Extra copies of chromosomes 7, 8, 12, 19, and 21 are recurrent in adamantinoma.

Adamantinoma of long bones is a rare neoplasm predominantly involving the tibia. Cytogenetic studies of adamantinoma are few. Cytogenetic or molecular cytogenetic analysis of four adamantinomas, and a review of eleven cases in the literature reveals extra copies of chromosomes 7, 8, 12, 19, and 21 as recurrent in this neoplasm. Adamantinoma may be confused with a variety of primary and metastatic epithelial and mesenchymal neoplasms. Observation of these aneuploidies may be useful in establishing the diagnosis of adamantinoma.

Evaluating marrow margins for resection of osteosarcoma. A modern approach.

Intraoperative evaluation of bone marrow margins by frozen section analysis is a common practice in the surgical treatment of osteogenic sarcoma. The purpose of this study was to assess the clinical use of intraoperative marrow margin evaluation to rule out occult intramedullary tumor extension in osteosarcoma surgery. One hundred twenty-eight consecutive patients with high grade osteosarcoma diagnosed between 1988 and 1996 (Group 1) were reviewed retrospectively and compared with 92 consecutive patients treated from 1979 to 1984 (Group 2). Eighty-five patients in Group 1 met the inclusion criteria of having high grade intramedullary lesions of the long bones observed on preoperative magnetic resonance imaging evaluation of the lesion and intraoperative frozen section analysis of the bone marrow margin. Thirty-three patients in Group 2 met the same inclusion criteria with the exception of having preoperative magnetic resonance imaging. Ninety-two marrow margins in Group 1 and 33 marrow margins in Group 2 were evaluated by frozen section. All 92 marrow margins in patients in Group 1 were negative by frozen section analysis and permanent histologic analysis. Of the 33 marrow margins in patients in Group 2, three (9.1%) were reported positive for tumor. Of these, one was found to be a false positive result on permanent pathologic examination. In addition, one false negative frozen section result was found, which was positive for tumor on permanent pathologic examination. The difference in true positive results of marrow margins between Group 1 and Group 2 was statistically significant. Intraoperative marrow margin evaluation by frozen section is not mandatory with modern imaging techniques. Preoperative evaluation of tumor extent using magnetic resonance imaging and intraoperative evaluation of the specimen by the pathologist (done by bivalving the specimen) are reliable methods to ensure adequate surgical margins in most cases of conventional osteosarcoma of the long bones.

Identification of a ring chromosome in a myxoid malignant fibrous histiocytoma with chromosome microdissection and fluorescence in situ hybridization.

We investigated the origin of a ring chromosome in a myxoid malignant fibrous histiocytoma (MFH) by microdissection and fluorescence in situ hybridization (FISH) analyses. Cytogenetically, only two ring chromosomes were observed; the smaller ring was seen more frequently. The latter was microdissected, and the material used for FISH. Hybridization of the microdissected labeled DNA to normal metaphase cells revealed that the signal localized only to 20q. Three signals were seen in the tumor cells using either the microdissected 20q probe or chromosome 20 centromeric probe, indicating the involvement of both the long arm and the centromere in the ring chromosome. The short arm of chromosome 20 did not appear to be involved in the formation of the ring chromosome.

Local host response in osteosarcoma after chemotherapy referred to radiographs, CT, tumour necrosis and patient survival.

PURPOSE: The necrotic effect of chemotherapy on primary osteosarcoma has been shown to be predictive of the final outcome. Little attention has been paid to the local response of the host (LHR), which reflects the tumour-host relationship. DESIGN: A four-step grading system was developed based on distinct histological patterns of the LHR around the lesion. These responses were correlated with the chemotherapy-induced necrosis or chemosensitivity and analysed in an attempt to ascertain their influence on the patient prognosis. The ability of conventional radiographs and computed tomography to measure LHR was studied. METHODS: The grading system was applied to macroslides of specimens obtained from 72 patients with stage II B primary osteosarcoma in various limbs after wide resection and complete courses of pre- and postoperative chemotherapy who were treated between 1985 and 1991 with a median follow-up of 5 years and 9 months. The histological specimens were blindly reviewed by two pathologists and two experienced musculoskeletal oncologists to assign a grade of response. The results were correlated with tumour necrosis, patient survival and response features on conventional radiographs and CT images. RESULTS: Significant correlation was found between LHR and tumour necrosis or chemosensitivity (r=0.55) and between LHR and CT response (r=0.56). There was no correlation between LHR and the findings on conventional radiographs. A grade 4 LHR was predictive of long-term survival. CONCLUSIONS: The LHR to preoperative chemotherapy has a prognostic influence on patient survival and can be predicted by CT.

Deletion 1p in a low-grade chondrosarcoma in a patient with Ollier disease.

Ollier disease is an uncommon, nonhereditary developmental disorder affecting enchondral ossification. Cytogenetic analysis of low-grade chondrosarcoma in a patient with Ollier disease (multiple enchondromatosis) revealed an interstitial deletion, del(1)(p11p31.2), as the only chromosome abnormality. This is the first cytogenetic study of a chondrosarcoma in a patient with Ollier disease. Such patients are at risk of developing chondrosarcoma and, because del(1p) is frequent in chondrosarcoma, it is suggested that this cytogenetic finding is associated with early chondrosarcomatous transformation.

Cytogenetic findings in 19 malignant bone tumors.

BACKGROUND: The majority of karyotypes observed in osteosarcomas (OS) and chondrosarcomas (CS) are complex. Specific chromosomal abnormalities have not yet been characterized in either tumor except for a ring chromosome in parosteal OS. The purpose of this study was to determine recurrent chromosomal abnormalities and establish a possible correlation between the cytogenetic changes and the pathologic findings. METHODS: Ten OS and nine CS were cytogenetically analyzed. Tumor samples were obtained from patients having a resection or incisional biopsy. Cytogenetic study of short term cell cultures included harvesting and G-banding, which were performed by routine methodologies. RESULTS: Clonal abnormalities were observed in six OS and six CS. Modal chromosome numbers ranged from near diploid to near tetraploid in both types of tumors. The structural rearrangements observed in OS involved mostly chromosomes 1, 2, 6, 12, and 17. Nonreciprocal translocations were the most frequent event. Two OS had a single clonal abnormality involving 11p15 and 14q32, respectively. Double minute chromosomes were observed in three cases. In CS, the most frequent structural abnormalities were nonreciprocal translocations and deletions involving numerous chromosomes. Rearrangements of 1p together with other abnormalities were observed in four CS. CONCLUSIONS: The karyotypes were usually complex consisting of numerical and structural changes, particularly in high grade tumors. Rearrangements of 11p15 and 14q32 in OS and possibly 1p in CS were found as primary cytogenetic aberrations. Cytogenetic analysis in more cases of OS and CS together with molecular studies are necessary to characterize further the consistent genetic changes in these tumors.

The synovial structure of breast-implant-associated bursae.

Over 2 million silicone breast prostheses have been implanted since they were introduced in the 1960s. After implantation, a fibrovascular tissue reaction referred to as a "capsule" is observed. Many consider this capsule to be a static structure, an effective barrier to the egress of foreign material. However, reports documenting the presence of silicone within lymph nodes of patients with apparently unruptured implants indicate that silicone may be transported away from the breast-implant capsule. To characterize the cells making up the breast-implant capsule, 183 capsules from 103 ruptured or bleeding implants and 80 intact implants were studied. Gross and light microscopic studies were performed on all, and selected capsules were subjected to ultrastructural study and Fourier-transform infrared spectroscopy. Light microscopic examination of the capsule revealed an organized, layered structure with an associated network of endothelia-lined spaces. The capsules varied in cellularity, depending on the type and integrity of the implant. The superficial cell layer of all capsules had cytoplasmic processes directed toward the surface. These long cytoplasmic processes contained vacuoles ultrastructurally, indicating phagocytic and pinocytotic capability. These cells bore immunological markers of bone marrow derived macrophage-type cells. The extracellular matrix of the surface layer consisted of an amorphous fibrillar protein lacking the ultrastructural periodicity of mature collagen. No cell-to-cell junctions were observed. Deeper capsular layers were characterized by fibroblast-type cells in a collagen matrix. No capsules studied contained basement membrane or basal lamina between the stroma of the capsule and the surface cells.(ABSTRACT TRUNCATED AT 250 WORDS)

Involvement of 10q22 and 11q13 in hibernoma.

A hibernoma studied cytogenetically had the karyotype 46,XY,t(9;10;11)(q34;q22;q13),t(17;19) (q21.3;q13). The findings are discussed and compared with those of the previous case described in the literature.

Osteosarcoma of the pelvis. A clinical and histopathological study of twenty-five patients.

Twenty-five patients who had an osteosarcoma of the pelvis were treated at the University of Florida between 1967 and 1990. Two of these patients had underlying Paget disease, and five had received previous radiation therapy to the pelvis. Common problems in this series of patients included delays and errors in establishment of the diagnosis, underestimation of the extent of the tumor on the radiographic staging studies, histopathological findings of local extension next to and into pelvic structures, widespread invasion into major pelvic veins, and microscopic foci of tumor in otherwise normal tissue. These problems led to difficulty in gaining local control of the tumor with any type of operative procedure. Of the eighteen patients who had a resection, only four had a contamination-free wide margin, and a local recurrence developed in thirteen. Only one patient, who had no evidence of disease eleven years after treatment, was alive at the time of writing. Because of the tendency for venous invasion, the radiographic staging studies should include a thorough evaluation of the blood vessels adjacent to the tumor.

Pulmonary function tests after whole-lung irradiation and doxorubicin in patients with osteogenic sarcoma.

PURPOSE: Because of the scarcity of information regarding long-term follow-up of pulmonary function after whole-lung irradiation, a prospective study was started at the University of Florida in 1979 to evaluate pulmonary function after treatment with whole-lung irradiation and doxorubicin in patients with osteogenic sarcoma. PATIENTS AND METHODS: Between 1979 and 1984, 57 osteogenic sarcoma patients with no evidence of metastatic disease at diagnosis received adjuvant therapy consisting of whole-lung irradiation (with the heart shielded) followed by Adriamycin (doxorubicin; Adria Laboratories, Columbus, OH). The whole-lung irradiation schema was 1,600 cGy in 10 fractions with 8-MV x-rays via anterior and posterior fields. This was followed by five cycles of Adriamycin for a total dose of 450 mg/m2. Pulmonary function tests (PFTs) consisting of spirometry, lung volumes, and diffusing capacity were obtained before the whole-lung irradiation, at 6 and 12 months after irradiation, and at yearly intervals thereafter. RESULTS: At the time of analysis, 28 of the 57 patients were available for study, with a mean follow-up of 42 months (range, 6 to 77 months). Follow-up pulmonary function testing revealed decreased forced vital capacity (FVC) and forced expiratory volume at 1 second (FEV1) during the first 6 to 12 months after whole-lung irradiation. These values returned to baseline during the second-year posttherapy and remained at baseline throughout the remainder of the follow-up period. Changes in lung volumes demonstrated a similar early trend, with significant decreases in total lung capacity (TLC) and functional residual capacity (FRC) at 6 to 12 months. These changes, however, did not improve significantly during the remainder of the follow-up period. Diffusing capacity of the lungs for carbon monoxide (DLCO) also reached a nadir at 6 to 12 months after whole-lung irradiation, with resolution by 2 years and maintenance of at least baseline values for the remainder of the follow-up period. CONCLUSIONS: Treatment with whole-lung irradiation and Adriamycin, as given in this study, caused no significant sequelae, as demonstrated by pulmonary function testing during the mean follow-up period of 42 months, although a mild, transient restrictive ventilatory defect occurred at 6 to 12 months after treatment.

Histologic anatomy of the triangular fibrocartilage.

The collagen arrangement of the triangular fibrocartilage complex was studied in 20 fresh cadaver wrists by means of standard and polarized light microscopy and scanning electron microscopy. The collagen fibres in the articular disk are arranged in undulating sheets oriented at oblique angles to each other. The fibers of the radioulnar ligaments are oriented longitudinally from the radial origin to the ulnar insertion. The origin of the articular disk from the radius is characterized by thick fibers 1 to 2 mm in length radiating from the radius into the articular disk. Five specimens were also injected with india ink. The radioulnar ligaments and the peripheral 15% to 20% of the articular disk are well vascularized, whereas the central 80% of the articular disk is avascular.

Spontaneous necrosis in osteosarcoma.

The percentage of necrosis in a primary osteosarcoma after the patient has received preoperative chemotherapy is prognostic and is usually used to select subsequent chemotherapy. However, the percentage of necrosis that occurs spontaneously, without preoperative chemotherapy, has not been adequately studied. The examination of histologic macrosections of 76 osteosarcomas from patients who had not received preoperative treatment and of 20 patients who had received preoperative chemotherapy revealed a significant difference in the percentage of necrosis. There was minimal spontaneous necrosis, but necrosis after preoperative chemotherapy was usually extensive. The larger the tumor, the greater the percentage of spontaneous necrosis; however, size did not correlate with the percentage of necrosis with preoperative chemotherapy. Survival among those patients who did not receive preoperative treatment correlated with the size and percentage of necrosis. Necrosis in an osteosarcoma in a patient who has received preoperative chemotherapy can be considered the result of chemotherapy and not a spontaneous event.

Preoperative irradiation for soft tissue sarcomas of the trunk and extremities in adults.

Between May 1978 and January 1987, 58 adult patients with previously untreated sarcomas of the trunk and extremities were treated with preoperative irradiation and surgery at the University of Florida. All patients had a minimum of 2 years of follow-up; 24 had a minimum of 5 years of follow-up. The preoperative dose was usually 5040 cGy, with 120-125 cGy per fraction delivered twice daily. Operations were performed 2 to 6 weeks after radiation therapy. Eight patients received adjuvant chemotherapy. The tumors were high grade in 52 (90%), measured greater than 10 cm in 45 (78%), and were extracompartmental in 49 (84%). The surgical margins were wide in 17, marginal in 31, and intralesional in 10 patients. A functional extremity was preserved in 47 of 54 patients who would have required an amputation had they been treated by operation alone. Five of 58 patients (9%) developed local failure; in three, the failure occurred outside of the irradiated volume. Survival rates (product-limit method) at 5 years according to grade and size of lesion were as follows: low grade, 100%; high grade, 10 cm or less in largest diameter, 68%; high grade, 11-20 cm, 39%. Data are insufficient for a 5-year analysis of high-grade lesions greater than 20 cm; to date, there are no 5-year survivors in these patients. Moderate and severe wound complications occurred in 16%. There were four pathological fractures in 52 long bones at risk.

The effect of local extent of the tumor on prognosis in osteosarcoma.

The effect of local extent of the tumor on disease-free survival was studied in fifty-one patients who had an osteosarcoma, all of whom were treated according to the same protocol. Disease-free survival was defined as the interval between entry into the protocol and local recurrence, metastasis, or death (whichever occurred first). All patients had a Stage-IIB intramedullary osteosarcoma--that is, a high-grade malignant tumor (Stage II) with cortical penetration (B). The tumors were subdivided on the basis of the amount of local extension: E1--the tumor touches but does not elevate or penetrate the periosteum; E2--the tumor elevates but does not penetrate the periosteum; E3--the tumor penetrates into, but not through, the periosteum; E4--there is minimum extraperiosteal extension without invasion of another structure, such as a muscle, tendon, or ligament; E5--the tumor invades one additional structure (a muscle, ligament, or tendon); and E6--the tumor invades two or more structures adjacent to the bone. When the tumor had invaded two or more adjacent structures (E6), the instantaneous risk of failure was estimated to be 5.9 times greater than when the tumor had a lesser extent (E1 through E5). The relative prognostic importance of the amount of local extension, the size of the tumor, and the sex and age of the patient was compared with forward step-wise Cox multivariate analysis. Only the amount of local extension had a significant effect on disease-free survival. The estimated cumulative probabilities of disease-free survival at five years, according to the Kaplan-Meier method, were 49.1 +/- 8.5 per cent for patients who had a Stage-IIB tumor, 79.8 +/- 9.3 per cent for those who had a Stage-IIB but not E6 tumor, and 17.6 +/- 11.3 per cent for those who had a Stage-IIB E6 tumor. These data suggest that, with respect to prognosis, there are two distinct subgroups of Stage-IIB osteosarcomas that can be objectively defined by the amount of local extension.

A clinicopathologic comparison of malignant fibrous histiocytoma and liposarcoma.

The diagnosis of MFH depends on the demonstration of histiocytic and fibroblastic functions. MFHs may phagocytose fat; therefore, lipid stains are useless. By electron microscopy, cytoplasmic lipid is membrane-bound. Immunohistochemical staining for vimentin and histiocytic markers may be helpful. Liposarcoma is diagnosed only when there is convincing evidence of synthesis and storage of fat by the tumor cells. By electron microscopy, cytoplasmic lipid is nonmembrane-bound. Both MFH and liposarcoma have subtypes. In MFH, the pleomorphic forms are the most common. Myxoid MFH is less common; all other types are rare. In liposarcoma, the myxoid types are by far the most common. The myxoid types of both MFH and liposarcoma may contain other elements that vary in degree and geographic distribution and that can raise the histologic grade. About 50% of liposarcomas are low-grade tumors; these are almost always purely myxoid. Low-grade myxoid liposarcoma has a much better prognosis than other types. Myxoid liposarcoma has a better prognosis than myxoid MFH. The peak incidence of MFH is in the seventh decade of life whereas that of liposarcoma is in the fifth decade. A substantial number (roughly 25% to 30%) of MFHs occur in the subcutaneous tissue. Clinically they are almost invariably mistaken for ganglion cysts. Liposarcoma, however, is likely to occur in or below the buttocks. Most are in the anterior thigh. Subcutaneous liposarcoma is extremely rare. A tumor in this area is likely to be either a more malignant myxoid MFH or one of the benign atypical lipomatous tumors. In both MFH and liposarcoma, the development of distant lesions is related to the tumor's histologic grade and size and to local recurrence. Favored metastatic sites of MFH are lung and lymph nodes. Favored sites of distant lesions in the myxoid/round cell types of liposarcoma are intra-abdominal, retroperitoneal, other soft-tissue areas (especially in the neck), and bone. Lymph node involvement is very rare. Because myxoid/round cell liposarcomas have a marked propensity to involve intra-abdominal sites, abdominal computed tomography and bone scan are recommended in the initial evaluation and follow-up of high-risk patients (those with high-grade tumors larger than 15 cm and those with local recurrence of intermediate- or high-grade tumors of any size).

Surgical treatment for osteosarcoma.

Fifty-three patients who had a high-grade osteosarcoma had either a limb-salvage resection or an amputation. They all received adjuvant therapy that consisted of administration of Adriamycin (doxorubicin) and whole-lung irradiation. At the time of follow-up, the surgical margin was assessed by examination of the surgical specimen. Each patient was followed for at least three years or until death. The data suggested that a wide surgical margin is adequate to control a primary osteosarcoma. When a wide surgical margin can be used and a functional limb can be salvaged, an amputation probably is not required.

Staging of intraosseous extent of osteosarcoma: correlation of preoperative CT and MR imaging with pathologic macroslides.

The accuracy of preoperative computed tomography (CT) and magnetic resonance (MR) imaging in the measurement of intraosseous tumor extent in 17 cases of osteosarcoma of an extremity was compared with macroslides of surgical specimens. Longitudinal intraosseous extension of tumor from the adjacent articular surface was measured on imaging studies and macroslides to the nearest millimeter. The average difference between macroslide and CT measurements (15 cases) was 16.5 mm +/- 10.7, and the average difference between macroslide and MR imaging measurements (17 cases) was 4.9 mm +/- 4.3. However, much of the latter difference appears to be caused by use of a different plane of section in MR imaging compared with that in macroslides, since in a subgroup of five specimens with an identical plane of section, the average difference was 1.8 mm +/- 1.6. The authors conclude that MR imaging is extremely accurate in the assessment of intraosseous extent of osteosarcoma.

Imaging of atypical lipomas of the extremities: report of three cases.

Atypical lipomas are soft tissue neoplasms which differ from simple lipomas in that they consist of mature fat cells interspersed with occasional areas containing a variable admixture of multinucleated cells, collagen bundles, and adipocytes with large, hyperchromic nuclei. Although these histopathologic features resemble those of liposarcoma, atypical lipomas occurring in the extremities differ from liposarcoma because they have no tendency for distant metastases. Three patients with atypical lipomas involving the posterior compartment of the thigh are presented. The lipomas were imaged with computed tomography (CT) and magnetic resonance imaging (MRI), using a 0.15-T whole body imager. Although all lesions appeared largely lipomatous, the three lesions simulated liposarcoma on both CT and MRI. On CT, each lesion demonstrated small areas of confluent soft tissue density; these areas were hyperintense with adipose tissue on strongly T2-weighted MRI. We conclude that atypical lipoma of the extremities may not be distinguishable from liposarcoma on imaging and that biopsy is necessary for differentiation.

Flow cytometric analysis of DNA in bone and soft-tissue tumors using nuclear suspensions.

Ninety-four bone and soft tissue tumors were analyzed for their DNA content using flow cytometry (FCM). A simple, rapid method for preparing isolated nuclear suspensions was used. Tissues, minced in a hypotonic solution containing detergent and propidium iodide as a fluorescent probe for DNA, provided in most instances high nuclear yields from only 0.02 to 0.03 g of solid tumor. Whereas all nonneoplastic samples had a diploid DNA content, various degrees of abnormal DNA distributions were detected in 90% of the neoplastic samples and were present in benign as well as malignant tumors. Our findings demonstrate that FCM DNA analysis is practical in most musculoskeletal tumors and support the observations of others that abnormal DNA content may serve as a general neoplastic marker in these tumors.