Rhabdomyosarcoma in Children: About 10 Cases

Introduction: Rhabdomyosarcoma (RMS) is a malignant soft-tissue tumor arising from striated muscle cells. It accounts for 60% - 70% of malignant mesenchymal tumors and 5% of pediatric cancers. Two-thirds of these cancers are diagnosed in children under 6 years of age, with a slight male predominance. Materials and Methods: This is a retrospective descriptive study of 10 cases of RMS collected in the pediatric hematology and oncology department of the Oujda university hospital, over a 5-year period, running from January 2018 to December 2022. Results: The median age at diagnosis was 3 years, with a sex ratio of 1. The mean time to diagnosis was 2 months. The most common site was the head and neck (50%), followed by the genitourinary tract (20%), the extremities (20%) and finally the abdomen (10%). The most frequent mode of discovery was a mass or swelling found in 90% of patients (all sites included), followed by exophthalmos in 30% of cases. At the diagnostic stage, CT scans were performed in 70% of cases and MRI in 5 patients (50%). Histological diagnosis was determined by immunohistochemical pathology in all our patients, with a predominance of embryonal (70%) versus alveolar (20%) and spindle cell types (10%). All patients underwent an extension workup, and a cervico-thoraco-abdominopelvic CT was performed in all patients (100%);MRI was performed in 2 patients (20%);lymph node involvement was present in 5 patients (50%). Metastases at the time of diagnosis were noted in only 1 patient (10%), who simultaneously presented with two metastatic sites;testicular and abdominal wall. Sixty percent of patients presented with advanced disease (high risk) and 40% with standard risk. Chemotherapy was used in all patients (100%), with upfront tumor resection performed in 40%. Fifty percent of patients received radiotherapy at a mean dose of 43 Gy, with the orbit the most frequently irradiated area (30%). All patients underwent CTscan and/or MRI and/or ultrasound surveillance. Follow-up during and after treatment was marked by complete remission in 8patients, loss of sight in one patient, and one patient died as a result of progressive disease. Conclusion: RMS is a malignant tumor of striated muscle. The epidemiological and clinical features of this tumor in our study are generally similar to those described in the literature. Management of these tumors requires multidisciplinary collaboration involving oncopediatric, radiologist, pediatric surgeon, pathologist and radiotherapist.

Attitudes and Practices of Lumbar Puncture among Students, Interns, and Residents of the Pediatric Department of the Mohammed VI University Hospital Center of Oujda, Morocco

Introduction: Even though lumbar puncture (LP) represents an important tool in the diagnosis of certain neurological diseases, this procedure is little practiced by our students. We aimed to evaluate the attitudes and practices of students, interns, and residents about LP, and to assess their feelings about how this procedure is taught. Materials and Methods: We conducted a cross-sectional study of 160 participants, using an anonymous questionnaire, to evaluate the attitudes and practices of students, interns and residents in the pediatrics department concerning lumbar puncture. Results: Half of the participants had never performed LP, usually because of the risk involved or lack of confidence, while 20% had performed it more than 4 times. None of the participants had learned to perform the procedure through simulation sessions, while (42%) had learned it under the supervision of a senior physician and had not been able to perform it successfully the first time. Most participants inform the patient about the procedure before performing it. Only 44% of participants acknowledged that LP can be performed under local anesthetic. The sitting position (60%) was by far the most commonly used. Most LPs were performed for diagnostic purposes. Most participants stated that the pediatric ward and the pediatric emergency department are among the departments that perform LP most frequently, and that they would be interested in taking part in simulated lumbar puncture sessions in children in the future. Conclusion: The results of our study show that LP is perceived by students as a risky procedure that is difficult to perform. Teachers should reconsider how this technical procedure is taught, by integrating simulation on mannequins into student training.

Pediatric Hepatoblastoma: A Case Report and Review of the Literature

Introduction and Objective: Hepatoblastoma is the most frequent malignant embryonal liver tumor, and its incidence is increasing. Children under 3 years of age are particularly affected. The etiology is largely unknown, but a genetic predisposition exists. This case highlights the clinical and imaging characteristics and management strategies. Case Report: We report the case of an 18 months old male admitted for exploration of an abdominal mass and was diagnosed with hepatoblastoma. Conclusion: Hepatoblastoma is an aggressive tumor that begins locally and eventually metastasizes to the brain, lungs, lymph nodes, and other organs. Tumor stage at presentation and resectability are the most significant prognostic factors.

Sacrococcygeal Teratoma a Rare Tumor in Children: Case Report

Sacrococcygeal teratomas (SCTs) are uncommon congenital tumors that typically develop in newborns, they are rarely associated with chromosomal abnormalities or other congenital anomalies. The majority of pediatric teratomas are benign in the neonatal age group, but the risk of malignancy increases with age. Diagnosis is based on a combination of clinical, radiological, and hormonal findings, but confirmed by anatomopathological study. Treatment is primarily surgical, with the aim of achieving complete resection to prevent recurrence. We present the case of a 22-month-old child who was admitted for management of a sacrococcygeal mass and was diagnosed with an immature teratoma.

Juvenile Xanthogranuloma: A Case Report with Literature Review

Juvenile xanthogranuloma (XGJ) is the most common form of non-Langerhansian histiocytosis. We report a pediatric case of multiple XGJ without visceral involvement in a 6-month-old female infant who was hospitalized with disseminated congenital skin nodules, of firm consistency, of variable diameter ranging from a few millimetres to 1 cm, involving the face, scalp, trunk and limbs. There was no mucosal localization. The remainder of the somatic examination was unremarkable. The diagnosis of disseminated XGJ was confirmed by skin biopsy without visceral or systemic involvement. Therapeutic abstention was recommended. This observation underlines the fact that XGJ is a rare clinical entity that should not be overlooked when faced with congenital skin nodules.

Ecthyma Gangrenosum in Patient with Bone Marrow Aplasia: A Case Report and Review of the Literature

Background: Ecthyma gangrenosum (EG) is an infrequent and discernible cutaneous disease caused by Pseudomonas aeruginosa. In situations where it is associated with septicemia in debilitated patients, the prognosis is usually unfavorable. Objective: In this case, we aim to verify risk factors, clinical, bacteriological and therapeutic characteristics of ecthyma gangrenosum and we review the literature to highlight the features of this rare condition and discuss the role of early diagnosis and treatment. Case Report: We describe the clinical case of a 4-year-old male with bone marrow aplasia who was presented with characteristic skin lesions of EG and developed sepsis later. Conclusion: EG is a cutaneous disease characterized by its aggressive nature. The presence of delayed diagnosis and therapy, along with sepsis, is closely linked to a high mortality rate. Treatment is empirically founded on an aggressive initial approach.