Diagnosis and management of an extremely rare solitary fibrous tumor in the retropubic space of Retzius: A case report.

INTRODUCTION AND IMPORTANCE: Solitary fibrous tumors (SFTs), rare mesenchymal tumors, occur in diverse locations. Retroperitoneal and pelvic SFTs are extremely uncommon, challenging to identify due to vague clinical and radiological features. We present a high-risk SFT case in the retropubic Retzius space. CASE PRESENTATION: A 56-year-old woman with diabetes and bronchial carcinoid tumor had abdominal pain. Physical exam revealed hypogastric and pelvic masses. MRI showed a 16.5x12x8cm mass near the bladder. Initial laparotomy deemed it unresectable; biopsy indicated grade 2 FNCLCC mesenchymal tumor. In a second surgery, a 20 cm mass was successfully removed from the Retzius space. Histopathology confirmed SFT. Due to vaginal involvement, adjuvant radiotherapy was given. After 12 months, clinical and radiological follow-up showed remission. Unusual SFT location and successful treatment highlight the importance of comprehensive assessment and multidisciplinary approach in managing these rare tumors. CLINICAL DISCUSSION: Solitary fibrous tumors (SFTs) are rare mesenchymal neoplasms, often found in extremities but seldom in the abdomen. Diagnosis is challenging due to vague symptoms and imaging. This case discusses an unusual SFT location in the pelvic space, requiring surgical removal. SFTs are typically benign but can be malignant, requiring wide resection and potential adjuvant therapy. CONCLUSION: Diagnosing SFTs can be difficult because of their non-specific clinical and radiological characteristics, but imaging studies and biopsies can aid in the diagnosis. The preferred treatment for SFTs is surgical removal with clear margins, and regular follow-up is essential to watch for any signs of recurrence.

Acute myeloid leukemia after 10 years of azathioprine treatment for Crohn's disease.

BACKGROUND: Azathioprine, which is an immunosuppressive agent commonly used for chronic inflammatory bowel disease, may be associated with an increased risk of certain cancers such as hematologic malignancies. CASE REPORT: A 50-year-old man with a 27-year history of Crohn's disease had been under azathioprine treatment at a dose of 2.5 mg/kg/day (150 mg/day) since 2007, after ileocecal resection to avoid postoperative recurrences. Ten years later, the patient presented with a 3-week history of worsening general condition and fever. The physical examination revealed skin paleness and fever. The biological assessment showed pancytopenia. Hematological toxicity of azathioprine was suspected. The drug was immediately stopped. A bone marrow biopsy with immunophenotyping studies confirmed the diagnosis of acute myeloid leukemia. Chemotherapy was indicated but the patient passed away 2 weeks later. CONCLUSION: Azathioprine may be implicated in therapy-related acute myeloid leukemia. Close monitoring of its hematological toxicity, as well as patient education to adhere to this monitoring program, are crucial to detect this life-threatening complication.

Spontaneous regression of an orbital Langerhans cell histiocytosis after biopsy: A case report. Spontaneous regression of an orbital Langerhans cell histiocytosis.

Langerhans histiocytosis or Langerhans cell histiocytosis (LCH) is a rare benign pathology representing less than 1% of orbital tumors. It can cause either localized or generalized lesions, leading to the destruction of hard and soft tissues. Eosinophilic granuloma is the most benign form and the predominant clinical presentation of LCH. We report a case of eosinophilic granuloma with orbital involvement in an 18-year-old male patient. Orbital radiotherapy was initially planned, but finally it was not performed due to a spontaneous regression of the lesion after the incisional biopsy. The presented case supports an expectant attitude given the possibility of a spontaneous regression after the biopsy, especially in small lesions. However, long-term follow-up is essential given the risk of recurrence.

Prognostic factors and the role of pelvic lymphadenectomy in uterine leiomyosarcomas.

OBJECTIVES: Leiomyosarcomas are relatively rare uterine smooth muscle tumors. Surgery is the most common therapy choice for uterine leiomyosarcomas. However, controversy exists over the appropriate initial surgical management, especially about the role of lymph node sampling. The aim of our study is to analyze the prognostic factors and the role of lymphadenectomy in overall survival and in disease-free survival. METHODS: We analyzed retrospectively 31 patients suffering from uterine leiomyosarcomas at Institute of Salah Azaiez during 2000-2014. Demographic and clinical features such as age, menopausal status, stage, tumor size, and management options were examined, and pathological characteristics such as mitotic count, lymphovascular space invasion, and tumor necrosis were evaluated. RESULTS: Out of 31 patients treated for uterine leiomyosarcomas, pelvic lymphadenectomy was done for 18 patients. No para-aortic lymphadenectomy was performed. Median number of resected lymph nodes was 13 ± 7 (range: 3-27). Lymphatic metastasis was observed in 2 out of 18 patients with clinical stage IA and IIIB. The distribution of different variables (age, International Federation of Gynecology and Obstetrics stage, tumor size, mitotic count, and adjuvant treatment) between the group of patients, who had or had not lymphadenectomy done, had no significant difference. The 5-year overall survival and disease-free survival were 61% and 50%, respectively. Clinical stage, presence of lymphovascular space invasion, and lymph nodal dissection were found to be relevant for disease-free survival on univariate analysis. Only age and menopausal status were found to be a prognostic factor for overall survival. CONCLUSION: Hence, routine lymph node dissection was not generally recommended. Our study demonstrates that lymphadenectomy has a statistically significant effect on disease-free survival but not on overall survival.