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Biomedical subjects

J M Mirra

Publications and source records attributed to J M Mirra.

At least 19 recordsLinked to original sources

Benign giant cell tumor of bone with osteosarcomatous transformation ("dedifferentiated" primary malignant GCT): report of two cases.

It is not uncommon for sarcomatous transformation of giant cell tumor (GCT) of bone to occur after radiation, but rarely does malignant transformation occur spontaneously, with less than 15 cases reported up to 1995. Only four of these cases have been documented in detail. We report two additional cases of GCT of bone spontaneously transforming or "dedifferentiating" into osteosarcoma without radiation therapy. The first case is absolutely unique and most interesting in that the dedifferentiation process occurred in one of multiple GCT lung metastases 6 years after successful eradication of a primary tibial tumor. The right lung was resected due to development of a large tumor, and at pathologic examination, demonstrated several small nodules of conventional GCT and a much larger, 14-cm mass composed of a mixture of GCT and high-grade osteosarcoma. The second case involved a physician, who had a large tumor in the sacrum with vague symptoms for 8 years. Open biopsy revealed conventional, benign GCT of bone with a secondary aneurysmal bone cyst. Complete curettage 2 weeks later revealed, in addition to areas of conventional, benign GCT a second component of very high grade osteosarcoma. Both patients died less than 1.5 years from diagnosis. This report of osteosarcomatous transformation of a conventional GCT of bone strengthens the theory that there is a mesenchymal cell line in GCT that may spontaneously transform to sarcoma.

Adult

Benign and malignant cartilage tumors of bone and joint: their anatomic and theoretical basis with an emphasis on radiology, pathology and clinical biology. I. The intramedullary cartilage tumors.

We reviewed 845 cases of benign and 356 cases of malignant cartilaginous tumors from a total of 3067 primary bone tumors in our database. Benign cartilaginous lesions are unique because the epiphyseal plate has been implicated in the etiology of osteochondroma, enchondroma (single or multiple), periosteal chondromas and chondroblastoma. In the first part of this paper, we will review important clinical, radiologic and histologic features of intramedullary cartilaginous lesions in an attempt to support theories related to anatomic considerations and pathogenesis.

Adolescent

Clear-cell chondrosarcoma with elevated alkaline phosphatase, mistaken for osteosarcoma on biopsy.

This paper report a case of a pelvic clear-cell chondrosarcoma that was initially diagnosed as clear-cell osteosarcoma after needle biopsy. Preoperatively, laboratory studies revealed an elevated alkaline phosphatase and needle biopsy showed clear cells producing osteoid. Internal hemipelvectomy was performed and final pathology was most consistent with clear-cell chondrosarcoma, despite a negative S-100 immunohistochemical stain. Distinguishing between these two entities is clinically important because treatment differs. In this case, the patient did not receive pre-or postoperative chemotherapy and is disease free more than 2 years later.

Acetabulum

Osteosarcoma and Ewing's sarcoma in a retinoblastoma patient.

Retinoblastoma, though uncommon, frequently is associated with other second malignant neoplasms, especially bone sarcomas. These second neoplasms can occur in sporadic cases of retinoblastoma, though they more commonly are associated with genetic forms of the disease. This is the first known report of 2 histologically distinct bone sarcomas in a patient with retinoblastoma.

Child

Tissue reaction to metal on metal total hip prostheses.

The periprosthetic tissue reaction to polyethylene wear debris in metal on polyethylene total hip replacements is strongly implicated as the cause of osteolysis. This has led to a renewed interest in metal on metal total hip replacements. However, little is known about the role of wear debris in failures of these prostheses. Capsular and interface tissues from 9 long and short term metal on metal total hip replacement retrievals were studied to assess the tissue reaction around these prostheses. As compared with metal on polyethylene cases, the extent of the granulomatous inflammatory reaction and the presence of foreign body type giant cells was much less intense in metal on metal cases, likely because of the lower numbers and overall smaller size of metal wear debris particles. This may lead to a better transport of the particles from the joint tissues and a lower incidence of periprosthetic osteolysis around metal on metal hip replacement.

Adult

Ribbing disease (multiple diaphyseal sclerosis): imaging and differential diagnosis.

OBJECTIVE: This study describes the clinical presentation and the course of Ribbing disease in six patients and illustrates imaging features on plain radiography, conventional and computed tomography, and 99mTc-methylene diphosphonate bone scans. MATERIALS AND METHODS: Between 1982 and 1990, six female patients presented with painful bony lesions that were believed to be Ribbing disease. Ten bones were affected: both tibiae in three patients, a unilateral tibia in one, both femora in one, and a unilateral femur in one. Plain radiographs and either conventional or computed tomography were available for all patients and 99mTc-methylene diphosphonate bone scans, for five patients. All patients underwent open biopsy and/or surgical decompression. RESULTS: The diagnosis was reached in all patients through a combination of clinical findings (lack of systemic signs of infection or laboratory values suggesting metabolic bone disease), imaging, histologic evaluation, and specimen cultures. Radiographs and tomographic studies showed benign-appearing endosteal and periosteal cortical thickening. Intense uptake of radionuclide tracer was confined to the shaft of all involved bones. All pathologic specimens revealed nonspecific changes that included a slow increase in the mass of cortical and endosteal bone. These specimens also assisted in excluding neoplastic or infectious causes for the new bone formation. CONCLUSION: Ribbing disease is a rare disorder that, on imaging studies, may simulate stress fracture, chronic infection, bone-forming neoplasia, or a systemic metabolic or endocrine disorder. Clinical and imaging features may suggest the correct diagnosis.

Adult

Multifocal pigmented villonodular synovitis in a child. A case report.

Pigmented villonodular synovitis is a well-described disease that almost universally involves a single site. This is a report of an unusual case of multiple site involvement of pigmented villonodular synovitis in a child. In addition to multiple site involvement, the case is unusual for several reasons: asymmetric involvement, involvement of both upper and lower extremities, involvement of the pes anserine tendons, and the patient is an otherwise healthy child.

Biopsy

Primary malignant mesenchymoma of bone: case report, literature review, and distinction of this entity from mesenchymal and dedifferentiated chondrosarcoma.

The seventh case of primary malignant mesenchymoma of bone is reported. It is compared to the other six cases in the literature and clinicopathologic pattern of this entity is developed. Primary malignant mesenchymoma of bone is compared to mesenchymal chondrosarcoma and dedifferentiated chondrosarcoma. Differences as well as similarities are discussed and a more current classification scheme for malignant mesenchymoma of bone is proposed.

Bone Neoplasms

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

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

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

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

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

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