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.

Background: CIC-rearranged sarcoma is a rare and aggressive type of undifferentiated round cell tumor characterized by CIC gene fusion, most commonly CIC::DUX4. This study presents a series of eleven cases, highlighting their clinicopathological features. Methods: Pathology records (2019 to 2024) were searched using “sarcoma with CIC”, identifying eleven cases, of which seven referred cases were initially misdiagnosed. Pathological and clinical analysis was conducted. Treatment was dictated upon multidisciplinary panel discussion based on tumor stage. Follow-up data (1–25 months) was available for all patients. Results: The cohort included six males and five females, with a median age of 43 years (range;14–53), with nine in soft tissue and two in bone. Tumor size ranged from 3.5 cm to 20.0 cm (mean: 9.8 cm). Most cases showed sheets of undifferentiated round- to oval-shaped cells. Two cases showed an Ewing-like pattern, and one case showed spindle cells in a fibrotic stroma transitioning to epithelioid cells. Necrosis was present in nine cases, and mitotic count ranged from 2 to 38/ 10HPFs (mean = 14.2). CD99 was positive in (10/11) cases and WT-1 in (6/9). NKX2.2, S100, and MDM2 were positive in rare cases. CIC::DUX4 fusion was detected in four cases. FISH for CIC gene rearrangement was positive in seven cases, two of them confirmed by methylation analysis. Metastasis at diagnosis was common (n = 8), primarily in the lungs, with later metastasis to the brain and bone. At time of final analysis, eight patients died within a median of 10 months (range: 1–19 months), while three were alive, two with stable disease (for a period of 6 and 25 months) and one with progression after 10 months. Significant correlation was seen between overall survival and the presence of metastasis at diagnosis (p value = 0.03). Conclusions: CIC-rearranged sarcomas are rare, high-grade tumors with predilection for soft tissue. Misdiagnosis is frequent, necessitating molecular confirmation. These tumors are treatment-resistant, often present with lung metastasis, and carry a poor prognosis, especially with initial metastasis.

Introduction: Even with the advancement of limb salvage surgery techniques, forequarter amputation (FQA) is still used in orthopedic oncology. Even though it might pose catastrophic sequelae on the patient’s lifestyle, debilitating one’s ability to perform regular tasks, FQA is still considered as a treatment of last resort for huge fungating tumors of the upper extremity. Case presentation: We present a case of an 18-year-old male patient, who was diagnosed in Libya with left proximal humerus fracture after a trivial trauma and underwent open reduction and internal fixation using k-wires as it was thought to be a simple fracture. Soon after, pain and swelling progressed severely and an open biopsy confirmed a diagnosis of osteosarcoma and imaging suggested metastatic disease to the lungs for which he was started on chemoradiotherapy. He was referred to our cancer center to continue his management and due to the aggressive nature of the tumor, the patient underwent palliative forequarter amputation followed by multiple lines of chemotherapy and radiotherapy, all of which failed to halt the progression of the disease. The patient was lost to follow up due to his decision to go back to Libya. Conclusion: “Whoops” surgeries are fixated upon repairing fractures without looking for the alarming signs on radiographs to exclude pathological entity. As in our case, the procedure done escalated the osteosarcoma into such a massive fungating tumor due to the violation of the osteosarcoma pseudo capsule, in which the only available option is to do a palliative forequarter amputation. Highlights:

Background: Central nervous system (CNS) solitary fibrous tumors (SFTs) are rare neoplasms with limited outcome data. We describe our institutional experience with adult CNS SFTs. Methods: We retrospectively reviewed adults (≥18 years) with histopathologically confirmed CNS SFTs treated at a tertiary referral center between 2005 and 2023. Clinical, radiologic, pathologic, treatment, and follow-up data were collected from the electronic medical record, and pathology was centrally reviewed. Results: Twelve patients were included. Median age at diagnosis was 38 years (range, 18–52), and 58.3% were male. The cohort included 11 intracranial tumors and 1 primary spinal tumor. WHO Grade 3 tumors were most common (n = 6, 50%). Initial treatment with subtotal resection was performed in six patients (50%), gross total resection in five (41.7%), and biopsy alone in one (8.3%). Adjuvant radiotherapy was administered in 58.3% (n = 7), and chemotherapy in one of the two patients with extracranial relapse. Six patients (50%) developed local recurrence, and two (16.7%) experienced extracranial relapse. Five- and ten-year overall survival rates were both 91.7%, and five- and ten-year recurrence-free survival rates were 73.3% and 50.3%, respectively. With a median follow-up of 12 years, late events included extracranial metastases at 8.9 and 9.4 years, and local recurrences at 11.1 and 17.2 years. Conclusions: CNS SFTs in adults show frequent local relapse and disease events more than a decade after treatment. Prolonged surveillance is warranted, and larger multicenter studies are needed to define prognostic factors and optimal treatment.

Background/Objectives: Radiation therapy for left-sided breast cancer exposes the heart and lungs to incidental radiation, increasing long-term risk of cardiopulmonary morbidity. This study evaluated the dosimetric impact of DIBH compared with free breathing (FB) and explored patient- and treatment-related predictors of benefit. Methods: We retrospectively analyzed 90 patients with left-sided breast cancer or chest wall irradiation planned under both FB and DIBH. Dosimetric parameters included mean heart dose (MHD), mean lung dose (MLD), heart V5, and lung V20. Univariable analyses assessed associations between dose reductions (Δ%) and clinical factors (age, BMI, fractionation, boost, nodal fields, and smoking). Multivariable regression identified independent predictors. Results: DIBH significantly reduced mean doses to the heart (FB 4.25 ± 1.47 Gy vs. DIBH 2.64 ± 1.28 Gy; Δ −1.60 Gy, p < 0.001, and Cohen’s d = 2.46) and lung (FB 14.00 ± 3.45 Gy vs. DIBH 11.64 ± 3.32 Gy; Δ −2.36 Gy, p < 0.001, and Cohen’s d = 2.73). Similarly, heart V5 (median Δ −10.5%, p < 0.001) and lung V20 (median Δ −5.5%, p < 0.001) were significantly improved. Higher BMI was independently associated with smaller relative reductions in MHD (−1.4% per unit, p < 0.001) and MLD (−0.31% per unit, p = 0.019). Hypofractionation was linked to greater MHD and MLD reductions (+8.9%, p = 0.007), (3.7%, p = 0.035), respectively. Supraclavicular field irradiation increased lung exposure, while internal mammary chain fields elevated both lung and heart doses. Age showed no significant influence. Conclusions: DIBH provides robust and consistent reductions in cardiac and pulmonary radiation doses during left-sided breast irradiation. The degree of sparing is modulated by BMI, fractionation, and nodal field inclusion.

Background: Uveal melanoma (UM) is a rare malignancy. Despite effective local control, 10–50% of patients develop metastatic disease, which is associated with poor prognosis. Real-world data remain lacking. This study evaluated the clinical characteristics, treatment patterns, and survival outcomes of metastatic UM in a comprehensive cancer center in Jordan. Methods: This retrospective study included adult patients with metastatic UM treated and followed at King Hussein Cancer Center in Jordan (2006–2025). Baseline characteristics and treatment patterns were collected. Descriptive and inferential statistical analyses were performed in JASP v0.95.4 and R v4.5.3. A two-sided p < 0.05 was considered statistically significant. Results: Among 125 patients with followed UM, 31 (24.8%) had metastatic disease and were included in the analysis. Median age at diagnosis was 51 years, 61.3% were males, 61.2% had an ECOG PS 0–1, and 51.6% had hepatic-only metastases. Median time to distant metastasis was 25 months. Median OS from initial diagnosis was 35 months, while median post-metastatic OS (PM-OS) was 10 months. First-line immunotherapy was associated with improved PM-OS compared to Dacarbazine (median, 11 vs. 5 months; p = 0.034; 24-month PM-OS, 44.4% vs. 0%). In exploratory analysis, patients who continued immunotherapy beyond radiologic progression, consistent with pseudoprogression, had longer PM-OS than those who discontinued therapy (median, 32 vs. 6 months; Log-rank p = 0.020). Conclusions: In this real-world cohort, metastatic UM was associated with poor outcomes and limited access to novel therapies including Tebentafusp. First-line immunotherapy may be associated with favorable survival outcomes, and hypothesis-generating exploratory analysis suggested potential benefit from immunotherapy continuation beyond radiologic progression.