Giant cell angiofibroma (GCA) was first described in 1995, as a previously unrecognized soft tissue tumor of the orbit. Since that time, rare extraorbital head and neck manifestations of GCA have been documented. To the best of our knowledge, we present the fourth documented case of GCA of the buccal cavity, constituting the largest dominant site of occurrence in the extraorbital head and neck.
This is a single-surgeon retrospective case report and review of the literature of patients with extraorbital head and neck manifestations of giant cell angiofibroma.
There have been 8 extraorbital head and neck manifestations of giant cell angiofibroma, 4 within the buccal cavity. GCA occurring outside the head and neck, seems to occur more often in females. On the contrary, head and neck manifestations seem to have a male predilection. Extraorbital head and neck manifestations of GCA occurs primarily in adulthood at a median age of 45 (range 29-61), which has a similar distribution to orbital and non-head and neck sites. The highest age distribution seems to be in the fifth decade of life.
Extraorbital head and neck manifestation of GCA represents a unique disease entity with a distinctive gender predilection different from that of other sites. In the review of the literature, it seems that the buccal mucosa is the most prevalent extraorbital head and neck site. Awareness of the lesion, and its close relationship with other soft tissue tumors is important in the differential diagnosis and management.
Giant cell angiofibroma, Rare head and neck tumor, Head and neck surgery
Giant cell angiofibroma (GCA) was first described by Dei Tos, et al. in 1995, as a previously unrecognized soft tissue tumor of the orbit [1]. It was pathologically characterized as having patternless round to spindle cell proliferation with moderate to high degree of cellularity. All cases further exhibited prominent vascularity, vessel wall hyalinization, and lacunar and irregularly shaped pseudovascular spaces containing granular material. Since that time, rare extraorbital head and neck manifestations of GCA were documented in the scalp, vocal cord, oral cavity, submandibular region, and parapharyngeal space [2-6]. To the best of our knowledge, we present the fourth documented case of GCA of the buccal cavity, constituting the largest dominant site of occurrence in the extraorbital head and neck.
A 29-year-old otherwise healthy African American male presented for evaluation of a right buccal mass present for 1 year. Based on patient history, the mass had exhibited slow, painless growth. The mass was located within the right buccal mucosa, measuring approximately 2.5 × 2.0 cm, approximately 2 cm posterior to the oral commissure. On exam, it was soft, freely mobile, non-fluctuant, without changes to the overlying mucosa. The patient underwent a transoral resection of the mass, with primary closure. Intraoperativley, a transoral incision was made just 1 cm anterior to lesion, and through blunt dissection the lesion was easily delivered from the buccal space. The patient was closed primarily, without any postoperative complications.
Microscopical analysis revealed a well delineated neoplasm composed of proliferating bland spindle cells with intervening collagen deposition and foci of giant cell formation. Irregular capillaries were also present. The neoplasm was found to be bcl-2, CD34, CD99, and Factor XIIIa positive, and s-100 and smooth muscle actin negative.
Giant cell angiofibroma was originally described as a unique orbital tumor by Dei Tos, et al. in a series of seven patients, of whom 6 were male, with a median age of 59 (range 24-73) [1]. It was thought only to occur in the orbit, and as such the original authors designated the lesion "Giant Cell Angiofibroma of the Orbit". All lesions were found in the eyelid, in the region close, or involving the lacrimal gland. Within their study they described the lesion as sharing similar characteristics but distinct from solitary fibrous tumors and giant cell fibroblastoma.
Since its original description, rare cases have been described in approximately 18 extraorbital location including the mediastinum, parascapular region, retroauricular sites, thigh, retroperitoneum, vulva, hip, forearm, groin [2,7]. Within the head and neck, extraorbital cases have been documented in the scalp, vocal cord, oral cavity, submandibular region, and parapharyngeal spaces (Table 1).
Table 1: Literature review of documented giant cell angiofibromas in the head and neck with pathologic description. View Table 1
In review of the case report literature, it was found that there is no gender predilection in extraorbital head and neck sites, in contrast to the original orbital lesion found mostly in males. Furthermore, GCA occurring outside the head and neck, in regions such as the thigh, retroperitoneum, hip and forearm, seems to occur more often in females [9,10]. Extraorbital head and neck manifestations of GCA occurs primarily in adulthood at a median age of 45 (range 29-61), which has a similar distribution to orbital and non-head and neck sites. The highest age distribution seems to be in the fifth decade of life.
Pathologic assessment of GCA from the extraorbital head and neck uniformly reveals an unencapsulated, well circumscribed lesion with the presence of round to spindle shaped cells and giant cells lining pseudovascular spaces. All lesions are benign appearing, with an indolent course, treated with whole specimen surgical excision, without documentation of recurrence. However, one documented case by Arifin, et al. revealed the presence of bony erosion on pathologic assessment and radiographic imaging of a lytic lesion [2]. However, despite the bony erosive changes, no documentation of malignant behavior or recurrence was evident. Furthermore, GCA lesions uniformly stain positive for CD34 and vimentin, with a no staining to cytokeratin, smooth muscle actin, s100, demisn, p63, CD31. Gonzalez-Perez, et al. further performed in situ hybridization studies to rule out synovial sarcoma, with probes to the SYT gene (18q11.2) that were determined to be negative [4].
Given the rarity of GCA in the extraorbital head and neck, the authors describe a tumor that has a propensity to occur in the soft tissue of the head and neck. In the review of the literature, it seems that the buccal mucosa is the most prevalent extraorbital head and neck site. Awareness of the lesion, and its close relationship with other soft tissue tumors is important in the differential diagnosis and management.
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