scholarly journals COMPUTER TOMOGRAPHY AND X-RAY DIAGNOSTICS OF PERIPHERAL PRIMITIVE NEUROEECTODERMAL TUMORS (PNET) IN CHILDREN

2021 ◽  
Vol 06 (01) ◽  
pp. 35-38
Author(s):  
Umid Ibragimov Umid Ibragimov ◽  
Sundetilla Sargelov Sundetilla Sargelov ◽  
Murat Ensepbaev Murat Ensepbaev ◽  
Amankul Taynekova Amankul Taynekova
Keyword(s):  
Soft Tissue ◽  
Primitive Neuroectodermal Tumor ◽  
Ewing’S Sarcoma ◽  
Ewing's Sarcoma ◽  
Soft Tissue Sarcomas ◽  
The United States ◽  
Neuroectodermal Tumor ◽  
Special Role ◽  
Peripheral Primitive Neuroectodermal Tumor ◽  
Medical Network

Relevance: Peripheral primitive neuroectodermal tumor (PNET) belongs to the group of malignant tumors that develop from migrating embryonic neural crest cells. PNET includes a number of nosological forms: Askin's tumor, esthesioneuroblastoma, the very peripheral primitive neuroectodermal tumor, Ewing's sarcoma . PNET accounts for 3–9% of all soft tissue tumors in children and 19% of all soft tissue sarcomas. In European countries and the United States, the frequency of their occurrence is 3.4 cases per year per 1 million children under 15 years of age, in Kazakhstan - 0.6-1.2 cases per 1 million child population. Rapid tumor growth, malignancy and early metastasis to other organs and systems predetermines its special role in oncology. Objective of the study: to improve the quality and availability of early diagnosis of sarcomas in children in medical institutions of the general medical network. Results of the study: 35 cases of peripheral PNET in children were analyzed. The age of the patients is from 1.5 to 17 years. The average age is 9.3 years. There were 1.3 times more boys among patients than girls. Extra-skeletal localization was found in one patient (3.6%). Radiographically, it is often almost impossible to make a differential diagnosis between the manifestations of Ewing's sarcoma and primary chronic or "healed" (antibiotic) forms of acute hematogenous osteomyelitis in the initial phase of the process, before the formation of the extraosseous soft tissue component. The process of bone damage is more often localized in its diaphysis and subsequently spreads to the metaphyses of the bone.

scholarly journals Radiation diagnostics of peripheral primitive neuroectodermal tumors (PNET) in children at the Scientific Center of Pediatrics and Pediatric Surgery (Almaty, Kazakhstan)

2020 ◽  
Vol 58 (4) ◽  
pp. 36-40
Author(s):  
U. IBRAGIMOV ◽  
S. SARGELOV ◽  
M. ENSEPBAEV ◽  
A. TAYNEKOVA
Keyword(s):  
Soft Tissue ◽  
Primitive Neuroectodermal Tumor ◽  
Ewing’S Sarcoma ◽  
Ewing's Sarcoma ◽  
Abdominal Cavity ◽  
Neuroectodermal Tumor ◽  
Peripheral Primitive Neuroectodermal Tumor ◽  
Tissue Component ◽  
Soft Tissue Component ◽  
Medical Network

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

Detection of circulating tumor cells in patients with Ewing's sarcoma and peripheral primitive neuroectodermal tumor.

1997 ◽  
Vol 15 (2) ◽  
pp. 583-588 ◽  
Author(s):  
D C West ◽  
H E Grier ◽  
M M Swallow ◽  
G D Demetri ◽  
L Granowetter ◽  
...  
Keyword(s):  
Bone Marrow ◽  
Tumor Cells ◽  
Primitive Neuroectodermal Tumor ◽  
Ewing’S Sarcoma ◽  
Ewing's Sarcoma ◽  
Residual Disease ◽  
Neuroectodermal Tumor ◽  
Peripheral Primitive Neuroectodermal Tumor ◽  
Rt Pcr ◽  
Relapsed Disease

PURPOSE To determine the feasibility of detecting Ewing's sarcoma (ES) or peripheral primitive neuroectodermal tumor (PNET) through a reverse-transcriptase polymerase chain reaction (RT-PCR) of the t(11;22)(q24;q12) fusion transcript in blood and bone marrow samples from patients with these neoplasms. PATIENTS AND METHODS Peripheral-blood (PB) and/or bone marrow aspirate (BM) samples were obtained from 28 patients with ES or PNET at initial presentation or at relapse. Patients were divided into two groups: newly diagnosed patients with nonmetastatic disease and those with metastatic/relapsed disease. RNA was extracted from fractionated BM and PB samples, and RT-PCR was performed for the EWS/HumFLI1 fusion mRNA was transcribed across the t(11;22) breakpoint. RESULTS Among the 16 patients with nonmetastatic disease, three of 16 were RT-PCR positive for EWS/HumFLI1 RNA in BM and three of 10 were positive in PB. The total number of nonmetastatic patients who were positive in either PB or BM was four of 16 (25%). Among patients with metastatic/relapsed disease, two of six were positive in BM and five of 10 were positive in PB. The total fraction of patients with metastatic/relapsed disease that was positive in either BM or PB was six of 12 (50%). CONCLUSION In this study, we show that it is possible to amplify the EWS/HumFLI1 RNA by RT-PCR from the BM and PB of a subset of patients with both nonmetastatic and metastatic ES or PNET, which implies that occult tumor cells are present at these sites. The true biologic and clinical meaning of this information is unknown. However, it does suggest a possible application of RT-PCR for the monitoring of residual disease in patients who are undergoing therapy for ES or PNET. This approach may permit early identification of patients who may benefit from alternative therapy or who may be spared possible overtreatment.

Peripheral primitive neuroectodermal tumor/Ewing's sarcoma of the craniospinal vault: case reports and review

2006 ◽  
Vol 37 (7) ◽  
pp. 845-853 ◽  
Author(s):  
Bret C. Mobley ◽  
Diane Roulston ◽  
Gaurang V. Shah ◽  
Karen E. Bijwaard ◽  
Paul E. McKeever
Keyword(s):  
Primitive Neuroectodermal Tumor ◽  
Ewing’S Sarcoma ◽  
Ewing's Sarcoma ◽  
Case Reports ◽  
Neuroectodermal Tumor ◽  
Peripheral Primitive Neuroectodermal Tumor
Sign in / Sign up

Export Citation Format

Share Document