Muhammad Zafrullah Arifin, et al.

Title Page: Original Article Manuscript title: Giant Cell Angiofibroma of Scalp: Benign Rare-Neoplasm with Bone Destruction Running title: The Scalp Giant Cell Angiofibroma Authors: Muhammad Zafrullah Arifin, MD., PhD1,2; Firman Priguna, MD1,2; Arwinder Singh Gill, MD1; Alexander Cahyadi, MD1; Bethy Suryawati Hernowo, MD., PhD2,3; Ahmad Faried, MD., PhD1,2,* Authors Affiliation:
1

Department of Neurosurgery; 2Oncology Working Group, Health Research Unit and Department of Pathology Anatomy, Faculty of Medicine Universitas PadjadjaranDr

Hasan Sadikin General Hospital, Bandung, Indonesia *All correspondence to: Ahmad Faried, MD., PhD Department of Neurosurgery, Faculty of Medicine Universitas PadjadjaranDr. Hasan Sadikin General Hospital, Jl. Pasteur No. 38, Bandung 40161, Indonesia E-mail: faried.fkup@gmail.com

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Abstract Inroduction: The incidence of extraorbital Giant Cell Angiofibroma (GCA) is quite rare and there is only one case in the world literature that located in scalp. The morphological hallmark is histopathological examination showing richly vascularized patternless spindle cell proliferation containing pseudovascular spaces and oret like multinucleate giant cells. A Case Report: We report one case of a 30-years-old female with main complaint of 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 tumors in the head and neck is challenging. Our case is unique that it is presented as a Giant cell angiobroma (GCA) of the scalp, which is extremely rare clinical entity and also demonstrating bone destruction. Keywords: scalp, giant cell angiofibroma, bone destruction

Beberapa hal yang saya ganti : 1. Banyak tulisan di abstract dan diskusi yang saya edit 2. Tulisan harus SCALP atau scalp ? 3. Histologic atau histopathologic atau pathologic ? 4. Tulisan legend untuk gambarnya apa saja ? Gambar IHC dan tulang yang diresorpsi bisa diperoleh? Nanti kita tambahkan dalam diskusinya.

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Introduction Giant cell angiofibroma (GCA) is a non-recurring benign neoplasm that first described in 1995 in a study of seven patients with a distinctive orbital tumor1. Since then, approximately 39 cases have been reported in the literature. Most of these lesions involving orbital region and predominates in males; extraorbital sites of disease predominates in female, including nasolacrimal duct, buccal mucosa, submandibular region, trunk, mediastinum, retroperitoneum, vulva, and extremities.1,2 Giant cell angiofibroma may belong to a solitary fibrous tumor group as it contains multinucleated giant stromal cells and angiectoid space. They may also have an indolent course, sometimes painful mass, slowly growing or remain stable in size over many years.2,5 Case Report A 30-year-old female presented with a bony swelling over left parietal region that enlarged in size for the past 2 years. There was no history of trauma, bone pain, systemic disease and neurological symptoms. Physical examination found the swelling 6x5cm in size, firm, with no tenderness when touched. The skin over the swelling area was not attached to the underlying soft tissue, with no dilatation in veins, scars, or sinuses. No bruit was heard over the swelling. Neurological examination was unremarkable and fundoscopy revealed no papilledema. CT examination showed a well-dened expanded lytic lesion with an electron density greater than the brain with no contrast enhancement, involving parietal bone with an associated soft tissue mass that extend extra cranially. The bone was markedly thinned and destroyed (Figure 1). We were unable to obtain an MRI scan. On clinical grounds, the

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decision was made to operate immediately. The mass was approached through a horse shoe scalp incision. The craniotomy was followed by a macroscopic total removal of a nodular tumor that destroyed the parietal bone and adherent to the dura mater but not inltrating. The patient was neurologically intact postoperatively. Gross pathologic examination revealing a firm, 25256mm, reddish specimen (Figure 2). Histopathologic analysis demonstrated a proliferation of spindle cells in a fibrous stroma with multiple pseudovascular spaces filled with various multinucleated giant cells. (Figure 3 and 4). Mitotic activity was inconspicuous. A diagnosis of GCA of the scalp was rendered on the basis of these findings. Ada gambar sediaan destruksi tulang??? Tambahan IHC ? The patient was discharged from the hospital uneventfully and no recurrence of the mass was detected at her one-year follow-up. Discussions Giant cell angiofibroma (GCA) was first described in 1995 by Dei Tos et al. as a distinctive orbital tumor occurring exclusively in male adults1. GCA is a benign neoplasm, mesenchymal lesion showing histological features intermediate in between, but distinct from, solitary fibrous tumor (SFT) and giant cell fibroblastoma (GCF) of soft tissue.1,6 However, it is now recognized that this lesion can also present in other

locations, and 18 cases were reported in extraorbital sites as a submandibular, parascapular, retroauricular, thigh, mediastinum, back, scalp, retroperitoneum, vulva, hip, forearm, groin, parotid, and neck.7-10 The median age of affected patients was 45 years old, with a range of 18 to 81 years. Interestingly, the extraorbital lesions predominates in female, corresponding to 66% of the cases.10 Patients typically present with a solitary

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painless soft tissue mass. Other symptoms were depend on the site of the tumor secondary to local mass effect, including pain, visual disturbances, and respiratory difficulty.1,9 Although GCA is considered as a benign neoplasm, but it has the potential to recur locally, especially after an incomplete resection. Histologically, the tumor displays a varying combination of the cellular area that composed of a bland round to spindle cells, collagenous or myxoid stroma with focal sclerotic areas, medium-sized to small thickwalled vessels, and multinucleated giant stromal cells, often lining angiectoid spaces (1, 4). The number of giant cells may vary from one tumor to another, and pseudovascular spaces may occasionally be absent. GCAs are invariably positive for

immunohistochemical staining with vimentin and CD34.1,2,7,9,11 Cytogenetic testing in one study revealed an associated chromosomal abnormality on 6q.12 Diagnostic imaging can play a role in determining tumor resectability, assessing surgical risks, and evaluating tumor recurrence. Computed tomography (CT) and magnetic resonance imaging (MRI) may help detecting exact lesion localization and extension, but ussually, diagnosis based on radiological evidence cannot be made. Diagnostic imaging can play a role in determining tumor resectability, assessing surgical risks, and evaluating tumor recurrence.10,11 In our case, from bone window CT scan, we discovered a bone discontinuity at a left parietal region, suggesting a lytic lesion which was proven intraoperatively. Although the internal table of the skull was deformed but there was no invasion into duramater. This is unique as these GCA entities are usually considered as a benign tumors. The bone surrounding the tumor appeared rarefied and removed with roungeurs to the border of normal bone. Several entities should be considered in the differential diagnosis of GCA. On the basis of both histology and immunohistology, the following diagnosis can usually be

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easily excluded: hemangiopericytoma (a much rarer entity when strict diagnostic criteria are applied, which usually appeared more cellular with 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).8,13 The best treatment is to surgically resect the tumor completely because local recurrence may occur after an incomplete resection.7 In summary, although GCA is a rare scalp tumor but it should be kept in mind when the histopathologic examination reveals a well-dened broblastic patternless CD34 (+) subcutaneous tumor mass. Conclusion Diagnosis of highly vascularized tumors of head and neck is challenging. Our case is quite unique in discussing a Giant cell angiobroma (GCA) of the scalp, which is an extremely rare clinical entity that may also demonstrate a bone destruction. Acknowledgement Authors thank Antonius Jenes Ridwan, MD., from Department of Pathology Anatomy, for extensive discussions and technical assistants. References 1. Dei Tos AP, Seregard S, Calonje E, Chan JK, Fletcher CD. Giant cell angiofibroma: A distinctive orbital tumor in adults. Am J Surg Pathol. 1995;19:1286-93.

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Keyserling H, Peterson K, Camacho D, Castillo M. Giant cell angiofibroma of the orbit. Am J Neuroradiol. 2004;25:1266-8.

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Pala EE, Beyhan R, Bayol U, Cumurcu S, Kucuk U. Giant cell angiofibroma in unusual localization: A case report. Case Report Pathol. 2012;2012:408575.

4. World Health Organization Classification of Tumours. Pathology and Genetics of

Tumours of Soft Tissue and Bone. International Agency for Research on Cancer (IARC) Press; Lyon, France 2006. Page 79-80. 5. Guillou L, Gebhard S, Coindre JM. Orbital and extraorbital giant cell angiofibroma: a giant cell-rich variant of solitary fibrous tumor? Clinicopathologic and immunohisto chemical analysis of a series in favor of a unifying concept. Am J Surg Pathol. 2000;24:971-9. 6. Gengler C, Guillou L. Solitary fibrous tumor and haemangiopericytoma: Evolution of a concept. Histopathology 2006;48:63-74. 7. Thomas R, Banerjee SS, Eyden BP, Shanks JH, Bisset DL, Hunt R, Byers RJ, Oogarah P, Harris M. A study of 4 cases of extraorbital giant cell angiofibroma with documentation of some unusual features. Histopathology 2001;39:390-6. 8. Husek K, Vesel K. Extraorbital giant cell angiofibroma. Cesk Patol. 2002;38: 11720. 9. Qian YW, Malliah R, Lee HJ, Das K, Mirani N, Hameed M. A t(12;17) in an extra orbital giant cell angiofibroma. Cancer Genet Cytogenet. 2006;165:157-60.

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10. De Andrade CR, Lopes MA, De Almeida OP, Leon JE, Mistro F, Kignel S. Giant cell angiofibroma of the oral cavity: A case report and review of the literature. Med Oral Patol Oral Cir Bucal 2008;13:E540-3. 11. Wiebe BM, Gottlieb JO, Holck S. Extraorbital giant cell angiofibroma. APMIS 1999; 107:695-8 12. Sonobe H, Iwata J, Komatsu T, Fukushima A, Hayashi N, Moriki T, Shimizu K, Ohtsuki Y. A giant cell angiofibroma involving 6q. Cancer Genet Cytogenet. 2000;116:47-9. 13. Farmer JP, Lamba M, McDonald H, Commons AS. Orbital giant cell angiofibroma: Immunohistochemistry and differential diagnosis. Can J Opthalmol. 2006;41:216-20.