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Summary: Clear cell sarcoma or melanoma of soft tissues is a rare tumor in young adults, mainly located in the extremities. We report a new case observed in a 15-year-old teenager who had injured her left knee 1 month before hospitalization. The patient consulted for deformation of the left knee with pain and limitation in flexion. A CT scan of the knee showed a hypodense mass of the anterior compartment of the left leg. The histological and immunohistochemical study of the biopsy fragment concluded in clear cell sarcoma of the soft tissue. The histogenesis of this tumor continues to be debated and raises diagnostic challenges for the pathologist who must eliminate melanoma metastases, whose prognosis is different. [ABSTRACT FROM AUTHOR]

We summarize here a 15-year-old Moroccan girl who was diagnosed with Lafora disease based on histopathological findings of skin biopsy. Diagnosis was confirmed by histopathological analysis of axillary skin biopsy, which revealed typical Lafora bodies in the epithelium of the sweat gland ducts (Fig). Epilepsy Res. 145, 2018, 169-177 3 M.A. Garcia-Gimeno, E. Knecht, P. Sanz, Lafora disease: a ubiquitination-related pathology. [Extracted from the article]

Résumé: La mucormycose est une infection fongique opportuniste rare, cliniquement polymorphe et rapidement extensive et destructrice. Elle est due à des champignons du groupe des mucorales présents dans l’environnement et survient en général sur un terrain immunodéprimé. Le diagnostic, souvent difficile et tardif, repose sur l’examen mycologique et anatomopathologique. Ce garçon de 10ans avait été admis pour une éruption érythémato-squameuse prurigineuse siégeant au niveau inguinal droit, associée à une adénopathie satellite et à un lymphœdème du membre inférieur droit. L’étude histologique de la biopsie cutanée avait révélé la présence d’une réaction granulomateuse avec des filaments mycéliens. L’examen mycologique du prélèvement de la lésion cutanée avait mis en évidence des filaments mycéliens de type mucorale et isolé une souche d’Absidia corymbifera. Dans le cadre du bilan d’extension, la tomodensitométrie (TDM) abdomino-pelvienne avait objectivé une extension musculaire avec englobement vasculaire et urétéral. Le diagnostic de mucormycose cutanée iléo-fémorale avec extension endo-pelvienne avait été retenu. Le bilan immunologique était normal. L’enfant avait été traité par de l’itraconazole pendant 3mois puis de l’amphotéricine B injectable pendant 1mois et l’évolution clinique et radiologique avait été favorable. La mucormycose est une infection fongique rare, notamment dans sa localisation cutanée. Elle survient exceptionnellement chez l’immunocompétent et se manifeste cliniquement par une éruption vésiculo-pustuleuse évoluant vers l’ulcération. Le diagnostic est confirmé par l’étude mycologique et anatomopathologique. Le traitement repose sur un antifongique associé à l’exérèse chirurgicale des tissus infectés et nécrotiques. [ABSTRACT FROM AUTHOR]

Summary: Mesenchymal hamartoma of the liver is a rare, benign tumor that presents mostly before the age of 2 years. Its pathogenesis is poorly understood. We present the case of a 2.5-year-old female patient who had a large cystic mass of the liver of which the hamartomatous nature was confirmed by the pathological examination of the surgical specimen. We discuss the clinicopathological, imaging, and histological features of this unusual tumor through a review of the literature. [Copyright &y& Elsevier]

Clear cell sarcoma of the kidney (CCSK) also called a “bone-metastasizing renal tumor of childhood” is the second common pediatric renal neoplasm. This tumor is associated with a higher rate of relapse and a wider distribution of metastases than Wilms’ tumor.Patients and methods. – We have reviewed records of 13 cases of CCSK among 277 renal tumors (5%) diagnosed at the children’s hospital of Rabat between 1990 and 2002.Results. – The median age at diagnosis was 14 months (5 months–9 years). The male to female ratio was 5.5:1.00. Abdominal mass, usually the first physical finding, was associated with hematuria in four cases. No congenital malformation syndrome or familial Wilms’ tumor were observed. Imaging studies found out seven right and six left intrarenal processes. Preoperative chemotherapy was given according to the SIOP9, SIOP93-01 and GFAOP 98 protocols. Twelve of 13 children underwent nephrectomy. Tumor measurements varied through 450–3450 g and 7–26 cm. The classic morphologic pattern was seen in nine cases (69%). The distribution local stage was I: three cases; II: three cases; III: six cases; IV: one case. Postoperative chemotherapy and radiotherapy (21 600–30 600 cGy) was done in 10 cases. With a median follow up of 44 months, four patients showed bone metastases (31%), four are alive in CR, four are lost for follow up and five dred.Conclusion. – CCSK remains the pediatric renal tumor most frequently misdiagnosed. Its aggressiveness and its ability to give bone metastases need to recognize early this diagnosis for an adapted treatment. [Copyright &y& Elsevier]

Summary: We report on a rare case of botryoid Wilms tumor extending into the duodenum. This uncommon macroscopic form of nephroblastoma consists entirely of a polypoid renal intrapelvic mass. The main differential diagnosis of this unusual tumor is botryoid rhabdomyosarcoma. A 14-month-old boy presented with a painful abdominal mass. Radiology revealed a large heterogeneous mass in the renal calyx, protruding into the ureter. A right radical nephroureterectomy was carried out. The tumor was found to extend into the 2nd portion of the duodenum. The pathologic diagnosis was mixed type nephroblastoma, SIOP 2001 stage III. The patient was given a course of postoperative chemotherapy. No local recurrence or metastatic spread has been detected after 2 years. Only few such cases have been previously reported, some of them extending into the bladder. To our knowledge, botryoid nephroblastoma has not previously been described as extending into the digestive system. [Copyright &y& Elsevier]

Abstract: Thymic hyperplasia in response to stress is a well known phenomenon. Thymic hyperplasia has also been described after chemotherapeutic treatment for malignancies in children. Case report. – A three-year-old girl was followed up from the age of 18 months for a left kidney nephroblastoma treated by combination of chemotherapy (vincristin, actinomycin and adriamycin) and surgery. Assessment at the end of treatment was normal. Four months after the end of treatment, pulmonary radiography showed mediastinal enlargement, which was shown to originate in the thymus at thoracic CT scan. A recurrence of the disease was suspected. Biopsy showed thymic hyperplasia without evidence of tumor cells. Mediastinal enlargement then disappeared spontaneously 2 months later. Conclusion. – Thymic hyperplasia occurring during remission of a cancer treated by chemotherapy is a diagnostic dilemma as it suggests mediastinal reccurence of the disease. Needle aspiration cytology is an appropriate investigation in thymic hyperplasia. No steroid therapy should be used before histologic diagnosis of thymic hyperplasia. [Copyright &y& Elsevier]