Sinonasal Hemangiopericytoma



Sinonasal Hemangiopericytoma


Jonathan B. McHugh, MD









Coronal CT scan shows a sinonasal hemangiopericytoma (HPC) filling the right nasal cavity image with bone erosion and extension into the adjacent ethmoid sinus image. The right maxillary sinus has associated sinusitis image.






Low-power image shows sinonasal HPC with intact surface image and a subepithelial mesenchymal proliferation with prominent vascularity image, extravasated blood image, and effacing subepithelial structures.


TERMINOLOGY


Abbreviations



  • Sinonasal hemangiopericytoma (HPC)


Synonyms



  • Glomangiopericytoma


  • Hemangiopericytoma-like tumor of sinonasal cavity


  • Sinonasal glomus tumor


  • Hemangiopericytoma


Definitions



  • Unique sinonasal mesenchymal neoplasm demonstrating perivascular myoid phenotype


ETIOLOGY/PATHOGENESIS


Histogenesis



  • Proposed cell of origin is unidentified modified perivascular glomus-like myoid cell


CLINICAL ISSUES


Epidemiology



  • Incidence



    • Accounts for < 0.5% of sinonasal neoplasms


  • Age



    • All age groups affected (range: 5-86 years)



      • 75% of cases occur in 6th-8th decades


      • Peak incidence in 6th-7th decades


  • Gender



    • Slight female predilection


Site



  • Most frequently involve nasal cavity



    • Often have concomitant paranasal sinus involvement


    • Right and left side equally effected


  • Rarely arise primarily in paranasal sinuses


Presentation



  • Unilateral polypoid intranasal mass


  • Nasal obstruction


  • Epistaxis


  • Congestion &/or difficulty breathing


  • Sinusitis


Treatment



  • Surgical approaches



    • Complete surgical excision is treatment of choice


  • Adjuvant therapy



    • Radiation therapy of unproven value


Prognosis



  • Excellent overall 5-year survival (> 90%)


  • Approximately 1/3 will recur/persist (range: 18-44%)



    • Recurrences can occur after many years



      • Mean interval to 1st recurrence is around 6.5 years (range: 1-17.5 years)


      • Long-term follow-up warranted


  • Malignant degeneration uncommon



    • Histologic features of malignant sinonasal hemangiopericytoma similar to soft tissue “hemangiopericytoma”



      • Large size (> 5 cm)


      • Marked pleomorphism


      • Necrosis


      • Bone invasion


      • > 4 mitoses/10 high-power fields


      • Ki-67 proliferation index > 10%


IMAGE FINDINGS


Radiographic Findings



  • Opacification filling nasal cavity ± adjacent sinuses


  • Bone erosion or sclerosis can be seen


  • Concomitant sinusitis not uncommon



MACROSCOPIC FEATURES


General Features



  • Often removed piecemeal


  • If resected intact, appears as polypoid solid mass with tan hemorrhagic cut surface


  • Surface mucosa typically intact


Size



  • Mean size is 3.5 cm (range: 1.5-8 cm)


MICROSCOPIC PATHOLOGY


Histologic Features



  • Subepithelial, well-demarcated but unencapsulated, cellular mesenchymal proliferation



    • Surface (Schneiderian) mucosa usually intact but may be eroded or show squamous metaplasia


    • Usually efface but may surround submucosal minor salivary glands


  • Composed of uniform, closely packed, round to spindle-shaped cells intimately associated with vascular component


  • Vascular channels are variable in size ranging from capillaries to patulous “staghorn” vessels



    • Prominent perivascular hyalinization is characteristic and seen in up to 90% of cases


  • Stroma is typically scant but may be myxoid in areas


  • Stromal edema may result in hypocellular zones with residual smaller cellular lobules


  • Most tumors have solid or fascicular growth, but mixed growth patterns are common



    • Whorled growth patterns can be seen in up to 10% of cases


  • Extravasated red blood cells are usually present


  • Inflammatory cells, including eosinophils, mast cells, lymphocytes, and plasma cells, are invariably present

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Jul 9, 2016 | Posted by in PATHOLOGY & LABORATORY MEDICINE | Comments Off on Sinonasal Hemangiopericytoma

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