Skin nonmelanocytic tumor
Vascular tumors
Hemangioendothelioma-retiform
Author: Christopher S. Hale, M.D.
Last author update: 1 August 2012
Last staff update: 16 April 2021
Copyright: 2003-2023, PathologyOutlines.com, Inc.
PubMed search: retiform hemangioendothelioma skin
Table of Contents
Definition / general | Case reports | Treatment | Gross description | Microscopic (histologic) description | Microscopic (histologic) images | Positive stains | Negative stains | Differential diagnosisCite this page: Hale CS. Hemangioendothelioma-retiform. PathologyOutlines.com website. https://www.pathologyoutlines.com/topic/skintumornonmelanocytichemangioendotheliomaretiform.html. Accessed June 9th, 2023.
Definition / general
- Low grade variant of angiosarcoma
- Usually distal extremities of young individuals
- Weiss and Goldblum use term “hobnail hemangioendothelioma” for retiform and Dabska type tumors, which they believe to be closely related
- Rarely multiple (Am J Dermatopathol 1996;18:606)
- 2 / 3 recur, particularly without wide local excision
- Low rate of metastases, no tumor related deaths (Am J Surg Pathol 1994;18:115)
Case reports
- 37 year old man with lesion on back (Case of the Week #107)
Treatment
- Wide local excision
Gross description
- Lesion of reticular dermis and subcutaneous tissue
Microscopic (histologic) description
- Retiform (net-like, similar to rete testis) pattern of blood vessels that disperse through reticular dermis and subcutis
- Vessels lined by monomorphic hobnail endothelial cells with scant cytoplasm and rounded, naked type nuclei
- Often prominent lymphocytic infiltrate
- No epithelioid areas or cytoplasmic vacuoles (Am J Surg Pathol 1994;18:115)
Microscopic (histologic) images
Case #107
Various images
CD31
Positive stains
- Endothelial cells - CD34 (strong), CD31, vWF
- Also VEGFR3 (Mod Pathol 2000;13;180)
Negative stains
- Endothelial cells - keratin
Differential diagnosis
- Angiosarcoma: may focally have low grade features, but also exhibits areas of marked atypia and pleomorphism; also dissects between individual collagen bundles and has mitotic activity)
- Hobnail hemangioma: smaller, more superficial and more localized, with papillary dermal vessels that disappear into reticular dermis
