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.
Subject(s)
Bone Neoplasms/diagnosis , Magnetic Resonance Imaging , Neuroectodermal Tumors, Primitive, Peripheral/diagnosis , Neuroectodermal Tumors, Primitive/diagnosis , Soft Tissue Neoplasms/diagnosis , Tomography, X-Ray Computed , Adolescent , Adult , Bone Neoplasms/mortality , Bone Neoplasms/therapy , Child , Child, Preschool , Combined Modality Therapy , Female , Follow-Up Studies , Humans , Infant , Male , Middle Aged , Neuroectodermal Tumors, Primitive/mortality , Neuroectodermal Tumors, Primitive/therapy , Neuroectodermal Tumors, Primitive, Peripheral/mortality , Neuroectodermal Tumors, Primitive, Peripheral/therapy , Soft Tissue Neoplasms/mortality , Soft Tissue Neoplasms/therapy , Survival RateABSTRACT
The evaluation of loose bodies in the elbow is usually done by means of clinical examination, radiography, and postarthrographic computed tomography (CT). The authors review their experience with magnetic resonance (MR) imaging in place of post-arthrographic CT for the evaluation of loose bodies in the elbow. The prospective interpretation of MR studies of the elbow in 20 patients was compared with arthroscopic findings. All elbows were imaged in multiple planes with thin sections, surface coils, and combinations of T1, T2, and proton-density weighting. The sensitivity for showing loose bodies with MR imaging was 100%, and the specificity was 67%. Because this was a nonblinded study, the results are biased and caution must be used when extrapolating these results to the general population. In this limited experience, MR imaging has reliably shown loose bodies in the elbow, and in the authors' institutions has replaced postarthrographic CT for that purpose.
