Nasal cavity, paranasal sinuses, nasopharynx

Other malignant tumors

Sinonasal glomangiopericytoma


Editorial Board Member: Bin Xu, M.D., Ph.D.
Deputy Editor-in-Chief: Kelly Magliocca, D.D.S., M.P.H.
Jinping Lai, M.D., Ph.D.

Last author update: 20 July 2022
Last staff update: 20 July 2022

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PubMed Search: Sinonasal glomangiopericytoma

Jinping Lai, M.D., Ph.D.
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Cite this page: Lai J. Sinonasal glomangiopericytoma. PathologyOutlines.com website. https://www.pathologyoutlines.com/topic/nasalHPC.html. Accessed December 6th, 2022.
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
Sites
Pathophysiology
Etiology
  • CTNNB1 mutations
Clinical features
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 axial)

Right nasal mass (MRI coronal)

Right nasal mass (MRI coronal)

Right nasal mass (MRI sagittal)

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
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

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

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

Subepithelial grenz zone, hemorrhagic stroma

Perivascular proliferation and hyalinization

Perivascular proliferation / hyalinization

Perivascular proliferation and staghorn vessels

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

Subepithelial grenz zone and whorled features

Perivascular hyalinization and staghorn vessels

Perivascular hyalinization and staghorn vessels

No mitosis and eosinophils

No mitosis, eosinophils

Beta catenin nuclear staining

Beta catenin nuclear staining


Positive cyclin D1

Positive cyclin D1

Positive CD99

Positive CD99

Positive SMA

Positive SMA

Low Ki67 index

Low Ki67 index

Cytology description
  • Smears show spindled, epithelioid or round cells with indistinct cell borders
Positive stains
Negative stains
Electron microscopy description
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
Additional references
Board review style question #1
Regarding sinonasal glomangiopericytoma, which of the following statements is true?

  1. Bilateral tumor is seen in 10% of patients
  2. It is much more common in male patients
  3. Majority of the tumors have a poor prognosis
  4. Maxillary sinus is the most affected
  5. 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.

Comment Here

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?

  1. Beta catenin nuclear staining
  2. CD31
  3. CD34
  4. S100 nuclear staining
  5. 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.

Comment Here

Reference: Sinonasal glomangiopericytoma
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