Clinical Impact of Pathological Features Including the Ki-67 Labeling Index on Diagnosis and Prognosis of Adult and Pediatric Adrenocortical Tumors.

Adrenocortical tumors (ACT) in the adult and pediatric population are generally considered distinct entities due to differences in molecular events related to tumorigenesis, clinical presentation, and outcome. Furthermore, pathological criteria used for diagnosis and prognostication of ACT in adults are usually inadequate for predicting the biological behavior of ACT in children. Here, we analyzed 146 adult and 44 pediatric (< 15y/o) ACT with long-term clinical follow-up and furthered current evidence on the clinical and pathological differences between pediatric and adult tumors. Predilection for female over male gender was observed in both cohorts, but more so in adults (84% vs. 61%, p = 0.003). Cushing syndrome was more frequent in adults (p < 0.001), whereas virilization, either isolated (p < 0.001) or combined to Cushing (p = 0.047), was more common in children. The Ki67 labelling index (LI) of pediatric adenomas and carcinomas was much higher than their corresponding tumors in adults (p < 0.001). Despite these differences, pathological analyses including the evaluation of Ki67 greatly improved patient prognostication in both age cohorts. Indeed, increased Weiss scores and Ki67 indexes correlated with poor overall- and disease-free survival in adult patients with carcinoma. Among the proliferative indexes tested, Ki67 LI ≥ 10% showed the highest hazard ratio (HR) for recurrence and the Ki67 LI ≥ 3% showed the highest HR for survival. In pediatric tumors, the Wieneke score (p < 0.001) and the Ki67 LI (p < 0.001) showed high accuracy for predicting biological behavior, and increased scores/indexes correlated with worse overall and disease-free survival. In this age cohort, Ki67 LI < 10% was able to rule out malignant behavior, whereas Ki67 LI ≥ 15% may be used to predict the patients with higher risks of recurrence and/or poor outcome.

Primary bone lymphoma in 24 patients treated between 1955 and 1999.

The authors present their experience in the treatment of 24 patients with primary bone lymphoma. Eighty-one patients treated between 1955 and 1999 were evaluated, and 57 were excluded because of misdiagnosis. The male to female ratio was 7:5 and the median age was 38.5 years (range, 18-69 years). Two patients had human immunodeficiency virus. Seventeen patients had intermediate-grade lymphomas (Working Formulation), nine patients had centroblastic subtype (Kiel), and 22 patients had B immunophenotype. Nine patients had combined treatment with chemotherapy and radiation therapy, and nine patients had chemotherapy alone. The mean followup was 13.2 years (range, 1.5-37.3 years). Three patients had surgery, two because of subtrochanteric fractures (one patient had closed reduction and internal fixation and the other patient had resection and endoprosthesis reconstruction). The third patient presented with myelocompression at the thoracic level, and he had decompression surgery. Only one patient had radiation therapy and two patients had no treatment. There have been no local recurrences in 17 patients (70.8%). Seven patients (29.2%) died with evidence of disease within a followup of 11 months. The results of the current study showed that patients with primary bone lymphoma have a good prognosis when they are treated with chemotherapy, regardless of whether radiation therapy was given. Surgery usually is appropriate for patients with fractures.