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Persistent Mullerian duct syndrome (PMDS) is a congenital disorder related to male sexual development. PMDS is usually diagnosed during an inguinal hernia cure. The diagnosis of PMDS following a testicular germ cell tumor is less common. We report the cases of three infertile male patients who were diagnosed with PMDS after surgery for germ cell tumors. They were 39, 27, and 37 years old men with a medical history of neglected cryptorchidism. All patients had a male karyotype and the ELISA test for the anti-Mullerian hormone was undetectable. Patients underwent chemotherapy followed by resection of residual mass in one patient. One patient is currently alive and disease-free. The two other patients died of systemic relapse. These cases highlight how early recognition and treatment of PMDS can prevent malignant germ cell tumors. The diagnosis of PMDS relies on a systemic assessment and analysis of mutations in the gene coding for AMH and AMHR-II. Key words: Persistent Müllerian duct syndrome (PMDS), anti mullerian hormone, germ cell neoplasm.

Objectives: Retroperitoneal sarcomas (RPSs) are rare mesenchymal tumors. The objective of this study was to discuss the different clinical, therapeutic and prognostic aspects of RPS in our institution., Methods: This was a retrospective study conducted at the Department of Medical Oncology in the Habib Bourguiba University Hospital in Sfax, including 19 patients who were treated for RPSs between 1999 and 2016., Results: The median age was 49 years (range: 18-83 years); 68.4% of the patients were female. The commonest symptom was abdominal pain (88%) and the median tumor size was 15 cm (range: 4-30 cm). Complete resection was achieved in only five cases (26.3%). The most common histological subtypes were liposarcoma (47.4%) and leiomyosarcoma (26.3%). Eight patients had a high-grade tumor (G2 = 2 or G3 = 6). Adjuvant radiotherapy was administered in 5 patients (26%). Seventeen patients were treated with chemotherapy; six received chemotherapy in an adjuvant treatment, three as a neoadjuvant treatment, and eight were treated during the palliative phase. Relapse was observed in 58% of cases. For all patients, the overall survival (OS) was 89.5% at 1 year and 40.3% at 5 years. Prognostic factors influencing OS were sex ( p  = 0.037), resection margins ( p  = 0.02), recurrence ( p  = 0.042), and radiotherapy ( p  = 0.023). In multivariate analysis, radiotherapy ( p  = 0.031) and histological subtype ( p  = 0.028) were independent factors influencing OS and disease-free survival (DFS) respectively., Conclusion: We showed that the treatment of RPSs relies on surgery with complete resection. Other factors, such as radiotherapy and the occurrence of relapse, also have an impact on OS. To facilitate surgery and to obtain negative resection margins, preoperative radiotherapy is indicated in selected patients with a high risk of relapse. Further prospective trials are warranted to select optimal therapies with less toxicity and better efficacy in reducing recurrences, mainly at a local level., (© 2022 [The Author/The Authors].)

Angiosarcoma is a rare malignant tumour occurring in less than 2% of soft tissue sarcomas. Angiosarcoma involving the kidney usually represents metastasis from skin or visceral primary lesions, while angiosarcoma primarily occurring in the kidney is a very rare neoplasm. We report a case of angiosarcoma of the right kidney in a 59-year-old male. The computed tomography scan showed a solid tumour with a low increased density after administration of contrast medium. Histological examination of the piece of nephrectomy confirmed the diagnosis.

Introduction: Cystic lymphangioma is a rare benign vascular tumor that may arise in various sites, revealed at any age. Abdominal locations represent less than 10% of the cases preferentially involving the mesentery. We report a case of primary lymphangioma arising from the kidney., Presentation of Case: A 50-year-old woman was admitted for severe left lumbar pain. Abdominal ultrasonography and computed tomography revealed a 6×10 cm multiloculated cystic mass with hydronephrosis, extending along the left renal hilum. Surgical exploration revealed a retroperitoneal cystic tumor. Anatomopathologic examination concluded it to be a cystic lymphangioma., Discussion: The cystic lymphangioma is a benign malformative tumor of the lymphatic system. Surgery is the best curative treatment with complete excision, the prognosis is excellent., Conclusion: Primary renal lymphangioma is exceedingly rare. Medical imaging has certain limits for the diagnosis which required histological confirmation. The treatment of choice is surgical., (Copyright © 2012 Surgical Associates Ltd. Published by Elsevier Ltd. All rights reserved.)

Mucinous tubular and spindle cell carcinomas (MTSCC) are low-grade renal epi-thelial neoplasms with approximately 100 documented cases reported in the literature. We report a case of MTSCC in a 79-year-old patient in association with a renal tuberculosis infection that has never been reported. Further investigations are needed to determine the frequency and true prognosis of these tumors.

Objective: To study the evolution of impaired renal function after external continent urinary diversion (Mitrofanoff principle) (ECUD-M) associated with ileocystoplasty., Patients and Methods: Over 18 years from 1992 to 2009, ECUD-M with ileocystoplasty was performed in 120 patients with mean age of 25.5 years. Renal impairment was evident in 43 patients (17 children and 26 adults)., Results: Ninety percent of patients demonstrated a neurologic bladder and mild to moderate renal failure. Initially, all patients underwent continuous bladder drainage for a mean of 3 weeks. Renal function improved in 35 patients, although with persistent mild renal insufficiency. The other patients demonstrated moderate persistent residual renal insufficiency. During a mean follow-up of 10 years (range, 1-18 years), renal function returned to normal in 13 patients, stabilized at lower values in 15, and remained moderate in 5. After a mean follow-up of 8 years (range, 6-12 years), renal failure gradually worsened, increasing to higher values in 6 patients and leading to hemodialysis in 4. One patient underwent living-donor kidney transplantation, with good evolution., Conclusion: ECUD-M with ileocystoplasty can lead to normalization unless stabilizationof impaired residual renal function by eliminating the obstructive factor provides self-adequate management of the diversion. The procedure delays for the need forhemodialysis therapy, and enables patients to prepare for kidney transplantation into a previously reconstructed lower urinary tract., (Copyright © 2011 Elsevier Inc. All rights reserved.)