Esophagus
Other malignancies
Hemangiopericytoma

Author: Feriyl Bhaijee, M.D. (see Authors page)
Editor: Israh Akhtar, M.D.

Revised: 30 January 2018, last major update September 2013

Copyright: (c) 2003-2018, PathologyOutlines.com, Inc.

PubMed Search: Hemangiopericytoma esophagus

Cite this page: Bhaijee, F. Hemangiopericytoma. PathologyOutlines.com website. http://www.pathologyoutlines.com/topic/esophagusHPC.html. Accessed February 21st, 2018.
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
Gross images

Images hosted on other servers:

Solitary fibrous tumor of soft tissue (not esophagus)

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:

Various images

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