Kidney tumor
Benign adult tumors
Hemangioblastoma


Topic Completed: 25 May 2021

Minor changes: 25 May 2021

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PubMed Search: Hemangioblastoma kidney

Lin Cheng, M.D., Ph.D.
Maria Tretiakova, M.D., Ph.D.
Page views in 2020: 1,898
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Cite this page: Cheng L, Tretiakova M. Hemangioblastoma. PathologyOutlines.com website. https://www.pathologyoutlines.com/topic/kidneytumormalignanthemangioblastoma.html. Accessed June 20th, 2021.
Definition / general
Essential features
  • Lipoblast-like epithelioid cells with abundant cytoplasmic microvacuoles
  • Complex network of thin walled vessels in arborizing or pericytomatous patterns
  • Positive for inhibin, S100, neuron specific enolase (NSE) and vimentin
Terminology
  • Also known as capillary hemangioblastoma
ICD coding
  • ICD-O: 9161/1 - hemangioblastoma
  • ICD-10: D30.0 - benign neoplasm of kidney
  • ICD-11: 2F35 - benign neoplasm of urinary organs (kidney)
Epidemiology
Sites
  • Extracranial locations include but are not limited to kidney, intestine, orbit, forearm, peritoneum, periadrenal soft tissue, flank and peripheral nerve (Am J Surg Pathol 2014;38:119)
  • CNS: most common in cerebellum
Pathophysiology / etiology
  • Unknown at this time
Clinical features
Diagnosis
  • Radiographic studies showing renal mass
  • Histological examination on renal biopsy or resection specimen
Laboratory
Radiology description
Radiology images

Images hosted on other servers:

Left kidney mass

Prognostic factors
Case reports
Treatment
  • Surgical resection
Gross description
  • Well circumscribed, lobulated, gray to brown, solid
Gross images

Images hosted on other servers:

Well demarcated tumor

Microscopic (histologic) description
  • Sheets, lobulated or nested growth pattern
  • Round to oval shaped cells containing microvacuoles in eosinophilic or clear cytoplasm (so called stromal cells), mimicking lipoblasts
  • Complex capillary network comprises mainly thin walled vessels
  • Some areas show spindle cells surrounding vessels (so called pericytomatous pattern) (Int J Surg Pathol 2012;20:519)
  • Focal areas may contain predominant spindle cells with minimal cytoplasm and vague vacuolization (Am J Surg Pathol 2014;38:119)
  • Usually mild pleomorphism, however, occasional marked nuclear pleomorphism, rhabdoid features or pseudointranuclear inclusions can be seen (Am J Surg Pathol 2014;38:119, Diagn Pathol 2012;7:39)
Microscopic (histologic) images

Contributed by Maria Tretiakova, M.D., Ph.D.

Lipoblast-like cells

Nuclear pleomorphism

Capillary network

Inhibin positive

Positive stains
Negative stains
Sample pathology report
  • Left kidney, partial nephrectomy:
    • Hemangioblastoma (see comment)
    • Surgical margins negative for tumor
    • Comment: Clinically, this patient presented with incidental finding of left kidney mass. No other lesions or history of any malignancies are identified. The histological sections of the renal mass show sheets of round to oval atypical cells with complex capillary network. Some of the atypical cells show abundant microvacuoles within clear cytoplasm. Immunohistochemistry studies show that the atypical cells are positive for inhibin, S100 and vimentin and negative for PAX8, CK8/18, HMB45 and MDM2. The overall findings are suggestive of a hemangioblastoma, likely renal primary. Clinical correlation is recommended.
Differential diagnosis
Additional references
Board review style question #1

Which of the following is the most commonly seen histological feature of hemangioblastoma?

  1. Admixture of adipose tissue, smooth muscle and thick walled vessels
  2. Lobules of mature adipocytes
  3. Spindle cells with cytoplasmic microvacuoles and complex capillary network
  4. Storiform fibrosis
  5. Sustentacular cells wrapped around chief cells, forming zellballen pattern
Board review style answer #1
C. Spindle cells with cytoplasmic microvacuoles and complex capillary network

Comment Here

Reference: Hemangioblastoma
Board review style question #2
Which of the following is the most likely immunophenotype of hemangioblastoma?

  1. PAX8+, AE1 / AE3+, inhibin-, HMB45-, S100-
  2. PAX8-, AE1 / AE3-, inhibin+, HMB45-, S100+
  3. PAX8-, AE1 / AE3-, inhibin-, HMB45+, S100+
  4. PAX8-, AE1 / AE3+, inhibin-, HMB45-, S100-
  5. PAX8-, AE1 / AE3-, inhibin-, HMB45-, S100-
Board review style answer #2
B. PAX8-, AE1 / AE3-, inhibin+, HMB45-, S100+

Comment Here

Reference: Hemangioblastoma
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