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Case report of Primary vulval cloacogenic carcinoma. Suneetha Ganta, Jennifer Hoh, R Wood UNIVERSITY HOSPITAL NORTH DURHAM References Wilkinson EJ:Premalignant.

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Presentation on theme: "Case report of Primary vulval cloacogenic carcinoma. Suneetha Ganta, Jennifer Hoh, R Wood UNIVERSITY HOSPITAL NORTH DURHAM References Wilkinson EJ:Premalignant."— Presentation transcript:

1 Case report of Primary vulval cloacogenic carcinoma. Suneetha Ganta, Jennifer Hoh, R Wood UNIVERSITY HOSPITAL NORTH DURHAM References Wilkinson EJ:Premalignant precancerous. and malignant tumors of vulva. Blaustein 's Pathology of the Female Genital Tract. Kurman RJ (ed). New York, Springer-Verlag, 4th Ed, 1994, pp 87-129 Kennedy JC, Majmudar B: Primary adenocarcinoma of the vulva, possibly cloacogenic. a report of two cases. J Reprod Med 1993; 38:113-116 We report a case of a 57year old woman with a vulval cloacogenic carcinoma initially presenting with a lump between the urethra and the clitoris. Cloacogenic carcinoma is a rare embryological tumor with only few cases of vulval or vaginal involvement. Historical Background: Hermann and Desfosses first drew to attention to the presence of transitional epithelium in the region of the dentate line of the anal canal; they believe this epithelium to represent remnants of embryonic cloaca. Grivalsky and Helwig reconfirmed these observations in 1956 and pointed this complex cloacogenic zone, and its adnexal structures can give rise to neoplasms with a variety of patterns. They coined the term “Transitional cloacogenic carcinoma”. Due to the intricate embryological plan of the anogenital region cloacogenic carcinoma is multicentric in nature, arising from the vagina, perineal skin,vulva, ecto cervix or anus. Herein we present a unique case of primary vulval cloacogenic carcinoma its manifestations, histological characteristics and management. Summary: In summary clocogenic carcinoma is a rare pelvic malignancy with multi centric origin. Usually limited to the pelvis the cloacogenic carcinoma presents as a mass, and has an indolent course if limited to the pelvis. Aggressive variants with extensive metastatic involvement including cardiac, pulmonary and hepatic involvement have been noted. Surgery and radiotherapy are the main modalities of treatment and the 5-year survival rate was 50%. Most common symptoms preceding the diagnosis of cloacogenic carcinoma are bleeding, pain, protruding mass, dysuria or change in bowel habits. Pruritis, recurrent abscess, weakness and fatigue are occasional symptoms. Duration of symptoms varies from months to years. If occurring in the rectum may accompany benign conditions such as fissure, fistula, or haemorrhoids. Treatment: A variety of surgical and radiotherapeutic modalities or both have been used in the management of cloacogenic carcinoma. In general the therapy for each patient was individualised in accord with physical findings. The current trend in the management of any gynaecological malignancy is towards a less radical surgical therapy.Modern individulisedtreatment requires careful consideration of the need to to minimize complications,reduce psychosexual sequelae and the choice of less disfiguring surgery without compromising disease free survival rate.Due to the lack of data available,the principles of management of primary cloacogenic carcinoma of the vulva are similar to those of vulvar cancer. Prognosis: The prognosis appears to be poor when pelvic or inguinal lymph nodes are involved at the time of definitive surgery no matter what treatment is employed. In the absence of nodal involvement the extent of local invasion and the size of the neoplasm appear to affect the prognosis directly. Five-year survival was up to 50%. CASE REPORT: A 57year old Caucasian woman presented with a 6month history of vaginal mass. She underwent wide local excision under GA, a bilobed cystic mass with no obvious capsule was identified.. Histology revealed a poorly differentiated invasive carcinoma with predominantly basiloid appearance with admixed area of squamoid differention.The lesion was considered to be Basaloid carcinoma.Surgery was uneventful.and complete. Radiotherapy was therefore not advised. She is currently being followed up as per protocols for vulval carcinoma. Discussion: Transitional cloacogenic carcinoma of the genital tract is characterized as a multicentric tumor that occurs within the ectocervix, vagina,vulva. Embryologically the cloaca can be identified as early as 6 weeks and is divided into dorsal rectum and a ventral region consisting of bladder and urogenital sinus. The Cloacogenic membrane is an endodermal-ectodermal plate formed by cloacal lining and surface ectoderm. The dorsal division invades through this plate at 7 weeks and forms the anal canal.The urogenital sinus grows upwards to meet and replace the Mullerian epithelium and becomes the vagina. The area between the anal and genital tract becomes perineum and ventrally, the urogenital sinus becomes the vagina and the urethra. The neoplasm’s that arise from this zone occur more frequently in the fifth and sixth decades of life.


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