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Biomedical subjects

J Mirra

Publications and source records attributed to J Mirra.

At least 19 recordsLinked to original sources

Progress in the recognition and treatment of soft tissue sarcomas.

Over the past 10 years there has been significant progress made in the recognition and treatment of soft tissue sarcomas. With the advent of CAT scans and MRI, preoperative delineation of soft tissue tumors has become readily available. The diagnostic use of these modalities in patients presenting with an ill-defined asymptomatic mass has been extremely helpful in terms of screening patients to decide whether or not a biopsy is indicated. These techniques have also provided a much clearer delineation of the anatomic extent of the primary tumor, which has been of great assistance both in radiation therapy treatment calculations as well as in preoperative surgical planning. The recognition that tumor grade is the dominant prognostic variable has resulted in the more common use of a grading system, and a more uniform reporting and stratification of end results. Recent studies with immunohistochemical staining have proven of value in determining the histogenesis of many tumors that in the past were difficult to classify accurately. Most recently the use of flow cytometry suggests that this will also be a valuable adjunct in determining tumor grade and thereby prognosis. The most recent investigations of molecular biologic evaluation of genetic DNA and RNA sequences, as well as of oncogenes are extremely interesting from a diagnostic standpoint and in demonstrating the potential of molecular biologic evaluation for understanding the origin of these tumors. Multimodality therapy with surgery, radiation, chemotherapy, or all three has resulted in a marked improvement in local tumor control for patients with soft tissue sarcomas. The combination of modalities has allowed smaller surgical excisions of the tumor and thereby preservation of the extremity and much of its function. There are currently several different methods of multimodality therapy used including neoadjuvant therapy and postoperative therapy, both of which have been proven efficacious. Chemotherapy is now playing an increased role in clinical investigation and treatment. The availability of Adriamycin, DTIC, cisplatin, and most recently ifosfamide has added several chemotherapeutic agents for use by the clinician. Combination chemotherapy and radiation is of value in the neoadjuvant setting, and several studies are now underway to determine whether postoperative adjuvant chemotherapy is of similar value in reducing systemic spread of disease. Finally, surgical resection of pulmonary metastases has been proven of value in 20% to 25% of patients who subsequently develop metastatic disease. As a result of these advances in several different treatment disciplines, the overall survival rate and quality of life of patients with soft tissue sarcoma have improved markedly over the past 10 years.(ABSTRACT TRUNCATED AT 400 WORDS)

Antineoplastic Combined Chemotherapy Protocols

Thallium-201 scintigraphy in bone sarcoma: comparison with gallium-67 and technetium-MDP in the evaluation of chemotherapeutic response.

This study attempts to characterize thallium-201 (201TI) uptake in patients with bone and soft-tissue sarcoma and to compare these findings with gallium-67 (67Ga) and bone scintigraphy with emphasis on evaluating tumor viability before and after chemotherapy. Thirty-eight patients with surgically-proven sarcomas were evaluated. All patients had gallium and thallium studies. Nineteen patients underwent pre- and post-chemotherapy thallium and evaluation. Seven patients also had technetium-99m-MDP (99mTc-MDP) bone scintigraphy comparisons. Pathologic changes pre- and postchemotherapy were graded on the basis of %tumor necrosis as defined histologically. Scintigraphic comparisons demonstrated a high degree of correlation with 201TI and poor correlation with 99mTc-MDP. Thallium-201 was superior to 99mTc-MDP and 67Ga in predicting tumor response to chemotherapy as determined by %tumor necrosis determined histologically. Gallium was superior to Tc-MDP in predicting response to chemotherapy. However, both 67Ga and 99mTc-MDP appear to be affected by factors other than tumor activity.

Adolescent

Results of core decompression for femoral head osteonecrosis.

The progression of femoral head osteonecrosis (FHON) culminates in destruction of the hip joint, a devastating consequence for young people who are predominantly affected. Core decompression, as a means of ameliorating the disease, was evaluated in 45 hips in 33 patients with nontraumatic FHON. The mean clinical and roentgenographic follow-up period was three years (range, 1-7 years). None of the ten Ficat Stage I hips progressed, 11 of 26 Stage II hips progressed, and five of nine Stage III hips progressed to coxarthrosis. In those hips in which there was an association with steroid therapy, there was 50% progression when steroids were continued, compared with 22% progression when steroids had been discontinued. Core decompression is prescribed for Ficat Stage I and II. Patients on continuous post-operative treatment with steroids for associated conditions should be advised of the high risk of progression.

Adult

The use of MRI to assist in diagnosis of pigmented villonodular synovitis of the knee joint.

Pigmented villonodular synovitis (PVNS) of the knee joint remains a difficult and elusive entity to define and characterize. This entity most often appears in the young adult knee with nonspecific clinical features, including a painful range of motion and perhaps a sensation of locking. Detection and diagnosis of this localized soft-tissue mass are difficult because plain roentgenograms may be totally within normal limits. The case of a 21-year-old woman illustrates the use of magnetic resonance imaging (MRI) as an effective technique to define and characterize PVNS. Advantages of MRI include high-resolution/high-contrast multiplanar sections that depict bone, marrow, ligaments and tendons, fat, menisci, and articular cartilage in one image. In addition, MRI is noninvasive and requires no ionized radiation. MRI is an excellent clinical tool for the evaluation of intraarticular tumors of the knee joint.

Adult

Alveolar soft part sarcoma following radiotherapy for a spinal hemangioma. A case report.

A case of alveolar soft part sarcoma arising some 20 years later in a site previously irradiated as a treatment for a spinal cord hemangioma is described. This is the first known case of radiation-associated alveolar soft part sarcoma, and it fulfills the criteria for a tumor to be radiation-induced. The coincidental finding of "viral-like" particles within some of the tumor cells was noted.

Head and Neck Neoplasms

Parasymphyseal insufficiency fractures of the os pubis.

Twelve parasymphyseal insufficiency fractures of the os pubis are described in eight osteopenic postmenopausal women, three of whom had rheumatoid arthritis. The fractures involved both right and left pubic bones in three women. Radiographically lesions often appear deceptively destructive and malignant. In six patients, biopsy was performed and showed no tumor. Biopsy can be avoided in an osteopenic woman with recent increased activity, pubic pain, and the characteristic pubic lesion.

Aged

Intranuclear branched cylindrical structures in human osteosarcoma cells.

A peculiar branched cylindrical structure within the nuclei of human osteosarcoma cells is reported. Although the described structures may be postulated to represent either "vermicellar bodies" or a response to cellular injury by chemotherapeutic agents, their origin and function are unknown to us.

Adult

Retroperitoneal sarcomas: a reappraisal of treatment.

Retroperitoneal sarcoma, regardless of the histopathologic grade of malignancy, has a high incidence of local recurrence that is usually fatal. Surgical resection is mandatory and resectability may be improved by a midline transabdominal approach. However, even with total tumor excision, recurrence is high. A preliminary study, combining resection and preoperative adriamycin, found to be effective for established disease, suggests potentially improved prognosis. Further clinical trials employing combined with adjuvant chemotherapy are warranted.

Adolescent

Neurofibrosarcoma.

Case histories of 10 patients with neurofibrosarcoma, including 14 (70%) with neurofibromatosis, evaluated over 10 years were reviewed to determine the incidence of local and systemic recurrence and the most effective means of therapy for this rare neoplasm. Initial therapy resulted in complete local disease control in only 11 (55%) patients. Local excision, or local excision plus radiation or chemotherapy resulted in local recurrence in 8 of 12 patients. Radical surgery alone, or radical surgery combined with radiation and chemotherapy resulted in local recurrence in 1 of 6. Even with complete local disease control, 7 of 16 (44%) patients died of metastases. Both A.J.C. Clinical Stage II and III patients had a similar poor prognosis. Associated neurofibromatosis did not worsen prognosis. These data suggest that both moderate and high-grade primary neurofibrosarcoma are highly malignant neoplasms and should be treated by radical resection. Preoperative intraarterial Adriamycin and radiation--found to be successful for other highly malignant sarcomas--may be of benefit. Since distant disease occurs despite local control, postoperative adjuvant chemotherapy trials are warranted.

Adolescent

The fate of pedicle osteocutaneous grafts in mandibulo-facial restoration.

To overcome some of the problems encountered in the repair of extensive composite defects of the head and neck, several means of transfering bone and soft tissue--while preserving the blood supply by means of a pedicle--have been clinically attempted. The superiority of pedicle osteocutaneous grafts compared to free grafts is demonstrated in a series of experiments in monkeys and dogs. Conventional and tetracycline labelled histological specimens, from animals sacrificed at intervals from two weeks to six months after grafting, illustrate the dynamic changes in the grafts. Pedicle supported grafts appeared superior to free grafts and partivipated actively in bone reconstruction.

Animals

Experimental E. coli arthritis in the rabbit. A model of infectious and post-infectious inflammatory synovitis.

Rabbit knee joints challenged with E. coli 06 underwent a self-limited infection lasting several weeks followed by a prolonged post-infectious inflammatory arthritis. The E. coli used did not possess collagenolytic activity nor did a variety of common aerobic clinical isolates. Articular cartilage destruction occurred by two basically different mechanisms: u) direct invasion of pannus at the juxtaarticular margins, and 2) fibrillation in cartilage to cartilage contact areas. Weekly measurement of intra-articular pH and temperature were correlated with bacteriologic findings and groww and microscopic pathologic events.

Animals

Acid, neutral, and alkaline hydrolases in arthritic synovium.

The levels of six lysosomal enzymes (acid phosphatase, beta-acetylglucosaminidase, cathepsin D, beta-galactosidase, arylsulfatase A, and beta-glucuronidase) and four neutral and alkaline hydrolases (esterase, inorganic phyrophosphatase, alkaline phosphatase, and 5'-nucleotidase) were measured in osteoarthritic, rheumatoid and control synovia. All enzyme levels in diseased synovium except esterase values in osteoarthritis were significantly elevated compared with controls. The mean values of the group of acid hydrolases and the group of neutral and alkaline hydrolases in osteoarthritic synovia were 1.9- and 2.0-fold greater than those of control specimens. In rheumatoid synovia, the values were 4.2- and 4.5 fold greater than control for the same enzymes. Levels in rheumatoid synovia were significantly higher than those in osteoarthritic synovia with the exception of 5'-nucleotidase. Only a limited correlation between the extents of inflammation present in the synovia and the levels of a lysosomal marker enzyme (cathepsin D) was observed. These results demonstrate that whatever the mechanism, increased levels of acid hydrolases as well as certain neutral and alkaline hydrolases are present in osteoarthritic and rheumatoid synovia, and these enzymes are probably contained in the synovial lining cells.

Acetylglucosaminidase

Limb salvage from a multidisciplinary treatment approach for skeletal and soft tissue sarcomas of the extremity.

Multimodality management of extremity skeletal and soft tissue sarcomas with preoperative intra-arterial Adriamycin and radiation therapy, radical surgical resection and postoperative chemotherapy or chemo-immunotherapy has resulted in preservation of a functional extremity in 13 or 14 patients. Seven of 8 patients with Stage IIIA and IIIB soft tissue sarcomas, managed with preoperative intra-arterial Adriamycin and radiation therapy, followed by en bloc soft tissue resection and 6 patients with bone sarcomas managed by preoperative treatment, followed by bone resection and replacement with cadaver bone allografts, remained free of disease from 4 to 34 months. The results of the combined modality approach were significantly better than the results obtained in patients managed by surgical resection alone, or by combination of operation with another single modality, both in terms of short term-recurrence free survival and salvage of a functional extremity.

Bone Neoplasms