Intra-operative radiation therapy for osteosarcoma in the extremities.

The outcome following intra-operative radiation therapy in the treatment of osteosarcoma in the extremity in 33 patients was evaluated for oncological and functional results. Local recurrence occurred in seven cases, six of which were in a non-irradiated region, indicating inappropriate planning of the radiation field. Twenty-one patients underwent either prosthetic replacement (14) or amputation (7). Irradiated tumours were left in situ in the remaining 12 patients. In this latter group no degenerative joint changes were observed radiologically. Twenty-six patients experienced local complications, of which fracture of the irradiated bone was the most significant. Associated intramedullary nailing showed encouraging results in preventing fracture. Although IORT is effective for the local control of osteosarcoma in extremities, critical patient selection and improvements of treatment protocol are required in order to obtain a satisfactory outcome.

Pulmonary cysts as the sole metastatic manifestation of soft tissue sarcoma: case report and consideration of the pathogenesis.

A 29-year-old woman with an unusual form of pulmonary metastasis from epithelioid sarcoma of the right forearm is presented. Since she manifested left pneumothorax due to metastatic pulmonary cyst 7 years ago, the only metastatic manifestation has been the presence of bilateral multiple thin-walled pulmonary cysts; no other types of pulmonary lesions, such as nodules, cavitary lesions with thick or irregular walls, or extrapulmonary metastases, have been found. Pathologic studies revealed metastatic proliferation of sarcoma cells in the wall of the pulmonary cysts and infiltration of malignant cells inside the microscopic cavitary metastases surrounded by normal lung parenchyma.

Transcatheter arterial embolization for malignant osseous and soft tissue sarcomas. II. Clinical results.

PURPOSE: To evaluate the clinical effects of transcatheter arterial embolization (TAE) on malignant bone and soft tissue tumors. METHODS: TAE was performed in 10 patients with primary bone and soft tissue sarcomas and in 31 patients with metastatic bone tumors. The embolized arteries were the internal iliac artery in 30 cases, the intercostal artery in six cases, the lumbar artery in five cases, the suprascapular artery in three cases, and the iliolumbar artery, the internal pudendal artery, and the lateral sacral artery in one case each. The embolized material was gelatin sponge particles. The chemotherapeutic drugs were usually 20-40 mg of doxorubicin for primary and metastatic tumors and 50-100 mg of cisplatin only for primary tumors. In addition, 50-60 Gy of 10-MV radiotherapy with or without radiofrequency (RF)-capacitive hyperthermia in four sessions was administered before TAE for primary tumors only. RESULTS: Even though the pain score increased immediately after TAE, 30 of 38 (79%) patients with pain (8 of 9 with primary tumors, and 22 of 29 with metastases) achieved pain control after TAE. A necrotic low-density area shown by computed tomography (CT) after TAE was found in 31 of 41 (76%) tumors [8 of 10 (80%) with primary tumors, and 23 of 31 (74%) with metastatic tumors]. The tumor size decreased in 14 of 25 (56%) primary and metastatic tumors after 3 months. Osteosclerotic changes appeared in two cases of metastatic tumors after 6 months. In five tumors resected after TAE, large areas of necrosis within the tumor were confirmed histologically. Transient local pain and numbness appeared after TAE, but were relieved by drug treatment within 1 week. No severe complications except a case of gluteal muscle necrosis were encountered after TAE. The 1-year survival rate of the patients with primary tumors was 38.1%, and the median survival was 18 months. The longest survival was 84 months. The 1-year survival rate of the patients with metastatic bone tumors was 38.9%; the median survival was 12 months. The longest survival was 24 months. CONCLUSION: TAE could be an effective treatment for pain control and local control of malignant bone and soft-tissue tumors.

MR appearance of parasymphseal insufficiency fractures of the os pubis.

OBJECTIVE: To clarify the MRI features of parasymphyseal insufficiency fractures of the os pubis. DESIGN AND PATIENTS: MRI was performed in four postmenopausal women with parasymphyseal insufficiency fractures. The diagnosis was confirmed with plain films in every patient. T1-weighted and T2-weighted images were obtained in four patients using a 1.5-T unit. Postcontrast T1-weighted imaging was also done in three patients. RESULTS AND CONCLUSIONS: MRI of pubic parasymphyseal insufficiency fracture characteristically demonstrates a hyperintense mass lesion with a hypointense rim on T2-weighted imaging, showing peripheral and septal enhancement after contrast administration. It is important to have this entity in mind in patients with osteoporosis, especially in patients with a history of pelvic irradiation for malignant disease, so as not to misinterpret it as a chondroid tumor or bone metastasis.

Enchondroma of the distal phalanx of the hand.

We saw five patients who had enchondroma of the distal phalanx, a relatively uncommon site for that lesion. Three patients had pain secondary to a pathological fracture and were managed with curettage and bone-grafting through a palmar longitudinal incision. The other two patients had severe deformities of the fingertip and nail. One was managed with disarticulation of the distal interphalangeal joint and the other, with curettage and grafting through a dorsal approach followed by reconstruction of the nail matrix. We believe that the palmar incision in the pulp of the finger has few, if any, complications.

Septum-like structures in lipoma and liposarcoma: MR imaging and pathologic correlation.

OBJECTIVE: To investigate the septum-like structures in predominantly lipomatous tumors, by correlating fat-suppressed MR images with histopathologic findings. DESIGN AND PATIENTS: The MR findings of three cases of well-differentiated liposarcoma (atypical lipoma), one case of lipoma-like component of dedifferentiated liposarcoma, and nine cases of lipoma were analyzed. T1-, T2-, and fat-suppressed T1-weighted images after Gd-DTPA administration were obtained. Surgical specimens from five patients (four with liposarcoma and one with lipoma) were also scanned with a MR unit, and compared with the pathologic findings. RESULTS AND CONCLUSIONS: Enhancement features of lipoma and liposarcoma were well visualized on fat-suppressed T1-weighted images after Gd-DTPA administration. The septum-like structures of liposarcoma are thick and enhanced considerably, while septa of lipoma are thin and enhanced only slightly. Pathologically, the septum-like structures of liposarcoma contained muscle fibers and the septa of lipoma represented fibrous capsule. Identification of well-enhanced septa in a predominantly lipomatous tumor helps to differentiate malignant tumors from lipomas. As the septum-like structures of liposarcoma contain a skeletal muscle component the tumor might need more extensive surgical procedures including resection of adjacent muscles.

MRI and scintigraphic features of extraabdominal desmoid tumors.

To determine whether extraabdominal desmoid can be correctly diagnosed using both magnetic resonance imaging (MRI) and scintigraphy with pentavalent technetium-99m dimercaptosuccinic acid and gallium-67 citrate, MRI (T1- and T2-weighted images) and scintigraphy were performed in 18 patients with 27 histologically proved extraabdominal desmoid tumors. The extraabdominal desmoid tumors were characterized by positive uptake of pentavalent technetium-99m dimercaptosuccinic acid and lack of uptake of gallium-67 citrate on scintigraphy. These tumors were isointense to skeletal muscle on T1-weighted MRIs and hyperintense on T2-weighted images. They also displayed septum-like internal inhomogeneity and surrounding hypointense capsular band. The combination of scintigraphy and MRI is of value in correctly diagnosing extraabdominal desmoid tumors except in the rare case of fibrotic verylow-grade sarcoma.

Loss of heterozygosity and tumor suppressor gene mutations in chondrosarcomas.

Loss of heterozygosity (LOH) of chromosomes 13q and 17p and mutations of the retinoblastoma (Rb) and p53 gene were studied in 28 tumors from 22 patients with chondrosarcomas. Allele loss at polymorphic loci on 13q was found in 36% of tumors and the frequency was much higher in grade II (56%) or high grade (40%) tumors than in grade I tumors (18%). LOH of 17p, which was detected in 25% of all tumors, was of low frequency in low grade tumors (8% in grade I and 20% in grade II), whereas 80% of tumors with high grade tumors were positive for LOH. These observations may imply that LOH on 13q and 17p contributes to the tumor development at different stages. In low grade cases (grade I and II), tumor recurrence was observed more frequently in primary tumors with LOH on 13q (86%) than those without (8%), suggesting the significance of LOH analysis in the assessment of biological behavior of tumors. Structural alteration of the Rb gene was found in one dedifferentiated tumor, and point mutations of the p53 gene were found in all of five high grade tumors, indicating that high grade chondrosarcomas were genetically equivalent to other high grade sarcomas such as osteosarcomas.

[Prospective randomized trial comparing 1/2 FAM (5-fluorouracil (5-FU) + adriamycin + mitomycin C) versus palliative therapy for the treatment of unresectable pancreatic and biliary tract carcinomas (the 2nd trial in non-resectable patients). Japanese Study Group of Surgical Adjuvant Therapy for Carcinomas of the Pancreas and Biliary Tract].

The efficacy of 1/2 FAM, which consists of 5-fluorouracil (5-FU), adriamycin (ADM) and mitomycin C (MMC), was compared with that of palliative treatment in patients with unresectable pancreatic and biliary tract carcinomas in a multicenter randomized trial. The patients assigned to 1/2 FAM group were treated with 5-FU 200 mg/m2/day IV, ADM 15 mg/m2/day IV and MMC 5 mg/m2/day IV. These 3 drugs were given concurrently as the initial dose within a week after palliative operation, and this regimen was repeated for at least 2 whole courses, at 4-week intervals before the next course of therapy. Those randomized to the control group were subjected to palliative treatment alone. Completely eligible for analysis were 42 cases of the 1/2 FAM group and 41 of the control group. There was no significant difference between the groups with respect to the overall and differentiated survival times according to the tumor sites and the clinical efficacy. As for the duration of 50% inhibition of tumor progression, a significantly better outcome was obtained in 1/2 FAM group. Tumor progression was most significantly inhibited in patients with gallbladder carcinoma. In 1/2 FAM group, tumor reduction was achieved in 1 CR and 2 PR patients. The most frequent adverse reaction was gastrointestinal manifestations, along with diarrhea and alopecia. 1/2 FAM did not contribute to the life prolongation, but inhibited the tumor progression for a significantly longer duration and, to a lesser extent, reduced the tumor size in unresectable pancreatic and biliary tract carcinomas. This regimen is suggested to be useful particularly in the treatment of the latter carcinoma.

Periosteal ganglion of the tibia.

Radiographic, computed tomographic and scintigraphic findings of a patient with separated, multilocular periosteal ganglion are reported. Multiple periosteal cystic masses with calcification in small parts of the cyst walls were demonstrated in the surface of the left tibia by plain radiograms and CT. The accumulations of technetium-99m hydroxymethylene diphosphonate and pentavalent technetium-99m dimercaptosuccinic acid were shown in the calcification in the periosteum and wall of the cyst. Needle puncture revealed that the masses were filled with jelly-like fluid. The masses were diagnosed as multiple ganglionic cysts at the periosteum of the left tibia.

MDM2 gene amplification in bone and soft-tissue tumors: association with tumor progression in differentiated adipose-tissue tumors.

We have studied 107 bone and soft-tissue sarcomas and 8 lipomas for amplification of the MDM2 gene. This gene was amplified in 3 out of 67 osteosarcomas, 3 out of 20 malignant fibrous histiocytomas, 4 out of 20 liposarcomas, and 4 out of 8 lipomas. The amplification was associated with overexpression of mRNA. In osteosarcomas, contrary to previous findings, all amplifications were observed in primary lesions. In liposarcomas, the amplification was seen exclusively in well-differentiated tumors with high frequency (4/5) but not in other subtypes (0/15). In addition, MDM2 amplification was also frequently found in deep-seated intra- or intermuscular lipomas (4/5). Hence, it is suggested that MDM2 amplification plays a significant role in the development of differentiated adipose-tissue tumors. Three well-differentiated liposarcomas with MDM2 amplification coexisted with high-grade dedifferentiated sarcomas, in which MDM2 amplification was also observed. Interestingly, in 2 of these cases, the grades of amplification correlated with the histological grades, indicating an important role of MDM2 overexpression in tumor progression.

3D-spiral CT of multiple exostoses.

We report pre- and post-operative three-dimensional (3D)-spiral CT images in a patient with multiple exostoses. Images of 3D-CT, which were performed using the integrated 3D software of the CT system, showed the exact shapes and locations of the individual tumors around the knee joint in comparison with the surgical findings and resected specimen. 3D-spiral CT images of multiple exostoses would be useful for the planning of surgical procedure.

Uptake of pentavalent technetium-99m dimercaptosuccinic acid in idiopathic synovial chondromatosis.

We reported two Tc-99m(V) DMSA scintigrams in patients with idiopathic synovial chondromatosis which affected the metacarpo-phalangeal joint and shoulder joint. Tc-99m(V) DMSA accumulated markedly and diffusely in the tumor. Tc-99m(V) DMSA scintigraphy would be valuable for deciding the optimal site for biopsy.

Involvement of muscle by malignant lymphoma: MR and CT appearances.

OBJECTIVE: The purpose of this study is to investigate the CT and MR findings of muscular involvement by malignant lymphoma and identify the CT and MR features that may assist in their diagnosis. MATERIALS AND METHODS: Magnetic resonance imaging was performed on four patients (five lesions) with pathologically proven non-Hodgkin lymphoma using a 1.5 T unit (Cases 1, 2, and 4) and a 0.5 T scanner (Case 3). Computed tomography scans were carried out on three patients (Cases 1, 3, and 4). RESULTS: The lesions that extended along muscle fascicles with preserved fat planes looking like swelling of the muscle were of slightly hyper- to isointensity relative to uninvolved muscles on T1-weighted images, of hyperintensity on T2-weighted images, and of low or isodensity on CT. Microscopically, lymphoma cells were seen clustering among normal and atrophic muscle fibers in a biopsy specimen of one patient. The lesions enhanced relatively homogeneously after Gd-DTPA injection. In three cases, vessels were coursing through the lesion on MRI and in two cases on enhanced CT. CONCLUSION: Magnetic resonance imaging proved useful to show the extension of involvement of muscular lymphoma compared with CT. The diagnosis of infiltration of muscle by lymphoma is entertained when a lesion of relatively homogeneous intensity and density extends along the muscle fascisles without obliteration of the fat planes and especially when vessels are identified within the lesion.

Diagnostic value of Tc-99m (V) DMSA for chondrogenic tumors with positive Tc-99m HMDP uptake on bone scintigraphy.

Technetium-99m (V) DMSA scintigraphy was performed in 17 patients with 37 chondrogenic tumors (13 osteochondromas, 14 enchondromas, and 10 chondrosarcomas) that had previously shown uptake of Tc-99m HMDP. Technetium-99m (V) DMSA showed high uptake by all chrondrosarcomas, but low or no uptake always indicated benign chondrogenic tumors. Technetium-99m (V) DMSA scintigraphy may be superior to Tc-99m HMDP scintigraphy for distinguishing benign and malignant chondrogenic tumors, and could also be useful for diagnosing the malignant transformation of chondrogenic tumors.

Autogenous callo-osseous grafts for the repair of osteochondral defects.

We describe a new method of biological repair of osteochondral defects. In rabbit knees an osteochondral defect was reconstructed with a callo-osseous graft made of a superficial sheet of medullary fracture callus attached to a base of cancellous bone. This was taken from the iliac bone of the same animal which had been osteotomised ten days earlier. The reparative tissues were evaluated for 24 weeks by quantitative histology, biochemical analysis of the uronic acid content, and immunohistochemical staining of collagen constituents. The callo-osseous graft provided significantly faster and better repair of the articular surface than an untreated defect or a callo-osseous graft in which the cells had been devitalised by irradiation before transplantation. Our findings indicate that the callo-osseous graft contributes to the repair process by providing both favourable extracellular matrices and pluripotential mesenchymal cells. Our study tested the hypothesis that early medullary callus generates hyaline cartilage instead of bone after transfer to an articular surface.

Solitary muscular involvement by tuberculosis: CT, MRI, and scintigraphic features.

The authors reported computed tomography (CT), magnetic resonance imaging (MRI) and scintigraphic findings of a patient with solitary muscular tuberculosis in the forearm. All these findings resembled those of other granulomatous inflammatory lesions in the soft tissue such as muscular sarcoidosis.

The effects of intraoperative radiotherapy on bone-healing ability in relation to different doses and postradiotherapy intervals.

PURPOSE: Alterations in bone-healing ability after intraoperative radiotherapy were investigated in relation to different doses and postradiotherapy intervals in an experimental model. METHODS AND MATERIALS: Bilateral osteotomy of the fibulae of 56 rabbits was carried out either immediately, 13 weeks or 39 weeks after unilateral intraoperative radiotherapy with 10-MV X ray using one of three doses, 25, 50, or 100 Gy. The healing of the fibulae was evaluated radiographically and histologically 13 weeks after the osteotomy. RESULTS: Bone-healing ability was irreversibly reduced in all irradiated fibulae and these damages occurred in a dose-dependent manner. The severity of the damage also tended to increase with the time interval between irradiation and osteotomy. This interval-related aggravation was characterized by augmented bone loss at the osteotomy ends and atrophy of the neighboring areas. CONCLUSION: These results serve as useful information concerning the surgical intervention of irradiated bone during and after intraoperative radiotherapy, and also after conventional radiotherapy.

Case report: uptake of pentavalent technetium-99m dimercaptosuccinic acid by pigmented villonodular synovitis: comparison with computed tomography, magnetic resonance imaging and gallium-67 scintigraphy.

The scintigraphic findings of a patient with pigmented villonodular synovitis are described and compared with the computed tomography and magnetic resonance imaging data. Intense uptake of pentavalent technetium-99m dimercaptosuccinic acid without gallium-67 citrate uptake indicates that pigmented villonodular synovitis has the features of a hyperplastic or neoplastic lesion rather than an inflammatory lesion from the point of view of nuclear medicine.