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SHORT COMMUNICATION  
Year : 2011  |  Volume : 22  |  Issue : 3  |  Page : 497
Hemangiopericytoma - The need for a protocol-based treatment plan


Department of Oral and Maxillofacial Surgery, Meenakshi Ammal Dental College and Hospital, Meenakshi Academy Of Higher Education and Research (MAHER), Alapakkam Main Road, Maduravoyal, Chennai, Tamil Nadu, India

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Date of Submission24-Nov-2010
Date of Decision11-Feb-2011
Date of Acceptance02-May-2011
Date of Web Publication3-Nov-2011
 

   Abstract 

Hemangiopericytoma is a vascular tumor which comprises only 1% of all vascular tumors. The frequency of occurrence in the head and neck accounts for about 16-33% of all hemangiopericytomas. In this paper we discuss the surgical management, the difficulties in decision-making and treatment-planning in a case of a maxillary tumor in a five-year-old boy with a two-year follow-up. A five-year-old boy presented with a large unilateral maxillary tumor with nasal obstruction. Computed tomography revealed a heterogeneous mass completely occupying the right maxillary sinus and displacing the lateral wall of the nose and nasal septum. The lesion was diagnosed as hemangiopericytoma after histopathological confirmation. The option of surgical resection (total maxillectomy) was carried out after evaluating the available literature. Various treatment modalities like surgery, chemotherapy and radiotherapy were taken into consideration as the tumor has an aggressive nature. Due to the inadequate literature on definitive treatment options for these types of tumors, there was difficulty in arriving at a protocol-based treatment plan.

Keywords: Head and neck, hemangiopericytoma, tumor, vascular

How to cite this article:
Krishnan M, Kumar K S, Sowmiya T. Hemangiopericytoma - The need for a protocol-based treatment plan. Indian J Dent Res 2011;22:497

How to cite this URL:
Krishnan M, Kumar K S, Sowmiya T. Hemangiopericytoma - The need for a protocol-based treatment plan. Indian J Dent Res [serial online] 2011 [cited 2014 Oct 22];22:497. Available from: http://www.ijdr.in/text.asp?2011/22/3/497/87086
Hemangiopericytoma (HPC) is a rare vascular tumor in the head and neck region arising from the vascular pericytes of Zimmerman, which occurs around capillaries and post-capillary venules [1] and accounts for 16-33% of vascular tumors in the head and neck. [2] Incidences in pediatric cases account for less than 10%. [3] Inadequate literature is available regarding the management of HPC. The complexities involved in diagnosis and treatment-planning are presented below.


   Case Report Top


A five-year-old male in apparent good health reported to our department with a slow-growing swelling on the right side of the face [Figure 1]. It was noticed about a year back with associated mild intermittent pain. The clinical examination revealed a diffuse swelling on the right side of the face extending supero-inferiorly from the infraorbital margin to the line drawn from the angle of the mouth to the tragus of the ear, medio-laterally from the philtrum to the perpendicular drawn from the lateral canthus of the eye, antero-posteriorly from the ala of the nose to the pre-auricular region. Ophthalmological evaluation revealed no abnormality. Nasal obstruction was apparent on the right side. There was no history of epistaxsis. Patient had paraesthesia on the right cheek, nose and upper lip. There was no associated lymphadenopathy. The skin over the swelling was pinchable with no secondary changes. The swelling was hard in consistency, non-tender, and non-pulsatile. Intra-orally the swelling extended antero-posteriorly from the right maxillary primary central incisor to the primary second molar and mediolaterally obliterating the right buccal vestibule up to the midline of the hard palate with mobility of teeth, inflammed gingiva [Figure 2].
Figure 1: Clinical presentation with swelling on the right side of the face

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Figure 2: Intraoral appearance of the lesion

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Radiologically, paranasal sinus view showed a radiopaque mass occupying the right maxillary sinus and obliterating the lateral wall of the nose. Computed tomography revealed a heterogeneous mass completely occupying the right maxillary sinus and displacing the lateral wall of the nose and nasal septum [Figure 3]. Incisional biopsy was done and the diagnosis was suggestive of HPC.
Figure 3: Preoperative CT scan-axial view

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Total maxillectomy of the right side was carried out under general anesthesia. The lesion was approached using a Weber Fergueson incision. The patient was rehabilitated with a maxillary obturator postoperatively. The patient was followed up every month during the first six months. Postoperative review up to two years showed uneventful healing with no evidence of recurrence until the present day. [Figure 4]. The histopathological examination confirmed the diagnosis of HPC [Figure 5].
Figure 4: Postoperative CT scan-coronal view

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Figure 5: H and E showing stag-horn appearance

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   Discussion Top


Hemangiopericytoma (Glomangiopericytoma) was described by Stout and Murray in 1942, [1] while studying the glomus tumor.

In children HPC comprises two different clinical entities. Forty percent of cases occur during the first year of life, [3] and are considered to be congenital-infantile HPC and less aggressive. Those occurring in children older than one year behave in a manner similar to adult HPC and are more aggressive. [4]

The etiology of HPC remains unknown. Previous history of trauma has raised the possibility that this may stimulate proliferation of pericytes following damage to the capillary network. Long-term steroid therapy, and arterial hypertension are been thought to cause HPC but none of these theories have been proved. [5] Cytogenetic abnormalities are being studied and most HPC are near diploid, and break points in 12q13, 12q24 and 19q13 are common with recurrent t(12;19)(q13;q13) translocation. [6]

HPCs are slow-growing, solitary and indolent lesions showing local infiltration. Due to their wide array of nonspecific symptoms the lesions could reach large proportions before the diagnosis is made. Moreover inadequate literature is available regarding the management of these tumors. According to histopathology, HPC-like lesions are generally divided into three groups. The present case falls into the second category of true HPC according to th Gengler et al. [7]

But this does not alter the management of these lesions as surgical resection seems to be the mainstay among treatment modalities. Radiotherapy and chemotherapy have shown mixed results. Early identification and management seems to be the key to these situations. If the lesions can be identified in the early stage the extent of surgical morbidity can be reduced which will directly reflect on the long-term wellbeing and quality of life. Emphasizing the need for a detailed assessment during the routine pediatric clinician visit might be a table-turner for the early diagnosis of these lesions.

Surgical resection is the mainstay of treatment. The surgical morbidity associated with the treatment is high because of the aggressiveness of the lesion. So the post-surgical rehabilitation remains a challenge. The infantile subtype is characterized by high response to chemotherapy. [8] Although a few unresectable lesions at diagnosis were treated with neoadjuvant chemotherapy with very good clinical responses the role of chemotherapy in the treatment is not well-established. These tumors are regarded as radio-resistant, although success has been reported by some authors. Adjuvant radiotherapy has a definite role in the treatment of HPC in both the primary setting and the recurrent setting. Radiation doses >50 Gy are required for local control of disease. [9]


   Conclusion Top


Due to the inadequate literature on the treatment options for these types of tumors, there was difficulty in arriving at a definitive treatment plan. The option of surgical resection was decided after evaluating the available literature. Surgical resection by itself poses a challenge for the surgical team because of the size, extent and proximity of the lesion to vital anatomical structures. The early diagnosis and management of these lesions can limit post-surgical morbidity. Experience gained from the management of these vascular tumors should be used to devise a protocol that can be adhered to during treatment planning, which will reduce the ambiguity surrounding the management of the lesion. This can go a long way in improving the quality of life of these patients.

 
   References Top

1.Stout AP. Hemangiopericytoma. A study of twenty-five new cases. Cancer 1949;2:1027-35.   Back to cited text no. 1
[PUBMED]    
2.Barnes L. Tumors and tumor-like lesions of the soft tissues. In: Barnes L. editor Surgical Pathology of the Head and Neck, 2 nd ed. New York: Marcel Dekker Inc; 2001. p. 890-1048.  Back to cited text no. 2
    
3.Walike JW, Bailey BJ. Head and neck hemangiopericytoma. Arch Otolaryngol 1971;93:345-53.   Back to cited text no. 3
[PUBMED]    
4.Kauffman SL, Stout AP. Hemangiopericytoma in children. Cancer 1960;13:695-710.  Back to cited text no. 4
[PUBMED]    
5.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. 5
    
6.Kumar R, Corbally M. Childhood hemangiopericytoma. Med Pediatric Oncol 1998;30:294-6.  Back to cited text no. 6
    
7.Gengler C, Guillou L. Solitary fibrous tumour and haemangiopericytoma: Evolution of a concept. Histopathology 2006;48:63-74.  Back to cited text no. 7
[PUBMED]  [FULLTEXT]  
8.Hallen M, Parada LA, Gorunova L, Pålsson B, Dictor M, Johansson B. Cytogenetic abnormalities in a hemanigiopericytoma of the spleen. Cancer Genet Cytogenet 2002;136:62-5.  Back to cited text no. 8
    
9.Del Rosario ML, Saleh A: Preoperative chemotherapy for congenital hemangiopericytoma and a review of the literature. J Pediatr Hematol Oncol 1997;19:247-50.  Back to cited text no. 9
    

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Correspondence Address:
Murugesan Krishnan
Department of Oral and Maxillofacial Surgery, Meenakshi Ammal Dental College and Hospital, Meenakshi Academy Of Higher Education and Research (MAHER), Alapakkam Main Road, Maduravoyal, Chennai, Tamil Nadu
India
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DOI: 10.4103/0970-9290.87086

PMID: 22048604

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    Figures

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



 

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