Key result
MDM2 immunohistochemistry confirms diagnosis of rare pulmonary vein intimal sarcoma.
Why the study?
Pulmonary vein intimal sarcoma is an extremely rare and aggressive neoplasm that can be challenging to diagnose.
Case Report (n=1)
Intimal sarcoma of the pulmonary vein is an extremely rare and aggressive neoplasm that can mimic atrial myxoma, highlighting the diagnostic importance of MDM2 immunohistochemistry.
MDM2 IHC may aid diagnosis of rare pulmonary vein sarcomas mimicking myxoma; leaves open standardized use in atypical atrial masses.
Intimal sarcoma is an uncommon, aggressive neoplasm originating in the tunica intima of large vessels. Although most cases arise in the pulmonary artery, primary involvement of the pulmonary veins is exceptional. We present the case of a 52-year-old woman who presented to the emergency department with acute heart failure and dyspnoea. Imaging studies initially suggested an atrial myxoma. Surgical resection of the lesion revealed a proliferation characterised by a relatively monotonous spindle-cell proliferation within the pulmonary vein and a separate zone showing greater pleomorphism, necrosis, and atypical mitoses within the left atrial cavity. Immunohistochemistry demonstrated nuclear positivity for MDM2, supporting the diagnosis. Pulmonary vein intimal sarcoma is an extremely rare entity that can be challenging to diagnose. Detection of MDM2 by immunohistochemistry is a key diagnostic tool.
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Sambuelli et al. (2026) conducted a case report in Intimal sarcoma of the pulmonary vein (n=1). MDM2 immunohistochemistry was evaluated. MDM2 detection by immunohistochemistry successfully confirmed the diagnosis of an extremely rare pulmonary vein intimal sarcoma in a 52-year-old woman.
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