Multiple Eruptive Epithelioid Hemangiomas

A Subset of Cutaneous Cellular Epithelioid Hemangioma With Expression of FOS-B

Mar Llamas-Velasco, Werner Kempf, Carlo Cota, Maria Teresa Fernández-Figueras, Joyce Lee, Gerardo Ferrara, Christian Sander, Philip E. Shapiro, Luis Requena, Heinz Kutzner

Research output: Contribution to journalArticle

6 Citations (Scopus)

Abstract

There is a wide clinicopathologic spectrum of vascular proliferations characterized by the presence of epithelioid endothelial cells, comprising epithelioid hemangioma (EH)—pseudomyogenic (epithelioid sarcoma-like) hemangioendothelioma (PM-HAE), epithelioid hemangioendothelioma, and epithelioid angiosarcoma. Immunohistochemical FOS-B expression as well as FOS-B rearrangement (fluorescent in situ hybridization [FISH]) have recently been described as diagnostically relevant underpinnings of EH (restricted to osseous lesions) and PM-HAE. The aim of this study was to clinicopathologically characterize and to elucidate FOS-B expression in patients with eruptive lesions of the cellular variant of cutaneous EH. All cases of cutaneous cellular EH (n=16) showed strong diffuse immunohistochemical expression of FOS-B, in conjunction with positivity for ERG and nestin. Expression of MYC, CAMTA-1, AE1/3, and MNF116 was negative in all cases. FISH investigations did not show any sign of rearrangements for CAMTA-1 or MYC amplification. Negative-control cases included 15 lobular hemangiomas, 5 epithelioid angiosarcomas, and 5 nodular Kaposi sarcomas, all of which were negative for FOS-B. Positive-control cases included 15 angiolymphoid hyperplasia with eosinophilia cases, all of them being positive. In contrast with what has been published so far, cutaneous variants of cellular EH exhibit positive immunostaining for FOS-B. Remarkably, FOS-B expression is not restricted to the intraosseous subset of EH. For differential diagnosis of epithelioid vascular tumors, we therefore suggest a helpful panel of antibodies including CAMTA-1, TFE-3, FOS-B, and AE1/AE3. We point out the telltale immunophenotypes: angiolymphoid hyperplasia with eosinophilia and EH (FOS-B+/others negative), PM-HAE (FOS-B+/AE1/AE3+/others negative), epithelioid hemangioendothelioma (CAMTA-1+ or TFE-3+/others negative). Remarkably, MYC is not expressed in these tumors, neither is there an MYC amplification by FISH. We suggest the term multiple eruptive EHs for this subset of cutaneous vascular tumors.

Original languageEnglish
JournalAmerican Journal of Surgical Pathology
DOIs
Publication statusPublished - Dec 20 2017

Fingerprint

Hemangioma
Skin
Fluorescence In Situ Hybridization
Epithelioid Hemangioendothelioma
Angiolymphoid Hyperplasia with Eosinophilia
Blood Vessels
Hemangiosarcoma
Polytetrafluoroethylene
Hemangioendothelioma
Nestin
Epithelioid Cells
Neoplasms
Kaposi's Sarcoma
Sarcoma
Differential Diagnosis
Endothelial Cells
Antibodies

Keywords

  • angiosarcoma
  • CAMTA-1 protein
  • FOS-B protein
  • genes
  • human
  • MYC
  • vascular neoplasm
  • ZPF36-FOS-B fusion protein

ASJC Scopus subject areas

  • Anatomy
  • Surgery
  • Pathology and Forensic Medicine

Cite this

Multiple Eruptive Epithelioid Hemangiomas : A Subset of Cutaneous Cellular Epithelioid Hemangioma With Expression of FOS-B. / Llamas-Velasco, Mar; Kempf, Werner; Cota, Carlo; Fernández-Figueras, Maria Teresa; Lee, Joyce; Ferrara, Gerardo; Sander, Christian; Shapiro, Philip E.; Requena, Luis; Kutzner, Heinz.

In: American Journal of Surgical Pathology, 20.12.2017.

Research output: Contribution to journalArticle

Llamas-Velasco, Mar ; Kempf, Werner ; Cota, Carlo ; Fernández-Figueras, Maria Teresa ; Lee, Joyce ; Ferrara, Gerardo ; Sander, Christian ; Shapiro, Philip E. ; Requena, Luis ; Kutzner, Heinz. / Multiple Eruptive Epithelioid Hemangiomas : A Subset of Cutaneous Cellular Epithelioid Hemangioma With Expression of FOS-B. In: American Journal of Surgical Pathology. 2017.
@article{037de4d07f6f4eb08fb2fadf0696ae4d,
title = "Multiple Eruptive Epithelioid Hemangiomas: A Subset of Cutaneous Cellular Epithelioid Hemangioma With Expression of FOS-B",
abstract = "There is a wide clinicopathologic spectrum of vascular proliferations characterized by the presence of epithelioid endothelial cells, comprising epithelioid hemangioma (EH)—pseudomyogenic (epithelioid sarcoma-like) hemangioendothelioma (PM-HAE), epithelioid hemangioendothelioma, and epithelioid angiosarcoma. Immunohistochemical FOS-B expression as well as FOS-B rearrangement (fluorescent in situ hybridization [FISH]) have recently been described as diagnostically relevant underpinnings of EH (restricted to osseous lesions) and PM-HAE. The aim of this study was to clinicopathologically characterize and to elucidate FOS-B expression in patients with eruptive lesions of the cellular variant of cutaneous EH. All cases of cutaneous cellular EH (n=16) showed strong diffuse immunohistochemical expression of FOS-B, in conjunction with positivity for ERG and nestin. Expression of MYC, CAMTA-1, AE1/3, and MNF116 was negative in all cases. FISH investigations did not show any sign of rearrangements for CAMTA-1 or MYC amplification. Negative-control cases included 15 lobular hemangiomas, 5 epithelioid angiosarcomas, and 5 nodular Kaposi sarcomas, all of which were negative for FOS-B. Positive-control cases included 15 angiolymphoid hyperplasia with eosinophilia cases, all of them being positive. In contrast with what has been published so far, cutaneous variants of cellular EH exhibit positive immunostaining for FOS-B. Remarkably, FOS-B expression is not restricted to the intraosseous subset of EH. For differential diagnosis of epithelioid vascular tumors, we therefore suggest a helpful panel of antibodies including CAMTA-1, TFE-3, FOS-B, and AE1/AE3. We point out the telltale immunophenotypes: angiolymphoid hyperplasia with eosinophilia and EH (FOS-B+/others negative), PM-HAE (FOS-B+/AE1/AE3+/others negative), epithelioid hemangioendothelioma (CAMTA-1+ or TFE-3+/others negative). Remarkably, MYC is not expressed in these tumors, neither is there an MYC amplification by FISH. We suggest the term multiple eruptive EHs for this subset of cutaneous vascular tumors.",
keywords = "angiosarcoma, CAMTA-1 protein, FOS-B protein, genes, human, MYC, vascular neoplasm, ZPF36-FOS-B fusion protein",
author = "Mar Llamas-Velasco and Werner Kempf and Carlo Cota and Fern{\'a}ndez-Figueras, {Maria Teresa} and Joyce Lee and Gerardo Ferrara and Christian Sander and Shapiro, {Philip E.} and Luis Requena and Heinz Kutzner",
year = "2017",
month = "12",
day = "20",
doi = "10.1097/PAS.0000000000001003",
language = "English",
journal = "American Journal of Surgical Pathology",
issn = "0147-5185",
publisher = "Lippincott Williams and Wilkins",

}

TY - JOUR

T1 - Multiple Eruptive Epithelioid Hemangiomas

T2 - A Subset of Cutaneous Cellular Epithelioid Hemangioma With Expression of FOS-B

AU - Llamas-Velasco, Mar

AU - Kempf, Werner

AU - Cota, Carlo

AU - Fernández-Figueras, Maria Teresa

AU - Lee, Joyce

AU - Ferrara, Gerardo

AU - Sander, Christian

AU - Shapiro, Philip E.

AU - Requena, Luis

AU - Kutzner, Heinz

PY - 2017/12/20

Y1 - 2017/12/20

N2 - There is a wide clinicopathologic spectrum of vascular proliferations characterized by the presence of epithelioid endothelial cells, comprising epithelioid hemangioma (EH)—pseudomyogenic (epithelioid sarcoma-like) hemangioendothelioma (PM-HAE), epithelioid hemangioendothelioma, and epithelioid angiosarcoma. Immunohistochemical FOS-B expression as well as FOS-B rearrangement (fluorescent in situ hybridization [FISH]) have recently been described as diagnostically relevant underpinnings of EH (restricted to osseous lesions) and PM-HAE. The aim of this study was to clinicopathologically characterize and to elucidate FOS-B expression in patients with eruptive lesions of the cellular variant of cutaneous EH. All cases of cutaneous cellular EH (n=16) showed strong diffuse immunohistochemical expression of FOS-B, in conjunction with positivity for ERG and nestin. Expression of MYC, CAMTA-1, AE1/3, and MNF116 was negative in all cases. FISH investigations did not show any sign of rearrangements for CAMTA-1 or MYC amplification. Negative-control cases included 15 lobular hemangiomas, 5 epithelioid angiosarcomas, and 5 nodular Kaposi sarcomas, all of which were negative for FOS-B. Positive-control cases included 15 angiolymphoid hyperplasia with eosinophilia cases, all of them being positive. In contrast with what has been published so far, cutaneous variants of cellular EH exhibit positive immunostaining for FOS-B. Remarkably, FOS-B expression is not restricted to the intraosseous subset of EH. For differential diagnosis of epithelioid vascular tumors, we therefore suggest a helpful panel of antibodies including CAMTA-1, TFE-3, FOS-B, and AE1/AE3. We point out the telltale immunophenotypes: angiolymphoid hyperplasia with eosinophilia and EH (FOS-B+/others negative), PM-HAE (FOS-B+/AE1/AE3+/others negative), epithelioid hemangioendothelioma (CAMTA-1+ or TFE-3+/others negative). Remarkably, MYC is not expressed in these tumors, neither is there an MYC amplification by FISH. We suggest the term multiple eruptive EHs for this subset of cutaneous vascular tumors.

AB - There is a wide clinicopathologic spectrum of vascular proliferations characterized by the presence of epithelioid endothelial cells, comprising epithelioid hemangioma (EH)—pseudomyogenic (epithelioid sarcoma-like) hemangioendothelioma (PM-HAE), epithelioid hemangioendothelioma, and epithelioid angiosarcoma. Immunohistochemical FOS-B expression as well as FOS-B rearrangement (fluorescent in situ hybridization [FISH]) have recently been described as diagnostically relevant underpinnings of EH (restricted to osseous lesions) and PM-HAE. The aim of this study was to clinicopathologically characterize and to elucidate FOS-B expression in patients with eruptive lesions of the cellular variant of cutaneous EH. All cases of cutaneous cellular EH (n=16) showed strong diffuse immunohistochemical expression of FOS-B, in conjunction with positivity for ERG and nestin. Expression of MYC, CAMTA-1, AE1/3, and MNF116 was negative in all cases. FISH investigations did not show any sign of rearrangements for CAMTA-1 or MYC amplification. Negative-control cases included 15 lobular hemangiomas, 5 epithelioid angiosarcomas, and 5 nodular Kaposi sarcomas, all of which were negative for FOS-B. Positive-control cases included 15 angiolymphoid hyperplasia with eosinophilia cases, all of them being positive. In contrast with what has been published so far, cutaneous variants of cellular EH exhibit positive immunostaining for FOS-B. Remarkably, FOS-B expression is not restricted to the intraosseous subset of EH. For differential diagnosis of epithelioid vascular tumors, we therefore suggest a helpful panel of antibodies including CAMTA-1, TFE-3, FOS-B, and AE1/AE3. We point out the telltale immunophenotypes: angiolymphoid hyperplasia with eosinophilia and EH (FOS-B+/others negative), PM-HAE (FOS-B+/AE1/AE3+/others negative), epithelioid hemangioendothelioma (CAMTA-1+ or TFE-3+/others negative). Remarkably, MYC is not expressed in these tumors, neither is there an MYC amplification by FISH. We suggest the term multiple eruptive EHs for this subset of cutaneous vascular tumors.

KW - angiosarcoma

KW - CAMTA-1 protein

KW - FOS-B protein

KW - genes

KW - human

KW - MYC

KW - vascular neoplasm

KW - ZPF36-FOS-B fusion protein

UR - http://www.scopus.com/inward/record.url?scp=85040358351&partnerID=8YFLogxK

UR - http://www.scopus.com/inward/citedby.url?scp=85040358351&partnerID=8YFLogxK

U2 - 10.1097/PAS.0000000000001003

DO - 10.1097/PAS.0000000000001003

M3 - Article

JO - American Journal of Surgical Pathology

JF - American Journal of Surgical Pathology

SN - 0147-5185

ER -