Informes de casos clínicos y médicos

A Case of Pulmonary Epithelioid Hemangioendothelioma with Surgical Resection

Abstract

Shinichiro Shimamatsu, Mitsuhiro Takenoyama, Makoto Edagawa, Ryo Toyozawa, Kaname Nosaki, Fumihiko Hirai, Masafumi Yamaguchi, Fumiyoshi Fushimi, Kenichi Taguchi, Takashi Seto and Yukito Ichinose

A 65-year-old female patient was found to have a nodular shadow on a chest X-ray. Computed tomography showed a well-defined tumor measuring 1.8 cm in diameter in the right middle lobe that was diagnosed to be adenocarcinoma by a transbronchial lung biopsy. The patient underwent right middle lobectomy and hilar and mediastinal lymph node dissection for stage IA primary lung cancer. The pathological diagnosis was pulmonary epithelioid hemangioendothelioma (PEH), which is a rare tumor of the lung. The postoperative course was uneventful, and she remains free of PEH recurrence at 26 months after the surgery. PEH is the currently preferred term for the neoplastic process originally described as intravascular bronchioloalveolar tumor in the lung. The estimated prevalence of epithelioid hemangioendothelioma is less than 1 in 1 million, PEH is a rare tumor derived from vascular endothelial cells, and it is difficult to diagnose it preoperatively. PEH is considered to have low-to-intermediate grade malignancy, but the tumor predominantly involves the liver, lungs, soft tissues, and can be multicentric, even resulting in systemic metastasis. We described a case of a single PEH found at a medical checkup that was treated with complete resection. Active surgical treatment is considered desirable for single cases.