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UNCOMMON CANCERS

UNCOMMON CANCERS. 13 APRIL 2012 DR ELIUD NJUGUNA. UNCOMMON CANCERS(?). TRUE RARE INCIDENCE DIAGNOSTIC LIMITATIONS PRACTICE BIAS GEOGRAPHICAL/REGIONAL BIAS POOR /INCORRECT DOCUMENTATION. Coding. Coding. Coding. Coding. Coding. Uncommon cancers. Unusual sites

gwendolyn-thompson
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UNCOMMON CANCERS

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  1. UNCOMMON CANCERS 13 APRIL 2012 DR ELIUD NJUGUNA

  2. UNCOMMON CANCERS(?) • TRUE RARE INCIDENCE • DIAGNOSTIC LIMITATIONS • PRACTICE BIAS • GEOGRAPHICAL/REGIONAL BIAS • POOR /INCORRECT DOCUMENTATION

  3. Coding

  4. Coding

  5. Coding

  6. Coding

  7. Coding

  8. Uncommon cancers • Unusual sites • Unusual behavioure.g rare metastatic patterns

  9. MERKEL CELL CA (MCC) • Aggressive rare small neuroendocrine cell skin tumour • Usually occurs from sixth decade of life • Commonly occurs in head and neck region • Involve reticular dermis and subcutaneous tissue and rarely involve papillary dermis • Usually invade vascular and lymphatic channels • 25-75% local recurrence after surgical excision • Single most prognostic factor is stage at diagnosis

  10. MCC • Probably missed due to diagnostic limitations-IHC • Management involves surgical excision +regional LN excision followed by chemo/XRT • XRT field should include original tumour volume +5cm margin, entire scar and nodal bed • I HAVE ONLY TWO PATIENTS LAST TWO YEARS

  11. CUTANEOUS T CELL LYMPHOMA (CTCL) • HAS TWO CLINICAL COMPONENTS ; a)MYCOSIS FUNGOIDES ( MF) b)SEIZARY SYNDROME (SZ) • CHARACTERIZED BY CD4 POSITIVITY • RISK FACTORS INCLUDE INDUSTRIAL CHEMICAL EXPOSURE AND GENETIC FACTORS • 2:1 M:F, 2:1 BLACKS : WHITES • COMMONEST AFTER SIXTH DECADE OF LIFE (our patients appear to be much younger)

  12. MF • Commoner in sun shaded areas but can appear anywhere in the body • Has 3 progressive clinical phases ; a)premycotic/patch b)infiltrated plaque/mycotic c)Fungoid/tumour • Pathology is accumulation of atypical lymphoid cells in the skin and the natural history may span six years to a few decades

  13. MF • TREATMENT IS SKIN IRRADIATION AND CHEMOTHERAPY(total skin electron beam irradiation, topical mechlorithamine chemotherapy, photochemotherapy with methoxsalem ,oral retinoidsegbexarotene, systemic chemotherapy) • PROGNOSIS REMAINS POOR ---MEDIAN SURVIVAL 5-10 YEARS • 4PATIENTS LAST TWO YEARS

  14. SZ • Characterized by presence of malignant T cells in peripheral blood • Treatment as in MF • Prognosis as in advanced MF

  15. CA PENIS • M0ST CANCERS ARISE IN THE GLANS AND PREPUSE • FREQUENTLY PRESENTS WITH INGUINAL ADENOPATHY • OFTEN HIV RELATED • USUALLY PRESENT WITH ULCERATIVE OR EXOPHYTIC PAPPILLARY LESIONS • MOST ARE WELL DIFFERENTIATED SQUAMOUS CELL CA

  16. CA PENIS • SIZE OF PRIMARY LESION AND LN STATUS MAIN PROGNOSTIC FACTORS • MANAGEMENT INVOLVES SURGERY(partial or total penectomy,circumcision for prepuce lesions ),RADIOTHERAPY (brachytherapy using irridium implants or external beam) and CHEMOTHERAPY • 3 PATIENTS ON TREATMENT

  17. URETHRAL CA • MAY PRESENT WITH HAEMATURIA ,OBSTRUCTION, DISCHARGE, DYSURIA, PROLAPSE OR ULCERATION • USUALLY SPREADS BY DIRECT EXTENSION TO SURROUNDING TISSUE • TREATMENT IS MAINLY SURGICAL +/-CHEMO/XRT

  18. VAGINAL CA • USUALLY PRESENTS WITH ABNORMAL VAGINAL BLEEDING • COMMON SITE IS UPPER 1/3 POSTERIOR WALL • EXHIBIT MUCOSAL SPREAD TO THE CERVIX / VULVA • COMMONLY INVOLVE MULTIPLE PELVIC/INGUINAL NODAL GROUPS • DISTANT METS IN 25% OF PATIENTS

  19. VAGINAL CA • 90% ARE EPIDERMOID CARCINOMAS (most are non keratinizing and moderately diff) ADENO CA ARE ABOUT 5%,OTHERS ARE ADENOID CYSTIC CA, SMALL CELL N.E.T, SARCOMAS, MELANOMA • SURGERY AND RADIOTHERAPY ARE THE MAIN TREATMENT MODALITIES • 6 PATIENTS LAST TWO YEARS

  20. URETERICTUMOURS • TEND TO OCCUR IN LOWER 1/3 • MAY BE MULTIFOCAL OR SPREAD DIRECTLY ALONG UROTHELIUM • >90% ARE TCC • SURGERY (radical nephrouretectomy)IS TREATMENT OF CHOICE. CONSERVATIVE SURGERY +RADIOTHERAPY WHERE NEPHRECTOMY IS NOT INDICATED • CHEMOTHERAPY-MVAC FOR METASTATIC DSE

  21. UNUSUAL NON EPITHELIAL H/N TUMOURS • GLOMUS TUMOURS-Arise in jugular bulb and along tympanic and auricular branch of tenth cranial nerve -CLASSIFIED AS TYMPANIC, JUGULARE OR CAROTID VAGAL -HITOLOGICALLY BENIGN BUT OCCASSIONALY EXTEND TO LUMEN OF VEINS TO REGIONAL NODES BUT RARELY TO DISTANT SITES -TREATMENT OPTIONS INCLUDE SURGERY, EMBOLIZATION AND XRT

  22. H/N contd • HEMANGIOPERICYTOMA -UNUSUAL VASCULAR TUMOUR THAT MAY OCCUR ANYWHERE IN THE BODY -HAS CHARACTERISTIC ANGIOGRAPHIC FEATURES ON ARTERIOGAPHY -MANAGEMENT IS LOCAL EXCISION+/- OR RADIOTHERAPY

  23. H/N contd • OTHER TUMOURS -CHORDOMAS(rare neoplasm of axial skeleton arising from remnants of primitive notochord-chordadorsalis) -LETHAL MIDLINE GRANULOMA(Associated with Epstein Barr virus-ulceration and necrosis of midline facial tissues CHLOROMA-Granulocytic sarcoma associated with AML

  24. ENDEMIC KS • ONLY 1% OF ALL KS CASES -VISCERAL IN 5% OF CASES -SLOW PROGRESSION -XRT/SXT MAINSTAY OF TREATMENT -SYSTEMIC CHEMOTHERAPY MAY BE USED IN WIDESPREAD DSE OR IN-FIELD RECURRENCE

  25. THANK YOU

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