Diagnosis Of Tenosynovial Giant Cell Tumor Research Articles

Pure muscle tenosynovial giant cell tumor mimicks a metastasis in patient with melanoma

Tenosynovial Giant Cell Tumor (TGCT) is a rare benign synovial tumor arising from the tendon sheath, bursae, synovium or adjacent soft tissue. TGCT typical occurs in intra-articular site but can be rarely extra- articular. A 50-years-old woman already affected by malignant melanoma was submitted to PET/CT scan for routine diagnostic examinations during follow up; an intense FDG focal uptake corresponding to peritrochanteric medial part of right iliopsoas muscle was detected. Corresponding MRI images demonstrated a lesion with well-defined margins, slightly hyperintense in T1, low to intermediate signal intensity in T2 weighted scans, no significant restriction in DWI, and intense enhancement in T1 after intravenous contrast medium administration. There wasn’t evidence of extra-lesional spread. MRI findings oriented for benign lesion, but according to intermediate signal intensity in T2, intense enhancement in T1 after intravenous contrast medium, the presence of intense FDG uptake as well as history of aggressive malignancy, a local excision was done; then the final diagnosis of TGCT has been proven. Our case shows TGCT lesions may reproduce malignant appearance on FDG-PET, while MRI may be useful tool to properly manage affected patients. Keywords: Tenosynovial giant cell tumor; Melanoma metastasis; Diffusion-MRI; FDG-PET/CT.

CHONDROID TENOSYNOVIAL GIANT CELL TUMOR OF THE TEMPOROMANDIBULAR JOINT

<h3>Background</h3> Tenosynovial giant cell tumor (TGCT) is a locally aggressive tumor arising from the synovium, most commonly associated with the large joints such as the knee, but they may involve the temporomandibular joint (TMJ). Compared to other sites, TGCT of the TMJ more frequently shows chondroid metaplasia and may mimic chondroid neoplasms. <h3>Case Report</h3> A 78-year-old man presented with several months of history of otalgia, tinnitus, and aural fullness. A computed tomographic scan of his brain showed a destructive, lytic lesion in the squamous part of the temporal bone extending into the zygomatic arch. A fine-needle aspiration biopsy produced a blood-stained smear with scattered pigmented histiocytes and multinucleated giant cells. A diagnosis of giant cell tumor was favored. The patient was commenced on denosumab; however, he showed no clinical improvement after 6 months. A core biopsy was then performed at Westmead Hospital, which revealed a cellular neoplasm comprising spindled and epithelioid cells containing hemosiderin pigment, multinucleated giant cells, and chondrocyte-like cells. Focally, calcific deposits were observed with a "chicken-wire" like pattern. The possible differential diagnoses of chondroblastoma and tenosynovial giant cell tumor were suggested. The histiocyte-like cells showed positive immunoperoxidase staining with CD68, while the larger epithelioid cells were positive for podoplanin (D2-40). The cells were negative for S-100 and H3-K36M, thereby confirming a diagnosis of TGCT. <h3>Conclusions</h3> Although rare, TGCTs are known to show chondroid metaplasia, particularly when involving the TMJ, and they may mimic a chondroblastoma. Immunohistochemical markers such as podoplanin, S-100, and H3F3 K36M are useful in distinguishing these entities.

Management of Tenosynovial Giant Cell Tumor: A Neoplastic and Inflammatory Disease.

Background:Patients with diffuse tenosynovial giant cell tumor (TGCT) face a high risk of recurrence, progression, and disability. This systematic review assesses the recent evidence of surgical, adjuvant, and systemic treatments for TGCT.Methods:We searched PubMed and Ovid with the terms “Giant cell tumor of tendon sheath” OR “pigmented villonodular synovitis” OR “tenosynovial giant cell” AND “treatment” OR “surgery.” Inclusion criteria: published 2013 to present; prospective or retrospective design; English language; > 20 patients with histopathological confirmed diagnosis of TGCT; and ≥ 1 efficacy and/or safety outcome from surgery, systemic drug therapy, or adjuvant 90yttrium radiosynoviorthesis.Results:Of the 434 studies identified, 25 met the inclusion criteria. Of 11 studies in patients with disease in the knee, nine examined surgical treatment approaches, and two evaluated adjuvant 90yttrium radiosynoviorthesis. Of 11 studies in patients with mixed sites of disease, six assessed surgical treatment approaches, and five evaluated systemic drug therapies. Three studies assessed surgery in patients with TGCT in the hand, hip, and ankle or foot.Discussion:The high rates of recurrence and risks associated with surgery emphasize the need for novel treatments in patients with symptomatic, advanced TGCT. Systemic therapy may be valuable as part of a multidisciplinary approach.

The role of a magnet-resonance tomography in diagnosis of a teno-synovial giant-cell tumor (the pigment villo-nodular synoviitis) of a knee joint

Objective. To study and analyze the modern possibilities and role of magnet-resonance tomography in diagnosis of teno-synovial giant-cell tumor (pigmented villo-nodular synoviitis) of a knee joint. To improve diagnosis and monitoring of the disease, using the estimation scale elaborated.&#x0D; Materials and methods. In the investigation 77 patients with pathohistologically verified teno-synovial giant-cell tumor (pigmented villo-nodular synoviitis) of a knee joint were included. The patients were distributed into two groups: the main - 52 patients, who were operated and examined, using diagnostic-treatment algorithm, proposed by the authors, and a control one - 25 patients, who were examined and treated in accordance to standard procedures.&#x0D; Results. Magnet-resonance tomography constitutes a «gold standard» in diagnosis of a pigment villo-nodular synovitis of a knee joint. There was established, that a distance between the graphic plots data, obtained in accordance to Ogilvie-Harris and Ogilvie-Harris-Kostogryz scales, which equals 2 and more, trusts the absence of the disease recurrence. A number «2» was selected not incidentally, because the estimate in 2 points in accordance to the scale equals a criterion «good», and in a 3 points - a criterion «excellent».&#x0D; Conclusion. Application of a function-prognosis scale of Ogilvie-Harris-Kostogryz is directly connected with magnet-resonance tomography. With the help of this scale it is possible to estimate the joint functional state, and, more than using the scale existing, to monitor the disease, as well as to mention about the operation radicalism and the risk of the recurrence occurrence.

Work productivity loss in patients with tenosynovial giant cell tumors in the United States.

e22527 Background: Tenosynovial giant cell tumor (TGCT) is a rare, locally aggressive and debilitating tumor that generally affects young working-age adults. This study assessed work productivity loss in TGCT patients. Methods: Incident patients aged 18-64 years with diagnosis of TGCT, who had earning and disability data, were identified in the OptumHealth database (Q1 1999 – Q1 2017). Patients were classified into surgical and non-surgical groups depending on the presence of joint surgery claim in postindex period. Control patients without TGCT were matched 10:1 with TGCT patients on age, gender, year of TGCT diagnosis, and follow-up duration. The number of days missed from work due to disability and medical visits post diagnosis was compared using Poisson regressions. General linear models were used to compare indirect costs associated with productivity loss. Results: A total of 1,395 TGCT patients (724 surgical; 671 non-surgical) were matched to 13,950 controls (36% female; mean age = 47 years). Both surgical and non-surgical TGCT patients had more comorbidities (mean Charlson Comorbidity Index (CCI): 0.3 vs 0.2; 0.4 vs 0.2; p &lt; 0.001), had greater use of analgesic drugs (44% vs 20%; 40% vs 21%; p &lt; 0.001) and MRI tests (47% vs 4%; 26% vs 3%; p &lt; 0.001) in the 12 months before TGCT diagnosis compared with controls. Both surgical and non-surgical TGCT patients missed more time from work due to disability and medical visits and had higher indirect cost associated with productivity loss than matched controls. Disability burden was greater in patients receiving surgery. Conclusions: Regardless of receiving surgery or not, TGCT is associated with significant work productivity loss. These findings highlight the unmet need for effective treatments to reduce disability and restore function in TGCT patients. [Table: see text]

Clinical characteristics and treatment outcomes in six cases of malignant tenosynovial giant cell tumor: initial experience of molecularly targeted therapy

BackgroundAlthough tenosynovial giant cell tumor (TGCT) is classified as a benign tumor, it may undergo malignant transformation and metastasize in extremely rare occasions. High aberrant expression of CSF1 has been implicated in the development of TGCT and recent studies have shown promising activity of several CSF1R inhibitors against benign diffuse-type TGCT; however, little is known about their effects in malignant TGCT.Case presentationInformation from six consenting patients (3 men, 3 women) with malignant TGCT presenting to Dana-Farber Cancer Institute for initial or subsequent consultation was collected. Median age at initial diagnosis of TGCT was 49.5 years (range 12–55), and median age at diagnosis of malignant TGCT was 50 years (range 34–55). Two patients developed malignant TGCT de novo, while four other cases showed metachronous malignant transformation. All tumors arose in the lower extremities (3 knee, 2 thigh, 1 hip). Five patients underwent surgery for the primary tumors, and four developed local recurrence. All six patients developed lung metastases, and four of five evaluable tumors developed inguinal and pelvic lymph node metastases. All six patients received systemic therapy. Five patients were treated with at least one tyrosine kinase inhibitor with inhibitory activity against CSF1R; however, only one patient showed clinical benefit (SD or PR). Five patients were treated with conventional cytotoxic agents. Doxorubicin-based treatment showed clinical benefit in all four evaluable patients, and gemcitabine/docetaxel showed clinical benefit in two patients. All six patients died of disease after a median of 21.5 months from diagnosis of malignant TGCT.ConclusionsThis study confirms that TGCT may transform into an aggressive malignant tumor. Lymph node and pulmonary metastases are common. Local recurrence rates are exceedingly high. Conventional cytotoxic chemotherapy showed clinical benefit, whereas tyrosine kinase inhibitors against CSF1R showed limited activity. Given its rarity, a prospective registry of malignant TGCT patients is needed to further understand the entity and to develop effective strategies for systemic treatment.

Disability burden in patients with tenosynovial giant cell tumors in the United States from employer perspective.

92 Background: Tenosynovial giant cell tumor (TGCT) is a rare locally aggressive tumor causing pain, swelling, joint destruction, and limited mobility. This study assessed the disability burden and the associated costs in TGCT patients from an employer’s perspective. Methods: A retrospective analysis was performed using medical and disability claims from the OptumHealth database. Incident patients 18-64 years old with a diagnosis of TGCT (as identified by ICD-9: 727.02, 719.2x; ICD-10: D48.1, D21.x, M12.2) were matched 1:10 to controls without TGCT based on age, gender, index year, and follow-up duration. Patients without earning and disability data were excluded. Days of work loss due to disability claims and absenteeism associated with medical visits were compared using Poisson regression models. Costs were compared using generalized linear models. Results: A total of 1,395 eligible TGCT patients were matched with 13,950 controls without TGCT. Despite similar demographics (36% female, mean age 45-47) and only slightly higher comorbidity burden (mean Charlson Comorbidity Index (CCI): 0.3 versus 0.2), TGCT patients had increased usage of analgesic drugs (44% versus 20%) and MRI tests (37% versus 3%), prior to their diagnosis, compared with controls. During follow-up, TGCT patients were more likely to have disability claims (15.1% vs. 5.6%; p &lt; 0.001), had more disability claim days (9.5 versus 2.0; p &lt; 0.001), medically related absenteeism days (9.9 versus 4.3; p &lt; 0.001), and total days of work loss (19.4 versus 6.3; p &lt; 0.001) per person-year compared with their matched controls. After adjusting for age, gender, index year and CCI score, the average annual indirect cost per person was greater for patients with TGCT than controls ($4,653 versus $1,902; p &lt; 0.001). Conclusions: In addition to the known problems of pain, limitation of mobility, and eventual joint destruction, TGCT patients had substantial indirect costs associated with increased work absenteeism and disability. These findings highlight the unmet need for more effective treatments to reduce not only the medical, but also the economic burden of TGCT.