Soft tissue
Fibrohistiocytic tumors
Tenosynovial giant cell tumor, localized type


Senior Author: Jerad M. Gardner, M.D.
Editor-in-Chief: Debra Zynger, M.D.
Michella Whisman, M.D.
Jerad M. Gardner, M.D.

Topic Completed: 25 June 2019

Revised: 2 July 2019

Copyright: 2019, PathologyOutlines.com, Inc.

PubMed Search: Tenosynovial giant cell tumor[title] AND (Humans[Mesh])

Michella Whisman, M.D.
Jerad M. Gardner, M.D.
Page views in 2018: 21,730
Page views in 2019 to date: 13,875
Cite this page: Whisman M, Gardner J. Tenosynovial giant cell tumor, localized type. PathologyOutlines.com website. http://www.pathologyoutlines.com/topic/softtissuegctlocal.html. Accessed July 20th, 2019.
Definition / general
  • Benign, well circumscribed proliferation of mononuclear cells admixed with variable proportions of osteoclast-like giant cells, foamy histiocytes, siderophages and inflammatory cells
Essential features
  • Composed of variable proportions of mononuclear cells, osteoclast-like giant cells, foamy histiocytes and hemosiderin laden histiocytes
  • Well circumscribed and lobulated, usually in close association with a tendon
  • Second most common tumor of the hand (after ganglion cyst)
Terminology
ICD coding
  • ICD10: D48.1 - Neoplasm of uncertain behavior of connective and other soft tissue
Epidemiology
Sites
  • Predominantly occurs on the digits (85%) (Orthop Traumatol Surg Res 2017;103:S91)
  • Other locations have been reported, such as wrist, ankle, knee, hip, elbow
    • Intra-articular localized tenosynovial giant cell tumors are most frequent in the knee
Pathophysiology
  • Originally believed to be reactive but now considered neoplastic
  • At least some are thought to be caused by the overexpression of CSF1 by a neoplastic component (comprising a subset of lesional cells) which leads to the recruitment and activation of additional, nonneoplastic cells to create a tumor mass (Proc Natl Acad Sci U S A 2006;103:690)
    • Majority of cases contain a subset of cells with a translocation involving CSF1 gene at 1p13
    • Translocation correlates with an overexpression of CSF1, a macrophage colony stimulating factor
    • The other lesional cells express the CSF1 receptor (CSF1R)
Clinical features
Radiology description
  • X-ray: (Radiol Med 2010;115:141)
    • Most commonly demonstrate a soft tissue mass, some of which demonstrates bony erosion
  • Ultrasonography: (Radiol Med 2010;115:141)
    • Lesion does not move with the tendon during flexion / extension
    • Solid, not cystic (helps to differentiate from ganglion cyst)
  • MRI findings: (Oncol Lett 2017;13:4459)
    • Well demarcated
    • Associated with a tendon (and encasing a tendon in some instances)
    • May have variability in signal intensity but will, in general, have foci of low intensity on T1WI and T2WI from hemosiderin
Radiology images

Images hosted on other servers:
Missing Image

Ultrasound of finger lesion (Case 16)

Missing Image

Hand MRI (Case 4 recurrent)

Case reports
Treatment
  • Surgical excision (usually curative but may recur)
Clinical images

Contributed by Mark R. Wick, M.D.

Digit lesion


Images hosted on other servers:
Missing Image

Mass over lateral right foot

Missing Image

Tumor at surgery

Gross description
Gross images

Contributed by Mark R. Wick, M.D.
Missing Image

Cut surface

Microscopic (histologic) description
  • Lobular architecture at low power with fibrous bands separating lobules
  • Polymorphous cell population with variable proportions: (Arch Pathol Lab Med 2012;136:901)
    • Large histiocytoid cells with ample eosinophilic cytoplasm, eccentrically placed nucleus with vesicular chromatin and relatively prominent nucleolus
      • Mitoses may be present and are sometimes abundant
      • A ring or halo of intracytoplasmic hemosiderin may be present around the periphery of these large histiocytoid cells
    • Osteoclast-like giant cells
    • Smaller mononuclear stromal cells with round or reniform nuclei
    • Foamy histiocytes
    • Hemosiderin laden histiocytes
      • May see inflammatory infiltrate but should not be a major component
  • Sclerotic collagenous background
Microscopic (histologic) images

Contributed by Michella Whisman, M.D.
Missing Image

Fibrous bands

Missing Image

Lobular appearance

Missing Image

Multinucleated giant cells

Missing Image

Foamy histiocytes


Missing Image

Mononuclear component

Missing Image

Pigment laden histiocytes

Missing Image

Foamy histiocytes

Missing Image

Mononuclear component

Cytology description
  • Moderately cellular smears with a variable admixture of cell populations: (Diagn Cytopathol 2012;40:E94)
    • Polygonal to spindled mononuclear cells in loose clusters or individually dispersed with moderate cytoplasm and round to oval nuclei
    • Scattered osteoclast like multinucleated giant cells
    • Foamy histiocytes
    • Hemosiderin laden histiocytes
  • Relatively scant background inflammation in most cases (Diagn Cytopathol 2012;40:E94)
Positive stains
Negative stains
Molecular / cytogenetics description
  • Translocations involving CSF1 gene at 1p13 are most common
Videos

Diagnostic features of both localized and diffuse forms of tenosynovial giant cell tumor

Sample pathology report
  • Right finger, index, excision:
    • Tenosynovial giant cell tumor, localized type (giant cell tumor of tendon sheath), 1.6 cm
Differential diagnosis
  • Diffuse type tenosynovial giant cell tumor
    • Essentially identical appearance to localized form at high magnification
    • Distinguished from localized form by large size, infiltrative growth or anatomic site (often intra-articular and in larger joints)
    • Villonodular architecture when intra-articular
  • Giant cell tumor of soft tissue
    • More uniform background of mononuclear cells
    • Typically have sheets of osteoclastic giant cells similar to giant cell tumor of bone
    • Less association with a tendon
  • Fibroma of tendon sheath
    • Slit-like vascular spaces at the periphery
    • Cracking artifact in collagenous background
    • Spindled to stellate fibroblasts / myofibroblasts
    • Lacks giant cells, large histiocytoid cells with eccentric nucleus, hemosiderin laden histiocytes and foamy histiocytes
Additional references
Board review question #1

    The image above comes from a finger mass of a 45 year old woman. Grossly, the lesion was well circumscribed and bosselated. Which of the following features must be identified to the make the diagnosis?

  1. Foamy macrophages
  2. Hemosiderin laden macrophages
  3. Histiocytoid mononuclear cells
  4. Inflammatory cells
  5. Multinucleated giant cells
Board review answer #1
C. Histiocytoid mononuclear cells. The pictured lesion is a tenosynovial giant cell tumor, localized type (also called giant cell tumor of tendon sheath). Although all the listed cell types can be seen in varying proportions within the tumor, the histiocytoid mononuclear cells are the neoplastic component and should always be present.

Comment Here

Reference: Tenosynovial giant cell tumor, localized type
Board review question #2
    Which of the following is true about a tenosynovial giant cell tumor, localized type?

  1. Both genders are affected equally
  2. The diagnosis can be made even in the absence of osteoclast-like giant cells
  3. Highly infiltrative lesion, which lead the majority to recur
  4. It is the most common tumor of the hand
  5. The patient always reports a history of trauma
Board review answer #2
B. The diagnosis can be made even in the absence of osteoclast-like giant cells. Some cases show a paucity of giant cells, which is why it is best to know the constellation of histologic features aside from the presence of giant cells in order to make the diagnosis in giant cell poor cases. Tenosynovial giant cell tumor, localized type, is the second most common tumor of the hand (ganglion cyst is most common). It shows a predilection for females (F:M=2:1). The tumors are usually well circumscribed. They may recur but simple excision is curative the majority of the time. Some patients report a history of trauma but not all.

Comment Here

Reference: Tenosynovial giant cell tumor, localized type
Back to top