Alveolar adenoma

8/3/2019 Alveolar adenoma

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1. Alveolar adenoma: biasanya pada perifer paru, terdiri dari ruang kistik kecil yangdibatasi oleh pneumosit tipe II dan berisi cairan.

2. Bronchial gland adenoma: termasuk oncocytoma dan adenoma kelenjar mucus.3. Papillary adenoma: tumbuh pada perifer paru, terbentuk dari pneumosit tipe II dan

sel clara.

4. Benign metastasizing leiomyoma : Jarang,


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- Disebut juga pulmonary chondroma- Tumor jinak yang terjadi pada orang dewasa.- Biasanya soliter, subpleural- Umumnya, muncul sebagai incidental coin lesion (lingkaran abnormal) dengan

pola popcorn dari gambaran Xray.

- Treatment: excision9. Multiple pulmonary leiomyomatous hamartoma:- Sangat jarang, hampir selalu pada wanita paruh baya dan asimtomatik.- DD : Reaktivasi proliferasi otot polos pada pneumonitis interstisial kronik, benign

metastasizing leiomyoma, leiomyosarcoma, lymphangioleiomyomatosi, proliferasi

otot pulmonal native.

10.Hemangioma- Biasanya pada anakanak- Endobronchial atau parenkim

11.Hemangiomatosis- Dapat muncul dengan gejala dari hipertensi pulmonal atau penyakit paru

interstitial.

- Prognosis buruk; dapat menjadi varian dari penyakit veno-occlusive.- DD: veno-occlusive disease

12.Inflammatory pseudotumor- Juga disebut inflammatory myofibroblastic tumor, plasma cell granuloma- Sering pada anak anak dan orang dewasa yang berusia 30 tahun serta

merupakan tumor paru yang paling sering pada anakanak usia 16 tahun dan

lebih muda.


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- Treatment: eksisi; jarang menyebabkan kematian karena local extension.- Faktor faktor diagnostik yang buruk: metastase, nekrosis >15% dari area

permukaan yang terkena, local recurrence, bizarre giant cells, > 3 tampilan

mikotik/50 HPF, tahap lanjut, high cellularity.

- DD: hemangiopericytoma, carcinoid tumor, plasmacytoma, amyloid tumor,metastatic carcinoma, TB in immunosuppressed patients, organizing

pneumonia, lipid pneumonia, benign and malignant fibrous histiocytoma,

other spindle cell tumors, mycobacterial pseudotumor

13.Inflammatory pseudotumor pada masa kanakkanak- Kebanyakan paling sering lesi paru yang terisolasi pada anak anak dan

asimtomatik.

- Jinak, walaupun beberapa kasus yang jarang telah melaporkan adanyakeganasan.

- Tatalaksana: eksisi atau raditerapi14.Langerhans cell histiocytosis- Also called eosinophilic granuloma, Langerhans cell granulomatosis, histiocytosis

X (H-X), Hand-Schuller-Christian disease, Letterer-Siwe disease

- Usually ages 20-39 years; strongly associated with smokers- 20% with multicentric disease (bone, skin, lymph nodes, spleen, pituitary) have

lung involvement

- 50% of cases only involve lung- Often associated with pneumothorax, Pneumocystis carinii pneumonia- Usually lung disease resolves or stabilizes, but 10-20% may progress to

respiratory failure

- DD: eosinophilic pleuritis (no Langerhans cells although mesothelial cells mayappear similar), reactive Langerhans cells in inflammatory conditions (no sheets

or groups of Langerhans cells), desquamative interstitial pneumonitis


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15.Leiomyoma- Rare, associated with HIV-infected children- DD: spindle cell carcinoma16.Lipoma- Usually endobronchial, usually men ages 50+

17.Lymphangiomyomatosis- Also called lymphangioleiomyomatosis- Rare, unknown etiology, may diffusely involve both lungs- Almost always in women, usually white and of reproductive age; rare cases in

men or postmenopausal women on hormone replacement

- Associated with tuberous sclerosis, renal angiomyolipomas- May involve mediastinal or periaortic lymph nodes- Derived from perivascular epithelioid cells (also angiomyolipomas, clear cell

[sugar] tumor of lung)

- Symptoms: dyspnea with pneumothorax or emphysema, without a smokinghistory

- Have severe impairment of diffusion with air trapping and expanding lungvolumes

- Disease is progressive, prognosis poor, death due to respiratory failure or corpulmonale

- Disease worsened by pregnancy or menstruation, improved post-menopause- Complications: respiratory insufficiency and death, spontaneous pneumothorax,

chylous pleural effusion; may be due to metastases or migration of progenitor

cells


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- Treatment: oophorectomy, hormone manipulation (progesterone or antiestrogens),lung transplantation (but may recur in lung allografts, Hum Path 2003;34:95)

- DD: metastatic endometrial sarcoma, benign metastasizing leiomyoma, idiopathicpulmonary hemosiderosis (all HMB45 negative), micronodular pneumocyte

hyperplasia

18.Micronodular pneumocyte hyperplasia- Associated with tubular sclerosis; may coexist with lymphangioleiomyomatosis- Usually women with shortness of breath- Considered a hamartoma- Not progressive- DD: lymphangioleiomyomatosis19.Paraganglioma- Rare in lung- Usually benign; rarely malignant- May be hyperplastic, not neoplastic- DD: carcinoid tumor (ribbons, festoons, rosettes)20.Sclerosing hemangioma- Also called sclerosing pneumocytoma- Usually adult women, ages 30-50 years, with incidental solitary nodule on chest

Xray

- Apparently derived from type II pneumocytes or with differentiation towardsthese cells

- Almost always benign, 2-4% have nodal metastases that dont appear to affectprognosis (Archives 2003;127:321)

- Sclerosis and hemorrhage are probably secondary changes


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- DD: primary and metastatic carcinoma, clear cell (sugar) tumor, carcinoidtumor, papillary adenoma, alveolar adenoma, epithelioid hemangioendothelioma,

Langerhans cell histiocytosis, meningiomas, meningothelial-like nodules

21.Solitary fibrous tumor- May be intrapulmonary and not pleural- Peaks at ages 50-69 years- Larger tumors associated with hypoglycemia, pleural effusion, pulmonary

osteoarthropathy (particularly if tumors 7cm+)

22.Squamous papilloma- Occur in large bronchi, often with associated tracheal or laryngeal lesions- Due to HPV- Often associated with dysplasia, carcinoma in situ or invasive squamous cell

carcinoma

- May contain mucus cells- DD: mucoepidermoid carcinoma (for papillomas with mucus cells)23.Dysplasia/carcinoma in situ

Dysplasia-general

- Usually associated with bronchial lesions; often present in uninvolved bronchusnear carcinoma

- No distinct criteria for dysplasia versus carcinoma in situ- Expert confirmation recommended before signing out case as carcinoma in situ24.Bronchioalveolar atypical adenomatous hyperplasia- Diagnostic variability exists - also called adenoma, well differentiated

bronchioalveolar carcinoma of Clara cell or type II pneumocyte type


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- Associated with coexisting bronchioloalveolar carcinoma and papillaryadenocarcinoma

- If malignant appearing but noninvasive, recommended to call bronchioloalveolarcarcinoma of nonmucinous type

Carcinoma

25.Carcinoma-general- 95% of lung tumors are bronchogenic carcinoma; also bronchial carcinoids,

mesenchymal, miscellaneous neoplasms

- In US, lung cancer is #1 cause of cancer death in men and women; causes 30% ofcancer deaths in men

- In 2002, 164,000 new cases in US; incidence in men is 74 per 100,000 vs. 31 per100,000 for women

- Peaks at ages 50-69 years; 2% occur before age 40- Young patients (age 40 or less) have higher incidence of 20q gains/amplifications

compared to older patients (56% vs. 8%), Mod Path 2002;15:372

- Cigarette smoking: causes most cases of lung cancer; relative risk of smokers vs.nonsmokers is 10:1; increases to 20:1 for >40 cigarettes/day; risk is strongly

related to number of cigarettes smoked, described in pack years (number of packs

per day x number of years smoking)

- Also associated with carcinomas of lip, tongue, pharynx, larynx, esophagus,bladder, pancreas, kidney, floor of mouth

- 10% of smokers have atypia or hyperplasia of bronchial epithelium- Carcinogens in tobacco smoke are benzo [a] pyrene (an initiator) and phenol

derivatives (promoters)

- Usually associated with squamous cell and small cell carcinoma, less likely withadenocarcinoma


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- Other causes: radiation exposure, uranium (RR with uranium exposure is 4:1 fornonsmokers, 10:1 for smokers vs. general population); asbestos (RR with asbestos

exposure is 5:1 for nonsmokers, 50-90:1 for smokers vs. general population),

exposure to nickel, chromate, coal, mustard gas, arsenic, beryllium, iron, vinyl

chloride, radon radiation, gold miners

- Causes of death for asbestos workers are: 20% lung cancer, 10% mesothelioma,10% GI carcinomas

- Symptoms: cough, weight loss, chest pain, shortness of breath, increased sputumproduction

- Systemic symptoms: Lambert-Eaton myasthenic syndrome (muscle weakness dueto antibodies to neuronal calcium channel), sensory peripheral neuropathy,

acanthosis nigricans, leukemoid reaction, hypertrophic pulmonary

osteoarthropathy (clubbing), superior vena cava syndrome (compression/invasion

of SVC causes venous congestion, circulatory compromise, dusky head, arm

edema), pain in distribution of ulnar nerve and Horners syndrome (enophthalmos,

ptosis, miosis, anhidrosis) due to apical lung tumors called Pancoast tumors

- Classification: broad classification is non-small cell carcinoma (80%) versus smallcell carcinoma (20%)

- 50% of non-small cell carcinomas are metastatic at diagnosis vs. 80% of small cellcarcinomas

- Many have mixed histologic subtypes- Scar cancers: scar is desmoplastic response to tumor- Spread: along bronchus distally and proximally, into lung parenchyma to

mediastinum or pleura, causing pleural seeding, pleural effusion, involvement of

diaphragm and chest wall

- Metastases: 50% have nodal involvement at resection (usually hilar, mediastinaland supraclavicular); also metastases to adrenals (50%), liver (30%), brain, bone;

also opposite lung, pericardium, kidneys


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- Treatment: complete excision for non-small cell lung carcinoma; radiation therapy(usually non-curative), chemotherapy (rarely curative, even for small cell

carcinoma)

- Survival: overall 5 year survival is 10-15%; only 30% have limited disease atdiagnosis making resection for cure an option

- For stage I non-small cell carcinoma, 5 year survival is 60%, poor prognosticfactors are p53+, HER2+, angiolymphatic invasion or tumor size > 3 cm, Hum

Path 2002;33:105

- Poor prognostic factors (clinical): high TNM stage, weight loss >10% of bodyweight, age < 40 years or women (usually are high stage), small cell or giant cell

subtypes, angiolymphatic invasion, pleural effusion, lack of lymphoplasmacytic

reaction; possibly ERCC1 genetic polymorphisms for non-small cell carcinoma

(Clin Lung Cancer 2009;10:118, Clin Cancer Res 2004;10:4939, Eur J

Cardiothorac Surg 2008;33:805)

- Favorable subtypes: non-mucinous bronchioloalveolar, well differentiatedsquamous cell

26.Acinic cell tumorBronchial gland tumor with low grade malignancy

- Very rare at this site, usually in parotid gland, less commonly in submandibularand sublingual glands

- May arise from pluripotent cells of tracheobronchial submucosal serous andmucus glands

- Treatment: surgical excision- DD: metastastic salivary gland tumor, primary lung adenocarcinoma, clear cell

(sugar) tumor of lung, oncocytic carcinoma, bronchial oncocytoma, bronchial

granular cell tumor, metastatic renal cell carcinoma

Adenocarcinoma

- Arises from terminal bronchioles


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- In US, 50% of lung carcinomas in women are adenocarcinoma; is the mostcommon subtype in nonsmokers

- 80% contain mucin- Grow slower than squamous cell; may be associated with scarring- 5 year survival: stage I - 69%, II - 40%, IIIA - 17%, IIIB - 5%, IV - 8%- Peripheral tumors with bronchioloalveolar and invasive areas < 5 mm had low

rates of vascular and pleural invasion and no nodal involvement, Am J Surg

Pathol 2003;27:937

- More likely TTF1 negative in males or smokers- DD: melanoma (may be mucin positive)References: Am J Surg Pathol 2003;27:150, Am J Surg Pathol 2002;26:767 (TTF1)

Sources for IHC testing: Clarient (Pulmotype testing)

Subtypes:

Bronchial surface cell type with little/no mucin

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8% of adenocarcinoma cases

Gross: cartilage bearing bronchus with polypoid growth or in distal airways

Micro: papillary or tubular groups of tall columnar cells resembling ciliated columnar

cells, but without cilia

EM: numerous mitochondria and smooth surfaced vesicles, basal bodies but usually

no cilia, no secretory granules


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Goblet cell type

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Gross: lobar pneumonia-like or nodular

Micro: tumor cells resemble goblet cells, with cytoplasmic mucin displacing the

nuclei to basal portion; usually in bronchioloalveolar pattern, rarely in papillary

pattern

Positive stains: lysozyme

Bronchial gland cell type

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Gross: cartilage bearing bronchus with polypoid growth or in distal airways as

nodular tumor

Micro: acini, tubules, ducts, cribriform patterns or solid nests of cuboidal or polygonal

cells, often with mucin; may have signet ring cells; resemble bronchial glands; nests

surrounded by myoepithelial type cells

Clara cell type

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Gross: pleura indented toward area of central fibrosis

Micro: papillary, tubular or bronchioloalveolar patterns of peg-shaped cells or low

columnar cells with tongue-shaped projections; fibrotic focus representing collapsed

alveoli may be present; variable nuclear atypia and mitotic activity; frequent

intranuclear eosinophilic inclusion bodies


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EM: microvilli on free cell surface of peg cells, scattered rough endoplasmic

reticulum, 200-900 nm round, dense, neurosecretory granules

DD: type II alveolar epithelial cell type

Type II alveolar epithelial cell type

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Gross: solitary nodular of rarely diffusely distributed tumor

Micro: papillary or bronchioloalveolar patterns of cuboidal to low columnar cells with

dome-shaped free cell border; finely vacuolated cytoplasm

EM: cytoplasmic lamellar inclusion bodies

Hepatoid

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Resembles hepatocellular carcinoma (HCC); more common in gastric

adenocarcinomas

Old age, high serum AFP, aggressive behavior, often with liver metastases

Micro: tubular and papillary adenocarcinoma with hepatic morphology; liver

metastases are difficult to distinguish from HCC

Positive stains: same as HCC - AFP, CK8, CK18, canalicular staining with polyclonalCEA

Positive stains: different from HCC - CK19, CK20, negative for HepPar1, negative

for CK7

Molecular: 4q-, 8p-, Xq+ (seen in hepatocellular carcinoma and hepatoblastoma)

References: Am J Surg Pathol 2003;27:1302


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Adenocarcinoma of fetal lung type

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Rare, high grade variant is very aggressive

May be a variant of pulmonary blastoma without malignant mesenchymal

components

Associated with upregulation of Wnt signaling pathway, Mod Path 2002;15:617

Micro: irregular tubular structures of columnar epithelial cells with clear cytoplasm

and oval nuclei, optically clear nuclei rich in biotin; resembles fetal lung in

pseudoglandular stage; fibrous stroma without atypia; no morules

Micro images: high grade

DD: well-differentiated fetal adenocarcinoma (has morules)

Adenocarcinoma - well differentiated fetal type

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Mean ~40 years

Better prognosis than pulmonary blastoma

Variant of bronchioloalveolar carcinoma

Associated with upregulation of Wnt signaling pathway, Mod Path 2002;15:617

Gross: well-defined, non-encapsulated, not related to visible bronchi

Micro: irregular tubular structures of columnar epithelial cells with clear cytoplasm

and oval nuclei, continuous with morular structures; nuclei in morules are optically

clear and rich in biotin; fibrous stroma without atypia

Micro images: image1

Positive stains: chromogranin A, synaptophysin, N-CAM


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Negative stains: p53

DD: adenocarcinoma of fetal type (no morules)

Adenocarcinoma vs. mesothelioma

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Difficult to differentiate histologically if adenocarcinoma does not produce mucin

Stains recommended to differentiate these 2 tumors: calretinin, cytokeratin 5/6 or

WT1 and CEA, MOC31 (or B72.3, Ber-EP4 or BG8), Am J Surg Pathol

2003;27:1031

Stains that are specific for adenocarcinoma vs. mesothelioma: MOC31 (100% vs.

8%), Ber-EP4 (100% vs. 18%), BG8 (96% vs. 7%), CEA (88% vs. 0%), B72.3 (84%

vs. 0%), TTF1 (74% vs. 0%), CD15/LeuM1 (72% vs. 0%)

Stains that are specific for mesothelioma vs. adenocarcinoma: calretinin (100% vs.

8%), cytokeratin 5/6 (100% vs. 2%), WT1 (93% vs. 0%), thrombomodulin (77% vs.

14%), N-cadherin (73% vs. ?)

Stains frequently positive in both tumors: EMA, E-cadherin, HBME, CD44S,

mesothelin, vimentin

EM: long, slender microvilli in mesothelioma

Adenoid cystic carcinoma

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May arise from submucus bronchial glands

Usually in large bronchi, may involve the trachea

Frequent metastases to regional lymph nodes and lung parenchyma

Prolonged course, but overall prognosis is poor


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Treatment: radiation therapy (palliative)

Gross: large, polypoid, intrabronchial mass or may grow subepithelially along the

bronchi causing thickened bronchial wall

DD: basaloid carcinoma with adenoid cystic carcinoma-like pattern (microcystic

spaces containing mucin, surrounded by small tumor cells)

Adenosquamous carcinoma

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Substantial amounts of squamous and glandular differentiation

Dont diagnose if one component is clearly minor


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More favorable prognosis: non-mucinous histology (hobnail cells, Clara cells),

localized disease

This diagnosis is usually limited to non-invasive tumors with lepidic spread (along

alveolar septa); if stromal, vascular or pleural invasion is seen, tumor is classified as

adenocarcinoma, mixed subtype

Gross: peripheral lung, one or more nodules (nodules may coalesce) or pneumonia-

like infiltrate

Gross images: multifocal tumor #1, #2

Micro: tall, columnar cells line up along alveolar septa, project into spaces with

papillary projections, but underlying lung architecture is preserved; variable anaplasia

but usually well differentiated

Composed of mucus-producing goblet cells, Clara cells or type II alveolar epithelial

cells

Mucinous type - composed of well differentiated columnar cells containing mucin

that line respiratory spaces; tumor cells are associated with bronchioles, not bronchi;

usually sharp demarcation between normal and tumor cells

Non-mucinous type - cuboidal cells with bright eosinophilic cytoplasm, prominent

nucleoli and nuclear atypia; apical spouts and hobnail cells often present; cilia are

rare; also PAS+ intranuclear inclusions; associated with interstitial fibrosis (may be

severe) and chronic inflammatory infiltrate; 13% have psammoma bodies

Micro images: noninvasive, nonmucinous (H&E, TTF1, CK7, CK20), mucinous

(H&E, TTF1, CK7, CK20), mixed mucinous and nonmucinous,

Virtual slides: tumor & nodal metastasis

Positive stains: alpha-1-antitrypsin for Clara cells in non-mucinous type, surfactant

for type 2 pneumocytes in non-mucinous type, TTF1 (80% of non-mucinous, 0% of

mucinous), CK7 (94-100%)

Negative stains: CK20 (but 25-90% of mucinous are CK20+)


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EM: mucinous - resemble bronchiolar goblet cells; non-mucinous - resemble Clara

cells or type 2 pneumocytes

DD: metastatic mucinous carcinoma

References: Mod Path 2002;15:538 (TTF1, CK7, CK20), Hum Path 2002;33:915

(CK7, CK20)

DD: metastatic adenocarcinoma

Epithelial-myoepithelial carcinoma

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Arises from submucosal bronchial glands, mimics the similar salivary gland tumor

Very rare, < 10 cases reported through April 2003

Ages 40-69 years

Low grade malignancy; long interval to recurrence or metastasis

Case report of 73 year old man with bronchial tumor, Archives 2003;127:e177

Gross: intraluminal polypoid mass in bronchus; may invade pulmonary parenchyma

Gross/micro images: image1

Micro: well circumscribed mass, pushing margin; tumor consists of ductlike

structures with inner epithelial cells and outer clear myoepithelial cells; no

myxoid/chondroid stroma, no perineural invasion

Positive stains: epithelial cells - cytokeratin, EMA; myoepithelial cells - S100, muscle

specific actin

Negative stains: HMB45

DD: mucoepidermoid carcinoma, acinic cell carcinoma, pleomorphic adenoma,

adenoid cystic carcinoma with a tubular pattern, myoepithelioma, myoepithelial


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carcinoma, clear cell (sugar) tumor, metastatic epithelial-myoepithelial carcinoma

(usually parenchymal, not endobronchial), metastatic clear cell carcinoma

Giant cell carcinoma

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Included in WHO classification of carcinomas with pleomorphic, sarcomatoid, or

sarcomatous elements


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Gross: usually peripheral lung, spherical tumor with well-defined borders and

bulging, fleshy, homogenous gray-white cut surface; no anthracosis; frequently

involves thoracic wall

Micro: large polygonal cells and anaplastic cells growing in solid nests without

obvious squamous or glandular differentiation; vesicular nuclei, prominent nucleoli,

moderately abundant cytoplasm, well defined cell borders

Virtual slides: anaplastic tumor cells

Positive stains: keratin5 (56%), calretinin (38%), thrombomodulin (25%), mesothelin

(13%), TTF1 (variable reports of staining)

EM: glandular (intracellular and extracellular lumina) and squamous (desmosomes,

tonofilaments) features often present although not obvious with H & E staining

Large cell neuroendocrine carcinoma

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Diagnostic criteria (Rosai): resembles a non-small cell carcinoma, but on closer

inspection has a hint of neuroendocrine architecture confirmed by special stains

Micro: larger tumor cells than atypical carcinoid, high nuclear grade, increased

mitotic activity (11+ per 10 HPF) and necrosis; poorly developed neuroendocrine

architecture with some pallisading or rosette-like structures present

Positive stains: neuroendocrine markers, CD117 (60%), TTF1 (50%)

Lymphoepithelioma-like carcinoma

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Rare, ~ 100 cases reported

In Taiwan study, most patients were women, Chinese, non-smokers, Am J Surg

Pathol 2002;26:715


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Case report of EBV+ tumor in 25 year old Italian man, Hum Path 2003;34:623

Otherwise rare in Caucasians, and usually EBV-

Tumor size may correlate with EBV serology titer

May have better prognosis than other nonsmall cell carcinomas of lung

Gross: well circumscribed nodules

Micro: syncytial growth of epithelial cells with large vesicular nuclei, prominent

eosinophilic nucleoli, accompanied by marked CD8+ lymphocytic infiltration;

predominantly pushing borders, permeative interface with adjacent lung; occasional

amyloid deposition

Positive stains: EBV, EBER-1, LMP (EBV latent membrane protein), bcl2, patchy

keratin

Negative stains: CD45/LCA (in tumor cells)

DD: metastatic nasopharyngeal carcinoma, lymphoma, inflammatory pseudotumor

Metastastic tumors to lung

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Lung is a common site of metastases

Usually multiple, bilateral, sharply outlined, rapidly growing, more pleomorphic and

necrotic than lung primaries, negative for TTF1

Often multiple discrete nodules in periphery of lung; also lymphangitis carcinomatosa

(peribronchial and perivascular patterns via lymphatics); rarely intralymphatic

microscopic foci that may cause pulmonary hypertension

Treatment: excision of isolated nodules

Nodular metastases: breast, GI tract, kidney, sarcoma, melanoma


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Lymphangitis carcinomatosis: stomach, breast, choriocarcinoma, pancreas, prostate;

case report with hepatocellular carcinoma, Archives 2003;127:e11

Central cavitation: squamous cell carcinoma of upper aerodigestive tract, colonic

adenocarcinoma, leiomyosarcoma

Intrabronchial masses: breast, kidney, colon

Tumor emboli: breast, stomach, liver, choriocarcinoma

Lepidic pattern: colon, pancreas

Gross images: multiple tumor nodules #1, #2

Gross/micro images: hepatocellular carcinoma

Virtual slides: metastatic adenocarcinoma, metastatic small cell carcinoma

Positive stains: CDX2 (colorectal carcinoma, Am J Surg Pathol 2003;27:141)

Negative stains: surfactant apoprotein

Metastatic endometrial stromal sarcoma

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Metastases to lung are rare, reports at Am J Surg Pathol 2002;26:440, Am J Surg

Pathol 2002;26:1142

Metastases occur a mean 10 years after diagnosis of uterine endometrial stromal

sarcoma

Excellent prognosis; patients only rarely die of disease

Note: uterine diagnosis often not disclosed to pathologist

Micro: well circumscribed, solid tumor composed of plump spindle cells in short

fascicles, often with hyalinized areas; may have cystic or sex cord-like areas


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Positive stains: vimentin, ER, PR, CD10; variable smooth muscle actin, desmin and

keratin

Negative stains: chromogranin, HMB45

DD: sclerosing hemangioma, carcinoid tumor, lymphangioleiomyomatosis,

endometriosis, hemangiopericytoma, lymphoma

Micropapillary adenocarcinoma

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Associated with nodal metastases of similar histology, intrapulmonary metastases,

non-smokers

Poorer survival than non-papillary for stage I (79% vs. 93%), Am J Surg Pathol

2003;27:101, Am J Surg Pathol 2002;26:358

Case report of incidental brain metastases with micropapillary structures, Archives

2003;127:e313

Micro: small papillary tufts without a fibrovascular core; associated with varied other

histologic subtypes

Micro images: papillary and micropapillary structures

Positive stains: CK7 (93%), TTF1 (80%), CK20 (13%)

Mucoepidermoid carcinoma

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May arise from submucus bronchial glands

Usually in large bronchi

May occur in children


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Usually considered to have low malignant potential with recurrence but only rarely

aggressive

Excellent prognosis after surgical removal

Gross: polypoid growth in major bronchi

Micro: low grade or high grade; mucus secreting cells, squamous cells, intermediate

type cells

DD: adenosquamous carcinoma arising from bronchial epithelium

Papillary carcinoma

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Includes goblet cell (mucin producing) type, Clara cell type, type II pneumocyte type

and bronchial surface epithelial cell type with or without mucin production

Micro: papillary structures with true fibrovascular core should comprise at least 75%

of tumor

Pleomorphic carcinoma

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Also called sarcomatoid carcinoma, spindle cell carcinoma, giant cell carcinoma,

pseudosarcoma, pulmonary blastoma, and carcinosarcoma

Included under recent WHO classification of carcinomas with pleomorphic,

sarcomatoid, or sarcomatous elements

90% men, 92% smokers

Diagnosis is based on histology, not immunostains


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Presumed epithelial origin, although epithelial and sarcomatous components express

common markers differently, Am J Surg Pathol 2003;27:1203

Nodal metastases common

Stage 1 tumors have same prognosis as other stage 1 non-small cell carcinomas;

pleomorphic tumors at higher stages may have worse prognosis by stage than other

non-small cell carcinomas, Am J Surg Pathol 2003;27:311

Giant cell tumors frequently metastasize to small intestine

Carcinosarcoma: carcinoma component is combined with a sarcoma, the latter

consisting of heterologous elements, such as malignant cartilage, bone, or muscle;

both components appear to have a common origin, Am J Surg Pathol 2002;26:510

Gross: 2-17 cm, necrosis and hemorrhage common

Micro: non small cell lung carcinoma with at least 10% neoplastic spindle or giant

cells, usually with epithelial cells; epithelial component 10-85%, usually

adenocarcinoma or large cell carcinoma, also squamous cell carcinoma; usually

poorly differentiated; spindle cells resemble MPNST, MFH or fibrosarcoma; giant

cells usually bizarre with multilobulated nuclei, abundant eosinophilic cytoplasm

accompanied by heavy neutrophilic infiltrate with occasional ingested white blood

cells; stroma often myxoid, frequent inflammatory infiltrate, collagen fibers;

numerous mitotic figures; necrosis common; vascular invasion in 58%

Positive stains: sarcomatoid component - CK7 (63%), TTF1 (43%), surfactant protein

A (6%); epithelial component - CK7 (76%), TTF1(59%), surfactant protein A (39%)

Negative stains: CK20

Molecular: extensive allelic loss in carcinosarcomas at 3p, 5q, 17p

Pulmonary blastoma

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Also called embryoma


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Considered a subtype of pleomorphic carcinoma / carcinosarcoma

Usually in adults (mean age 43 years), 20% less than 20 years old

Metastases common; 2/3 die within 2 years

In infants/children, epithelial component is benign appearing or minimal; stroma may

be rhabdomyoblastic or chondroid; tumors may be cystic and may involve pleura and

lung

Case report of 16 year old white boy, Archives 2002;126:875

Gross: peripheral, solitary, well circumscribed, large

Micro: biphasic tumor in which epithelial and mesenchymal components have a

primitive, fetal-type appearance; well formed tubular glands surrounded by cellular

stroma of embryonal appearance; resembles Wilms tumor and fetal lung at 10-16

weeks; glandular cells are tall, columnar, often with clear cytoplasm and subnuclear

and supranuclear cytoplasmic vacuoles; morules with ground-glass nuclei are

common; stroma may differentiate towards striated muscle, smooth muscle, cartilage

Micro images: biphasic tumor

Positive stains: PAS (glycogen in epithelial cells)

Sebaceous carcinoma

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Case report of 78 year old man with slow growing, endobronchial tumor, Am J Surg

Pathol 2002;26:795

Micro: sebaceous differentiation with lobulated and infolded architecture, light and

dark zones composed of basaloid cells with sharp cytoplasmic borders, vesicular

nuclei and cytoplasm varying from scant to abundant with small vesicles

Positive stains: oil red O (lipid)

Negative stains: PAS-diastase, mucicarmine


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DD: metastatic tumor from head and neck, mucoepidermoid carcinoma, squamous

cell carcinoma with sebaceous differentiation

Small cell carcinoma

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Also called undifferentiated carcinoma, oat cell carcinoma

10-20% all of lung carcinoma, 45,000 new cases per year in US

Usually men, median age 60 years, 99% occur in smokers, very aggressive with early

mediastinal lymph node involvement

Apparently derived from primitive cells of basal bronchial epithelium with partial

differentiation towards neuroendocrine cells

Associated with paraneoplastic syndromes due to production of ADH (hyponatremia),

ACTH (Cushings syndrome), parathyroid hormone (hyperparathyroidism), calcitonin

(hypocalcemia), gonadotropins (gynecomastia), serotonin (carcinoid syndrome),

encephalomyelitis, sensory neuropathy, Lambert-Eaton syndrome

Diagnosis is based on H & E staining, not the presence of neuroendocrine markers

Some pathologists report the presence of a large cell component or classify as

undifferentiated or squamous cell carcinoma of small cell type if small tumor cells

with hyperchromatic, coarsely granular or vesicular nuclei, small but distinct nucleoli,

scanty but identifiable cytoplasm, distinct cell border, primarily negative for

neuroendocrine stains

5 year survival (n=55): stage I - 33%, II - 44%, IIIA - 22%, IIIB - 0%, IV - 12%

Biopsies often crushed; cytology may be helpful

Flow cytometry: CD56+ (neural cell adhesion molecule), CD45-, which differentiates

from lymphoma, Archives 2003;127:461

Treatment: chemotherapy, radiation; cure rates of 15-25% for limited disease; most

live 1 year; preoperative chemotherapy and surgery if T1-2, N0-1, M0


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Poor prognostic factors: elevated serum LDH, alkaline phosphatase, albumin,

hemoglobin, white blood count

Gross: usually central/hilar; white-tan, soft, friable, extensive necrosis; peripheral

nodules have fairly well-defined border and fleshy cut surface

Gross images: central tumor #1, #2, spreading along bronchi

Micro: sheets, ribbons, clusters, rosettes or peripheral pallisading of small to medium

sized (2-4x neutrophils) round/oval cells with minimal cytoplasm, salt and pepper

chromatin without prominent clumps, hyperchromatic, indistinct nucleoli, nuclear

molding, smudging, frequent mitotic figures; Azzopardi phenomena (basophilic

nuclear chromatin spreading to wall of blood vessels), indistinct cell borders; stroma

is scanty, vascular, delicate; no glands, replacement of epithelium is less common

than subepithelial growth; necrosis and apoptotic debris are common

More cytoplasm is present in cells in metastases or resections than in small biopsies

May have larger cells with similar morphology, small mixtures of squamous cell

carcinoma or adenocarcinoma; rarely scattered giant cells, prominent nucleoli

Micro images: flow, H&E, stains (not necessarily lung), classic; H&E, CD117

Virtual slides: small cell carcinoma, primary tumor, bone marrow metastasis, liver

metastasis

Positive stains: pan-keratin (100%, dot like pattern), CK-BB (91%), TTF1 (89%),

histamine decarboxylase (78%, Mod Path 2003;16:72), neuron specific enolase

(77%), CD117 (75%, 50% after chemotherapy), chromogranin (58%, may be weak),

synaptophysin (57%), calretinin (49%), thrombomodulin (27%), keratin5 (27%),

CD57/Leu7 (variable), gastrin releasing peptide, N-CAM/CD56, bcl-2 (variable)

Negative stains: CD3, CD20, CD45, CD99/MIC2, pancreatic polypeptide, vimentin,

mesothelin, p63

EM: occasional round, membrane bound, dense core neurosecretory granules, 100-

200 nm in diameter; may have bundles of tonofibrils; may form glandular spaces


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DD: atypical carcinoid tumor (less nuclear atypia,


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Central cases appear to arise from bronchial epithelial dysplasia; peripheral cases

usually lack dysplasia

Peripheral cases with alveolar space filling pattern have better prognosis, no

lymphatic invasion or nodal metastases, Am J Surg Pathol 2003;27:978

Symptoms: bronchial obstruction (pneumonitis, atelectasis)

Cytology: often positive in sputum

5 year survival after resection: stage I-64%, stage II-48%, stage IIIA-28%, stage IIIB-

6%, stage IV-0%

Gross: usually central portion of lung affecting larger bronchi but may be peripheral;invades peribronchial soft tissue, lung parenchyma and nearby lymph nodes; may

compress pulmonary artery and vein; peripheral tumors often have nodular growth

with central necrosis and cavitation; surrounding lung may exhibit lipid pneumonia,

bronchopneumonia, atelectasis; calcification is unusual

Gross images: cavitating tumor #1, #2, tumor obstructing bronchus; endobronchial

tumor, tumor extending to pleura

Micro: sheets or islands of large polygonal malignant cells containing keratin

(individual cells or keratin pearls) and intercellular bridges; adjacent bronchial

dysplasia or carcinoma in situ is common; at advancing tumor border, tumor cells

usually destroy alveoli or fill alveolar spaces; rarely spread beneath basement

membrane; may have focal areas of intracytoplasmic mucin; rarely oncocytes, foreign

body giant cells (reacting to keratin), pallisading granulomas, extensive neutrophilic

infiltration, lepidic growth pattern at tumor periphery, clear cell change (glycogen);

Classify as well, moderately or poorly differentiated based on amount of

keratinization present in predominant component

Peripheral tumor types: alveolar space filling (tumor cells fill alveoli but dont destroy

elastic septa), expanding type (growth destroys elastic septa) or mixture

Variants: small cell (small tumor cells with focal keratinization, distinct nucleoli,

sharply outlined tumor nests, less necrosis than small cell carcinoma), clear cell


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(numerous clear tumor cells containing glycogen), well-differentiated papillary,

basaloid (see below), spindle cell (see below)

Micro images: moderately differentiated, well differentiated, with keratin pearls

Micro images (Mod Path subscribes): cytology

Virtual slides: bronchial tumor, esophageal metastasis; keratinizing-moderately

differentiated

Positive stains: keratin, keratin5 (87-100%), EMA, thrombomodulin (87-100%),

S100, CD15, CEA, p53, p63, HPV, mesothelin (16-31%)

Negative stains: vimentin (usually), TTF1 (usually)

EM: abundant tonofilaments, complex desmosomes, basal lamina

DD: squamous metaplasia with atypia (Hum Path 2002;33:1052)

References: Hum Path 2002;33:921 (p63)

Sources for IHC testing: Clarient (Pulmotype testing)

Early lung carcinoma of hilar type

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Arises proximal to sub segmental bronchi (i.e. major bronchi), confined to bronchial

wall with no lymph node metastases

Are usually squamous cell carcinomas

May be polypoid, nodular, superficially infiltrating or mixed

Longitudinal mucosal folds show changes at tumor border; superficial tumor has

thickened and fused folds

Five year survival is 90% or more if no second squamous cell carcinoma present


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Early squamous cell carcinoma of peripheral type

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Defined as tumor 2 cm or less in peripheral lung with no lymph node or distal

metastases

Only rarely identified in practice, since these tumors grow rapidly

Often have glandular cell characteristics

Basaloid squamous cell carcinoma

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Very aggressive clinical course

Spindle cell squamous cell carcinoma

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Also called sarcomatoid carcinoma

Carcinoid and related tumors

Carcinoid tumor

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Also called well-differentiated neuroendocrine carcinoma


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Occasionally occurs as part of MEN syndrome

May infiltrate or spread to local lymph nodes, but doesnt affect prognosis

Rarely produces carcinoid syndrome (flushing, diarrhea, cyanosis)

10 year survival is 50%

In children, involve lung or liver, may metastasize regardless of histology or

classification as carcinoid vs. neuroendocrine carcinoma, Archives 2003;127:1200

Gross: either central (polypoid and endobronchial in major bronchi) or peripheral

(solid/nodular); usually well defined, smooth, ivory to pink cut surface, no necrosis

Micro: nests or trabeculae of medium sized polygonal cells of low nuclear grade,

round to oval finely granular nuclei and lightly eosinophilic cytoplasm, may have

rosettes or small acinar structures with variable mucin; scanty vascular stroma,

occasionally amyloid stroma with bone; no/minimal mitotic activity or necrosis

Micro images: B-carcinoid, A-carcinoid

Positive stains: TTF-1 (43-53%, Appl Immunohistochem Mol Morphol 2007;15:407,

AJCP 2005;123:394)

Central carcinoid tumor

Most common type, usually slow growing, associated with obstruction, infection,

hemorrhage

Usually adults, but also most common lung tumor of children

5% metastasize, usually to regional lymph nodes; rarely distant osteoblastic

metastases to bone

10 year survival 70%

Cytology often negative since tumor is covered by mucosa

Treatment: surgical resection


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Gross: solitary, intrabronchial polypoid mass that may infiltrate bronchial wall,

covered by intact mucosa; gray-yellow cut surface, cartilage may be present

Gross images: bronchial carcinoid

Micro: nests or cords of uniform, bland cells with central nuclei and moderate

granular cytoplasm, prominent vasculature, stroma may be delicate fibrovascular,

hyalinized or exhibit calcification; angiolymphatic invasion common; rarely mitotic

figures, rosettes or papillary architecture, endocrine atypia, melanin granules

May have paraganglioma appearance with S100+ sustentacular cells

May have oncocytic features (abundant granular eosinophilic cytoplasm with

numerous mitochondria by EM)

Virtual slides: bronchial carcinoid

Positive stains: keratin, serotonin, neuron-specific enolase, chromogranin A and B,

synaptophysin, CD57/Leu7, pancreatic polypeptide, N-CAM

Negative stains: mucin (except in glandular lumina), TTF1 (usually, Hum Path

2004;35:825)

Molecular: 1/3 are aneuploid, which doesnt appear to affect prognosis

EM: dense core secretory granules that vary in shape and size

DD: small cell carcinoma (if extensively crushed)

Peripheral carcinoid tumor

Arise in peripheral lung, often beneath the pleura

Usually asymptomatic and incidental

Excellent prognosis

Rare nodal metastases are usually cured by excision

Treatment: lobectomy (since multiple tumors are common)


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Gross: multiple, nonencapsulated, gray-tan nodules, bulging, brown-tan cut surface,

not associated with a bronchus

Micro: disorderly spindle cells resembling smooth muscle; moderate pleomorphism

and mitotic activity; prominent stroma; amyloid and melanin often present

Positive stains: Congo Red (amyloid), TTF-1 (usually), calcitonin (often)

Atypical carcinoid tumor

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Also called moderately differentiated neuroendocrine carcinoma

Diagnostic criteria (Rosai): resembles a carcinoid but with atypical features

More aggressive than typical carcinoid tumors: nodal metastases in 70% vs. 5%

5 year survival is 49-69%

Micro: carcinoid tumors with increased mitotic activity (2-10 per 10 HPF), nuclear

pleomorphism or foci of necrosis

Positive stains: more intense neuroendocrine staining than small cell carcinoma; also

positive for pancreatic polypeptide

EM: numerous large neurosecretory granules

Tumorlet

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Nodular proliferation of small spindle cells near bronchioles

May be associated with bronchiectasis or other causes of scarring

Almost always benign

By definition 5 mm or less


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Positive stains: same as carcinoid

EM: same as carcinoid

Lymphoma and lymphoid infiltrates

BALT lymphoma

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Lymphoma of Bronchus Associated Lymphoid Tissue

Rare type of low-grade, primary pulmonary MALT lymphoma

Often need gene rearrangement studies to diagnose

Indolent with good prognosis

Case report of 41 year old woman with clonality by gene rearrangement but not by

flow cytometry, Archives 2003;127:115

Micro: centrocyte-like lymphocytic proliferation in bronchiolar submucosa with

lymphoepithelial lesions, reactive lymphocytes and plasma cells; bland appearance

Micro images: H&E with lymphoepithelial lesions

DD: reactive hyperplasia

Burkitts lymphoma

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Case report of 33 year old man with cystic fibrosis and Burkitts lymphoma after

double lung transplant, Am J Surg Pathol 2003;27:818

Diffuse large B cell lymphoma


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top

Presents as large parenchymal mass accompanied by necrosis, occasionally by

intrabronchial mass

May evolve from MALT lymphoma or arise without preexisting lesions

5 year survival is 50%

Micro: pleomorphic large lymphoid cells, usually noncleaved

Positive stains: CD20

Negative stains: CD3, CD5, CD26

Follicular bronchitis / bronchiolitis

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Associated with immunodeficiency (congenital, AIDS), rheumatoid arthritis and

Sjogrens syndrome

Mild shortness of breath

Treatment: steroids, uncertain if chemotherapy if helpful

Micro: lymphoid follicles and plasma cells around distal bronchi and bronchioles that

infiltrate fibromuscular wall and may compress the lumen; alveoli relatively

unaffected

Hodgkins lymphoma

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Very rare (


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Case report of localized pulmonary consolidation in 21 year old woman, Archives

2003;127:e49

Usually good prognosis, but fatal cases occur

Gross: nodular or multinodular lesions in upper lung

Micro: typical Reed-Sternberg cells, mononuclear cells, lacunar variants in

background of mixed inflammatory infiltrate; rarely granulomatous inflammation

simulating tuberculosis

Micro images: 3A: CD15; 3B: CD30

Positive stains: CD15, CD30

Negative stains (Reed-Sternberg like cells): CD45, CD20, CD3, EMA

DD: tuberculosis, Wegeners granulomatosis, organizing pneumonia, T cell

lymphoma, anaplastic lymphoma

Lymphomatoid granulomatosis

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Also called angioimmunoproliferative lesion

Lymphoproliferative disorder that is malignant per se or likely to become malignant,

resembles post-transplant lymphoproliferative disorders

Middle aged men and women with well defined, bilateral rounded mass densities on

chest Xray

Associated with transplant-related immunosuppression, Sjogrens syndrome, HIV

80% of cases have extrapulmonary involvement (skin, CNS, kidneys, liver, spleen,

adrenal glands, heart, GI tract)

Rarely, there is only extrapulmonary involvement

Usually no hilar or mediastinal lymph node involvement


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May be an EBV related, B cell proliferation; T cells present are usually reactive

May have reversal of T helper / suppressor ratio

Large number of atypical lymphoid cells is a poor prognostic factor

Median survival 14 months; death due to sepsis, destruction of lung tissue; similar

infiltrates found in other organs

Treatment: chemotherapy

Micro: nodular inflammatory infiltrate of large atypical lymphoid cells (prominent

nuclei, mitotic activity), plasma cells, immunoblasts, involving walls of pulmonary

vessels (angioinvasive, angiocentric, angiodestructive); no multinucleated giant cells

Positive stains: EBV (50-70%)

DD: Wegeners granulomatosis (giant cells, necrosis)

MALT lymphoma

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Lymphoma of Mucosal Associated Lymphoid Tissue - a type of marginal zone

lymphoma

Also called lymphoma of bronchial associated lymphoid tissue (BALT)

Called pseudolymphoma in older literature

Etiology: in pediatric HIV patients with lymphoid interstitial pneumonitis,chemokines and cytokines may recruit inflammatory cells, either representing an early

stage of MALT or providing a microenvironment for the evolution of a monoclonal

B-cell population (Mod Path 2001;14:929)

In adults, lung MALT is usually low-grade, median age 68 years (range 34-88), often

associated with autoimmune disorders, monoclonal gammopathies, hepatitis C,

Helicobacter pylori gastritis; 44% involve mediastinal nodes


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Usually indolent with excellent prognosis; may recur locally, rarely transforms, few

die of disease

20% have monoclonal gammopathy, 30% have pleural effusions

Flow cytometry of tumor cells is useful in phenotyping; may even be helpful on

peripheral blood

Case reports: 41 year old woman with multiple lung nodules, negative flow cytometry

but clonal IgH gene rearrangement (Arch Pathol Lab Med 2003;127:115)

Treatment: local resection for limited disease, chemoradiotherapy for advanced

disease

Gross: solitary discrete mass, occasionally multiple nodules

Gross images: contributed by anonymous - discrete tan mass #1; #2; #3

Micro: nodular pattern of monotonous, mature lymphocytes with germinal centers

that infiltrate overlying epithelium (lymphoepithelial lesions) and around vessels,

pleura and alveolar septa; cells may be monocytoid or resemble centrocytes; also

reactive plasma cells, variable fibrosis and epithelioid granulomas; also colonization

of germinal centers by tumor cells; rarely granulomatous vasculitis

Micro images: contributed by anonymous - monotonous population of small-

intermediate cells; lymphoepithelial lesion

Figure 1-CT with multiple pulmonary nodules; 2A-lymphocytic proliferations with

some germinal centers; 2B-lymphoepithelial lesions associated with bronchial mucosa

Positive stains: CD20, CD43

Negative stains: CD5, CD10, CD23

DD: benign lymphoid aggregates / reactive disorder: architecture is preserved,

associated with immunosuppression and collagen vascular disease; not monoclonal by

flow cytometry or PCR

References: Am J Surg Pathol 2001;25:997, Am J Surg Pathol 2002;26:76


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Nasal type NK-T cell lymphoma

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Case report of orbital tumor with lung metastases, Hum Path 2003;34:290

Plasmacytoma

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Associated with nodal or bone involvement and myeloma

Micro: mature and atypical plasma cells

SLL/CLL

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Small lymphoblastic lymphoma / chronic lymphocytic leukemia

Usually asymptomatic pulmonary nodules or infiltrates in elderly

Involves the lung at autopsy in 30% of cases, may cause significant pulmonary

impairment

Gross: well defined, encapsulated mass, homogenous gray cut surface

Micro: monomorphic infiltrate with pseudofollicles

DD: reactive lymphoid infiltrate (polymorphic lymphocytes, germinal centers, otherinflammatory cells)

Waldenstrm macroglobulinemia

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B cell neoplasm (usually lymphoplasmacytic lymphoma) plus serum IgM monoclonal

paraprotein


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Usually elderly patients with indolent clinical course

Median survival 5 years

Involves bone marrow (100%), liver, spleen, lymph nodes (15-20%); also

hyperviscosity syndrome in 15%

Lung an unusual site, Am J Surg Pathol 2003;27:1104.

Diagnosis: serum protein electrophoresis

Other malignancies

Angiosarcoma

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Presents as mass or diffuse pulmonary infiltrates

DD: metastatic tumor

Desmoplastic small round cell tumor

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Case report in 22 year old man, Archives 2002;126:1226

Micro: nests of small round tumor cells within a cellular and vascular collagenous

stroma

Micro images: H&E, stains

Positive stains: AE1/AE3, EMA, CAM5.2, vimentin, desmin, neuron-specific

enolase, WT1

Negative stains: thrombomodulin, calretinin

Molecular: t(11;22)(p13;q12) [EWS-WT1 gene fusion]

Molecular images: RT-PCR


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EM: intracytoplasmic whorls of intermediate filaments

EM images: cytoplasmic perinuclear aggregates of intermediate filaments

Epithelioid hemangioendothelioma

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Formerly called intravascular bronchioloalveolar tumor

80% women, usually young adults

Neoplastic, but usually not metastatic

Progressive growth, usually remains within thoracic cavity, may cause death from

respiratory insufficiency

Other sites: liver, bone

10% have peripheral eosinophilia

Poor prognosis if vascular spread, pleural involvement, severe symptoms

Case history: Archives 2003;127:e319

Gross: multiple round, well demarcated nodules < 2 cm, often in lower lung, with a

gray-white peripheral rim; may spread along pleura or pericardium and resemble

mesothelioma

Micro: central hyalinized stroma, eosinophilic amorphous material or coagulative

necrosis with variable calcification surrounded by thin rim of plump eosinophilicendothelial cells; clusters fill alveoli, apparently through pores of Kohn, and

occasionally bronchioles, arteries, veins; nuclei are bland, round/oval, may have

cytoplasmic vacuoles; no/minimal mitoses; lung architecture preserved

Gross/micro images: H&E, D-Factor VIII

Positive stains: Factor VIII, CD31, CD34; variable ER and PR


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EM: endothelial features - well developed basal lamina, pinocytotic vesicles,

occasional Weibel-Palade bodies

DD: metastatic tumor from liver or other sites (destroy lung architecture, high mitotic

rate), sclerosing hemangioma (destroys lung architecture, negative vascular markers)

Hemangiopericytoma

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Rare, often a misdiagnosis

Micro: blunt spindle cells with scant cytoplasm, separated by arborizing vascular

spaces arranged in a stag-horn pattern

DD: metastatic endometrial stromal sarcoma

Histiocytic-dendritic neoplasm

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Case report (letter), Am J Surg Pathol 2002;26:1373

HIV associated polymorphic lymphoproliferative disorders

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Resemble posttransplant lymphoproliferative disorders in solid organ transplant

recipients, Am J Surg Pathol 2003;27:293

Kaposis sarcoma

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Usually associated with AIDS


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Tumor follows lymphatic channels

Leiomyosarcoma

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Usually is metastatic, not primary

Melanoma

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Rare, must rule out metastatic disease

Rhabdomyosarcoma

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Pleomorphic subtype more common in adults, embryonic subtype more common in

children

Synovial sarcoma

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Rare

Case report with SYT-SSX2 and rapidly progressive course, Archives 2003;127:e201

Case report of poorly differentiated tumor with rhabdoid features, Archives

2003;127:e160

Associated with chest pain, hemoptysis, dyspnea, cough, fever

40-55% die of disease after 20 years follow-up, due to metastases to liver, CNS, bone

or invasion of adjacent organs


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Tumors with SYT-SSX2 fusion proteins have better prognosis with 89% 5 year

survival vs. 42% for SYT-SSX1 fusion protein

Micro: monophasic spindle cells or biphasic with epithelial and spindle cell

component; monophasic tumors have compact fascicles of hyperchromatic spindle

cells with hemangiopericytoma-like areas; often punctuated by small arteries and

capillaries in an irregular distribution; poorly differentiated tumors have small, round

blue cells resembling Ewings sarcoma/PNET; rarely has rhabdoid features

Micro images: H&E, bcl2, CD99, CD117, poorly differentiated tumor: H&E and

vimentin

Positive stains: CD117 (in dispute)

Molecular: t(X;18)(p11.2;icq11.2); produces either SYT-SSX1 or SYT-SSX2 fusion

genes

DD: monophasic tumor resembles fibrosarcoma, hemangiosarcoma, leiomyosarcoma,

spindle cell carcinoma, carcinosarcoma or sarcomatoid carcinoma (primary or

metastatic)

Alveolar adenoma

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