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Table of Contents    
Year : 2012  |  Volume : 60  |  Issue : 5  |  Page : 536-539

Primary solitary malignant hemangioendothelioma of vertex: A rare calvarial tumor

1 Department of Neurosurgery, King Edward Memorial Hospital and Seth Gordhandas Sunderdas Medical College, Mumbai, India
2 Department of Neuropathology, Tata Memorial Hospital, Parel, Mumbai, India
3 Department of Neuropathology, King Edward Memorial Hospital and Seth Gordhandas Sunderdas Medical College, Mumbai, India

Date of Web Publication3-Nov-2012

Correspondence Address:
Amit Mahore
Department of Neurosurgery, King Edward Memorial Hospital and Seth Gordhandas Sunderdas Medical College, Mumbai
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/0028-3886.103215

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How to cite this article:
Mahore A, Epari S, Katariya NG. Primary solitary malignant hemangioendothelioma of vertex: A rare calvarial tumor. Neurol India 2012;60:536-9

How to cite this URL:
Mahore A, Epari S, Katariya NG. Primary solitary malignant hemangioendothelioma of vertex: A rare calvarial tumor. Neurol India [serial online] 2012 [cited 2022 Sep 27];60:536-9. Available from: https://www.neurologyindia.com/text.asp?2012/60/5/536/103215


A 43-year-old male noticed painful vertex swelling while combing hair 3 months prior to presentation to our clinic. The frequency and severity of pain increased to an extent to disturb his sleep. Neurological examination was normal. Neuroimaging revealed an intensively enhancing calvarial lesion in the midline and parasagittal location, with destruction of skull bone but without parenchymal abnormality [Figure 1]a-c. Plain X-ray and computed tomography (CT) of the chest and CT and positron emission tomography (PET) scan of the abdomen did not reveal any abnormality. Initially, the lesion was biopsied and then totally removed, with the negative tissue margins. The bone, galea and parasagittal dura were excised, whereas the endosteal layer of the dura over the sagittal sinus was peeled off. Duraplasty was done using the fascia lata. Histology of the lesion revealed a malignant pleomorphic tumor with foci of reactive metaplastic bone [Figure 2]a composed of sheets of spindle-shaped cells with scattered, large, pleomorphic cells and mitotic activity [Figure 2]b-d. Few tumor cells showed intracytoplasmic red blood cell containing lumen [Figure 2]c. The tumor cells were positive for CD31 [Figure 2]d and CD34 [Figure 2]e,f. These features were suggestive of angiosarcoma and malignant hemangioendothelioma. The patient received adjuvant radiation; however, he refused to take chemotherapy. The patient remained well at 18 months follow-up, without any recurrence [Figure 1]d.
Figure 1: (a) Coronal image of computed tomography scan of the head suggestive of abnormal intradiploic lytic lesion causing expansion and destruction of both tables of the skull bone. (b) Post-contrast T1-weighted coronal and (c) sagittal image of magnetic resonance (MR) scan of the brain showing irregular osteolyticmass of the skull with heterogeneous enhancement. (d) Post-contrast T1-weighted coronal image of MR scan of the brain showing no recurrence on follow-up

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Figure 2: Photomicrographs showing a malignant pleomorphic tumor with foci of reactive metaplastic bone (a) (H and E, × 100) composed of sheets of spindle-shaped cells with scattered large pleomorphic cells and mitotic activity (b-d) (H and E, × 400) arrowhead for pleomorphic cells. Few tumor cells show intracytoplasmic red blood cells containing lumen (as shown by arrows in c). The tumor cells are positive for CD31 (d) (IHC × 200)and CD34 (e,f) (IHC × 400)

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Primary neoplasms of the skull are rare, representing 2.6% of primary neoplasms of bone, and primary malignant neoplasms are even less common, accounting for only 0.8% of primary malignant neoplasms of bone. [1],[2],[3] Hemangioendothelioma is a rare neoplasm of bone and skull in an uncommon site. Among primary malignant bone tumors, only 0.5% are hemangioendotheliomas. There is a male predominance, and the median age at presentation is 32 years. About 50% of these lesions affect the long tubular bones, usually of the lower extremities. [1],[2],[3],[4] The common presenting symptoms are pain and a local mass or swelling. [1],[3],[4],[5],[6] The lesions are characterized by a rich network of anastomosing vessels lined by atypical endothelial cells. Immunohistochemistry is helpful in confirming the diagnosis by identifying factor VIII-related antigen, which is a marker for vascular endothelial cells. Three histological grades (I-III) can be identified on the basis of the degree of tumor cell differentiation. Tumor cells are well differentiated in grade I lesions, and differentiation decreases with increasing malignancy. Grade I lesions may remain stable over many years even without treatment. Treatment usually involves curettage or excision. Grade II hemangioendotheliomas often have a worse prognosis, and are usually treated by resection and radiation. Multicentricity, which is common, usually indicates grade I or II lesions. However, there can be, frequently, areas of both grades II and III within the same lesion. This accounts for the variable malignant potential and unpredictable clinical behavior of grade II lesions. Low-grade lesions may progress to high-grade tumors. Grade III lesions demonstrate undifferentiated pleomorphic cells with atypical mitotic figures. Epithelioid sarcomas are the main differential consideration pathologically. Although the malignant cells are epithelioid, these do not line vascular spaces and are not positive with factor VIII-related antigen immunostaining. Grade III lesions are usually solitary, with poor prognosis. [1],[3],[4],[5],[6] Our patient had grade III lesion and was treated with excision and adjuvant radiation. Hemangioendotheliomas of the skull have a worse prognosis than the lesions elsewhere because of secondary involvement of the brain. [1],[2],[3] Metastases to bones and lungs are common with grade III lesions. Therefore, follow-up should include magnetic resonance imaging of the region of surgery for evaluation of recurrence as well as bone scans and chest radiography for evaluation of metastatic disease. The two commonly used terms are hemangioendotheliomaand angiosarcoma. Hemangioendotheliomais the preferred terminology as angiosarcomaimplies a high-grade malignancy, and not all these lesions are of high grade. [1],[2],[3],[4],[5],[6] Plain radiographs reveal lytic lesions, usually multiple, with no surrounding sclerosis. In the skull, the frontal location is most common. CT scans may demonstrate expansion of bone because of involvement of the diploic space and enhancement. Nuclear bone scan shows areas of increased activity. Magnetic resonance images reveal multiple lesions of the diploic space, with extension through the inner and outer tables. The lesions are isointense with gray matter on T1-weighted and proton density-weighted images, and of increased signal on T2- weighted images. On T2-weighted images, the lesions may have a lobulated and septated appearance due to the coalescence of multiple lesions. These lesions show intense enhancement after administration of contrast. [6]

  References Top

1.Young RJ, Brown NJ, Reed MW, Hughes D, Woll PJ. Angiosarcoma.Lancet Oncol 2010;11:983-91.  Back to cited text no. 1
2.Geschickter CF. Primary tumors of the cranial bones. Am J Cancer 1936;26:155-80.  Back to cited text no. 2
3.Volpe R, Mazabraund A. Hemangioendothelioma (angiosarcoma) of bone: A distinct pathologic entity with an unpredictable course? Cancer 1982;49:727-36.  Back to cited text no. 3
4.Terada T. Fatal poorly differentiated angiosarcoma of the scalp. Int J Clin Exp Pathol 2010;3:541-4.  Back to cited text no. 4
5.Chou YC, Chang YL, Harnod T, Chen WF, Su CF, Lin SZ, et al. Primary angiosarcoma of the cranial vault: A case report and review of the literature. Surg Neurol 2004;61:575-9.  Back to cited text no. 5
6.Bourekas EC, Cohen ML, Kamen CS, Tarr RW, Lanzieri CF, Lewin JS. Malignant hemangioendothelioma (angiosarcoma) of the skull: Plain film, CT, and MR appearance. AJNR Am J Neuroradiol 1996;17:1946-8.  Back to cited text no. 6


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