Shyamaly K J, Mutanabbi M, Talukder M K, Farzana N, Riaaz R, Kawser C A
Dr Kohinoor Jahan Shyamaly, Resident Phase-B, Department of General Paediatrics, Bangabandhu Sheikh Mujib Medical University (BSMMU), Dhaka, Bangladesh.
Mymensingh Med J. 2018 Jul;27(3):665-668.
Primitive neuroectodermal tumors are malignant tumor of pluripotent cells of neural crest. It has diverse clinical presentation and aggressive clinical behaviour. Clinical features may provide some clue but imaging studies such as MRI of brain; tissue histopathology, immune histochemistry and cytogenetic are essential to confirm the diagnosis. Here we describe a girl of 4½ years age admitted with the complaints of, low back pain for 2 months, gradually worsening weakness in both lower limbs for the same duration along with urinary incontinence and constipation for 1 month. On examination she was afebrile, mildly pale, no lymphadenopathy, having normal vital signs and anthropometrically well thriving. Back and spine examination showed angulation of spine involving T₁₁ - L₄, without paravertebral swelling. Neurological examination of lower limbs revealed decreased muscle tone, diminished muscle power (3/5), both knee and ankle jerks were also diminished and bilaterally equivocal planter response, sensory function of all modalities were impaired. MRI findings were suggestive of ependymoma. Consultation was done with department of neurosurgery and they did the relevant surgery. Tissue histopoathology and immune histochemistry confirmed the diagnosis of primitive neuroectodermal tumor. Finally the child was referred to Paediatric Haemato-oncolgy department for further management.
原始神经外胚层肿瘤是神经嵴多能细胞的恶性肿瘤。它具有多样的临床表现和侵袭性的临床行为。临床特征可能提供一些线索，但诸如脑部MRI等影像学检查、组织组织病理学、免疫组织化学和细胞遗传学对于确诊至关重要。在此，我们描述一名4岁半的女孩，因以下症状入院：腰痛2个月，双下肢逐渐加重的无力持续相同时间，伴有1个月的尿失禁和便秘。检查时，她体温正常，轻度苍白，无淋巴结肿大，生命体征正常，体格发育良好。背部和脊柱检查显示脊柱成角，累及T₁₁ - L₄，无椎旁肿胀。下肢神经学检查显示肌张力降低，肌力减弱（3/5），膝反射和踝反射也减弱，双侧巴宾斯基征可疑，所有感觉功能均受损。MRI结果提示室管膜瘤。与神经外科进行了会诊，他们实施了相关手术。组织组织病理学和免疫组织化学确诊为原始神经外胚层肿瘤。最后，该患儿被转诊至儿科血液肿瘤学部门进行进一步治疗。
