Nasal cavity, paranasal sinuses, nasopharynx
Other tumors
Hemangiopericytoma - sinonasal type

Author: Nat Pernick, M.D. (see Authors page)

Revised: 23 May 2018, last major update November 2004

Copyright: (c) 2004-2018, PathologyOutlines.com, Inc.

PubMed Search: Hemangiopericytoma sinonasal type

Cite this page: Pernick, N. Hemangiopericytoma - sinonasal type. PathologyOutlines.com website. http://www.pathologyoutlines.com/topic/nasalHPC.html. Accessed October 17th, 2018.
Definition / general
  • Uncommon tumor of pericytic myoid differentiation (unlike soft tissue hemangiopericytoma)
  • WHO calls it glomangiopericytoma; also called myopericytoma (J Laryngol Otol 2007;121:786)
  • Mean age 63 years, usually ages 40+ but range of 5 - 86 years; slight female predominance
  • Usually presents with airway obstruction or epistaxis in nasal cavity or paranasal sinus
  • Recurs locally (18%), no metastases; death due to disease is rare
Case reports
Treatment
  • Excision plus followup
Gross description
  • Resembles allergic polyp
  • Unilateral, unencapsulated, mean 3 cm, red to gray-pink, soft, edematous, fleshy to friable, often hemorrhagic
Microscopic (histologic) description
  • 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, occasionally are multinucleated
  • Minimal atypia, no necrosis, no / rare mitotic activity
  • Vessels are prominent with staghorn appearance and perivascular hyalinization
  • Often mast cells and eosinophils
  • Surface epithelium is intact and usually respiratory
Microscopic (histologic) images

Images hosted on PathOut server:

Bland spindled and round / oval cells with prominent blood vessels

CD34 highlights vessels but tumor cells are negative

Smooth muscle actin is diffusely positive



Images hosted on other servers:

Various images

Negative stains
Electron microscopy description
  • Basal lamina surrounds individual cells, tapered cytoplasmic extensions, orderly bundles of filaments
Electron microscopy images

Images hosted on other servers:

(A) intracytoplasmic thin filaments with dense bodies seen in dendritic thin cytoplasm
occasional intercellular junctions (arrow); irregular basement membrane-like material
(B) tumor cells are separated from capillary; (C) by continuous basement membrane

Differential diagnosis
  • Glomus tumor: also a pericytic tumor with similar staining but extremely rare in sinonasal region, composed of compact epithelioid cells
  • Lobular capillary hemangioma: lobular pattern at low power, has spindled fibroblasts and prominent vessels but overall granulation tissue-like appearance with only small capillaries
  • Solitary fibrous tumor: similar vascular pattern but tumor has patternless pattern, ropy keloidal collagen, thin walled vascular spaces, D2-40- (Virchows Arch 2006;448:459)