Soft tissue
So called fibrohistiocytic
Tenosynovial giant cell tumor, localized type
Authors: Michella Whisman, M.D., Jerad M. Gardner, M.D.
Editor-in-Chief: Debra L. Zynger, M.D.
Topic Completed: 25 June 2019
Minor changes: 22 April 2021
Copyright: 2019-2021, PathologyOutlines.com, Inc.
PubMed Search: Tenosynovial giant cell tumor[title] AND (Humans[Mesh])
Table of Contents
Definition / general | Essential features | Terminology | ICD coding | Epidemiology | Sites | Pathophysiology | Clinical features | Radiology description | Radiology images | Case reports | Treatment | Clinical images | Gross description | Gross images | Microscopic (histologic) description | Microscopic (histologic) images | Cytology description | Positive stains | Negative stains | Molecular / cytogenetics description | Videos | Sample pathology report | Differential diagnosis | Additional references | Board review style question #1 | Board review style answer #1 | Board review style question #2 | Board review style answer #2Cite this page: Whisman M, Gardner J. Tenosynovial giant cell tumor, localized type. PathologyOutlines.com website. https://www.pathologyoutlines.com/topic/softtissuegctlocal.html. Accessed October 16th, 2021.
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
- Tenosynovial giant cell tumor, localized type per IARC: WHO Classification of Tumours of Soft Tissue and Bone (Medicine), 4th Edition, 2013
- Also referred to as giant cell tumor of tendon sheath
ICD coding
- ICD10: D48.1 - Neoplasm of uncertain behavior of connective and other soft tissue
Epidemiology
- Fourth or fifth decade of life most frequent
- F>M (2:1) (Orthop Traumatol Surg Res 2017;103:S91)
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
- Skin colored nodule without specific features; most are slow growing and painless (Orthop Traumatol Surg Res 2017;103:S91)
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:
Ultrasound of finger lesion (Case 16)
Hand MRI (Case 4 recurrent)
Case reports
- 9 year old girl with multiple lesions within a single digit (Case Rep Orthop 2016;2016:1834740)
- 27 year old man with a localized, intra-articular tenosynovial giant cell tumor presenting as a tear in the anterior cruciate ligament (J Clin Orthop Trauma 2015;6:140)
- 28 year old woman with an intra-articular lesion treated with arthroscopic resection (Medicine (Baltimore) 2015;94:e1887)
- 49 year old woman with a right lateral foot mass (J Orthop Case Rep 2015;5:68)
- 78 year old man with multifocal lesions of the same hand following trauma (J Hand Microsurg 2015;7:233)
Treatment
- Surgical excision (usually curative but may recur)
Clinical images
Contributed by Mark R. Wick, M.D.
Digit lesion
Images hosted on other servers:
Mass over lateral right foot
Tumor at surgery
Gross description
- Well circumscribed with a lobulated appearance (Arch Pathol Lab Med 2012;136:901)
- Variegated cut surface (yellow, tan, red-brown) (Arch Pathol Lab Med 2012;136:901)
Gross images
Contributed by Mark R. Wick, M.D.
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
- May be absent or rare in some cases (giant cell poor) (Ann Clin Lab Sci 2014;44:222)
- 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
- Large histiocytoid cells with ample eosinophilic cytoplasm, eccentrically placed nucleus with vesicular chromatin and relatively prominent nucleolus
- Sclerotic collagenous background
Microscopic (histologic) images
Contributed by Michella Whisman, M.D.
Fibrous bands
Lobular appearance
Multinucleated giant cells
Foamy histiocytes
Mononuclear component
Pigment laden histiocytes
Foamy histiocytes
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
- Large histiocytoid mononuclear cells: clusterin, focal / patchy desmin (71%) (Am J Surg Pathol 2009;33:1225)
- Small stromal mononuclear cells: CD163
- Osteoclast-like giant cells: CD68, CD45, TRAP
Negative stains
Molecular / cytogenetics description
- Translocations involving CSF1 gene at 1p13 are most common
- Multiple partners have been described (Int J Oncol 2014;44:1425)
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 style 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?
- Foamy macrophages
- Hemosiderin laden macrophages
- Histiocytoid mononuclear cells
- Inflammatory cells
- Multinucleated giant cells
Board review style 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.
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Board review style question #2
- Which of the following is true about a tenosynovial giant cell tumor, localized type?
- Both genders are affected equally
- The diagnosis can be made even in the absence of osteoclast-like giant cells
- Highly infiltrative lesion, which lead the majority to recur
- It is the most common tumor of the hand
- The patient always reports a history of trauma
Board review style 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.
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