Reduced recurrence rate and comparable functionality after wide resection and reverse total shoulder arthroplasty with allograft-prosthetic composite versus curettage for proximal humeral giant cell tumor: a multicenter retrospective study.

BACKGROUND: Giant cell tumors of bone (GCTBs) are rare, aggressive tumors, and the proximal humerus is a relatively rare location for GCTBs; limited evidence exists on which surgical approaches and reconstruction techniques are optimal. In the largest case series to date, we evaluated the recurrence rate of proximal humeral GCTBs and the functional outcomes of different resection and reconstruction options in this multicenter study. METHODS: All 51 patients included in this study received initial surgical treatment for proximal humeral GCTBs from January 2007 to December 2020, with a minimum 2-year follow-up period. Local recurrence and functional outcomes were statistically analyzed in relation to demographic, clinical, and primary surgical variables. Functional outcomes were reported by patients and were assessed by the Musculoskeletal Tumor Society score and QuickDASH instrument (shortened version of the Disabilities of the Arm, Shoulder and Hand instrument). RESULTS: The mean follow-up period was 81.5 months (range, 30-191 months), and the overall recurrence rate was 17.6% (9 of 51 patients). The majority of recurrences (n = 7) occurred in the first 2 years of follow-up. The intralesional curettage group (n = 23) showed a statistically significant difference in the recurrence rate compared with the en bloc resection group (n = 28) (34.8% vs. 3.6%, P = .007). Among shoulders receiving en bloc resection, 16 were reconstructed with hemiarthroplasty; 8, reverse total shoulder arthroplasty (rTSA) with allograft-prosthetic composite (APC) reconstruction; and 4, arthrodesis. On the basis of intention-to-treat analysis, the mean functional Musculoskeletal Tumor Society scores of the groups undergoing curettage, rTSA with APC, hemiarthroplasty, and arthrodesis were 26.0 ± 3.1, 26.0 ± 1.7, 20.3 ± 2.8, and 22.5 ± 1.3, respectively (P < .001 [with P < .001 for curettage vs. hemiarthroplasty and P = .004 for rTSA with APC vs. hemiarthroplasty]) and the mean QuickDASH scores were 14.0 ± 11.0, 11.6 ± 4.5, 33.1 ± 11.8, and 21.6 ± 4.7, respectively (P < .001 [with P < .001 for curettage vs. hemiarthroplasty and P = .003 for rTSA with APC vs. hemiarthroplasty]). CONCLUSIONS: On the basis of our data, en bloc resection followed by reverse shoulder arthroplasty showed a lower recurrence rate and no significant difference in functional outcome scores for proximal humeral GCTBs compared with intralesional curettage. Therefore, we believe that rTSA with APC may be reasonable for the initial treatment of proximal humeral GCTBs.

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.

Uterine giant cell tumor of soft tissue: A case report.

INTRODUCTION: Giant cell tumor of soft tissue (GCT-ST) is a rare primary soft tissue tumor. GCT-ST mainly occurs in the trunk and extremities. There is no standard treatment for GCT-ST. This paper reports a rare case of primary uterine GCT-ST. CASE PRESENTATION: A 48-year-old female patient underwent a transabdominal subhysterectomy for uterine leiomyoma. Postoperative pathological examination showed GCT-ST with unclear tissue boundary (10.0 × 6.0 × 5.0 cm). A small amount of GCT-ST tissue could be seen on the local edge of the leiomyoma. Residual tumor tissue was found around the uterine cavity. The patient reported persistent lower abdominal distension pain 3 months after the operation. Pelvic and abdominal imaging showed a huge tumor and multiple pelvic and abdominal organ metastasis. No pulmonary metastasis was found. Exploratory surgery revealed widespread metastases in the abdominal and peritoneal cavities, involving both ovaries, right tubal serous membrane, appendix serous membrane, bladder, pelvic peritoneum, and abdominal wall incision. After surgery, the patient had 6 cycles of docetaxel and carboplatin but stopped treatments due to economic reasons. The patient died 3 months later because of multiple organs failure. CONCLUSION: GCT-ST is generally benign but has unpredictable behavior. A massive recurrence with wide invasion is possible after subtotal resection.

Partial sternectomy with reconstruction of a giant cell tumor of the sternum, a case report, Saudi, Arabia.

BACKGROUND: Giant cell tumor (GCT) is a relatively common and locally aggressive benign bone tumor that rarely affects the sternum. CASE PRESENTATION: We report a case of giant cell tumor of the sternum in a 28-year-old Saudi with painful swelling at the lower part of the sternum. Subtotal sternectomy and reconstruction with a neosternum using two layers of proline mesh, a methyl methacrylate prosthesis, and bilateral pectoralis muscle advancement flaps were performed. CONCLUSIONS: Giant cell tumor of the sternum is a rare diagnosis. Surgical resection with negative margins is the ideal management. To avoid defects or instability of the chest wall, reconstruction of the chest wall with neosternum should be considered.

A Mass on the Sole Revealing a Giant-Cell Tumor of the Tendon Sheath.

A 61-year-old woman presented with a 3-year history of painless soft-tissue mass on the right sole. The patient reported gradual growth, with a rapid increase in size over the past few months, leading to difficulty in walking. She had no history of past trauma. Examination revealed a 4-cm ovoid mass located over the ball of the foot. It was firm in consistency, with well-defined margins, a smooth surface, and an overlying normal skin (Figure 1). An ultrasound image revealed an eccentric, hypoechoic, nonvascular subcutaneous lobular mass. A magnetic resonance imaging (MRI) of the foot revealed a well-defined mass arising from the flexor tendon sheath of the right foot. The lesion was heterogeneously hyperin-tense on T1- and T2-weighted images with an avid contrast enhancement. All of the surrounding soft tissues indicated normal signal intensity patterns. There was no associated bony destruction. Histopathologic examination after complete excision of the mass established a well-circumscribed lesion composed of osteoclast-like giant cells and mononuclear cells in a hyalinized stroma, consistent with a giant cell tumor of the tendon sheath (GCT-TS) (Figure 2). There was no recurrence during a 6-month follow-up period (Figure 3).

Multimodal management of tenosynovial giant cell tumors (TGCT) in the landscape of new druggable targets.

Tenosynovial giant cell tumor (TGCT) is a rare, benign, locally aggressive synovial based neoplastic process that can result in functional debilitation and end-stage arthrtitis. Although surgical resection is the primary treatment modality, novel systemic therapies are emerging as part of the multimodal armamentarium for patients with unresectable or complex disease burden. This review discusses the pathogenesis of TGCT, potential druggable targets and therapeutic approaches. It also evaluates the safety and efficacy of different systemic therapies.

Tenosynovial giant cell tumour of the finger: a case report.

Giant cell tumour most commonly occuring in epiphysis of the long bone, present and with pain, tenderness and swelling. It is a solitary lesion with restricted movement and tenderness over the lesion. The tendon sheath is where tenosynovial giant cell tumours typically develop. Because of its remarkably peculiar position, we present a case of giant cell tumour (GCT) tenosynovial of bone in the middle phalaynx in a 33-year-old female with complaints of swelling, pain in ring finger of left hand since 2 months which is rarely seen. After clinical, radiological, pathological investigations tenosynovial giant cell tumour was diagnosed. Following fine needle aspiration cytology, histopathology was utilized to confirm the tumour's diagnosis which was later treated as resection of excision of the tumour with allo/autograft reconstruction. Our case report showed no evidence of recurrence in 2 years of follow-up. Hence our case report proves that early and complete resection of the tumour shows evidence of regain of complete range of motion and decrease recurrence rate.

Giant Cell Tumour of Tendon Sheath of Distal Phalanx of Thumb: A Case Report.

Giant cell tumour of tendon sheath is an uncommon benign soft tissue tumour. Histopathological examination plays a crucial role in the definitive diagnosis of giant cell tumour although pre-operative imaging supports its suspicion. We report a case of a giant cell tumour of the tendon sheath in a 26-year-old man as a painless, firm, localized, slow-growing benign soft tissue tumour of the thumb; managed by complete excision. The patient continues to do well at 7 months post-surgery with no complaints and no signs of recurrence. Giant cell tumour of the phalanges is a locally aggressive entity; therefore delayed or missed diagnosis of giant cell tumour especially of the thumb distal phalanx can be extremely debilitating. Hence, high degree of suspicion and early en bloc resection is the key to its management. Keywords: case reports; giant cell tumors; tendons; thumb.

3D-printed Titanium Prosthetic Reconstruction of Unilateral Bone Deficiency After Surgical Resection of Tumor Lesions in the Upper Cervical Spine: Clinical Outcomes of Three Consecutive Cases and Narrative Review.

MAIN POINTS: Operational excision of tumor lesions in the upper cervical spine remains a tremendous challenge to surgeons due to the local complex anatomic relationships. Meanwhile, no commercially available device has been specially designed to address bone deficiency after surgical resection. Here, we described the reconstruction of unilateral bone deficiency after surgical resection of a giant cell tumor of the tendon sheath originating from the lateral atlantoaxial joint with the employment of a 3D printing technique and reviewed the relevant literature. In our study, 3 patients with giant cell tumor of the tendon sheath in the upper cervical spine achieved complete tumor removal, and received unilateral bone reconstruction with one-armed 3D-printed titanium prosthesis. During the follow-up, these patients remained neurologically intact and got back to a normal life without wearing the braces. Images demonstrated the satisfactory placement of 3D-printed prosthesis with no failure of fixation and no subsidence. In addition, 6 articles describing the employment of 3D-printed prostheses or models for tumor surgery in the upper cervical spine were reviewed, and satisfactory clinical outcomes were reported in these studies. Hence, 3D-printed titanium prosthetic reconstruction of bone deficiency in the upper cervical spine was a safe and effective technique. LEVEL OF EVIDENCE: Level IV.

Giant cell tumor of the patellar tendon sheath in childhood: case report.

The aim of this study was to report a rare case of a giant cell tumor of the patellar tendon sheath. This indicates the diagnostic procedures and treatment options for giant cell tumors of the patellar tendon. This study reported a case of a 13-year-old male patient with a giant cell tumor of the tendon sheath. In our case, open arthrotomy was performed with complete surgical excision of the lesion. Histopathological examination revealed a giant cell tumor. At the last follow-up, 2 years after surgery, no complications were reported. The giant cell tumor of the patellar tendon sheath is an uncommon benign tumor. It mimics common knee symptoms. A differential diagnosis is definitely a challenge. Available operation approaches have demonstrated similar results, which lead to symptom relief and a low recurrence rate.

Complications and prognosis of primary thoracic and lumbar giant cell tumors treated by total tumor resection.

BACKGROUND: Spinal giant cell tumor (SGCT) is a relatively rare primary tumor. En bloc resection is the preferred surgical procedure for it due to its aggressiveness, meanwhile leading to more complications. We reported the characteristics of perioperative complications and local control of total tumor resection including en bloc resection and piecemeal resection for primary thoracic and lumbar spinal giant cell tumors in a single center over 10 years. METHODS: This is a retrospective cross-sectional and cohort study. Forty-one consecutive patients with SGCTs who underwent total tumor resection from 2010 to 2020 at our institution and were followed up for at least 24 months were reviewed. Surgery data, complication characteristics and local tumor control were collected and compared by different surgical procedure. RESULTS: Forty-one patients were included, consisting of 18 males and 23 females, with a mean age of 34.2 years. Thirty-one had thoracic vertebra lesions, and 10 had lumbar vertebra lesions. Thirty-five patients were primary cases, and 6 patients were recurrent cases. Eighteen patients were treated by total en bloc spondylectomy (TES), 12 patients underwent en bloc resection according to WBB surgical system, and 11 patients underwent piecemeal resection. The average surgical time was 498 min, and the mean estimated blood loss was 2145 ml. A total of 58 complications were recorded, and 30 patients (73.2%) had at least one perioperative complication. All patients were followed up after surgery for at least 2 years. A total of 6 cases had postoperative internal fixation failure, and 4 cases presented local tumor recurrence (9.8%). CONCLUSIONS: Although the surgical technique is difficult and accompanied by a high rate of perioperative complications, en bloc resection can achieve favorable local control in SGCT. When it is too difficult to complete en bloc resection, thoroughly piecemeal resection without residual is also acceptable, given the relatively low recurrence rate.

Rare Presentation of Giant Cell Tumors of the Tendon Sheath: Bilateral Involvement of Tendoachilles.

Giant cell tumor of tendon sheath arises from the synovium of tendon sheaths, joints, or bursa, mostly affects adults between 30 and 50 years of age, and is slightly more common in females. It corresponds to a localized form of pigmented villonodular synovitis (PVNS). Typically occur in the hand where they represent the second most common type of soft tissue tumors after synovial ganglions. Bilateral giant cell tumor of tendon sheath of tendoachilles is a rare presentation. We report the case of a 22-years-old female presenting with pain in both ankles without any history of trauma. On clinical examination, tenderness on both tendoachilles and local indurations were observed. Ultrasonography showed focal thickening of Achilles tendon bilaterally, and Doppler demonstrated increased flow in peritendinous area. MRI findings showed that most of the tumor had intermediate signal intensity and portions of the tumor had low signal intensity. Fine needle aspiration cytology confirmed the diagnosis of giant cell tumor of tendon sheath. Excision biopsy was done with no recurrence on subsequent follow-up.

Outcomes of Surgery for Benign Tumours in The Upper Extremity.

Benign tumours of the upper extremity are common in hand surgeons' practice. The most commonly diagnosed are giant-cell tumours of the tendon sheath and lipomas. THE OBJECTIVE: of this study was an investigation into the distribution of tumours in the upper limb, their symptomatology and outcomes of surgery, particularly regarding the rate of recurrence. MATERIAL AND METHODS: A total of 346 patients, 234 women (68%) and 112 men (32%), who had undergone surgery for tumours located in the upper extremity which were not ganglion cysts were enrolled into the study. The follow-up assessment was performed at a mean of 21 months (range 12-36) post-operatively. RESULTS: The most common tumour in this study was giant cell tumour of the tendon sheath - 96 cases (27.7%), followed by lipoma - 44 cases (12.7%). Most lesions - 231 (67%) were localized in the digits. A total of 79 (23%) recurrences were noted, the most common after surgery for rheumatoid nodules - 43.3% and the giant-cell tumours of the tendon sheath - 31.3%. The independent factors increasing risk of recurrence following the tumour's resection were: histological type of the lesion - the giant-cell tumour of the tendon sheath (p=0.0086) and the rheumatoid nodule (p=0.0027), as well as a combination of incomplete (non-radical) and not "en block" resection of tumours. A brief review of the literature referring to the presented material is offered.

Comparison of the prognostic factors of total en bloc spondylectomy and total piecemeal spondylectomy in patients with Enneking stage III giant cell tumor in the thoracic and lumbar spine.

PURPOSE: To compare total en bloc spondylectomy with marginal margins against piecemeal spondylectomy with intralesional margins in the surgical treatment of Enneking stage III spinal giant cell tumor (GCT) in terms of local recurrence. METHODS: A retrospective survival analysis of patients with Enneking stage III GCT who underwent TES with marginal margins or total piecemeal spondylectomy with intralesional margins was performed between January 2006 and April 2020. Local recurrence-free survival (LRFS) was the time between the date of surgery and recurrence. Factors with p-values < 0.05 in the univariate analysis were included in the multivariate analysis using proportional hazard analysis. RESULTS: Sixty patients (25 men and 35 women) with a mean age of 35.6 (range 11-71) years were included. The mean follow-up duration was 93 (range 24-198) months. Two patients were lost to follow-up 6 and 14 years after the procedure. Over a 10-year period, the recurrence rate was 13.3%. The 2-, 5-, and 10-year LRFS rates were 95%, 88%, and 78%, respectively. Univariate analysis identified total piecemeal spondylectomy and no adjuvant radiotherapy as prognostic factors for LRFS. Multivariate Cox-regression models showed a significant association between local recurrence and total piecemeal spondylectomy and no adjuvant radiotherapy. CONCLUSION: TES with marginal margins is better than total piecemeal spondylectomy with intralesional margins owing to its lower postoperative recurrence rate. Adjuvant radiotherapy should be administered to reduce postoperative recurrence rates.

The exploration of surgical outcomes in patients with giant cell tumor of the tendon sheath in spine: An epidemiological, radiological, and follow-up investigation.

PURPOSE: This study aimed to characterize giant cell tumor of the tendon sheath (GCTTS) in spine more fully and further validate the therapeutical effect of surgcial resection for treating this neoplasm. METHODS: Patients diagnosed with spinal GCTTS and received surgical resection in our hospital between January 2009 and September 2021 were identified retrospectively. The clinical data and radiological images were summarized and the clinical outcomes of patients with a follow-up period of more than 12 months were analyzed. RESULTS: Thirty patients with benign GCTTS and one with malignant GCTTS were included. Preoperative radiological images were available in 28 of 30 benign cases. Benign lesions were revealed as soft tissue masses centering on the facet joint with osteolytic bone destruction in 26 patients on CT, and as prevertebral or intramuscular masses without bone erosion in 2. MRI showed the signal of isointensity or hypointensity on T1 weighted images (T1WI) in 25 patients and slightly hyperintense in three. On T2 weighted images (T2WI), 17 lesions displayed homogeneous hypointense signal, and eight lesions possessed heterogeneous signals. The remaining three lesions featured slightly hyperintense signal on T2WI. Follow-up data were available in 23 of 30 benign cases treated with gross-total resection, and two patients experienced recurrence. CONCLUSIONS: Spinal GCTTS should be suspected in cases with features such as the mass mainly involving the posterior bone elements, the lack of intralesional calcification, T2-weighted dark signals, and free of any cancer. Gross-total resection is an effective means for treating spinal GCTTS.

Tenosynovial giant cell tumor.

Tenosynovial Giant Cell Tumor (TGCT) is a group of typically benign lesions arising from the synovium of joints, bursae and tendon sheaths. Depending on their growth pattern and clinical course, they are divided into localized and diffuse types. It is predominantly caused by a mutation in the stromal cells of the synovial membrane leading to overexpression of the colony stimulating factor 1 that recruits CSF1R-expressing cells of the mononuclear phagocyte lineage into the tumor mass. The lesions contain mainly histiocyte-like and synovial cells accompanied by varying numbers of multinucleated giant cells, mononuclear cells, foam cells, inflammatory cells and hemosiderin deposits. The gold standard for detect- ing and monitoring the disease is MRI, where the characteristic hemosiderin accumulation can be best appreciated, but it is a histological examination that is most conclusive. The main treatment is surgical resection of all pathological tissue, but radio- and chemotherapy are also viable options for certain groups of patients.

Predictors of Functional Recovery among Musculoskeletal Oncology Patients Undergoing Lower Extremity Endoprosthetic Reconstruction.

BACKGROUND AND OBJECTIVES: Functional outcomes are important for oncology patients undergoing lower extremity reconstruction. The objective of the current study was to describe patient reported function after surgery and identify predictors of postoperative function in musculoskeletal oncology patients undergoing lower extremity endoprosthetic reconstruction. METHODS: We performed a cohort study with functional outcome data from the recently completed Prophylactic Antibiotic Regimens in Tumor Surgery (PARITY) trial. We utilized the 100-point Toronto Extremity Salvage Score (TESS), which was administered pre-operatively and at 3, 6 and 12 months post-operatively. Higher scores indicate better physical functioning, and the minimally important difference is 11 points. We calculated mean functional scores at each timepoint after surgery and developed a logistic regression model to explore predictors of failure to achieve excellent post-operative function (TESS ≥ 80) at 1 year after surgery. RESULTS: The 555 patients included in our cohort showed important functional improvement from pre-surgery to 1 year post-surgery (mean difference 14.9 points, 95%CI 12.2 to 17.6; p < 0.001) and 64% achieved excellent post-operative function. Our adjusted regression model found that poor (TESS 0-39) pre-operative function (odds ratio [OR] 3.3, 95%CI 1.6 to 6.6); absolute risk [AR] 24%, 95%CI 8% to 41.2%), older age (OR per 10-year increase from age 12, 1.32, 95%CI 1.17, 1.49; AR 4.5%, 95%CI 2.4% to 6.6%), and patients undergoing reconstruction for soft-tissue sarcomas (OR 2.3, 95%CI 1.03 to 5.01; AR 15.3%, 95%CI 0.4% to 34.4%), were associated with higher odds of failing to achieve an excellent functional outcome at 1-year follow-up. Patients undergoing reconstruction for giant cell tumors were more likely to achieve an excellent functional outcome post-operatively (OR 0.40, 95%CI 0.17 to 0.95; AR -9.9%, 95%CI -14.4% to -0.7%). CONCLUSIONS: The majority of patients with tumors of the lower extremity undergoing endoprosthetic reconstruction achieved excellent function at 1 year after surgery. Older age, poor pre-operative function, and endoprosthetic reconstruction for soft tissue sarcomas were associated with worse outcomes; reconstruction for giant cell tumors was associated with better post-operative function. LEVEL OF EVIDENCE: Therapeutic Level IV.

Is pancreatic giant cell tumor resistant to standard chemotherapy?

Pancreatic giant cell tumors (PGCTs), undifferentiated pancreatic carcinoma are rare tumors of the pancreas. PGCTs consist of osteoclastic, pleomorphic and mixed variants. PGCT is usually diagnosed at an advanced stage. PGCT has a worse prognosis than pancreatic ductal adenocarcinoma. Although surgery can be curative, there is no standard treatment approach for advanced PGCT. We present a case of PGCT that is resistant to standard therapy and progresses in a short time.