Skin nonmelanocytic tumor

Vascular tumors

Cutaneous epithelioid angiomatous nodule



Last author update: 8 May 2023
Last staff update: 26 June 2023

Copyright: 2022-2024, PathologyOutlines.com, Inc.

PubMed Search: Cutaneous epithelioid angiomatous nodule

Vasudevan D. Mahalingam, D.O., M.S.
Grace Y. Wang, M.D.
Page views in 2023: 4,763
Page views in 2024 to date: 837
Cite this page: Mahalingam VD, Wang GY. Cutaneous epithelioid angiomatous nodule. PathologyOutlines.com website. https://www.pathologyoutlines.com/topic/skinnonmelanocytictumorCEAN.html. Accessed March 29th, 2024.
Definition / general
  • Benign vascular tumor comprised of solid sheets of epithelioid endothelial cells in the superficial dermis
  • May be mitotically active
  • Strongly positive for vascular markers
  • Benign clinical behavior with no reported adverse outcomes
Essential features
Terminology
  • CEAN
ICD coding
  • ICD-10: L98.8 - other specified disorders of the skin and subcutaneous tissue
  • ICD-11: XH8SM9 - cutaneous epithelioid angiomatoid nodule
Epidemiology
  • Very rare (< 70 cases reported in the English literature) (J Cutan Pathol 2022;49:765)
  • Wide age range (14 - 84 years old)
  • Slight male predominance (M:F = 1.4:1)
Sites
Pathophysiology
Etiology
  • Unknown at this time
Clinical features
  • Most commonly a solitary lesion but may be multiple
  • Red to violaceous vascular nodule; may be painful
Diagnosis
  • Histopathology is the gold standard for definitive diagnosis
Radiology description
  • Dermal based, palpable; imaging studies are usually not necessary
  • In cases involving nasal cavity, a homogenous mass is seen on noncontrast CT
Prognostic factors
Case reports
Treatment
  • Surgical excision
Clinical images

Images hosted on other servers:

Solitary vascular papule

Arose within a port wine stain

Arose within a port wine stain

Gross description
Frozen section description
  • Frozen sections usually not performed
Microscopic (histologic) description
  • Dermal based (Acta Derm Venereol 2017;97:135, Am J Dermatopathol 2008;30:16)
  • Well circumscribed
  • Nodular proliferation of epithelioid cells with vesicular chromatin, prominent nucleoli and abundant eosinophilic to clear cytoplasm
  • Mitotic figures can be present but not numerous (up to 5/10 high power fields) and without atypical forms
  • In exceptional cases, moderate cytologic has been reported (Diagn Pathol 2018;13:50)
  • Vascular channel formation is focal but intracytoplasmic lumina are common
  • Vascular channels are lined by a single layer of constituent cells
  • Mild background chronic lymphoplasmacytic inflammation with scattered eosinophils
  • Hemosiderin deposition may be seen
Microscopic (histologic) images

Contributed by Grace Y. Wang, M.D.
Dermal nodule

Dermal nodule

Mixed inflammation with hemosiderin deposition

Mixed inflammation with hemosiderin deposition

Epithelioid cells

Epithelioid cells

Intracytoplasmic lumina

Intracytoplasmic lumina


Well circumscribed nodule

Well circumscribed nodule

Vascular channels

Vascular channels

CD31

CD31

CD34

CD34

Sample pathology report
  • Skin, chest, biopsy:
    • Cutaneous epithelioid angiomatous nodule (see comment)
    • Comment: The biopsy demonstrates a well circumscribed nodule, comprised of sheets of epithelioid cells in the superficial dermis. The epithelioid cells are relatively monomorphous and have intracytoplasmic vacuoles. Rare vascular channel formation is noted. A mild stromal lymphocytic and eosinophilic inflammatory infiltrate is present at the periphery. Immunohistochemistry shows that the epithelioid cells are positive for CD34 and ERG. The overall histopathologic and immunohistochemical findings are consistent with cutaneous epithelioid angiomatous nodule, a benign vascular neoplasm with a very low risk of local recurrence and no metastatic potential.
Differential diagnosis
Board review style question #1

A 35 year old man presents with a painful cutaneous upper arm lesion (shown above). What is the diagnosis?

  1. Bacillary angiomatosis
  2. Cutaneous epithelioid angiomatous nodule
  3. Epithelioid angiosarcoma
  4. Epithelioid hemangioma
Board review style answer #1
B. Cutaneous epithelioid angiomatous nodule. Pictured is a dermal based proliferation of epithelioid cells with abundant eosinophilic cytoplasm and chronic inflammation. Answer D is incorrect because there are focal areas of single layered vascular channel formations but they are not prominent. Answer A is incorrect because there are no associated neutrophils or bacteria identified. Answer C is incorrect because there are no readily appreciated mitotic figures and marked cytologic atypia.

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Reference: Cutaneous epithelioid angiomatous nodule
Board review style question #2
What is the clinical behavior of cutaneous epithelioid angiomatous nodule?

  1. Indolent
  2. Locally aggressive
  3. Potentially metastatic
  4. Recurs frequently
Board review style answer #2
A. Indolent. Cutaneous epithelioid angiomatous nodule is a benign vascular proliferation that has not been shown to metastasize and does not generally recur following excision.

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Reference: Cutaneous epithelioid angiomatous nodule
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