bims-myxlip 2024-09-29 papers

Indian J Surg Oncol. 2024 Sep;15(Suppl 3): 429-433

Pan Afr Med J. 2024 ;48 56

Metastatic myxoid round cell liposarcoma of the buttock: a case report.

Niare Ndour, Mamadou Sarr, Assane Diop, Mamadou Diouldé Kanté, Coumba Ndiaye, Astou Diouf, Fatou Diagne, Moussa Diallo, Fatimata Ly.

Liposarcoma is a rare primitive mesenchymal tumor, developed at the expense of adipose tissue and with a preferential location in the thigh. We report an observation of liposarcoma in the buttock. A 56-year-old man, presented with a tumor of the right buttock for 2 years. Examination revealed an inflammatory, ulcerated tumor in the upper-external quadrant of the right buttock, measuring about 8 cm. Bilateral inguinal adenopathies were associated. The diagnostic hypotheses were: a squamous cell carcinoma, a cutaneous lymphoma, and cutaneous metastases. An anatomical examination confirmed the diagnosis of myxoid round-cell liposarcoma. The extension work-up appeared compatible with secondary pleuropulmonary, hepatic, cutaneous, and lymph node neoplastic localizations. The patient was treated with chemotherapy with the Adriamycin-carboplatin protocol. The evolution was rapidly fatal after a few weeks after the first course of chemotherapy. It should be evoked in front of any ulcerated tumor of the buttock.

Keywords: Myxoid liposarcoma; buttock; case report; metastasis; round cell

DOI: https://doi.org/10.11604/pamj.2024.48.56.40400

Curr Oncol. 2024 Sep 12. 31(9): 5384-5398

Cardiac Metastasis from Myxoid Liposarcoma Managed Successfully with Chemotherapy and Radiotherapy: Case Report and Review of the Literature.

Georgios M Stergiopoulos, Brittany L Siontis, Ivy A Petersen, Matthew T Houdek, Thanh P Ho, Scott H Okuno, Steven I Robinson.

BACKGROUND: Liposarcoma, one of the most prevalent sarcoma histologies, is recognized for its tendency for extra-pulmonary metastases. While oligometastatic cardiac disease is rarely reported, it poses a unique challenge as oligometastatic sarcomas are often managed with surgical resection.
CASE REPORT: We present a case of a 62-year-old man diagnosed with an oligometastatic myxoid liposarcoma (MLPS) to the heart 19 years after the primary tumor resection from the lower limb. The metastatic mass, situated in the pericardium adjacent and infiltrating the left ventricle, was not managed surgically but with a combination of chemotherapy and radiotherapy. The patient's disease remains stable to date, for more than 10 years.
LITERATURE REVIEW: We conducted a review of the literature to determine the preferred management approach for solitary cardiac metastases of sarcomas. We also conducted an in-depth analysis focusing on reported cases of MLPS metastasizing to the heart, aiming to extract pertinent data regarding the patient characteristics and the corresponding management strategies.
CONCLUSIONS: Although clinical diagnoses of solitary or oligometastatic cardiac metastases from sarcomas are infrequent, this case underscores the significance of aggressive management employing chemotherapy and radiotherapy for chemosensitive and radiosensitive sarcomas, especially when surgical removal is high-risk. Furthermore, it challenges the notion that surgery is the exclusive therapeutic option leading to long-term clinical benefit in patients with recurrent sarcomas.

Keywords: cardiac metastasis; chemotherapy; liposarcoma; myxoid liposarcoma; pericardial metastasis; radiotherapy; sarcoma

DOI: https://doi.org/10.3390/curroncol31090398

ESMO Open. 2024 Sep 20. pii: S2059-7029(24)01496-0. [Epub ahead of print]9(10): 103726

Translocation in bone and soft tissue sarcomas: a comprehensive epidemiological investigation.

K Kawaguchi, M Endo, E Shimada, K Kohashi, T Hirose, A Nabeshima, T Fujiwara, A Kawai, Y Oda, Y Nakashima.

BACKGROUND: Limited epidemiological research has focused on translocations in soft tissue sarcomas, with no studies on bone sarcomas. This study aimed to clarify the epidemiology, prognosis, and genetic information of translocation-related sarcoma (TRS) and non-TRS patients.
MATERIALS AND METHODS: This retrospective cohort study used data from the Bone and Soft Tissue Tumor Registry in Japan (BSTTRJ) (2001-2019), the Kyushu University Hospital (KUH) repository (2001-2021), and a publicly available online dataset (MSK). The patients were categorized into TRS and non-TRS groups, and epidemiological, prognostic, and mutational diversity were compared.
RESULTS: This study included 25 383 participants, of whom 4864 (19.2%) were TRS and 20 519 (80.8%) were non-TRS patients. TRS patients had significantly younger onset ages (median: 43 years, interquartile range: 29-59 years) than non-TRS patients (median: 63 years, interquartile range: 46-73 years). In the MSK cohort, microsatellite instability and tumor mutation burden scores in non-TRS were higher than in TRS, although they were rather low compared with the pan-cancer analysis. In the BSTTRJ cohort, survival analyses with the propensity score matching revealed that patients with TRS had better overall [hazard ratio (HR): 0.71, 95% confidence interval (CI) 0.63-0.81], metastasis-free (HR: 0.75, 95% CI 0.67-0.84), and recurrence-free (HR: 0.47, 95% CI 0.39-0.57) survival.
CONCLUSIONS: This study highlights differences in the epidemiology and genetic rearrangements of sarcoma.

Keywords: bone sarcoma; epidemiology; sarcoma; soft tissue sarcoma; translocation-related sarcoma

DOI: https://doi.org/10.1016/j.esmoop.2024.103726

Cell Stem Cell. 2024 Sep 17. pii: S1934-5909(24)00296-0. [Epub ahead of print]

The landscape of drug sensitivity and resistance in sarcoma.

Sarcomas are rare malignancies with over 100 distinct histological subtypes. Their rarity and heterogeneity pose significant challenges to identifying effective therapies, and approved regimens show varied responses. Novel, personalized approaches to therapy are needed to improve patient outcomes. Patient-derived tumor organoids (PDTOs) model tumor behavior across an array of malignancies. We leverage PDTOs to characterize the landscape of drug resistance and sensitivity in sarcoma, collecting 194 specimens from 126 patients spanning 24 distinct sarcoma subtypes. Our high-throughput organoid screening pipeline tested single agents and combinations, with results available within a week from surgery. Drug sensitivity correlated with clinical features such as tumor subtype, treatment history, and disease trajectory. PDTO screening can facilitate optimal drug selection and mirror patient outcomes in sarcoma. We could identify at least one FDA-approved or NCCN-recommended effective regimen for 59% of the specimens, demonstrating the potential of our pipeline to provide actionable treatment information.

Keywords: high-throughput drug screening; organoids; patient-derived models; precision medicine; sarcoma; tumor organoids

DOI: https://doi.org/10.1016/j.stem.2024.08.010