罕见侵袭性原发外阴泄殖腔源性癌：病例报告1例和文献回顾

　　UNUSUALLY AGGRESSIVE PRIMARY CLOACOGENIC CARCINOMA OF THE VULVA: A CASE REPORT AND LITERATURE REVIEW

　　A. Agrawal^1,2,3, R. Chibbar^2,4, C.K. Giede^1,2,3, K.A. Wood⁵

　　¹Obstetrics and Gynecology, University of Saskachewan, Saskaton, ²Royal University Hospital, ³Saskachewan Cancer Centre, ⁴Department of Pathology, ⁵College of Medicine, University of Saskachewan, Saskatoon, SK, Canada

　　Primary cancer of the vulva is rare accounting for less than 5% of all lower female genital tract cancers. Primary cloacogenic carcinoma of the vulva is extremely rare with less than 20 cases reported in English literature. These tumors are thought to arise from embryonic or ectopic rests of cloacogenic tissue. Here we present a case of metastatic cloacogenic carcinoma of the vulva.

　　Case: A 49 year old nulligravida woman presented with multiple small vulvar lesions . Metastatic work up showed spread to the lung and bilateral bulky inguinal and pelvic lymph nodes. Based on histology and immunoprofile of vulvar biopsy a metastatic adenocarcinoma from the gastrointestinal tract was suggested. After work up for gastrointestinal pathology was negative, she was treated with concurrent chemo-radiation to the vulva.

　　She received second line chemotherapy and laparoscopic bilateral pelvic lymph node debulking for persistent disease.

　　Discussion: Only a few cases have reported adenocarcinoma arising from cloacal remnants and majority of these were described small solitary non-metastatic tumors. Majority were treated with radical vulvectomy and bilateral inguinal lymph node excision. In our case radical vulvectomy was not possible due to entire vulva, lower vagina involvement.

　　Conclusion: Primary cloacogenic carcinoma of the vulva is very rare, based on literature, it is considered to be of indolent nature but in our case, the tumor was aggressive with metastatic spread. It provides evidence that although it is very rare it can be aggressive. It is important for pathologist and clinicians to be aware of this entity.

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