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Biomedical subjects

J M Mirra

Publications and source records attributed to J M Mirra.

At least 37 records · Page 2Linked to original sources
PubMed1995

Giant bone island of femur. Case report, literature review, and its distinction from low grade osteosarcoma.

A 42-year old male with the largest reported giant bone island (10.5 cm in length) is presented. Due to its ominous size, association with some degree of pain and increased uptake on radionuclide bone scan, a biopsy was considered necessary definitively to rule out a slow-growing osteosarcoma or blastic metastasis. Documentation of growth in adult patients of conventional and giant bone islands, coupled with evidence of increased radionuclide uptake, makes the clinicoradiological distinction between bone islands and blastic malignancies difficult. Guidelines for biopsy versus serial radiographic follow-up of such lesions are addressed.

Adult↗
PubMed1995

Paget's disease of bone: review with emphasis on radiologic features, Part I.

Distinctive clinical, radiologic and pathologic features are seen in Paget's disease of bone. These distinct features can be divided into three phases; initial phase, midphase, and late phase. The clinical features may vary from patients being asymptomatic (involving a single bone) to patients having severe, multiple bone involvement with systemic disease. Radiologically, there are unique features which can differentiate Paget's disease from other bone diseases. The radiologic features follows the histologic findings on light microscopy. The correlation of the different phases with the clinical, radiologic and pathologic features of Paget's disease allows for early accurate diagnosis and treatment.

Adult↗
PubMed1995

Paget's disease of bone: review with emphasis on radiologic features, Part II.

Part I discusses the correlation between the clinical, radiology and histologic features with the three phases (incipient, mid- and late phase) of Paget's disease. In this section, we will discuss in detail the radiologic features by location as well as aberrant radiographic presentations in addition to secondary tumors such as post radiation sarcomas and giant cells tumors which occur in Paget bone. Because Paget's disease generally affects people in their middle and late ages, the differential diagnosis often includes metastatic disease and the differentiation can often be quite challenging. Moreover, metastatic disease to bones afflicted with Paget's disease can further add diagnostic confusion. These critical aspects will be discussed in this section of Paget's disease of bone.

Adult↗
PubMed1995

Chondroblastoma arising from a nonepiphyseal site.

Chondroblastoma is a rare, benign primary bone tumor usually involving secondary centers of enchondral ossification. The consistent epiphyseal location in the great majority of chondroblastomas signifies that the tumor may arise from an aberrant germ cell of the physeal plate. This case report describes a chondroblastoma located in a atypical nonphyseal location, namely in the right fourth metatarsal base. Cases such as these imply that the cell of origin may not exclusively be derived form the physeal plate. The clinical-radiographic significance is that historically chondroblastoma is considered an epiphyseal lesion, but rare cases such as the one reported here and those of the skull demonstrate that this is not always a characteristic of these tumors. The implication from a histogenetic standpoint is that chondroblastoma may, on rare occasions, occur in an area of an enchondrally formed bone other than adjacent to the physeal plate. We are presently investigating the histogenetic relationship of the chondroblastoma (CB) of bone to CB of soft tissue, giant-cell tumor of tendon sheath (GCT-TS), pigmented villonodular synovitis (PVNS) and chondroma of tendon sheath (chondroma of soft parts). We now have collected about 15 cases of GCT-TS and PVNS with extensive areas of chondroid and/or cartilage differentiation that cannot be distinguished from CB of bone by histologic or electron microscopic features alone. From these interesting observations we are developing the histogenetic concept that all of these lesions are interrelated to multipotential mesenchymal and/or synovial cells of the tendon sheath.(ABSTRACT TRUNCATED AT 250 WORDS)

Adult↗
PubMed1994

Case report 823: Fibro-osseous pseudotumor of the scapula.

A unique lesion discovered in the scapula of a 36-year-old woman is presented. The lesion has microscopic features resembling those of fibrous dysplasia and osteoid osteoma which we believe is identical to an entity previously only documented in the rib (fibro-osseous lesion of rib). We are of the opinion that the lesion probably represents a reactive response to trauma rather than a neoplasm and suggest the name of "fibro-osseous reparative pseudotumor" for this entity.

Adult↗
PubMed1994

Case report 827: Miliary osteosarcomatosis.

We presented the case of a 26-year-old woman with a blastic, miliary form of osteosarcomatosis involving the axial skeleton, skull, and long bones to the elbow and knee joint regions who subsequently developed hypocalcemic tetany. Radiologically the lesions mimicked the spread of breast carcinoma, and because of the distribution of the lesions we surmised that the spread was by means of Batson's vertebral plexus of veins. The literature on multiple osteosarcomas was reviewed and an updated clinical subclassification of patients with multiple skeletal osteosarcomas presented.

Adult↗
PubMed1994

Pulmonary metastases of stage IIB extremity osteosarcoma and subsequent pulmonary metastases.

PURPOSE: This study investigated prognostic factors in nonmetastatic high-grade extremity osteosarcoma and the prognosis following resection of subsequent pulmonary metastases, with emphasis on the effect of chemotherapy-induced tumor necrosis. PATIENTS AND METHODS: We reviewed 111 consecutive patients with high-grade nonmetastatic extremity osteosarcoma treated with preoperative chemotherapy and surgical resection, with additional review of 36 patients who had subsequent pulmonary metastases resected. RESULTS: The overall 5-year survival rate was 53%. In resected primary tumors, tumor-free resection margin (P < .001) and increasing chemotherapy-induced tumor necrosis (> 90% threshold, P < .003) correlated with increased metastasis-free survival. Relative risk factors for metastases were as follows: tumor-containing resection margin (most likely to metastasize); poor response to preoperative chemotherapy and/or lack of postoperative chemotherapy (next worse prognosis); and excellent response to preoperative chemotherapy (> or = 90% necrosis) combined with postoperative chemotherapy (best prognosis). The 5-year survival rate following pulmonary metastasis resection was 23%, whereas a 0% 4-year survival rate followed development of bony metastases (P < .001). The extent of tumor necrosis in resected pulmonary metastases did not affect prognosis. Survival was best in patients with three or fewer pulmonary nodules (P < .048), four or fewer recurrent pulmonary nodules (P < .047), unilateral pulmonary metastases (P < .037), or longer intervals between primary tumor resection and metastases (P < .082). CONCLUSION: Intensive preoperative and postoperative chemotherapy combined with complete resection of both primary and metastatic pulmonary osteosarcomas is justified, with a goal of 100% tumor necrosis and excision. Although current treatment regimens allow effective salvage therapy for a few patients with pulmonary metastases, more effective systemic treatment is needed.

Adolescent↗
PubMed1994

Pulmonary metastasis of benign giant cell tumor of bone. Six histologically confirmed cases, including one of spontaneous regression.

Benign giant cell tumor of bone, despite being classified as benign, has the unusual ability to metastasize. Metastasis of such a tumor has been thought to be rare, with only approximately 50 such cases having been reported. However, as awareness of the metastatic potential of these tumors has increased, and methods of detection have improved, metastasis of benign giant cell tumor has been increasingly recognized. Six patients with pulmonary metastasis of giant cell tumor have been treated at a Los Angeles hospital since 1980. This represents 9.1% of all patients treated for benign giant cell tumor of bone over the same period at this institution, a higher rate than that encountered in previously published series. The early detection and treatment of this tumor is important, because those with complete resection of tumor have the best prognosis. The nature of these pulmonary metastases remains unpredictable, however, as evidenced by two of the cases in this series: one of spontaneous regression, and another of death caused by pulmonary failure.

Adolescent↗
PubMed1993

Extraskeletal telangiectatic osteosarcoma.

The first three well-documented cases of pure, extraskeletal telangiectatic osteosarcoma of the soft tissues are presented in this article. The distinctive gross features were a predominance of large, blood-filled spaces, which, on histologic examination, were large blood-filled cavities in association with cyst-like walls containing anaplastic spindle cells and definite osteoid production in two of three cases. One of the cases is of additional clinical interest because the teen-aged daughter of the woman patient had died 1 year previously of a high-grade intramedullary osteosarcoma of bone.

Adult↗
PubMed1993

Case report 797: "Dedifferentiated" lipoma-like liposarcoma of soft tissue with focal transformation to high-grade "sclerosing" osteosarcoma.

We have presented the first case of a lipoma-like liposarcoma of the soft tissues with high-grade malignant transformation to a high-grade osteosarcoma, the latter component of which responded well to systemic chemotherapy. With 4 months of follow-up the patient is without evidence of disseminated disease. The various types of liposarcoma are listed and several described in some detail. Of particular importance is the potential for development of a highly malignant sarcoma from a relatively indolent low-grade liposarcoma, with even osteosarcoma developing.

Aged↗
PubMed1993

Case report 771: Melorheostosis in association with desmoid tumor.

Melorheostosis is a rare congenital disease, some 300 cases have been reported in the literature. The main purpose of this case report is twofold: (1) to present an unusual case of melorheostosis of the right hand, beginning in infancy, with serial radiographic studies to show the gradual progression of the disease; and (2) to present the first case with the development of "aggressive" fibromatosis (desmoid tumor) of the adjacent soft tissues, associated with melorheostosis.

Child↗
PubMed1993

Serial thallium-201 scintigraphy in osteosarcoma. Correlation with tumor necrosis after preoperative chemotherapy.

In 27 patients with extremity lesions (24 osteosarcoma, three malignant fibrous histiocytoma), a notable decrease in thallium-201 uptake was correlated with a good response to preoperative chemotherapy of the primary tumor. A good response is indicated by a greater than 95% tumor necrosis. Serial quantitative thallium-201 uptake of malignant bone tumors in patients receiving preoperative chemotherapy therefore can accurately predict a good histologic response and prognosis. Serial thallium scintigraphy can furthermore identify poor responses within two weeks after the initiation of treatment, or can prompt an early change in preoperative chemotherapy and facilitate limb salvage surgery.

Adolescent↗
PubMed1992

The fibroma-like variant of epithelioid sarcoma. A fibrohistiocytic/myoid cell lesion often confused with benign and malignant spindle cell tumors.

Five cases of a previously undescribed variant of epithelioid sarcoma are presented. This variant differs from the usual lesion in its absence of the typical necrobiotic nodular epithelioid pattern. It is instead composed of deceptively bland fibrohistiocytic and myoid cells arranged in a fibroma-like or dermatofibroma-like pattern with an affinity for osseous involvement. The clinical presentation, ultrastructural features, and presence of vimentin and low molecular weight keratin within the tumor cells justifies their designation as an epithelioid sarcoma variant.

Adolescent↗
PubMed1992

Case report 748: Chondroblastoma of the femur with an aneurysmal bone cyst.

A case of chondroblastoma associated with an aneurysmal bone cyst has been described. The radiographic appearance of the lesion understandably reflects the combined features of both tumors. Up to one-half of all cases of aneurysmal bone cysts are found in association with other tumors, benign or malignant, and up to 15% of chondroblastomas are combined with an aneurysmal bone cyst (4).

Adolescent↗