Bilotta Clio, Perrone Giulio, Maresi Emiliano, De Lisi Giovanni, Di Pasquale Pietro, Piro Ettore, Argo Antonina, Zerbo Stefania
Department of Health Promotion, Mother and Child Care, Internal Medicine and Medical Specialties, Section of Legal Medicine, University of Palermo, Palermo, Italy.
Division of Cardiology, Paolo Borsellino, G. F. Ingrassia Hospital, Palermo, Italy.
Front Pediatr. 2021 Nov 10;9:718157. doi: 10.3389/fped.2021.718157. eCollection 2021.
There are still no guidelines about pediatric cardiac cancers. The purpose of this work is to provide new scientific data facilitating the differential diagnosis of a rare cardiac tumor with an unusual presentation, such as the cardiac inflammatory myofibroblastic tumor (IMT). A 3-year-old male child presented with several symptoms including unconsciousness, vomiting, and drowsiness. A clinical and neurological examination revealed a unilateral (right) motor delay and positive unilateral Babinski sign. Electrocardiogram (ECG) was normal. The total body computed tomography (CT) scans showed hypodensity in the left temporal-parietal lobe, a large hypodense area in the right frontal lobe, and a second area in the left frontal lobe were found with head CT. A magnetic resonance (MR) also noted cerebral areas of hypointensity. The echocardiographic images revealed an ovoid mass, adherent to the anterolateral papillary muscle. The histological exams, performed with hematoxylin-eosin, Masson's trichrome, Alcian blue PAS, Weigert and Van-Gieson stain, allowed observing the microscopic structure of the neoplastic mass. The immunohistochemical analysis was performed through subsequent antibodies: anti-vimentin, anti-actina, anti-ALK, anti-CD8, anti-CD3, anti-CD20, anti-kappa and lambda chains, and anti CD68 antibodies. The healthcare professionals diagnosed a cardiac IMT with brain embolism. The ventricular localization, observed through radiological exams, required a differential diagnosis with fibroma and rhabdomyoma, the presence of brain embolism with sarcoma, and its morphology with fibroma. Neurological symptoms might be attributed to encephalitis, primitive cerebral cancer, such as astrocytoma or neuroblastoma, cerebral metastases due to any malignancy, or embolic stroke. New studies are encouraged to better define IMT behavior and draw up guidelines confirming the crucial role of multidisciplinary approach and treatment protocol selected on the basis of the characteristics of the tumors, in the case of this rare type of cancer.
目前仍没有关于儿童心脏肿瘤的指南。这项工作的目的是提供新的科学数据，以促进对一种表现不寻常的罕见心脏肿瘤，如心脏炎性肌纤维母细胞瘤（IMT）的鉴别诊断。一名3岁男童出现了包括昏迷、呕吐和嗜睡在内的多种症状。临床和神经学检查发现单侧（右侧）运动迟缓以及单侧巴宾斯基征阳性。心电图（ECG）正常。全身计算机断层扫描（CT）显示左侧颞顶叶低密度，右侧额叶有一大片低密度区，头部CT还发现左侧额叶有第二个区域。磁共振成像（MR）也显示脑区有低信号。超声心动图图像显示一个椭圆形肿块，附着于前外侧乳头肌。通过苏木精-伊红染色、马松三色染色、阿尔辛蓝过碘酸雪夫染色、魏格特染色和范吉森染色进行组织学检查，得以观察肿瘤肿块的微观结构。免疫组织化学分析通过后续抗体进行：抗波形蛋白抗体、抗肌动蛋白抗体、抗间变性淋巴瘤激酶（ALK）抗体、抗CD8抗体、抗CD3抗体、抗CD20抗体、抗κ和λ链抗体以及抗CD68抗体。医护人员诊断为心脏IMT伴脑栓塞。通过影像学检查观察到的心室定位需要与纤维瘤和横纹肌瘤进行鉴别诊断，脑栓塞的存在需要与肉瘤进行鉴别诊断，其形态需要与纤维瘤进行鉴别诊断。神经症状可能归因于脑炎、原发性脑癌，如星形细胞瘤或神经母细胞瘤、任何恶性肿瘤导致的脑转移或栓塞性中风。鼓励开展新的研究，以更好地界定IMT的行为，并制定指南，确认多学科方法和根据肿瘤特征选择的治疗方案在这种罕见癌症病例中的关键作用。
