|Year : 2013 | Volume
| Issue : 2 | Page : 131-134
A case of recurrent malignant hemangiopericytoma of the hard palate
Saeed Nezafati1, Shirin Fattahi2, Faranak Moradi Abbasabadi2
1 Department of Oral and Maxillofacial Surgery, Faculty of Dentistry, Tabriz University of Medical Sciences, Tabriz, Iran
2 Department of Oral and Maxillofacial Pathology, Faculty of Dentistry, Tabriz University of Medical Sciences, Tabriz, Iran
|Date of Web Publication||3-Jan-2014|
Faranak Moradi Abbasabadi
Department of Oral and Maxillofacial Pathology, Faculty of Dentistry, Daneshgah Street, Tabriz, Eastern Azerbaijan
Source of Support: None, Conflict of Interest: None
Hemangiopericytoma is an aggressive vascular tumor with a high propensity for recurrence and metastasis. The tumor is extremely rare in the head and neck region. We present a case of hemangiopericytoma on the right side of the hard palate in a 28-year-old male patient, which recurred after 5 years. This report demonstrates the malignant nature of this neoplasm. Computed tomography scans revealed a mass on the right side of the hard palate, which extended into the nasal cavity. Microscopic features of malignant hemangiopericytomas have been described as ovoid or spindle shaped tumor cells surrounded by reticular fibers that proliferate around recognizable capillaries. The examination of resected recurrent tumor was consistent with hemimaxillectomy.
Keywords: Hard palate, hemangiopericytoma, hemimaxillectomy
|How to cite this article:|
Nezafati S, Fattahi S, Abbasabadi FM. A case of recurrent malignant hemangiopericytoma of the hard palate. J Orofac Sci 2013;5:131-4
|How to cite this URL:|
Nezafati S, Fattahi S, Abbasabadi FM. A case of recurrent malignant hemangiopericytoma of the hard palate. J Orofac Sci [serial online] 2013 [cited 2020 Apr 5];5:131-4. Available from: http://www.jofs.in/text.asp?2013/5/2/131/124260
| Introduction|| |
Hemangiopericytoma is a relatively slow-growing vascular tumor with locally invasive tendencies and variable malignant potential, constituting less than 1% of all neoplasms.  It typically occurs in the soft-tissues of the extremities and trunk and is rarely seen in the oral cavity.  Involvement of the palate is extremely rare, and only a few cases have been reported to date. ,
All osseous hemangiopericytoma are now regarded as malignant, but varying metastatic potential.  The diagnosis of hemangiopericytoma is made based on its characteristic architectural pattern and configuration in ultrastructural or immunohistochemical studies. 
This report presents a malignant and recurrent case of hemangiopericytoma in the hard palate.
| Case Report|| |
A 28-year-old male patient presented to Imam Reza Hospital, Tabriz University of Medical Sciences, Tabriz, Iran, in November 2011, with a mass on the right side of his hard palate.
According to the history of the patient, he had a tumor in this area 5 years previously [Figure 1]. Then hemangiopericytoma had been diagnosed and treated with hemimaxillectomy. The patient had been disease-free for 5 years and had not referred for regular follow-ups.
|Figure 1: The first computed tomography scan image revealed a mass on the right side of the hard palate|
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On physical examination, a pink non-tender pedunculated mass covered with healthy mucosa was seen adjacent to the upper second molar on the right side [Figure 2].
|Figure 2: Intra-operative photograph showing a well-circumscribed mass in the palate|
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Computed tomography (CT) scans of the maxillofacial region revealed a mass on the right side of the hard palate, extending into the nasal cavity, causing bone erosion [Figure 3]. Other assessments, including chest radiography, computerized neck scan and bone scintigraphy, were unremarkable. Under general anesthesia with nasotracheal intubation, the tumor was completely excised together with partial resection of the remaining palatal bone using a transoral approach.
|Figure 3: Computed tomography scanning revealed a mass on the right side of the hard palate, causing erosion of the surrounding bone and extending into the nasal cavity and maxillary sinus|
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Macroscopic examination of the specimen showed a solitary gray-brown soft-tissue, measuring 8 cm × 5 cm × 9 cm in size [Figure 4].
|Figure 4: Macroscopically, the lesion was a solitary grey-brown soft tissue mass, measuring 8 cm × 5 cm × 9 cm in diameter|
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Microscopically, the sections showed tightly packed cells that surrounded endothelium-lined vascular channels. The cells demonstrated pleomorphic nuclei. In some sections, four mitoses per 10 high-power fields and necrotic areas were present [Figure 5].
|Figure 5: Microscopic examination of the tumor shows tightly packed cells surrounding the thin-walled endothelium (H&E, ×200)|
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Immunohistochemically, the tumor cells were intensely positive for CD34 and vimentin [Figure 6] and completely negative for S-100, CD45, chromogranin, synaptophysin, cytokeratin, muscle specific actin and desmin.
Considering the results of immunohistochemical staining, neural, neuroendocrine, lymphoid, epithelial, and monophasic synovial sarcomas were ruled out. The final diagnosis was malignant hemangiopericytoma.
Six months after surgery, the patient had no sign and symptoms and recurrence [Figure 7].
| Discussion|| |
Hemangiopericytoma is a soft-tissue tumor derived from mesenchymal cells with pericytic differentiation. The tumor may occur anywhere in the body, with the most common anatomic locations being the lower extremities, the pelvis, and the head and neck region.  About 15-25% of all hemangiopericytomas occur in the head and neck lesions, especially in the nasal cavity, paranasal sinuses, orbital region, parotid gland, and neck, and rarely occur in the pharynx. 
It usually presents as a painless slow-growing mass, which is often nodular and well-circumscribed. The overlying skin or mucosa is generally normal in appearance and without obvious discoloration.  In our case, the external surface was smooth, and the cut surface had a homogeneous brown tan with geographic areas of necrosis.
On CT examinations, hemangiopericytoma in the head and neck region can be described as an expansile bone-remodeling lesion with a variably enhancing, fairly homogeneous appearance.  In our case, CT scans revealed a mass on the right side of the hard palate, extending into the nasal cavity, causing bone erosion.
On histopathology, it is usually fairly well-circumscribed, and exhibits tightly packed cells that surround endothelium-lined vascular channels. The cells are haphazardly arranged and demonstrate round to ovoid nuclei and indistinct cytoplasmic borders. The blood vessels often show irregular branching, leading to a characteristic "staghorn" and "antlerlike" appearance. The identification of four or more mitoses per ten high-power fields suggests a rapidly growing tumor capable of metastasis. The presence of necrosis also suggests malignancy.
In immunohistochemical findings reticulin stain demonstrates a dense reticulin network that surrounds the vessels and individual tumor cells. 
In our case, histopathology demonstrated a lack of circumscription and high mitotic rate, with a tendency toward necrosis, hemorrhage and invasion; immunohistochemical findings were positive for CD34 and vimentin, and negative for S-100, CD45, chromogranin, synaptophysin, cytokeratin, EMA and desmin in tumor cells, leading to report that the case was a malignancy.
The potential for malignant behavior, based on histological evaluation, is often difficult to predict. The differentiation between benign and malignant hemangiopericytomas can be difficult since histologically or cytologically benign-appearing hemangiopericytomas can prove malignant. 
The number of mitotic figures are variable and of prognostic significance.
The prognosis of hemangiopericytoma is unpredictable, and its benign or malignant nature is unpredictable, and its benign or malignant nature is not easily discernible histologically, especially in first-time lesion. 
It metastasizes via the lymphatics or by hematogenous spread, with the lung being a common site.
The treatment of choice is wide local excision, and long-term follow-up is advised.  In our case, because of the high incidence of local recurrence, hemimaxillectomy was performed along with radiation therapy and chemotherapeutic agents.
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[Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5], [Figure 6], [Figure 7]