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Tendon Sheath Giant Cell Tumor

Giant cell tumors of the tendon sheath are benign, circumscribed tumors that may either arise from the tendon sheath or from the synovium. These lesions are part of the fibriohistiocytic tumor cluster. Most commonly, they are localized, but may also be diffuse. The diffuse form mainly affects lower extremities, while localized tumors are more often encountered in the hand. Recurrences after excision may occur.


Presentation

Giant cell tumors of the tendon sheath are described as firm, lobulated masses that may cause no symptoms or patients may present with impaired mobility of the affected segment with distal paresthesia. Pain is uncommon. Lesions usually have a slow progression rate, but faster growth (within weeks) has also been reported. They usually occur in the distal interphalangeal joints rather than larger articulations [1] [2], especially on their palmar or plantar side [3], near the extensor or flexor tendon sheath and may extend and implicate both volar and dorsal parts of the digits. Metastasis has not been described, but recurrence after excision may be as high as 44%. Clinical examination reveals the fact that they are solitary or multinodular masses, adherent to the underlying and overlying structures, or skin may be mobile over the lesion. The examiner should try to determine if the joint capsule, nerves or vessels are compressed by the tumor or if adjacent bone erosion is present. Ankles, elbows, wrists and knees may also be involved [4]. In children upper and lower extremities are equally affected [5], but the disease more often appears in 30 to 50 years old individuals, slightly more frequently in women. The right hand is more often affected. Transillumination is negative in giant cell tumors of the tendon sheath, making differential diagnosis with cysts easier.

Swelling
  • […] and extra articular should be considered as a differential diagnosis in swelling of hand and fingers.[scopemed.org]
  • On examination proper, swelling extended from the Metacarpo-phalangeal joint to interphalangeal joint of left thumb. Swelling was globular in shape with the overlying skin being normal.[file.scirp.org]
  • Depending on the type of hip arthritis, symptoms can range from pain and swelling in the joints to difficulty using or moving the joint in a normal way.[northwell.edu]
  • […] of patients presenting with knee pain and swelling.[ispub.com]
  • , boil Kun reading compounds 腫れる 【はれる】 to swell (from inflammation), to become swollen 腫れ 【はれ】 swelling, boil 腫れる 【はれる】 to swell (from inflammation), to become swollen 腫らす 【はらす】 to cause to swell, to inflame 腫れ物 【はれもの】 tumor, tumour, swelling, boil, abscess[jisho.org]
Paresthesia
  • Giant cell tumors of the tendon sheath are described as firm, lobulated masses that may cause no symptoms or patients may present with impaired mobility of the affected segment with distal paresthesia. Pain is uncommon.[symptoma.com]
Paresthesia
  • Giant cell tumors of the tendon sheath are described as firm, lobulated masses that may cause no symptoms or patients may present with impaired mobility of the affected segment with distal paresthesia. Pain is uncommon.[symptoma.com]

Workup

Clinical examination must be followed by an imaging workup in order to establish the diagnosis of a giant cell tumor of the tendon sheath. The first step is to perform a plain radiography, that shows a well circumscribed, sometimes calcified tumor that may cause bone erosion [6] due to its pressure effect and, not due to direct invasion.

If radiological features are inconclusive then a magnetic resonance imaging scan should be performed. This examination will establish the soft tissue character of the pathological process [7] and may highlight decreased signal intensity in certain areas, due to hemosiderin accumulation.

Echography offers information about tumor size, internal vascularization- which is often rich, echogenity (hypo [8] or hyperechoic [9]), location and relationship with neighboring structures [10]. Due to the fact that tumors are usually superficial, they can be well characterized using this method. Tumor borders are usually clear.

If clinical presentation dictates excision, the diagnosis will also be confirmed by means of histology. Tumors are usually 0.5 to 5 cm in diameter and composed of rounded or polygonal cells, collagen, inflammatory cells histiocytes, giant cells and xanthoma cells, rich in hemosiderin [11] embedded in a fibrous matrix [12]. A fibrous capsule surrounds this. The tumor has low mitotic activity and necrosis is rarely seen. Mitotic activity is not a predictor for future recurrence.

Treatment

  • Part 2 is a long-term treatment phase in which all participants receive open-label pexidartinib. There was also a follow-up period added to Part 2.[clinicaltrials.gov]
  • Patient Population: Prevalence and Epidemiology Knee // Shoulder & Elbow // Hip // Spine // Foot & Ankle // Hand & Wrist Fragility Fractures: Diagnosis and Treatment Shoulder & Elbow The Characteristics of Surgeons Performing Total Shoulder Arthroplasty[mdedge.com]
  • Because of its destructive nature, immediate treatment upon diagnosis is recommended. Radical resection proved to be an excellent choice for superior local control.[ncbi.nlm.nih.gov]
  • Surgery to remove the tumor is a common treatment, though the tumors tend to recur. Fibroma of tendon sheath List of cutaneous conditions[en.wikipedia.org]
  • Tendon sheath (usually volar) or interphalangeal joint Large joints (10%) Ankle, knee, wrist, elbow Bursa Intraarticular tumors called localized PVNS Presentation Painless mass Slow growing Uncommon findings: Triggering, carpal and ulnar tunnel syndromes Treatment[basicmedicalkey.com]

Prognosis

  • Large joints (10%) Ankle, knee, wrist, elbow Bursa Intraarticular tumors called localized PVNS Presentation Painless mass Slow growing Uncommon findings: Triggering, carpal and ulnar tunnel syndromes Treatment Surgical approaches Complete local excision Prognosis[basicmedicalkey.com]
  • Prognosis GCTTS is a slowly proliferative tumor that may infiltrate the overlying dermis and erode the bone by pressure or direct invasion. Although rare, a malignant GCTTS can occur. Most giant-cell tumors are treated with surgical excision.[humpath.com]
  • Univariate prognostic analysis revealed that p-AKT, p-mTOR, and p-S6RP expressions were associated with poor prognosis.[hyoka.ofc.kyushu-u.ac.jp]
  • Signal characteristics T1: low signal T2: low signal T1 C (Gd): often show moderate enhancement 6 GE: low and may demonstrate blooming Treatment and prognosis Tenosynovial giant cell tumors are usually benign and local surgical excision usually suffices[radiopaedia.org]
  • LM foam cells, multinucleated giant cells (may be scarce), /-tendon, /-hemosiderin-laden macrophages LM DDx giant cell lesions Gross circumscribed mass - yellow-brown to tan Site hand - classic site Prognosis good (benign), can be malignant (rare) Giant[librepathology.org]

Etiology

  • […] pigmented villonodular synovitis (PVNS), nodular tenosynovitis Definitions Benign soft tissue tumor of synovial origin Polymorphous population of neoplastic stromal cells, macrophages, and osteoclast-like giant cells Well circumscribed, noninvasive ETIOLOGY[basicmedicalkey.com]
  • Pigmented Villonodular Synovitis Pigmented villonodular synovitis (PVNS) is a term given to a group of benign, locally invasive lesions of the synovium of joints, bursae, and tendon sheaths.[ 39 ], [ 40 ] Involvement of the TMJ is rare.[ 39 ] The etiology[eref.thieme.de]
  • Abstract quote BACKGROUND AND OBJECTIVES: Giant cell tumor of the tendon sheath (GCTTS) is a lesion of uncertain etiology.[thedoctorsdoctor.com]
  • Despite the undetermined etiology, the clinical presentation, diagnosis, and surgical treatment of GCT-TS are described.[ijhas.in]

Epidemiology

  • Patient Population: Prevalence and Epidemiology Knee // Shoulder & Elbow // Hip // Spine // Foot & Ankle // Hand & Wrist Fragility Fractures: Diagnosis and Treatment Shoulder & Elbow The Characteristics of Surgeons Performing Total Shoulder Arthroplasty[mdedge.com]
  • ETIOLOGY/PATHOGENESIS Histogenesis Neoplastic growth Balanced translocation involving 1p13 (CSF1 gene) in many tumors CSF1 overexpression by neoplastic stromal cells Recruitment and activation of intratumoral macrophages by CSF1R activation CLINICAL ISSUES Epidemiology[basicmedicalkey.com]
  • […] bursae and tendon sheath; giant cell tumor of bone, giant cell tumor of tendon sheath and pigmented villonodular synovitis may have common cell lineage ( Ultrastruct Pathol 2006;30:151 ) See also joints chapter (fibrous histiocytoma of tendon sheath) Epidemiology[pathologyoutlines.com]
  • Epidemiology GCTTS affects individuals between the age of 30 to 50 years and is more often seen in women than in men. GCTTS is an idiopathic benign tumor of the synovial membrane and is one of the most common soft tissue tumors in the hand.[humpath.com]
Sex distribution
Age distribution

Prevention

  • Temporal bone flap is a reliable plug for rigid support in preventing brain hernia and cerebrospinal fluid leak.[ncbi.nlm.nih.gov]
  • Prakash P, Kotwal, et al (2000) Giant-cell tumour of the tendon sheath: is radiotherapy indicated to prevent recurrence after surgery? Br J Bone Joint Surg 82B:571–573 Google Scholar 16.[link.springer.com]
  • Giant cell tumours of tendon sheath of hand: causes and strategies to prevent recurrence. Hand Surg. 2011; 16 (2): 149-54[ DOI ][ PubMed ] 11. Hamdi MF, Touati B, Zakhama A.[shafaorthoj.com]
  • If the tumor is incorporated into the tendon sheath or joint, some excision of these structures may also be necessary to ensure complete removal of the tumor and to prevent recurrence.[hand411.com]
  • Is Radiotherapy Indicated to Prevent Recurrence after Surgery? The Bone & Joint Journal, 82, 571-573.[file.scirp.org]

References

Article

  1. Uriburu I, Levy V. Intraosseous growth of giant cell tumors of tendon sheath of the digits: report of 15 cases. J Hand Surg. 1998;23:732–736.
  2. Garg B, Kotwal P. Giant cell tumour of the tendon sheath of the hand. J Orthop Surg. 2011;19(2):218-220.
  3. Briët J, Becker S, Oosterhoff T, et al. Giant cell tumor of tendon sheath. Arch Bone Jt Surg. 2015;3(1):19-21.
  4. Findling J, Lascola N, Groner T. Giant cell tumor of the flexor hallucis longus tendon sheath: a case study. J Am Podiatr Med Assoc. 2011;101(2):187-189.
  5. Gholve P, Hosalkar H, Kreiger P, et al. Giant cell tumor of tendon sheath: largest single series in children. J Pediatr Orthop. 2007;27:67–74.
  6. Karasick D, Karasick S. Giant cell tumor of tendon sheath: spectrum of radiologic findings. Skeletal Radiol. 1992;21:219–224.
  7. Bancroft L, Peterson J, Kransdorf M. Imaging of soft tissue lesions of the foot and ankle. Radiol Clin North Am. 2008;46(6):1093-1103.
  8. Ho C, Chiou H, Chou Y, et al. Peritendinous lesions: the role of high-resolution ultrasonography. Clin Imaging. 2003;27:239–250.
  9. Peh W, Wong Y, Shek T, et al. Giant cell tumour of the tendon sheath of the hand: a pictorial essay. Australas Radiol. 2001;45:274–280.
  10. Middleton W, Patel V, Teefey S, et al. Giant cell tumors of the tendon sheath: analysis of sonographic findings. AJR Am J Roentgenol 2004;183:337–339.
  11. Iyer V, Kapila K, Verma K. Fine-needle aspiration cytology of giant cell tumor of tendon sheath. Diagn Cytopathol. 2003;29(2):105-110.
  12. Ly J, Carlson C, Lagatta L, et al. Giant cell tumor of the peroneus tendon sheath. AJR Am J Roentgenol. 2003;180(5):1442.

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Last updated: 2019-07-11 20:13