Department of Pathology and Laboratory Medicine, Nationwide Children's Hospital, The Ohio State University College of Medicine, Columbus, Ohio.
Division of Hematopathology, Mayo Clinic, Rochester, Minnesota.
Pediatr Dev Pathol. 2021 May-Jun;24(3):264-268. doi: 10.1177/1093526621999022. Epub 2021 Mar 8.
We report comprehensive characterization of an unusual collision tumor comprising extramedullary plasmacytomas and nasopharyngeal angiofibroma in a pediatric patient, which has yet to be reported. Histologically, the nasopharyngeal angiofibroma showed typical morphology with a diffuse, dense plasmacytic infiltrate in the stroma. The neoplastic plasma cells showed a spectrum of well-differentiated, plasmablastic, and anaplastic morphology, Epstein-Barr virus encoded RNA (EBER) positivity, and aberrant immunophenotype. Fluorescence in situ hybridization using a plasma cell myeloma targeted panel detected gains of 1q21.3 ( x3), 3q27 ( x4), and 11q22.3 ( x3), but no rearrangement of and . A 50-gene next generation sequencing lymphoma panel failed to detect any pathogenic mutation. Plasmacytoma with EBER positivity and plasmablastic morphology must be distinguished from plasmablastic lymphoma due to different clinical management and prognosis. This case highlights the importance of a thorough pathological evaluation of a mass lesion with synchronous neoplastic processes.
我们报告了一例儿童罕见的髓外浆细胞瘤和鼻咽血管纤维瘤合并的全面特征,目前尚未有报道。组织学上,鼻咽血管纤维瘤表现为典型形态,基质中有弥漫性密集的浆细胞浸润。肿瘤浆细胞表现出不同分化程度、浆母细胞样和间变形态、EB 病毒编码 RNA(EBER)阳性和异常免疫表型。使用浆细胞骨髓瘤靶向 panel 的荧光原位杂交检测到 1q21.3(x3)、3q27(x4)和 11q22.3(x3)的增益,但 和 没有重排。50 个基因下一代测序淋巴瘤 panel 未检测到任何致病性突变。由于不同的临床管理和预后,EBER 阳性和浆母细胞样形态的浆细胞瘤必须与浆母细胞淋巴瘤相鉴别。该病例强调了对具有同步肿瘤过程的肿块进行彻底的病理评估的重要性。