Epithelioid myoepithelioma of lacrimal gland.
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Biomedical subjects
Publications and source records attributed to Diego Strianese.
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BACKGROUND/AIMS. Localized orbital non-Hodgkin's lymphoma is a rare form of extranodal lymphoma. The aim of this study was to evaluate the outcome of 48 patients presenting with stage I-E non-Hodgkin's lymphoma (NHL). METHODS. Out of 118 charts of patients with lymphoproliferative lesions, those of 48 patients with stage I-E orbital lymphoma seen over a 22-year period from 1977 through 1999 were reviewed. RESULTS. Twenty-five patients were male and 23 were female. Their mean age was 58.7 (12-85) years. The mass was localized inside the orbit in 37 cases and in the lacrimal gland region in 11 cases; bilateral involvement was observed in two cases. The duration of the symptoms at the time of presentation ranged from 10 days to 10 years with a mean duration of 18 months. The 24 cases with low-grade lymphoma are all alive without disease, whatever therapeutic approach was performed (biopsy, radiotherapy, chemotherapy). In intermediate and high-grade NHL the rate of recurrence after chemotherapy has been 50% and 33%, respectively, compared to 75% and 50% after biopsy alone and 75% after radiotherapy (not administered in high-grade forms). CONCLUSION. The therapeutic approach to localized orbital non-Hodgkin's lymphoma must take the histologic grading into consideration. The 24 cases with low-grade lymphoma had a good prognosis. The 17 cases with the intermediate-grade form and the 7 cases with the high-grade form had a better prognosis when treated with chemotherapy.
BACKGROUND: As for rhabdomyosarcoma (RMS) of other anatomic regions, the evaluation of traditional clinicopathological parameters does not allow the unequivocal outcome prediction of the single cases of orbital RMS. We investigated the role of DNA ploidy and immunohistochemical expression of p53, bcl-2, MDR-1 and Ki67 (MIB1) in the prognostic evaluation of orbital rhabdomyosarcomas. MATERIALS AND METHODS: The study population consisted of 11 selected cases. Serial sections of each tumor, stained with Feulgen's technique, were analyzed for the DNA content, using the QUANTIMET 500c Leica analyzer, QWINVO200A software. The results were compared with the immunohistochemical expression of p53 (wild plus mutated, W&M and mutated), bcl2, MDR-1 and Ki67 (MIB1), and with follow-up data. RESULTS: The statistical analysis of results showed that the cases of tetraploid and/or multiploid RMS, overexpressing p53 (W&M and mutated) and MDR-1, were characterized by an overall worse prognosis. On the contrary, the tumors with a favourable clinical course showed hyperexpression of MIB1 and absence of mutated p53 expression. Significantly higher MIB1 expression was found in the relapse-free group of tumors, with respect to the RMS with relapse (both in primary tumors and relative relapses, p<0.05). This finding could justify the higher sensibility to pharmacological therapy of RMS of the first group. The group of RMS with a worse prognosis (primary tumors and relapses) showed instead p53 overexpression (W&M and mutated), MDR-1 expression and multiploidy, with high 5cEE values and tetraploid peaks. No significant difference was found concerning the expression of bcl-2 among the two groups of RMS (p>0.05). CONCLUSION: The evaluation of DNA ploidy, p53, MIB1 and MDR-1 expression could be used for subtyping of orbital RMS into two prognostically different subcategories, respectively RMS responder to the therapy, with favourable clinical outcome, and RMS with a worse prognosis, requiring more aggressive therapeutic protocols.