ABSTRACT
Background: Rhabdomyosarcoma (RMS) is a rare high-grade malignant tumor and the most common soft-tissue sarcoma, which occurs in young girl over 5 years old. Multimodality treatment associating with surgery, chemotherapy, and/or radiotherapy culminate in a >70% overall 5-year survival. This is the first case reported in 30 years of practice in Côte d'Ivoire, low- and middle-income country (LMIC). Objective: To summarize clinical data, the significant alternative chemotherapy efficiency and difficulties related to the prognosis evaluation in an LMIC. Case: A 2-year-old girl had been examined for a large mass in the vulvar region and clitoris. We carried out a biopsy for histopathologist exam. This allows pathologic, genetic, and biological characterization of nonmetastatic botryoid rhabdomyosarcoma. A multidisciplinary team decision of neoadjuvant chemotherapy was retained combining vincristine, cyclophosphamide, and actinomycin D or alternatively with Adriamycin. After 3 weeks of chemotherapy, significant volumetric reduction of tumor was observed. Yet a surgical removal was proposed but not performed because the patient has no longer consulted our medical center and was lost to follow-up. Therefore, we cannot assess the long-term evolution and prognosis. Conclusion: Embryonal RMS (ERMS) of clitoris is a rare malignant tumor of infant. Histology and immunohistochemistry are essential for diagnostic but unavailable in our context. We want to emphasize on the difficulties encountered in treatment and prognosis assessment. The primary free surgical removal of the vulva with adjuvant chemotherapy and/or radiotherapy must then be implemented in our practice.
ABSTRACT
Background. The risk of ovarian cancer is increased in the association of ovarian tumor, ascites, and hydrothorax with the significant elevated tumor marker CA-125. However, this association can be observed in a rare clinical and benign pathological entity, that is Demons-Meigs' syndrome. Objective. To describe a rare case of Demons-Meigs' syndrome observed in our department. Methods. A black African woman of 35 years old, seventh gravida and fourth parous, underwent a total abdominal hysterectomy with bilateral salpingoophorectomy for large bilateral ovarian masses associated with significant ascites, bilateral pleural effusion, and particular highly elevated tumor marker CA-125 (1835 UI/mL) in a pronounced general alteration condition. Results. The postoperative course was uneventful characterized by a complete remission of hydrothorax and ascites with normal level of CA-125 three months after tumor excision. Histology of both masses revealed a bilateral ovarian fibrothecoma, a benign tumor of the ovary, thus confirming the diagnosis of Demons-Meigs' syndrome. Conclusion. The Demons-Meigs syndrome, although it strongly mimics the clinical picture of malignant metastatic ovarian cancer, remains a disease with benign prognosis after surgical tumor resection. This is a rare condition that must be known and recognized by practitioners to avoid unnecessary practices.
