MEDIASTINAL TUMORS

CONTENTS

• ANATOMY AND CLINICAL CORRELATION • APPROACH TO A MEDIATINAL TUMOR• OVERVIEW OF DIFFERENT MEDIASTNAL TUMOR

ANATOMY OF MEDIASTINUM • BORDERS OF MEDIASTINUM • COMPARTMENTS OF MEDIASTINUM • CONTENTS OF MEDIASTINUM

ANTERO SUPERIOR • THYMUS

• LYPMH NODES• FAT

MIDDLE• PERICARDIUM

• HEART • ASCENDING AORTA

• SVC • PULMONARY VEIN• PULMONARY ARTERY • PHRENIC NERVE• BIFURCATION OF

TRACHEA • LYMPHNODE

POSTERIOR• OESOPHAGUS

• DESCENDING AORTA• AZYGOS VEIN

• HEMI AZYGOS VEIN• ACCESORY AZYGOS VEIN• SYMPATHETIC CHAIN• VAGUS NERVE• THROACIC DUCT • LYMPH NODES

CLINICAL CORRELATION • ANTERO SUPERIOR COMPARTMENT THYMOMA ( ADULT ) GERM CELL TUMOR LYMPHOMA RETROSTERNAL GOITRE• MIDDLE COMPARTMENT LYMPHOMA TUMORS OF BLOODVESSELS TUMORS OF HEART• POSTERIOR COMPARTMENT NEUROGENIC TUMORS ( CHILDREN ) LYMPHOMA

CONTENTS

• ANATOMY AND CLINICAL CORRELATION • APPROACH TO A MEDIASTINAL TUMOR• OVERVIEW OF DIFFERENT MEDIASTINAL TUMOR

APPROACH TO MEDIASTINAL TUMORS

CLINICAL PRESENTATION

INCIDENTALLY NOTICED SYMPTOMS DUE TO

COMPRESSION SYSTEMIC SYMPTOMS

APPROACH TO MEDIASTINAL TUMORS

DIAGNOSIS

AGE

SYMPTOMSIMAGING STUDIES

(ANATOMICAL LOCATION )

THYMOMA - COMMON IN ADULTS NEUROGENIC TUMORS – COMMON IN CHILDREN

PRESSURE SYMPTOMS • STRIDOR• DYSPNEA• COUGH

• HEMOPTYSIS • FACE AND UPPER EXTREMITY SWELLING

• CARDIAC TAMPONADE• PLEURAL EFFUSION

• DYSPHAGIA • HORNER SYNDROME

SYSTEMIC SYMPTOMS • FEVER , NIGHT SWEATS , WEIGHT LOSS – LYPHOMA

• DIPLOPIA , PTOSIS , DYSPHAGIA – MYASTHENIA GRAVIS

X-RAY PA AND LATERAL VIEWCT CHEST WITH CONTRAST

TUMOR MARKERS ANTI ACETYL CHOLINE ANTIBODIES - MYASTHENIA

GRAVIS α-FETO PROTEIN AND β-HCG – GERM CELL

TUMORS

MRI - CONFIRM INVASION OR SPINAL CANAL INVOLVEMENT

FNAC / CORE BIOSPSY

APPROACH TO MEDIASTINAL TUMORS

TREATMENT

SURGERY –

CHEMOTHERAPY AND RADIATION

MINIMALLY INVASIVE • VATS , ROBOTIC

OPEN APPROACH• MEDIAN STERNOTOMY• THOROCOTOMY

CONTENTS

• ANATOMY AND CLINICAL CORRELATION • APPROACH TO A MEDIASTINAL TUMOR• OVERVIEW OF DIFFERENT MEDIASTINAL TUMOR

OVERVIEW OF DIFFERENT MEDIASTINAL TUMORS

• THYMOMA• NEUROGENIC TUMORS• GERM CELL TUMORS• LYMPHOMA

THYMOMA

• SEEN BETWEEN 40 – 60 YRS• ASSOCIATED WITH PARANEOPLASTIC SYNDROMES LIKE MYASTHENIA , RED CELL APLASIA, HYPOGAMMAGLOBLINEMIA, SLE• 2 STAGINGS – MASAOKA AND WHO

Stage I – Completely encapsulated .Stage II – Capsular invasion. IIA: Microscopic capsular invasion IIB: Macroscopic invasion into surrounding fatty

tissue or adherent to but not through the mediastinal pleura or pericardium

Stage III – Pericardial or lung involvement. IIIA: Macroscopic invasion into pericardium or lung

without great vessel invasion IIIB: Macroscopic invasion into pericardium or lung

with great vessel invasionStage IV – Disseminated disease. IVA: Pleural or pericardial dissemination IVB: Lymphatic or hematogenous metastases

TREATMENT : STAGE 1 AND 2 – COMPLETE RESECTION STAGE 3 AND 4 – COMPLETE RESECTION + POST OP RADIATION POST OP CHEMOTHERAPY – CISPLATIN,

DOXORUBICIN, CYCLOPHOSPHAMIDE

IN UNRESECTABLE TUMOR – PRE OP CHEMOTHERAPY [OR] RADIOTHERAPY IS DONE .

OVERVIEW OF DIFFERENT MEDIASTINAL TUMORS

• THYMOMA• NEUROGENIC TUMORS• GERM CELL TUMORS• LYMPHOMA

NEUROGENIC TUMORS • ORIGIN SYMPATHETIC GANGLIA :– GANGLIOMA GANGLIO-NEUROBLASTOMA NEUROBLASTOMANERVE SHEATH :– NEUROLEMOMA(SCHWANOMA) NEUROFIBROMA NEUROSARCOMAPARAGANGLION CELLS :- PHEOCHROMOCYTOMA

NEUROBLASTOMA• CHILDHOOD TUMOR

• COMMONLY SEEN IN ADRENAL MEDULLAOTHER SITE – SYMPATHETIC CHAIN • ARISE FROM NEUROBLASTIC CELLS

• C/F – MASS IN LOIN OR CHEST (PRESSURE SYMPTOM)

OTHER MANIFESTATIONS - RACOON EYE SIGN , OPSOMYOCLONUS SYNDROME , HYPERTENSION

, FLUSHING , DIARRHOEA (VIP) • INVESTIGATION – CT OR MRI , VANILYL

MANDELIC ACID (VMA ) , HOMOVANILIC ACID (HVA) , MIBG( META IODO BEZYL GUANIDINE)

SCAN , BONE MARROW BIOPSY

GRADING OF NEUROBLASTOMABASED ON AGE , EXTENT OF DISEASE , HISTOLOGY ,

N-MYC ONCOGENE

LOW RISK 90% - SURGICAL EXCISION INTERMEDIATE RISK 70 % – EXCISION AND

RADIOTHERAPY HIGH RISK 30 % – DEBULKING , RADIATION AND

CHEMOTHERAPY (CISPLATIN , DOXORUBICIN , CYCLOPHOSPHAMIDE ) AND SECOND LOOK SURGERY

NEUROGENIC TUMORS • ORIGIN SYMPATHETIC GANGLIA :– GANGLIOMA GANGLIO-NEUROBLASTOMA NEUROBLASTOMANERVE SHEATH :– NEUROLEMOMA(SCHWANOMA) NEUROFIBROMA NEUROSARCOMAPARAGANGLION CELLS :- PHEOCHROMOCYTOMA

PHEOCHROMOCYTOMA

• TUMOR ARISES FROM CHROMAFFIN CELLS• SEEN COMMONLY IN ADRENAL MEDULLA

EXTRA ADRENAL ( PARAGANGLIONS ) • THESE TUMOR SECRETES NORADRENALINE AND

ADRENALINE • RULE OF 10’S :– 10% - MALIGNANT ,

EXTRAADRENAL , BILATREAL , FAMILIAL , MULTIPLE , NON HYPERTENSIVE

• CLINICAL FEATURES – HEADACHE , PALPITATION , FLUSHING , WEAKNESS , HYPERTENSION ,

• ASSOSIATED WITH MEN 2 , VHL , NF TYPE 1 .

• INVESTIGATION – 24 HR URINE OR BLOOD SAMPLE - METANEPHRINE &

NORMETANEPHRINE MRI FOR LOCALISATION MIBG SCAN • TREATMENT :– INTIALLY α- BLOCKER (PHENOXYLBENZAMINE) IS GIVEN FOR 10 DAYS THEN START β – BLOCKERS SURGICAL EXCISION OF TUMOR IS DONE .

MALIGNANT PHEOCHROMOCYTOMA – SURGERY + I-131 MIBG + POST OP CHEMOTHERAPY - MITOTANE + CYCLOPHOSPHAMIDE , VINCRISTINE , DACARBAZINE .

NEUROGENIC TUMORS • ORIGIN SYMPATHETIC GANGLIA :– GANGLIOMA GANGLIO-NEUROBLASTOMA NEUROBLASTOMANERVE SHEATH :– NEUROLEMOMA(SCHWANOMA) NEUROFIBROMA NEUROSARCOMAPARAGANGLION CELLS :- PHEOCHROMOCYTOMA

NEUROFIBROMA•ARISE FROM SCHWANN CELLS AND CONNECTIVE TISSUES •TYPES – CUTANEOUS – SMALL , HARD , MULTIPLE NODULES PLEXIFORM – LARGE , HARD MASS NON TENDER , PARAESTHESIA ,MOVE PERPENDICULAR TO THE NERVE•COMMONLY ASSOCIATED WITH NEUROFIBROMATOSIS TYPE 1 – CHROMOSOME 17 MUTATION , NF-1 GENE .OTHER FEATURES – AXILLARY FRECKLES , LISCH NODULES, CAFÉ AU LAIT SPOT . INVESTIGATION – CT/MRI , SLIT LAMP , MOLECULAR TESTING . TREATMENT – SURGICAL EXCISION

NEUROGENIC TUMORS • ORIGIN SYMPATHETIC GANGLIA :– GANGLIOMA GANGLIO-NEUROBLASTOMA NEUROBLASTOMANERVE SHEATH :– NEUROLEMOMA(SCHWANOMA) NEUROFIBROMA NEUROSARCOMAPARAGANGLION CELLS :- PHEOCHROMOCYTOMA

SCHWANNOMA•ARISE FROM SCHWANN CELLS , BENIGN TUMORS•CAN OCCUR AT ANY SITE •ASSOCIATED WITH NEUROFIBROMATOSIS TYPE - 2 CAUSING ACOUSTIC NEUROMA . •C/F – HEARING LOSS , PARAESTHESIA , SOMETIMES TENDER .• INVESTIGATION – CT/MRI , AUDIOMETRY .• TREATMENT – SURGICAL EXCISION

NEUROGENIC TUMORS • ORIGIN SYMPATHETIC GANGLIA :– GANGLIOMA GANGLIO-NEUROBLASTOMA NEUROBLASTOMANERVE SHEATH :– NEUROLEMOMA(SCHWANOMA) NEUROFIBROMA NEUROSARCOMAPARAGANGLION CELLS :- PHEOCHROMOCYTOMA

OVERVIEW OF DIFFERENT MEDIASTINAL TUMORS

• THYMOMA• NEUROGENIC TUMORS• GERM CELL TUMORS• LYMPHOMA

OVERVIEW OF DIFFERENT MEDIASTINAL TUMORS

• THYMOMA• NEUROGENIC TUMORS• GERM CELL TUMORS• LYMPHOMA• CYSTS

GERM CELL TUMORS • DUE TO FAILURE OF MIGRATION OF GERM CELLS

•GERM CELL TUMORS

•TERATOMA IS THE COMMONEST IN MEDIASTINUM •C/F – PRESSURE SYMPTOMS •INVESTIGATION – CT / MRI , X-RAY •TREATMENT – SURGICAL EXCISION SEMINOMATOUS TUMOR – RADIOSENSITIVE AND CHEMOSENSITIVE NON SEMINOMATOUS – CHEMOSENSITIVE CHEMOTHERAPY – BEP (BLEOMYCIN , ETOPOSIDE , CISPLATIN)

SEMINOMATOUS

NON- SEMINOMATOUS

OVERVIEW OF DIFFERENT MEDIASTINAL TUMORS

• THYMOMA• NEUROGENIC TUMORS• GERM CELL TUMORS• LYMPHOMA

OVERVIEW OF DIFFERENT MEDIASTINAL TUMORS

• THYMOMA• NEUROGENIC TUMORS• GERM CELL TUMORS• LYMPHOMA

•LYMPHOMA•2 TYPES – HODGKIN’S AND NON-HODGKIN’S•SITE :– HODGKIN’S - MAINLY CERVICAL OTHER SITE – MEDIASTINUM , AXILLARY , INGUINAL NON- HODGKIN’S – WALDEYER’S RING , EPITROCHLEAR , ABDOMINAL ( MALT ) • C/F – PRESSURE SYMPTOMS , FEVER , WEIGHT LOSS , NIGHT SWEATS, ANAEMIA , BONE PAIN . • INVESTIGATION – X-RAY , CT •TREATMENT – RADIOTHERAPY CHEMOTHERAPY – MOPP ( MUSTINE ,ONCOVINE {VINCRISTINE} , PROCARBAZINE, PREDNISOLONE) ABVD - ADRIAMYCIN , BLEOMYCIN , VINBLASTINE DACARBAZINE

OVERVIEW OF DIFFERENT MEDIASTINAL TUMORS

• THYMOMA• NEUROGENIC TUMORS• GERM CELL TUMORS• LYMPHOMA

MEDIASTINAL CYSTS

• PERICARDIAL CYST • BRONCHOGENIC CYST • ENTERIC CYST • THYMIC CYST