YAP1-KMT2A融合肉瘤是一种罕见的软组织肉瘤新亚型，好发于青壮年，形态学与硬化性上皮样纤维肉瘤（SEF）有一定程度重叠，但不表达MUC4。本例患者为34岁女性，左腋下无痛性肿块半年。肿瘤周界相对清楚，由大小形态相对一致的上皮样细胞呈小簇状、短条索状、梁状、巢状或弥漫片状分布于致密胶原化间质中，局部可见血管外皮瘤样结构和地图样坏死。瘤细胞无显著异型性，核分裂象约15个/10 HPF。瘤细胞表达上皮细胞膜抗原、CD99、cyclin D1和BCOR，MUC4阴性。荧光原位杂交显示KMT2A基因易位，二代测序显示YAP1-KMT2A基因融合。常规病理工作中，若遇到形态类似SEF，但不表达MUC4的肿瘤，要考虑到YAP1-KMT2A融合肉瘤的可能性，并行分子检测予以明确。.

OBJECTIVE: Angiosarcomas of the face are rare but present significant treatment challenges due to their origin in the supportive tissues of blood or lymphatic vessels. Achieving optimal balance between oncological efficacy and aesthetic outcomes requires a multidisciplinary approach, particularly in cases where radical R0 resection is necessary. Delays often occur, especially during histopathological examinations, which can complicate primary plastic reconstruction before definitive pathological findings.
METHODS: To address this issue, we present a case with the use of porcine-derived acellular dermal matrix for temporary soft tissue coverage as a viable option in a case of angiosarcoma of the face. This is particularly useful in situations where frozen sections risk the loss of critical anatomical structures and intraoperative diagnosis is not feasible. This approach allowed for satisfactory wound coverage and granulation during diagnostic phases, paving the way for oncologically manageable situations and functional rehabilitation.
CONCLUSIONS: Temporary soft tissue coverage with porcine-derived acellular dermal matrix is a valuable option in tumor surgery of rare and complex situations.

HMGA2::NCOR2 keratin-positive giant cell tumors in children with response to imatinib in an infant.

Liposarcomas are some of the most challenging soft tissue tumors and are subclassified into multiple subtypes with special histologic and molecular features. The peculiarities of each histopathological subtype influence the clinical behavior, management, and treatment of these neoplasms. For instance, well-differentiated liposarcomas are common soft tissue malignancies and usually display a favorable outcome. On the other hand, pleomorphic liposarcoma is the rarest, yet the most aggressive subtype of liposarcoma. This histopathological diagnosis may be challenging due to the scarce available data and because pleomorphic liposarcomas can mimic other pleomorphic sarcomas or other neoplasms of dissimilar differentiation. Nevertheless, the correct diagnosis of pleomorphic liposarcoma is of utmost importance as such patients are prone to develop local recurrences and metastases. Treatment usually consists of surgical excision along with radiotherapy and follow-up of the patients. Therefore, this review aims to assess the complex clinical, histological, and immunohistochemical features of liposarcomas in order to establish how these characteristics influence the management and prognosis of the patients, emphasizing the particularities of pleomorphic liposarcoma.

UNASSIGNED: Myxofibrosarcoma (MFS) is a rare type of soft tissue sarcoma that is locally aggressive and has a high risk of recurrence. The effectiveness of perioperative radiotherapy (RT) in preventing local recurrence (LR) of MFS remains uncertain. This retrospective study aimed to evaluate the impact of perioperative radiotherapy on local recurrence in patients with MFS.
UNASSIGNED: A total of 75 patients diagnosed with MFS and treated at a single institution were included in the study. Patient data, including demographics, tumor characteristics, and treatment variables, were collected from electronic medical records. The primary endpoint was the occurrence of local recurrence.
UNASSIGNED: Among the patients, 25/75 (33.3%) received radiation therapy, while 50/75 (66.7%) did not. Local recurrence in the radiated group was 28% (7/25) compared to 36% (18/50) in the non-irradiated group (p = 0.20). The LR rate trended higher in patients who received RT postoperatively (adjuvant) (6/12, 50%) than preoperatively (neoadjuvant) (1/13, 7.6%) (p = 0.124). Of the 54 patients with negative margins, the local recurrence rate was lower in the radiated group (1/12, 8.33) than the non-irradiated group (9/36, 25%) (p = 0.034). A subgroup analysis based on tumor grade did not reveal any significant differences in recurrence rates between the radiated and non-irradiated groups. Furthermore, there was no significant difference in recurrence rates between the irradiated and non-irradiated groups at the one-year (p = 0.32), two-year (p = 0.24), and five-year (p = 0.32) follow-up marks.
UNASSIGNED: Although radiotherapy demonstrated a trend toward reduction in recurrence rates in patients with MFS in this study, the observed difference did not reach statistical significance. Neoadjuvant radiation appears to be more effective than adjuvant radiation. However, there was a significant reduction in recurrence in patients with negative margins who received radiation demonstrating that effective surgical resection continues to be the most important intervention in patients with myxofibrosarcoma. Level of Evidence: III.

BACKGROUND: Superficial soft tissue metastasis (S-STM) of malignant tumors is uncommon and often brings great pain to patients. However, current treatment options are limited. The purpose of this study was to explore the clinical efficacy and prognostic factors of CT-guided radioactive iodine-125 (125I) seed implantation (RISI) for the treatment of S-STM.
METHODS: We retrospectively evaluated 132 patients with S-STM who received RISI between June 2010 and July 2022. Local tumor progression-free survival (ltPFS), tumor response, pain control and complication were analyzed. The independent factors affecting ltPFS were screened out using a layered Cox proportional hazards model.
RESULTS: The median follow-up time was 8.3 months (interquartile range [IQR], 4.5-15.3 months). The objective response rate (ORR) was 81.8%. The median ltPFS was 9.1 (95% CI: 6.6, 11.6) months. The Cox proportional hazard regression model revealed that the independent factors influencing ltPFS included KPS score, primary tumor, metastases, boundary, density and postoperative D90 (All P < 0.05). After RISI, the rate of pain relief was 92.3%. 66 (84.6%) patients reported pain marked relief, and 6 (7.7%) experienced pain moderate relief. No severe adverse events associated with RISI were observed during follow-up.
CONCLUSIONS: CT-guided RISI was associated with high local control and pain relief without severe adverse events and should be considered as a reliable palliative treatment modality for S-STM.
BACKGROUND: Trial registration Retrospectively registered.

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Soft-tissue sarcomas represent a cohort of rare and heterogeneous malignant tumors that could affect various body parts, with a higher incidence in the lower extremity. When these tumors are surgically removed, both the superficial and deep lymphatic pathways could also be damaged and might require immediate reconstruction to prevent lymphatic complications. In the present report, we describe a case of a patient affected by a high-grade (G3) spindle cell pleomorphic rhabdomyosarcoma of the upper medial thigh. A 22 × 20 cm mass was removed with exposure of the deep femoral vessels and the great saphenous vein. After intraoperative indocyanine green lymphography, it was determined that the superficial lymphatic vessels were intact, but the deep lymphatic system was unavoidably damaged. As a reconstructive procedure, we performed a pedicled SCIP-based vascularized lymphatic vessel transfer and vascularized lymph node transfer to restore the deep lymphatic system and dead space obliteration. The procedure was successful, and no signs of lymphatic impairment were observed during the two-year follow-up period. We believe that this novel approach might be helpful in cases of large and profound defects that involve the deep lymphatic system. The combination of these two techniques could help restore deep lymph drainage, minimizing the risk of superficial system overload and lymphatic dysfunction. No other cases have been described so far employing the same approach. Considering the obtained results, this procedure might be worth further investigation.

The widespread use of advanced molecular techniques has led to the identification of several tumor types with PLAG1 gene fusions some of which also affect the skin and soft tissues. Herein, we present a 38-year-old female with a subcutaneous tumor affecting her forearm, which does not seem to fit into any currently recognized entity. It was a well-circumscribed tumor measuring 6 × 4,5 × 4 cm. It had a thick capsule composed of bland spindle cells forming palisades and Verocay body-like structures within a myxocollagenous background. Scattered calcifications were dispersed throughout the lesion. No cytological atypia, mitotic activity, or necrosis were present. Targeted NGS revealed a SOX10::PLAG1 fusion and fluorescent in situ hybridization confirmed the presence of PLAG1 gene rearrangement. The neoplastic cells showed a diffuse immunohistochemical expression of S100, SOX10, and PLAG1, as well as patchy desmin and CD34 positivity. The methylation profile of this tumor did not match any other entity covered by the DKFZ sarcoma classifier and apart from the gain of chromosome 12, the copy number profile was normal. The tumor was completely excised, and the patient has been free of disease for 4 years since the excision. While more cases are needed to confirm this tumor as a distinct entity, we propose a provisional name \"SOX10::PLAG1-rearranged calcifying spindle cell tumor.\"

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