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.

Macrophage Activation Syndrome as the Primary Presentation of Pediatric Systemic Lupus Erythematosus: A Case Report and Review of the Literature

Macrophage activation syndrome (MAS), in its secondary form, often complicates rheumatic diseases but rarely constitutes a mode of revelation. Systemic lupus erythematosus (SLE) is a systemic autoimmune disease of unknown etiology that primarily affects women in adulthood. MAS is a serious condition that may be the first presentation of SLE. Here, we report the case of a 4-year-old female with MAS as the primary manifestation of Systemic Lupus Erythematosus (SLE). In this case, we outline the characteristics of a complex case of SLE that was initially accompanied with MAS, and also review the literature to discuss the clinical, biological, and therapeutic aspects of this condition.

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.

Extra Dural Hematoma of the Dorso-Lumbar Region in a Hemophiliac: A Rare Entity

Introduction and objective: Hemophilia is a genetic bleeding disorder inherited as a recessive train linked to the male gender. Bleeding into the central nervous system in patients with hemophilia is an extremely dangerous condition that can be directly life-threatening, if left untreated. Extradural hematoma of the dorso-lumbar region is rare but potentially deadly disease in children. This condition can result in severe neurological deficits. We aim to discuss the clinical, radiological and progressive clinical aspects of this illness. Case report: We report the case of a 5-year-old child with severe hemophilia A treated for extradural hematoma of the dorso-lumbar region resulting from trauma. A spinal magnetic resonance imaging revealed an extradural hematoma. The patient was successfully treated with intensive replacement therapy and did not require surgical intervention. Conclusion: Extradural hematoma is a rare complication of hemophilia, that needs to be looked for in children who have bleeding disorders. For the best neurological outcome, early diagnosis is crucial.

Mandibular Embryonal Rhabdomyosarcoma Confused with a Vascular Malformation: About a Case

Malignant mesenchymal tumors (MTM) in children represent 5% to 10% of malignant tumors in children. They constitute a heterogeneous group of tumors of various differentiations depending on their supposed tissue of origin. They mainly include tumors of muscular origin, those derived from connective, vascular, nervous, or adipose tissue. Rhabdomyosarcoma (RMS) is the most common mesenchymal tumor in children and adolescents (60% to 70% of them). And it accounts for 5.8% of all malignant solid tumors in children. Almost half of rhabdomyosarcomas occur in the head and neck. The prognosis for this type of tumor is particularly poor. A case of rhabdomyosarcoma in the mandible with extension to the abdominal wall and unilateral testis in a 6-month-old infant is reported with evolution since birth. It is a purplish lesion at the level under the right chin which was initially taken for vascular malformation, evolving very quickly towards a mandibular mass deforming the painful face with inflammatory signs, followed by the appearance of a hard swelling under the skin on the left flank taking on the same aspect of the mandibular mass. This observation illustrates the need to know how to systematically think about tumor causes in the face of atypical aspects and to carry out an anatomopathological examination.

Late Onset Combined Immune Deficiency (LOCID) Revealed by a Haemolytic Anaemia in a Child: A Case Report

Variable Common Immune Deficiency (VCID) is a very heterogeneous condition both clinically and immunologically. It is a group of molecular abnormalities responsible for a defect in antibody production leading to hypogammaglobulinemia often associated with autoimmune and/or lymphoproliferative manifestations. Late Onset Combined Immune Deficiency (LOCID) is a type of Variable Common Immune Deficiency (VCID) defined by a defect in antibody production (IgG and IgA ± IgM type), profound CD4 T-cell lymphopenia and frequent opportunistic infections. LOCID has been considered as a distinct entity from VCID due to its particular clinical and immunological profile.

Neuromyelitis Optica Revealed by Headache in a Child: A Case Report

Devic’s neuromyelitis optica (NMO) is a rare inflammatory disease of the central nervous system that results in optic neuropathy and myelitis. Optic neuritis represents the mode of entry into the disease in more than two thirds of cases. It is a rare entity in children. There is no effective treatment at present, but some molecules can be used, such as corticosteroids, immunosuppressants and plasma exchange. The prognosis in children is generally favorable. Devic’s neuromyelitis is a condition of unknown etiopathogeny which is functionally critical and requires early and appropriate treatment. We report the case of a 12-year-old girl who presented to emergency with a headache and decreased visual acuity, whose investigations led to the diagnosis of Devic’s neuromyelitis optica.

Intracardiac Thrombosis Secondary to the Silent Killer: A Case Report about Monoxide Carbon Poisoning in a Child

Carbon monoxide (CO) poisoning is a frequent cause of emergency room admissions, especially during winter days, the symptoms are varied ranging from a simple headache to a serious cardiac and neurological impairment that can be deadly. Diagnosis is based on the circumstances of occurrence as well as the dosage of carboxyhemoglobin in the blood. Exposure to CO has serious consequences, neurological and cardiac manifestations are not negligible and vary from repolarization disorders to heart attack. Treatment is urgent with normobaric or hyperbaric oxygen therapy. We report a case of a 2-year male child admitted to the emergency room for CO intoxication with an intracardiac thrombus subsequently complicated by an ischemic stroke with a fatal outcome in order to highlight this complication rarely described in literature.

Hematochezia Revealing an Adenocacinoma

Colorectal carcinoma (CRC) is a common pathology in the adult population but remains a very rare neoplasia in childhood with delayed diagnosis and advanced stage with grim prognosis that can be fatal, research regarding pediatric adenocarcinoma remain very rare. IT represents approximately 1% of pediatric neoplasms. It is usually associated with predisposing genetic factors;this pathology is closely linked with the inheritance of familial syndromes. The pediatric clinical presentations are nonspecific, with a grim prognosis. This review aimed to report a case of a 10-year-old girl who presented with constipation and hematochezia evolving since three months’ diagnosis with rectal adenocarcinoma.

Unexplained Crying Episodes Reveling a Cystic Nephromas

Cystic nephromas (CNs) are uncommon, benign renal neoplasms reported in infants/young children of both genders and in adult females, concerning the pediatric population few studies have been conducted. The diagnosis of cystic nephroma is based on clinical signs, imaging tests, and anatomo-pathological study. In children, CNs can appear as a palpable abdominal mass in most of cases, hematuria or recurrent urinary infections. They are characterized by multycystic architecture and the exclusive presence of mature nephrogenic elements. Treatment is surgical with a very good prognosis in most of cases. We are going to report a case of a 13-month-old girl child diagnosed with Cystic nephromas who presented to pediatric emergency with unexplained crying episodes in order to increase clinicians awareness about this rare tumor.

Unilateral Ptosis in a Child with Wilm’s Tumor Induced by Vincristine

Vincristine is a chemotherapy drug belonging to the group of Vinca alkaloids which also includes vinblastine and vindesine. It is used in hematological malignancies and solid tumors. The Vinca alkaloids are neurotoxic, usually causing peripheral neuropathy, and rarely cranial neuropathies. We report a case of a 33-month-old male child diagnosed with Wilms’ tumor, who had an isolated unilateral right ptosis following vincristine, with a good improvement after stopping it.

Aorto-Pulmonary Window, an Unrecognized Cause of Heart Failure in Infants: A Case Report

Introduction: The aorto-pulmonary window is a rare congenital heart disease. It is a defect between the ascending aorta and the trunk of the pulmonary artery upstream of the pulmonary bifurcation. Once the diagnosis is made, surgery must be performed quickly to avoid progression to pulmonary arteriolitis. We report the case of a 4-month-old infant in whom we made the diagnosis of aorto-pulmonary window type I. He was able to benefit from an open heart surgery at FES with an excellent result after closing the window. Conclusion: The aorto-pulmonary window is a rare congenital heart defect, the diagnosis of which must be made as early as possible in order to avoid the progression to pulmonary hypetension and heart failure.

Case Report: The Paratesticular Rhabdomyosarcoma in Children

Rhabdomyosarcoma is a rare and aggressive mesenchymal tumor, whose paratesticular location is the most common genitourinary site. It is the prerogative of the child and the young adult. On physical examination, a painless scrotal tumefaction is observed. His diagnosis is essentially provided by testicular ultrasound. His therapeutic strategy is codified and based on the combination of chemotherapy and surgical excision or radiotherapy. The precocity of the management and the accuracy of the extension and radiology tests are important for the vital and functional prognosis. We report a case of paratesticular rhabdomysarcoma of alveolar type, diagnosed in front of a large left painless scrotal mass, which has been evolving for less than one month in a 7-year-old child. An inguinal orchiectomy was performed. A histological examination of the excised tissue revealed an epididymal RMS of the alveolar type. Our patient had 9 chemotherapy sessions with vincristine, actinomycin C and cyclophosphamide. Each chemotherapy session took place over four days, with a 21-day cycle. He was evaluated for 4 years and demonstrated good clinical improvement. The experience gained from the index case is crucial for the management of patients with intratesticular rhabdomyosarcoma who must always ensure that the radical inguinal orchiectomy with lymph node dissection is covered by chemotherapy and/or radiotherapy. This will potentially reduce the chances of tumor recurrence and/or metastasis, thus improving patient prognosis.

Acute Lymphoblastic Leukemia in Childhood Following Pyoderma Gangrenosum

Pyoderma gangrenosum (PG) is a rare but serious ulcerating skin disease that occurs either idiopathically or associated with various systemic diseases and malignant tumors. Although the association of PG with myeloid malignant tumors is well known, their association with lymphoid malignant tumors, especially in children is extremely rare. We present here a case of PG in a 4-year and 4 months female child, diagnosed in the Pediatrics department, which had initially evolved well with oral corticosteroid therapy and a local dermocorticoid. The development was marked 7 months after stopping corticosteroid treatment by the occurrence of acute lymphoblastic leukemia. This case of PG is presented in the intention of increasing awareness of this uncommon condition with may occurring in association with haematological malignancy. PG may precede, develop concurrently with, or follow various conditions.

Exceptional Complication of a Benin Cranial Trauma in a 3-Year-Old Child

Internal carotid artery (ICA) dissection with associated stroke is rare event in children. The usual mechanism is either direct trauma or sudden neck movements. We describe the case of a 3-year-old patient admitted with right hemiplegia following a stroke. The diagnosis was confirmed by ANGIO-MRI. During treatment with anticoagulants and antiplatelet agents, the patient showed significant improvement in the right-sided hemiplegia. Minor head trauma is a possible pathological mechanism for ICA dissection in children. However, the scenario is extremely rare.