An Update on Clinicopathological and Molecular Features of Plexiform Fibromyxoma

Abstract

Plexiform fibromyxoma is a rare and newly described gastric mesenchymal tumor with only 121 reported cases in the literature. Our understanding of plexiform fibromyxoma requires updating since the first case has been reported by Takahashi et al. 12 years ago. The present review summarized reported cases in the literature, and both clinical and pathological aspects of plexiform fibromyxoma were comprehensively discussed. Plexiform fibromyxoma usually causes nonspecific or bleeding signs or symptoms, and therefore clinical recognition of the disease is challenging. Plexiform fibromyxoma is of benign nature without any metastasis or recurrence reported, and more conservative surgical treatment should be considered.

Gastric Plexiform Fibromyxoma Arising in the Cardia in an Adolescent Male: A Rare Tumor with an Unusual Location

“…It is more common in adults than in children with a ratio of 7 : 1 and equal male to female predisposition [ 2 ]. More cases were reviewed in 2019 by Su et al, who collected a total of 121 cases, providing a more comprehensive updating of PF [ 3 ]. They stated that the age range of the patients was broad ranging from 5 to 81 years (mean age 43.17 ± 18.00 years, median age 46 years), with most patients middle aged, with a peak around 30-60 years.…”

“…Plexiform fibromyxoma (PF) is a rare benign gastric mesenchymal tumor, described for the first time in 2007 by Takahashi et al, who named it plexiform angiomyxoid myofibroblastic tumor of the stomach [ 1 ]. It affects adults mainly, with a few cases observed in children and adolescents [ 2 , 3 ]. It is usually located at the antrum, with occasional cases found in the body of the stomach or rarely presenting primarily in the duodenum [ 4 , 5 ], the esophagus [ 6 ], the jejunum [ 7 ], the colon [ 8 ], and the gallbladder [ 9 ].…”

“…Occasionally, this tumor presents with bleeding, obstruction, and perforation. In addition, it could be found incidentally during endoscopy or radiological images [ 2 , 3 , 10 ]. In a retrospective histologic review of approximately 4200 GISTs from 1970 to 1999, Miettinen et al found ten cases and added two additional cases of benign gastric antral fibromyxoid tumors with a peculiar multinodular, plexiform pattern, and they proposed the name plexiform fibromyxoma.…”

“…Definite diagnosis is usually confirmed after surgical excision and histopathological examination of the specimen [ 10 ]. Treatment is surgical resection of the tumor; however, the type of surgery depends on the size and location of the tumor [ 3 , 10 ]. Herein, we report the twelfth case of gastric plexiform fibromyxoma in the pediatric population with an unusual location in the cardia, a location reported for the first time for this tumor.…”

A Malignant Neoplasm From the Jejunum With a MALAT1-GLI1 Fusion and 26-Year Survival History

“…[4][5][6][7][8] Plexiform fibromyxomas arise in the stomach, are composed of bland spindle cells within a myxoid matrix, express SMA, and lack metastatic potential. 12 Gastroblastomas arise in the stomach, have both spindle cells often expressing SMA and epithelial components expressing cytokeratin and CD56, and have malignant potential. 11,[13][14][15][16][17][18] MALAT1-GLI1, ACTB-GLI1, and PTCH1-GLI1 gene fusions, or GLI1 amplifications, have also been reported in epithelioid tumors from diverse sites including soft tissue, skeletal muscle, bone, and retroperitoneum with variable S100 protein, cytokeratin, and CD56 expression and the potential for metastatic behavior.…”