TUMORI RARI DEL DISTRETTO EPATO-BILIARE

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tumori primitivi rari della colecisti • Tumori epiteliali

– Adenoma tubulare– Sottotipi rari dell’adenocarcinoma

• papillare, mucinoso, a cellule chiare e a cellule ad anello con castone

– Carcinoma spinocellulare– Carcinoma adenosquamoso– Carcinoma a piccole cellule

• Tumori non epiteliali– carcinoide– sarcomi– melanoma

• Carcinosarcoma

Adenoma tubulare della colecisti

Rare tumor; papillar or tubular; in middle aged and older patients, although it may develop even in children.

•Gallbladder adenoma with focal adenocarcinoma: a case report Recenti Prog Med. 2007 Oct;98(10):506-8. •Tubular adenoma of the gallbladder with squamous metaplasia Srp Arh Celok Lek. 2006 Mar-Apr;134(3-4):159-61

•Tubular adenoma of the gallbladder with spindle cell metaplasia. J Korean Med Sci. 1998 Jun;13(3):295-8. •Pyloric gland type adenoma of the gallbladder with squamoid spindle cell metaplasia. Pathol Res Pract. 1996 Sep;192(9):963-9; discussion 970-1.

•Tubular adenoma of the gall bladder. J Indian Med Assoc. 1995 Oct;93(10):402.

Sottotipi dell’adenocarcinoma

• Papillary carcinomas, a subtype of adenocarcinomas, comprise 5% of gallbladder cancer

• Mucinous and the signet ring

• Clear cell carcinomas which must be distinguished from metastatic renal cell carcinoma

Papillary carcinomas of the gallbladder. Arch Pathol Lab Med 2005; 129:905-9

Unusual malignant epithelial tumours of the gallbladder. Semin Diagno Pathol 1996; 13(4):326-38

Clear cell carcinoma of the gallbladder. A hisological and immunohistochemical study. Pathol Res Pract 1995; 191(12):1259-65

Carcinoma spinocellulare della colecisti • Rare, 1.4 - 3.3%• Predominant in females;

proportion of 3:1 over males

• Fourth and sixth decades• A worse prognosis than

adenocarcinoma• Squamous cells may

develop from metaplastic squamous epithelium or squamous differentiation of adenocarcinoma.

• Bulky local disease and direct liver invasion.

Pure squamous cell carcinoma of the gall bladder. Indian J Pathol Microbiol. 2007 Jul;50(3):599-600.

Carcinoma adenosquamoso

• very rare gallbladder carcinoma

• less than 3.5% of gallbladder carcinomas

• characterised by invasive growth, a reduced tendency for lymph node metastasis, an increased tendency for hepatic infiltration or liver metastasis,

• poorer prognosis than adenocarcinoma.

Adenosquamous and squamous carcinoma of the gallbladder Cir Esp. 2007 Apr;81(4):202-6.

Carcinoma a piccole cellule

• Small cell carcinomas of the gallbladder are extremely rare.

• Most present with unresectable, metastatic disease.

Small cell carcinoma of the gallbladder: a case report and review of 53 cases in the literature. Hepatogastroenterology. 2001 Nov-Dec;48(42):1588-93.

Carcinoma combinato: a piccole cellule e adenocarcinoma

• Neoplastic glands are surrounded by cells characteristic of small-cell carcinoma

• The histogenesis is a subject of controversy.

• Three hypotheses proposed: – (a) two components arising

simultaneously from two different precursor cells;

– (b) one component producing the other;

– (c) a single totipotent progenitor cell producing both components

Combined neuroendocrine cell carcinoma and adenocarcinoma of the gallbladder: report of a case. J Nippon Med Sch. 2006 Apr;73(2):101-5.

Carcinoma neuroendocrino• Rare neuroendocrine carcinoma of the gall bladder.

Coincidental occurrence of an endometrioid ovarian adenocarcinoma] Dtsch Med Wochenschr. 2009;134(1-2):19-22

• Mixed (composite) glandular-endocrine cell carcinoma of the gallbladder. HPB (Oxford). 2001;3(1):7-9

• Moderately differentiated neuroendocrine carcinoma of the gallbladder. Arch Surg. 2008;143(1):95-6

• Case report: neuroendocrine carcinoma of the gallbladder causing hyperinsulinaemic hypoglycaemia. Clin Radiol. 2007 Apr;62(4):391-4

• MDCT findings in neuroendocrine carcinoma of the gallbladder: a case report. Abdom Imaging. 2006 Sep 12

• High-grade neuroendocrine carcinomas of the gallbladder and bile duct: Report of four cases with pathological correlation. J Comput Assist Tomogr. 2006 Jul-Aug;30(4):604-9

• MDCT findings in neuroendocrine carcinoma of the gallbladder: case report. Abdom Imaging. 2007 Jan-Feb;32(1):105-7

• Combined neuroendocrine cell carcinoma and adenocarcinoma of the gallbladder: report of a case. J Nippon Med Sch. 2006 Apr;73(2):101-5

• Neuroendocrine carcinoma of gallbladder: report of 2 cases. Hepatobiliary Pancreat Dis Int. 2005;4(1):144-6

• Large cell neuroendocrine carcinoma of the gallbladder: report of two cases. Am J Surg Pathol. 2000 Oct;24(10):1424-8

• Neuroendocrine carcinoma and adenocarcinoma of the gallbladder: report of a case with immunocytochemical study G E N. 1990 Apr-Jun;44(2):146-50

Carcinoide

• Less than 50 carcinoid tumors of the gallbladder have been reported

• Less than 1% of all carcinoid tumors arising from different parts of the body

• Most are localized• Usually lack specific symptoms• Incidentally detected after a

cholecystectomy• Rarely do they manifest with

carcinoid syndrome

Carcinoid tumor of the gall bladder. Ann Diagn Pathol. 2007 Apr;11(2):113-6.

Carcinosarcoma

• 68 cases in published literatures• 52 females and 16 males

(F:M=3.25:1)• 27 Japanese• Mean age 68.8 years (median

68 years, range 45-91 years)• Adenocarcinoma the most

common epithelial component (79.2%) squamous cell carcinoma the least common (9.4%)

• Spindle cell type the most common mesenchymal component (44.6%) and osteoid was the least common (5.4%)

Prognostic significance of race and tumor size in carcinosarcoma of gallbladder: a meta-analysis of 68 cases. Int J Clin Exp Pathol. 2008 Jan 1;1(1):75-83.

Tumore neuroectodermico primitivo

• Intralumina polypoid mass of the gallbladder with heterogenous enhancement (arrowhead).

• Diffuse sheets of monotonous small round cells

• Tumor cells have coarse or fine powdery chromatin, scanty cytoplasm, and inconspicuous nucleoli

• The tumor cells demonstrate a diffuse, positive membranous immunoreactivity for MIC2 gene product (A) and positive cytoplasmic immunoreactivity for synaptophysin (B)

Song DE et al Arch Pathol Lab Med. 2004;128:571–573

Melanoma primitivo

• 28 reported cases• Fourth or fifth decade • Prognosis poor• In an autopsy study by

Das Gupta and Brasfield, gallbladder metastases were found in 15% of patients with metastatic melanoma of the gastrointestinal tract

Malignant melanoma of the gallbladder: a report of two cases and review of the literature. Samplaski MK, et al. J Gastrointest Surg. 2008 Jun;12(6):1123-6