Hibernomas are rare benign soft tissue tumors that are included in the broad spectrum of lipomatous neoplasms. The tumors are derived of brown fat, and the clinical and imaging presentation can mimic other neoplastic conditions. We discuss a 20-year experience at a single academic institution to define the clinical presentation, imaging, and management of these rare neoplasms. A retrospective review of all cases of histologically proven hibernoma over a 20-year period was performed. Clinical presentation, demographics, radiologic reports and images, and pathology reports were all reviewed and collected. We identified 19 cases of hibernoma. The clinical presentation and radiographic characteristics are presented. Our findings also demonstrated that local recurrence of these benign soft tissue tumors was rare, and local recurrence was only documented in one of the 19 cases, which was most likely due to inadequate initial resection rather than true recurrence. Hibernomas are composed of brown fat, in which the imaging can be misleading. Once diagnosed, surgical resection is usually curative.

Purpose To assess the healing of horizontal cleavage tears of the meniscus 1 year after surgical repair and to determine what modality is best to determine healing. Methods Patients were prospectively followed for 12 months after surgical meniscus repair using a circumferential compression stitch. Inclusion criteria were preoperative magnetic resonance imaging (MRI) evidence of horizontal cleavage tear, age between 18 and 50 years, and no concomitant anterior cruciate ligament reconstruction. Patients were excluded if they were >50 years old, had a meniscus tear pattern other than horizontal cleavage tear, and underwent concomitant ligament reconstruction. MRIs were performed 1-year postoperatively for evaluation of repair healing. Preoperative and postoperative MRIs of tears were evaluated blindly by a musculoskeletal radiologist. In-office needle arthroscopy was performed at 6 months post-operatively. Results Eight patients were included and had surgery between March 2016 and November 2017. There were 4 medial and 4 lateral meniscus tears. No patients had recurrence of preoperative symptoms or evidence of retear. Six repairs evaluated by in-office needle (at 5.9 months postsurgery) arthroscopy demonstrated complete healing. Seven of the 8 patients had grade III changes on preoperative MRI, and 1 patient had grade IIc changes. On postoperative MRI, 5 of 7 patients had grade III changes, 1 patient had IIc changes, and 1 had IIb changes. There was no significant difference in the proportion of patients with grade III changes preoperatively compared with postoperatively ( P = .57). One of 8 patients with preoperative MRIs demonstrated extrusion where no patients demonstrate postoperative MRI evidence of extrusion ( P = .47). Conclusions Horizontal cleavage meniscal tears repaired with a circumferential compression stitch demonstrate healing on in-office needle arthroscopy 6 months after surgery. No evidence of incomplete or failed healing was found. MRI at 1 year after surgery demonstrated residual tear evidence for all patients. Level of Evidence IV, therapeutic case series.

Sarcomas harboring EWSR1-NFATc2 fusions have historically been categorized and treated as Ewing sarcoma. Emerging evidence suggests unique molecular characteristics and chemotherapy sensitivities in EWSR1-NFATc2 fusion positive sarcomas. Comprehensive genomic profiles of 1024 EWSR1 fusion positive sarcomas, including 14 EWSR1-NFATc2 fusions, were identified in the FoundationCore® database. Additional data from the Gene Expression Omnibus, the Genomics of Drug Sensitivity in Cancer and The Cancer Genome Atlas datasets were included for analysis. EWSR1-NFATc2 fusion positive sarcomas were genomically distinct from traditional Ewing sarcoma and demonstrated upregulation of the mTOR pathway. We also present a case of a 58-year-old male patient with metastatic EWSR1-NFATc2 fusion positive sarcoma who achieved 47 months of disease stabilization when treated with combination mTOR and VEGF inhibition. EWSR1-NFATc2 fusion positive sarcomas are molecularly distinct entities with overactive mTOR signaling; which may be therapeutically targetable. These findings support the use of precision medicine in the Ewing family of tumors.

The molecular findings in Ewing sarcoma have greatly expanded in recent years. Furthermore, this is particularly true for the subset termed “Ewing-like” undifferentiated round cell sarcomas in which new translocations have been reported since the fourth edition of the WHO Classification of Tumours of Soft Tissue and Bone. Amid this expanding genetic landscape, we report a case of extraskeletal undifferentiated round cell “Ewing-like” sarcoma in a 27-year-old female. The patient presented with a large lung mass accompanied on staging imaging by deposits suspicious for metastatic disease in the humerus, calvarium, and lymph nodes of the neck and chest. Biopsy of the lung mass revealed a densely packed monotonous proliferation of round, uniform neoplastic cells with scant cytoplasm. By immunohistochemistry, the tumor cells were diffusely positive for CD99, synaptophysin, TLE1, EMA, and MUC4 and negative for FLI1, PAX7, AE1/3, S100, SOX10, WT1, p63, desmin, and HMB45. Fluorescence in situ hybridization demonstrated rearrangement of the EWSR1 gene. Next-generation sequencing based assay revealed an EWSR1-CREB3L1 fusion. Taken together, the histomorphologic and molecular findings were considered consistent with an undifferentiated round cell sarcoma with an EWSR1-CREB3L1 fusion. Although described in entities such as sclerosing epithelioid fibrosarcoma, low-grade fibromyxoid sarcoma, and small cell osteosarcoma, this has not been previously described in undifferentiated round cell (“Ewing-like”) sarcoma. This finding adds to the growing list of undifferentiated round cell sarcomas with Ewing-like morphologic phenotype–associated fusion genes and may contribute to further defining and characterizing the different subset of tumors in the Ewing family of tumors.

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