Our Experience with Sinonasal Glomangiopericytoma in North India: A Case Series

Abstract

Glomangiopericytoma (GPC) is a rare benign sinonasal tumor originating from Zimmerman’s Pericytes surrounding capillaries and accounting for less than 0.05% of all sinonasal tumors. Glomangiopericytoma has low malignant potential (5–10%) and is mostly diagnosed in the 6th or 7th decade of age with slight female preponderance. We presented here a case series of 5 patients with sinonasal GPC. This research was conducted at a tertiary healthcare centre in North India. In our case series, all the patients were evaluated and underwent endoscopic surgical resection. All patients underwent digital subtraction angiography (DSA) and preoperative embolization. The coblation technique used for haemostasis proved very effective and time-saving. All patients exhibited cytoplasmic SMA positivity (a marker of GPC) and CD34 negativity, while one patient exhibited a high Ki-67 index (> 10%), which is a predictor of aggressive tumor behavior. None of the patients showed any recurrence in follow-up. We recommend performing complete endoscopic surgical excision to prevent recurrence. The use of DSA, preoperative embolization, and intraoperative use of the coblation technique provides a cleaner surgical field and reduced operating time.

Introduction

Glomangiopericytoma (GPC) is a rare benign sinonasal tumor originating from Zimmerman’s Pericytes surrounding capillaries and accounting for less than 0.05% of all sinonasal tumors [1, 2]. GPC is classified as a distinct entity in 2005 by the World Health Organization [3]. GPC is usually localized to the nasal cavity, with paranasal extension, however, the skull base involvement is rare [4]. Sphenoid and ethmoidal sinuses are most commonly involved if there is a paranasal extension [5]. The most common symptoms are epistaxis, headache, nasal obstruction, and sinusitis [4]. The etiology is still unclear but the role of previous trauma, pregnancy, corticosteroids, and hypertension has been mentioned in the literature [5]. We present here a series of 5 cases of GPC. Written and informed consent were taken from each of the participants.

Case 1

22 years old male presented with complaints of right-sided nasal obstruction for 2 years. On evaluation, the general examination and vitals were within normal limits. No abnormality was detected on the anterior rhinoscopy. However, Diagnostic Nasal Endoscopy (DNE) revealed a firm reddish mass arising from the posterior end of the nasal septum on the right side and extending up to the posterior end of the right middle turbinate and right sphenoethmoidal recess (Fig. 1a).

Fig. 1.

Images of Case 1. a Lesion visualized on DNE, b intraoperative endoscopic image of lesion of the second patient, c DSA and preoperative embolization, Pre-embolization, and d DSA and preoperative embolization, post-embolization

Contrast-Enhanced Magnetic Resonance Imaging (CEMRI) revealed a well-defined lesion 23 × 14 × 20 mm in the nasopharynx, superiorly based at the skull base and projecting into the right post choana, superolaterally abutting the sphenopalatine foramen and cranial part of the medial pterygoid plate, medially abutting the posterior part of the nasal septum, anteriorly abutting posteromedial part of the posterior wall of the maxillary sinus and post part of Inferior turbinate, posteriorly limited by nasopharyngeal mucosa. The patient underwent digital subtraction angiography (DSA) and preoperative embolization (Fig. 1c, d), followed by a coblation-assisted endoscopic excisional biopsy under general anesthesia (Fig. 1b). The postoperative period was uneventful. The HPE (histopathological examination) showed fragmented tissue partially lined by respiratory epithelium and subepithelial tissue depicting a poorly circumscribed lesion. The lesion was composed of monotonous oval to spindle cells with interspersed blood vessels many of which had thick hyalinised walls. The tumor cells had a syncytial pattern arranged around the blood vessels. The tumor cells had oval nuclei with dark chromatin. (Fig. 2a–c). A Reticulin stain highlighted the reticulin fibres restricted to around the blood vessels while being absent around individual tumor cells. Masson Trichrome stain highlighted the vasculature and lack of intercellular collagen. Immunohistochemistry with SMA (Clone 1A4, Pathnsitu) showed diffuse cytoplasmic immunoreactivity in the tumor cells. Immunohistochemistry with CD34 (Clone EP88, Pathnsitu) showed lack of staining in the tumor cells while the internal control (endothelial cells) showing membranous immunoreactivity (Fig. 3a–d). A diagnosis of Right Sinonasal GPC was made based on HPE findings. The patient was followed up for 12 months and remained symptom-free.

Fig. 2.

Photomicrographs of HPE. a Fragmented tissue partially lined by respiratory epithelium and subepithelial tissue shows a poorly circumscribed lesion. (Hematoxylin and Eosin stain, 100 × magnification), b The lesion is composed of monotonous oval to spindle cells with interspersed blood vessels many of which have thick hyalinised walls. (Hematoxylin and Eosin stain, 100 × magnification), c The tumor cells have a syncytial pattern arranged around the blood vessels. The tumor cells have oval nuclei with dark chromatin

Fig. 3.

Photomicrographs of Immunohistochemistry. a Reticulin stain highlights the reticulin fibres restricted to around the blood vessels, while being absent around individual tumor cells. (400 × magnification), b Masson Trichrome stain highlights the vasculature (400 × magnification) and lack of intercellular collagen (400 × magnification), c Immunohistochemistry with SMA (Clone 1A4, Pathnsitu) showing diffuse cytoplasmic immunoreactivity in the tumor cells (400× magnification), d: Immunohistochemistry with CD34 (Clone EP88, Pathnsitu) showing lack of staining in the tumor cells while the internal control (endothelial cells) showing membranous immunoreactivity

Case 2

A 41-year-old female patient with no known comorbidities was admitted with complaints of left-sided nasal obstruction, unilateral left-sided nasal discharge, and recurrent left-sided epistaxis for 8 months. On evaluation, the general examination and vitals were within normal limits. No abnormality was detected in the external nasal framework. Anterior rhinoscopy and DNE showed a soft tissue mass arising from the left middle turbinate extending into the nasopharynx posteriorly. Mass was non-tender, firm, and bleed to touch on palpation. The patient was further evaluated with routine blood work followed by CEMRI. The routine investigations were within normal limits, while CEMRI revealed a well-defined lobulated lesion 5.7 × 2.4 × 4.0 cm epicentered in the left nasal cavity and extending into the nasopharynx, superiorly extending into anterior ethmoid cells and abutting medial wall of orbit causing remodelling of turbinates, nasal septum and medial wall of maxillary antrum causing blockade of left frontal, maxillary and sphenoid drainage. (Fig. 4a–c). The patient underwent DSA and embolization of the feeding vessel (Internal maxillary artery). Then, the patient underwent an endoscopic excisional biopsy under general anesthesia, and the tissue was sent for HPE. The patient was followed up for 15 months and remained symptom-free.

Fig. 4.

Axial section of T1 (a, b) and T2 (c) weighted CEMRI images of Case 2 with the white arrow pointing to the lesion

Case 3

A 55-year-old male patient with no known comorbidities was admitted with complaints of right-sided recurrent epistaxis for 3 years and nasal obstruction for 1 year. On evaluation, the general examination and vitals were within normal limits. No abnormality was detected in the external nasal framework. A reddish vascular mass was seen filling the right nasal cavity on the anterior rhinoscopy. The diagnostic nasal endoscopy revealed a fleshy firm mass filling the right nasal cavity, arising from the nasal septum extending towards the sphenoethmoidal recess and reaching up to the nasopharynx through the posterior choana. The patient was further investigated with routine blood work which was within normal limits. contrast-enhanced computed tomography (CECT) revealed a well-differentiated enhancing soft tissue mass in the right nasal cavity originating from the septum involving the right nasal cavity reaching the cribriform plate superiorly, laterally not occluding the middle meatus and posteriorly up to the nasopharynx. The mass measured 42 mm × 12.6 mm × 35.7 mm in size with no areas of calcification. The patient underwent DSA and embolization of the feeding vessel (Internal maxillary artery). The patient underwent a coblation-assisted endoscopic excisional biopsy under general anesthesia (Fig. 5a, b) and the tissue was sent for histopathological examination. The postoperative period was uneventful. A diagnosis of Right Sinonasal GPC was made based on HPE findings. The patient was followed up for 8 months and remained symptom-free.

Fig. 5.

Preoperative (a) and Intraoperative (b) endoscopic images of the lesion of Case 3

Case 4

A 52-year-old male presented with recurrent left-sided epistaxis for 5 months. On evaluation, the general examination and vitals were within normal limits. No abnormality was detected in the external nasal framework. A small reddish vascular mass of approximately 1 cm × 1 cm was seen in the left nasal cavity arising from the anterior end of the inferior turbinate on the anterior rhinoscopy and DNE. Mass was firm, non-tender, and bled on touch. CECT revealed a 7 mm × 6 mm × 8 mm enhancing soft tissue mass arising from the anterior end of the left inferior turbinate. The patient underwent endoscopic excision biopsy under general anesthesia and the tissue was sent for histopathological examination. The postoperative period was uneventful. A diagnosis of Left Sinonasal GPC was made based on HPE findings. The patient was followed up for 6 months and remained symptom-free.

Case 5

A 7-year-old male presented with recurrent left-sided epistaxis for 2 months. On evaluation, the general examination and vitals were within normal limits. No abnormality was detected in the external nasal framework. A small reddish vascular mass of approximately 0.5 cm × 0.5 cm was seen in the left nasal cavity arising from the anterior end of the middle turbinate on the anterior rhinoscopy and DNE. Mass was firm, non-tender, and bled on touch. CECT revealed a 4 mm × 5 mm × 4 mm enhancing soft tissue mass arising from the anterior end of the left middle turbinate. The patient underwent endoscopic excision biopsy under general anesthesia and the tissue was sent for histopathological examination. The postoperative period was uneventful. A diagnosis of Left Sinonasal GPC was made based on HPE findings. The patient was followed up for 6 months and remained symptom-free.

Discussion

Glomangiopericytoma has low malignant potential (5–10%) and is mostly diagnosed in the 6th or 7th decade of age with slight female preponderance [6, 7]. This was in contrast with our experience as we did not find any female preponderance or common age group. Occasionally, GPC may present with characteristics similar to sinonasal polyps [6]. GPC are submucosally present and hence, can easily be differentiated from more common mucosal lesions such as squamous cell carcinoma or inverted papilloma [8]. Thus, the differential diagnosis for submucosal tumors should include capillary hemangiomas, nasopharyngeal angiofibromas, hemangiopericytomas, desmoid-type fibromatosis, and glomus tumors [8–10].

Avid homogenous enhancement along with erosive bone remodeling especially in sinonasal cavities is seen on CECT and CEMRI. CEMRI shows variable T2 appearance with hyperintensity and intermediate signal [8]. On HPE, GPC exhibits uniformly proliferated oval to short spindle-shaped cells under the epithelium. GPC shows cytoplasmic SMA, Vimentin, and nuclear beta catenin positivity on immunohistochemistry. However, it is negative for CD34, CD99, CD117, AE1/AE3, Bcl-2, S-100 protein, Factor VIIIR Ag, and STAT6 [9]. In our case series, all patients exhibited cytoplasmic SMA positivity and CD34 negativity, while one patient exhibited a high Ki-67 index (> 10%), which is a predictor of aggressive tumor behavior [9, 11]. Surgical resections using the endoscopic or combined approach form the mainstay of treatment for GPC. Our patients showed no recurrence to date; however, local recurrences may occur and mostly arise within 5 years of resection [12]. Rarely, chemoradiation can be used in cases with metastatic disease as adjuvant or palliative therapy.

The endoscopic approach provides clear visualization of the nasal cavity and paranasal sinuses, minimal trauma to healthy mucosa facilitating rapid healing, and reduced postoperative facial and nasal as well as paranasal deformities. We performed preoperative DSA and embolization of the feeding vessel in all cases which reduced the intraoperative bleeds and improved the time for surgery by providing a clear surgical field. The use of the coblation technique drastically reduced intraoperative bleeding and proved an extremely useful tool for the control of vascular tumors of nasal and sinonasal cavities. Postoperative follow-ups should be done every 3 to 6 months with DNE and/or MRI for early identification of any local recurrence [12].

The limitations of our study were the small number of cases, which can be explained by the rarity of GPC and data from a single tertiary healthcare centre. Another limitation was limited follow-up which was due to the constraint of resources and disproportionately large dependent clientele. Large-scale multi-institutional studies with prolonged follow-up are mandated.

Conclusion

We concluded that endoscopic surgical resection forms the mainstay of the treatment. The use of preoperative embolization and intraoperative use of the coblation technique improved the surgical field and reduced operative time and complications. Long-term follow-up is mandated for early detection and management of recurrence. Our case series supports and reaffirms the under-represented literature evaluation of imaging and treatment of sinonasal glomangiopericytoma.

Abbreviations

GPC

Glomangiopericytoma

CEMRI

Contrast-enhanced magnetic resonance imaging

CECT

Contrast-enhanced computed tomography

DNE

Diagnostic nasal endoscopy

DSA

Digital subtraction angiography

Funding

None.

Declarations

Conflict of interest

The authors declare that there is no conflict of interest.

Ethical standards

The authors assert that all the procedures contributing to the present work comply with the ethical standards of the relevant national and institutional guidelines on human experimentation and with the Helsinki Declaration of 1975, as revised in 2008.