Neoadjuvant concurrent chemoradiotherapy with and without hyperthermia in retroperitoneal sarcomas: feasibility, efficacy, toxicity, and long-term outcome

Purpose Retroperitoneal (RPS) sarcomas are associated with poor local and abdominal tumor control. However, the benefit of preoperative radio- or chemotherapy alone for these entities is currently unclear. Moreover, as intermediate- and high-grade sarcomas have a tendency toward early metastasis, exploration of neoadjuvant strategies is of high importance. This analysis reports the results of our 20-year single-institution experience with preoperative neoadjuvant concurrent chemoradiation. Methods From 2000–2019, 27 patients with intermediate- or high-grade RPS (12 dedifferentiated liposarcoma, 10 leiomyosarcoma, 5 others) were treated with radiotherapy (median dose: 50.4 Gy; range 45–75 Gy) and two cycles of chemotherapy (doxorubicin 50 mg/m2 BSA/d3 q28 and ifosfamide 1.5 g/m2 BSA/d1‑5 q28) in neoadjuvant intent. Chemotherapy consisted of doxorubicin alone in two cases and ifosfamide alone in one case. Fifteen patients (56%) additionally received deep regional hyperthermia. Results The median follow-up time was 53 months (±56.7 months). 92% of patients received two cycles of chemotherapy as planned and 92% underwent surgery. At 5 and 10 years, abdominal-recurrence-free survival was 74.6% (±10.1%) and 66.3% (±11.9%), distant metastasis-free survival was 67.2% (±9.7%) and 59.7% (±11.1%), and overall survival was 60.3% (±10.5%) and 60.3% (±10.5%), respectively. CTC grade III and IV toxicities were leukocytopenia (85%), thrombocytopenia (33%), and anemia (11%). There were no treatment-related deaths. Conclusion Neoadjuvant chemoradiotherapy with and without hyperthermia for retroperitoneal sarcomas is feasible and provided high local control of intermediate- and high-grade sarcoma.

A Transposition Flap Reconstruction after Resection of a Soft-Tissue Sarcoma in the Buttock

Introduction:Large defects following resection in the gluteal region are challenging. Of note, there are a limited number of fairly morbid options for reconstruction. Case Report:A 65-year-old female presented with complaints of an enlarging mass in the left buttock over the past several months. A high-grade sarcoma was diagnosed based on a biopsy. The final diagnosis was an undifferentiated pleomorphic sarcoma based on the resected tumor. An 11-cm tumor with surrounding tissues, including the great gluteal muscle, was resected, which resulted in a 17-cm full thickness defect. The defect was reconstructed with a transposition flap elevated from the lateral thorax. A transposition flap can cover large buttock defects without sacrificing other muscles. Conclusion:Moreover, a transposition flap is esthetically acceptable because most of the operative scar is within the buttock area. A transposition flap reconstruction is one of the several options for large defects after soft-tissue sarcoma resection in the buttock. Keywords:Buttock, resection, reconstruction, musculocutaneous flap, soft-tissue sarcoma.

High-Grade Sarcoma Arising in the Background of Growing Teratoma Syndrome; A Rare Case

Introduction/Objective Growing teratoma syndrome is a rare condition seen in patients with a history of immature teratomas or mixed germ cell tumor status post-treatment. It usually occurs within the first two years of diagnosis but rarely can be seen years later. Methods/Case Report We report a case of a 48-year-old female with a previous history of ovarian malignancy in adolescence status post-TAH/BSO and chemotherapy, now presenting with a 23 cm multi cystic septate mass with some solid components in the subdiaphragmatic area, extending into the liver on imaging. Nodular implants along the surface of the right hepatic lobe and multiple peritoneal implants were seen. Histology of peritoneal implants showed fibro adipose tissue admixed with rare scattered glandular elements, epithelial fragments, and mature neuroglial tissue, consistent with mature teratoma. Liver biopsy revealed short fascicles of spindled, epithelioid and rhabdoid tumor cells admixed with foci of the myxoid stroma. Scattered moderate cytologic atypia, atypical mitosis, and necrosis were appreciated. Tumor cells showed strong and diffuse reactivity to vimentin while being negative for epithelial, neural/mesenchymal, mesothelial, sex cord-stromal, and germ cell differentiation markers. Differential diagnostic considerations included spindle cell transformation of a germ cell tumor or high-grade sarcoma, not otherwise specified arising in the background of growing teratoma syndrome. FISH testing for isochromosome 12p was negative excluding sarcomatoid germ cell tumor. Results (if a Case Study enter NA) NA Conclusion This case study highlights the importance of considering the rare complication of high-grade sarcoma arising in the background of growing teratoma syndrome.

Malignant ossifying fibromyxoid tumor of the calvaria: illustrative case

BACKGROUND Ossifying fibromyxoid tumor (OFMT) is a rare entity of soft tissue tumor that most commonly occurs in the subcutaneous tissues of trunk or extremities with occasional cases involving the head and neck; however, primary involvement of the skull has not been reported. While historically considered slow-growing benign to intermediate malignant, few cases of atypical or malignant features have been described. OBSERVATIONS Herein, the authors present a case of malignant OFMT with primary skull and transcranial extension. The tumor caused lytic calvarial destruction with intra- and extracranial soft tissue components. Gross total resection was performed, and histopathology revealed malignant OFMT with 40 mitoses per 50 high-power fields and moderate nuclear atypia. LESSONS OFMT can rarely occur in the head and neck and, as reported herein, may involve the skull with intracranial extension. While no uniformly recognized histological criteria for malignancy exist, a three-tiered classification has been proposed: typical, atypical, and malignant, based on features such as hypercellularity, mitotic activity, infiltrative growth, and/or nuclear atypia. Malignant variants should be considered along the high-grade sarcoma spectrum with elevated risk for recurrence or metastatic spread. Routine adjuvant radiotherapy is not typically recommended; however, surveillance imaging is advised.

A phase 1b study of avelumab plus DCC-3014, a potent and selective inhibitor of colony stimulating factor 1 receptor (CSF1R), in patients with advanced high-grade sarcoma.

11549 Background: Select sarcomas are infiltrated with immunosuppressive myeloid cells. DCC-3014 is an inhibitor of the CSF1R kinase that decreases tumor infiltrating myeloid cells in preclinical models. We hypothesized that DCC-3014 combined with the anti-PDL1 inhibitor avelumab would be safe and tolerable, decrease immunosuppressive myeloid cells, and increase cytotoxic T cells. Methods: This investigator initiated, open label, single center, phase I study of DCC-3014 plus avelumab in patients (pts) with unresectable or metastatic sarcoma utilized a standard 3+3 dose escalation design. DCC-3014 was administered on days 1-3 (loading dose of 20, 30, or 50 mg) followed by oral daily maintenance (10, 14, or 20 mg) in 28-day cycles; 800 mg of IV avelumab was administered q2weeks. The primary endpoint was to determine the recommended phase 2 dose (RP2D). Secondary endpoints defined the adverse event (AE) profile and assessed clinical efficacy. Peripheral blood CD14+Lin-HLA-DRlo myeloid-derived suppressor cells (MDSCs) were measured by flow cytometry. Results: 13 pts were treated; median age was 61 (range 32 – 71), 8 were female, and median prior lines of therapy was 5 (range 2 – 10). Histologic subtypes included leiomyosarcoma (LMS, n = 7), undifferentiated pleomorphic sarcoma (2), dedifferentiated liposarcoma (LPS, 2), synovial sarcoma (1), and pleomorphic LPS (1). The Table lists treatment-related AEs (TRAEs) of any grade (G) occurring in ≥ 10% of pts and all G ≥ 3 TRAEs, sorted by frequency. All pts had at least 1 TRAE. Seven pts (54%) had a G ≥ 3 TRAE. Most TRAEs were either G ≤ 2 or expected on-target effects of CSF1R inhibition. 1 of 6 pts on the highest dose level had a dose limiting toxicity (G4 elevated AST with abdominal pain) that resolved with treatment cessation. The highest dose level was declared the RP2D. Best objective response by RECIST 1.1 was stable disease in 3 pts; 2 had LMS and were treated at the highest dose level. At baseline, the mean proportion of monocytes in peripheral blood samples with an MDSC phenotype was 12.2% (range 7.1 – 19.9). 5 of 7 pts with serial blood samples had decreased circulating MDSCs (mean decrease of 26.9% from baseline to last time point). Conclusions: DCC-3014 combined with avelumab was safe and tolerable. Study therapy decreased circulating MDSCs in select patients; T cell analyses will be reported. Study expansion at the RP2D is ongoing. Clinical trial information: NCT04242238. [Table: see text]

HIGH GRADE SARCOMA PRESENTING AS SUBDURAL EMPYEMA: CASE REPORT

Sarcoma is very rare, and even more unusual is the association between sarcomas and subdural collection. In this report, we present a case of sarcoma presented as subdural empyema.

Challenging retrovesical mass in men: report of a rare liposarcoma case with concurrent COVID-19 infection

We describe a case of retrovesical liposarcoma in a male patient with concurrent COVID-19. A 50-year-old man had lower urinary tract symptoms and dull pain along his right gluteus. Due to COVID-19 infection, management was delayed. During self-isolation, the patient developed urinary retention and his pain level was an eight on the Visual Analogue Scale. A urinary catheter and an epidural catheter were inserted without any difficulty. Abdominal-pelvic MRI revealed a retrovesical mass suspected of liposarcoma with clear borders from surrounding organs. Following two consecutive negative SARS-CoV-2 PCR tests, we proceeded with surgery. Histopathology was dedifferentiated liposarcoma. Postoperatively, the patient suffered reactivation of COVID-19, and he was eventually discharged after two consecutive negative results on the PCR test on Post Operative Day (POD)-10. Retrovesical dedifferentiated liposarcoma is rare and considered as high-grade liposarcoma. Although surgery may exacerbate COVID-19 infection, surgical resection of symptomatic high-grade sarcoma is prioritised and performed as soon as no infection detected.