Spontaneous uterine perforation secondary to uterine Malignant mixed mullerian tumor (MMMT) in a young unmarried female of north Indian origin: Case report and Review of literature

Carcinosarcoma presenting as uterine Perforation

Abstract

Carcinosarcomas are highly aggressive tumors occurring after menopause, at a median age of 62 years. Most frequent presenting symptom is postmenopausal bleeding, occuring in 80% to 90% cases. Less common symptoms are vaginal discharge, abdominal or pelvic pain, weight loss, and passage of tissue from vagina. On physical examination, uterine enlargement is present in 50% to 95% of patients. Diagnosis determined by biopsy of endocervical mass or endometrial curettage or most often made post-operatively by histopathological examination and immuno-histochemical study.
Appropriate treatment includes surgical staging followed by total hysterectomy with bilateral salpingo-oophorectomy, removal of pelvic and aortic lymph nodes, omentectomy and peritoneal cytology. High rate of relapse and metastasis necessitate adjuvant therapy. Due to the aggressiveness of the tumor adjuvant chemotherapy is recommended even in stage 1 and 2 lesions. The most effective protocol for chemotherapy is still under study.
Taxanes and cisplatin based chemotherapy as well as ifosphamide, along with whole pelvic irradiation, might lead to increased survival in patients with metastatic carcinosarcoma especially in stage 3 and 4. On literature search, we found only two cases of carcinosarcoma who presented with tumor rupture and acute abdominal emergency with histopathological diagnosis of carcinosarcoma uterine cervix and uterine corpus. This is a rare case with unusual presentation in a young unmarried female with no prior risk factors presenting directly as an acute abdominal emergency with uterine rupture in shock.
Our present case expanded the clinical spectrum of uterine adenocarcinomas and highlights the atypical presentation of MMMT, which may be complicated by tumour rupture and pose a surgical emergency. The preventive measures, screening, and treatment measures that apply to the usual cervical adenocarcinoma may not be suitable for mesonephric tumours. More effort is needed to clarify the etiology and nature of mesonephric adenocarcinomas and MMMTs.