21-year-old woman with an Unusual Tumor
Presented by Henry Foong, M.D.
Ipoh, Malaysia
October 6, 2001

History: Miss T is a 21-year-old student who has had mildly tender purpuric indurated patches on the right arm for 5 years. The lesions have increased in size and number over the previous two years. Lately new lesions appeared on the right lower arm. She is otherwise well and has no constitutional symptoms.

Examination: Ms. T has three purpuric lesions, 4-5cm by 5-6 cm in diameter, with ill-defined margins on the extensor surface of the right arm. Her regional nodes were not enlarged. There were no other significant findings.

A skin biopsy was done and it showed features consistent with epithelioid hemangioendothelioma. These show skin several nodular lesions in the deeper dermis, apparently following the courses of capillary structures. These nodules often include some vascular (capillary) lumina within them. They consist of pale vesicular cells consistent with endothelial cells but there have a vague epitheloid or histiocytic appearance. The nuclei are irregular and sometimes have clear vacuoles. There is no pleomorphism or mitotic figures. Spindle cell differentiation or kaposiform areas are not present. The epidermis is unremarkable.

Discussion:
A review of the literature using MEDLINE confirmed that this is a rare tumor with only 41 citations so far.

Epithelioid hemangioendothelioma arising in the skin is rare, with only 18 cases previously reported, 3 of which had associated bony involvement. There was only 1 fatality reported in a case of cutaneous epitheloid hemangioendothelioma. The patient died of lymph node and lung metastases 5 months after surgery. The common sites of involvement are the extremities, trunk, face, genitalia and scalp.

Questions:
This is a rare tumor usually affecting the bone, liver or lung. I would welcome other diagnostic suggestions and recommendations for therapeutic intervention that may be useful to offer this young woman.

Comments:
From David Goldman, M.D. 10/8/01:

What treatments have been tried with or without success in the other 18 cases that were cited? Has anyone tried embolization therapy? radiation? Mohs micrographic surgery?--just thoughts

From Andrew Carlson, M.D. 10/9/01:

Considering the clinical features and pathological findings, this is a case of TUFTED HEMANGIOMA aka angioblastoma (of Nakagawa) or in the english literature as progressive capillary hemangioma. This vascular tumor can be conceptualized as a intradermal variant of lobular capillary hemangioma/pyogenic granuloma. Tufted hemangioma is a benign tumor as no reports exist of "malignant transformation". Although there are reports of partial or complete regression, most cases are persistent. Treatment of tufted angioma is difficult, various modalities like glucocorticosteroids, Interferon-alpha, flashlamp-pumped pulsed dye laser and excision have been described with varying results.

Epithelioid hemangioendothelioma is a low grade angiosarcoma that can be difficult to differentiate from metastatic adenocarcinoma and is characterized by nests and cords of plump epithelioid to spindle cells in a fibromyxoid stroma replacing and infiltrating the dermis. Intracellular lumen formation is typical and found in so called blister cells that have numerous and coalescing cytoplasmic vacuoles.

From Daniel Carter, M.D. 10/9/01:

The second micro has a somewhat "glomeruloid" appearance, so a Glomeruloid Hemangioma as is seen in POEMS Syndrome could be considered.

From Robert Feinstein, M.D. 10/10/01:

The clinical photos do not show purpura well. However, the areas appear to be lichenified. I do not have anything else to add to the medical picture, but it certainly is an interesting case.

References:

1. Quante M, Patel NK, Hill S, et al. Epithelioid hemangioendothelioma presenting in the skin: a clinicopathologic study of eight cases. Am J Dermatopathol. 1998;20:541-546.

2. Bettencourt MS. Burton CS. Prieto VG. Bossen EH. Shea CR. Recurrent papules on the left upper extremity of a 50-year-old man. Archives of Dermatology. 137(3):365-70, 2001 Mar.

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