Metachronous Cancer of Carcinosarcoma of the Uterus with Adenocarcinoma of Ovary: A Rare Case Report

Abstract

Carcinosarcoma of the uterus (also known as malignant mixed Mullerian tumor, MMMT). Uterine carcinosarcoma is a rare, highly aggressive, rapidly progressing neoplasm associated with a poor prognosis that has not significantly improved in the past 30 years despite advances in imaging and adjuvant therapies.

Our case is a Metachronous cancer where diagnosed two cancers at different time and she is on etoposide orally daily, She will evaluation after three months to see the response.

Keywords: Uterine carcinosarcoma; Metachronous cancer; Malignant mixed Mullerian tumor; Etoposide

Introduction

Endometrial cancer is usually diagnosed at an early stage because patients present for consultations at the hospital with abnormal vaginal bleeding or discharge. It has been reported that primary cancers of the endometrium and ovary coexist in approximately 10% of all women with ovarian cancer and in 5% of all women with endometrial cancer [1]. A malignant mixed Müllerian tumor (MMMT), also known as malignant mixed mesodermal tumor. MMMT is a malignant neoplasm found in the uterus, the ovaries, the fallopian tubes and other parts of the body that contains both carcinomatous and sarcomatous components [2]. Synchronous endometrial and ovarian cancer (SEOC) is defined as the simultaneous presence of these two cancers at the time of diagnosis as opposed to metachronous cancer where these two cancers are diagnosed at different chronologic time points [3]. To date, discrimination between these pathologies is possible only after surgery by histopathological examination [4]. The overall prognosis of uterine carcinosarcoma is poor, even with the best of care, due to its aggressive behavior [5].

Case Presentation

A 60-year-old female, post menopause, presented with vaginal offensive since two months pre diagnosis, not associated with pain or fever or inguinal lymph nodes enlargement. Comorbidity is AHT on regular treatment, She had no cancer history in her family. Abdomen and pelvic CT-scan results - pre operation on 25-1-2021 was Bulk uterus 15.5x10x9 cm, diffuse inhomogenicity of uterine-the process involved all parts of uterus (Fundus, body, cervix), no adnexal masses, no ascites. She is underwent to total abdominal hysterectomy with bilateral salpingo-oophorectomy (TAH+BSO), the histopathology result was carcinosarcoma (malignant mixed mesodermal tumor (MMMT), the Cervical and Ovaries were no significant pathological changes and the Immunohistochemistry (IHC) was carcinosarcoma (Endometrial mixed mesodermal tumor-MMMT. The results were; Pan CK, CK7, EMA are positive and Her2, ER, PR were negative. Ejection Fraction of her heart was 61%, the tumor markers were CEA=1.85, CA125=18.

CT-Scans post operation

Chest-CT scan was normal and the abdomen and pelvic were no residual or metastasis. She had received six cycles of Paclitaxel with carboplatin, with well tolerance and good response and she reached to complete cure. The evaluation after completed her treatment by CT-scans were normal, no any residual or metastasis. Unfortunately, after three months our patient was complained from abdominal pain, vomiting and diarrhea, not responded to any medical treatments, later on she developed signs of intestinal obstruction, so she entered to Operation Theater as an emergency case. On operation appeared mass in her intestine, which complete resected. The pathologic study and IHC revealed adenocarcinoma ovarian origin, CEA 4, CA125 was high 320. The diagnosis is ovarian cancer. Our patient was very ill and unfit for aggressive chemotherapy protocol. So we advised to her Etoposide 50mg tab orally daily and recommended to do CA125 monthly and evaluate by CT-Scans after three months from this treatment.

Discussion

Uterine sarcomas represent about 8% of uterine cancers, with an incidence of about 0.4 per 100,000 women [6]. Uterine carcinosarcoma (formerly called malignant mixed mullerian tumor) is a rare tumor of the gynecologic tract, the name is derived from observations of the embryonic female genitalia accounts for <5% of uterine malignancies and typically arises in the uterine corpus or in the cervix [7]. Uterine carcinosarcomas are highly aggressive, rare, biphasic tumors composed of epithelial and mesenchymal elements believed to arise from a monoclonal origin [8]. It is rarely encountered and accounts for only 1-3% of all uterine malignant tumors [9]. Our case was diagnosed as carcinosarcoma and after months from the cure it appeared ovarian carcinoma. Risk factors associated with this disease are similar to those encountered in endometrial carcinoma, and include obesity and prolonged estrogen exposure [10]. On the contrary, oral contraceptives are reported to provide a protective effect against these tumours [11]. Chemotherapy effectiveness in sarcomas differs greatly from that in endometrial carcinomas, with increased toxicity [12].

Chemotherapy is used but there is uncertainty as to the best treatment [13]. Our case treated by Paclitaxel and carboplatin with good response and tolerance. Pazopanib 800mg oral daily achieved objective response in about 6% of patients with metastatic soft tissue sarcoma in a phase III trial [14]. In this case we started Etoposide 50mg orally daily. CT is the preferred modality for staging, followup, and evaluation of distant metastases [15]. No correlation was observed between biomarkers and tumor response or progression [16]. The prognosis is often poor, with 30-40% of cases having extra uterine involvement at the first presentation [17].

Conclusion

Carcinosarcomas though rare, representing less than 5% of all uterine tumors. It is an aggressive neoplasm that has an extremely poor prognosis, 5-year survival is about 35%. It is difficult to make the differential diagnosis of this tumor because of its rarity and lack of specific imaging features that might distinguish it from other malignant uterine tumors. It has limited therapeutic options. A rare case of uterine carcinosarcoma is reported in this study with Metachronous cancer adenocarcinoma of ovarian origin.

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