A Rare Case of Pulmonary Epithelioid Hemangioendothelioma Presenting with Skin Metastasis

Abstract

Epithelioid hemangioendothelioma (EHE) is a well-differentiated and rare vascular tumor. Systemic metastases are uncommon. Herein, we present a patient with skin metastasis of pulmonary EHE (PEH) that was treated by wide excision. A 76-year-old male was evaluated due to pulmonary thromboembolism and a solitary pulmonary nodule. A biopsy was performed and pathological examination of the mass confirmed EHE. No metastasis was observed. The patient returned to care approximately two years later due to a painful nodule in the right lower leg. A skin biopsy showed metastatic EHE from the lung. We used a safety margin of 1 cm based on clinical experience, because no prior case had been reported regarding the resection margin appropriate for primary cutaneous EHE and skin metastases of PEH. At four months after surgery, the patient recovered without complications or recurrence. Skin metastasis of PEH is extremely rare, and only two cases have been reported in the literature. In this case, we report a rare case of PEH with histologically diagnosed skin metastasis that was successfully treated by curative resection. It is expected that this case report will provide a helpful contribution to the extant data regarding PEH metastases.

Primary cutaneous epithelioid hemangioendothelioma with lymph node metastasis

“…Cutaneous EHE may result from an internal tumor that metastasizes to the skin or may be a primary lesion with or without associated systemic involvement. 4 , 5 , 6 , 9 As in the case with our patient, tumor can spread from the skin to other structures through the lymphatics. In general, EHE is considered to be of moderate malignant potential and intermediate potential to hemangiomas and angiosarcomas in both metastatic and mortality rates, although more data specific to cutaneous EHE are needed.…”

“…Reports of EHE presenting as an erythematous dermal nodule, ulceration, or purplish-red papule or nodule highlight a wide array of morphologies ( Fig 4 ). 1 , 4 , 5 , 6 , 8 , 9 Interestingly, multiple EHEs have been reported in a single patient, although most cases are of solitary lesions. 8 Tumors can be found anywhere on the body, and a spectrum of involvement sites has been reported.…”

Pulmonary Epithelioid Hemangioendothelioma: Two Case Reports of a Rare Neoplasm with an Unpredictable Prognosis

“…This patient was diagnosed with PEH after presenting with cough and weight loss and died less than 9 months afterwards despite treatment with radiation, oral kinase inhibitor, and bronchial stenting vena cava syndrome [8], mediastinal nodal methastases [9], and pleural invasion [10]. Systemic metastases or synchronic lesions involving central nervous system [11], skin [12], liver, spleen, and bone have also been reported [13].…”

Case report: A rare presentation of high risk epithelioid hemangioendothelioma on leg treated with surgical excision

“…Hemangioendotheliomas are considered to be tumors of intermediate malignancy because of their propensity to recur and occasionally metastasize, but at a rate lower than that of classical angiosarcomas [2]. There are several subtypes of hemangioendotheliomas; kaposiform hemangioendothelioma, papillary intralymphatic angioendothelioma, retiform pseudomyogenic hemangioendotheioma, and EHE [3].…”

“…Lesions presenting in this fashion can represent either a primary lesion or a secondary site of metastasis. Due to the limited literature on the subject, it is not clear which age group is most affected, but it seems to appear in young to middle aged individuals more often [[1], [2], [3]]. There have been reported cases of EHE, to our knowledge, from age 9–93 [1].…”