Frequency of Plexiform Fibromyxoma relative to gastrointestinal stromal tumor: A single center study

Arslan, Mustafa Erdem; Hua, Li; Jennings, Timothy A.; Lee, Edward C.; Nigam, Ankesh; Lee, Hwajeong

doi:10.1016/j.anndiagpath.2020.151568

Cited by 11 publications

(20 citation statements)

References 19 publications

Supporting

Mentioning

Contrasting

Unclassified

Order By: Relevance

“…PFs have been reported as either case reports or small case series. To date, a total of 130 PF cases have been reported in the English literature[ 1 , 6 , 16 - 22 ]. Given the fact that only nineteen cases had been described by 2010 (approximately three years after the first description of the entity), this remarkable increase in its incidence in a short period of time may be attributable to the increased awareness of PF by clinicians and pathologists[ 1 , 7 ].…”

Section: History and Nomenclaturementioning

confidence: 99%

“…The most common site for PF is gastric antrum[ 6 , 9 ]. In addition, it has been reported in the duodenum, jejunum, gallbladder and mediastinum[ 1 , 3 - 6 , 25 , 26 ].…”

Section: Clinical Presentationmentioning

confidence: 99%

“…Plexiform fibromyxoma (PF) is a recently described mesenchymal tumor of the gastrointestinal (GI) tract. Recently our group reported that the incidence of PF relative to GI stromal tumor (GIST), a close mimic of PF, is 1.7 % over a span of 20 years[ 1 ]. The tumor is mostly found in the gastric antrum, however PF involving other segments of the GI tract have been reported[ 1 - 5 ].…”

Section: Introductionmentioning

confidence: 99%

“…Recently our group reported that the incidence of PF relative to GI stromal tumor (GIST), a close mimic of PF, is 1.7 % over a span of 20 years[ 1 ]. The tumor is mostly found in the gastric antrum, however PF involving other segments of the GI tract have been reported[ 1 - 5 ]. Due to its rarity, the pathogenesis and molecular alterations of PF are largely unknown[ 6 ].…”

Section: Introductionmentioning

confidence: 99%

“…Due to its rarity, the pathogenesis and molecular alterations of PF are largely unknown[ 6 ]. However, its incidence appears to be rapidly increasing in the literature probably due to a growing awareness of this entity[ 1 , 7 ]. While PF behaves in a benign manner, its histologic mimics such as GISTs, smooth muscle tumors and nerve sheath tumors show malignant potential[ 8 , 9 ].…”

Section: Introductionmentioning

confidence: 99%

See 4 more Smart Citations

Plexiform fibromyxoma: Review of rare mesenchymal gastric neoplasm and its differential diagnosis

Arslan

et al. 2021

WJGO

Self Cite

View full text Add to dashboard Cite

Plexiform fibromyxoma (PF) is a very rare mesenchymal neoplasm of the stomach that was first described in 2007 and was officially recognized as a subtype of gastric mesenchymal neoplasm by World Health Organization (WHO) in 2010. Histologically, PF is characterized by a plexiform growth of bland spindle to ovoid cells embedded in a myxoid stroma that is rich in small vessels. The lesion is usually paucicellular. While mucosal and vascular invasion have been documented, no metastasis or malignant transformation has been reported. Its pathogenesis is largely unknown and defining molecular alterations are not currently available. There are other mesenchymal tumors arising in the gastrointestinal tract that need to be differentiated from PF given their differing biologic behaviors and malignant potential. Histologic mimics with spindle cells include gastrointestinal stromal tumor, smooth muscle tumor, and nerve sheath tumor. Histologic mimics with myxoid stroma include myxoma and aggressive angiomyxoma. Molecular alterations that have been described in a subset of PF may be seen in gastroblastoma and malignant epithelioid tumor with glioma-associated oncogene homologue 1 ( GLI1 ) rearrangement. The recent increase in publications on PF reflects growing recognition of this entity with expansion of clinical and pathologic findings in these cases. Herein we provide a review of PF in comparison to other mesenchymal tumors with histologic and molecular resemblance to raise the awareness of this enigmatic neoplasm. Also, we highlight the challenges pathologists face when the sample is small, or such rare entity is encountered intraoperatively.

show abstract

Section: History and Nomenclaturementioning

confidence: 99%

“…The most common site for PF is gastric antrum[ 6 , 9 ]. In addition, it has been reported in the duodenum, jejunum, gallbladder and mediastinum[ 1 , 3 - 6 , 25 , 26 ].…”

Section: Clinical Presentationmentioning

confidence: 99%