Giant Cell Tumor of Tendon Sheath

KEY FACTS
Nonmelanocytic Tumors of Skin:
Fibrous and "Fibrohistiocytic" Tissue Tumors

TERMINOLOGY • Around large joints (10%)

• Synonyms: Localized-type tenosynovial giant cell, nodular • Benign but recurs locally (15-20%)
tenosynovitis, localized pigmented villonodular synovitis • Intraarticular tumors called localized PVNS
(PVNS) MACROSCOPIC
• Benign soft tissue tumor of synovial origin
• Average size: 1.1 cm (range: 0.5-6.0 cm)
• Arises from tendon sheath, intraarticular site, or bursa
MICROSCOPIC
ETIOLOGY/PATHOGENESIS
• Well-demarcated nodular lesion
• Neoplastic growth
• Multinodular with fibrous septa
• Balanced translocation involving 1p13 (CSF1 gene)
• Polymorphous population of epithelioid cells,
○ CSF1 overexpression by neoplastic stromal cells
macrophages, and osteoclast-like giant cells
○ Autocrine activation of neoplastic cells via CSF1R
• Stromal fibrosis
○ Recruitment and activation of intratumoral
• Average mitotic rate: 5 per 10 figures/high-power field)
macrophages via CSF1R
TOP DIFFERENTIAL DIAGNOSES
CLINICAL ISSUES
• Diffuse-type tenosynovial giant cell tumor (PVNS)
• 2nd most common tumor of hand
• Giant cell tumor of soft tissue
• Any age; peak: 3rd-4th decade
• Fibroma of tendon sheath
• Digits (85%)
• Dermatofibroma

Clinical Presentation of GCTTS Gross Appearance of GCTTS

(Left) Giant cell tumor of
tendon sheath giant cell tumor
of tendon sheath (GCTTS)
typically presents as a
painless, slow-growing mass
that arises from a tendon
sheath, most often on the
volar aspect of a finger, such
as this thumb mass ſt. (Right)
Grossly, GCTTS is well
demarcated, yellow to red-
brown, and typically has a
lobular configuration with
surface clefting as depicted.
Average size is 1.1 cm, ranging
from 0.5-6.0 cm.

Superficial Dermal GCTTS GCTTS at High Magnification

(Left) In this example of
GCTTS, the tumor involves the
superficial dermis, mimicking a
dermatofibroma. Unlike
GCTTS, however,
dermatofibroma does not
contain prominent osteoclast-
like giant cells ﬈. (Right) High
magnification shows a
polymorphous cell population
including large epithelioid
cells with abundant
eosinophilic cytoplasm and
eccentric vesicular nuclei ﬉,
mononuclear stromal cells
with smaller round or reniform
nuclei ﬈, and osteoclast-like
giant cells ﬊. Note the
hemosiderin deposits ﬈.

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