Skin nonmelanocytic tumor
Vascular tumors
Cutaneous epithelioid angiomatous nodule
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PubMed Search: Cutaneous epithelioid angiomatous nodule
Table of Contents
Definition / general | Essential features | Terminology | ICD coding | Epidemiology | Sites | Pathophysiology | Etiology | Clinical features | Diagnosis | Radiology description | Prognostic factors | Case reports | Treatment | Clinical images | Gross description | Frozen section description | Microscopic (histologic) description | Microscopic (histologic) images | Positive stains | Negative stains | Sample pathology report | Differential diagnosis | Additional references | Board review style question #1 | Board review style answer #1 | Board review style question #2 | Board review style answer #2Cite this page: Mahalingam VD, Wang GY. Cutaneous epithelioid angiomatous nodule. PathologyOutlines.com website. https://www.pathologyoutlines.com/topic/skinnonmelanocytictumorCEAN.html. Accessed September 21st, 2023.
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
- Located in superficial dermis, well circumscribed, sheets of epithelioid cells with intracytoplasmic vacuoles
- Significant histomorphologic overlap with epithelioid hemangioma (EH) / angiolymphoid hyperplasia with eosinophilia (ALHE)
- Essential to distinguish from other more aggressive epithelioid neoplasms
- Treatment is simple excision
Terminology
- CEAN
ICD coding
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
- Most commonly trunk and extremities (J Cutan Pathol 2022;49:765)
- Can also occur on head and neck
- Nasal cavity
Pathophysiology
- Largely unknown at this time
- Some experts suggest it may be reactive (Am J Dermatopathol 2004;26:14)
- Some experts consider it to be a variant of EH / ALHE (Am J Dermatopathol 2008;30:16)
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
- Generally does not recur (Am J Dermatopathol 2004;26:14, J Cutan Pathol 2022;49:765)
- No reports of distant metastasis
Case reports
- 19 year old man with left foot mass (World J Clin Cases 2020;8:600)
- 28 year old woman with right breast nodule (Indian Dermatol Online J 2019;10:463)
- 30 year old man with nodule in pre-existing port wine stain on the chest (Acta Derm Venereol 2017;97:135)
- 84 year old man with multiple papules on scalp and neck (Am J Dermatopathol 2011;33:831)
Treatment
- Surgical excision
Clinical images
Images hosted on other servers:
Solitary vascular papule
Arose within a port wine stain
Gross description
- Well circumscribed, solid, hemorrhagic nodule in the dermis (Indian Dermatol Online J 2019;10:463)
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
Mixed inflammation with hemosiderin deposition
Epithelioid cells
Intracytoplasmic lumina
Well circumscribed nodule
Vascular channels
CD31
CD34
Positive stains
Negative stains
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
- Epithelioid hemangioma (EH) / angiolymphoid hyperplasia with eosinophilia (ALHE):
- More commonly affects head and neck (Adv Anat Pathol 2019;26:186)
- Considerable histopathologic overlap with CEAN
- More prominent, well formed vasculature
- May involve deep dermis and subcutis
- Hobnail-like endothelial cells
- FOS gene rearrangement may be found in a subset of intraosseous and soft tissue lesions (Am J Surg Pathol 2015;39:1313)
- FOSB fusion with ZNF36 or WWTR1 reported in rare EHs with atypical features (Genes Chromosomes Cancer 2014;53:951)
- Some experts think that CEAN is a variant of EH / ALHE
- Epithelioid angiosarcoma:
- Infiltrative, architecturally complex (Adv Anat Pathol 2019;26:186)
- Significant cytologic atypia
- Mitotic figures, including atypical forms
- May have necrosis
- Destruction of native structures
- Epithelioid hemangioendothelioma:
- Cords and nests of epithelioid, lightly eosinophilic cells (Adv Anat Pathol 2019;26:186)
- Nonvasoformative
- Fibromyxoid stroma
- Cytologic atypia and mitotic figures common
- WWTR1::CAMTA1 fusion (Genes Chromosomes Cancer 2011;50:644)
- YAP1::TFE3 fusion (Genes Chromosomes Cancer 2013;52:775)
- Nonvascular neoplasms:
Additional references
Board review style question #1
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
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Reference: Cutaneous epithelioid angiomatous nodule
Board review style question #2
What is the clinical behavior of cutaneous epithelioid angiomatous nodule?
- Indolent
- Locally aggressive
- Potentially metastatic
- 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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Reference: Cutaneous epithelioid angiomatous nodule
