Atypical Presentation of Tenosynovial Giant Cell Tumor on the Hallucal Flexor Tendon Sheath: A Case Report.

Tenosynovial giant cell tumor (TGCT) is a rare type of neoplasm that may be locally aggressive but is most often benign and can be divided into two subtypes: localized and diffuse. It tends to develop in the joints, bursae, and tendon sheaths primarily in the digits of the hand and less commonly in the forefoot. This soft-tissue mass has many possible differential diagnoses, including lipoma, ganglion cyst, plantar fibroma, and various sarcomas; surgical excision is usually indicated to reach a definitive diagnosis and rule out malignancy. We report a rare case of a 30-year-old woman with atypical plantar hallucal pain and a palpable mass on the plantar lateral aspect of the left hallux. Surgical excision and histopathologic evaluation confirmed a TGCT of the left hallucal flexor tendon sheath. Although it bears clinical resemblance to several other soft-tissue masses, TGCT has numerous pathognomonic features evident with advanced imaging and histologic analysis that help the physician obtain an accurate diagnosis and proceed with appropriate treatment.

Diffuse-Type Tenosynovial Giant Cell Tumor An Update of Current Management Strategies.

Diffuse-type giant cell tumor (Dt-GCT), formerly known as pigmented villonodular synovitis, is the more aggressive entity belonging to the spectrum of benign proliferative lesions of synovial origin that may affect the joints, bursae, and tendon sheaths. Diffuse-type giant cell tumor's importance stems from its local aggressiveness and sequelae if left untreated. This review briefly describes Dt-GCT's clinical features, its imaging and pathology findings, and provides an extensive discussion of its available treatments. The management approaches of Dt-GCT can be divided into surgical management and non-surgical management, which includes radiation therapy or more novel molecular and biologic therapies. We also present an algorithm based on disease presentation and site involved to guide treatment.

MRgFUS ablation of a recurrent tenosynovial giant cell tumor in the foot using ExAblate 2100 system in combination with patient immobilization device.

INTRODUCTION: Magnetic Resonance-guided Focused Ultrasound (MRgFUS) treatment for certain anatomy locations can be extremely challenging due to patient positioning and potential motion. This present study describes the treatment of a recurrent tenosynovial giant cell tumor of the plantar forefoot using the ExAblate 2100 system in combination with patient immobilization device. METHODS: Prior to the treatment, several patient immobilization devices were investigated. Vacuum cushions were selected and tested for safety and compatibility with the treatment task and the MR environment. RESULTS: During the treatment, one vacuum cushion immobilized the patient's right leg in knee flexion and allowed the bottom of the foot to be securely positioned on the treatment window. Another vacuum cushion supported the patient upper body extended outside the scanner bore. 19 sonications were successfully executed. The treatment was judged to be successful. No immediate complications were observed. CONCLUSIONS: MRgFUS treatment of a recurrent tenosynovial giant cell tumor of the right plantar forefoot was successful with the use of patient immobilization vacuum cushions. IMPLICATIONS FOR PRACTICE: The immobilization system could be utilized to aid future MRgFUS treatment of lesions in challenging anatomic locations. Various sizes of the vacuum cushions are available to potentially better accommodate other body parts and treatment configurations.

Arthroscopic Excision of a Localized Tenosynovial Giant Cell Tumor of the Knee: A Case Report.

BACKGROUND AND AIM: A tenosynovial giant cell tumor (TGCT) is a proliferative lesion of the synovial membrane of the joints, tendon sheaths and/or bursae. There are two described subtypes, including the localized and diffuse forms. A TGCT can also be intraarticular or extraarticular. An intraarticular localized tenosynovial giant cell tumor (L-TGCT) of the knee is characterized by nodular hyperplasic synovial tissue that can remain asymptomatic for a long time, but as the mass grows, it may cause mechanical symptoms that may require surgical treatment. The aim of our study is to present a rare case of an L-TGCT of the knee joint treated with an arthroscopic excision. CASE REPORT: We describe the case of a 17-year-old female with pain, swelling and knee locking in the absence of trauma. The magnetic resonance imaging (MRI) displayed a well-circumscribed small mass in the anterior medial compartment, adherent to the infrapatellar fat pad. The lesion presented the typical MRI characteristics of an intraarticular localized TGCT. The patient was treated with an arthroscopic mass removal and partial synovectomy. The gross pathology showed an ovoid nodule that was covered by a fibrous capsule; a histopathology examination confirmed the diagnosis. The patient was able to return to normal daily activities one month after surgery; at the three-year follow-up, she was free of symptoms with no evidence of disease on the MRI. CONCLUSION: In patients with a small-dimension L-TGCT in the anterior compartment of the knee that presents an MRI pattern and causes mechanical symptoms, an arthroscopic en-bloc excision can be performed that results in good outcomes and a rapid return to preinjury levels.

Medium-term Outcomes of Excision Using Surgical Microscope of Tenosynovial Giant Cell Tumors of the Hand.

BACKGROUND/AIM: The recurrence rate following the excision of tenosynovial giant cell tumors (TSGCT) of the hand is very high. Intraoperative application of a surgical microscope has been reported. However, to date, there are no reports of medium-term outcomes related to this technique. This study aimed to evaluate the medium-term outcomes of tumor excision using surgical microscope for TSGCT of the hand. PATIENTS AND METHODS: A total of 27 patients, who underwent an initial surgery for histologically-confirmed TSGCT of the hand, between 2008 and 2020, were included and evaluated. The mean follow-up time postoperatively was 6.8 years. Tumor recurrence and preoperative tumor characteristics were assessed. RESULTS: All tumors were adherent to tendons, tendon sheaths, neurovascular structures or periarticular ligaments and capsules. Bony lesions were observed in 11 tumors. The surgical microscope was used in 13 tumors. Recurrences were observed in three tumors (overall recurrence rate: 11%). Tumor characteristics were similar in both groups, but the recurrence rate in the group treated using the surgical microscope was 0%, whereas the recurrence rate in the group treated without the surgical microscope was 21%. Re-operations using the surgical microscope for recurrent tumors were performed, without recurrence postoperatively. CONCLUSION: Among patients with TSGCT of the hand treated with tumor excision using the surgical microscope, the postoperative recurrence rate was 0%. Based on the results of this study, the surgical microscope might be used for excision of TSGCTs of the hand.

Oncological and functional outcomes of modified arthroscopic resection for intra-articular tenosynovial giant cell tumor of the knee using multiple portals.

BACKGROUND: Arthroscopic resection of tenosynovial giant cell tumor (TS-GCT) presents favorable outcomes. However, there are reportedly higher recurrence rates in patients who had incomplete resection. To minimize incomplete resection, we established a multiple portal approach depending on the location of the disease. In this study, we aimed to retrospectively evaluate the clinical outcomes of arthroscopic resection for both localized and diffuse types of TS-GCT of the knee. METHODS: From 2009 to 2019, 13 patients who underwent arthroscopic synovectomy of the knee and were histologically diagnosed with TS-GCT were included in this study. The pre- and postoperative range of motion (ROM) of the knee was measured. The Japanese Orthopaedic Association (JOA) score and the Knee Injury and Osteoarthritis Outcome Score (KOOS) were assessed at the final follow-up examination. Magnetic resonance imaging was performed to detect incomplete resection or local recurrence. RESULTS: Among the 13 patients, seven and six had localized and diffuse type TS-GCT, respectively. Regarding the knee ROM, preoperative knee flexion in patients with the localized type was limited compared with that in those with the diffuse type. However, the ROM was significantly improved in patients with both types postoperatively. The JOA score and KOOS of patients with both types at the final follow-up were favorable, and there were no significant differences between both types. There was neither recurrence nor incomplete resection in any patient for both types. CONCLUSION: All patients, regardless of the TS-GCT type, achieved favorable outcomes after arthroscopic surgery; especially, the failure rate was 0%.

Tenosynovial giant cell tumor: a case report.

BACKGROUND: This case reports the synchronous diagnosis of two rare unrelated diseases; leiomyosarcoma and tenosynovial giant cell tumor of the knee. It focuses on the challenges of diagnosing tenosynovial giant cell tumor, including cognitive biases in clinical medicine that delay diagnosis. It also demonstrates the pathogenic etiology of tenosynovial giant cell tumor, evidenced by the transient deterioration of the patients' knee symptoms following the administration of prophylactic granulocyte colony-stimulating factor given as part of the chemotherapeutic regime for leiomyosarcoma. CASE PRESENTATION: A 37-year-old Caucasian man presented with a left groin lump and left knee pain with swelling and locking. Investigations including positron emission tomography-computed tomography and biopsy revealed leiomyosarcoma in a lymph node likely related to the spermatic cord, with high-grade uptake in the left knee that was presumed to be the primary site. His knee symptoms temporarily worsened each time granulocyte colony-stimulating factor was administered with each cycle of chemotherapy for leiomyosarcoma to help combat myelosuppressive toxicity. Subsequent magnetic resonance imaging and biopsy of the knee confirmed a tenosynovial giant cell tumor. His knee symptoms relating to the tenosynovial giant cell tumor improved following the completion of his leiomyosarcoma treatment. CONCLUSIONS: Tenosynovial giant cell tumor remains a diagnostic challenge. We discuss the key clinical features and investigations that aid prompt diagnosis. The National Comprehensive Cancer Network clinical practice guidelines for soft tissue sarcoma have recently been updated to include the pharmacological management of tenosynovial giant cell tumor. Our case discussion provides an up-to-date review of the evidence for optimal management of patients with tenosynovial giant cell tumor, with a particular focus on novel pharmacological options that exploit underlying pathogenesis.

Outcomes of Tenosynovial Giant Cell Tumor of the Foot and Ankle.

BACKGROUND: Tenosynovial giant cell tumor (TGCT) is a benign proliferative disease affecting synovial membranes. There are 2 forms, localized (L-TGCT) and diffuse (D-TGCT), which although histologically similar behave differently. It is locally invasive and is treated in most cases by operative excision. The aim of this study was to assess current practice, how the patients' presentation affected their outcome, as well as review the recurrence rates and complications. METHODS: A retrospective analysis of 123 cases was performed in patients treated between 2003 and 2019 with TGCT of the foot and/or ankle. Data were collected on age at presentation, radiologic pattern of disease, location of disease, treatment provided, and recurrence rates. The minimum follow-up was 2 years with a mean of 7.7 years. RESULTS: There were 61.7% female patients with a mean age of 39 (range, 11-76) years. L-TGCT accounted for 85 (69.1%) cases and D-TGCT for 38 (30.9%). The most prevalent preoperative symptoms were a palpable mass (78/123) and pain (65/123). Radiologically confirmed recurrence in the operative group was noted in 14.5% (16/110) cases. This comprised 4% (3/75) of operatively treated L-TGCT and 37% (13/35) of operatively treated D-TGCT. Patients with pain on presentation and those with erosive changes on presenting magnetic resonance imaging (MRI) were more likely to have persistent postoperative pain (P < .001 for both). Where patients had both preoperative pain and erosive changes, 57.1% had postoperative pain. Thirteen cases were managed nonoperatively where symptoms were minimal, with 1 case requiring surgery at a later date. CONCLUSION: Outcomes of TGCT management are dependent on the disease type, extent of preoperative erosive changes, and presence of preoperative pain. These data are useful for counseling patients regarding the outcomes of surgical intervention and help guide the timing of intervention. LEVEL OF EVIDENCE: Level III, retrospective cohort study.

Imaging characteristics of tenosynovial giant cell tumors on 18F-fluorodeoxyglucose positron emission tomography/computed tomography: a retrospective observational study.

BACKGROUND: 18F-fluorodeoxyglucose positron emission tomography/computed tomography (18F-FDG-PET/CT) is useful for assessing location, metastasis, staging, and recurrence of malignant tumors. Tenosynovial giant cell tumor (TSGCT) is a benign tumor; however, some studies have reported that TSGCTs have a high uptake of FDG. Few studies have reported on the detailed evaluation of TSGCT using 18F-FDG-PET/CT. The purpose of the current study is to evaluate the image characteristics and locations, particularly where possible, with or without, extra-articular invasion from TSGCT of the knee in 18F-FDG-PET/CT could occur. METHODS: We retrospectively reviewed the patients with TSGCT who were diagnosed pathologically either by biopsy or surgical specimen. Furthermore, we evaluated the difference of the maximum standardized uptake value (SUVmax) between diffused TSGCT with extra-articular invasion and TSGCT with intra-articular localization in the knee. RESULTS: The study consisted of 20 patients with TSGCT. The mean SUVmax of TSGCT was 12.0 ± 6.50. There were five patients with TSGCT arising in the knee with extra-articular invasion and six with TSGCT with intra-articular localization. The mean SUVmax of TSGCT with extra-articular invasion and those with intra-articular localization were 14.3 ± 6.00 and 5.94 ± 3.89, respectively. TSGCT with extra-articular invasion had significantly higher SUVmax than TSGCT with intra-articular localization (p < 0.05). CONCLUSIONS: TSGCT revealed high FDG uptake. Furthermore, SUVmax was higher in diffused TSGCT with extra-articular invasion than in intra-articular localized TSGCT; this may reflect its local aggressiveness.

Tenosynovial Giant Cell Tumor (TSGCT) of the hip: MRI accuracy and results of surgical treatment.

Tenosynovial Giant Cell Tumor (TSGCT) or formerly pigmented villonodular synovitis (PVNS) is a rare nonmalignant tumor of the synovia seldom affecting the hip. MRI and surgical resection are the gold standards in its diagnosis and treatment. However, the accuracy of MRI is unknown, and only few reports on its surgical treatment results exist. The goal of the study was to investigate the MRI accuracy, results after surgical treatment, and natural history of untreated MRI-diagnosed hip TSGCT. Twenty-four consecutive patients with suspected TSGCT on hip MRI, between December 2006 and January 2018, were identified from our medical database. Six refused to participate. About 18 patients with a minimal follow-up of 18 months were enrolled. Charts were reviewed for histopathology results, specific treatment and recurrence. At the last follow-up, all patients had a clinical (Harris Hip Score [HHS]) and radiological examination (x-ray and MRI). Out of 18 patients with suspected TSGCT on MRI, with a mean age of 35y (range 17-52), 14 had surgi- cal resection and 4 refused surgery 1 of whom had a CT-guided biopsy. Out of 15 cases with biopsies, in 10 TSGCT was confirmed. Three surgically-treated patients showed recurrence on MRI after 24, 31 and 43 months. Two non-treated patients showed progression after 18 and 116 months. At the last follow-up (65 m; range 18-159), the mean HHS with or without recurrence was 90 and 80pts (ns). Operative vs. non-operative treatment showed HHS of 86 and 90pts (ns). In the conservatively-treated group, HHS with and without progression was 98 and 82pts (ns), respectively. MRI-suspected TSGCT of the hip was confirmed with biopsy in two-thirds of the cases. Surgical treatment showed recurrence in more than one-third of the patients. Two out of four untreated patients showed progression of the TSGCT-suspected lesion.

Arthroscopic resection of localized tenosynovial giant cell tumor in the deep infrapatellar bursa: a case report.

Tenosynovial giant cell tumor (TGCT) is a rare disease characterized by the proliferation of the synovial membrane of a joint, tendon sheath, or bursa. TGCTs in joints are subdivided into the diffuse or localized type. The localized TGCT most frequently affects the knee and may occur in any knee compartment. The most common localization is the Hoffa's fat pad, followed by the suprapatellar pouch and the posterior capsule. Here, we describe a case of a histopathologically proven TGCT of the knee, found in an unusual localization in the deep infrapatellar bursa, which was diagnosed by magnetic resonance imaging. The tumor was entirely arthroscopically resected. The patient had no further complaints following the operation, and there was no recurrence at the 18-month follow-up. Even though TGCT of the knee is uncommon, it should not be overlooked by orthopedic and trauma surgeons, and excision should be regarded as a reliable treatment option. The form of surgical treatment, either open or arthroscopic, should be determined based on a combination of the surgeon's preference and the best approach to the anatomical location of the disease.

Image findings of tendon sheaths affected by diffuse tenosynovial giant cell tumors of the skull base.

OBJECTIVE: This study investigated radiographic images and the differential diagnosis of intracranial diffuse tenosynovial giant cell tumor (D-TGCT) in order to better understand the disease and improve the rate of preoperative diagnosis. PATIENTS AND METHODS: Images and clinical data of patients with D-TGCT were retrospectively analyzed. Routine Computer Tomography (CT), routine Magnetic Resonance Imaging (MRI), and contrast-enhanced MRI were performed for nine cases. Susceptibility-weighted imaging (SWI) was also performed for one case. RESULTS: We reviewed nine patients (6 males and 3 females) aged between 24 and 64 years, with a mean age of 47.33 ± 14.92 years. The most frequent complaints were hearing loss (5/9, 55.6%), pain (4/9, 44%), masticatory symptoms (2/9, 22.2%), and mass (4/9, 44.4%), with a mean duration of 22 ± 21.43 months. All cases were centered on the base of the skull, and showed hyper-density soft-tissue mass with osteolytic bone destruction on CT. The tumor signal mainly showed iso-intensity or hypo-intensity on T1WI compared with that in the brain parenchyma in all patients. On T2WI, nine lesions mainly showed hypo-intensity. Among these nine lesions, three displayed cystic region showing hyper-intensity on T2WI and hypo-intensity on T1WI (Figure 2A, 2B) in the lesion. Nine lesions showed hypo-intensity on DWI sequences. SWI images presented low signal in two cases, showing the "flowering effect". Nine patients showed heterogeneous enhancement, and two patients had meningeal thickening. CONCLUSIONS: Intracranial D-TGCT is extremely rare, but must be differentiated from other tumors. Osteolytic bone destruction in the area of the skull base with hyper-density soft-tissue mass and hypo-intensity on T2WI images are indicative of D-TGCT.

Effectiveness of arthroscopic excision based on the distribution of the tenosynovial giant cell tumor around knee joint.

BACKGROUND: The mainstay treatment for tenosynovial giant cell tumor (TGCT) is open excision. However, open excision is associated with the risk of stiffness, infection, neurovascular injury, and prolonged hospital stay and rehabilitation. The purpose of this study was to evaluate the efficacy of arthroscopic excision of tenosynovial giant cell tumor (TGCT) of the knee joint, including the diffuse type of TGCT. METHODS: Patients who underwent arthroscopic excision of TGCT between April 2014 and November 2020 were retrospectively analyzed. TGCT lesions were divided into 12 distributions (nine intra- and three extra-articular lesions). The distribution of TGCT lesions, portals used, degree of excision, recurrence, and magnetic resonance imaging (MRI) scans were evaluated. The prevalence of intra-articular lesions in diffuse TGCT was also analyzed to validate the existence of a connection between intra- and extra-articular lesions. RESULTS: Twenty-nine patients were included in the study. Fifteen patients (52%) had localized TGCT, and 14 patients (48%) had diffuse TGCT. The recurrence rates for localized, and diffuse TGCT were 0%, and 7%, respectively. Intra-articular posteromedial (i-PM), intra-articular posterolateral (i-PL), and extra-articular posterolateral (e-PL) lesions were found in all patients with diffuse TGCT. The prevalence rates of i-PM and i-PL lesions among e-PL lesions were both 100% (p = 0.026 and p < 0.001, respectively). Diffuse TGCT lesions were managed with posterolateral capsulotomy and viewed from the trans-septal portal. CONCLUSIONS: Arthroscopic excision of TGCT was effective in both localized and diffuse TGCT. However, diffuse TGCT was associated with posterior and extra-articular lesions. Therefore, technical modification such as posterior, trans-septal portal, and capsulotomy were required. STUDY DESIGN: Retrospective case series; level Ⅳ.

Tenosynovial giant cell tumors of digits: MRI differentiation between localized types and diffuse types with pathology correlation.

OBJECTIVE: To compare the MRI findings between the localized- and diffuse-type tenosynovial giant cell tumors (TSGCTs) of digits with pathology correlation. METHODS: Twenty-eight patients with newly diagnosed TSGCTs of digits (22 localized and 6 diffuse types) who underwent preoperative MRI and surgical excision were included from Jan. 2015 to September 2021. MRI findings regarding nodularity, margins, morphology of hypointensity with pathology correlation, and disease extent (bone erosion, articular involvement, muscle involvement, tendon destruction, and neurovascular encasement) were assessed. RESULTS: Diffuse type was significantly larger (P = 0.006), more multinodular on both MRI and pathology (P = 0.038, both) with significant agreement, and infiltrative on both MRI and pathology (P < 0.001, both) with substantial agreement, and showed central granular on MRI and strong hemosiderin deposition on pathology (P = 0.022 and P = 0.021) with moderate agreement than localized type. Localized type showed significantly more frequent peripheral capsules on both MRI and pathology (P < 0.001, both) with moderate agreement than diffuse type. However, the septum on both MRI and pathology showed no statistically significant difference between the two groups (P = 0.529 and P = 0.372) without significant agreement. The disease extent was more severe in the diffuse type than the localized type regarding articular involvement (P < 0.001), muscle involvement (P < 0.001), and tendon destruction (P = 0.010). No statistically significant differences were found between the two groups regarding bone erosion (P = 0.196) or neurovascular bundle encasement (P = 0.165). CONCLUSIONS: Diffuse-type TSGCTs of digits presented as locally aggressive lesions with larger, multinodular, infiltrative masses exhibiting stronger hemosiderin deposition and more severe disease extents of articular, muscle, and tendon involvement than the localized type.

FNAC study of giant cell tumor of tendon sheath (localized tenosynovial giant cell tumor): Clinico-radiological correlation and cytopathological features.

BACKGROUND: Localized tenosynovial giant cell tumor (GCT) or giant cell tumor of tendon sheath (GCTTS), is a benign nodular lesion that arises from the synovium of the tendon sheath of the hands and foot. GCTTS is characterized by the presence of multinucleated giant cells and proliferation of synovial-like mononuclear cells. A clinical diagnosis of GCTTS is kept as a differential when a firm, nodular mass shows decreased signal intensity on both T1-and T2-weighted MR imaging. Treatment is usually marginal excision of the mass. MATERIAL AND METHODS: It is a retrospective study, observed in the past 3 years at a tertiary care hospital. Those cases were included in the study in which histopathological confirmation was available or if clinico-radiological features were confirmatory of the diagnosis of GCTTS when correlated with cytological features. RESULTS: There was a total of 24 cases, out of which 16 were females and 8 males. The tumor was located in the upper limb in 21 cases and in 3 cases the tumor was present in the lower limb. In the upper limb, 18 cases were on the right side and three cases were on the left side. In the lower limb, 1 case was present on the left and 2 on the right side. The cytomorphology consisted of mononuclear stromal cells, multinucleated giant cells, and hemosiderin-laden macrophages in variable numbers. CONCLUSION: It is important to accurately diagnose and categorize giant cell-containing lesions because their prognosis depends on the exact categorization of the tumor.

Tenosynovial giant cell tumours of the upper and lower cervical spine: two case reports.

INTRODUCTION: Tenosynovial giant cell tumours (TSGCTs) usually arise from the synovial membranes of tendon sheaths, bursa, and joints. They are rarely found in the spine. Lesions of the upper cervical spine (C1/2) are extremely rare, with only 13 previous cases reported in the literature. Of these, all previous anterior upper cervical cases (C1/2) have been deemed unresectable and have been managed with immunotherapy or radiological surveillance. CASE PRESENTATION: We report two cases of TSGCST in the cervical spine: one with a lesion at C1/2 and another at C6/7. DISCUSSION: The location of our C1/2 lesion was unique, allowing for a new endoscopic endonasal tissue biopsy method and a new transoral surgical approach for successful gross total resection. Our C6/7 lesion had a more typical location and was removed via a C6/7 laminectomy.

Tenosynovial Giant Cell Tumor Mimicking Acute Septic Arthritis of the Hip: A Case Report.

A 12-year-old boy presented to the pediatric emergency department with a 5-day history of atraumatic, progressively worsening right hip pain and inability to ambulate. He was afebrile and had elevated inflammatory markers (Erythrocyte Sedimentation Rate [ESR]: 42 mm/hr, C-Reactive Protein [CRP]: 6.6 mg/dL) with a normal white blood cell count of 6050 cells/mm3. Given the clinical concern for septic arthritis, joint aspiration of the right hip was done and demonstrated a bloody appearance with a WBC count of 54,999 cells/mm3 and RBC count of 7,000 cells/mm3. MRI of the right hip demonstrated an intra-articular mass suggestive of tenosynovial giant cell tumor/pigmented villonodular synovitis. Subsequent biopsy and excision of the mass confirmed the diagnosis. The acute presentation of tenosynovial giant cell tumor with features mimicking septic arthritis is uncommon. This rare presentation of an already uncommon diagnosis should be considered in a child with an equivocal presentation for severe hip pain because misdiagnosis may lead to unnecessary or inadequately planned surgical treatment of the condition.

Localized tenosynovial giant cell tumor: a rare case of snapping hip.

We report on a 40-year-old male with a 9-month-long history of snapping of his right hip caused by a previously undescribed etiology of internal extra-articular snapping hip, namely due to a localized tenosynovial giant cell tumor. Both dynamic ultrasound evaluation and MRI proved to be crucial in the diagnosis of this rare entity. Auto-provocation of the snapping showed an anterior hip mass moving posteriorly to the psoas tendon which elucidated the pain and clicking sensation. Subsequent MRI demonstrated a peripheral low-intensity rim due to hemosiderin deposition around the synovial mass which is indicative for pigmented villonodular tenosynovitis. Treatment consisted of arthroscopic shaver burr resection. Immediately postoperatively, the snapping sensation could not be provoked anymore by the patient. The purpose of reporting on this case report is to emphasize several successive learning points. First, dynamic ultrasound aids in diagnosis and differentiation of the types of snapping hip. Second, specific MRI features are suggestive of tenosynovial giant cell tumor, recognizing these traits may prevent delayed diagnosis and subsequent aggravated clinical course. Third, localized pigmented villonodular tenosynovitis around the hip may present as an internal extra-articular snapping hip and is of consideration in the differential diagnosis of recurrent snapping hip.