Primary Peritoneal Carcinosarcoma: A Case Report

Introduction: Carcinosarcoma, also known as malignant mixed Mullerian tumor (MMMT) is a malignant biphasic neoplasm consisting of carcinomatous and malignant non-epithelial components of mesenchymal origin. MMMTs typically arise from the female genital tract in patients over 40 years old. Primary extragenital MMMTs are extremely rare with published literature totaling 40 reported cases. The primary peritoneal carcinosarcoma is an aggressive tumor as patients with this tumor have an average survival of 7.6 months. Surgical debulking is the mainstay of treatment for these tumors and systemic chemotherapy is advised in all cases.Case: A 48-year-old Amish female presented with 5 day history of bloating and abdominal pain superimposed on a 1 year history of worsening fatigue and intermittent bloody bowel movements. She was found to have a pelvic mass on physical exam. Computed tomography scan of the abdomen and pelvis that demonstrated stricturing of the sigmoid colon, and a large multi-cystic mass in the midline pelvis measuring 12.5 × 9.9 × 11.7 cm. Colonoscopy showed stenosis due to external compression without intraluminal lesion. CEA and CA 125 levels were elevated and CA 19-9 was normal. Exploratory laparotomy was performed with en-bloc resection of a 15 cm mass originating from the sigmoid colon mesentery with several other small tumor deposits throughout the mesentery. Pathology diagnosed primary peritoneal carcinosarcoma Mullerian-type with three positive lymph nodes.Conclusion: Malignant mixed Mullerian tumor (carcinosarcoma) caries a universally grim prognosis. Herein, we report a unique case of primary peritoneal carcinosarcoma and discuss the work-up and surgical management of this rare tumor.

Primary Carcinosarcoma of the Spleen: A Rare Case Report of Incidental Finding after Splenic Trauma

Primary carcinosarcoma of the spleen is a rare, aggressive splenic malignancy. To date, seven cases have been reported in the literature. We report a first case of primary carcinosarcoma of the spleen in France. A 75-year-old woman with a medical history of hysterectomy for uterine adenocarcinoma presented with left hypochondrium pain following blunt abdominal trauma. A splenic mass was noted on computed tomography (CT) scan. A splenectomy was performed by laparotomy. Histology revealed a malignant mixed Mullerian tumor. The PET scan allowed us to confirm that it was a primary lesion of the spleen. She is currently undergoing adjuvant chemotherapy despite the tumor progression. The interest of this case lies in the rarity of primary carcinosarcomas of the spleen and the circumstances of its diagnosis.

Carcinosarcoma of the Uterus: An Elusive Diagnosis

Introduction: Carcinosarcoma or Malignant Mixed Mullerian Tumor (MMMT) of the uterus is a rare malignant tumor comprising both carcinomatous and sarcomata’s components. Worldwide it accounts for two to five percentages of all uterine malignancies. However, there is a paucity of reports in Nepalese literature. Case report: A 62 year’s postmenopausal woman with diabetes and hypertension presented with urinary symptoms for two months and passage of fleshy mass per vagina for two days. Ultrasound was inconclusive, whereas, Magnetic Resonance Imaging (MRI) showed an endo-cervical mass. Pre-operative biopsy of the masssuggested leiomyosarcoma. Abdominal hysterectomy with bilateral salpingo-oophorectomy and pelvic andpara-aortic lymphadenectomy was performed. Per-operatively, an atrophic uterus, a dimple in the funds, andballooning of the lower uterine segment and vagina were noted. The cut section showed an exophytic polypoidalmass with base in the fundus. Histopathology revealed Stage IA carcinosarcoma with aggressive mitotic figures. Immunohistochemistry confirmed the diagnosis of carcinosarcoma with a heterogonous rhabdomyosarcomatouscomponent. Adjuvant chemotherapy and radiation therapy was advised. Conclusion: The preoperative diagnosis of carcinosarcoma or MMMT was difficult and might be missed on biopsy as well. Owing to its aggressive nature and higher rates of post-surgical recurrence, carcinosarcoma accounts for around one-fifth of deaths due to uterine malignancies. Surgery is the primary treatment modality, yet much study is needed before evidence-based adjuvant management for improving its outcome is established.

Ovarian malignant mixed Müllerian tumor: a rare case report from Tanzania

Malignant mixed Müllerian tumor of the ovary is rare aggressive tumor that is histologically defined by the presence of malignant epithelial and stromal components. We report a 37-year-old woman who consulted our facility complaining of abdominal distention and a painful palpable mass over her lower abdomen. Physical examination including computerized tomography revealed a complex cystic mass lesion on the left ovary with extensive omental involvement. Ovarian cancer was suspected and the patient underwent debulking surgery. The histopathology of the specimen revealed a high-grade tumor composed of both malignant epithelial and sarcomatous elements. Both epithelial and stromal components stained positive for p53 immunostaining. Before the initiation of chemotherapy, on 5th day postoperation, the patient was found unresponsive. The stage of the disease seems to be the most important prognostic factor, thus emphasis should be made to identify it in earlier stages.

Renal Hemangioblastoma with Mixed Mullerian tumor of endometrium: A tale of two rare primary tumors

Introduction: Renal hemangioblastoma (RH) is a very rare benign tumor. Hemangioblastoma most commonly occur in the central nervous system (CNS) and only few cases of RH have been reported as they occur most commonly as asymptomatic masses found incidentally. Mixed Mullerian Tumor (MMT) of the uterus is a rarer and aggressive form of uterine malignancy. The detection of two primary rare tumors incidentally is a rare entity. Case presentation: A 50 years female presented with abnormal uterine bleeding which on endometrial sampling was diagnosed as a rare variety of endometrial cancer i.e., MMT or uterine carcinosarcoma. On preoperative imaging, a renal mass was also detected which was highly vascular and was mimicking Renal Cell Carcinoma (RCC). Fine needle aspiration cytology (FNAC) was done from the renal mass to differentiate between RCC and metastasis but it showed only blood cells. Patient underwent staging laparotomy for endometrial cancer and frozen section examination of the renal mass which was inconclusive with few atypical cells and thus patient underwent radical nephrectomy too. Histopathological examination revealed it to be a RH which is a very rare benign tumor. Discussion: RH is a rare benign tumor which doesn’t require any treatment in majority of the patients. Only 26 cases of RH outside the CNS have been reported till date. MMT is a rare aggressive uterine tumor with an incidence of 1-2 % of all uterine neoplasms, which metastasizes early and thus early identification and treatment is the key. RH needs to be differentiated from RCC to avoid over treatment. Morphological findings are similar in both, preoperative FNAC, PET scan and intraoperative frozen section can be utilized to differentiate in between two in well circumcised tumors and in high suspicion. Occurrence of renal mass as an incidental finding in the preoperative work up of uterine malignancy directed us to the differentials of metastasis or another histologically distinct primary tumor. The presence of two rare primary tumors i.e., RH and MMT in the same patient which are unrelated is a rare entity.