Hemangiopericytoma of the Oral Cavity After a Ten-Year Follow-Up

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274 Annals of Clinical & Laboratory Science, vol. 37, no. 3, 2007 Case Report: Hemangiopericytoma of the Oral Cavity after a Ten-year Follow-up

Emiliano Maresi,1 Silvia Tortorici,2 Maria Campione,1 Maria L. Buzzanca,2 Francesco Burruano,2 Filiberto Mastrangelo,3 and Stefano Tetè3 Departments of 1Pathology and 2Oral and Maxillofacial Surgery, Policlinico P. Giaccone, University of Palermo, Palermo, Italy; 3Department of Oral Sciences, University of Chieti, Chieti, Italy

Abstract. Hemangiopericytoma (HPC) is a mesenchymal tumour that may be benign, malignant, or occur in an intermediate form. We report an unusual case of hemangiopericytoma located in the buccal mucosal region. The histopathologic features showed increased cellularity, necrosis, hemorrhage, low proliferation index, and 4 or less mitotic figures per 10 high-power fields. Since this histological pattern suggests an intermediate form characterized by unpredictable clinical behavior, life-long follow-up is essential. In this patient no recurrences or distant metastases were evident at 10-yr follow-up.

Keywords: hemangiopericytoma, tumor of oral cavity

Introduction mandible, floor of mouth, hard palate, buccal mucosa, retromolar gingiva, parapharyngeal spaces, Hemangiopericytoma (HPC) is a soft tissue tumor and larynx [2-15]. In a metanalysis by Brockbank arising from the pericytes of Zimmermann, which [16], from 1949-1979 only 35 cases were reported are modified smooth-muscle cells surrounding in the oral cavity, including: tongue (9 cases), upper capillary vessels. These pericytes are located outside jawbone (5 cases), lips (4 cases), buccal region (3 the reticulin sheath of the endothelium. Since the cases), cheeks (3 cases); gingiva (3 cases), parotid vascular pattern expressed by such tumors is gland (1 case), and multifocal lesions (1 case) common to other tumors, the diagnosis of HPC is HPC can occur in any age group and there is based on the following morphologic criteria: on no sex predilection. The biological behavior of immunohistochemical analysis, the tumor cells are HPC is unpredictable. Malignant forms are negative for α-smooth muscle actin, desmin, S-100 characterized by necrosis, cellular pleomorphism, protein, and cytokeratin, are intensely positive for high proliferation index, and mitoses >4 per 10 vimentin, and are focally positive for CD34. high-power fields. The absence of necrosis, cellular Described in 1942 by Stout [1], HPC occurs pleomorphism, and mitoses <4 per 10 high-power most commonly in the soft tissues of the upper and fields does not necessarily indicate benignancy; in lower extremities, and the retroperitoneum. fact, tumors with benign histologic appearance Location in the head-neck region is uncommon have been reported to metastasize [17-22]. (16%) with lesions reported arising in the orbit, nasal sinus tract, parotid gland, tongue, lip, maxilla, Case Report

Address correspondence to Professor Stefano Tetè, Depart- A 20-yr-old Caucasian female presented with a 3- ment of Oral Sciences, University of Chieti, Via dei Vestini mo history of a hazelnut-sized swelling in the 31, 66100 Chieti, Italy; tel 39 0871 3554095; fax 39 0871 mouth. The mass had a wide base implant, a tense- 3554095; e-mail [email protected]. elastic consistency, was mobile in relation to deep

0091-7370/07/0300-0274. $1.50. © 2007 by the Association of Clinical Scientists, Inc. Hemangiopericytoma of the oral cavity 275 and superficial tissues, and was located at the upper Discussion vestibule in the incisor area. The oral mucosa that covered the mass was strained and moderately HPC is a rare mesenchymal tumor that occurs as a telangiectatic on palpation; pulsation was not localized mass; its diameter can range from 1 to 20 evident. Surgical excision of the lesion was cm; it has slow growth and is usually asymptomatic. performed after ligation of the vascular peduncle. Based on the medical history and clinical findings On macroscopic examination the lesion was an of some patients, various etiological factors have ovoid mass (diameter about 4 cm) circumscribed been suggested (eg, hypertension, hormonal or by a thin, transparent fibrous capsule; sectioning metabolic imbalance [2,18-23], and trauma [1]) but revealed a variegated cut-surface with red, yellow, the etiology of HPC is unknown. and grey areas (Fig. 1). Microscopically the The differential diagnosis between hemagioperi- neoplasm was characterized by abundant and cytoma and other tumor types characterized by a compact cellular proliferation having a fusiform ramified and pericytoma-like vascular proliferation and monomorphic-like appearance; no cytological (Table 1) requires immunohistochemical stains to atypias were evident; on the other hand, a rich net exclude epithelial, muscle, and neural neoplasms. of capillary vessels having a staghorn-like shape Vimentin is the only marker that is consistently was found, with lumina surrounded by endothelial expressed in HPC; with the exception of CD34, cells (Fig. 2). The tumor cells had a pale cytoplasm which may stain the tumor cells, vascular markers with indistinct cell borders and ovoid or round (factor VIII, CD31) stain only the endothelial cells nuclei; some tumor cells were extended and of the blood vessels. An immunohistochemical fusiform (Fig. 3). profile can be useful in cases of Schwannoma, The mass was entirely circumscribed by a neurofibroma, peripheral nerve sheath tumor, fibrous capsule that did not appear to be infiltrated angioleiomyoma, and solitary myofibroma, where by tumor cells. Although cellularity was mainly the morphological differences are not clear. The high and compact, focal areas were observed in tumor that most closely resembles HPC is the which the cells were distorted due to hemorrhagic solitary fibrous tumor, whose cells also stain for and necrotic phenomena or due to deposition of vimentin and CD34; histologically some differences mixoid material and collagen fibres (Fig. 4). The exist: (a) HPC shows homogeneously higher cell- cells were haphazardly arranged and surrounded by ularity and staghorn-like vessels throughout the a dense reticulum; nevertheless, they tended to lesion, (b) SFT shows varying cellularity and often assume a circumferential course around the vessels. thick and keloid-like hyalinization, and (c) Sporadic lymphocytes were seen among the cells. numerous mast cells are found in SFT and not in Mitotic figures were <4 per 10 high-power fields. HPC [17,31-36]. Immunohistochemically the tumor cells showed The clinical behavior of HPC varies from case diffuse positivity for CD34 (Fig. 5) and vimentin, to case. According to the histological findings these but were negative for smooth muscle actin, desmin, tumors are classified as low-grade, intermediate- cytokeratins, CD 31, S100 protein, and EMA grade, or high-grade based on mitoses, cellularity, High cellularity, low proliferation index, and cellular pleomorphism. HPC cases with high- hemorrhagic necrosis, and <4 mitoses per 10 high- grade malignancy have been reported with lymph power fields all suggested an uncertain but not very node involvement and bone, pulmonary, and aggressive biological behavior, as reported in the hepatic metastases, at a frequency that ranges from literature [15,17]. The patient needed to be followed 12 to 56%; metastases usually occur within 5 yr closely for an extended period. Upon follow-up after initial diagnosis and are rare after 10 yr [24]. examination at 10 yr post-resection there was no Recurrence has been reported in 20 to 57% of evidence of tumor recurrence or metastasis, HPC cases approximately 17 mo after the initial confirmed by negativity of the head-facial computed treatment [2,25-30]. The prognosis of HPC is tomographic (CT) scan, bone scintigraphy, and unpredictable; the histologic features are poorly chest X-ray, correlated with the clinical behavior of the tumor, 276 Annals of Clinical & Laboratory Science, vol. 37, no. 3, 2007

Fig. 1. Ovoid tumor mass of about 4 cm diameter, circum- Fig. 2. Microscopy shows networks of capillary vessels with scribed by a thin, transparent fibrous capsule. The cut-surface a staghorn-like shape, whose lumen is lined by flat endothelial has variegated color with red, yellow, and grey areas. cells, surrounded by proliferation of fusiform monomorphic cell without atypia (H&E, original magnification x150).

Fig. 3. The tumor cells present a pale cytoplasm with indistinct Fig. 4. Deposits of collagen fibers among the tumor cells cell borders and ovoid or round nuclei (H&E, original (H&E, original magnification x200) magnification x 200).

although Enzinger and Smith [2] suggested as malignant indices: tumor diameter >6.5 cm, mitotic figures >4 per 10 high-power fields, increased cellularity, cellular pleomorphism, necrosis and/or hemorrhage. Surgical excision of the HPC lesion represents a treatment of choice, with preceding ligation of the vascular bundle that nourishes the neoplastic tissue, achieving reduction of the size of the neoplasm as well as its removal [25,37-42]. The efficacy of radiation therapy is controversial because HPC is considered to be radioresistant; however, radiation therapy can be useful in the treatment of Fig. 5. Immunohistochemical staining of tumor cells shows aggressive HPC and incomplete resections. diffuse and high positivity for CD34 antigen (original magni- Chemotherapy and immunotherapy have been fication x200). Hemangiopericytoma of the oral cavity 277

Table 1. Immunohistologic findings in tumors with a hemangiopericytoma-like pattern.

Tumors Vimentin Factor XIIIa Actin Desmin CD31 CD34 Cytokeratin S100 NSE CD68

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