Malik Faizan, Bernieh Anas, El Jamal Siraj M, Saad Ali G
Department of Pathology and Laboratory Medicine, 12326University of Tennessee Health Science Center, Memphis, TN, USA.
Division of Pathology, 2518Cincinnati Children's Hospital, Cincinnati, OH, USA.
Pediatr Dev Pathol. 2022 May-Jun;25(3):304-315. doi: 10.1177/10935266211063989. Epub 2022 Jan 4.
Cranial fasciitis (CF) is a benign (myo)fibroblastic proliferation of children. Typical presentation consists of a rapidly growing solitary mass on the temporal or parietal cranium in the first 2 years of age. CF is characterized by a rapid growth followed by a relative slowdown and even growth arrest. The finding of somatic gene rearrangements demonstrating clonality in CF together with its clinical behavior places it in the category of diseases recently termed "transient neoplasia." Histological, immunohistochemical, and molecular findings of 18 patients with CF were retrospectively studied. The tumor typically presented as a painless rapidly enlarging mass in the temporal region. Sixty-six percent of the cases harbored gene rearrangement. Nine patients were treated with gross total resection (GTR) and 9 with subtotal tumor resection (STR). Two patients treated with GTR had recurrence. Five patients treated with STR had progression-free disease for at least 10 months after surgery and in four patients the tumor regressed spontaneously a median 16 months after surgery. In this largest series to date, we reported the clinicopathological, immunohistochemical, and molecular findings of 18 pediatric cases of CF with emphasis on the clinical growth pattern of these tumors.
颅骨筋膜炎（CF）是一种儿童期的良性（肌）纤维母细胞增生性疾病。典型表现为在2岁前颞部或顶骨颅骨上出现快速生长的孤立性肿块。CF的特点是生长迅速，随后生长相对减缓甚至停止生长。CF中存在体细胞基因重排显示克隆性，连同其临床行为，使其归入最近被称为“暂时性肿瘤形成”的疾病类别。对18例CF患者的组织学、免疫组化和分子学结果进行了回顾性研究。肿瘤通常表现为颞部无痛性迅速增大的肿块。66%的病例存在基因重排。9例患者接受了全切除（GTR），9例接受了次全肿瘤切除（STR）。2例接受GTR治疗的患者复发。5例接受STR治疗的患者术后至少10个月无疾病进展，4例患者肿瘤在术后中位16个月时自发消退。在这个迄今为止最大的系列研究中，我们报告了18例儿童CF病例的临床病理、免疫组化和分子学结果，重点关注这些肿瘤的临床生长模式。
