Date post: | 05-Apr-2017 |
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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