A 5‐year survivor of endarterectomy for sclerosing undifferentiated intimal sarcoma of the pulmonary artery: Importance of clinical suspicion and careful histologic evaluation

Author:

Amemiya Kisaki1ORCID,Nishihira Morikazu12,Ishibashi‐Ueda Hatsue1,Ohta‐Ogo Keiko1,Ogo Takeshi2,Ikeda Yoshihiko1,Hatakeyama Kinta1,Sasaki Hiroaki3,Ogino Hitoshi34

Affiliation:

1. Department of Pathology National Cerebral and Cardiovascular Center Suita Japan

2. Department of Cardiovascular Medicine National Cerebral and Cardiovascular Center Suita Japan

3. Department of Cardiovascular Surgery National Cerebral and Cardiovascular Center Suita Japan

4. Department of Cardiovascular surgery Tokyo Medical University Hospital Tokyo Japan

Abstract

AbstractWe present a diagnostically challenging case of intimal sarcoma of the pulmonary artery (PA) due to the histologic finding of a sclerosing appearance with no appreciable spindle/pleomorphic cell proliferation. Initial endarterectomy specimens were composed of sclerosing extracellular matrix with a few bland cells, some recanalization, and fibrin thrombi, impeding the confirmation of intimal sarcoma as these findings were also consistent with chronic thromboembolic pulmonary hypertension. However, the patient experienced recurrence 5 years later, and the second endarterectomy specimens revealed more firm and solid mass and the proliferation of atypical spindle/pleomorphic cells within a myxomatous matrix in the distal PA, leading to the definitive diagnosis of undifferentiated intimal sarcoma of the PA. The archival specimens from the endarterectomy confirmed intense MDM2 expression by immunohistochemistry, suggesting its role as a potential diagnostic marker for intimal sarcoma. This case highlights that prominent sclerosing extracellular matrix with very few atypical cells should raise the possibility of intimal sarcoma of the PA and that high index of suspicion, generous sampling, and ancillary tests are critical for accurate diagnosis. In this case, the tumor was incidentally removed by endarterectomy, resulting in 5 years of survival.

Publisher

Wiley

Subject

Pulmonary and Respiratory Medicine

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