What caused a 65-year-old man with poorly controlled hypertension to develop an obstruction in his left nasal cavity 2 years ago? That’s what Chia-Hsiang Fu, MD, PhD, of Chang Gung Memorial Hospital and Chang Gung University in Guishan, Taiwan, and colleagues needed to determine when the man presented to their outpatient department.
The patient’s history did not include any trauma or surgery that might account for the obstruction. His systolic blood pressure ranged from 130 to 160 mm Hg, despite taking oral treatment with amlodipine 5 mg/valsartan 80 mg and indapamide 1.5 mg once a day, the team reported in JAMA Otolaryngology–Head & Neck Surgery.
On physical examination, Fu and co-authors noted a large tumor “originating from the olfactory groove in the left nasal cavity, with feeding vessels pushing the middle turbinate outward.” They performed an MRI that showed a strongly enhancing mass with well-defined margins located in the left nasal cavity. Surgery performed to further assess the mass revealed that “the tumor abutted the superior end of the nasal septum near the cribriform plate.”
The tumor was excised with a minimum margin of 5 mm, with the widest margin along the perichondrium of the nasal septum. Surgeons cauterized the tumor’s feeding vessels during the surgery.
On macroscopic examination, the mass was whitish to pink-tan in color and 4.8 × 3.0 × 1.8 cm in size. Histopathology findings included “sheetlike proliferation of monomorphic ovoid to spindle tumor cells containing ectatic vascular spaces,” noted Fu and colleagues. Immunohistochemical staining results were positive for smooth-muscle actin, cyclin D1, and nuclear expression of β-catenin, and negative for epithelial membrane antigen, cytokeratin, and CD34.
The team considered several possible diagnoses, including lobular capillary hemangioma, inverted papilloma, and solitary fibrous tumor, before agreeing on the diagnosis of glomangiopericytoma (GPC).
Fu and colleagues explained that this relatively rare mesenchymal neoplasm — which accounts for less than 0.5% of all sinonasal tumors — occurs submucosally in the nasal cavity and paranasal sinus. Its smooth surface and resemblance to an inflammatory nasal polyp can delay endoscopic diagnosis.
Initially, when it was described in 1924, sinonasal GPC was classified as a “hemangiopericytoma-like neoplasm with perivascular myoid differentiation,” and was considered to pose little risk of malignancy, the group wrote.
Due to its typically benign clinical course and nonspecific presentation, often with nasal obstruction and epistaxis, GPC tends to be quite large in size by the time it is diagnosed, the authors noted. GPC often occurs in patients in their 50s and 60s, and is slightly more common in women than men.
Fu and co-authors said that they diagnosed this patient based on pathology findings of “a diffuse, subepithelial proliferation of bland, uniform, closely packed spindled cells surrounding capillaries with perivascular hyalinization,” which they noted is why it was initially categorized as a hemangiopericytoma.
They clarified that presentation of GPC differs from that of inflammatory nasal polyps, which usually occur bilaterally and rarely have feeding vessels.
Diagnosis of GPC is confirmed by “positive immunohistochemical stain for smooth-muscle actin, cyclin D1, and nuclear expression of β-catenin,” they wrote.
GPC and lobular capillary hemangiomas occur submucosally and present as polypoid lesions with smooth surfaces, and both are characterized by a solid and soft texture, which can make differentiation challenging. Histologic examination can help distinguish between lobular capillary hemangioma, which presents as lobular vascular proliferation of capillary-sized vessels, and inverted papilloma, which is characterized by a “mucosal origin with cerebriform, lobulated, and firm appearance,” the authors explained.
Inverted papilloma affects two to three men for every one woman, and usually develops in the maxillary sinus and lateral nasal wall. Inverted papilloma can be distinguished from GPC on histologic findings of “squamous epithelial cells within the sinonasal tract,” the authors noted.
Finally, although solitary fibrous tumors resemble GPC histologically, these tumors are immunoreactive for CD34.
While the cause of GPC remains unknown, hypervascularity associated with factors including hypertension, trauma, pregnancy, and corticosteroid use has been reported. In this patient’s case, the authors suggested that a long history of poorly controlled hypertension may have played a role.
“Complete surgical resection with adequate margins remains the primary management strategy in patients with GPC,” they wrote. Reported recurrences are relatively rare — mostly attributed to incomplete resection — and occur within 1 year of surgery.
Angiographic embolization of GPC feeding vessels prior to surgery has been proposed as a means of reducing the volume of bleeding during surgery, they added. Radiotherapy may be used in addition to surgery to provide local control of the disease.
Chemotherapy has also been added to the treatment regimen for patients with metastatic disease, the incidence of which has been reported to range from 5% to 10%, Fu and co-authors noted. “It is noteworthy that this observation predates the formal classification of GPC as a distinct clinical entity in 2005, thus potentially introducing a risk of diagnostic misclassification. Because of its rarity, no formal guidelines have been published for sinonasal GPC, to our knowledge.”
The team reported that over 3 years of regular follow-up after surgery, this patient had no tumor recurrence or decreased olfaction.
Fu and co-authors reported no conflicts of interest.
JAMA Otolaryngology–Head & Neck Surgery
Source Reference: Ho Y-T, et al “Patient with unilateral nasal obstruction and a nasal mass” JAMA Otolaryngol Head Neck Surg 2023; DOI: 10.1001/jamaoto.2023.3817.