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Relevance: Peripheral primitive neuroectodermal tumor (PNET) belongs to the group of malignant tumors that&#x0D;develop from migrating embryonic neural crest cells. PNET&#x0D;includes several nosological forms: Askin’s tumor, esthesioneuroblastoma, the very peripheral primitive neuroectodermal tumor, and Ewing’s sarcoma [1]. PNET accounts&#x0D;for 3–9% of all soft tissue tumors and 19% of all soft tissue&#x0D;sarcomas in children [2, 3]. In Europe and the US, PNETs account for 3.4 cases per year per 1 million children below 15&#x0D;years; in Kazakhstan – 0.6-1.2 cases per 1 million child population. Rapid tumor growth, malignancy, and early metastasis to other organs and systems predetermine the PNET’s&#x0D;specific role in oncology [4].&#x0D;The purpose was to improve the quality and availability of early sarcoma diagnostics in children in medical institutions of the general medical network.&#x0D;Results: 35 cases of peripheral PNET in children were analyzed. The age of the patients was 1.5 to 17 years, the average age – 9.3 years. Boys were 1.3 times more than girls. One&#x0D;patient (3.6%) had extra-skeletal tumor localization. Children with stage IIB prevailed – 46.4% of cases (13 children).&#x0D;Radiographical differentiation between Ewing’s sarcoma and primary chronic or “healed” (antibiotic) acute&#x0D;hematogenous osteomyelitis in the initial phase of the process is almost impossible before the extraosseous soft tissue&#x0D;component is formed. The bone damage process is more&#x0D;often localized in the bone diaphysis and subsequently&#x0D;spreads to its metaphyses.&#x0D;Conclusion: PNET originates most often from the chest&#x0D;wall, so it is advisable to start the X-ray examination from&#x0D;the chest. In terms of radiation semiotics, PNET is similar to&#x0D;Ewing’s sarcoma and Askin’s tumor; therefore, an additional&#x0D;immunohistochemical study of the tumor tissue is required.&#x0D;An important indirect diagnostic criterion in Ewing’s sarcoma&#x0D;is the predominance of the soft tissue component over the&#x0D;bone manifestations. Extended CT and MRI studies with contrast enhancement (chest, abdominal cavity, pelvis, and the&#x0D;primary lesion area) and skeletal scintigraphy are required to&#x0D;clarify the extent of changes, stage the tumor accurately, and&#x0D;assess the tumor dynamics after treatment. The above conclusions generally coincide with the available literature data.

onkologiâ i radiologiâ kazahstana

Radiation diagnosis of peripheral&#x0D; primitive neuroectodermal tumors&#x0D; (PNET) in children under&#x0D; the conditions of the scientific center of&#x0D; pediatrics and pediatric surgery&#x0D; (Almaty, Kazakhstan)

Radiation diagnosis of peripheral primitive neuroectodermal tumors (PNET) in children under the conditions of the scientific center of pediatrics and pediatric surgery (Almaty, Kazakhstan)