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Year : 2022  |  Volume : 9  |  Issue : 2  |  Page : 65-68

Total metabolic regression after everolimus in an adult patient with pseudomyogenic hemangioendothelioma

1 Department of Oncology, National Taiwan University Hospital, Taipei, Taiwan
2 Department of Dermatology, National Taiwan University Hospital and National Taiwan University College of Medicine, Taipei, Taiwan
3 Department of Pathology, National Taiwan University Hospital, Taipei, Taiwan

Correspondence Address:
Dr. Tom Wei-Wu Chen
Department of Oncology, National Taiwan University Hospital, No.7, Chung Shan S. Rd., Taipei 100225
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/2311-3006.346409

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Pseudomyogenic hemangioendothelioma (PHE) is a rare vascular tumor harboring the pathognomonic SERPINE1-FOSB fusion. Most patients are treated primarily with surgical resection, but some patients require systemic therapy due to multiple metastases at initial presentation or multifocal recurrence after definitive resection. The most optimal treatment strategy for the disease has yet to be clearly defined; however, mammalian target of rapamycin inhibitors show promise-given–reported anecdotal responses from case reports of pediatric patients. We present an adult patient with multifocal PHE who was successfully treated with everolimus with a significant clinical response.

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