Mremi Alex, Nyoni Vimbikai, Elisante Joseph, Sadiq Adnan, Nkoronko Mugisha
Department of Pathology, Kilimanjaro Christian Medical Centre, Moshi, Tanzania; Faculty of Medicine, Kilimanjaro Christian Medical University College, Moshi, Tanzania.
Faculty of Medicine, Kilimanjaro Christian Medical University College, Moshi, Tanzania; Department of General Surgery, Kilimanjaro Christian Medical Centre, Moshi, Tanzania.
Int J Surg Case Rep. 2023 Jul;108:107896. doi: 10.1016/j.ijscr.2023.107896. Epub 2023 Jan 12.
Plexiform fibromyxoma (PF) is a rare benign mesenchymal neoplasm of stomach recognized by the WHO. The tumor often arises in the antrum and pyloric region of the stomach. Morphologically, PF tumors display bland spindle cells in myxoid or fibromyxoid stroma and can be misdiagnosed as gastrointestinal stromal tumor (GIST).
A-21-year old female presented to emergency department with peritonitis due gastric tumor leading to gastric perforation and pus collection in the abdomen. Partial gastrectomy was performed. Histopathology, immunohistochemical (IHC) and fluorescent in-situ hybridization assessment of the specimen confirmed the diagnosis of PF. One year postoperative, the patient remains symptoms free.
A great majority of gastric mesenchymal tumors are GIST. Histopathologically, PF tumors display a multinodular and plexiform architecture with arborizing vasculature. Cytologically these tumors show bland spindle cells in myxoid or fibromyxoid stroma with a rare or no mitotic figures. Thus, PF may easily be under recognized or misinterpreted without the pathologists' knowledge of this entity. Misinterpreting PF as GIST can lead to inappropriate treatment including unnecessary surgery and/or chemotherapy, which is an expensive. Recommended treatment is surgical excision. Metastases and recurrence following complete excision have not been described. This case highlights unexpected presentation in a young female where other competing diagnoses were more plausible before considering PF diagnosis which could not have been established without advanced diagnostic techniques.
PF is a rare mesenchymal tumor with nonspecific clinical characteristics. It is principally located in the gastric antrum and prepyloric regions, however other parts of the body may be affected. PF tumors should be separated from GIST, nerve sheath tumors, and other fibromyxoid neoplasms. The worth in writing lies in epidemiological custodianship for such a unique presentation of a rare gastric neoplasm.
丛状纤维黏液瘤（PF）是一种世界卫生组织认可的罕见的胃部良性间叶性肿瘤。该肿瘤常发生于胃窦和幽门区域。在形态学上，PF肿瘤在黏液样或纤维黏液样基质中表现为温和的梭形细胞，可能被误诊为胃肠道间质瘤（GIST）。
一名21岁女性因胃肿瘤导致胃穿孔和腹腔积脓而到急诊科就诊。进行了胃部分切除术。对标本进行组织病理学、免疫组织化学（IHC）和荧光原位杂交评估，确诊为PF。术后一年，患者无症状。
绝大多数胃间叶性肿瘤是GIST。在组织病理学上，PF肿瘤表现为多结节和丛状结构，血管呈树枝状分布。在细胞学上这些肿瘤在黏液样或纤维黏液样基质中显示温和的梭形细胞，有罕见的或无有丝分裂象。因此，如果病理学家不了解这种实体，PF可能很容易未被识别或被误解。将PF误诊为GIST会导致不适当的治疗，包括不必要的手术和/或化疗，这是昂贵的。推荐的治疗方法是手术切除。完全切除后未见转移和复发的报道。该病例突出了在一名年轻女性中的意外表现，在考虑PF诊断之前，其他竞争性诊断似乎更合理，而没有先进的诊断技术就无法确诊PF。
PF是一种罕见的间叶性肿瘤，具有非特异性临床特征。它主要位于胃窦和幽门区域，然而身体的其他部位也可能受累。PF肿瘤应与GIST、神经鞘瘤和其他纤维黏液样肿瘤相鉴别。撰写本文的价值在于对这种罕见胃肿瘤的独特表现进行流行病学记录。
