<strong>Introduction</strong><span style="font-family:Verdana;"><strong>:</strong> The</span><span style="font-family:Verdana;"> </span><span styl...<strong>Introduction</strong><span style="font-family:Verdana;"><strong>:</strong> The</span><span style="font-family:Verdana;"> </span><span style="font-family:;" "=""><span style="font-family:Verdana;">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. </span><b><span style="font-family:Verdana;">Conclusion</span></b><span style="font-family:Verdana;">: The</span></span><span style="font-family:Verdana;"> </span><span style="font-family:Verdana;">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.</span>展开更多
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 a...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.展开更多
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 canc...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展开更多
Cutaneous presentation revealing acute myeloid leukemia (AML) is rare, and the prognosis is poor. We report a case of an 11-month-old male who presented with cutaneous lesions present as diffuse purplish papulo-nodula...Cutaneous presentation revealing acute myeloid leukemia (AML) is rare, and the prognosis is poor. We report a case of an 11-month-old male who presented with cutaneous lesions present as diffuse purplish papulo-nodular lesions, revealing M5 acute myeloid leukemia. Skin biopsy and immunohistochemical examination, combined with routine blood analysis and bone marrow examination, contributed to early diagnosis. However, despite intensive chemotherapy treatment, the prognosis was poor and death occurred during the first months of treatment.展开更多
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 hypog...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.展开更多
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 ori...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.展开更多
Costal Ewing sarcoma is a rare primary malignant bone tumor with delayed diagnosis and complex treatment. The diagnosis, evoked in front of swelling, a parietal pain, supported on the tomodensitometry, rests on the an...Costal Ewing sarcoma is a rare primary malignant bone tumor with delayed diagnosis and complex treatment. The diagnosis, evoked in front of swelling, a parietal pain, supported on the tomodensitometry, rests on the anatomopathological examination. The treatment is based on the combination of chemotherapy surrounding complete surgical removal of the tumor. Radiotherapy should remain reserved for a few specific indications because of its complications. We report the case of three children who presented with a pleural effusion revealing Ewing’s sarcoma affecting the ribs.展开更多
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...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.展开更多
Rhabdomyosarcoma is a rare and aggressive mesenchymal tumor, whose paratesticular location is the most common genitourinary site. It is the preroga</span><span style="font-family:Verdana;">tive o...Rhabdomyosarcoma is a rare and aggressive mesenchymal tumor, whose paratesticular location is the most common genitourinary site. It is the preroga</span><span style="font-family:Verdana;">tive of the child and the young adult. On physical examination, a painless</span><span style="font-family:Verdana;"> 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.</span><b> </b><span style="font-family:Verdana;">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</span></span></span><span style="font-family:Verdana;"><span style="font-family:Verdana;"><span style="font-family:Verdana;">-</span></span></span><span style="font-family:Verdana;"><span style="font-family:Verdana;"><span style="font-family:Verdana;">year</span></span></span><span style="font-family:Verdana;"><span style="font-family:Verdana;"><span style="font-family:Verdana;">-</span></span></span><span><span><span style="font-family:""><span style="font-family:Verdana;">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 </span><span style="font-family:Verdana;">days, with a 21-day cycle. He was evaluated for 4 years and demonstrated</span> <span style="font-family:Verdana;">good clinical improvement. The experience gained from the index case is</span><span style="font-family:Verdana;"> crucial for the management of patients with intratesticular rhabdomyosarcoma who must always ensure that the radic展开更多
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 ...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</span></span><span style="font-family:Verdana;"><span style="font-family:Verdana;"><span style="font-family:Verdana;"> and </span></span></span><span style="font-family:Verdana;"><span style="font-family:Verdana;"><span style="font-family:Verdana;">4</span></span></span><span><span><span style="font-family:""> </span></span></span><span style="font-family:Verdana;"><span style="font-family:Verdana;"><span style="font-family:Verdana;">month</span></span></span><span style="font-family:Verdana;"><span style="font-family:Verdana;"><span style="font-family:Verdana;">s</span></span></span><span style="font-family:Verdana;"><span style="font-family:Verdana;"><span style="font-family:Verdana;"> female child, </span></span></span><span style="font-family:Verdana;"><span style="font-family:Verdana;"><span style="font-family:Verdana;">diagnosed</span></span></span><span style="font-family:Verdana;"><span style="font-family:Verdana;"><span style="font-family:Verdana;"> 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.</span></span></span><span><span><span style="font-family:""> </span></span></span><span style="font-family:Verdana;"><span style="font-family:Verdana;"><span style="font-family:Verdana;">This case of PG is presented in the intention of increasing awareness of this uncommon condition with may oc</span></span></span><span style="font-family:Verdana;"><span style="font-family:Verdana;"><span style="font-family:Verdana;">c</span></span></span展开更多
文摘<strong>Introduction</strong><span style="font-family:Verdana;"><strong>:</strong> The</span><span style="font-family:Verdana;"> </span><span style="font-family:;" "=""><span style="font-family:Verdana;">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. </span><b><span style="font-family:Verdana;">Conclusion</span></b><span style="font-family:Verdana;">: The</span></span><span style="font-family:Verdana;"> </span><span style="font-family:Verdana;">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.</span>
文摘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.
文摘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
文摘Cutaneous presentation revealing acute myeloid leukemia (AML) is rare, and the prognosis is poor. We report a case of an 11-month-old male who presented with cutaneous lesions present as diffuse purplish papulo-nodular lesions, revealing M5 acute myeloid leukemia. Skin biopsy and immunohistochemical examination, combined with routine blood analysis and bone marrow examination, contributed to early diagnosis. However, despite intensive chemotherapy treatment, the prognosis was poor and death occurred during the first months of treatment.
文摘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.
文摘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.
文摘Costal Ewing sarcoma is a rare primary malignant bone tumor with delayed diagnosis and complex treatment. The diagnosis, evoked in front of swelling, a parietal pain, supported on the tomodensitometry, rests on the anatomopathological examination. The treatment is based on the combination of chemotherapy surrounding complete surgical removal of the tumor. Radiotherapy should remain reserved for a few specific indications because of its complications. We report the case of three children who presented with a pleural effusion revealing Ewing’s sarcoma affecting the ribs.
文摘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.
文摘Rhabdomyosarcoma is a rare and aggressive mesenchymal tumor, whose paratesticular location is the most common genitourinary site. It is the preroga</span><span style="font-family:Verdana;">tive of the child and the young adult. On physical examination, a painless</span><span style="font-family:Verdana;"> 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.</span><b> </b><span style="font-family:Verdana;">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</span></span></span><span style="font-family:Verdana;"><span style="font-family:Verdana;"><span style="font-family:Verdana;">-</span></span></span><span style="font-family:Verdana;"><span style="font-family:Verdana;"><span style="font-family:Verdana;">year</span></span></span><span style="font-family:Verdana;"><span style="font-family:Verdana;"><span style="font-family:Verdana;">-</span></span></span><span><span><span style="font-family:""><span style="font-family:Verdana;">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 </span><span style="font-family:Verdana;">days, with a 21-day cycle. He was evaluated for 4 years and demonstrated</span> <span style="font-family:Verdana;">good clinical improvement. The experience gained from the index case is</span><span style="font-family:Verdana;"> crucial for the management of patients with intratesticular rhabdomyosarcoma who must always ensure that the radic
文摘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</span></span><span style="font-family:Verdana;"><span style="font-family:Verdana;"><span style="font-family:Verdana;"> and </span></span></span><span style="font-family:Verdana;"><span style="font-family:Verdana;"><span style="font-family:Verdana;">4</span></span></span><span><span><span style="font-family:""> </span></span></span><span style="font-family:Verdana;"><span style="font-family:Verdana;"><span style="font-family:Verdana;">month</span></span></span><span style="font-family:Verdana;"><span style="font-family:Verdana;"><span style="font-family:Verdana;">s</span></span></span><span style="font-family:Verdana;"><span style="font-family:Verdana;"><span style="font-family:Verdana;"> female child, </span></span></span><span style="font-family:Verdana;"><span style="font-family:Verdana;"><span style="font-family:Verdana;">diagnosed</span></span></span><span style="font-family:Verdana;"><span style="font-family:Verdana;"><span style="font-family:Verdana;"> 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.</span></span></span><span><span><span style="font-family:""> </span></span></span><span style="font-family:Verdana;"><span style="font-family:Verdana;"><span style="font-family:Verdana;">This case of PG is presented in the intention of increasing awareness of this uncommon condition with may oc</span></span></span><span style="font-family:Verdana;"><span style="font-family:Verdana;"><span style="font-family:Verdana;">c</span></span></span
