Endoscopic resection of invasive glomangiopericytoma following preoperative embolisation

Abstract

We present a case of a 60-year-old woman with a 2-year history of nasal obstruction and unilateral recurrent epistaxis. Anterior rhinoscopy identified a unilateral hypervascular lobular mass occupying the entire left nasal cavity. Imaging studies documented an extensive soft tissue density lesion in the left nasal cavity with complete infiltration of the anterior ethmoid, limited indentation of the medial wall of the orbit, bone demineralisation of the cribriform plate and involvement of the anterior cranial fossa. The diagnosis of locally invasive glomangiopericytoma was obtained preoperatively through biopsy of the lesion, which resulted in a self-limited epistaxis that was easily controlled by nasal packing, confirming the vascular nature of the lesion. We discuss the diagnostic work up, imaging and surgical approach of this tumour, and review the recent literature on endoscopic treatment of these lesions.

Background

Glomangiopericytoma (GPC) is a rare highly vascular mesenchymal tumour with myoid phenotype (smooth muscle actin (SMA)-positive) representing less than 0.5% of all nasal lesions.^1–6 Formerly known as sinonasal type haemangiopericytoma (HPC), its nomenclature was updated by the WHO in 2005, taking into account the nasal prevalence of the tumour, its perivascular origin from modified myoepithelial cells, histology and immunohistochemistry resemblance to glomus tumours.^{2 7} The GPC is classified as a soft tissue tumour with low malignant potential, and is clinically and pathologically different from the more aggressive, extranasal soft tissue HPC/solitary fibrous tumour. Typically exhibiting a benign local behaviour, GPC may express a locally aggressive progression and the risk of metastasis.^{2 8} Treatment is complete endoscopic endonasal resection of the tumour with free surgical margins, which decreases the risk of local recurrence.^7–12

Case presentation

A 60-year-old woman presented with a 2-year history of progressive symptoms of unilateral nasal obstruction and recurrent epistaxis formerly treated with nasal packing. She also had complaints of headaches, and persistent forehead and maxillary facial fullness. On examination, anterior rhinoscopy identified a unilateral lobular hypervascular mass occupying the entire left nasal cavity, not sensitive, but easily friable with minimal manipulation with multiple adhesion to the nasal septum and floor of the nasal cavity. The skin of the nose and face had no abnormalities and there were no palpable cervical lymph nodes.

Investigations

Imaging tests, CT scan of paranasal sinuses, MRI and angio-MRI documented the presence of an extensive vascular soft tissue density lesion of the left nasal cavity, with infiltration of the ethmoid and without involvement of pterygomaxillary fossa; with limited indentation of the medial wall of the left orbit, bone demineralisation of the left cribriform plate and intradural extension with limited involvement of the anterior cranial fossa (figure 1).

Figure 1.

(A) MRI T2 fast spin echo (FSE). Pediculated lesion in the anterior limit of the cribriform plate (*). (B) Coronal MRI fat suppression (FS) showing suspected invasion of papyraceous and contact with medial rectus muscle of the left eye (*). (C) Parasagittal MRI FSE T2, showing of the involvement of in the anterior frontonasal area and anterior cranial fossa (*).

A specimen biopsy was performed causing a self-limited epistaxis, which was easily controlled by nasal packing. On histological examination, the tumour was initially found to be consistent with angiofibroma, showing an epithelial lining, and vascular architecture with a fusiform cell background stroma with no atypia, no mitosis and no necrosis. Immunohistochemical staining revealed that the tumour was positive SMA and vimentin, and lacked expression with desmin, CD34 and S-100, establishing the conclusive diagnosis of sinonasal GPC (figure 2).

Figure 2.

Histological features of GPC. The well-defined tumour with respiratory epithelial lining and spindle to oval shaped cells interspersed with a rich vasculature with a stag horn configuration (HE, X100). (A) Vimentin positive reaction on immunohistochemical staining.

Differential diagnosis

GPCs are usually misinterpreted as vascular angiofibroma-like lesions, although the differential diagnosis must include nasosinusal polyposis, lobular capillary haemangioma, leiomyoma, leiomyosarcoma, synovial sarcoma, glomus tumours, inverted papillomas and solitary fibrous tumours.^{1 2 5–7 10 13}

Treatment

Due to the vascular nature of the lesion, preoperative embolisation was performed, which removed 90% of the vascularisation of the tumour originating from the infraorbital artery.

Complete piecemeal resection of the tumour was achieved through an endonasal endoscopic approach using a microdebrider (Xomed, Jacksonville, Florida, USA) and radiofrequency (Coblation—EVac 70 Xtra Plasma Wand). The papyracea was intact without intraorbital involvement. The tumour was found to arise from the anterior ethmoidal roof. At this point, the mass was invading the cribriform plate and extending intracranially. To access the intracranial component of the tumour, we performed an endoscopic transethmoidal craniectomy, which allowed the identification of normal dura and complete tumour dissection from the normal brain tissue (figures 3–7). During intracranial dissection, the surgeon must be aware of peritumoural and intratumoural vessels that may contribute to the tumour vascular supply (figure 7).

Figure 3.

Transnasal endoscopic aspect of the left nasal cavity depicting non-pulsatile tumour with adhesions to the septum and floor as result of prior nasal packing for epistaxis. (A) Coblation-assisted ablation to reduce the size and devascularise the tumour. IT: inferior turbinate; NS: nasal septum; T: tumour.

Figure 4.

Transethmoidal endoscopic aspect of the left nasal cavity with identification of the skull base defect and intracranial involvement. EF: ethmoidal fovea.

Figure 5.

Detail of the drilling of the internal limit of the skull base defect (A) identification of the normal dura mater (D) at the periphery of the drilling. EF, ethmoidal fovea; FS: fat suppression.

Figure 6.

Transethmoidal endoscopic aspect of the left nasal cavity with identification of the skull base defect after partial removal of the tumour. Control of bleeding from the anterior ethmoidal artery (AEA). (A) Detail on coagulation of the AEA with radiofrequency. LP: lamina papyracea; EF, ethmoidal fovea.

Figure 7.

Transethmoidal endoscopic aspect of the left nasal cavity with debridement of the intracranial component of the tumour. (A) Detail of the controlled excision of the dura mater within the limits of the tumour. EF, ethmoidal fovea.

The reconstruction of the resulting skull base defect was performed using a multilayer technique with placement of DuraForm inlay, autologous fascia lata and septal mucosa overlay. The nasal fossa was packed with Merocel, which was removed 24 h after surgery. The blood loss during surgery was minimal and easily controllable without the need for transfusion during the intraoperatively as well as postoperatively period.

Outcome and follow-up

A complete resection with surgical free margins was achieved by endoscopic endonasal surgery. The final histopathology report confirmed the diagnosis of GPC without signs of malignant transformation. No complications, no cerebrospinal fluid leak and no locoregional recurrence were observed during the postoperative follow-up.

Discussion

The GPC is a rare mesenchymal tumour revealing a marked perivascular growth. These lesions predominantly involve the nasal cavity and present rather benign local behaviour, with rare metastasis (3%) despite a high recurrence rate.^{2 5 7 9 13 14} Extension into the paranasal sinuses is rare and extrasinusal involvement is extremely rare, although bone erosion and sclerosis may occur.^{2 9 14} This finding was described in one case at the internal wall of the orbit by Jung et al;¹⁵ in the skull base in one case by Sun et al;¹⁶ and in six cases involving the cribriform plate by Tessema et al.¹¹ To our knowledge, there are no reported cases in the literature describing bone destruction with intracranial invasion associated with this type of tumour. The size and the invasive behaviour of the tumour may represent malignant transformation, which, although infrequent, is described in tumours showing nuclear pleomorphism, high proliferative index and necrosis.^{2 6 7 9 12 14}

No specific age and no gender predilection has been associated with this tumour, although a slight predominance in females and occurrence during the seventh decade of life have been previously reported.^{1 2 7 8 13 14} The aetiology is unknown, though some authors suggest a relationship with trauma, hypertension, pregnancy or chronic use of corticosteroids.^{9 17}

Patients often complain of unilateral nasal obstruction, recurrent epistaxis and history of previous nasal packing, which may lead to mucosal adhesions (figure 3) between tumour and adjacent normal tissue areas.^{7 10 11 13 14} The patient reported here had a 2-year history of nasal obstruction, frequent epistaxis and facial fullness. She had no predisposing factor except hypertension.

These tumours tend to present a vascular, polypoid, lobular and oedematous aspect, and are easily friable. Due to their vascular nature, preoperative biopsy of these lesions tends to be avoided, because it often results in profuse bleeding.^{2 10 14 16}

The diagnosis is based on histopathology. GPCs, although sharing the structural branched vascular pattern of the more aggressive extranasal HPCs, are histologically different, as they present epithelioid cells with a characteristic perivascular hyalinisation. They are submucosal non-encapsulated tumours with highly diffuse cellular stroma interspersed with large branching vessels with a distinctive stag horn configuration (figure 2).^{7 9 13 14 16 18} GPCs express limited or absent mitotic activity and lack atypia, necrosis and haemorrhage, usually seen in HPCs.^{5 7 14} Due to their histological properties overlapping with other vascular tumours, it is of utmost importance to perform immunohistochemical staining to achieve confirmation of the diagnosis. The GPC typically shows a diffuse reactivity with SMA, vimentin and factor XIIIa and lacks expression with desmin, CD34, Bcl2, FVIII-R Ag, CD99, CD117 and S-100.^{2 5 9 13 14 18}

If the final diagnosis of GPC is established before surgery, angiography with selective embolisation is advantageous in surgical planning, to avoid the risk of blood loss during the surgery.^{6 8 12 19 20}

The treatment for GPC is local excision while assuring negative margins, which ensures an excellent 5-year survival rate of over 90%.^{1 2 10 12 16} Preoperative embolisation and intraoperative endoscopic-assisted resection using radiofrequency and a microdebrider have advantages in devascularisation and bleeding control, and allow safe and well-controlled endoscopic tumour ablation.

Local recurrence is variable and can occur in up to 71% of cases after incomplete excision, and in up to 22% of cases with complete excision, implying life-long postoperative surveillance follow-up.^{1 2 6–11 14 16 18}

Learning points.

• Glomangiopericytoma (GPC) is a vascular soft tissue tumour, specific to sinonasal passages, with low malignant potential and biologically distinct from extra-nasal haemangiopericytomas.

• Its aetiology is unknown but may involve past trauma, hypertension, pregnancy and the use of corticosteroids.

• Malignant GPC is uncommon and usually presents with large size, bone invasion, nuclear pleomorphism and increased mitotic activity or necrosis.

• Preoperative embolisation has an advantage in devascularisation and bleeding control, and allows safe and complete endoscopic tumour ablation.

• Due to a high risk of recurrence, life-long postoperative surveillance follow-up is advised.