DEFINITION AND PATHOGENESIS
- Aka "benign synovioma"---no relationship to synovial sarcoma; tenosynovial giant cell tumor, localized type, nodular tenosynovitis, fibrous histiocytoma of synovium, fibrous xanthoma of synovium, pigmented nodular synovitis)
- First described by Chassaignac as "cancer of tendon sheath"
- Unclear as to etiology
- Localized form
- Intraarticular form (most in the infrapatellar fat)
- Diffuse form (florid proliferative synovitis, extraarticular PVNS)
- Most common neoplasm of the hand
- Most common between 30-50 years, F>M, 20-40 yrs in diffuse form
- Develop over a long period, often without change for several years
- Fixed to deep structures
- Distal lesions adjacent to IPJs
- Has been the reported cause of osteomalacia in a single pt
- Compressive neuropathy reported
- Carpal tunnel syndrome
- Ulnar tunnel syndrome at Guyon's canal
- Reported to be a cause of trigger wrist
- Reported as a cause of hallux valgus with EDL involvement
- Reported at the site of a previous tendon laceration
- Circumscribed soft tissue mass in ½ of cases
- 10-11% with cortical erosion or perforation (more common in lesions of the feet)
- Hypoechoic nodules with homogeneous or heterogeneous echogenicity
- T1-weighted images: muscle isointensE
- T2-weighted images: hypointense with black regions (hemosiderin)
- ? homogeneous enhancement
- + uptake on FDG-PET scan often seen when staging is done for a malignant dx
- Lobulated, 0.5-4cm in size, larger lesions on the feet
- Mottled cut surface, pink to gray with yellow-brown areas of lipid and hemosiderin
- Dense capsule continuous with nodule septae does not totally contain tumor, especially where it is adjacent to synovial membrane
- Moderately cellular with sheets of rounded to polygonal cells
- Multinucleated giant cells numerous to sparse (esp. In recurrent lesions)
- Formed by fusion of mononuclear cells with 3-60 nuclei
- Xanthoma cells contain hemosiderin granules
- Hyalinization, and cartilaginous/osseous metaplasia rarely found
- Mitotic figures in 50%, with ? 3/hpf in > 10%
- Tumor thrombi in1-5% within small veins (does not indicate malignancy)
- nm23 expression (antimetastasis and antiinvasion gene) associated with a significant risk of LR
- p63 expression
- EM: both Type A (synovial cell) and Type B (fibroblastic cell) synovial membrane cell types present
- Cell surface markers suggest osteoclastic lineage
- Flow cytometry: diploid pattern in 50% of localized form, aneuploid pattern in 50% of diffuse form
DIFFERENTIAL CLINICOPATHOLOGIC DIAGNOSIS
- Foreign body granuloma (more inflammatory cells)
- Necrobiotic granuloma (degenerating collagen rimmed by histiocytes and proliferating capillaries)
- Tendinous xanthoma (hyperlipidemia with intratendinous xanthomas, "cholesterol clefts")
- Fibroma of tendon sheath
- Calcifying aponeurotic fibroma
- Epithelioid sarcoma with numerous giant cells (+ keratin)
DISEASE COURSE AND TREATMENT
- 10-20% locally recur, possibly higher with longer followup
- Possibly related to more cellular lesions with ? mitoses
- More common intralesional excisions, where tumor is left at deep margins
- 20 Gy in 10 divided doses has been reported to ? LR in selected pts (no long term FU however)
- Local excision with a small cuff of normal tissue
- Surface mold brachytherapy has been used after resection
- Amputation of digit for extensive disease may be required
TENOSYNOVIAL GIANT CELL TUMOR, DIFFUSE TYPE
- (Proliferative Synovitis, Florid Synovitis, Extraarticular Pig-mented Villonodular Synovitis, Pigmented Villonodular Bursitis)
- PVNS or GCTTS when it occurs extraarticularly or within a bursal sac, arising from the synovium of a tendon sheath or a bursa
- ½ of pts <40yrs of age, F>M (slightly)
- Sxs often of long duration include pain and local tenderness
- X-rays may show adjacent osteopenia, cortical erosion
- Bone scan may show ? soft tissue uptake
MULTIFOCAL GIANT CELL TUMOR OF TENDON SHEATH
- Multifocal involvement of the same tendon in different locations has been described
- Bilateral thumb involvement has been reported
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