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Review Article
- A comprehensive review of ossifying fibromyxoid tumor: insights into its clinical, pathological, and molecular landscape
- Kyriakos Chatzopoulos, Antonia Syrnioti, Mohamed Yakoub, Konstantinos Linos
- J Pathol Transl Med. 2026;60(1):6-19. Published online January 14, 2026
- DOI: https://doi.org/10.4132/jptm.2025.10.02
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Abstract
PDF - Ossifying fibromyxoid tumor (OFMT) is a rare mesenchymal neoplasm first described in 1989. It typically arises in the superficial soft tissues of the extremities as a slow-growing, painless mass. Histologically, it is commonly characterized by a multilobular architecture composed of uniform epithelioid cells embedded in a fibromyxoid matrix, often surrounded by a rim of metaplastic bone. While classic cases are readily identifiable, the tumor's histopathological heterogeneity can mimic a range of benign and malignant neoplasms, posing significant diagnostic challenges. Molecularly, most OFMTs harbor PHF1 rearrangements, commonly involving fusion partners such as EP400, MEAF6, or TFE3. This review underscores the importance of an integrated diagnostic approach- incorporating histopathological, immunohistochemical, and molecular data- to accurately classify OFMT and distinguish it from its mimics. Expanding awareness of its morphologic and molecular spectrum is essential for precise diagnosis, optimal patient management, and a deeper understanding of this enigmatic neoplasm.
Case Study
- Hepatic carcinoma expressing inhibin: case report of a proposed novel entity and review of the literature
- Antonia Syrnioti, Evangelia Athanasiou, Prodromos Hytiroglou
- J Pathol Transl Med. 2022;56(4):225-230. Published online June 15, 2022
- DOI: https://doi.org/10.4132/jptm.2022.04.07
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- 3 Web of Science
- 3 Crossref
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Abstract
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- Hepatic carcinoma expressing inhibin is a recently described neoplasm with varied architecture, including trabecular, pseudoglandular, follicular/microcystic, organoid, solid and tubular patterns of growth. We report a case of hepatic carcinoma expressing inhibin that occurred in a 47-year-old woman presenting with epigastric and back pain. The tumor was located in the left hepatic lobe and measured 12 cm in diameter. On immunohistochemical stains, the neoplastic cells were positive for inhibin, as well as cytokeratins 7, 8/18 and 19. There was mild focal expression of synaptophysin, and lack of expression of hepatocytic markers. The histogenesis of hepatic carcinoma expressing inhibin is presently uncertain. From a practical point of view, this neoplasm can potentially cause diagnostic pitfalls by simulating other primary or metastatic tumors, such as hepatocellular carcinoma, cholangiocarcinoma, neuroendocrine tumors, and follicular carcinoma of thyroid gland. Performing inhibin immunostain could assist in the differential diagnosis of liver tumors with unusual histologic features.
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Citations
Citations to this article as recorded by
- Cytologic Findings of Cholangioblastic Variant of Intrahepatic Cholangiocarcinoma: A Rare Variant and Cytologic Pitfall
Eleonora Fiorletta Quiroga, Maria Luisa C. Policarpio‐Nicolas
Diagnostic Cytopathology.2026; 54(1): 43. CrossRef - Cholangioblastic Cholangiocarcinoma (NIPBL::NACC1 Cholangiocarcinoma)
Pedram Argani, Kiyoko Oshima, Robert A. Anders, Raul S. Gonzalez, Osman Yilmaz, Munita Bal, Lisa Rooper, Jessica Hicks, Angelo De Marzo, Jeffrey Gagan, Chengsong Zhu, Doreen N. Palsgrove
American Journal of Surgical Pathology.2025; 49(4): 303. CrossRef - Primary Peritoneal Hepatoid Adenocarcinoma: A Multidisciplinary Approach for a Rare Case Scenario
Mahmoud A. Elseadany, Fatmaelzahraa Abdelfattah Denewar, Reham Mohamed Nagib, Raghda Tarek
Indian Journal of Gynecologic Oncology.2025;[Epub] CrossRef
- Cytologic Findings of Cholangioblastic Variant of Intrahepatic Cholangiocarcinoma: A Rare Variant and Cytologic Pitfall
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