Soft tissue
Fibroblastic / myofibroblastic tumors
Hemangiopericytoma

Author: Komal Arora, M.D., Vijay Shankar, M.D. (see Authors page)

Revised: 24 August 2016, last major update November 2012

Copyright: (c) 2002-2016, PathologyOutlines.com, Inc.

PubMed Search: Hemangiopericytoma [title] soft tissue

Cite this page: Hemangiopericytoma of soft tissue. PathologyOutlines.com website. http://www.pathologyoutlines.com/topic/softtissuehemangiopericytoma.html. Accessed December 6th, 2016.
Definition / General
  • Rare, controversial entity
  • In soft tissue, most lesions are actually solitary fibrous tumor, monophasic synovial sarcoma or myofibromatosis (Am J Surg Pathol 2010;34:777)
  • Diagnosis of exclusion (Histopathology 2006;48:63)
  • Historically defined as lesions with thin walled, branching vascular pattern, although this pattern is common in many lesions
  • Probably not a lesion of pericytes (cells arranged around capillaries and venules), except at sinonasal location
  • See also discussions in these chapters: Bone, Breast, CNS, Heart, Nasal cavity, Thyroid gland

Infantile / congenital hemangiopericytoma
Lipomatous hemangiopericytoma
Epidemiology
Lipomatous hemangiopericytoma
  • Uncommon
  • 2/3 males, usually thigh, lower extremity and retroperitoneum
  • Only rarely recurs, does not metastasize
Clinical Features
  • Slowly enlarging painless mass of middle aged adults, more common in women
  • Most common in deep soft tissue, particularly pelvic retroperitoneum, also limb or limb girdles and head and neck
  • Occasionally associated with hypoglycemia (J Clin Endocrinol Metab 1996;81:919)
  • 20 - 50% metastasize to lungs, liver, bone
  • 5 year survival was 86% in 2002 (Cancer 2002;95:1746)
  • 70% have benign behavior
  • Poor prognostic factors may be 4+ mitotic figures / 10 HPF, necrosis, nuclear pleomorphism with size > 5 cm
Case Reports

Lipomatous hemangiopericytoma
Clinical Images

Images hosted on PathOut servers:

CT scan, paranasal sinus, courtesy of Mark R. Wick, M.D.

Glomangiopericytoma, courtesy of Mark R. Wick, M.D.



Images hosted on other servers:

Jaw mass

Tongue lesion

Infantile / congenital
hemangiopericytoma

Gross Description
  • Well circumscribed, gray / white to red / brown cut surface, fleshy or spongy with hemorrhage, cystic degeneration, variable necrosis, up to 15 cm

Lipomatous hemangiopericytoma
  • Solid, tan-yellow
Gross Images

Images hosted on PathOut servers:

Courtesy of Mark R. Wick, M.D.



Images hosted on other servers:

Greater omentum

Jejunum mesentery

Diaphragm and the segment VII of the liver

Encapsulated omental tumor

Lipomatous hemangiopericytoma - thigh tumor

Micro Description
  • Uniformly cellular (similar to cellular areas of solitary fibrous tumor) with numerous, variably ectatic or compressed, thin walled branching vessels with gaping sinusoidal spaces (staghorn configuration)
  • Tumor cells are spindled to round with small amounts of pale or eosinophilic cytoplasm, indistinct margins, bland vesicular nuclei
  • May have extensive fibrosis, hyalinization, myxoid change
  • Variable mitotic activity
  • No atypia

Infantile / congenital hemangiopericytoma
  • Immature cytology, frequent mitotic figures, necrosis, possibly neoplastic endothelial cells

Lipomatous hemangiopericytoma
  • Well circumscribed, patternless cellular areas, prominent hemangiopericytoma-like vessels, mature adipose tissue, variable collagen
Micro Images

Scroll to see all images:


Images hosted on PathOut servers:

Courtesy of Mark R. Wick, M.D.



Glomangiopericytoma (last 5 images)



Lipomatous hemangiopericytoma - Case of the Week #16, retroperitoneal mass



Images hosted on other servers:

Staghorn vessels and spindle cells

Staghorn vessels are prominent

Jaw mass



Various images

Rectal mass

Reticulin stain

GMS



Lipomatous hemangiopericytoma - thigh tumor



Lipomatous hemangiopericytoma - mediastinal tumor

Lipomatous hemangiopericytoma - paratesticular

Lipomatous hemangiopericytoma - stomach



Cytology Description
  • Cellular; single and tightly packed clusters of oval to spindle cells aggregated around branched capillaries
  • Often basement membrane material present
  • Nuclei are uniform, oval, with finely granular chromatin and indistinct nucleoli
  • No mitotic figures, no necrosis (Cancer 1999;87:190)

Lipomatous hemangiopericytoma
Positive Stains

Lipomatous hemangiopericytoma
Negative Stains

Lipomatous hemangiopericytoma
Electron Microscopy Description
  • Has fibroblastic or undifferentiated spindle cell features
  • No true pericytic differentiation (pericytic features are cytoplasmic filaments and processes, pinocytotic vesicles, basal lamina, poorly formed intercellular junctions)

Lipomatous hemangiopericytoma
  • Features of pericytes, no lipoblasts
Molecular / Cytogenetics Description
  • 12q13-15 alterations in some cases
Differential Diagnosis

Lipomatous hemangiopericytoma