About a Large Botryoid Rhabdomyosarcoma in a Little Girl: Management Difficulties and Literature Review.

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.

Gastrointestinal Stromal Tumor Associated with Neurofibromatosis Type 1 Simulating a Neurofibrosarcoma in a Black African Adult Patient.

Gastrointestinal stromal tumor (GIST) is the most prevalent mesenchymal tumor encountered in patients with neurofibromatosis type I (NF1). This association is rarely reported in black African adult patients. Clinical signs such as abdominal pain, intestinal bleeding, and palpable abdominal nodules may contribute to the diagnosis of GIST. However, these clinical signs may also indicate the presence of other solid intestinal tumors such as a neurofibrosarcoma which is an intestinal complication of NF1. The immunohistochemistry staining of the resected tissue is mandatory that allows establishing a correct diagnostic of GIST. The immunohistochemistry is also a challenging method in African countries with limited resources. Herein, we report a case of multiple intestinal involvement of GIST associated with NF1 revealed by a recurrent intestinal bleeding in a black African adult patient. The GIST diagnosis was suspected on a computed tomography scan images and laparotomy findings, which were confirmed by histopathological and immunochemistry examinations of the resected nodules. The immunohistochemistry staining of the tissue was positive for CD34 and CD117 indicating the presence of GIST in black African patient with NF1. The surgical treatment was consisted of a double intestinal resection with anastomosis that removed all palpable nodules located on the intestinal antimesenteric wall. No additional chemotherapy was administered to the patient who is so far under follow-up. The patient has not presented any episode of intestinal bleeding since the surgical treatment. We emphasize in this case report the importance of a recurrent intestinal bleeding in patient with NF1 that may indicate the presence of GIST and, the need of performing a large intestinal resection, as the most valuable treatment in limited resource countries.

Metastatic thyroid carcinoma presented as a large craniofacial mass: Case report and CT findings.

Cranial metastases are uncommon locations of thyroid papillary carcinoma. They significantly reduce patients' survival, especially in a context of late management. We report the case of a 46-year-old woman who presented an anterior cervical swelling, then a progressive large craniofacial mass for more than 5 years without any treatment. The imaging performed showed a large craniofacial tissue mass with frontotemporal and right orbital bone destruction extended to the face sinuses and the underlying extradural space without brain involvement; the second thyroid tumor right site had similar imaging characteristics to the cranial mass. The diagnosis of papillary carcinoma with craniofacial metastasis was retained after cranial biopsy and thyroid cytology. At this stage, a cranial surgery has not been proposed but a palliative hormonal treatment. The patient died 2 weeks after diagnosis. This case illustrates the relevant role of imaging in the assessment of thyroid tumors and the value of early management for improving patients' survival.

Ovarian tuberculosis mimicking a malignant tumour.

There has been reported increased incidence of ovarian tuberculosis in the tropics since the advent of HIV/AIDS disease. We report a case of bilateral ovarian tuberculosis associated with a single right kidney of uncertain origin in an immunocompetent 15-year-old generally healthy-looking girl. Abdominopelvic scan was equivocal about the diagnosis of the lesion as it failed to differentiate it from malignancy. Tuberculin and histopathology were necessary to confirm the diagnosis of ovarian tuberculosis. Antituberculous medical therapy successfully resolved the disease.

Bilateral Ovarian Fibrothecoma Associated with Ascites, Bilateral Pleural Effusion, and Marked Elevated Serum CA-125.

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.

Severe skin forms of psoriasis in black africans: epidemiological, clinical, and histological aspects related to 56 cases.

Bacground. Psoriasis is an erythematosquamous dermatosis of chronic development. In sub-Saharan Africa, few studies have been focused on complicated forms of psoriasis. Objective. The aim is to describe epidemiological, clinical, and histological features of severe skin forms of psoriasis in Cote d'Ivoire. Material and Methods. The study was both cross-sectional and descriptive, that focused on patient admitted to the dermatology unit for complicated psoriasis, from January 1st, 1986, to December 31th, 2007. Results. Fifty-six patients admitted to hospital for severe skin forms of psoriasis were recorded and included in our study over 7.503 patients hospitalized during the study period. They represented 0.75% of cases. The average age was 39.6 ± 3.3 years. There were 49 male (87.5%) and 7 female patients (12.5%) with a sex ratio of 7. At socioprofessional level, 48 patients (87.5%) were from category 1. Patients' history was dominated by the psoriasis vulgaris. Physical and general signs were dominated by itching (58.9%). The three severe skin forms were observed with predominant erythrodermic psoriasis (60.7%). Fifteen patients (34.9%) were HIV positive. Conclusion. Severe skin forms of psoriasis are rare in our setting. But in the quarter of HIV-positive patients, they are dominated by the erythrodermic psoriasis.