Case Report
Copyright ©The Author(s) 2020. Published by Baishideng Publishing Group Inc. All rights reserved.
World J Clin Cases. May 26, 2020; 8(10): 2009-2015
Published online May 26, 2020. doi: 10.12998/wjcc.v8.i10.2009
Effective combined therapy for pulmonary epithelioid hemangioendothelioma: A case report
Xiu-Qin Zhang, Heng Chen, Shu Song, Yan Qin, Li-Ming Cai, Fang Zhang
Xiu-Qin Zhang, Li-Ming Cai, Fang Zhang, Department of Respiratory Medicine, Affiliated Hospital of Jiangnan University, Wuxi 214000, Jiangsu Province, China
Heng Chen, Department of Hematology Medicine, Wuxi People’s Hospital, Wuxi 214000, Jiangsu Province, China
Shu Song, Department of Pathology Medicine, Shanghai Public Health Clinical Center, Fudan University, Shanghai 200000, China
Yan Qin, Department of Pathology Medicine, Affiliated Hospital of Jiangnan University, Wuxi 214000, Jiangsu Province, China
Author contributions: Zhang XQ and Chen H contributed equally to this work; Zhang XQ and Chen H conducted the study and drafted the manuscript; Song S and Qin Y performed the pathological examination of P-EHE; Cai LM was involved in follow-up of the patient; Zhang F participated in the design of case report.
Supported by Suitable Technology Project of Wuxi, No. T201911.
Informed consent statement: The patient provided informed consent at the time of investigation.
Conflict-of-interest statement: The authors of this work declare no conflict of interest.
CARE Checklist (2016) statement: The authors have read the CARE Checklist (2016), and the manuscript was prepared and revised according to the CARE Checklist (2016).
Open-Access: This article is an open-access article that was selected by an in-house editor and fully peer-reviewed by external reviewers. It is distributed in accordance with the Creative Commons Attribution NonCommercial (CC BY-NC 4.0) license, which permits others to distribute, remix, adapt, build upon this work non-commercially, and license their derivative works on different terms, provided the original work is properly cited and the use is non-commercial. See: http://creativecommons.org/licenses/by-nc/4.0/
Corresponding author: Fang Zhang, MD, Chief Physician, Department of Respiratory Medicine, Affiliated Hospital of Jiangnan University, 200 Huihe Street, Binhu District, Wuxi 214000, Jiangsu Province, China. kaikai20130912@sina.com
Received: February 13, 2020
Peer-review started: February 13, 2020
First decision: April 1, 2020
Revised: April 13, 2020
Accepted: April 24, 2020
Article in press: April 24, 2020
Published online: May 26, 2020
Abstract
BACKGROUND

Pulmonary epithelioid hemangioendothelioma (P-EHE) is a rare disease. Thus far, consensus on a standard treatment for P-EHE has not been established given its low incidence worldwide. Apatinib combined with chemotherapy with doxorubicin/cyclophosphamide has been used as an effective combination treatment for human malignancies. However, the efficacy of this combination has not been reported in P-EHE cases.

CASE SUMMARY

We present the case of a 64-year-old woman with chest tightness, cough, and chest pain. Computed tomography showed multiple unresectable pulmonary nodules. She had been misdiagnosed with lung carcinoma and underwent gefitinib treatment at a hospital. Subsequently, the patient underwent a cardiothoracic surgery for further disease investigation. CD31, CD34, and Vimentin expression were detected in the resected nodule specimens by immunohistochemical analyses, and pathological analyses confirmed the diagnosis of P-EHE. Following this, four cycles of apatinib combined with chemotherapy with doxorubicin/cyclophosphamide were initiated. The patient demonstrated stabilization of multiple bilateral nodules and showed a dramatic improvement in the clinical presentation after combination treatment. The patient could not tolerate the side effects of chemotherapy. Therefore, she then continued apatinib monotherapy, which is ongoing to date. The patient was stable at the last follow-up after 24 mo.

CONCLUSION

Apatinib combined with chemotherapy with doxorubicin/cyclophosphamide may be an effective therapeutic option for P-EHE treatment.

Keywords: Pulmonary epithelioid hemangioendothelioma, Apatinib, Doxorubicin, Cyclophosphamide, Combination therapy, Prognosis, Case report

Core tip: Pulmonary epithelioid hemangioendothelioma (P-EHE) is a rare disease. Patients with P-EHE demonstrate multiple unilateral or bilateral nodules that are often misdiagnosed as lung cancer or suspected to represent lung metastases. Meanwhile, currently, no standard treatment for this rare disease exists, and patients with P-EHE show a poor prognosis. Therefore, an effective therapeutic strategy for these patients is urgently required.