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  • Histology  (1)
  • Neuroectodermal bone tumours  (1)
  • 1
    ISSN: 1432-2307
    Keywords: Neuroectodermal bone tumours ; Immunochemistry ; Electron microscopy
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary Peripheral neuroectodermal tumours (PNET) of bone are rare and mimick those seen in soft tissue (peripheral neuroepithelioma of soft tissue). Their differential diagnosis from Ewing's sarcoma (Es) is extremely difficult by optical means. Here we report 14 new cases of PNET of bone (other than Askin's neoplasm) located primarily in the limbs, pelvic girdle and scapula. Clinically and radiologically they displayed Ewing's sarcoma-like features: mean age was 14.4 years, male/ female ratio being 3:11. Metastasis was present in 6 cases at diagnosis (5 with bone metastasis). Prognosis was poor; thirteen patients died; only one with a metatarsal located tumour is alive and free of disease. The mean survival rate was 25 months following diagnosis and treatment with radio- and multimodal chemotherapy. Histologically the 14 cases displayed Homer-Wright rosettes and pseudorosette-like structures, as well as a fibrillary background and lobular pattern. Immunohistochemistry revealed positivity in a number of neural markers when using paraffin-embedded material: NSE, B-2-microglobuline, HNK-1 (leu-7) and E-36 antibodies. At EM level the cell cytoplasms evidenced dense-core granules with neurosecretion, neurotubules and intermediate filaments like those seen in peripheral neuroepithelioma.
    Type of Medium: Electronic Resource
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  • 2
    ISSN: 1432-2307
    Keywords: Ewing's sarcoma ; Bone ; Histology ; Prognosis
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary A retrospective multifactorial analysis on 261 previously untreated patients with Ewing's sarcoma (Es) of bone has been carried out in order to ascertain the prognostic value of several histological variables on survival. Among those cases accepted as Es, 208 (80% of the patients) were considered to be “typical Es”, while 40 (15%) displayed a large cell predominance, being subclassified as “atypical large cell Es”. Furthermore, 13 patients (5%) possessed tumours of endothelial-like appearance. Eleven cases which displayed a mixed histological configuration were finally included within one of the three previous groups according to their predominant histological pattern. After adjustment for therapeutic regimens and initial location of the tumour, only two histological characteristics remain significant; i.e. the presence of necrosis (p=0.002) and, to a lesser degree, the presence of filagree “en damier” pattern (p=0.08), both of which are of poor prognostic value. From this study, it can be assumed that the morphological (and possibly histogenetical) heterogeneity of Es of bone has no prognostic influence on survival.
    Type of Medium: Electronic Resource
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