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Solid form of epithelioid hemangioma: a case report.

Roh J, Song MJ, Lee MW, Park CS - Korean J Pathol (2014)

View Article: PubMed Central - PubMed

Affiliation: Departments of Pathology, Asan Medical Center, University of Ulsan College of Medicine, Seoul, Korea.

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Epithelioid hemangioma (EH) is an uncommon benign vascular tumor, of controversial etiology that usually presents as a slowly growing nodule on the face or digit of a middle-aged woman... EH has been described in the literature as an inflammatory angiomatous nodule or, an atypical or pseudopyogenic granuloma, when infiltration of various inflammatory cells is predominant, and a histiocytoid hemangioma5 when cobble stone-like endothelial cells are conspicuous... The lesions slowly grew in size over the course of 4 months... The patient was otherwise healthy with no significant medical conditions... The differential diagnoses for cutaneous lesions that mostly consist of epithelioid cells include poorly differentiated squamous cell carcinoma, melanoma, epithelioid vascular tumor, atypical fibroxanthoma, cutaneous leiomyosarcoma, epithelioid fibrous histiocytoma, and epithelioid sarcoma... A few reports have indicated its aggressive clinical behavior... Immunohistochemical staining for HHV-8 confirm the diagnosis... In this patient, diagnosis was potentially confusing owing to the proliferation of solid endothelial cells... However, the mass did not have the typical myxohyaline or sclerotic stroma of EHE... EA is a highly malignant tumor and more than half of patients die within the first year... In our case, histologic features such as a lobular architecture, peripheral maturation, absence of necrosis or atypical mitotic figures, and the lack of significant nuclear atypia decreased the index of suspicion for high-grade malignancy... One study reported that 80% of solid form of EH cases were interpreted as malignant vascular tumors by at least one contributing pathologist... To our knowledge, our present study is the first report of a solid form of EH in the Korean pathologic literature... It is important that the solid form of EH be distinguished from malignant vascular tumors to avoid overly aggressive intervention.

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Histologic features of the second lesion. (A) A recurrent lesion shows typical histologic characteristics of epithelioid hemangioma. (B) Vascular structures that comprise the lesion are lined by prominent epithelioid endothelial cells. The stroma contains mixed inflammatory cells, including many eosinophils. (C) A focal solid component is present in the center of the lesion.
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f3-kjpathol-48-5-394: Histologic features of the second lesion. (A) A recurrent lesion shows typical histologic characteristics of epithelioid hemangioma. (B) Vascular structures that comprise the lesion are lined by prominent epithelioid endothelial cells. The stroma contains mixed inflammatory cells, including many eosinophils. (C) A focal solid component is present in the center of the lesion.

Mentions: Approximately 1 year later, a 0.7-cm-sized nodule with the same clinical features as the nodules seen on initial physical exam developed. Histologically, this was a superficial ill-defined lesion with remarkably different morphologies to previous lesions (Fig. 3A). Most of the lesion demonstrated typical histologic features of EH (Fig. 3B). Vascular structures had prominent endothelial cells protruding deeply into the lumen, creating “tombstones” appearance. Mixed inflammatory cells, including many eosinophils, were dispersed between the tumor cells. A central, focal, and solid component similar to the previous biopsy was present (Fig. 3C). The epithelioid endothelial cells were also immunopositive for CD31. The cells expressed Factor VIII and CD34 to a much lesser extent than CD31. D2-40 immunostaining was negative (data not shown). The patient has survived more than 30 months since the first biopsy. There has not been any recurrence of the lesions after application of a topical corticosteroid.


Solid form of epithelioid hemangioma: a case report.

Roh J, Song MJ, Lee MW, Park CS - Korean J Pathol (2014)

Histologic features of the second lesion. (A) A recurrent lesion shows typical histologic characteristics of epithelioid hemangioma. (B) Vascular structures that comprise the lesion are lined by prominent epithelioid endothelial cells. The stroma contains mixed inflammatory cells, including many eosinophils. (C) A focal solid component is present in the center of the lesion.
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Related In: Results  -  Collection

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f3-kjpathol-48-5-394: Histologic features of the second lesion. (A) A recurrent lesion shows typical histologic characteristics of epithelioid hemangioma. (B) Vascular structures that comprise the lesion are lined by prominent epithelioid endothelial cells. The stroma contains mixed inflammatory cells, including many eosinophils. (C) A focal solid component is present in the center of the lesion.
Mentions: Approximately 1 year later, a 0.7-cm-sized nodule with the same clinical features as the nodules seen on initial physical exam developed. Histologically, this was a superficial ill-defined lesion with remarkably different morphologies to previous lesions (Fig. 3A). Most of the lesion demonstrated typical histologic features of EH (Fig. 3B). Vascular structures had prominent endothelial cells protruding deeply into the lumen, creating “tombstones” appearance. Mixed inflammatory cells, including many eosinophils, were dispersed between the tumor cells. A central, focal, and solid component similar to the previous biopsy was present (Fig. 3C). The epithelioid endothelial cells were also immunopositive for CD31. The cells expressed Factor VIII and CD34 to a much lesser extent than CD31. D2-40 immunostaining was negative (data not shown). The patient has survived more than 30 months since the first biopsy. There has not been any recurrence of the lesions after application of a topical corticosteroid.

View Article: PubMed Central - PubMed

Affiliation: Departments of Pathology, Asan Medical Center, University of Ulsan College of Medicine, Seoul, Korea.

AUTOMATICALLY GENERATED EXCERPT
Please rate it.

Epithelioid hemangioma (EH) is an uncommon benign vascular tumor, of controversial etiology that usually presents as a slowly growing nodule on the face or digit of a middle-aged woman... EH has been described in the literature as an inflammatory angiomatous nodule or, an atypical or pseudopyogenic granuloma, when infiltration of various inflammatory cells is predominant, and a histiocytoid hemangioma5 when cobble stone-like endothelial cells are conspicuous... The lesions slowly grew in size over the course of 4 months... The patient was otherwise healthy with no significant medical conditions... The differential diagnoses for cutaneous lesions that mostly consist of epithelioid cells include poorly differentiated squamous cell carcinoma, melanoma, epithelioid vascular tumor, atypical fibroxanthoma, cutaneous leiomyosarcoma, epithelioid fibrous histiocytoma, and epithelioid sarcoma... A few reports have indicated its aggressive clinical behavior... Immunohistochemical staining for HHV-8 confirm the diagnosis... In this patient, diagnosis was potentially confusing owing to the proliferation of solid endothelial cells... However, the mass did not have the typical myxohyaline or sclerotic stroma of EHE... EA is a highly malignant tumor and more than half of patients die within the first year... In our case, histologic features such as a lobular architecture, peripheral maturation, absence of necrosis or atypical mitotic figures, and the lack of significant nuclear atypia decreased the index of suspicion for high-grade malignancy... One study reported that 80% of solid form of EH cases were interpreted as malignant vascular tumors by at least one contributing pathologist... To our knowledge, our present study is the first report of a solid form of EH in the Korean pathologic literature... It is important that the solid form of EH be distinguished from malignant vascular tumors to avoid overly aggressive intervention.

No MeSH data available.


Related in: MedlinePlus