13 april 2012 dr eliud njuguna. uncommon cancers(?) true rare incidence diagnostic limitations...
TRANSCRIPT
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