Fukazawa Mitsuharu, Koga Hiroshi, Hiroshige Shoji, Matsumoto Toshifumi, Nakazono Yuichi, Yoshikawa Yasuji
Department of Pediatrics.
Department of Surgery.
Medicine (Baltimore). 2019 Jan;98(3):e14186. doi: 10.1097/MD.0000000000014186.
Plexiform fibromyxoma (PF) is a rare gastric mesenchymal tumor, with approximately 80 cases reported to date. Gastrointestinal stromal tumor, the most common primary mesenchymal tumor of the stomach, shows different biological and clinical characteristics between adult and pediatric patients.
This systematic literature review was conducted to elucidate the pathological and clinical features of pediatric PF compared to adult PF.
MEDLINE (1948 to March 2018) and EMBASE (1947 to March 2018) were searched, and all English articles that reported clinical data on PF patients were identified. Two authors independently reviewed the articles and extracted data to assess immunohistochemistry, sex, chief complaint, tumor size, tumor-related mortality, and tumor recurrence and metastasis.
A total of 41 reports with 80 PF patients (of whom 70 were adult PF and 10 were pediatric PF patients) confirmed by histological and immunohistochemical findings were included. Of a total of 80 tumors, 62 (78%) were located in the gastric antrum, 42 (65%) presented with ulceration, and 48 (74%) were resected by partial gastrectomy. Median tumor size of the resected specimen was larger in pediatric PF than in adult PF cases (5.3 cm vs 4.0 cm, P = .036). However, there was no difference between pediatric and adult PFs in immunohistochemical expression, sex predominance, chief complaint, tumor-related mortality, and tumor recurrence and metastasis during the follow-up periods.
Other than increased tumor growth in pediatric PFs, PF is a single disease entity with similar pathological features and benign clinical behavior regardless of onset age.
丛状纤维黏液瘤（PF）是一种罕见的胃间质瘤，迄今为止报道的病例约有80例。胃肠道间质瘤是胃最常见的原发性间质瘤，在成人和儿童患者中表现出不同的生物学和临床特征。
进行这项系统的文献综述，以阐明儿童PF与成人PF相比的病理和临床特征。
检索MEDLINE（1948年至2018年3月）和EMBASE（1947年至2018年3月），并识别所有报告PF患者临床数据的英文文章。两位作者独立审阅文章并提取数据，以评估免疫组织化学、性别、主要症状、肿瘤大小、肿瘤相关死亡率以及肿瘤复发和转移情况。
纳入了41篇报告，其中80例PF患者（70例为成人PF，10例为儿童PF患者）经组织学和免疫组织化学检查确诊。在总共80个肿瘤中，62个（78%）位于胃窦，42个（65%）出现溃疡，48个（74%）通过胃部分切除术切除。儿童PF切除标本的肿瘤大小中位数大于成人PF病例（5.3厘米对4.0厘米，P = 0.036）。然而，儿童和成人PF在免疫组织化学表达、性别优势、主要症状、肿瘤相关死亡率以及随访期间的肿瘤复发和转移方面没有差异。
除了儿童PF中肿瘤生长增加外，PF是一种单一的疾病实体，无论发病年龄如何，都具有相似的病理特征和良性临床行为。
