Korean J Obstet Gynecol.  2012 Aug;55(8):586-589. 10.5468/KJOG.2012.55.8.586.

Primary epithelioid hemangioendothelioma arising at vulva: A case report

• Lee HS
• Ahn JH
• Lee MH

• KMID: 2078110
• DOI: http://doi.org/10.5468/KJOG.2012.55.8.586

Abstract

Vulvar epithelioid hemangioendothelioma as primary site is extremely rare tumor that may be confused with other mesenchymal tumors due to their rarity and its histologic features. We report a case of 46-year-old woman who presented to hospital with left vulvar mass. The mass was located in subcutaneous area of vulva, and well marginated contrast enhanced on radiologic examination. Local excision was performed. After surgery, she has remained free of disease without recurrence for 6 months. Here we report a case of primary vulvar epithelioid hemangioendothelioma as very rare site and this is the first case in Korea.

Keyword

Epithelioid hemangioendothelioma; Vulvar neoplasm; Sarcoma

MeSH Terms

Female
Hemangioendothelioma, Epithelioid
Humans
Korea
Middle Aged
Recurrence
Sarcoma
Vulva
Vulvar Neoplasms

Figure

• 

  Fig. 1 Computerized tomography shows well marginated contrast enhanced mass, measuring 2.2×1.5 cm in sized, on subcutaneous portion of left vulvar area.

• 

  Fig. 2 Gross appearance of epithelioid hemangioendothelioma. Note a well-circumscribed nodule (A) with tan yellow soft, focally myxoid, lipoma-like gross cut surface (B).

• 

  Fig. 3 Light microscopic findings show epithelioid tumor cells with variable atypia organized in nests and hyalinized stroma (A, H&E, ×400) and tumor cells having intracytoplasmic vacuolation (arrows) (B, H&E, ×400).

Reference

1. De Young BR, Wick MR, Fitzgibbon JF, Sirgi KE, Swanson PE. CD31: an immunospecific marker for endothelial differentiation in human neoplasm. Appl Immunohistochem. 1993. 1:97–100.

2. Elder DE, Elentitsas R, Johnson BL, Murphy GF, Xu X. Lever's histopathology of the skin. 2005. 9th ed. Philadelphia (PA): Lippincott Williams & Wilkins.

3. Yousem SA, Hochholzer L. Unusual thoracic manifestations of epithelioid hemangioendothelioma. Arch Pathol Lab Med. 1987. 111:459–463.

4. da Silva BB, Lopes-Costa PV, Furtado-Veloso AM, Borges RS. Vulvar epithelioid hemangioendothelioma. Gynecol Oncol. 2007. 105:539–541.

5. Enzinger FM, Weiss SW. Soft tissue tumors. 1995. 3rd ed. St. Louis (MO): Mosby.

6. Angervall L, Kindblom LG, Karlsson K, Stener B. Atypical hemangioendothelioma of venous origin. A clinicopathologic, angiographic, immunohistochemical, and ultrastructural study of two endothelial tumors within the concept of histiocytoid hemangioma. Am J Surg Pathol. 1985. 9:504–516.

7. Naqvi J, Ordonez NG, Luna MA, Williams MD, Weber RS, El-Naggar AK. Epithelioid hemangioendothelioma of the head and neck: role of podoplanin in the differential diagnosis. Head Neck Pathol. 2008. 2:25–30.

8. Kabukçuoğlu F, Kabukçuoğlu Y, Livaoğlu A, Ozağari A, Armagan R, Kuzgun U. Epithelioid hemangioendothelioma of bone. Acta Orthop Traumatol Turc. 2006. 40:324–328.

9. Granter SR, Nucci MR, Fletcher CD. Aggressive angiomyxoma: reappraisal of its relationship to angiomyofibroblastoma in a series of 16 cases. Histopathology. 1997. 30:3–10.

10. Nucci MR, Granter SR, Fletcher CD. Cellular angiofibroma: a benign neoplasm distinct from angiomyofibroblastoma and spindle cell lipoma. Am J Surg Pathol. 1997. 21:636–644.

11. Gherman CD, Fodor D. Epithelioid hemangioendothelioma of the forearm with radius involvement: case report. Diagn Pathol. 2011. 6:120.

12. Al-Faky YH, Al Malki S, Raddaoui E. Hemangioendothelioma of the eyelid can mimic chalazion. Oman J Ophthalmol. 2011. 4:142–143.

13. Läuffer JM, Zimmermann A, Krähenbühl L, Triller J, Baer HU. Epithelioid hemangioendothelioma of the liver: a rare hepatic tumor. Cancer. 1996. 78:2318–2327.

14. Makhlouf HR, Ishak KG, Goodman ZD. Epithelioid hemangioendothelioma of the liver: a clinicopathologic study of 137 cases. Cancer. 1999. 85:562–582.