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MOGS Chronicles | Volume 1 | Issue 1 | September 2024 21IntroductionHerlyn-Werner-Wunderlich syndrome also known as obstructed hemivagina and ipsilateral renal agenesis (OHVIRA) syndrome, is a rare congenital anomaly characterized by a triad of Uterus didelphys, obstructed hemivagina and ipsilateral renal anomaly.Malignancies in OHVIRA syndrome are rare with only 6 reported cases in literature, all of which were cervical cancer.Case ReportWe present a case of a 42 years old divorced, nulligravida, who came with complaints of persistent mid-menstrual blackish vaginal bleeding from last 6 months. At the age of 28 years, she was diagnosed with OHVIRA syndrome i.e. Uterus didelphys with a non-communicating horn with hematosalpinx along with ipsilateral renal agenesis and managed surgically by drainage of hematocolpos with marsupialization of hemivagina. She was a known case of hypothyroidism on treatment. There was no history of cancer in the family.Initial pelvic examination revealed foul-smelling discharge and a mass arising from the cervix. Ultrasound abdomen and pelvis revealed pyometra measuring 8.3 %u00d7 8.5 %u00d7 5.8 cms ~214 cc seen in endometrial cavity with left kidney. Magnetic resonance imaging [Figure 1] showed a normal sized uterine horn with normal endometrial stripe with a small anterior wall uterine fibroid. E/o bicollis noted, distended vaginal cavity with large heterogeneous polypoidal mass with heterogeneous post-contrast enhancement. This is seen extending towards the left cervical cavity with presence of vessels in the cranial end. There is beaking seen in upper end of vagina with circumferentially thickened and mildly t2 hyperintense vaginal walls, with possibility of lower vaginal narrowing.Positron emission tomography scan showed a hypermetabolic polypoidal mass arising from the uterine cervix extending into the vagina. Patient underwent examination under anesthesia with excision of mass which was seen involving the anterior and lateral walls of the cervix and a frozen section was sent which was suggestive of malignancy, s/o possibility of poorly differentiated carcinoma [Figures 2 and 3]. Histopathology reported carcinosarcoma of the vagina. Immunohistochemistry showed the carcinoma component-high grade adenocarcinoma expressing ck, ema. pax8 and p53 (strong diffuse expression mutant expression), ini 1 and p16. The sarcomatous component- high-grade sarcoma with immunoprofile of a homologous high grade stromal sarcoma. The tumor cells express cd10 and cyclin d1 strongly. They are immuno negative for pr, sma, desmin, s-100 protein, cd 34 and ck.Malignancies in obstructed hemivagina and ipsilateral renal agenesis are rare, with only six reported cases in literature, all of which have been cervical cancers. A 42 years old, divorced, nulligravida, with a history of Uterus didelphys with a non-communicating horn with hematosalpinx along with ipsilateral renal agenesis with drainage of hematocolpos with marsupialization of hemivagina at the age of 28 years. Presented with persistent mid-menstrual blackish vaginal bleeding. Initial pelvic examination revealed foulsmelling discharge and a mass arising from the cervix. Magnetic resonance imaging pelvis showed a distended vaginal cavity with a large polypoidal mass extending towards the left cervical cavity. Positron emission tomography scan showed a hypermetabolic polypoidal mass arising from the uterine cervix extending into the vagina. Mass which was seen involving the anterior and lateral walls of the cervix was excised. Histopathology report was suggestive of carcinosarcoma of the vagina. A radical hysterectomy with pelvic lymph node dissection was planned. Tumor was inoperable with no discernable plane of dissection extending up to the introitus. The patient is undergoing chemotherapy. This is a rare case of a vaginal carcinosarcoma in a Mullerian anomaly with an aggressive course.Keywords: Herlyn-Werner-Wunderlich syndrome, Obstructed hemivagina and ipsilateral renal agenesis, Vaginal carcinosarcoma, Vaginal malignancyCarcinosarcoma in OHVIRA syndrome: an EnigmaRahat Sayyad1, Nozer Sheriar1, Nikhil Dharmadhikari2, Zubin Sheriar11Department of Obstetrics and Gynecology, Holy Family Hospital, Holy Family Hospital St Andrew%u2019s Road, Bandra (West), Mumbai, Maharashtra, India, 2Department of Surgical Oncology, Faculty of Obstetrics and Gynaecology, Holy Family Hospital, St Andrew%u2019s Road, Bandra (West), Mumbai, Maharashtra, IndiaCase ReportAddress for correspondence: Dr. Rahat Sayyad, Holy Family Hospital, Holy Family Hospital St Andrew%u2019s Road, Bandra (West), Mumbai, Maharashtra, India. Mobile: +91-8779555401. E-mail: rahat.sayyad@gmail.comAbstract