Plexiform Fibromyxoma in the Stomach: Immunohistochemical Profile and Comprehensive Genetic Characterization

Author:

Di Mauro Annabella1ORCID,Rega Rosalia Anna1,Leongito Maddalena2ORCID,Albino Vittorio2,Palaia Raffaele2,Gualandi Alberto1,Belli Andrea2ORCID,D’Arbitrio Imma1,Moccia Pasquale1,Tafuto Salvatore3ORCID,De Chiara Annarosaria4,Ottaiano Alessandro5ORCID,Ferrara Gerardo1ORCID

Affiliation:

1. Pathology Unit, Istituto Nazionale Tumori, IRCCS Fondazione “G. Pascale”, 80131 Napoli, Italy

2. Department of Gastro-Hepato-Pancreato-Biliary Surgery, Istituto Nazionale Tumori, IRCCS Fondazione “G. Pascale”, 80131 Napoli, Italy

3. Sarcomas and Rare Tumors Unit, Istituto Nazionale Tumori, IRCCS Fondazione “G. Pascale”, 80131 Naples, Italy

4. Histopathology of Lymphomas and Sarcomas SSD, Istituto Nazionale Tumori, IRCCS Fondazione “G. Pascale”, 80131 Naples, Italy

5. Division of Innovative Therapies for Abdominal Metastases, Istituto Nazionale Tumori, IRCCS Fondazione “G. Pascale”, 80131 Naples, Italy

Abstract

Plexiform fibromyxoma (PF), also referred to as plexiform angiomyxoid myofibroblast tumor, is an exceedingly rare mesenchymal neoplasm primarily affecting the stomach. Herein, we present a case of PF diagnosed in a 71-year-old male with a history of lung cancer, initially suspected to have a gastrointestinal stromal tumor (GIST) of the stomach, who subsequently underwent subtotal gastrectomy. The histopathological and molecular features of the tumor, including mutations in ABL1, CCND1, CSF1R, FGFR4, KDR, and MALAT1-GLI1 fusion, are elucidated and discussed in the context of diagnostic, prognostic, and therapeutic considerations.

Publisher

MDPI AG

Reference26 articles.

1. Bosman, F.T., Carneiro, F., Hruban, R.H., and Theise, N.D. (2010). WHO Classification of Tumours of the Digestive System, International Agency for Research on Cancer (IARC) Press. [4th ed.].

2. Plexiform fibromyxoma: A distinctive benign gastric antral neoplasm not to be confused with a myxoid GIST;Miettinen;Am. J. Surg. Pathol.,2009

3. Plexiform Angiomyxoid Myofibroblastic Tumor of the Stomach;Takahashi;Am. J. Surg. Pathol.,2007

4. Gastric plexiform angiomyxoid myofibroblastic tumor;Sing;Pathol. Int.,2010

5. Plexiform angiomyxoid myofibroblastic tumor of the stomach: Report of two cases and review of the literature;Kang;Korean J. Pathol.,2012

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