Since the original description of pathogenic germline DICER1 variation underlying PPB, the spectrum of extrapulmonary neoplasms known to be associated with DICER1 has continued to expand and now includes tumors of the ovary, thyroid, kidney, eye and brain among other sites. This report documents our experience with another manifestation: a primitive sarcoma that resembles PPB and DICER1 -associated sarcoma of the kidney. These tumors are distinguished by their unusual location in the peritoneal cavity, associated with visceral and/or parietal mesothelium. A total of seven cases were identified through pathology review in children presenting at a median age of 13 years (range 3 to 14 years). Primary sites of origin included the Fallopian tube (4 cases), serosal surface of the colon (one case), and pelvic sidewall (2 cases). One case had pathologic features of type I PPB, another type Ir (regressed) PPB and the remaining 5 had features of type II or III PPB with a mixed primitive sarcomatous pattern with or without cystic elements. All had a pathogenic DICER1 variation identified in germline and/or tumor DNA. PPB-like peritoneal tumors represent a newly described manifestation of DICER1 pathogenic variation whose pathologic features are also recapitulated in DICER1 -related renal sarcoma, cervical embryonal rhabdomyosarcoma, and some Sertoli-Leydig cell tumors with heterologous elements. Tumors arising from the Fallopian tube or elsewhere in the abdomen/pelvis, especially those with heterogeneous rhabdomyosarcomatous and/or cartilaginous differentiation, should prompt consideration of germline and tumor DICER1 testing.

This study examined how navigational strategies, map drawing, and map reading skills may be related in spatial perception performance of 124 U.S. undergraduate men and women who completed one of two versions of Collaer and Nelson's Judgment of Line Angle and Position-15 test and Piaget's Water Level Test. Analysis indicated sex differences in performance were eliminated when self-perceptions of map reading, map drawing, and navigational skills were used as covariates. The men used an orientation (cardinal), whereas the women used a landmark way-finding strategy. Introducing a fine motor skill to solve the Judgment of Line Angle and Position-15 eliminated the sex difference. The data suggest spatial perception is in part influenced by map reading and way-finding strategies.

Brain tumors have been the most common pediatric solid tumor and leading cause of morbidity and mortality. Improved survival emphasizes the importance of adverse treatment effects especially related to nutrition and exercise. Although studies have examined nutrition and exercise outcomes, few randomized trials exist. This narrative review included a systematic literature search with analysis of controlled or single group studies examining clinical and quality-of-life impact of nutrition or exercise interventions. Seven articles were included. Three nutrition studies demonstrated improvement with proactive feeding tubes, nutritional supplementation, and nutritional status. Two exercise studies showed improvement in measures of fitness and neuroanatomy with exercise in pediatric brain tumor survivors; two cohort studies demonstrated a link between quality of life and physical activity. Preliminary studies show nutrition and exercise may improve physical well-being and quality of life, suggesting future controlled studies are warranted to inform clinical care of children with brain tumors.