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| LETTER TO EDITOR |
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| Year : 2012 | Volume
: 60
| Issue : 3 | Page : 344-346 |
Epithelioid hemangioendothelioma of sphenoid bone: A case report of an unusual case
Sharique Ahmed1, Sridhar Epari1, Milap Shah2, K Srinivas Rao3
1 Department of Pathology, Tata Memorial Centre, Mumbai, India
2 Department of Pathology, Yashoda Hospital, Secunderabad, India
3 Department of Radiation Oncology, Yashoda Hospital, Secunderabad, India
| Date of Submission | 16-Mar-2012 |
| Date of Decision | 17-Mar-2012 |
| Date of Acceptance | 11-May-2012 |
| Date of Web Publication | 14-Jul-2012 |
Correspondence Address:
Sridhar Epari
Department of Pathology, Tata Memorial Centre, Mumbai
India
 Source of Support: None, Conflict of Interest: None  | Check |
DOI: 10.4103/0028-3886.98537
How to cite this article:
Ahmed S, Epari S, Shah M, Rao K S. Epithelioid hemangioendothelioma of sphenoid bone: A case report of an unusual case. Neurol India 2012;60:344-6 |
Sir,
Epithelioid hemangioendotheliomas (EHEs) are intermediate grade sarcomas of endothelial differentiation. Primary EHE of skull bones is extremely rare. [1]
A 42-year-old woman presented with diplopia and headache of 3 months duration. She was found to have ptosis and decreased visual acuity of right eye. Computed tomography (CT) scan and magnetic resonance imaging (MRI) showed a 2.8 cm-sized sellar lesion with areas of mineralization [Figure 1]. Endocrine evaluation was normal. Surgical debulking of the tumor was done through frontotemporal craniotomy. The tumor was seen involving the sphenoidal sinus, sphenoid bone, and clivus. Histological evaluation revealed a malignant tumor composed of irregular oval to pleomorphic cells of epithelioid morphology admixed with elongated ovoid tumor cells arranged in sheets and focally lining the slit-like vascular spaces with a variably hyalinized myxochondromatous stroma in the background [Figure 2]. The tumor cells showed eosinophilic cytoplasm with cytoplasmic vacuoles containing an occasional erythrocyte in few cells [Figure 2]d. Scattered foci of metaplastic calcification were seen. Mitotic activity was very occasional and no necrosis seen. The tumor cells were immunopositive [Table 1] for CD31, CD34, and Fli-1 [Figure 3]a-c, suggesting endothelial cell differentiation] and showed focal positivity for epithelial membrane antigen [Figure 3]d and cytokeratin [Figure 3]e. S100 protein and brachyury were negative. | Figure 1: Computerized tomography scan images (a and b) show sellar– suprasellar mass with calcification. Magnetic resonance images (c) do confirm the same.
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 | Figure 2: Photomicrographs showing a tumor with irregular short slitlike spaces lined by tumor cells with foci of destroyed bone [(a) H and E stain, ×200; (c) H and E stain, ×400] and interspersed with chondroidlike myxoid stroma [(b) H and E stain, ×200; (d) H and E stain, ×400]. Spindle- to polygonal-shaped tumor cells seen embedded within the myxoid stroma with the presence of occasional intracytoplasmic vacuole containing an erythrocyte [(d) H and E stain, ×400]
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 | Figure 3: Photomicrographs of the tumor showing immunohistochemical profile of diffuse strong positivity for CD31 [(a) immunohistochemistry (IHC), ×400 inset highlights the positivity of vacuolated cells for CD31], CD34 [(b) IHC, ×400 inset highlights the positivity of vacuolated cells for CD34], and Fli-1 [(c) IHC, ×200]. Focal areas of tumor show positivity for epithelial membrane antigen [(d) IHC, ×200] and cytokeratin [(e)IHC, ×200]
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 | Table 1: Details of the panel of the antibodies used for immunohistochemical evaluation
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Hemangioendotheliomas can occur at any age in soft tissue, viscera, and bone. [1],[2],[3] Histologically, they are further classified as epithelioid, kaposiform (spindle cell), retiform, and composite variants. EHEs, first described by Weiss and Enzinger, are the most aggressive variants with a higher incidence of recurrence and metastases. [4] EHEs, histologically sometimes show distinctive light blue chondroid-like to deep pink hyaline-like matrix and rarely even metaplastic bone. This may cause erroneous histological diagnosis of chondro- or osteosarcoma, especially in the primary bone lesions. They exhibit immunoreactivity for CD31, CD34, Fli-1 (differentiating from chondrosarcoma and osteosarcomas) and in about 25%-30% of cases of EHEs show positivity for epithelial markers. [4] Immunopositivity for epithelial marker is a known pitfall for getting misdiagnosed as carcinomas. Thus, a restricted panel could be potentially misleading. Although carcinomas in general tend to show diffuse and intense staining in contrast to the patchy staining in carcinoma-mimickers, as was seen in the present case. The tumor was immunonegative for S-100 protein and brachyury (coupled with positivity for vascular endothelial markers) excluding the (radiological) differentials of chordoma and chondrosarcoma. Absence of necrosis and frequent mitoses excludes the possibility of epithelioid angiosarcoma. In the current case, histology (intracytoplasmic lumen with erythrocytes, focal typical vascular differentiation) and the immunoreactivity for CD31, CD34, and Fli-1 confirms the diagnosis of EHE and a final diagnosis of primary sphenoid bone EHE was made.
EHE can affect any portion of the skeleton but cranium is very rare site. Joo et al. [5] reported a review of 22 primary skull bone-based hemangioendotheliomas and sphenoid bone was also one of them. In that series, it has been shown to occur commonly in second and third decades with slight male preponderance. Other than this, the authors could find only three published reports of suprasellar EHE, suggesting the rarity of occurrence. [6],[7],[8] Moreover, this is the only case with detailed histomorphological illustration and radiological correlation. This case illustrates the primary skull bone tumors as being one of the uncommon differentials for suprasellar masses.
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| 1. | Dorfman HD, Czerniak B. Vascular lesions. In: Bone tumors. Mosby: St. Louis; 1998. p. 729-814. 
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| 2. | Weiss SW, Goldblum JR, editors. Hemangioendothelioma: Vascular tumors of intermediate malignancy. In: Enzinger and Weiss's soft tissue tumors. 4th ed. St. Louis: Mosby Yearbook; 2001. p. 891-915.
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| 3. | Mentzel T, Beham A, Calonje E, Katenkamp D, Fletcher CD. Epithelioid hemangioendothelioma of skin and soft tissues: Clinicopathologic and immunohistochemical study of 30 cases. Am J Surg Pathol 1997;21:363- 74.
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| 4. | Weiss SW, Enzinger FM. Epithelioid hemangioendothelioma: A vascular tumor often mistaken for a carcinoma. Cancer 1982;50:970-81.
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| 5. | Joo M, Lee GJ, Koh YC, Park YK. Hemangioendothelioma of the sphenoid bone: A case report. J Korean Med Sci 2001;16:241-4.
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| 6. | Peal GS, Takoi Y, Tindal GT, O'Briens MS, Payne NS, Hoffman JC. Benign hemangioendothelioma involving the central nervous system: "Strawberry Nevus" of the neuroaxis. Neurosurgery 1980;7:249-56.
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| 7. | Baehring JM, Dickey PS, Bannykh SI. Epithelioid hemangioendothelioma of the suprasellar area: A case report and review of the literature. Arch Pathol Lab Med 2004;128:1289-93.
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| 8. | Hamlat A, Casallo-Quilliano C, Saikali S, Lesimple T, Brassier G. Epithelioid hemangioendothelioma of the infundibular-hypothalamic region: Case report and literature review. J Neurooncol 2004;67:361-6.
[PUBMED] [FULLTEXT] |
[Figure 1], [Figure 2], [Figure 3]
[Table 1]
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