David Present

Malignant fibrous histiocytoma of soft tissue. An analysis of 78 cases located and deeply seated in the extremities

Seventy‐eight cases of malignant fibrous histiocytoma (MFH) of soft tissues are reported. All tumors were deeply located in the extremities. An analysis is presented with regard to sex, age, site, macroand microscopic features. The staging was assessed according to Ennekings classification (IB, one case; IIA, 25 cases; IIB, 42 cases; III, 10 cases). The recurrence rate after surgical treatment was high (37.5%) with significant differences after inadequate surgery (70%), wide excision (38%), or amputation (0%). Adjunctive radiotherapy rarely was effective in preventing local recurrence. The overall 5‐year survival rate was 36%. The prognosis was worse in (1) tumors larger than 5 cm in diameter, (2) tumors that recurred early (less than 1 year) after surgery, and (3) tumors that had inadequate surgical treatment (intralesional or marginal excision).

Giant-cell tumor of bone with pulmonary metastases: Six case reports and a review of the literature

Giant-cell tumor of bone rarely metastasizes to the lung. In three of six cases, lesions in lung tissue were histologically benign. In 39 such cases reported in the literature, the treatments were surgical extirpation, chemotherapy, and radiation therapy. Resection was indicated to definitely diagnose the pulmonary lesions as benign giant-cell tumors. Radiation therapy and/or chemotherapy may be beneficial as adjuvant treatment, especially where the lesions are anatomically inaccessible. Some pulmonary lesions spontaneously regress even in the absence of definitive treatment.

Eosinophilic granuloma: MRI manifestations

The appearance on magnetic resonance imaging (MRI) of 16 cases of pathologically proven eosinophilic granuloma were reviewed retrospectively and correlated with the radiographic appearance of the lesion. The most common MR appearance (ten cases) was a focal lesion, surrounded by an extensive, ill-defined bone marrow and soft tissue reaction with low signal intensity on T1-weighted images and high signal intensity on T2-weighted images, considered to represent bone marrow and soft tissue edema (the flare phenomenon). The MRI manifestations of eosinophilic granuloma, especially during the early stages, are nonspecific, and may simulate an aggressive lesion such as osteomyelitis or Ewings sarcoma, or other benign bone tumors such as osteoid osteoma or chrondroblastoma.

The correlation between the radiologic staging studies and histopathologic findings in aggressive stage 3 giant cell tumor of bone.

The histologic features of aggressive Stage 3 benign giant cell tumor of bone were correlated with their radiologic staging studies. Our series includes 24 patients treated at the University of Florida, Department of Orthopaedic Oncology, from January 1979 to July 1983. Particularly in 13 cases, results of routine specimen histologic as well as of the histologic study of macrosections containing the entire resected specimen (the tumor and surrounding bone and soft tissue) were evaluated. Surgical staging studies including plain radiographs, bone scintigrams, computerized axial tomography scans, tomography, and angiography were used to delineate the anatomic location of the lesion. Within this group of giant cell tumors, the general histologic features resemble those of the classic giant cell tumor. However, certain aggressive features best demonstrated on the macrosections, such as cortical and subchondral invasion, capsular and reactive bony zone iniltration, “digital extension” of the tumor, and neovascularity correlated well with the anatomic localization and aggressiveness found on the staging studies. Those findings emphasize the value of staging studies in the delineation of the histologic potential of these benign aggressive lesions.

Clear cell chondrosarcoma of bone - A report of 8 cases

We report 8 cases of clear cell chondrosarcoma from 350 cases of chondrosarcoma (2.2%) seen at Istituto Rizzoli. The lesion is always low grade in nature and occurs in an epiphyseal location, usually in the proximal femur or humerus. This relatively inaccessible location may hinder the exact diagnosis. Wide resection is the treatment of choice, although 3 of our patients underwent intralesional or marginal procedures. At present all patients are free of disease. However, a longer follow-up is essential because of the slow-growing nature of this sarcoma.

Tumoral calcium pyrophosphate deposition disease

A report of two patients in which a soft tissue mass, initially regarded as a malignant tumor, was shown to be the result of calcium pyrophosphate deposition disease. The first case, a woman aged 71 years, presented with a mass involving the right fifth finger. In the second case, also a women aged 71 years, the lesion involved the tissues adjacent to the right hip. Each lesion consisted of a mass of highly cellular tissue containing deposits of calcium pyrophosphate dihydrate crystals. the clinical, radiological, and pathological features of the two cases are compared with those of seven similar cases reported in the literature.

The istituto Rizzoli experience with small cell osteosarcoma

Osteosarcoma has been divided into numerous varieties based on clinical, radiographic, and histologic characteristics. Small cell osteosarcoma is a distinct subtype with a histologic appearance in which the bulk of the lesion is composed of round malignant cells with minimal areas of spindle cells. Osteoid production is always present. The lesion may mimic Ewings tumor and other lesions in which round cells are seen. We report the clinical, radiologic, and pathologic features of this tumor, as well as the clinical outcome and response to therapy. Cancer 64:2591–2599, 1989.

Expression of metalloproteinases and tissue inhibitors of metalloproteinases in giant cell tumor of bone: An immunohistochemical study with clinical correlation

The clinical behavior of giant cell tumors (GCTs) is unpredictable. To gain insight into this tumors biological behavior, matrix metalloproteinases (MMPs) and tissue inhibitors of metalloproteinases (TIMPs) were studied. These substances play essential roles in wound healing and neoplastic invasion and metastasis. Paraffin-embedded tissue was collected from 18 cases of histologically benign GCT, with 17 treated by curettage and 1 by resection. Eight cases showed no recurrence after a minimum of 2.5 years, and 10 had local recurrence. One showed metastasis. Antibodies to MMP-9, MMP-2, TIMP-1, and TIMP-2 were applied by immunohistochemical methods. In all cases, MMP-9 was strongly expressed in giant cells predominantly in a diffuse pattern and was strong but focal in stromal cells. MMP-2 decorated stromal cells and giant cells heterogeneously. TIMP-1 was variably expressed in giant cells of the nonrecurrent cases and was strongly present in a diffuse or patchy distribution in the stromal cells in 6 of 8 cases. However, in 9 of 10 recurrent cases, TIMP-1 was expressed weakly by both giant and stromal cells. TIMP-2 was variably expressed in the giant cells of the nonrecurrent cases, but 6 of 8 nonrecurrent cases showed strong stromal cell positivity for TIMP-2. Weak staining for TIMP-2 was observed in 7 of 10 recurrent cases in the stromal cells and 9 of 10 recurrent cases in the giant cells. These results indicate that expression of MMPs and TIMPs differs in giant cells and stromal cells in the same tumor. More significantly, in contrast to the nonrecurrent giant cell tumors, there is an imbalance in the MMPs and TIMPs in the recurrent tumors with a net excess of MMPs. This unopposed expression of MMPs in GCTs may play a role in breakdown of extracellular matrix and tissue invasion. Finally, these markers may prove useful in predicting behavior in these tumors.

Intracapsular and paraarticular chondromas.

Intracapsular and paraarticular chondromas are rare benign tumors. The authors report four such lesions, three of which occurred about the knee and one at the hip. Radiographs showed calcified soft-tissue masses in the infrapatellar region of the knee in three patients. In the hip, the lesion was not visible on conventional radiographs. Three lesions originated from the joint capsule and one was extracapsular. These lesions should be differentiated from synovial chondromatosis and other calcified lesions occurring about the joints. After local excision, the tumors usually do not recur.

Dedifferentiated peripheral chondrosarcomas

Peripheral dedifferentiated chondrosarcoma (CS) is an exceedingly rare variant of the highly malignant entity of dedifferentiated chondrosarcoma. Only five such cases have previously been reported. Seven cases are analyzed and evaluated for the presentation and natural history of this highly malignant lesion when it arises in a tumor that was previously an osteochondroma. Both peripheral and central dedifferentiated chondrosarcomas are high‐grade malignant lesions and require wide or radical surgical margins for adequate treatment. Despite adequate resection, survival is poor; five of the seven cases presented herein died of metastatic spread of their disease. These cases of the peripheral variant of dedifferentiated chondrosarcoma occurred in patients who were younger than patients with central dedifferentiated chondrosarcomas and may be present with longer duration of symptoms because they occur in previously long‐standing benign osteochondromas. As such, they may be easily overlooked clinically and radiographically. Therefore, careful histologic analysis of all cartilage lesions arising on the surface of bone is essential to prevent overlooking foci of high‐grade sarcomatous dedifferentiation.