- Department of Neurosurgery, Universitas Padjadjaran–Dr. Hasan Sadikin General Hospital, Bandung, Indonesia
- Oncology Working Group, Health Research Unit, Universitas Padjadjaran–Dr. Hasan Sadikin General Hospital, Bandung, Indonesia
- Department of Pathology Anatomy, Faculty of Medicine, Universitas Padjadjaran–Dr. Hasan Sadikin General Hospital, Bandung, Indonesia
Oncology Working Group, Health Research Unit, Universitas Padjadjaran–Dr. Hasan Sadikin General Hospital, Bandung, Indonesia
Department of Pathology Anatomy, Faculty of Medicine, Universitas Padjadjaran–Dr. Hasan Sadikin General Hospital, Bandung, Indonesia
DOI:10.4103/2152-7806.119079Copyright: © 2013 Arifin MZ This is an open-access article distributed under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited.
How to cite this article: Arifin MZ, Tjahjono FP, Faried A, Gill AS, Cahyadi A, Hernowo BS. Giant cell angiofibroma of the scalp: A benign rare neoplasm with bone destruction. Surg Neurol Int 30-Sep-2013;4:131
How to cite this URL: Arifin MZ, Tjahjono FP, Faried A, Gill AS, Cahyadi A, Hernowo BS. Giant cell angiofibroma of the scalp: A benign rare neoplasm with bone destruction. Surg Neurol Int 30-Sep-2013;4:131. Available from: http://sni.wpengine.com/surgicalint_articles/giant-cell-angiofibroma-of-the-scalp-a-benign-rare-neoplasm-with-bone-destruction/
Background:The incidence of extraorbital giant cell angiofibroma (GCA) is rare, with only one case located in the scalp reported in the literature. The morphological hallmark is histopathological examination showing richly vascularized pattern-less spindle cell proliferation containing pseudovascular spaces and floret-like multinucleate giant cells.
Case Description:We report a case of a 30-year-old female with a primary complaint of a painless solitary nodule arising on the left parietal region of the scalp. Complete tumor removal through surgical intervention was achieved, and the postoperative period was uneventful.
Conclusion:Diagnosing a highly vascularized tumor in the head and neck is challenging. Our case is unique in that it is presented as a GCA of the scalp, which is an extremely rare clinical entity, and also demonstrated bone destruction.
Keywords: Bone destruction, giant cell angiofibroma, scalp
Giant cell angiofibroma (GCA) is a nonrecurring benign neoplasm that was first described in 1995 in a study of seven patients with a distinctive orbital tumor.[
A 30-year-old female presented with a bony swelling over her left parietal region that had increased in size over the past 2 years. She had no history of trauma, bone pain, systemic disease, or neurological symptoms. Physical examination found a swelling that was 6 × 5 cm in size, soft, and without any tenderness [
Clinical picture of the patient that had a bony swelling over the left parietal region (a). A CT scan at regio left parietal reveal bone discontinuity that showed bony destruction (b). On macroscopic examination, reddish mass, size 6 × 5 cm and the temporal muscle, size 2 × 1 cm that had been infiltrated with GCA, were removed (c)
Radiological examination of a skull x-ray showed a lytic lesion that involving the left temporoparietal bone, and computed tomography (CT) scan confirmed the presence of extensive bone destruction [
The tumor mass was approached through a horseshoe scalp incision [Figure
The histopathological examination showed both cellular areas with especially oval and round- to spindle-shaped cells intermixed with floret-like giant cells in no particular pattern and hypocellular areas. Giant cells often lined the pseudovascular spaces [Figure
A giant cell angiofibroma of the scalp presenting as a varying combination of cellular areas composed of bland round to spindle cells, collagenous or myxoid stroma with focal sclerotic areas, medium-sized to small thick-walled vessels, and multinucleated giant cells, often lining angiectoid spaces; arrow giant cell (H and E, stain ×100 and ×200, respectively) (a-b). The lesion was observed eroded through the bone, as shown that the lysis of the bone without reformation (c). The lesion was highly vascularized with varying caliber of blood vessels and immunohistochemistry showed CD34 positive staining (d)
She was discharged from the hospital uneventfully, and no recurrence of the mass was detected at her one-year follow-up.
GCA was first described in 1995 by Dei Tos et al. as a distinctive orbital tumor occurring exclusively in male adults.[
Although GCA is considered a benign neoplasm, it has the potential to recur locally, especially after an incomplete resection. Histologically, the tumor displays a varying combination of a cellular area composed of bland round- to spindle-shaped cells, collagenous or myxoid stroma with focal sclerotic areas, medium-sized to small thick-walled vessels, and multinucleated giant stromal cells often lining the angiectoid spaces.[
Diagnostic imaging can play a role in determining tumor resectability, assessing surgical risks, and evaluating tumor recurrence.[
Several entities should be considered in the differential diagnosis of GCA. On the basis of both histology and immunohistology, the following diagnoses can usually be easily excluded: hemangiopericytoma (a much rarer entity when strict diagnostic criteria are applied, which is usually more cellular and had a predominant staghorn vascular pattern and weaker CD34 positivity); fibrous histiocytoma (storiform pattern, more collagenous tissue and usually CD34 negative); schwannoma (Antoni A and B areas, Verocay bodies, strong S100 positivity); orbital meningioma (syncytial sheets or whorls of cells, EMA positive); and multinucleate cell angiohistiocytoma (usually dermal-based, CD34-negative, and CD68-positive interstitial cells but CD68-negative giant cells).[
The best treatment appears to be surgical resection with complete tumor removal, as local recurrence may occur after incomplete resection.[
GCA is a benign tumor with a predilection for the orbit and head and neck regions in adults. Diagnostic imaging can play a role in determining tumor resectability, assessing surgical risks, and evaluating tumor recurrence. In our case, its imaging appearance was not specific and suggested a highly vascular soft tissue tumor. We performed surgical resection, and the clinical course was uneventful. GCA is uncommon but should be included in the differential diagnosis of a subcutaneous mass of the scalp.
The authors thank Agung B. Sutiono, from Department of Neurosurgey, and Antonius J. Ridwan, from Department of Pathology Anatomy, Faculty of Medicine, Universitas Padjadjaran–Dr. Hasan Sadikin General Hospital, Bandung, West Java, Indonesia for fruitful discussions and Jasprit Kaur for technical assistance.
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