Type I and Ir pleuropulmonary blastoma (PPB): A report from the International PPB/DICER1 Registry

被引:13
|
作者
Nelson, Alexander T. [1 ,2 ,3 ,4 ]
Harris, Anne K. [1 ,2 ,3 ]
Watson, Dave [5 ]
Miniati, Doug [6 ]
Finch, Mike [5 ]
Kamihara, Junne [7 ]
Mitchell, Sarah G. [8 ]
Wilson, David B. [9 ]
Gettinger, Katie [9 ]
Rangaswami, Arun A. [10 ]
Campos, Jose M. [11 ]
Lederman, Sara [1 ,2 ,3 ,4 ]
Feltis, Brad A. [12 ]
Vasta, Lauren M. [13 ]
Harney, Laura A. [14 ]
Stewart, Douglas R. [13 ]
Dehner, Louis P. [15 ]
Messinger, Yoav H. [1 ,2 ,3 ]
Hill, D. Ashley [16 ,17 ]
Schultz, Kris Ann P. [1 ,2 ,3 ]
机构
[1] Childrens Minnesota, Int Pleuropulm Blastoma DICER1 Registry, Minneapolis, MN USA
[2] Childrens Minnesota, Int Ovarian & Testicular Stromal Tumor Registry, Minneapolis, MN USA
[3] Childrens Minnesota, Canc & Blood Disorders, Minneapolis, MN USA
[4] Univ Minnesota, Med Sch, Minneapolis, MN USA
[5] Childrens Minnesota, Res & Sponsored Programs, Minneapolis, MN USA
[6] Kaiser Permanente Northern Calif, Div Pediat Surg, Roseville, CA USA
[7] Boston Childrens Hosp, Dana Farber Canc Inst, Pediat Oncol, Boston, MA USA
[8] Emory Univ, Sch Med, Childrens Healthcare Atlanta, Aflac Canc & Blood Disorders Ctr, Atlanta, GA USA
[9] Washington Univ, Sch Med, Dept Pediat, St Louis Childrens Hosp, St Louis, MO 63110 USA
[10] Univ Calif San Francisco, Div Pediat Hematol & Oncol, San Francisco, CA 94143 USA
[11] Hosp Dr Sotero del Rio, Div Pediat Surg, Santiago, Chile
[12] East Tennessee State Univ, Div Pediat Surg, Johnson City, TN USA
[13] NCI, Clin Genet Branch, Div Canc Epidemiol & Genet, Rockville, MD USA
[14] Westat Corp, Rockville, MD USA
[15] Washington Univ, Med Ctr, Dept Pathol & Immunol, Lauren V Ackerman Lab Surg Pathol, St Louis, MO USA
[16] Childrens Natl Med Ctr, Dept Pathol & Lab Med, Washington, DC 20010 USA
[17] ResourcePath LLC, Sterling, VA USA
基金
美国国家卫生研究院;
关键词
DICER1; DICER1 tumor predisposition; lung cyst; pleuropulmonary blastoma; type I PPB; type Ir PPB; SOMATIC DICER1 MUTATIONS; GERM-LINE; OVARIAN; PATHOLOGY; TUMOR;
D O I
10.1002/cncr.34593
中图分类号
R73 [肿瘤学];
学科分类号
100214 ;
摘要
BackgroundPleuropulmonary blastoma (PPB) is the most common lung cancer of infancy and early childhood. Type I PPB is a purely cystic lesion that has a microscopic population of primitive small cells with or without rhabdomyoblastic features and may progress to type II or III PPB, whereas type Ir lacks primitive small cells. MethodsChildren with suspected PPB were enrolled in the International PPB/DICER1 Registry. Pathology was centrally reviewed, and follow-up was ascertained annually. ResultsBetween 2006 and 2022, 205 children had centrally reviewed type I or Ir PPB; 39% of children with type I and 5% of children with type Ir PPB received chemotherapy. Outcomes were favorable, although 11 children (nine with type I and two with type Ir PPB) experienced progression to type II/III (n = 8) or regrowth of type I PPB at the surgical site (n = 3), none of whom received chemotherapy before progression. Age and cyst size in combination were more suitable than either factor alone in predicting whether a particular lesion was type I or Ir PPB. ConclusionsFor young children with type I PPB, outcomes are favorable, but complete resection is indicated because of the risk for progression. Chemotherapy may be useful in a subset of children at increased risk for recurrence/progression. Efforts to risk stratify children with type I PPB to optimize outcomes while reducing treatment-related side effects are underway.
引用
收藏
页码:600 / 613
页数:14
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