UNCOMMON CANCERS

13 APRIL 2012DR ELIUD NJUGUNA

UNCOMMON CANCERS(?)TRUE RARE INCIDENCEDIAGNOSTIC LIMITATIONSPRACTICE BIASGEOGRAPHICAL/REGIONAL BIASPOOR /INCORRECT DOCUMENTATION

Coding TITLES

CODE NAME

C53 CERVIX

C15 OESOPHAGUS

C50 BREAST

C11 NASO PHARYNX

C56 OVARY

C61 PROSTATE

C16 STOMACH

C18 COLON

C20 RECTUM

C21 ANUS

Coding

C21 ANUS

C32 LARYNX

C46 KAPOSIS SARCOMA

C51 VULVA

C52 VAGINA

C90 MULTIPLE MYELOMA

C22 LIVER

C91 LYMPHOID LEUKEMIA

C85 OTHER & UNSPECIFIED TYPES OF NON HODGDINS LYMPOMA

C92 MYLEOID LEUKEMIA

CodingTITLES

CODE NAME

C79 SECONDARY MALIGNANT NEOPLASM OF OTHER SITES

C34 LUNG

C43.4 SCALP

C05 PALATE

C14.0 ORAL CAVITY

C02 TOUNGUE

C06 MOUTH

C69 EYE

C49.0 EAR

C64 KIDNEY

C41.0 FACE

Coding TITLES

CODE NAME

C73 THYROID

C71.9 BRAIN

C26.1 SPLEEN

C43 SKIN

C00.4 LOWER LIP

C41.1 MAXILLA

C60 PENIS

C07 PAROTID

C25 PANCREAS

C76.0 NECK

Coding C69.6 ORBIT

C09 TONSIL

C67 BLADDER

C41.8 OVERLAPPING LESION OF BONE AND ARTICULAR CARTILAGE

C76.5 LOWER LIMB

C41.9 BONE AND ARTICULAR CARTILAGE

C76.0 HEAD, FACE, AND NECK

C95 LEUKEMIA OF UNSPECIFIED CELL TYPE

C76.1 THORAX

C98 SARCOMA

Uncommon cancers

Unusual 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

diagnosis

MCCProbably 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 YEARS

4 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 CA

CA 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 TREATMENT

URETHRAL CAMAY PRESENT WITH

HAEMATURIA ,OBSTRUCTION, DISCHARGE, DYSURIA, PROLAPSE OR ULCERATION

USUALLY SPREADS BY DIRECT EXTENSION TO SURROUNDING TISSUE

TREATMENT IS MAINLY SURGICAL +/-CHEMO/XRT

VAGINAL 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 PATIENTS

VAGINAL 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, MELANOMA

SURGERY AND RADIOTHERAPY ARE THE MAIN TREATMENT MODALITIES

6 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 INDICATED

CHEMOTHERAPY-MVAC FOR METASTATIC DSE

UNUSUAL 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

RADIOTHERAPY

H/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 tissues

CHLOROMA-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

THANK YOU