Retiform hemangioendothelioma D48.1

Author: Prof. Dr. med. Peter Altmeyer

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Last updated on: 07.08.2021

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Hobnail hemangioendothelioma

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Calonje, 1994

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Extremely rare, intralymphatic growing vascular tumor. To what extent this tumor type is clearly different from the hemangioendothelioma of the Dabska type (this type primarily affects children) is currently still controversial.

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All age groups from the 1st-8th decade of life can be affected. Women >Men. In a smaller series (n=8), the mean age was 40 years (range: 13-69 years) (Lao IW et al. 2015).

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Lower and upper extremties; trunk (also genitals) and scalp. One case occurred in chronic lymphedema. Another case occurred after radiotherapy for carcinoma of the cervix (Calonje E et al 1994) . But also internal organs such as the skeletal system and the spleen can be primarily affected (Lao IW et al. 2015)

Clinical features
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Not specific. See below Hemangioendothelioma, Dabska type. The vascular tumor manifests as a slowly growing, exophytic, nodular or plaque-like, bluish translucent or skin-colored, elevation. Pain is reported in isolated cases (Zhang G et al. 2010).

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Striking morphological features are the presence of long, branched blood vessels arranged in a reticular pattern. The vascular spaces are lined by monomorphic Hobnail endothelial cells. Characteristic is a duet lymphocytic infiltrate , as well as the focal appearance of papillary structures which are also present in endovascular or intralymphatic papillary angioendothelioma(Dabska tumor).

In immunohistochemistry, the cases consistently show positivity for endothelial markers, including CD31, human coagulation factor VIII, CD34.

Differential diagnosis
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Retiform hemangioendothelioma proves to be a low-grade neoplasm. Postoperative recurrences are common due to the frequent multicentric growth (extent of the tumor is underestimated). The metastasis rate is low. Tumor-related deaths do not occur (Calonje E et al. 1994).

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  1. Calonje E et al. (1994) Retiform hemangioendothelioma. A distinctive form of low-grade angiosarcoma delineated in a series of 15 cases. Am J Surg Pathol 18: 115-125
  2. Chundriger Q et al (2021) Retiform hemangioendothelioma: a case series and review of the literature. J Med Case Rep 15:69
  3. El Darouti M et al (2000) Retiform hemangioendothelioma. Int J Dermatol 39: 365-368
  4. Kaplan GO et al (2021) Papillary intralymphatic angioendothelioma: An extremely rare tumor. Jt Dis Relat Surg 32:245-248.

  5. Lao IW et al (2015) Retiform hemangioendothelioma: a clinicopathologic analysis of 8 cases. Zhonghua Bing Li Xue Za Zhi 44:480-485.

  6. Mentzel T et al (2002) Tumors of the lymphatic vessel of the skin and soft tissue. Pathologist 23: 118-27
  7. Mentzel T et al (1994) Vascular tumors of the skin and soft tissue. Overview of newly characterized entities and variants.Pathologe 15: 259-270
  8. Mondal A et al (2020) Retiform hemangioendothelioma: An uncommon vascular neoplasm. Indian J Pathol Microbiol 63:122-124.
  9. Schommer M et al (2000) Retiform hemangioendothelioma: another tumor associated with human herpesvirus type 8? J Am Acad Dermatol 42: 290-292
  10. Zhang G et al (2010) A case of retiform-hemangioendothelioma with unusual presentation and aggressive clinical features. Int J Clin Exp Pathol 3:528-533.


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Last updated on: 07.08.2021