Uncommon Pediatric Tumors of the Thorax

Messinger, Yoav; Schultz, Kris Ann P.; Dehner, Louis P.

doi:10.1002/9781118464557.ch66

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2014

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“…PPB is believed to progress along a continuum from Type I, a purely cystic lesion diagnosed at a median age of 9 months, to Type II PPB, a mixed cystic and solid lesion diagnosed at a median age of 36 months, to Type III PPB, a solid, aggressive sarcoma diagnosed at a median age of 43 months . Survival rate decreases along the continuum with overall survival (OS) of >90% for Type I PPB , 75% for Type II PPB and 58% for Type III PPB . For Type I PPB, treatment includes complete surgical excision followed by observation alone or complete surgical excision followed by intermediate intensity chemotherapy.…”

Section: Introductionmentioning

confidence: 99%

Judicious DICER1 testing and surveillance imaging facilitates early diagnosis and cure of pleuropulmonary blastoma

Schultz

Harris

Williams

et al. 2014

Pediatr Blood Cancer

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Pleuropulmonary blastoma (PPB) and Sertoli-Leydig cell tumor (SLCT) are both associated with germline mutations in DICER1. In this brief report, a maternal history of SLCT led to identification of a deleterious DICER1 mutation in the patient and her asymptomatic infant. Radiographic screening revealed a large Type I PPB which was completely resected. Identification of DICER1 mutation carriers and imaging of children at risk for PPB may allow detection of PPB in its earliest and most curable form, leading to increased likelihood of surgical cure and decreased risks of treatment-related late effects.

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Section: Introductionmentioning

confidence: 99%