Prognosis of Malignant Pheochromocytoma and Paraganglioma (MAPP-Prono Study): A European Network for the Study of Adrenal Tumors Retrospective Study.
Обработка статьи · этапы, ошибки и управление
Ошибки и ограничения
История · было → стало
Состояние обновляется каждые 5 секунд, пока блок открыт. Процент показывается только там, где известен объём работы. Пауза останавливает продолжение этапа; текущий запрос к источнику может завершиться.
Hescot Segolene, Curras-Freixes Maria, Deutschbein Timo, van Berkel Anouk, Vezzosi Delphine, Amar Laurence, de la Fouchardière Christelle, Valdes Nuria, Riccardi Fernando, Do Cao Christine, Bertherat Jerome, Goichot Bernard, Beuschlein Felix, Drui Delphine, Canu Letizia, Niccoli Patricia, Laboureau Sandrine, Tabarin Antoine, Leboulleux Sophie, Calsina Bruna, Libé Rossella, Faggiano Antongiulio, Schlumberger Martin, Borson-Chazot Francoise, Mannelli Massimo, Gimenez-Roqueplo Anne-Paule, Caron Philippe, Timmers Henri J L M, Fassnacht Martin, Robledo Mercedes, Borget Isabelle, Baudin Eric, European Network for the Study of Adrenal Tumors (ENS@T)
Аннотация
PATIENTS AND METHODS: Retrospective multicenter study of MPP characterized by a neck-thoraco-abdomino-pelvic CT or MRI at the time of malignancy diagnosis in European centers between 1998 and 2010.
RESULTS: One hundred sixty-nine patients from 18 European centers were included. Main characteristics of patients with MPP were: primary pheochromocytoma in 53% of patients; tumor- or hormone-related symptoms in 57% or 58% of cases; positive plasma or urine hormones in 81% of patients; identification of a mutation in SDHB in 42% of cases. Metastatic sites included bone (64%), lymph node (40%), lung (29%), and liver (26%); mean time between initial and malignancy diagnosis was 43 months (range, 0 to 614). Median follow-up was 68 months and median survival 6.7 years. Using univariate analysis, better survival was associated with head and neck paraganglioma, age <40 years, metanephrines less than fivefold the upper limits of the normal range, and low proliferative index. In multivariate analysis, hypersecretion [hazard ratio 3.02 (1.65 to 5.55); P = 0.0004] was identified as an independent significant prognostic factor of worst OS.
CONCLUSIONS: Our results do not confirm SDHB mutations as a major prognostic parameter in MPP and suggest additional key molecular events involved in MPP tumor progression. Aside from SDHB mutation, the biology of aggressive MPP remains to be understood.