Mediastinal tumors

27
MEDIASTINAL TUMORS

Transcript of Mediastinal tumors

Page 1: Mediastinal tumors

MEDIASTINAL TUMORS

Page 2: Mediastinal tumors

CONTENTS

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

Page 3: Mediastinal tumors

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

Page 4: Mediastinal tumors

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

Page 5: Mediastinal tumors

CONTENTS

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

Page 6: Mediastinal tumors

APPROACH TO MEDIASTINAL TUMORS

CLINICAL PRESENTATION

INCIDENTALLY NOTICED SYMPTOMS DUE TO

COMPRESSION SYSTEMIC SYMPTOMS

Page 7: Mediastinal tumors

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

Page 8: Mediastinal tumors

APPROACH TO MEDIASTINAL TUMORS

TREATMENT

SURGERY –

CHEMOTHERAPY AND RADIATION

MINIMALLY INVASIVE • VATS , ROBOTIC

OPEN APPROACH• MEDIAN STERNOTOMY• THOROCOTOMY

Page 9: Mediastinal tumors

CONTENTS

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

Page 10: Mediastinal tumors

OVERVIEW OF DIFFERENT MEDIASTINAL TUMORS

• THYMOMA• NEUROGENIC TUMORS• GERM CELL TUMORS• LYMPHOMA

Page 11: Mediastinal tumors

THYMOMA

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

Page 12: Mediastinal tumors

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

Page 13: Mediastinal tumors

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 .

Page 14: Mediastinal tumors

OVERVIEW OF DIFFERENT MEDIASTINAL TUMORS

• THYMOMA• NEUROGENIC TUMORS• GERM CELL TUMORS• LYMPHOMA

Page 15: Mediastinal tumors

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

Page 16: Mediastinal tumors

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

Page 17: Mediastinal tumors

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 .

Page 18: Mediastinal tumors

• 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 .

Page 19: Mediastinal tumors

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

Page 20: Mediastinal tumors

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

Page 21: Mediastinal tumors

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

Page 22: Mediastinal tumors

OVERVIEW OF DIFFERENT MEDIASTINAL TUMORS

• THYMOMA• NEUROGENIC TUMORS• GERM CELL TUMORS• LYMPHOMA

Page 23: Mediastinal tumors

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

Page 24: Mediastinal tumors

OVERVIEW OF DIFFERENT MEDIASTINAL TUMORS

• THYMOMA• NEUROGENIC TUMORS• GERM CELL TUMORS• LYMPHOMA

Page 25: Mediastinal tumors

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

Page 26: Mediastinal tumors

OVERVIEW OF DIFFERENT MEDIASTINAL TUMORS

• THYMOMA• NEUROGENIC TUMORS• GERM CELL TUMORS• LYMPHOMA

Page 27: Mediastinal tumors

MEDIASTINAL CYSTS

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