A series of 111 mesenchymal chondrosarcomas was reviewed. The ages of the patients ranged from 5 to 74 years, and approximately 60% of them were in the second and third decades of life. There was no significant sex predilection. Seventy-two tumors, including 5 that involved multiple skeletal sites, arose in bone. Thirty-eight tumors were found in extraskeletal sites. At initial diagnosis, multifocal involvement, both in bone and in soft tissue, was observed in one case. Roentgenographically, the lesions in bone frequently resembled ordinary chondrosarcomas, showing osteolytic and destructive appearances with stippled calcification. Tumors in extraskeletal sites were almost always identified as calcified masses. Histologically, a combination of cellular zones composed of undifferentiated small cells and chondroid zones typically presented a bimorphic appearance that was virtually pathognomonic in most cases. Ablative surgical treatment seemed to be the procedure of choice. The value of irradiation or chemotherapy (or both) was difficult to assess in the current study. Prognosis for patients with mesenchymal chondrosarcoma is usually poor, and long-term follow-up is necessary. In a group of 23 patients from the Mayo Clinic, the 5-year survival rate was 54.6% and the 10-year survival rate was 27.3%.
A total of 422 patients with malignant lymphoma of bone who were seen at the Mayo Clinic from 1907 through 1982 were placed into four major groups based on stage of disease. There was one group with primary lymphoma of bone, one group with multifocal osseous lymphoma, and two groups with lymphoma of bone and nodal or soft tissue (or both) disease. The last-mentioned two groups were separated on the basis of time of onset of osseous lymphoma in relation to the nonosseous disease. The stage of disease was the single most important prognostic indicator of overall survival in malignant lymphoma of bone. The 5- and 10-year survival rates were, respectively, 58% and 53% for patients with primary bone lymphoma, 22% and 12.5% for patients with bone and nodal or soft tissue (or both) disease, and 42% and 35% for patients with multifocal osseous disease. Features having no significant prognostic value were sex of the patient, histologic grade of the lymphoma (according to the Working Formulation and the Kiel system), and presence of T-cell features or cleaved cells (or both). This study is not able to adequately address efficacy of treatment. In fact, treatment may be very important in outcome.
Background. Langerhans' cell histiocytosis (LCH) of bone is a disorder of histiocytic proliferation with variable and often unpredictable behavior. Method. The authors evaluated the clinical and pathologic features of 263 patients (172 children, 91 adults) with biopsy‐proven LCH examined during an 80‐year period at the Mayo Clinic. Only patients with bone involvement pathologically and/or radiographically were included in the study. Clinical follow‐up was available for 245 patients and ranged from 3 months to 50 years (mean, 12 years: median, 10 years). Chi‐square tests were used to determine associations between age, gender, extent of osseous involvement, visceral disease, and pathologic features. Survival analyses were performed by univariate and multivariate Cox regression methods. Results. Age at presentation ranged from 2 months to 71 years with a clear predominance in children. The most common presenting complaint was pain, often worse at night. The skull was the most frequent osseous site in children and adults. Diabetes insipidus was documented in 40 patients. Forty‐four children developed skeletal recurrence and/or new bone lesions, 19 of whom had diabetes insipidus. Fourteen children and 3 adults died either directly or indirectly from LCH. One adult patient developed systemic amyloidosis. All but two of these pediatric patients were 3 years of age or younger at presentation. All children with hepatosplenomegaly (7 patients) and/or thrombocytopenia (9 patients) died. Nine of the 14 children who died presented initially with three or more bone lesions. Conclusions. The clinical behavior of LCH of bone is often unpredictable; however, young age at diagnosis, hepatosplenomegaly, thrombocytopenia, and polyostotic (≥3 bones involved) disease are associated with a poor prognosis (P < 0.005). Recrudescence in children, but not in adults, strongly correlates with the presence of diabetes insipidus (P < 0.0005).
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