الفهرس 
INTRODUCTIONOnly 14 pages are availabe for public view
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Abstract
Desmoid-type fibromatosis is a locally aggressive growth of fibroblastic and myofibroblastic cells, with a potential to recur and invade local organs.
Recurrence in desmoid tumors is a major problem and causing patient morbidity. Few parameters are available to predict tumor recurrence and still, none of which is conclusive.
On the molecular level, most sporadic desmoid tumors are associated with mutations of the beta-catenin gene (CTNNB1). Moreover, the high incidence of desmoid tumors in women and the high rate of reported cases during or following pregnancy and spontaneous regression in postmenopause have raised the possibility of female sex hormones including estrogen as major players in the pathogenesis of desmoid tumors.
The aim of the present work was to assess the expression of beta-catenin and estrogen receptor beta in desmoid type fibromatosis and to correlate it with other clinico-pathological parameters of the tumor.
The present retrospective study was conducted upon the selected thirty cases of desmoid type fibromatosis submitted to the Department of Pathology, Faculty of Medicine, Alexandria University and to D.O.C from January 2013 to December 2018.
Age of the studied cases ranged from 21-51 years with mean value of 35.37 ± 8.20. Four patients were males (13.3%) and 26 (86.7%) were females. Size of the tumor ranged between 2-20 cm. with a mean value of 9.25 ± 4.89. About 63% of the cases presented by abdominal masses and 37% of the cases had extra abdominal masses. Mitotic figures and mild atypia were noted in 53.3% of the cases.
Both ERβ and beta-catenin expression in desmoid tumors was assessed using immunostaining technique. For each antibody staining intensity and density were assessed separately.
For beta-catenin, positive nuclear staining was detected in all studied cases with marked heterogeneity as regard staining intensity and density. There was no significant relation between beta-catenin expression and each of patients’ sex or age or tumor size or site or mitosis and/or atypia.
For ERβ, positive nuclear staining was detected in 83.3% of the studied cases with marked heterogeneity as regard staining intensity and density. Similarly, there was no significant relation between ER expression and each of patients’ sex or age or tumor size or site or mitosis and/or atypia.
Cases were followed up for a period of two years. Ten patients (33.3%) developed recurrence. When recurrent and non-recurrent cases were compared as regard the clinico-pathological parameters of the tumor, it was found that recurrence was encountered more in male sex, extra-abdominal cases and in tumors showed atypia and/or mitosis, but those results were not statistically significant.
In our study the only significant predictor of tumor recurrence was the size of the tumor with a sensitivity of 80%, specificity of 75% and at a cut off value of 8.5cm.
Recurrent cases showed lower ERβ expression, than non-recurrent cases but the difference between the two groups was not statistically significant. On the other hand, no statistically significant correlation could be detected between beta-catenin expression and tumor recurrence in our study.
In conclusion, this study confirmed the immunohistochemical expression of beta-catenin and ERβ in tumor cells of the majority of studied desmoid cases. These results introduce beta-catenin and ERβ as potential therapeutic targets in desmoid tumors. Further studies with a large sample size and follow-up are recommended to validate the current results. Strong intensity of nuclear beta-catenin staining serves as a useful diagnostic tool for desmoid-type fibromatosis. The high expression of ER in desmoid-type fibromatosis provides a biological mechanism for the anti-estrogenic compounds to treat fibromatosis. Beta-catenin and ERcannot predict the prognosis of desmoid-type fibromatosis or recurrence. Also in our study the size of the desmoid tumor was found to be a significant prediction of recurrence with high sensitivity and specificity.