Kidney tumor

Benign adult tumors

Glomus tumor



Topic Completed: 14 October 2021

Minor changes: 14 October 2021

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PubMed Search: Glomus tumor kidney

Lin Cheng, M.D., Ph.D.
Maria Tretiakova, M.D., Ph.D.
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Cite this page: Cheng L, Tretiakova M. Glomus tumor. PathologyOutlines.com website. https://www.pathologyoutlines.com/topic/kidneytumorglomus.html. Accessed December 8th, 2021.
Definition / general
Essential features
  • Variable proportion of glomus cells, blood vessels and smooth muscle cells
  • Glomus cells are uniform, round to ovoid, with abundant cytoplasm, distinctive cell borders and centrically located nuclei
  • Glomus cells forming nests or trabeculae and surrounding dilated thin walled vessels
Terminology
  • Subtypes include solid glomus tumor, glomangioma (glomuvenous malformation) and glomangiomyoma
  • Other related names: glomangiomatosis, symplastic glomus tumor, glomus tumor of uncertain malignant potential and malignant glomus tumor (glomangiosarcoma)
ICD coding
  • ICD-O: 8711/0 - glomus tumor, NOS
  • ICD-O: 8711/3 - glomus tumor, malignant
  • ICD-10: D30.00 - benign neoplasm of unspecified kidney
  • ICD-11: 2E81.Y & XH47J2 - other specified benign vascular neoplasms: glomus tumor, NOS
Epidemiology
Sites
  • Renal primary is very rare
  • Most common extrarenal sites are subungual areas
Pathophysiology
  • Uncertain in kidney
  • In soft tissue counterpart, resembles the perivascular modified smooth muscle cells of the normal glomus body
Etiology
Clinical features
Diagnosis
  • Imaging study of renal mass and histological examination
Radiology description
Radiology images

Images hosted on other servers:

A: heterogeneous
enhancing mass
B: unevenly increased
FDG uptake

Well defined heterogenous lesion

Well defined heterogenous lesion

Bilateral inhomogeneous lesions

Bilateral inhomogeneous lesions

Prognostic factors
Case reports
Treatment
  • Surgical resection
Gross description
  • Well circumscribed and often encapsulated, brownish or white-tan solid mass
  • Usually homogenous cut surface, may show cystic, hemorrhagic or myxoid areas (Arch Pathol Lab Med 2005;129:1172)
Gross images

Images hosted on other servers:
Well defined pale mass

Well defined pale mass

Myxoid areas

Myxoid areas

Microscopic (histologic) description
  • Contains 3 components: glomus cells, blood vessels and smooth muscle cells
    • Glomus cells: small and uniform, round to oval shaped, with distinctive cell borders, centrally located punched out nuclei, light eosinophilic or amphophilic cytoplasm (Hum Pathol 2017;64:106)
    • Blood vessels: usually thin walled, capillary sized, dilated or branching
    • Smooth muscle cells: elongated, mature looking (Arch Pathol Lab Med 2005;129:1172)
  • Subtyping depends on the proportion of the 3 components:
    • Solid glomus tumor:
      • Glomus cells predominant
      • Cuffs of uniform glomus cells forming nests or trabeculae and surrounding thin walled vessels
    • Glomangioma:
      • Vessels predominant
      • Cavernous hemangioma-like vasculatures surrounded by small clusters or rare layers of glomus cells
    • Glomangiomyoma:
  • Histologically malignant: atypical mitotic figures or moderate to high nuclear atypia and ≥ 5 mitotic figures/50 high power fields (Am J Surg Pathol 2001;25:1)
  • Glomus tumor with uncertain malignant potential: does not meet criteria for malignancy but has at least 1 atypical feature other than nuclear pleomorphism
Microscopic (histologic) images

Contributed by Maria Tretiakova, M.D., Ph.D.
Capsule and nests

Capsule and nests

Small uniform tumor cells

Small uniform tumor cells

Abundant vasculature

Abundant vasculature

Rare mitotic figure

Rare mitotic figure


Positive SMA

Positive SMA

Positive collagen IV

Positive collagen IV

Negative S100

Negative S100

Electron microscopy description
Electron microscopy images

Contributed by Debra L. Zynger, M.D.
Basal lamina

Basal lamina



Images hosted on other servers:
Thin microfilament, dense bodies

Thin microfilament, dense bodies

Sample pathology report
  • Right kidney, partial nephrectomy:
    • Benign glomus tumor, 1.5 cm; margins of resection free of involvement (see comment)
    • Comment: Clinically, this patient presented with flank pain and microhematuria. CT showed a heterogenous enhancing right renal mass. No other lesions or history of any malignancies are identified. The histological examination shows nests of small uniform tumor cells surrounding dilated capillary sized, thin walled vessels. The tumor cells are round to ovoid, with distinctive cell borders, amphophilic cytoplasm and centrally located nuclei. No mitosis is seen. Immunohistochemistry studies show that the tumor cells are positive for SMA and negative for pancytokeratin, synaptophysin and chromogranin. Collagen IV immunostain shows pericellular positivity. The overall findings are consistent with a benign glomus tumor, likely renal primary.
Differential diagnosis
  • Myopericytoma:
    • Also belongs to pericytic / perivascular tumors
    • Bland, myoid appearing spindle cells showing concentric perivascular growth
    • May have PDGFRB mutations
  • Juxtaglomerular cell tumor:
    • Young patients with severe hypertension, hyperkalemia and elevated plasma renin
    • Overlapped features with glomus tumor by light microscopy
    • Expresses KIT in ~50% cases, unlike glomus tumor (Hum Pathol 2013;44:47)
    • Rhomboid shaped renin crystals on electron microscopy
  • Paraganglioma:
    • Zellballen / organoid growth pattern
    • Sustentacular cells are positive for S100 and chief cells are positive for neuroendocrine markers
    • Negative for cytokeratin, SMA and collagen IV
    • Misnomers, such as glomus jugulare, glomus tympanicum, etc., are paragangliomas
  • Well differentiated neuroendocrine tumor:
    • Nests or organoid architecture, plasmacytoid cells with stippled chromatin
    • Positive for cytokeratin and neuroendocrine markers
    • Negative for SMA and collagen IV
  • Solitary fibrous tumor:
    • Fibroblastic spindle cell tumor with patternless pattern and staghorn vasculature
    • Positive for STAT6 immunostain
  • Angiomyolipoma:
    • Triphasic tumor with mature adipose tissue, smooth muscle and thick walled vessels
    • Positive for melanocytic markers (HMB45, MelanA, MITF), smooth muscle markers (SMA, calponin) and cathepsin K
Board review style question #1

Which of the following describes the histological features of a glomus tumor?

  1. Fibroblastic tumor with patternless pattern and staghorn vasculature
  2. Myoid appearing spindle cells showing concentric perivascular growth
  3. Nests of uniform round cells with distinctive cell borders and centrically located nuclei surrounding the dilated thin walled vessels
  4. Sustentacular cells and chief cells forming zellballen pattern
  5. Triphasic tumor with mature adipose tissue, smooth muscle and thick walled vessels
Board review style answer #1
C. Nests of uniform round cells with distinctive cell borders and centrically located nuclei surrounding the dilated thin walled vessels

Comment Here

Reference: Glomus tumor
Board review style question #2

Which immunostain is positive in glomus tumor?

  1. Collagen IV
  2. HMB45
  3. Pancytokeratin
  4. STAT6
  5. Synaptophysin
Board review style answer #2
A. Collagen IV

Comment Here

Reference: Glomus tumor
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