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Nasal cavity, paranasal sinuses, nasopharynx

Other malignant tumors

Sinonasal glomangiopericytoma

Author: Jinping Lai, M.D., Ph.D.

Editorial Board Member: Bin Xu, M.D., Ph.D.

Deputy Editor-in-Chief: Kelly Magliocca, D.D.S., M.P.H.

Last author update: 20 July 2022

Last staff update: 20 July 2022

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PubMed Search: Sinonasal glomangiopericytoma

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Table of Contents

Cite this page: Lai J. Sinonasal glomangiopericytoma. PathologyOutlines.com website. https://www.pathologyoutlines.com/topic/nasalHPC.html. Accessed May 30th, 2023.

Definition / general

Uncommon soft tissue tumor of perivascular myoid differentiation with hemangiopericytoma-like vasculature

Essential features

Also called glomangiopericytoma, sinonasal type
Nasal cavity is usually affected, present with nasal obstruction, mass, epistaxis and sinusitis
CTNNB1 mutation with beta catenin oncogenic activation (beta catenin nuclear staining on IHC) (Head Neck Pathol 2019;13:298, Yale J Biol Med 2021;94:593)
Excellent long term outcome with surgery alone, recurrence rate ~27%

Terminology

Previous name: sinonasal type hemangiopericytoma
Glomus tumor
Intranasal myopericytoma

ICD coding

ICD-10: D38.5 - neoplasm of uncertain behavior of other respiratory organs

Epidemiology

Rare, comprising < 0.5% of sinonasal primary neoplasms (Thompson: Diagnostic Pathology - Head and Neck, 2nd Edition, 2016)
Broad age range (5 - 90 years); mean in seventh decade
F > M (1.2:1)
No difference in outcome based on gender after surgery

Sites

Nasal cavity is usually affected, turbinate and septum occasionally affected in isolation (Thompson: Diagnostic Pathology - Head and Neck, 2nd Edition, 2016)
Maxillary and ethmoid sinuses may also be affected in conjunction with nasal cavity
Bilateral tumor uncommon (~5%)

Pathophysiology

May arise from pericytes associated with vessels of nasal cavity
Mutational activation of beta catenin (Head Neck Pathol 2019;13:298, Yale J Biol Med 2021;94:593)
Associated with cyclin D1 overexpression (Pathol Res Pract 2019;215:983)

Etiology

CTNNB1 mutations

Clinical features

Nasal obstruction, mass, polyps, difficulty breathing
Sinusitis, discharge, change in smell
Nasal bleeding (J Pak Med Assoc 2020;70:2469)
Headache
Rare association with oncogenic osteomalacia (BMC Endocr Disord 2022;22:31)

Diagnosis

Clinical symptoms and polypoid mass identified in the nasal cavity
CT and MRI showing a polypoid mass of nasal cavity (paranasal sinus) accompanied with bone erosion or sclerosis
Biopsy showing soft tissue with perivascular myoid differentiation, positive for beta catenin nuclear staining, cyclin D1 and SMA, negative for vascular markers in the tumor cells (Pathol Res Pract 2019;215:983)

Laboratory

In the majority of cases, lab tests do not help
In rare cases, associated with osteomalacia: serum hypophosphatemia and elevated alkaline phosphatase (BMC Endocr Disord 2022;22:31)

Radiology description

CT and MRI showing a mass of nasal cavity (paranasal sinus), could be lobulated

Radiology images

Contributed by Jinping Lai, M.D., Ph.D.

Right nasal mass (MRI axial)

Right nasal mass (MRI coronal)

Right nasal mass (MRI sagittal)

Prognostic factors

Excellent prognosis after surgery (5 year survival ~90%)
Recurrence rate ~27%, long term clinical follow up advocated as recurrences may develop late (Thompson: Diagnostic Pathology - Head and Neck, 2nd Edition, 2016)
Recurrences are associated with long duration of the symptoms, bone invasion and profound nuclear pleomorphism

Case reports

42 year old woman with osteomalacia caused by sinonasal glomangiopericytoma and coexisting parathyroid adenoma (BMC Endocr Disord 2022;22:31)
57 year old man with sinonasal FUS::ERG rearranged Ewing sarcoma mimicking glomangiopericytoma (Case Rep Oncol 2020;13:1393)
60 year old man with new onset, right arm and leg weakness / numbness, was found to have a left ethmoid sinus glomangiopericytoma with extension in the olfactory fossa (Yale J Biol Med 2021;94:593)
70 year old man with nasal blockage and epistaxis underwent endoscopic sinus surgery (J Pak Med Assoc 2020;70:2469)
74 year old man with a reddish nasal mass with CTNNB1 p.S37C mutation (Head Neck Pathol 2019;13:298)

Treatment

Surgery (polypectomy or wide surgical resection) is the treatment of choice (J Pak Med Assoc 2020;70:2469)
Radiation could be used for nonsurgical candidates

Clinical images

Contributed by Jinping Lai, M.D., Ph.D.

Nasal endoscopy

Gross description

Polypoid mass, mean 3 cm, beefy red to grayish pink with hemorrhage
Soft, edematous and fleshy cut surface

Gross images

Contributed by Jinping Lai, M.D., Ph.D.

Cut surface

Frozen section description

Frozen sections of the tumor show subepithelial cytologically bland spindle and round cell proliferation separated from the surface respiratory epithelium, hemorrhagic stromal, perivascular hyalinization and staghorn vessels

Frozen section images

Contributed by Jinping Lai, M.D., Ph.D.

Subepithelial grenz zone, hemorrhagic stroma

Perivascular proliferation and hyalinization

Perivascular proliferation / hyalinization

Perivascular proliferation and staghorn vessels

Microscopic (histologic) description

Surface respiratory epithelium or metaplastic squamous epithelium is intact
Subepithelial proliferation separated from surface (grenz zone)
Diffuse growth with fascicular, solid or focally whorled pattern of spindled or round / oval tumor cells that arrange themselves around prominent, small, thin walled submucosal blood vessels
Cells have variable cytoplasm and nuclei, indistinct cell borders and occasionally are multinucleated
Minimal atypia, no necrosis, no / rare mitotic activity
Vessels are prominent with staghorn appearance and perivascular hyalinization
Inflammatory cells including mast cells and eosinophils are often present

Microscopic (histologic) images

Contributed by Jinping Lai, M.D., Ph.D.

Subepithelial grenz zone and whorled features

Perivascular hyalinization and staghorn vessels

No mitosis, eosinophils

Beta catenin nuclear staining

Positive cyclin D1

Positive CD99

Positive SMA

Low Ki67 index

Cytology description

Smears show spindled, epithelioid or round cells with indistinct cell borders

Positive stains

Beta catenin: nearly all tumor cells with nuclear and cytoplasmic staining (Pathol Res Pract 2019;215:983, Yale J Biol Med 2021;94:593)
Cyclin D1, CD99 (Pathol Res Pract 2019;215:983)
Vimentin (98%), smooth muscle actin (92%), muscle specific actin (77%), factor XIIIa (78%), laminin (52%)
D2-40 (Virchows Arch 2006;448:459)

Negative stains

ERG, CD31, CD34 (usually), factor VIII, keratin

Electron microscopy description

Basal lamina surrounds individual cells, tapered cytoplasmic extensions, orderly bundles of filaments (Thompson: Diagnostic Pathology - Head and Neck, 2nd Edition, 2016)

Molecular / cytogenetics description

CTNNB1 mutation: PCR and gene sequencing show single nucleotide substitutions with exon 3 codons of the CTNNB1 glycogen serine kinase 3 beta phosphorylation region

Sample pathology report

Right nasal cavity mass, biopsy:
- Nasal glomangiopericytoma (see comment)
- Comment: Sections of the nasal mass biopsy show cytologically bland spindle cell proliferation with scattered staghorn vessels and perivascular hyalinization. The tumor cell proliferation is separated from the overlying respiratory epithelium. No mitotic figures or tumor necrosis is identified. The tumor cells are positive for beta catenin (nuclear staining), cyclin D1, CD99 and SMA, negative for desmin, CD31, ERG, CD34 and AE1 / AE3. The histologic features and immunoprofile support the diagnosis.

Differential diagnosis

Glomus tumor:
- Also a pericytic tumor with similar staining but extremely rare in the sinonasal region
- Composed of compact epithelioid cells
- Positive for SMA but negative for beta catenin nuclear staining
Lobular capillary hemangioma:
- Lobular pattern at low power
- Has spindled fibroblasts and prominent vessels but overall granulation tissue-like appearance with only small capillaries
- Positive for CD34 and CD31, negative for beta catenin nuclear staining
Solitary fibrous tumor:
- Similar vascular pattern but tumor has patternless pattern, with ropy keloidal collagen and thin walled vascular spaces
- Positive for CD34 and STAT6, negative for beta catenin nuclear staining
Ewing or Ewing-like sarcoma:
- Tumor cells have clear cytoplasm and similar histology with positive CD99 immunoreactivity
- Negative beta catenin nuclear staining and positive for EWSR1 rearrangement (Case Rep Oncol 2020;13:1393)

Additional references

Head Neck Pathol 2020;14:459

Board review style question #1

Regarding sinonasal glomangiopericytoma, which of the following statements is true?

Bilateral tumor is seen in 10% of patients
It is much more common in male patients
Majority of the tumors have a poor prognosis
Maxillary sinus is the most affected
Nasal cavity is usually affected

Board review style answer #1

E. Nasal cavity is usually affected; maxillary and ethmoid sinuses may also be affected in conjunction with nasal cavity. The female to male ratio is 1.2 to 1. Patients with bilateral tumor are uncommon (~5%). The majority of the tumors have a good prognosis.

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Reference: Sinonasal glomangiopericytoma

Board review style question #2

A 50 year old woman presented with right nasal obstruction and occasional nose bleeding. Physical examination showed a red to grayish polypoid soft mass in her right nasal cavity and a biopsy was performed. A histology photo is shown above. Which of the following immunostains should be positive for the diagnosis of nasal glomangiopericytoma?

Beta catenin nuclear staining
CD31
CD34
S100 nuclear staining
STAT6 nuclear staining

Board review style answer #2

A. Beta catenin nuclear staining should be positive in the tumor cells, as sinonasal glomangiopericytoma is related to mutational beta catenin activation. Positive CD34 and STAT6 are helpful for diagnosis of solid fibrous tumor. Positive S100 and muscular markers are helpful for diagnosis of biphenotypic sinonasal sarcoma.

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Reference: Sinonasal glomangiopericytoma

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