UNCOMMON CANCERS

UNCOMMON CANCERS13 APRIL 2012DR ELIUD NJUGUNAUNCOMMON CANCERS(?)TRUE RARE INCIDENCEDIAGNOSTIC LIMITATIONSPRACTICE BIASGEOGRAPHICAL/REGIONAL BIASPOOR /INCORRECT DOCUMENTATIONCoding TITLESCODENAMEC53CERVIXC15OESOPHAGUSC50BREASTC11NASO PHARYNXC56OVARYC61PROSTATEC16STOMACHC18COLONC20RECTUMC21ANUS3Coding C21ANUSC32LARYNXC46KAPOSIS SARCOMAC51VULVAC52VAGINAC90MULTIPLE MYELOMAC22LIVERC91LYMPHOID LEUKEMIAC85OTHER & UNSPECIFIED TYPES OF NON HODGDINS LYMPOMAC92MYLEOID LEUKEMIACodingTITLESCODENAMEC79SECONDARY MALIGNANT NEOPLASM OF OTHER SITESC34LUNGC43.4SCALPC05PALATEC14.0ORAL CAVITYC02TOUNGUEC06MOUTHC69EYEC49.0EARC64KIDNEYC41.0FACECoding TITLESCODENAMEC73THYROIDC71.9BRAINC26.1SPLEENC43SKINC00.4LOWER LIPC41.1MAXILLAC60PENISC07PAROTIDC25PANCREASC76.0NECKCoding C69.6ORBITC09TONSILC67BLADDERC41.8OVERLAPPING LESION OF BONE AND ARTICULAR CARTILAGEC76.5LOWER LIMBC41.9BONE AND ARTICULAR CARTILAGEC76.0HEAD, FACE, AND NECKC95LEUKEMIA OF UNSPECIFIED CELL TYPEC76.1THORAXC98SARCOMA

Uncommon cancersUnusual sitesUnusual behaviour e.g rare metastatic patterns

MERKEL CELL CA (MCC) Aggressive rare small neuroendocrine cell skin tumourUsually occurs from sixth decade of lifeCommonly occurs in head and neck regionInvolve reticular dermis and subcutaneous tissue and rarely involve papillary dermisUsually invade vascular and lymphatic channels25-75% local recurrence after surgical excisionSingle most prognostic factor is stage at diagnosisMCCProbably missed due to diagnostic limitations-IHCManagement involves surgical excision +regional LN excision followed by chemo/XRTXRT field should include original tumour volume +5cm margin, entire scar and nodal bed

I HAVE ONLY TWO PATIENTS LAST TWO YEARS

CUTANEOUS T CELL LYMPHOMA (CTCL)HAS TWO CLINICAL COMPONENTS ; a)MYCOSIS FUNGOIDES ( MF) b)SEIZARY SYNDROME (SZ)CHARACTERIZED BY CD4 POSITIVITYRISK FACTORS INCLUDE INDUSTRIAL CHEMICAL EXPOSURE AND GENETIC FACTORS2:1 M:F, 2:1 BLACKS : WHITESCOMMONEST AFTER SIXTH DECADE OF LIFE (our patients appear to be much younger)

MFCommoner in sun shaded areas but can appear anywhere in the bodyHas 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

MFTREATMENT IS SKIN IRRADIATION AND CHEMOTHERAPY(total skin electron beam irradiation, topical mechlorithamine chemotherapy, photochemotherapy with methoxsalem ,oral retinoids eg bexarotene, systemic chemotherapy)PROGNOSIS REMAINS POOR ---MEDIAN SURVIVAL 5-10 YEARS4 PATIENTS LAST TWO YEARS SZCharacterized by presence of malignant T cells in peripheral bloodTreatment as in MFPrognosis as in advanced MF

CA PENISM0ST CANCERS ARISE IN THE GLANS AND PREPUSEFREQUENTLY PRESENTS WITH INGUINAL ADENOPATHYOFTEN HIV RELATEDUSUALLY PRESENT WITH ULCERATIVE OR EXOPHYTIC PAPPILLARY LESIONSMOST ARE WELL DIFFERENTIATED SQUAMOUS CELL CACA PENISSIZE 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 TREATMENTURETHRAL CAMAY PRESENT WITH HAEMATURIA ,OBSTRUCTION, DISCHARGE, DYSURIA, PROLAPSE OR ULCERATIONUSUALLY SPREADS BY DIRECT EXTENSION TO SURROUNDING TISSUETREATMENT IS MAINLY SURGICAL +/-CHEMO/XRTVAGINAL CAUSUALLY PRESENTS WITH ABNORMAL VAGINAL BLEEDINGCOMMON SITE IS UPPER 1/3 POSTERIOR WALLEXHIBIT MUCOSAL SPREAD TO THE CERVIX / VULVACOMMONLY INVOLVE MULTIPLE PELVIC/INGUINAL NODAL GROUPSDISTANT METS IN 25% OF PATIENTSVAGINAL CA90% 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, MELANOMASURGERY AND RADIOTHERAPY ARE THE MAIN TREATMENT MODALITIES6 PATIENTS LAST TWO YEARS

URETERIC TUMOURSTEND TO OCCUR IN LOWER 1/3MAY BE MULTIFOCAL OR SPREAD DIRECTLY ALONG UROTHELIUM>90% ARE TCCSURGERY (radical nephrouretectomy)IS TREATMENT OF CHOICE. CONSERVATIVE SURGERY +RADIOTHERAPY WHERE NEPHRECTOMY IS NOT INDICATEDCHEMOTHERAPY-MVAC FOR METASTATIC DSEUNUSUAL NON EPITHELIAL H/N TUMOURSGLOMUS 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

H/N contdHEMANGIOPERICYTOMA-UNUSUAL VASCULAR TUMOUR THAT MAY OCCUR ANYWHERE IN THE BODY-HAS CHARACTERISTIC ANGIOGRAPHIC FEATURES ON ARTERIOGAPHY-MANAGEMENT IS LOCAL EXCISION+/- OR RADIOTHERAPYH/N contdOTHER TUMOURS-CHORDOMAS(rare neoplasm of axial skeleton arising from remnants of primitive notochord-chorda dorsalis)-LETHAL MIDLINE GRANULOMA(Associated with Epstein Barr virus-ulceration and necrosis of midline facial tissuesCHLOROMA-Granulocytic sarcoma associated with AML

ENDEMIC KSONLY 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

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