Esophagus
Other malignancies
Hemangiopericytoma


Topic Completed: 1 September 2013

Revised: 11 March 2019

Copyright: 2003-2019, PathologyOutlines.com, Inc.

PubMed Search: Hemangiopericytoma esophagus


Feriyl Bhaijee, M.D.
Israh Akhtar, M.D.
Page views in 2018: 826
Page views in 2019 to date: 1,214
Cite this page: Bhaijee F, Akhtar I. Hemangiopericytoma. PathologyOutlines.com website. http://www.pathologyoutlines.com/topic/esophagusHPC.html. Accessed August 24th, 2019.
Definition / general
  • Ubiquitous mesenchymal tumor showing uncertain line of differentiation (not true microvascular pericytes)
Terminology
Epidemiology
  • Extremely rare in esophagus (only 3 reported cases)
  • HPC / SFT commonly arises in middle aged adults; mean age 50 years
  • No sex predilection
Etiology
  • Unknown
Clinical features
  • Asymptomatic mass or stricture
  • Dysphagia, odynophagia
  • Hematemesis
  • Tumor induced hypoglycemia
    • Rare paraneoplastic syndrome due to production of insulin-like growth factor
Diagnosis
  • Biopsy or resection
Radiology description
  • Barium swallow shows large intraluminal mass in lower cervical and upper thoracic esophagus (frontal and lateral projections); note tracheal displacement anteriorly (Br J Radiol 1995;68:1031)
  • Axial CT shows intraluminal mass in thoracic esophagus with focal low density necrosis
Prognostic factors
  • Risk factors for local recurrence
    • Size > 5 cm
    • Infiltrative growth pattern
    • > 4 mitoses/10 HPF
    • Hypercellularity
    • Cellular atypia, nuclear pleomorphism
    • Tumor necrosis
Case reports
Treatment
  • Complete surgical excision
Gross description
  • Solitary, lobular, soft tissue mass with light tan, firm cut surfaces
  • Usually well circumscribed but malignant tumors may be infiltrative
Microscopic (histologic) description
  • Hyalinized, branching, staghorn, thin walled vessels lined by flattened endothelium (so called HPC-like vasculature)
  • Bland spindled to ovoid tumor cells in patternless pattern, surrounded by reticulin fibers
  • Rare mitotic figures (1 - 3/10 HPF)
  • Malignant HPC: infiltrative growth pattern, hypercellularity, nuclear pleomorphism, hyperchromasia, necrosis, increased mitotic activity (> 3/10 HPF), atypical mitoses
Microscopic (histologic) images

Images hosted on other servers:

Not esophagus:

Reticulin stain

Virtual slides

Images hosted on other servers:

HPC: soft tissue

Cytology description
  • Cellular clusters of ovoid to spindle cells surrounding branched blood vessels
    • Uniform nuclei with fine granular chromatin and indistinct nucleoli
    • Pale, delicate cytoplasm
    • Metachromatic basement membrane material often present
  • No significant atypia, necrosis or mitotic activity
Cytology images

Images hosted on other servers:

Figures 1 - 3

Positive stains
Negative stains
Electron microscopy description
  • Undifferentiated spindle cell or fibroblastic features
Molecular / cytogenetics description
  • No consistent genetic events reported
Differential diagnosis
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