INTRODUCTION:Pulmonary epithelioid hemangioendothelioma (PEHE) is a rare condition with very poor prognosis. To date approximately 50 cases have been described in the literature.We report a case of PEHE in which the tumour re-occurred despite adequate resection and no evidence of metastasis at the time of surgery. If there is progression of the disease, to date there is no single effective treatment.
CASE PRESENTATION:62 y/o retired Caucasian policeman with a PMH of Hypertension,DM & tuberculosis presented with non productive cough for one month. He denied any weight loss, change in voice or hemoptysis. He had no known allergies and was non smoker with no alcohol or illicit drug abuse. A chest x-ray showed a mass in the left posterior lobe. A CT scan revealed 4 × 3 cm irregular soft tissue posterior mass, a subsequent PET scan focal hypermetabolic uptake, corresponding to the area of the mass with no other systemic uptake. The patient underwent thoracotomy and left lower lobe lobectomy. Histology revealed cells arranged in small groups in a myoid stroma, with areas of surrounding necrosis confirming PEHE. Patient remained well 3 years into his follow-up, when he suddenly developed left hip pain. A subsequent biopsy confirmed the reoccurrence of PEHE and PET scan showed multiple bony uptake and lytic lesions (including sternum, left iliac crest and right sacrum bone) with new multiple pulmonary nodules. He is 1 ½ year in his follow-up with poor response to chemotherapy, there is minimum regression of the disease and areas of new uptake in ribs, left ileum and S1 vertebra.
DISCUSSIONS:Dail et al first described PEHE in 1975 (1).Weiss et al were the first to use the term PEHE. It can arise from many organ systems, including liver, bone, and soft tissues simultaneously or sequentially. According to the last WHO Classification of Tumors, PEHE is classified as a malignant soft tissue tumor.PEHE is often diagnosed incidentally, presenting symptoms are usually referable to an enlarging mass.Some lesions may be painful, this is thought to be related to vascular occlusion. It is four times more common in women, with a mean onset of 35 years; 50% are < 40 years old. Patients with fibrinous pleuritis and extrapleural proliferation of tumor cells or spindle tumor cells generally have a much worse prognosis. Death due to PEHE occurs from, 13% in soft tissue to 35% in liver and 70% in lung.The most characteristic feature of PEHE on chest radiograph or CT is the presence of multiple perivascular nodules with well or ill-defined margins in both lungs. They are found in relation to small and medium-sized vessels and bronchi. Histologically, PEHE exhibits an infiltrative growth pattern with ill defined margins. Approximately 50% involve/arise from blood vessels. When they involve vessels, they expand the vessel, filling the lumen with debris, and then infiltrating in a whirling pattern through the vessel wall into the surrounding tissue. It consists of cords and strands of epithelioid cells that are typically set in a variably myxohyaline stroma (2).
CONCLUSION:PEHE are aggressive tumors of pulmonary vasculature. Surgical excision is attempted if there is no distant metastasis. The effectiveness of chemotherapy and radiotherapy is ill defined at this time. The prognosis is very poor if the tumor progress with high mortality.
DISCLOSURE:Amir Khan, None.