Sino-nasal hemangiopericytoma: a case series and systematic literature review

Hemangiopericytoma is a rare tumor of the sino-nasal tract. Its clinical behavior is controversial. Whereas some describe an indolent course, others consider it to be an aggressive lesion with a tendency toward rapid local recurrence. Here, we describe our experience in the management of sino-nasal hemangiopericytoma (SN-HPC), comparing our experience with the current literature, and evaluating signs and tools to improve diagnosis and treatment.

All cases of SN-HPC between 2010 and 2020 were extracted and reviewed from our institutional electronic medical records. SN-HPC cases from PubMed and EMBASE between 2010 and 2020 were analyzed in a systematic literature review using the preferred reporting items for systematic review and meta-analysis (PRISMA) guidelines. Data regarding demographics, presentation, diagnosis, treatment, and outcome were collected.

We identified four cases of SN-HPC in the nasal cavity in our institution and an additional 53 cases in previous reports. The mean age at the time of diagnosis was 59 years, with a 1.2:1 male to female ratio. SN-HPC mostly appears unilaterally, arising in the ethmoid sinus (42.1%). The most common presenting symptoms were epistaxis (47.3) and nasal obstruction (47.3%). Both computed tomography (CT) and magnetic resonance imaging (MRI) were required for diagnosis and for tailoring the treatment plan. Endoscopic surgical excision was used in 85.9% of the patients, and in 15.7%, an additional preoperative embolization was performed, which was associated with septal necrosis in one patient (2.6%). The recurrence rate was 7%.

Although previous reports attribute an aggressive tumoral behavior to SN-HPC, our experience and the literature review support a more indolent course with low recurrence rates following complete endoscopic resection. Preoperative embolization can be useful in certain cases, but due to potential complications, it should not be routinely indicated.