Background: Malignant peripheral nerve sheath tumor is a rare and aggressive form of sarcoma that arises from a peripheral nerve, mostly in association with neurofibromatosis type 1. Half of the cases were reported in the extremities, with the lungs being the most common site of metastasis. We report a rare case of successful limb salvage surgery performed for a large exophytic malignant peripheral nerve sheath tumor of the right forearm with wide surgical margins followed by split-thickness skin graft and later a flexor carpi radialis tendon transfer to extensor digitorum communis tendon. Case presentation: A 51-year-old Bedouin Arabic man presented to our institution with an incompletely excised, large, fungating, malignant peripheral nerve sheath tumor occupying most of his right forearm. Staging imaging showed multiple lung nodules. He underwent wide local excision followed by skin graft and tendon transfer as a palliative measure to preserve the function of his dominant limb. The operation was performed without any complications, and the patient had an excellent postoperative result. Afterward, he was started on multiple lines of chemotherapy that failed because of disease progression, and the patient died 7 months after the operation. Conclusion: Clinicians must consider the possibility of soft tissue sarcoma even in a patient with a small, slow-growing, superficial mass. Furthermore, a wrong open biopsy or nononcological surgical procedure may lead to possible contamination and ultimately a more radical procedure than would have originally been necessary, where this can be prevented by an early referral to a highly specialized sarcoma center.

Introduction. Amputation for subungual malignancy (SUM) was thought to be the gold standard in preventing recurrence and metastasis. The rationale behind this aggressive treatment was never based on scientific evidence. Even though multiple recent studies supported more conservative management by illustrating successful results of the digit salvage technique, especially for “in situ” SUM, this salvage approach is not well supported for the more aggressive type of the “invasive” SUM; herein, we salvaged two cases of “invasive” SUM. Case Presentation. We present two cases of invasive SUM without radiographic evidence of intraosseous involvement, where we avoided digit amputation for both invasive subungual squamous cell carcinoma of the thumb and invasive subungual melanoma of the ring finger. Both were salvaged by using a triple technique under awake local anesthesia which included (I) radical excision of the nail bed unit including both eponychium and periosteum, (II) dorsal cortical bone shaving using a high-speed burr for the distal phalanx, and (III) flap coverage. Brunelli flap was used for the thumb in the first case, and V-Y plasty combined with proximal nail fold advancement flap was used for the ring finger in the second case. There was no evidence of local or distant recurrence, with a good functional outcome after 2.5 years in the first case and 2 years in the second. Conclusion. Ensuring complete resection with negative margins while preserving the functionality of the affected digit is considered to be the optimal challenge in treating “invasive” subungual malignancies. These two case reports contribute by reporting a successful digit salvage. The safety of this procedure could be confirmed by larger series and longer follow-up periods.

The aim of this study is to evaluate tumor volume changes during preoperative radiotherapy and to assess the role of adaptive radiation. Background: Contemporary neoadjuvant radiotherapy utilizes image guidance for precise treatment delivery. Moreover, it may depict changes in tumor size and shape. Materials and methods: Between 2016 and 2018, 23 patients aged ≥18 years with soft tissue sarcoma were treated with neoadjuvant radiation followed by surgical resection. The tumor volumes (cc) were measured using the Pinnacle planning system prior to starting radiotherapy and during treatment, the changes in volume and absolute differences were estimated. Moreover, patient's position on the machine was evaluated to assess setup offsets. The triggers for plan adaptation were >1 cm expansion or unacceptable setup offsets. Results: The mean tumors volume at presentation was 810 cc (range, 55-4000). At last cone beam CT the tumor volume had changed in 14 patients (61%); it was stable in nine patients (39%). Disease regression was documented in eight patients (35%), with median shrinkage of −20.5% (range, −2 to −29%), while tumor progression was observed in six cases (26%), the median change was 12.5% (range, +10 to +25%). Adaptive radiation was required in four patients (17%). For the remaining 19 cases (83%), the dose distribution was adequate to cover target volumes. Conclusions: Change in soft tissue sarcoma volume during radiation is not uncommon. Image guidance should be used to reduce setup errors and to detect differences in tumor volume. Image guidance and adaptive radiation are paramount to ensure optimal radiation delivery.

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