Glomangiopericytoma of the pterygomandibular space: An unusual case

doi:10.1016/j.bjoms.2006.10.002

British Journal of Oral and Maxillofacial Surgery

Volume 45, Issue 8, December 2007, Pages 673-675

https://doi.org/10.1016/j.bjoms.2006.10.002 Get rights and content

Abstract

We describe a case of glomangiopericytoma located in the pterygo-mandibular space, a rare anatomical region for this neoplasm to develop. The lesion is classified as a separate variant from the classic haemangiopericytoma, which is characterised by more aggressive biological behaviour.

Introduction

Haemangiopericytomas are rare vascular tumours that originate from pericytes. Haemangiopericytoma-like tumours were defined by Compagno and Hyams¹ as a group of tumours that develop in the head and neck region. They have a better prognosis despite their similarity to the conventional tumour. They often have histological features that look different from haemangiopericytomas because they express a more myogenic differentiation that resembles a glomus tumour. The term glomangiopericytoma or sinonasal-type haemangiopericytoma is preferred to describe them.²

We present a case of glomangiopericytoma of the pterygomandibular space. It is worthy to note because it is rare to find such tumours in that region, and it is difficult to differentiate the diagnosis from histologically dissimilar lesions that are found in the same area.

Section snippets

Case report

A 42-year-old woman was admitted to our Unit with a swelling of the right side of the face. She gave a history of referred pain associated with paraesthesia. On examination there was a swelling in the masseter-parotid region, which was hard on palpation, but there was no enlargement of the adjoining lymph nodes.

A computed tomogram (CT) with contrast showed a mass composed of heterogeneous tissue in the area of the right ramus of the mandible, which had been thinned and fractured at its apex by

Discussion

Stout and Murray,³ in an analysis of 691 vascular neoplasms, identified 9 patients with haemangiopericytomas. These lesions originate from pericytes, and so the tumours can affect any area of the organism in which capillaries are present.

Histologically they have a prominent pericytic vascular pattern, with thin-walled, branching vessels often with a “staghorn” configuration.

However, many benign and malignant soft tissue tumours share this vascular pattern (haemangiopericytoma-like).

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There are more references available in the full text version of this article.

Cited by (11)

Glomangiopericytoma-type glomus tumour/myopericytoma of the lip
2019, British Journal of Oral and Maxillofacial Surgery
Citation Excerpt :
Glomangiopericytoma was first described in 1998 as a soft tissue tumour that was characterised by branching haemangiopericytoma-like vessels surrounded by glomus-like cells.2 To date, the term glomangiopericytoma is also used as a synonym for a sinonasal haemangiopericytoma-like tumour, a perithelial myoid tumour that is virtually specific to the sinonasal area 9,10 and differs from a glomangiopericytoma-type glomus tumour/myopericytoma in the nuclear expression of β-catenin as a result of gain-of-function CTNNB1 mutations. 10 Despite this, glomangiopericytoma-type glomus/myopericytoma is recognised as a specific histological entity,1 and glomangiopericytoma-like areas can be detected in other pericytic (perivascular) tumours, including glomus tumours, myopericytomas, myofibromas, and angioleiomyomas.1–8
We describe the case of a 46-year-old man who presented with a four-month history of a reddish, otherwise asymptomatic, nodular lesion that involved the mucosal side of the upper lip. The lesion consisted of myoid glomus-like cells arranged around branching and thin-walled haemangiopericytoma-like vascular structures, and was classified as glomangiopericytoma. No recurrences occurred during an eight-month follow-up. To the best of our knowledge, this tumour has never previously been described in the mucosa of the lip.
Venous malformation arising in the pterygomandibular space: A case report
2019, Journal of Oral and Maxillofacial Surgery, Medicine, and Pathology
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Because mass lesions that develop in PMS are located at such an anatomically intricate site, some cases may have been treated as lesions occurring in a part of the masticator space [6] or parapharyngeal space [7] depending on the lesion size. However, our search of the literature revealed only 8 such cases [8–15]. Histologically, the lesions occurring in PMS are diverse, with reports of malignant tumor [8,14], vascular tumor [9], neurogenic tumors [11,13,15], and so on [10,12].
We report a rare case of venous malformation in the pterygomandibular space. A 49-year-old Japanese woman experienced discomfort on opening her mouth since 3 months but had not sought treatment previously. At initial examination, no submandibular lymph node enlargement, trismus, perceptual dysfunction were noted.
The surface mucosa on the lateral wall of the left middle pharynx was normal, and no mass could be palpated in the oral cavity. Panoramic X-ray examination revealed a clearly demarcated, elliptical, radiolucent area on the left mandibular ramus. Computed tomography revealed a clearly demarcated, nearly spherical mass of 17 × 11 × 16 mm near the mandibular foramen of the left mandibular ramus. Magnetic resonance imaging revealed signal hypointensity, which was approximately similar to that of muscle tissues, on T1-weighted images and signal hyperintensity on T2-weighted images. On contrast T1-weighted images, a mass with relatively good contrast effects and some internal areas of low concentration was revealed. A clinical diagnosis of benign tumor in the left pterygomandibular space was made, and enucleation was performed using intraoral approach under general anesthesia. The tumor was dark purple and encapsulated. It showed color-fading and was not pulsatile. Although venous vascular malformation was suspected, en bloc extraction including the capsule could be performed because no adhesion with surrounding tissue or blood vessels was observed. Histopathological diagnosis was venous malformation. Although neurological deficit symptoms in the lower lip and tongue were observed postoperatively, subsequent improvement was noted. Currently, at >5 postoperative years, no recurrence has been found.
The midfacial glomangiopericytoma - Case report
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That's why it is an equivalent term for sinonasal-type hemangiopericytoma.4 The term hemangiopericytoma only previously used for such type of tumors has been abandoned as they have better prognosis despite their similarity to the conventional tumors.3 Glomangiopericytoma tumors also differ from conventional soft tissue hemangiopericytomas in location, biologic behavior and histologic features – they express more myogenic differentiation.
Glomangiopericytoma is classified as a borderline low malignancy tumor but recurrence rate is reported to be about 16.8%–30%, probably caused by the lack of radical resection.¹ It is a rare tumor arising from pericytes surrounding capillaries. The tumor has a predilection for the nasal cavity and paranasal sinuses.
We report a case of glomangiopericytoma with an aim to present how it can invade and destroy both maxillary bones and lower part of nasal cavity. Due to its dimensions the tumor required extensive resection within tumor-free margins. On the basis of immunohistochemical staining the tumor was diagnosed as glomangiopericytoma.
Strict follow-up of 16 months after radical resection revealed no recurrence of the tumor.
Glomangiopericytoma of the nasal cavity
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The WHO unified similar concepts into the category of glomangiopericytoma in 2005 [3]. Glomangiopericytoma is defined as a sinonasal tumor demonstrating perivascular myoid phenotype, but only three cases of glomangiopericytoma have been described [7–9]. Although trauma, hypertension and long term steroid use are said to be possible causes of the tumor [4], the patient did not have the history of trauma hypertension and steroid use.
Glomangiopericytoma is a rare tumor arising from the pericytes surrounding capillaries, and accounts for less than 0.5% of all sinonasal tumors. A 60-year-old male patient presented with a glomangiopericytoma of the nasal cavity manifesting as nasal obstruction and epistaxis, which was treated successfully with endoscopic excision. Histological examination showed multiplication of spindle-shaped to oval cells which reacted strongly to immunostaining for α-smooth muscle actin. Glomangiopericytoma is categorized as a borderline low malignancy tumor, which tends to recurrence. Strict follow-up is required, especially if complete resection is not achieved.
Sinonasal Glomangiopericytoma-A Case Report
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CT and MRI findings of glomangiopericytoma in the head and neck: Case series study and systematic review
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Short communicationGlomangiopericytoma of the pterygomandibular space: An unusual case

Abstract

Introduction

Section snippets

Case report

Discussion

Am J Otolaryngol

J Oral Maxillofac Surg

Hemangiopericytoma-like intranasal tumors. A clinicopathologic study of 23 cases

Am J Clin Pathol

Sinonasal haemangiopericytoma-like tumour: a sinonasal glomus tumour or a haemangiopericytoma?

Histopathology

Haemangiopericytoma: a vascular tumour featuring Zimmerman's pericytes

Ann Surg

Short communication
Glomangiopericytoma of the pterygomandibular space: An unusual case