Sinonasal glomangiopericytoma

Last revised by Joachim Feger on 22 Jun 2024

Glomangiopericytomas (GPC), also known as sinonasal-type hemangiopericytomas, are rare vascular tumors that typically arise in the nasal cavity and paranasal sinuses characterized by a perivascular myoid pattern 1.

Glomangiopericytomas (GPCs) are often referred to as sinonasal hemangiopericytomas. However, they are distinct from conventional hemangiopericytomas in their location, biological behavior, and histological features 2.

Glomangiopericytomas are rare, accounting for less than 0.5% of sinonasal tumors. They primarily affect older adults and have a slight female predominance 1.

Patients typically present with symptoms such as nasal obstruction, epistaxis (nosebleeds), and facial pain. Less commonly, they may experience anosmia, headaches, and proptosis if the tumor extends to adjacent structures 3.

Glomangiopericytomas are characterized by a proliferation of spindle-shaped cells around blood vessels.

Glomangiopericytomas primarily occur in the nasal cavity and paranasal sinuses. Rarely, they can be found in other locations such as the spinal cord or the pterygomandibular space 4,5.

Macroscopically, glomangiopericytomas appear as reddish, polypoidal masses. They are often friable and prone to bleeding upon manipulation 6.

Microscopically, glomangiopericytomas consist of closely packed spindle cells arranged around a distinctive vascular network. The cells are uniform, with bland nuclei, and are surrounded by thin-walled, branching vessels, often described as having a "staghorn" appearance 3.

Immunohistochemistry is positive for α-smooth muscle actin, indicating myoid differentiation 1.

They can show an unspecific soft tissue mass in the nasal cavity.

CT is useful for evaluating the extent of the tumor and any potential bone involvement. Glomangiopericytomas typically appear as well-defined masses with uniform enhancement after contrast administration 1.

Provides superior soft tissue contrast and is useful for defining the tumor's relationship with adjacent structures 5.

  • T1: isointense to muscle

  • T2: heterogeneous high signal intensity

  • T1 C+ (Gd): vivid enhancement

The primary treatment for glomangiopericytomas consists of complete surgical resection. Endoscopic surgery is preferred due to its minimally invasive nature and effectiveness in complete tumor removal. The prognosis is generally good, with a low rate of malignancy and high survival rates if the tumor is fully excised. However, recurrence rates can be as high as 30%, necessitating long-term follow-up 7.

General imaging differential considerations include 2,8:

  • solitary fibrous tumor: the heterogeneity and presence of cystic or necrotic areas can help distinguish SFTs from glomangiopericytomas, which tend to have more uniform enhancement

  • leiomyoma: the homogenous low signal on T2-weighted images can help differentiate leiomyomas from glomangiopericytomas, which generally show a more heterogeneous signal

  • angiofibroma: the presence of flow voids and intense enhancement on MRI can help distinguish angiofibromas from glomangiopericytomas, which typically do not exhibit flow voids

  • lobular capillary hemangioma: the small size and superficial location, combined with intense homogeneous enhancement, can help differentiate these from glomangiopericytomas, which are typically larger and deeper

  • schwannoma: the presence of cystic areas and the "target sign" (central low signal with peripheral high signal on T2-weighted images) can help differentiate schwannomas from glomangiopericytomas

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