Characteristics, combinations, treatments, and survival of second primary hematological neoplasm: a retrospective single-center cohort of 49 patients (Hemo2study)

2019 ◽  
Vol 98 (10) ◽  
pp. 2367-2377
Author(s):  
Thomas Chalopin ◽  
Nicolas Vallet ◽  
Flavie Arbion ◽  
Carole Barin ◽  
Emmanuelle Rault ◽  
...  
2021 ◽  
Vol 39 (15_suppl) ◽  
pp. 7049-7049
Author(s):  
Gaurav Goyal ◽  
Aldo A. Acosta-Medina ◽  
Marie Hu ◽  
Jithma P. Abeykoon ◽  
Aishwarya Ravindran ◽  
...  

7049 Background: Langerhans cell histiocytosis (LCH) can manifest as single system (SS) disease, multisystem (MS) disease, or pulmonary LCH (smoking-related). There is a paucity of data on prognostic factors including risk organ (RO) involvement (liver, spleen, and bone marrow) in adult LCH, which we sought to address in this study. Methods: Single-center retrospective study of patients ≥18y diagnosed with LCH from 1998 to 2020. Univariate and multivariate analyses for progression free survival (PFS) and overall survival (OS) were conducted using age, sex, organ involvement, LCH subtype, year of diagnosis, BRAF V600E status, and treatments. Results: We included 219 patients with LCH; median age 43y (range 19-88), females 51%, SS unifocal (23%), SS multifocal (6%), pulmonary (31%) and MS (40%). Commonly involved organs included lung (53%), bone (42%), skin (24%), pituitary (16%), and CNS (12%). BRAF V600E was positive in 40/88 (46%). Median follow-up duration was 6.1y (95% CI, 5.1- 7.1). On univariate analysis, factors associated with worse PFS were bone LCH, RO involvement, multifocal/MS LCH, and radiation therapy alone; those with worse OS included RO involvement, MS disease, BRAF V600E+, and age ≥45y at diagnosis. In multivariate analysis, BRAF V600E and age ≥45y at diagnosis were associated with worse mortality (Table). Median PFS was not reached (NR-NR) for SS unifocal LCH, 5mo (0-12.7) for SS multifocal LCH, 110mo (84.7-135.3) for pulmonary LCH, and 27mo (17.2-36.8) for MS LCH. 5-year OS was 97.4% for SS unifocal LCH, 100% for SS multifocal LCH, 96.1% for pulmonary LCH, and 79.9% for MS LCH. 41 (18.7%) developed a second primary malignancy (SPM), of which 11 were hematologic neoplasms. There was a trend towards a higher prevalence of SPMs in patients with BRAF V600E (28% vs. 17%; p = 0.22). Conclusions: In our large single-center study, PFS for multifocal and MS LCH was worse than SS unifocal or pulmonary LCH. RO involvement was not associated with outcomes in multivariate analysis. Overall prognosis was excellent for all subtypes except MS LCH. BRAF V600E and older age were associated with worse OS. The prevalence of SPMs was very high and needs to be explored further.[Table: see text]


2014 ◽  
Vol 111 ◽  
pp. S33
Author(s):  
R. Micera ◽  
N. Simoni ◽  
E. Cagni ◽  
F. Vigo ◽  
A. Podgornii ◽  
...  

2014 ◽  
Vol 34 (2) ◽  
pp. 262-266
Author(s):  
Özge GÜMÜŞSOY ◽  
Ahmet ÖZET ◽  
Meltem BAYKARA ◽  
Bülent ÇETİN ◽  
Uğur COŞKUN ◽  
...  

2018 ◽  
Vol 7 (2) ◽  
pp. 257-267
Author(s):  
Peiyuan Dong ◽  
Linan Deng ◽  
Xing Xin ◽  
Dan Luo ◽  
Zhaojun Liu ◽  
...  

2020 ◽  
Vol 82 (3) ◽  
pp. 736-738
Author(s):  
Amrita Goyal ◽  
Daniel O'Leary ◽  
Kavita Goyal ◽  
Nathan Rubin ◽  
Kimberly Bohjanen ◽  
...  

2017 ◽  
Vol 12 (1) ◽  
pp. S599
Author(s):  
Fatmir Caushi ◽  
Danjela Xhemalaj ◽  
Hasan Hafizi ◽  
Ilir Skenduli ◽  
Jona Shkurti ◽  
...  

Medicina ◽  
2021 ◽  
Vol 57 (5) ◽  
pp. 494
Author(s):  
Murat Koçer ◽  
Sadık Muallaoğlu ◽  
Bülent Çetin ◽  
Hasan Şenol Coşkun ◽  
Nermin Karahan ◽  
...  

Background and Objectives: In this study, we investigated the frequency and type of second primary malignant tumors (SPMTs) accompanying gastrointestinal stromal tumors (GISTs), patient and tumor characteristics, and follow-up and survival data. Materials and Methods: We included 20 patients with SPMTs from a total of 103 patients with GISTs in a single center in Turkey. At the time of GIST diagnosis, patient age, sex, presentation symptoms, localization, pathological features of the tumor, stage, recurrence risk scoring for localized disease, treatments received, time of SPMT association, follow-up times, and survival analysis were recorded for each patient. Localization, histopathology, and stage of SPMT accompanying GISTs were also recorded accordingly. Results: SPMT was detected in 19.4% of patients with GISTs. Of the patients, 50% were men and 50% were women. The mean age at the time of diagnosis of GIST was 63.8 ± 10.81 years (range: 39–77 years). Of the GISTs, 60% were localized in the stomach, 25% in the small intestine, and 70% were at low risk. Of the SPMTs, 60% were in the gastrointestinal system. SPMTs were diagnosed as synchronous with GISTs in 50% of the patients. The mean follow-up period of the patients from the diagnosis of GIST was 45.6 (0.43–129.6) months. When the data were finalized, 5% died due to GIST, 35% died due to SPMT, and 15% died due to non-disease-related causes. Conclusions: SPMT was detected in 19.4% of patients with GISTs. GISTs were frequently located in the stomach, and most of them were at low risk. The most common SPMTs were gastrointestinal system tumors, and their coexistence was found to be synchronous. Most patients died due to SPMT during follow-up.


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