[Iliofemoral cutaneous mucormycosis with endopelvic extension in an immunocompetent child]. / Mucormycose cutanée ilio-fémorale avec extension endo-pelvienne chez un enfant immunocompétent.

Mucormycosis is a rare opportunistic fungal infection with clinical polymorphism and is rapidly extensive and destructive. It is caused by fungi of the mucorales group in the environment and generally arises in the context of immunosuppression. Often difficult and late, diagnosis is based on mycological and histological examination. We report the case of a 10-year-old patient admitted for a pruritic erythematous scaly eruption located in the right inguinal area associated with satellite lymphadenopathy and lymphedema of the right lower limb. The histological study of the cutaneous biopsy revealed a granulomatous reaction with filaments. The mycological examination of the collection of the cutaneous lesion showed mucorales filaments and a stump of Absidia corymbifera was isolated. Abdomino-pelvic CT showed muscular extension with vascular and ureteral englobement. The diagnosis of cutaneous mucormycosis was made. Immunological investigations were normal. Treatment included itraconazole for 3months followed by IV amphotericin B for 1month, with favorable clinical and radiological progression. Mucormycosis is an uncommon fungal infection whose cutaneous localization is rare. It occurs exceptionally in immunocompetent patients and is clinically manifested by a vesicular and pustular rash progressing to ulceration. The diagnosis is confirmed by mycological and histological studies. Treatment consists of antifungal therapy associated with surgical excision of necrotic and infected tissue.

[Botryoid Wilms tumor extending into the duodenum]. / Néphroblastome botryoïde étendu au duodénum.

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.

[Clear cell sarcoma of soft tissues: a case report]. / Le sarcome à cellules claires des parties molles à propos d'un cas.

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.

[Mesenchymal hamartoma of the liver in a child: a case report]. / Hamartome du foie chez l'enfant : à propos d'un nouveau cas.

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.

[Thymic hyperplasia following treatment for nephroblastoma]. / Hypertrophie thymique après une chimiothérapie pour un néphroblastome.

UNLABELLED: 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.

[Clear cell sarcoma of the kidney. A study of 13 cases]. / Sarcome rénal à cellules claires. A propos d'une série de 13 cas.

UNLABELLED: 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 died. 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.

[Chondromyxoid fibroma of bone: a rare benign bone tumor in children]. / Le fibrome chondromyxoïde de l'os: une tumeur osseuse rare de l'enfant.

Chondromyxoid fibroma is a rare benign tumor that is typically found in the metaphyseal ends of long tubular bones, such as the tibia. The radiographic appearances are those of a single, lytic lesion with lobulated margins, septations, cortical expansion and a sclerotic rim. The classic histological feature of a chondromyxoid fibroma is stellate or spindle-shaped cells arranged in lobules in a myxoid or chondroid background. Two cases are presented here: 8, and 12-year-old patients, both with lesions in the proximal tibia. The first case showed an unusual feature: it was diaphyseal chondromyxoid fibroma. In the second case, the lesion was metaphyso-diaphyseal. The differential diagnosis includes chondroblastoma, myxoma, aneurysmal cyst as well as chondrosarcoma. A surgical conservative treatment with complete excision is recommended even in case of recurrence.

[Pleuropulmonary blastoma: possible interest of neoadjuvant chemotherapy. A case report]. / Blastome pleuropulmonaire: intérêt possible d'une chimiothérapie néoadjuvante. A propos d'un cas.

Pleuropulmonary blastoma is a very exceptional anatomoclinical and histological entity recently distinguished from adult pneumoblastoma. This tumor, observed in children aged less than 15, can involve the lung, the pleura, or the mediastinum and is characterized by a very poor prognosis. We report the case of a 4-year-old girl who developed pleuropulmonary blastoma which was discovered in a context of respiratory distress. Standard chest x-ray revealed an opacity covering the entire left lung. Histology and immunohistology led to the diagnosis of pleuropulmonary blastoma with several components: blastematous, malignant mesenchymatous with pluridirectional differentiation, and benign epithelial tissue. Treatment consisted in preoperative chemotherapy and radiotherapy to reduce tumor volume. This neoadjuvant treatment is not widely reported and its relatively favorable result allowed tumor resection. This approach might be useful in similar cases.

[Visceral leishmaniasis and malnutrition: a case report]. / Leishmaniose viscérale et malnutrition: à propos d'une observation.

BACKGROUND: Visceral leishmaniasis occurring in malnourished subjects can have an uncommon course, which explains difficulties in its diagnosis. CASE REPORT: A 22-month-old infant was admitted because of malnutrition and prolonged fever. The bacteriological investigation was negative. When his nutritional status improved, he developed a splenomegaly. The medullogram confirmed the diagnosis of visceral leishmaniasis. The course was then favorable with treatment by pentavalent antimonial. CONCLUSION: Malnutrition constitutes a risk factor of opportunist parasitic disease such as leishmaniasis. Its diagnosis can be very difficult.