Congenital peripheral primitive neuroectodermal tumor: A case treated successfully with multimodality treatment

Abstract

Neonatal tumors comprise less than two percent of childhood malignancies. Most are solid tumors, most common histologies being teratoma and neuroblastoma. We encountered a child who was detected to have a right arm mass on antenatal sonogram, which was diagnosed to be a primitive neuroectodermal tumor involving the triceps on fine needle aspiration cytology performed in the post-natal period. The child was successfully treated with multimodality treatment consisting of surgery, chemotherapy and radiotherapy. We also discuss briefly the problems associated with therapy in neonatal period. A review of all cases reported to have congenital Ewing's sarcoma family of tumors is presented. Novel therapies are needed to improve efficacy and decrease the devastating side effects of treatment in this age group.

Ewing Sarcoma Presenting as a Congenital Scalp Mass

“…Unfortunately, this patient succumbed to her disease at 3 months of age. The oldest living patient with no remaining evidence of his congenital disease was treated with surgical resection and chemoradiotherapy consisting of 36 weeks of VAC/IE and adjuvant tumor bed irradiation (Goyal et al, 2014). Given the negative margins obtained postresection as well as our patient's negative metastatic workup, we are eager to see how she progresses following completion of chemotherapy.…”

Sarcoma de Ewing congênito: relato de um caso

“…A forma congênita pode se manifestar em qualquer parte do corpo, porém observamos maior incidência no tronco 1 . Esses tumores acometem mais frequentemente pacientes na segunda década de vida, cerca de 80% são encontrados abaixo dos 20 anos, sendo que a forma congênita é extremamente rara, visto que até o momento foram encontrados relatos de 17 casos na literatura [1][2][3] . Eles se originam do neuroectoderma e são compostos por células neuroepiteliais indiferenciadas ou pouco diferenciadas com capacidade de diferenciação em células neuronais, neurogliais ou outras formas mesenquimais 2 .…”

“…Os tumores da família Ewing podem ocorrer em qualquer idade; porém, cerca de 70 a 80% dos casos ocorrem até os 20 anos e 14% deles são diagnosticados em idade inferior a 5 anos; além disso, a forma congênita é extremamente rara [1][2][3] . São histologicamente caracterizados pela presença de pequenas células arredondadas e azuis em tecidos moles e ossos e incluem sarcoma de Ewing, tumor neuroectodérmico primitivo periférico, neuroepitelioma periférico e tumor de Askin 1,2 .…”

Solid Cancers in the Premature and the Newborn: Report of Three National Referral Centers

“…This highlights the importance of selecting the most appropriate surgical approach based on the knowledge of the natural history of each malignancy; a conservative, nonradical approach is appropriate for infantile fibrosarcoma, infantile hemangiopericytoma, and neuroblastoma, 6,7,9,10 whereas a more radical procedure is required for most renal, brain, and liver tumors, as well as other sarcomas. 11,12,21,22 In summary, neonatal solid tumors represent a small group of malignancies that typically present with an aggressive clinical behavior; however, their outcome is generally favorable, owing to their unique biological features. Early diagnosis and management by expert multidisciplinary teams that integrate perinatal medicine experts with pediatric and surgical oncologists with knowledge of the natural history of each disease are critical for the outcome of these patients.…”