Hemangioendothelioma epitheloides C49.-

Author: Prof. Dr. med. Peter Altmeyer

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Last updated on: 29.10.2020

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hemangioendothelioma epithelioid; malignant epithelioid haemangioendothelioma

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Rare, low-grade malignant tumour of the blood vessels, which grows predominantly in the superficial and deep soft tissues and the muscles of the extremities and also in internal organs (lungs, liver, bones, mediastinum).

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Occurs in middle age; sporadically also in childhood; no sex preference.

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Deep soft tissues: only rarely direct skin infection.

Clinical features
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  • Only rarely isolated skin infestation; manifestation mostly in combination with underlying soft tissue infestation.
  • Solitary, soft tissue masses located in the deep soft tissues; little pain. Cutaneous involvement manifests itself in uncharacteristic red, coarse papules and nodules reminiscent of a satellite of the Granuloma pyogenicum. The tumours often tend to recur postoperatively. 20-30% of the tumors metastasize, mostly in lymph nodes, lung or liver.

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  • Infiltration through large, epitheloid, partly spindle cells, which occur in fascicular or nest-like cell assemblies. Tumor cells with prominent nuclei and clearly protruding eosinophilic cytoplasm. Again and again intracytoplasmic vacuoles appear, occasionally with erythrocytes. Mature vascular lumina are rarely encountered. Typical is a mucoid or chondroid stroma. Circumscribed areas with angiosarcoma-like structures.
  • Immunohistology: Vascular markers (factor VIII; CD31; CD34) and cytokeratin occasionally positive.
  • Cytogenetically, a translocation t(1;3)(p36.3;q25) is considered an important diagnostic tool.

Differential diagnosis
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Radical excision (if not multifocal) with sufficient safety distance. Therapeutic approaches with interferon alfa (3 times/week 3 million IU), liposomal doxorubicin or also radiotherapy have been reported (radiotherapeutic measures are to be described as poor).

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Dermal tumours have a better prognosis than visceral ones; poor prognosis in case of visceral involvement. In metastasized patients about 50% die as a result of metastasis!

Case report(s)
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  • The 35-year-old patient (locksmith) reported painless red nodule on the back of the hand, which initially existed for 1 several months. Within a few eruptive shots, numerous painless, soft to bulging elastic, red or blue papules and nodes with a smooth surface, limited to the hand and forearms. During the initial examination, solid, well defined nodules up to 3 cm in size were palpated in the subcutis and even deeper. Rö/MRT: osseous destruction in the middle steel bone and in the distal radius. Other diagnostic examinations without laboratory: No esoinophilia (differentiation to Kimura disease).
  • Histology: Lobular, relatively mature vascular tumor (CD31+) with irregular vascular structures and moderate proliferation.
  • Therapy: As surgical repair seemed to be impossible, therapy with interferon-alfa (3 times/week 3 million IU); temporarily slight reduction of proliferation activity, after renewed progression radiotherapy (2 Gy ED, GD: 40 Gy) without success. Therefore use of liposomal doxorubicin (Caelyx: fortnightly, 20 mg/m2 i.v.); including a clear and satisfactory response.

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  1. Anderson PJ et al (2003) The use of sentinel node biopsy in the management of epitheloid haemangioendothelioma of the lip. Oral Oncol 39: 531-533
  2. Gosheger G et al (2002) The multicentric epitheloid hemangioendothelioma of bone: a case example and review of the literature. J Cancer Res Clin Oncol 128: 11-18
  3. Gray MF, Rosenberg AE, Dickersin GR et al (1990) Cytokeratin expression in epithelial vascular neoplasms. Hum Path 21: 211-214
  4. Li H et al (2015) Epithelioid hemangioendothelioma: a clinicopathologic analysis of 13 cases. Zhonghua Bing Li Xue Za Zhi 44:386-389.
  5. Park SM et al (2016) Cutaneous epithelioid hemangioendothelioma treated with Mohs micrographic surgery. Int J Dermatol doi: 10.1111/ijd.13299.
  6. Quante M et al (1998) Epithelioid hemangioma presenting in the skin. A clinicopathological study of eight cases. Am J Dermatopathol 20: 541-546
  7. Resnik KS, Cantor GR, Spielvogel RL et al (1993) Cutaneous epitheloid hemangioendothelioma without systemic involvement. At J Dermatopathol 15: 272-275


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Last updated on: 29.10.2020