XXXX : clinical and laboratory observations.

@entity1 @entity8449 @entity146 @entity5 @entity699 @entity1325 @entity1636 @entity342 @entity204 @entity3800 @entity4304
BACKGROUND: The clinical features and natural history of @entity1636 are highly dependent on the type of center reporting their experience. Observations from oncology services suggest a high incidence of nonfunctioning @entity5 , whereas reports from endocrine clinics emphasize excessive corticoid and @entity342 production in the majority of @entity5 . The incidence rate and natural history of childhood @entity4304 generally has been under emphasized. METHODS: Over the past 17 years, the authors have evaluated and treated 47 @entity1 with @entity1636 referred to the University of @entity1325 Paulo, 22 of whom were @entity1 . RESULTS: There is a bimodal age incidence of @entity4304 , with the disease peaking in the first and fourth decades of life. Childhood @entity4304 is characterized by a high rate of incidence of virilization, marked overproduction of @entity3800 , and a less @entity146 clinical course, and appears to be more amenable to surgical and other therapeutic modalities. By contrast, @entity1636 occurring in adults presents more commonly as a mixed @entity699 , with overproduction of corticoids and @entity3800 and a far more @entity146 clinical course, leading to rapid @entity204 within months or years. Nonfunctioning @entity1636 is less common; it generally occurs in older adults and exhibits a rapid downhill course. Modern day imaging methods have improved the diagnosis and staging of @entity4304 greatly. In the authors' experience, the histologic criteria of Weiss appeared to predict @entity5 prognosis most accurately, whereas immunologic markers, cytoskeletal markers, DNA ploidy, cell phase markers, and oncogenic probes have yielded inconsistent results to date. Surgical removal of a @entity5 remains the best hope for long term survival. Medical therapy with @entity8449 and its successors in @entity1 with Stage III or IV (MacFarlane system as modified by Sullivan et al.) disease appear to have added little to longevity or quality of life. CONCLUSIONS: When diagnosed in @entity1 , @entity4304 is associated with virilism and a less @entity146 natural history; however, when it occurs in adults, the disease presents more commonly as a mixed @entity699 and has a virulent course. The Weiss histologic criteria appear to correlate best with disease prognosis, but other histochemical, cell cycle, and genetic markers have not, to date, aided in disease management.
