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A rare angiosarcoma: retiform haemangioendothelioma

  • F O'Duffy (a1), C Timon (a1) and M Toner (a2)



We report the case of a rare angiosarcoma, retiform haemangioendothelioma, in an 18-year-old young man, which presented as a recurrent ulcerating lesion of the left pinna.


Case report and literature review of retiform haemangioendothelioma. This is a low grade angiosarcoma with a high local recurrence rate and low metastasis rate, and was first described in 1994 by Calonje et al.


This patient represents only the third report of lymph node metastasis in a case of retiform haemangioendothelioma. To date, 31 cases of the tumour have been reported. Histological diagnosis of this group of vascular neoplasms can be challenging, as their histopathological appearance is intermediate between haemangioma and angiosarcoma.


Surgical excision remains the primary treatment modality, with adjuvant radiotherapy recommended in patients with large tumour size, local recurrence and lymph node metastasis, as seen in this case.


Corresponding author

Address for correspondence: Mr Fergal O'Duffy, Royal Victoria Hospital, Adelaide Rd, Dublin 2, Ireland Fax: +353 14900643 E-mail:


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1Calonje, E, Fletcher, CD, Wilson-Jones, E, Rosai, J. Retiform haemangioendothelioma. A distinctive form of low-grade angiosarcoma delineated in a series of 15 cases. Am J Surg Pathol 1994;18:115–25
2Tan, D, Kraybill, W, Cheney, RT, Khoury, T. Retiform haemangioendothelioma: a case report and review of literature. J Cutan Pathol 2005;32:634–7
3Bhutoria, B, Konar, A, Chakrabarti, S, Das, S. Retiform hemangioendothelioma with lymph node metastasis: a rare entity. Indian J Dermatol Venereol Leprol 2009;75:60–2
4Duke, D, Dvorak, AM, Harris, T, Cohen, LM. Multiple retiform haemangioendotheliomias. A low-grade angiosarcoma. Am J Dermatopathol 1996;18:606–10
5Darouti, ME, Marzouk, SA, Sobhi, RM et al. Retiform haemangioendothelioma. Int J Dermatol 2000;42:290–2
6Schommer, M, Herbst, RA, Brodersen, JP, Kiehl, P, Katenkamp, D, Kapp, A et al. Retiform haemangioendothelioma: another tumour associated with human herpes virus type 8? J Am Acad Dermatol 2000;39:365–8
7Caljonje, E, Fletcher, CDM. Tumours of blood vessels and lymphatics. In: Fletcher, CDM, ed. Diagnostic Histopathology of Tumours, 2nd edn.London: Churchill Livingstone, 2000;1:4586
8Zhang, G, Lu, Q, Yin, H, Wen, H, Su, Y, Li, D et al. A case of retiform-hemangioendothelioma with unusual presentation and aggressive clinical features. Int J Clin Exp Pathol 2010;3:528–33
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The Journal of Laryngology & Otology
  • ISSN: 0022-2151
  • EISSN: 1748-5460
  • URL: /core/journals/journal-of-laryngology-and-otology
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