Indian journal of surgical oncology · 2024
Hypercalcemic Ovarian Carcinoma (Small Cell Carcinoma of the Ovary, Hypercalcemic Type (SCCOHT)): A Case Series and Review of Literature of a Rare Malignancy.
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Kilic Gunes Ebru, Yavasoglu Filiz, Kucukyurt Selin, Aykas Fatma, Yigit Kaya Sureyya, Kok Yilmaz Munevver Irem, Yildirim Murat, Andic Neslihan, Gunes Ahmet Kursad, Karakus Volkan, Sevindik Omur Gokmen, Ozkurt Zubeyde Nur, Gunduz Eren, Ayli Meltem
Аннотация
Mantle Cell Lymphoma (MCL) is a rare subtype of non-Hodgkin lymphoma (NHL), accounting for 5-7% of all lymphomas. Currently, there is no standardized treatment option for older patients (> 65 years), and optimizing MCL treatment in this group remains a significant clinical challenge. In this multicenter retrospective study, we aimed to evaluate the outcomes of first-line treatments, overall survival (OS), progression-free survival (PFS), and factors influencing survival in patients aged 65 years and older diagnosed with MCL. Patients aged ≥ 65 years diagnosed with MCL between November 2016 and January 2024 in six centers were included in this study. A total of 77 patients were included in the study, with 60 males (77.9%) and 17 females (22.1%). The median age was 71 years (range: 65-89). At diagnosis, 96.1% of patients had Stage III-IV disease, and 36.4% had an ECOG performance score of 2-3. Based on the MIPI-c score, 48% of patients had high-risk disease. As induction chemotherapy, 44.2% received R-CHOP, 13% received RB, 13% received R-CVP, and 29.8% received alternating R-CHOP/R-DHA-platinum. Additionally, 19.5% of patients underwent ASCT, and 28.6% received Rituximab maintenance after induction therapy or ASCT. The overall response rate (ORR) was 71.4%. After a median follow-up of 32 months, the median PFS was 18 months, and the median OS was 42 months. In multivariate analysis, independent risk factors for inferior PFS were LDH > ULN (HR: 2.09, 95% CI: 1.05-4.16, p = 0.035), Ki67 ≥ 30% (HR: 2.90, 95% CI: 1.38-6.10, p = 0.005), blastoid variant (HR: 1.86, 95% CI: 1.09-5.12, p = 0.040), and maintenance treatment (HR: 0.31, 95% CI: 0.12-0.75, p = 0.010). Independent risk factors for inferior OS were the maintenance treatment (HR: 0.29, 95% CI: 0.09-0.94, p = 0.040) and POD24 (HR: 2.04, 95% CI: 1.06-4.19, p = 0.044). There remains no universally accepted standard treatment for older MCL patients, and the disease remains incurable with current treatment modalities. However, advancements in targeted therapies and the optimization of maintenance strategies hold promise for improving survival in older and unfit patients. Future studies focusing on incorporating targeted therapies into first-line treatment and identifying high-risk factors that influence survival will be crucial in tailoring effective and individualized treatment approaches for this population.