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Peripheral primitive neuroectodermal tumors. CT and MRI evaluation.
Eur J Radiol. 1996 Feb; 21(3):225-32.EJ

Abstract

The clinical, radiographic, and pathologic features of 17 patients with documented peripheral primitive neuroectodermal tumor (PNET) were evaluated in a retrospective study. The age at diagnosis ranged from 9 months to 46 years (median, 15.8 years). Primary sites of involvement were the abdomen (n = 8), extremities (n = 5), chest (n = 1), temporal bone (n = 1), maxilla (n = 1), and diploe (n = 1). At the time of diagnosis, six patients had distant metastases; all of these patients died, with an average survival of 8.8 months. Radiologic workup included standard radiographs, ultrasound, computed tomography (CT), magnetic resonance imaging (MRI), and bone scintigraphy. The radiographic appearance of these tumors was not specific for differentiation of PNETs from other types of bone and soft tissue tumors. The typical appearance resembled large non-calcified, soft tissue masses with cystic or necrotic areas. Heterogeneous enhancement with intravenous contrast agents was evident on CT, as was an intermediate signal intensity on Tl-weighted images and hyperintense signal on T2-weighted and STIR sequences. After gadolinium administration, variable enhancement was seen. MRI and CT were useful in predicting resectability, in detecting distant metastases, and in the evaluation of response to treatment. Surgery was performed in all cases, either for definitive diagnosis or for therapy. All patients received combined chemotherapy and radiotherapy and five patients received autologous bone marrow transplantation. Clinical follow-up was obtained over a mean period of 3 years (range 1 day to 6 years). Prognosis was poor with a median survival of 3.4 years. Our experience in 17 patients with peripheral neuroectodermal tumors indicates that although their radiologic features are non-specific, they should be included in differential diagnosis of soft tissue tumors of aggressive behavior, especially in a young age group. CT and MRI are useful in delineating the extent and resectability of tumor and in monotoring treatment.

Authors+Show Affiliations

Department of Radiology, Mostoles Hospital, Madrid, Spain.No affiliation info availableNo affiliation info available

Pub Type(s)

Journal Article
Research Support, Non-U.S. Gov't

Language

eng

PubMed ID

8777915

Citation

Ibarburen, C, et al. "Peripheral Primitive Neuroectodermal Tumors. CT and MRI Evaluation." European Journal of Radiology, vol. 21, no. 3, 1996, pp. 225-32.
Ibarburen C, Haberman JJ, Zerhouni EA. Peripheral primitive neuroectodermal tumors. CT and MRI evaluation. Eur J Radiol. 1996;21(3):225-32.
Ibarburen, C., Haberman, J. J., & Zerhouni, E. A. (1996). Peripheral primitive neuroectodermal tumors. CT and MRI evaluation. European Journal of Radiology, 21(3), 225-32.
Ibarburen C, Haberman JJ, Zerhouni EA. Peripheral Primitive Neuroectodermal Tumors. CT and MRI Evaluation. Eur J Radiol. 1996;21(3):225-32. PubMed PMID: 8777915.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - Peripheral primitive neuroectodermal tumors. CT and MRI evaluation. AU - Ibarburen,C, AU - Haberman,J J, AU - Zerhouni,E A, PY - 1996/2/1/pubmed PY - 1996/2/1/medline PY - 1996/2/1/entrez SP - 225 EP - 32 JF - European journal of radiology JO - Eur J Radiol VL - 21 IS - 3 N2 - The clinical, radiographic, and pathologic features of 17 patients with documented peripheral primitive neuroectodermal tumor (PNET) were evaluated in a retrospective study. The age at diagnosis ranged from 9 months to 46 years (median, 15.8 years). Primary sites of involvement were the abdomen (n = 8), extremities (n = 5), chest (n = 1), temporal bone (n = 1), maxilla (n = 1), and diploe (n = 1). At the time of diagnosis, six patients had distant metastases; all of these patients died, with an average survival of 8.8 months. Radiologic workup included standard radiographs, ultrasound, computed tomography (CT), magnetic resonance imaging (MRI), and bone scintigraphy. The radiographic appearance of these tumors was not specific for differentiation of PNETs from other types of bone and soft tissue tumors. The typical appearance resembled large non-calcified, soft tissue masses with cystic or necrotic areas. Heterogeneous enhancement with intravenous contrast agents was evident on CT, as was an intermediate signal intensity on Tl-weighted images and hyperintense signal on T2-weighted and STIR sequences. After gadolinium administration, variable enhancement was seen. MRI and CT were useful in predicting resectability, in detecting distant metastases, and in the evaluation of response to treatment. Surgery was performed in all cases, either for definitive diagnosis or for therapy. All patients received combined chemotherapy and radiotherapy and five patients received autologous bone marrow transplantation. Clinical follow-up was obtained over a mean period of 3 years (range 1 day to 6 years). Prognosis was poor with a median survival of 3.4 years. Our experience in 17 patients with peripheral neuroectodermal tumors indicates that although their radiologic features are non-specific, they should be included in differential diagnosis of soft tissue tumors of aggressive behavior, especially in a young age group. CT and MRI are useful in delineating the extent and resectability of tumor and in monotoring treatment. SN - 0720-048X UR - https://www.unboundmedicine.com/medline/citation/8777915/Peripheral_primitive_neuroectodermal_tumors__CT_and_MRI_evaluation_ L2 - https://linkinghub.elsevier.com/retrieve/pii/0720-048X(95)00731-5 DB - PRIME DP - Unbound Medicine ER -