Ronald G. Swee

Systemic mast cell disease : Analysis of 58 cases and literature review

Based on study of 58 histologically proved cases of SMCD, we believe that the prognosis of most SMCD patients can be anticipated at the time of initial diagnosis by using 5 independent significant predictors developed in a multivariate model. Our study confirms the significance of several previously reported poor prognostic factors: absence of skin involvement and the presence of hepatosplenomegaly, cytologic atypia, and a hypercellular bone marrow. However, in contrast to previous reports we did not find a uniform correlation between the presence or absence of skin involvement and prognosis. The observation that anemia was strongly related to so many prognostic variables may be due to the number of patients in our study with associated hematologic disorders. Alternatively, this evidence of ineffective erythropoiesis may support the concept that SMCD is a myeloid stem cell disorder and frequently affects other hematopoietic cell lines. The observation that death occurs within the first 3 years in most fatal cases of SMCD suggests that these patients should be followed carefully for this interval after initial diagnosis, especially if poor prognostic features are present. Currently there is no curative therapy for SMCD.

MESENCHYMAL CHONDROSARCOMA OF BONE AND SOFT TISSUE. A REVIEW OF 111 CASES

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%. Cancer 57:2444–2453, 1986.

Significance of systemic mast cell disease with associated hematologic disorders

A clinical and hematopathologic review of 66 patients with systemic mast cell disease (SMCD) was undertaken to investigate the frequency and the clinical significance of associated hematologic disorders. Twenty‐two patients were found to have a second hematologic disorder, 19 of which involved the myeloid cells (ten dysmyelopoietic syndromes, five myeloproliferative disorders, three acute nonlymphocytic leukemias, and one chronic neutropenia), and three of which involved the lymphoid cells (three malignant lymphomas). A chromosome analysis of the bone marrow revealed abnormalities characteristic of neoplastic myeloid disorders in four patients. Five‐year survival for patients with hematologic disorders was 28% compared with 61% for other SMCD patients (P = 0.004). Patients with hematologic disorders differed significantly from other SMCD patients in that they were about 7 years older (P = 0.039), and they presented more commonly with anemia (P < 0.001) and constitutional symptoms (P = 0.007). These patients also had less frequent skin symptoms (P = 0.003) and urticaria pigmentosa (P = 0.018). By definition, patients with hematologic disorders had a greater percent of hematopoiesis (P < 0.001) and decreased fat cells (P = 0.011) on bone marrow biopsies. A multivariate model demonstrated that the following independent variables were associated with the presence of hematologic disorders: low hemoglobin (P = 0.001), the absence of hepatomegaly (P = 0.016), high leukocyte count (P = 0.021), and the presence of pathologic fractures (P = 0.051). The frequent coexistence of SMCD with dysplastic and neoplastic disorders of myeloid cells is consistent with the concept that SMCD itself is a disorder of myeloid cells and that the mast cell may be myeloid in origin.

Osteoid osteoma. Detection, diagnosis, and localization

One hundred surgically proved cases of osteoid osteoma were reviewed, with emphasis on the modalities used in the detection, diagnosis, and localization of this benign tumor. These included plain radiography, tomography, radionuclide imaging, and angiography. Diagnosis may require all of these techniques. Radionuclide imaging is valuable when no abnormalities are visible on the plain radiographs.

Bone sarcomas associated with Ollier's disease.

Of 55 patients with Olliers disease seen at the Mayo Clinic between 1907 and 1985, 16 had malignant bone neoplasms: 12 chondrosarcomas, two dedifferentiated chondrosarcomas, one chordoma, and one osteosarcoma. One patient had a chondrosarcoma in two different bones. These findings suggest that approximately 30% of patients with Olliers disease will develop a malignant bone neoplasm, most probably chondrosarcoma. The prognosis for most patients is good. Five of the 16 patients survived more than 13 years after treatment.

Chondrosarcoma of small bones of the hands and feet

Cartilaginous tumors of the hands and feet are not uncommon. Most are enchondromas, but they tend to show high cellularity, enlargement of nuclei, and many double‐nucleated cells. Hence, differentiation between a benign lesion and chondrosarcoma may be difficult.

Giant cell reaction (giant cell reparative granuloma) of the small bones of the hands and feet

Giant cell reactions (giant cell reparative granulomas) are uncommon lesions that predominantly involve the small bones of the hands and feet. In 1980, only 13 cases of this entity had been described. Three patients with similar giant cell reactions were identified from the Mayo Clinic files, and an additional 27 patients were collected from the consultation files of one of the authors. Clinical information and follow-up data for these 30 patients suggest that the biologic behavior and, therefore, treatment of giant cell reactions differ from those of true giant cell tumors in the small bones of the hands and feet.

Osteochondromyxoma of bone : A congenital tumor associated with lentigines and other unusual disorders

This article describes the clinical and pathologic features of four unusual bone tumors. Three were congenital or most likely so; the fourth, detected at age 1 year, was probably of considerable duration. The patients, three boys and one girl, each presented with a painless mass. Two had the Carney complex, a familial lentiginous and multiorgan tumorous syndrome; another probably had this disorder; the fourth did not show it, but his mother did. The tumors occurred in the nasal region (n = 2) and the diaphysis of the tibia and radius (n = 1 each). Roentgenographically, three had benign characteristics; the fourth, malignant features. Grossly, the tumors were gelatinous, cartilaginous, and bony. Microscopically, they featured benign-appearing polymorphic cells with few division figures arranged in sheets and lobules set in a myxomatous, cartilaginous, osseous, and hyaline fibrous matrix. Cellularity was low to moderate. The tumors eroded bone, one infiltrated between bony trabeculae, and three had soft tissue extension. Complete resection of one tumor was curative; incomplete excision of two tumors resulted in local recurrence (intracranial and fatal) in one and persistence in the other; the fourth tumor remains under observation after biopsy. No tumor metastasized.

High grade surface osteosarcoma

High grade surface osteosarcoma is a rare subtype of osteosarcoma arising on the surface of bone, accounting for only 8.9% of surface osteosarcomas at the study institution.

Sarcomas Arising in Paget Disease of Bone: A Clinicopathologic Analysis of 70 Cases

CONTEXT Sarcomatous transformation is a rare complication of Paget disease of bone. Prognosis in patients with other types of sarcomas arising in bone has improved in the last several decades because of therapeutic advances. However, because of the rarity of Paget sarcoma, outcome studies in these patients are limited. OBJECTIVE To determine whether prognosis for Paget sarcoma has improved. DESIGN Seventy cases of sarcomas arising in the setting of Paget disease were collected, and the histologic and clinical findings were reviewed. Clinical follow-up was obtained in 67 cases. RESULTS Sarcoma arising in Paget disease tended to arise in older men (46 men, 24 women; age range, 31-88 years; mean age, 66 years) and predominated in the axial skeleton (n = 37), especially in the pelvis. Thirty-three patients had a clinical history of Paget disease ranging in duration from 16 months to 30 years (mean, 15 years). No significant difference in incidence between monostotic (n = 33) and polyostotic (n = 36) disease was noted. Most tumors were osteosarcomas (88%). All tumors were high grade. Follow-up information was obtained in 67 of 70 cases (range of follow-up, 1-252 months). Survival ranged from 1 month to 20 years, with a 5-year survival rate of 10%. CONCLUSIONS Prognosis remains poor in patients with Paget sarcoma. There is no significant correlation between the number of bones involved with Paget disease or the duration of disease and development of Paget sarcoma. Poor prognosis in Paget sarcoma is unrelated to site or stage at presentation.