Mediastinal tumors
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Transcript of Mediastinal tumors
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MEDIASTINAL TUMORS
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CONTENTS
• ANATOMY AND CLINICAL CORRELATION • APPROACH TO A MEDIATINAL TUMOR• OVERVIEW OF DIFFERENT MEDIASTNAL TUMOR
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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
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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
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CONTENTS
• ANATOMY AND CLINICAL CORRELATION • APPROACH TO A MEDIASTINAL TUMOR• OVERVIEW OF DIFFERENT MEDIASTINAL TUMOR
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APPROACH TO MEDIASTINAL TUMORS
CLINICAL PRESENTATION
INCIDENTALLY NOTICED SYMPTOMS DUE TO
COMPRESSION SYSTEMIC SYMPTOMS
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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
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APPROACH TO MEDIASTINAL TUMORS
TREATMENT
SURGERY –
CHEMOTHERAPY AND RADIATION
MINIMALLY INVASIVE • VATS , ROBOTIC
OPEN APPROACH• MEDIAN STERNOTOMY• THOROCOTOMY
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CONTENTS
• ANATOMY AND CLINICAL CORRELATION • APPROACH TO A MEDIASTINAL TUMOR• OVERVIEW OF DIFFERENT MEDIASTINAL TUMOR
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OVERVIEW OF DIFFERENT MEDIASTINAL TUMORS
• THYMOMA• NEUROGENIC TUMORS• GERM CELL TUMORS• LYMPHOMA
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THYMOMA
• SEEN BETWEEN 40 – 60 YRS• ASSOCIATED WITH PARANEOPLASTIC SYNDROMES LIKE MYASTHENIA , RED CELL APLASIA, HYPOGAMMAGLOBLINEMIA, SLE• 2 STAGINGS – MASAOKA AND WHO
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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
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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 .
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OVERVIEW OF DIFFERENT MEDIASTINAL TUMORS
• THYMOMA• NEUROGENIC TUMORS• GERM CELL TUMORS• LYMPHOMA
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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
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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
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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 .
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• 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 .
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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
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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
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NEUROGENIC TUMORS • ORIGIN SYMPATHETIC GANGLIA :– GANGLIOMA GANGLIO-NEUROBLASTOMA NEUROBLASTOMANERVE SHEATH :– NEUROLEMOMA(SCHWANOMA) NEUROFIBROMA NEUROSARCOMAPARAGANGLION CELLS :- PHEOCHROMOCYTOMA
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OVERVIEW OF DIFFERENT MEDIASTINAL TUMORS
• THYMOMA• NEUROGENIC TUMORS• GERM CELL TUMORS• LYMPHOMA
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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
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OVERVIEW OF DIFFERENT MEDIASTINAL TUMORS
• THYMOMA• NEUROGENIC TUMORS• GERM CELL TUMORS• LYMPHOMA
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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
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OVERVIEW OF DIFFERENT MEDIASTINAL TUMORS
• THYMOMA• NEUROGENIC TUMORS• GERM CELL TUMORS• LYMPHOMA
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MEDIASTINAL CYSTS
• PERICARDIAL CYST • BRONCHOGENIC CYST • ENTERIC CYST • THYMIC CYST