Citations

Diffuse-type tenosynovial giant cell tumors’ (D-TGCTs) intra- and extra-articular expansion about the knee often necessitates an anterior and posterior surgical approach to facilitate an extensive synovectomy. There is no consensus on whether two-sided synovectomies should be performed in one or two stages. This retrospective study included 191 D-TGCT patients from nine sarcoma centers worldwide to compare the postoperative short-term outcomes between both treatments. Secondary outcomes were rates of radiological progression and subsequent treatments. Between 2000 and 2020, 117 patients underwent one-stage and 74 patients underwent two-stage synovectomies. The maximum range of motion achieved within one year postoperatively was similar (flexion 123–120°, p = 0.109; extension 0°, p = 0.093). Patients undergoing two-stage synovectomies stayed longer in the hospital (6 vs. 4 days, p < 0.0001). Complications occurred more often after two-stage synovectomies, although this was not statistically different (36% vs. 24%, p = 0.095). Patients treated with two-stage synovectomies exhibited more radiological progression and required subsequent treatments more often than patients treated with one-stage synovectomies (52% vs. 37%, p = 0.036) (54% vs. 34%, p = 0.007). In conclusion, D-TGCT of the knee requiring two-side synovectomies should be treated by one-stage synovectomies if feasible, since patients achieve a similar range of motion, do not have more complications, but stay for a shorter time in the hospital.

AbstractBackgroundCompared to other sarcomas, myxoid liposarcoma (ML) is known to be radiosensitive, with improved oncologic outcomes. Although these tumors “shrink” following radiotherapy, there is a paucity of data examining the degree of radiosensitivity and oncologic outcome. The purpose of the study was to evaluate pre‐ and postradiotherapy tumor volume to determine if size reduction impacts outcome.MethodsWe reviewed 62 patients with ML undergoing surgical resection combined with preoperative radiotherapy, with pre‐ and postradiotherapy MRI. This included 34 (55%) males, with a mean age of 47 ± 14 years. All tumors were deep to the fascia, and 12 (19%) patients had tumors with a >5% round‐cell component.ResultsThe mean volume reduction was 54% ± 29%. Compared to patients with >25% volume reduction, patients with reduction ≤25% had worse 10‐year disease specific survival (86% vs. 37%, p < 0.01), in addition to an increased risk of metastatic disease (HR 4.63, p < 0.01) and death due to disease (HR 4.52, p < 0.01).ConclusionLack of volume reduction is a risk factor for metastatic disease and subsequent death due to disease in patients with extremity ML treated with combined preoperative radiotherapy and surgery. This data could be used to stratify patients for adjuvant therapies and follow‐up intervals.

AbstractPatients with soft tissue tumors of the lower extremities are at greater risk to develop postoperative disruption of lymphatic vessels. Currently, there is no widely effective cure for lymphatic dysfunction. Therefore, the best strategy is to prevent it and reconstruct efficient drainage as soon as the original pathway is damaged. We present a report of three prophylactic LVA cases after sarcoma resection in the lower limb, and a literature review to show the feasibility of prophylactic LVAs. The patients were 35, 73, and 77 years old, respectively, at the time of the procedure. All three patients had sarcoma in the medial thigh and underwent radiation therapy before the surgery. The locations of the LVAs include the medial thigh and medial and lateral calf. During the surgery, methylene blue and/or indocyanine green were injected to identify lymphatic vessels. Postoperative recovery was uneventful immediately after the surgery. At follow‐up visits, all three patients reported improved functions with no significant swelling in the lower limb. One patient experienced a surgical wound infection that resolved after antibiotic admission. Two patients had a history of cardiac diseases, a major risk factor for developing postoperative lymphedema, but these two patients did not develop lymphedema with the treatment of prophylactic LVAs. These results suggest that prophylactic LVA may be an effective strategy to prevent secondary lymphedema after sarcoma resection. Further investigation is warranted.

AbstractSegmental loss of the sciatic nerve secondary to oncologic resection or trauma is detrimental to hamstring and leg function. The diameter of this nerve and the length of its axons spanning the lower extremity create significant challenges in reconstruction and optimizing return of sensory or motor function. The purpose of this report is to describe outcomes of a free vascularized sural nerve graft to preserve hamstring function in a large proximal sciatic nerve defect beginning at the greater sciatic foramen. A 44‐year‐old female underwent neoadjuvant chemotherapy and radiation for treatment of a left sciatic nerve synovial cell sarcoma. The patient underwent R0 resection of the proximal left sciatic nerve resulting in a 15 cm defect. An ipsilateral vascularized sural nerve graft was used to reconstruct the medial aspect of the sciatic nerve, prioritizing the tibial division, in an effort to restore hamstring function and plantar sensation. A 5 cm allograft nerve was added to the cutaneous branches of the sural nerve graft to better span the large defect and reconstruct the lateral aspect of the nerve. The patient's postoperative course was uneventful. At 1‐year follow‐up, the patient showed MRC grade 4/5 strength with knee flexion and steady gait pattern with a left ankle‐foot orthosis. Outcomes support the use of a single vascularized nerve graft alongside acellular nerve allograft to restore motor function in large diameter and large defect mixed nerve injuries.

AbstractBackgroundMerkel cell carcinoma (MCC) is a rare, aggressive cutaneous malignancy that usually occurs in the head/neck or extremities. However, there are reports of MCC developing in the lymph nodes or parotid gland without evidence of a primary cutaneous lesion.MethodsWe reviewed 415 patients with biopsy‐proven MCC. Patients with MCC of unknown primary (n = 37, 9%, MCCUP) made up the study cohort. The primary endpoints of the study were rate of recurrence, disease‐free survival, and overall survival.ResultsPatients with MCCUP presented with tumors in lymph nodes (n = 34) or parotid gland (n = 3). Nodal disease was most commonly detected in the inguinal/external iliac (n = 15) or axillary (n = 14) regions. The mean age at diagnosis was 70 years and 24% were female. Patients presented with distant metastases in 24.3% of cases. Patients with stage IIIA disease treated with regional lymph node dissection (RLND) had a lower risk of disease recurrence (hazard ratio 0.26, p = 0.046). Recurrence‐free survival was 59.3% at 5 years. Disease‐specific survival was 63.3% at 5 years.ConclusionPatients with MCCUP have a high risk of recurrence and mortality. The optimal treatment for MCCUP has yet to be elucidated, although therapeutic RLND appears beneficial for these patients.

AbstractIntroductionThe distal radius is a common location for giant cell tumors (GCTs) of bone. Management includes intralesional curettage or wide excision, however, long‐term comparisons of treatment options are limited. The purpose of the current study was to evaluate our institutions' outcomes of treatment of these tumors.MethodsWe reviewed 24 GCT of the distal radius in 23 patients (12 males: 11 females) with a mean age of 42 years at the time of surgery. Functional outcomes were collected including the Musculoskeletal Tumor Society Score (MSTS), QuickDash, the Visual Analog Scale (VAS), and the Patient Rated Wrist Evaluation (PRWE). The mean follow‐up was 13 years.ResultsTumor grade included Campanacci Grade II (n = 14) and Grade III (n = 10). Treatment included extended intralesional curettage (n = 16) and wide excision (n = 8). Reconstruction mainly included bone grafting/cement (n = 16) or free vascularized fibula radiocarpal arthrodesis (n = 5). At most recent follow‐up, there was no difference in MSTS, VAS, and PRWE (p > 0.05) between patients undergoing a joint sparing or arthrodesis. Patients undergoing arthrodesis had a lower QuickDASH score (13.7 vs. 20.8, p = 0.04)ConclusionsTreatment for GCT of the distal radius is individualized however in the setting of articular surface involvement, arthrodesis can lead to superior functional results at long‐term follow‐up.

Aims Socioeconomic and racial disparities have been recognized as impacting the care of patients with cancer, however there are a lack of data examining the impact of these disparities on patients with bone sarcoma. The purpose of this study was to examine socioeconomic and racial disparities that impact the oncological outcomes of patients with bone sarcoma. Methods We reviewed 4,739 patients diagnosed with primary bone sarcomas from the Surveillance, Epidemiology and End Results (SEER) registry between 2007 and 2015. We examined the impact of race and insurance status associated with the presence of metastatic disease at diagnosis, treatment outcome, and overall survival (OS). Results Patients with Medicaid (odds ratio (OR) 1.41; 95% confidence interval (CI) 1.15 to 1.72) and uninsured patients (OR 1.90; 95% CI 1.26 to 2.86) had higher risks of metastatic disease at diagnosis compared to patients with health insurance. Compared to White patients, Black (OR 0.63, 95% CI 0.47 to 0.85) and Asian/Pacific Islander (OR 0.65, 95% CI 0.46 to 0.91) were less likely to undergo surgery. In addition, Black patients were less likely to receive chemotherapy (OR 0.67, 95% CI 0.49 to 0.91) compared to White patients. In patients with chondrosarcoma, those with Medicaid had worse OS compared to patients with insurance (hazard ratio (HR) 1.65, 95% CI 1.06 to 2.56). Conclusion In patients with a bone sarcoma, the cancer stage at diagnosis varied based on insurance status, and racial disparities were identified in treatment. Further studies are needed to identify modifiable factors which can mitigate socioeconomic and racial disparities found in patients with bone sarcomas. Cite this article: Bone Joint Res 2022;11(5):278–291.

Radiation-associated sarcoma of the pelvis and/or sacrum (RASB) is a rare but challenging disease process associated with a poor prognosis. We hypothesized that patients with RASB would have worse surgical and oncologic outcomes than patients diagnosed with primary pelvic or sacral bone sarcomas. This was a retrospective, multi-institution, comparative analysis. We reviewed surgically treated patients from multiple tertiary care centers who were diagnosed with a localized RASB. We also identified a comparison group including all patients diagnosed with a primary localized pelvic or sacral osteosarcoma/spindle cell sarcoma of bone (POPS). There were 35 patients with localized RASB and 73 patients with POPS treated with surgical resection. Patients with RASB were older than those with POPS (57 years vs. 38 years, p < 0.001). Patients with RASB were less likely to receive chemotherapy (71% for RASB vs. 90% for POPS, p = 0.01). Seventeen percent of patients with RASB died in the perioperative period (within 90 days of surgery) as compared to 4% with POPS (p = 0.03). Five-year disease-specific survival (DSS) (31% vs. 54% p = 0.02) was worse for patients with RASB vs. POPS. There was no difference in 5-year local recurrence free survival (LRFS) or metastasis free survival (MFS). RASB and POPS present challenging disease processes with poor oncologic outcomes. Rates of perioperative mortality and 5-year DSS are worse for RASB when compared to POPS.

Background: Osseous reconstruction following total spondylectomy/vertebrectomy in the thoracic spine is indicated to restore spinal stability. To assist with bony reconstruction, vascularized bone flaps including free vascularized fibula flaps and pedicled rib flaps can be used. However, there are limited data comparing various techniques. The authors aimed to evaluate the outcomes of free vascularized fibula flaps and pedicled rib flaps used for thoracic spinal reconstruction. Methods: The authors reviewed 44 vascularized bone flaps [10 anterior pedicled rib flaps, 25 posterior pedicled rib flaps, and nine vascularized fibula flaps] for corpectomy defects spanning T1 to L1 between January of 1999 and June of 2018. Mean age and follow-up were 46 ±17 years and 74 ± 52 months, respectively. Results: The union rate was 93 percent, with a similar mean time to union among the three groups: free vascularized fibula flaps, 9 ± 4 months; anterior pedicled rib flaps, 9 ± 6 months; and posterior pedicled rib flaps, 9 ± 5 months (p = 0.95). Surgical-site complications were found in 27 vascularized bone flaps (61 percent), and reoperations and revisions were performed in 14 (32 percent) and 10 (23 percent) vascularized bone flaps, respectively. No differences were identified among anterior pedicled rib flaps, posterior pedicled rib flaps, and vascularized fibula flaps with regard to complication, reoperation, and revision rates. Conclusions: Free vascularized fibula flaps and pedicled rib flaps provide durable reconstruction for thoracic spinal defects. Union, time to union, revision, and reoperation rates were similar among anterior and posterior pedicled rib flaps and vascularized fibula flaps. Given the variability in indication and defect types among the flap cohorts, the authors’ results should be interpreted carefully as an insight into the outcomes of different vascularized bone flaps for the unique cases of thoracic spinal reconstruction rather than to compare the different flaps used. CLINICAL QUESTION/LEVEL OF EVIDENCE: Therapeutic, III.

Summary: Hand aneurysms in infants are extremely rare and, unlike their adult counterparts, cannot be explained by repetitive trauma to the palm. When they occur, they are most often located in the ulnar artery. Usually there is no history of trauma, and an alternative diagnosis like malignancy must be excluded. Helpful physical findings to aid diagnosis include rapid appearance of a mass over the course of a few days, pulsatile nature, and location along the ulnar side of the hand. These cases can be challenging and excisional biopsy may be required if the lesion is thrombosed and does not have a characteristic appearance on imaging. Aneurysms located in the palmar arch or common digital arteries are usually treated with simple excision, while those located in the ulnar artery often require repair or reconstruction. Here we present a unique case of an infant with a pseudoaneurysm involving the superficial palmar arch on the radial side of the hand with a likely congenital etiology. Diagnostic challenges and treatment options are discussed.