Plexiform Fibromyxoma: A Rare Gastric Tumor

Casmir Wambura,Salim Surani

doi:10.1155/2017/4014565

Abstract

Plexiform fibromyxoma is a rare and distinctive benign mesenchymal neoplasm that occurs in the gastric antrum. This tumor has a potential for misdiagnosis as gastrointestinal stromal tumor (GIST). It causes mucosa and vascular ulcerations without advancement of the tumor. Cytological bland spindle cells within a variably myxoid stroma characterize the histology of the tumor. We report the case of a 41-year-old African Tanzanian lady who presented with melena and recurrent anemia. Endoscopy and imaging studies revealed antral mass with initial suspicion of a GIST. However, immunohistochemically it turned to be a plexiform fibromyxoma. Follow-up evaluation 12 months after surgery revealed no evidence of recurrence or metastasis. This is a very uncommon tumor, which, to our knowledge, has been reported only once in Africa. The clinicians need to be aware of this rare occurrence to avoid misdiagnosis as GIST tumor.

Highlights

Plexiform fibromyxoma is a recently described gastric tumor with peculiar plexiform pattern, bland spindle cells, and a myxoid stroma invading the blood vessels [1]
The tumor cells are positive for α smooth muscle actin (SMA) and variable for CD10 and consistently negative for KIT, DOG1, CD34, Desmin, and S100 protein [1, 3, 6]
Plexiform fibromyxoma is a distinctive benign gastric antral neoplasm that should be separated from gastrointestinal stromal tumor (GIST), nerve sheath tumors, and other fibromyxoid neoplasms [7]

Summary

Introduction

Plexiform fibromyxoma is a recently described gastric tumor with peculiar plexiform pattern, bland spindle cells, and a myxoid stroma invading the blood vessels [1]. Typical plexiform intramural growth has multiple micronodules containing paucicellular to moderately cellular myxoid to collagenous and fibromyxoid neoplastic elements [4]. A prominent plexiform capillary pattern is typically present. An extramural component includes subserosal nodule, which can be cellular and solid and can have plexiform spindle cell proliferation. No KIT or platelet-derived growth factor receptor alpha mutations are present [2]. None of the patients developed recurrence or metastases. Plexiform fibromyxoma is a distinctive benign gastric antral neoplasm that should be separated from GIST, nerve sheath tumors, and other fibromyxoid neoplasms [7]. We hereby present a case of a 41-year-old female presenting with recurrent anemia

Case Description

Findings

Discussion

Conclusion