Pulmonary epithelioid hemangioendothelioma

05/2018

MUDr. Petr Jakubec, Ph.D.

Klinika plicních nemocí a tuberkulózy FN a LF UP Olomouc

SUMMARY

Pulmonary epithelioid hemangioendothelioma (PEH) is a rare sarcomatous tumor. The disease affects more women and has the highest incidence in lower middle age. There is currently no diagnostic clinical or laboratory PEH marker. The clinical picture of the disease is nonspecific and very variable. The PEH diagnosis is based on histological, immunohistochemical examination of a biopsy tumor sample. Because of the low degree of malignancy, a relatively good prognosis for solitary disorder and sometimes spontaneous remission occurs. The opposite is the case for multiple lesions, including metastases or multicenter affections of different organs, where they behave very aggressively, and any treatment is usually ineffective. The only standard treatment is resection for operable lesions. There is currently no demonstrably effective PEH therapy. The course of the disease is very variable, and the prognosis of the disease is unpredictable.

KEY WORDS

epithelioid hemangioendothelioma, lung, diagnostics, management, prognosis