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

  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]

  […] 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]

  Conclusion To conclude this entity has to be kept in mind when there is a swelling in the metacarpal area. [npplweb.com]

• Surgical Procedure

  The Procedure The surgical excision of giant cell tumors is generally preformed in an out patient surgery center. [ahni.com]

  The surgical procedure was performed under brachial plexus block anesthesia with tourniquet of upper limb. [dovepress.com]

  Surgical procedures and prognostic factors for local recurrence of soft tissue sarcomas. Sugiura H, Nishida Y, Nakashima H, Yamada Y, Tsukushi S, Yamada K. J Orthop Sci. 2014 Jan;19(1):141-9. [meidai-seikei.jp]

• Knee Pain

  Discussion Localized, intra-articular GCTTS of the knee joint is a rare lesion and a rare cause of anterior knee pain. In a retrospective study on 207 GCTTS cases, only eight cases of knee involvement have been reported [4]. [cureus.com]

  […] of patients presenting with knee pain and swelling. [ispub.com]

  Aksoy et al 1 reported a case of a 54-year-old woman with a 2-year history of right knee pain and no history of knee injury. Clinical examination and radiographs were negative. [healio.com]

  Relwani J, Factor D, Khan F, Dutta A: Giant cell tumour of the patellar tendon sheath--an unusual cause of anterior knee pain: a case report. Knee 10:145-8,2003. [orthopaedicsone.com]

• Joint Effusion

  Coexisting joint effusions may be inspected on radiograms. Some studies found that extrinsic erosion of bone was found in up to 20% of patients [7]. [cureus.com]

  Masses are usually solitary and well-defined, and joint effusion is usually absent. 6 The pigmented villonodular synovitis represents the diffuse intra-articular form 6 and has a high rate of local recurrence in the extrasynovial localization. 7 Magnetic [healio.com]

  Because of pain and persistent joint effusion, the patient underwent another synovectomy at our institution, and the histologic evaluation of the specimen confirmed the diagnosis of D-TGCT without atypical features. [clinicalsarcomaresearch.biomedcentral.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]

• Confusion

  Considerable confusion exists about the surgical treatment and diagnosis of these disorders. This review evaluates the presentation, surgical therapy and recurrence of these three proliferative disorders. [thedoctorsdoctor.com]

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.

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]

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]

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]

With proper treatment, many children with giant cell tumors can be cured of their disease. Why choose St. Jude for your child’s giant cell tumor treatment? St. Jude surgeons are leaders in cancer and tumor treatment research. [stjude.org]

The treatment of choice is a total excision of the lesion without leaving residual tumor tissue, in order to reduce the risk of recurrence. [cureus.com]

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]

Univariate prognostic analysis revealed that p-AKT, p-mTOR, and p-S6RP expressions were associated with poor prognosis. [hyoka.ofc.kyushu-u.ac.jp]

With a review of the literature on this subject they study the clinical and radiological signs, as well as the treatment and prognosis of this condition. [link.springer.com]

PROGNOSIS Biological Behavior • Rarely involve skin • May erode bone infrequently • Malignant change • May occur (very rarely) • Some reported cases • Recurrence varies from 4% to 44% • Recurrence rate is higher under some conditions, as; • Diffuse, poorly [tumorsurgery.org]

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]

Trauma, inflammation, metabolic disease, and neoplastic etiology are considered etiological factors [ 3 ]. It is the second most common tumor of the hand, but it is very uncommon in children under 10 years of age [ 1, 3 ]. [e-aps.org]

[…] 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]

Its etiology is unknown. We report a case of diffuse intra-articular PVNS of the right knee in a 38-year-old man. Ultrasound and magnetic resonance imaging (MRI) features of the disease are described. [ncbi.nlm.nih.gov]

(Etiology) The exact cause and formation mechanism of Giant Cell Tumor of Tendon Sheath is unknown Some of the theories proposed to offer an explanation of its development relate to: Metabolic defects Infections Abnormal body immunity Blood vessel anomalies [dovemed.com]

As is true for most soft-tissue tumors, the etiology of giant cell tumors of the tendon sheath is unknown. [emedicine.medscape.com]

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]

Epidemiology Giant cell tumors of the tendon sheath are the second most common tumors in the hand; simple ganglion cysts are the most common. [emedicine.medscape.com]

Temporal bone flap is a reliable plug for rigid support in preventing brain hernia and cerebrospinal fluid leak. [ncbi.nlm.nih.gov]

Complete removal of the affected tissue is of utmost importance in preventing recurrence [3,5,6]. Osseous involvement, radiologically evidenced by the presence of bony erosions is a risk factor for local recurrence [7]. [jocr.co.in]

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]

Current medical research has not established a way of preventing Giant Cell Tumor of Tendon Sheath Regular medical screening at periodic intervals with blood tests, scans, and physical examinations, are mandatory for those who have already endured the [dovemed.com]

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]

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