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Pathology & Laboratory Medicine

URMC / Pathology & Laboratory Medicine / Education / Case of the Month / Case 76: Lytic Bone Lesions / Diagnosis

 

Diagnosis

Diagnosis

Metastatic Epithelioid Angiosarcoma (EAS)

Discussion

Epithelioid angiosarcoma (EAS) typically arises in the deep soft tissue but may occur in the thyroid, skin, adrenal, or bone.  Soft tissue examples tend to occur in adult men and may be associated with Schwannoma, foreign bodies or trauma, and certain malignancies after radiation or chemothaeapy, synthetic vascular grafts, or with orthopedic hardware or prostheses. Cutaneous primary EAS is rare and has a predilection for the scalp or forehead of adult men.  Some cutaneous examples have also been associated with prior radiation therapy and lymphedema. Similar to our case, there have been reports of primary cutaneous EAS with multiple metastases at diagnosis.

EAS is an aggressive high-grade tumor, which presents a diagnostic challenge due to its diverse histological manifestations and potential mimicry of other malignancies, specifically metastatic carcinoma. Unlike classic angiosarcomas, which often display obvious vasoformative foci, EAS may present with epithelioid endothelial cells and only subtle vasoformative foci, if any. Additionally, the expression of keratins in EAS further complicates the diagnosis. In combination (keratin positivity, epithelioid morphology, and lack of clear vasoformation), all of these factors may lead to an erroneous diagnosis of metastatic carcinoma, which is far more common.

Thus, comprehensive histopathological examination coupled with immunohistochemical analysis becomes imperative for accurate diagnosis and appropriate management. Vascular marker expression patterns are useful for the diagnosis of EAS. Among vascular markers, CD31 has been reported to be the most sensitive. However, other vascular markers are useful such as CD34, ERG and factor VIII.

Adverse prognostic factors associated with EAS include advanced age, large tumor size, retroperitoneal primary site, and high proliferative index. Unfortunately, the overall prognosis of EAS is poor; more than 50% of patients with EAS die from the disease within 2 to 3 years of diagnosis.

In summary, EAS presenting as skeletal metastases is extremely rare but should be included in the differential diagnosis of epithelioid neoplasms of the bone (and other sites), especially in small specimens in older patients.

References

1. Sakamoto, A., Takahashi, Y., Oda, Y. et al. Aggressive clinical course of epithelioid angiosarcoma in the femur: a case report. World J Surg Onc 12, 281 (2014). 

2. Baliaka A, Balis G, Michalopoulou-Manoloutsiou E, Papanikolaou A, Nikolaidou A. Primary angiosarcoma of bone. A case report. Hippokratia. 2013;17:180-2.

3. Meis-Kindblom JM, Kindblom LG. Angiosarcoma of soft tissue: a study of 80 cases. Am J Surg Pathol. 1998;22:683–679.

4. Miettinen M, Fetsch JF. Distribution of keratins in normal endothelial cells and a spectrum of vascular tumors: implications in tumor diagnosis. Hum Pathol. 2000;31:1062-1067.

5. Hart J, Mandavilli S. Epithelioid angiosarcoma: a brief diagnostic review and differential diagnosis. Arch Pathol Lab Med. 2011;135:268-272.

6. Kikuchi A, Satoh T, Yokozeki H. Primary cutaneous epithelioid angiosarcoma. Acta Derm Venerol. 2008;88:422-423.

7. Kim M, Yan JH, Suh DH. A case of cutaneous epithelioid angiosarcoma with multiple metastases. Int J Dermatology. 2022;61:e101.

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