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Year : 2015  |  Volume : 4  |  Issue : 3  |  Page : 192-195

Hemangiopericytoma in an 11-year-old patient: Case report and review

1 Department of Oral and Maxillofacial Surgery, Sibar Institute of Dental Sciences, Guntur; Department of Oral and Maxillofacial Surgery, Mamata Dental College and Hospital, Khammam, Andhra Pradesh, India
2 Department of Oral and Maxillofacial Surgery, Mamata Dental College and Hospital, Khammam, Andhra Pradesh, India
3 Department of Oral and Maxillofacial Surgery, Sibar Institute of Dental Sciences, Guntur, Andhra Pradesh, India

Date of Web Publication15-Sep-2015

Correspondence Address:
Kruthi Nanduri
Department of Oral and Maxillofacial Surgery, Sibar Institute of Dental Sciences, Takkellapadu, Guntur, Andhra Pradesh
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Source of Support: Nil., Conflict of Interest: None declared.

DOI: 10.4103/2277-8632.165402

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Hemangiopericytoma (HPC) is a rare vascular neoplasm originating from vascular pericytes. Though they can occur anywhere in the body, their incidence in head and neck region is low and is rare in children. Both benign and malignant variants are present, and they have a tendency to metastasize. We report a case of an HPC of the submandibular region in an 11-year-old female patient. Diagnosis and management are discussed along with a focus on previous literature.

Keywords: Benign, hemangiopericytoma, malignant, metastasis, submandibular

How to cite this article:
Nanduri K, Gaddipati R, Vura N, Lingamaneni KP. Hemangiopericytoma in an 11-year-old patient: Case report and review. J NTR Univ Health Sci 2015;4:192-5

How to cite this URL:
Nanduri K, Gaddipati R, Vura N, Lingamaneni KP. Hemangiopericytoma in an 11-year-old patient: Case report and review. J NTR Univ Health Sci [serial online] 2015 [cited 2022 Jan 16];4:192-5. Available from: https://www.jdrntruhs.org/text.asp?2015/4/3/192/165402

  Introduction Top

Hemangiopericytoma (HPC) is an uncommon mesenchymal tumor, which was first described by Stout and Murray in 1942. It arises from pericytes of Zimmerman, which form capillaries and regulate luminal size.[1],[2] Although this tumor can occur anywhere in the body within the vascular tissue, it is common in lower extremities, pelvis and retroperitonium while only 15-20% occur in head and neck region.[2] The tumor has no gender prediliction and is common in fourth to sixth decade of life and 82% are Caucasians [3],[4] HPC is extremely rare in pediatric age group with the incidence <10%.[4] The etiology of the tumor is unclear though various theories have been proposed like trauma that stimulates the proliferation of pericytes, long-term use of corticosteroids and vascular hypertension, but unproven.[5] There is some ambiguity regarding the management of these lesions, and the available literature is also less. We report a case of HPC of the submandibular region in an 11-year-old patient. The purpose of this report is to describe the clinical presentation, diagnosis and management of this lesion with a focus on previous literature.

  Case Report Top

An 11-year-old female patient presented to the department of oral and maxillofacial surgery with a chief complaint of painless swelling in the lower jaw on the right side since 2 years, which was gradually increasing in size. A detailed case history has been taken that denied any significant medical history, family history, or personal history. On extra-oral examination, a firm, nontender swelling measuring about 4 cm × 2 cm in size was seen at the right submandibular region [Figure 1]. The swelling was mobile, noncompressible, and the skin overlying was normal and pinchable. There was no regional or distant lymphadenopathy.
Figure 1: Preoperative clinical photograph demonstrating the swelling

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Intra oral examination did not reveal any significant findings except absence of the following teeth deciduous canine, deciduous second molar and first permanent molar on the right side. After the routine hematological investigations, which were within normal limits, the patient was subjected to radiographic evaluation.

Orthopentamogram did not reveal anything significant except mild bony erosion at the lower border of the mandible at the body region on the right side. Computed tomography (CT) showed a minimal soft tissue swelling at the mandibular body region with bony erosion of mandible on the buccal side [Figure 2]. Magnetic resonance imaging study (MRI) also revealed a soft tissue mass of 4.5 cm × 2.2 cm in size extending into the submandibular region [Figure 3]. Ultrasonography (USG) of the submandibular region (USG) revealed slight increase in vascularity and fine-needle aspiration cytology showed smears of less cellularity, occasional cystic macrophages, fibrocollagenous tissue fragments and blood elements. An incision biopsy was done that showed spindle shaped cells of a solid tumor with fibrous tissue and numerous small vessel formations marking the diagnosis of HPC. The patient was subjected to surgical excision of the tumor under general anesthesia. Through extra oral submandibular approach tumor mass exposed and excised completely [Figure 4], and the rough area of the mandible was smoothened. The excised specimen was sent for histopathological examination, which confirmed the preoperative diagnosis of HPC. Mitotic figures and other dysplastic changes were absent on histopathological examination [Figure 5]. The patient was under regular review and during follow-up after 1-year, mild facial asymmetry was noticed [Figure 6] and on examination, a small, firm swelling was identified at the site of previous surgery and MRI was taken, which revealed a local recurrence of the lesion involving floor of the mouth [Figure 7]. The patient is being subjected to radiotherapy.
Figure 2: Computed tomography scan showing bony erosion, marked by arrow

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Figure 3: Magnetic resonance imaging showing the swelling marked by arrow

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Figure 4: Excised specimen

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Figure 5: Histopathological picture on ×40 magnification

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Figure 6: One-year postoperative clinical picture showing facial asymmetry

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Figure 7: Magnetic resonance imaging showing recurrence marked by arrow

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  Discussion Top

Hemangiopericytomas account for no >1% of all vascular tumors.[6] Though uncommon in head and neck region (10-15%), tumors involving tongue, floor of the mouth, cheek, lips, nose, paranasal sinuses, maxilla and mandible, pharynx, parotid and orbital region have been reported by Bastakis and Rice.[7] In the present report, it is seen in the submandibular region. As it arises from capillary pericytes, it can occur in any part of the body containing capillaries. HPCs can be both benign and malignant, and the distinction is often difficult. HPCs occurring in infants younger than 1-year behave differently from those occurring in children older than 1-year and adults.[4] Infantile HPCs have been reported to behave like a benign tumor, while the adult variants simulate other soft tissue sarcomas in their presentation and prognosis.[2] The present case falls into the second category though changes of malignancy or dysplasia were not evident on histopathological examination. Clinical presentation of nonspecific pain is a late symptom associated with an enlarging mass, which is not reported by our patient. They are usually deep seated and common in muscle and subcutaneous tissue [8] as in our case.

Hemangiopericytomas have a tendency to metastasize early or late through vascular and lymphatic route.[8] Local recurrences are also common. The rate of metastasis of a malignant variety is reported to be >75% (including both local and distant).[9] Distant metastasis however, follow one or more local recurrences and a rare case of metastatic HPC to lung after 20 years of its initial occurrence in mandible was reported by Ravenel and Goodman,[9] which indicates the emphasis on long-term follow-up. A rare case of HPC of the ethmoid sinus causing oncogenic osteomalacia has been reported by Beech et al. in 2007.[10] Conventional radiographs are rarely diagnostic of HPC. CT and MRI are the usual techniques for evaluating these tumors. There are seen as large soft tissue masses with homogenous enhancement or large cystic spaces. Speckled calcifications are also seen. Angiography reveals their vascular nature.[3],[9]

There is some ambiguity regarding the management of these lesions and only a few reports regarding its clinical management have been published since HPC is very rare in children. However, surgery is considered the main stay of treatment though radiotherapy and chemotherapy have a role to play. Infantile HPCs respond well to chemotherapy, but its effect on adult type is unclear.[2] Only two cases of congenital maxillary HPCs have been reported so far by Gotte et al. in 1999 and Yamanishi et al. in 2007. Rodriguez-Galindo et al. reported three cases of infantile HPC, which responded very well to chemotherapy with vincristine, actinomycin-D and cyclophosphamide.[11] Most common treatment for HPC is wide surgical excision. In pediatric nonrhabdomyosarcoma soft tissue sarcomas, including HPC, the margin of resection >1 cm reduces local recurrence,[2] which has been done in our case. Some authors have suggested presurgical embolization to reduce the risk of intra operative bleeding.[3] Lee et al., 1997 and Florence et al., 2000 have suggested enucleation of the tumor if the biopsy shows a low grade malignancy, but wide excision is often followed. Initially, the tumor was thought to be radio-resistant,[8] but the recent literature supports radio therapy as an adjuvant with doses between 60 and 70 Gy in case of incomplete excision of tumor, local recurrences and high grade malignancy.[3],[12] Staples et al. has reported high rate of local recurrence in patients treated with surgery alone compared with those underwent both surgery and radiotherapy, which is consistent with our experience.

In our case, as histopathological examination revealed a benign variant and complete surgical removal was done ensuring the safe margins (>1 cm) and the patient is being subjected to radiotherapy as there is a local recurrence 1-year postoperatively. Prognosis is usually favorable, but depends on the mitotic activity of the tumor and size, as those measuring <6.5 cm as in the present report carry a better prognosis.[12] The 2 and 5 years survival rates were reported as 93% and 86% respectively by Espat et al.[13]

  Conclusion Top

Wide surgical excision is the most accepted management of HPC. Early diagnosis reduces the postsurgical morbidity and improves the prognosis. Radiotherapy should be considered as an adjunct to surgery to prevent recurrence. Since, local recurrences are very common and distant metastasis has reported to occur 20 years after primary occurrence, long-term follow-up, both clinically and radiologically is mandatory.

  References Top

Bhutia O, Choudhury AR. Hemangiopericytoma of mandible. J Oral Maxillofac Pathol 2008;12:26-8.  Back to cited text no. 1
  Medknow Journal  
Yamanishi T, Nishio J, Inoue M, Yasui M, Toribe Y, Takeuchi M, et al. A case of congenital maxillary hemangiopericytoma: A case report. J Oral Maxillofac Surg 2007;65:549-52.  Back to cited text no. 2
Thiele OC, Freier K, Flechtenmacher C, Rohde S, Hofele C, Mühling J, et al. Haemangiopericytoma of the mandible. J Craniomaxillofac Surg 2010;38:597-600.  Back to cited text no. 3
Sinha A, Rawson K, Singh G. A rare case of hemangiopericytoma in the maxilla of a 4 year old child. J Indian Acad Oral Med Radiol 2010;22:S64-6.  Back to cited text no. 4
Krishnan M, Kumar KS, Sowmiya T. Hemangiopericytoma - The need for a protocol-based treatment plan. Indian J Dent Res 2011;22:497.  Back to cited text no. 5
[PUBMED]  Medknow Journal  
Anand R, Gupta S. Hemangiopericytoma of maxilla in a pediatric patient: A case report. J Dent Child (Chic) 2010;77:180-2.  Back to cited text no. 6
Batsakis JG, Rice DH. The pathology of head and neck tumors: vasoformative tumors, part 9B. Head Neck Surg 1981; 4 : 326-39.  Back to cited text no. 7
Tatum RC, Skinner HG, Adrian J. Hemangiopericytoma of the parotid: report of a rare case with literature review. Quintessence International 1986; 17:399-403.  Back to cited text no. 8
Ravenel JG, Goodman PC. Late pulmonary metastasis from hemangiopericytoma of mandible unusual findings on CT and MR imaging. Am J Roentgenol 2001;177:244-5.  Back to cited text no. 9
Beech TJ, Rokade A, Gittoes N, Johnson AP. A haemangiopericytoma of the ethmoid sinus causing oncogenic osteomalacia: A case report and review of the literature. Int J Oral Maxillofac Surg 2007;36:956-8.  Back to cited text no. 10
Rodriguez-Galindo C, Kirk Ramsey BS, Jenkins JJ, Poquette CA, Kaste SC, Merchant TE, et al. Hemangiopericytoma in children and infants. Cancer 2000;88:198-204.  Back to cited text no. 11
Bianchi B, Poli T, Bertolini F, Sesenna E. Malignant hemangiopericytoma of the infratemporal fossa: Report of a case. J Oral Maxillofac Surg 2002;60:309-12.  Back to cited text no. 12
Espat NJ, Lewis JJ, Leung D, Woodruff JM, Antonescu CR, Shia J, et al. Conventional hemangiopericytoma: Modern analysis of outcome. Cancer 2002; 95:1746-51.  Back to cited text no. 13


  [Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5], [Figure 6], [Figure 7]


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