We present a case of adamantinoma that originated from the fibula and had a large soft tissue component measuring approximately 6 cm. Clinical, radiological, and pathological investigations initially suggested that the tumor might be a bone-invading synovial sarcoma. To the best of our knowledge, no other case of fibular adamantinoma with such a large soft tissue component has been reported in the literature.

OBJECTIVE: Spinal chordoma treatment guidelines recommend resection. However, in patients in whom gross-total resection (GTR) is achieved, the benefits of radiation therapy (RT) are unclear. Therefore, the authors performed a systematic review to determine if RT is associated with postoperative progression-free survival (PFS) or overall survival (OS) after achieving GTR of spinal chordoma.
METHODS: The PubMed database was searched for studies including individualized data of patients undergoing GTR with or without RT for spinal chordoma. Patients < 18 years of age or those who underwent stereotactic body RT were excluded. Qualitative assessment was performed using Newcastle-Ottawa Scale guidelines. Log-rank tests for time-to-event data and a Cox proportional-hazards model were generated for a multivariable statistical model.
RESULTS: Complete data of 132 patients were retrieved, with 37 (28%) patients receiving adjuvant RT and 95 (72%) not receiving adjuvant RT. The mean follow-up was not statistically significantly different between those undergoing RT and not undergoing RT (54.02 months and 65.43 months, respectively). Patients were more likely not to undergo RT if their disease was located in the sacrum versus the mobile spine (p < 0.001). When controlling for age ≥ 65 years, male sex, disease location, and treatment year ≥ 2010, patients undergoing RT had similar PFS and OS when compared with those not undergoing RT on multivariable survival analysis (HR 0.935 [95% CI 0.703-2.340], p = 0.844 and HR 2.078 [95% CI 0.848-5.090], p = 0.110, respectively). However, age ≥ 65 years was associated with poorer OS in adjusted analyses (HR 2.761 [95% CI 1.185-6.432], p = 0.018) relative to patients < 65 years of age.
CONCLUSIONS: After achieving GTR of spinal chordoma, the utility of RT on PFS and OS remains unclear. Age ≥ 65 years appears to be associated with OS in spinal chordoma patients. Additional multicenter prospective studies are needed to determine the utility of RT in this patient population.

Background: Nowadays, limb-sparing procedures are the gold standard in the treatment of soft-tissue sarcomas of the limbs. Wide tumor resection with appropriate oncological margins, reconstruction, and stabilization of the involved bone and joint and restoration of the soft tissue lost are essential in order to obtain good clinical and functional outcomes. Tumor excision and soft-tissue reconstruction performed in one-step surgery is chosen by many centers as the preferred approach; however, according to our experience in some selected patients, two-step surgery performed using a dermal regeneration template first and then a margin revision, taking into account the definitive results of the anatomopathological exam conducted over the surgical specimen from the previous surgery, associated with definitive reconstruction surgery over a healthy bed of granulated tissue, showed many potential benefits. Methods: A retrospective observational study was conducted on thirteen patients who underwent a two-step reconstruction procedure using dermal substitution after soft-tissue sarcoma excision. Results: Clinically, the enrolled patients achieved excellent contour and cosmesis of their surgical wounds, with a mean VSS value of 3.07. During the follow-up period, no local recurrences were observed in any patient. Conclusions: Two-step surgery represents the most suitable solution to allow surgical radicality with minimal recurrency and adequate soft-tissue reconstruction, avoiding the possibility of wasting autologous tissue. Our patients generally embraced this approach and the management that followed.

UNASSIGNED: The management of distal radius giant cell tumors (GCTs) remains challenging, and the optimal approach is still a matter of debate. This systematic review and meta-analysis aimed to compare the outcomes of extended curettage and wide resection, the mainstays of treatment.
UNASSIGNED: Medline (via PubMed), Cochrane Library, Web of Science, Google Scholar, ClinicalTrials.gov, and Embase databases were searched for comparative studies that assessed extended curettage with adjuvant therapy and wide resection with reconstruction in patients with GCTs of the distal radius up to April 2023. Data were collected and analyzed on rates of local recurrence, metastasis, overall complications, and functional outcomes. The Newcastle-Ottawa scale was used to appraise the risk of bias within each study.
UNASSIGNED: Fifteen studies (n = 373 patients) were included and analyzed. Patients who underwent curettage were more likely to develop recurrence (risk ratio [RR] = 3.02 [95% confidence interval; CI, 1.87-4.89], P < .01), showed fewer complications (RR = 0.32 [95% CI, 0.21-0.49], P < .01), and showed greater improvement in Visual Analog Scale and lower Disabilities of the Arm, Shoulder, and Hand scores (P < .00001) than those who underwent wide resection. No significant difference was found regarding metastasis (RR = 1.03 [95% CI, 0.38-2.78], P = .95).
UNASSIGNED: Regarding the surgical approach to GCT of the distal radius, curettage with adjuvant therapy was associated with a higher likelihood of recurrence compared with wide resection with reconstruction. Nevertheless, the curettage approach resulted in significantly lower rates of operative complications, decreased pain scores, and better functional outcomes in comparison to the resection group.

Walking ability may be fairly well maintained after sciatic nerve resection combined with wide resection of soft tissue sarcoma, therefore, surgeons should not hesitate to perform sciatic nerve resection to achieve an adequate surgical margin.

Low-grade myofibroblastic sarcoma (LGMS) is a rare soft-tissue malignant neoplasm originating from the stromal cells that is predominantly comprised of atypical myofibroblasts. The present study reports the case of a patient with low-grade myofibroblastic sarcoma in the soft tissue of the knee. The patient exhibited a soft, painless mass on the medial side of the left knee. During surgery, a yellow-white mass above the posterior margin of the sartorius muscle was identified. This mass, which was irregular and adherent to surrounding tissues, had a blurry boundary, indicative of invasive growth. The morphology of the mass significantly differed from conventional lipomas and was lacking typical features. Immunohistochemical staining confirmed the diagnosis of a malignant LGMS. The patient experienced no recurrence over 1 year of follow-up and reported complete recovery of knee range of motion. The present study suggests that an incisional biopsy may be performed if LGMS is suspected based on patient symptoms and imaging results. In addition, long-term follow-up is crucial for the timely detection and management of any recurrence, playing a key role in the patient\'s ongoing care and treatment.

Malignant peripheral nerve sheath tumors are soft tissue sarcomas originating from peripheral nerves. They are more frequently diagnosed in individuals with neurofibromatosis and tend to affect young men more often than women. The most common sites for these tumors within the peripheral nerve sheath are in the pelvis and the distal femur. Although chemotherapy and radiotherapy are not frequently used, it should be noted that in some cases, postoperative radiotherapy and chemotherapy may be beneficial. The primary treatment approach typically involves the complete surgical removal of the tumor. Here, we discuss the case of our patient whom we successfully treated with extensive resection and followed up with postoperative radiotherapy at our clinic.

BACKGROUND: Details of improved gait ability after wide resection of soft tissue sarcomas that necessitate removal of portions of the quadricep muscle have not yet been reported. We describe a patient with improved gait ability following a rehabilitation program after wide resection of a soft tissue sarcoma that included four components of the quadricep muscle.
METHODS: An 85-year-old Japanese man underwent wide resection of an undifferentiated pleomorphic sarcoma that included portions of the quadriceps femoris muscle. The rectus femoris, vastus medialis, sartorius, and vastus intermedius were separated in the maximally bulging region of the tumour. Three weeks postoperatively, gait exercise was initiated using a rigid knee orthosis with a dual-adjustable lock knee. The contraction loading of the knee extension muscle was controlled by adjusting the hinge motion range of the orthosis as follows: fully extended, fixed knee 0°-30°, and free range. Under this regimen, he could walk independently without a rigid orthosis within 5 weeks postoperatively but could not sit on his heels during daily living activities. At six months, there was no clinical evidence of recurrent tumours or complications.
CONCLUSIONS: Postoperative gait ability might be affected by not only the number of resected muscles but also by the function of the separated muscles and the cross-sectional area of the remaining muscle. Gradually loaded exercise of the knee extension muscles using an orthosis could result in an improved gait motion for patients who undergo wide resection of a sarcoma that includes four components of the quadriceps femoris.

Surgical principles in sarcoma are a wide resection, including surrounding tissues and maximisation of the function of the affected limb. Rotator cuff muscles are biomechanically important structures acting as a force couple in movement of the shoulder joint. Thus, conjoined tendons are essential for motion capability in absence of the supraspinatus muscle. This article reports a case of a large undifferentiated pleomorphic sarcoma (UPS) at the suprascapular fossa in a 78-year-old man. After diagnosis of sarcoma, he underwent wide, en-bloc excision preserving conjoined tendons of rotator cuff muscles and low-dose radiation therapy for surveillance of local recurrence. All dissection was performed to avoid contaminating the tumour and involved the whole supraspinatus except the conjoined tendons. We report a case of UPS at the suprascapular fossa, which showed a good result after a wide resection preserving conjoined tendons of rotator cuff muscles. Level of Evidence: Level V (Therapeutic).

Giant cell tumor (GCT) of the bone is a benign, locally aggressive neoplasm of epiphyseal origin. Most common sites for GCTs include the distal femur, proximal tibia, and the distal end of radius with the distal humerus being involved rarely. GCT is predominantly managed by extended curettage followed by adjuvant therapy to reduce recurrence. Juxta-articular GCTs are difficult to manage due to the destruction of the articular cartilage and subchondral bone which necessitates the need for joint reconstruction or fusion to salvage the joint. Aggressive and recurrent GCTs can be managed by wide resection of the tumor to reduce local recurrence followed by joint reconstruction or fusion. Joint reconstruction using a total elbow arthroplasty has been described for limb salvage as it provides a good functional outcome. We present a case of an aggressive GCT of the distal humerus that was treated using wide resection with humero-ulnar arthrodesis as an alternative in situations where joint reconstruction is not possible due to the unavailability of the prosthesis or socio-economic factors. The patient was asymptomatic after two years of follow-up, had no signs of recurrence, and had good hand functions.