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Biomedical subjects

J M Mirra

Publications and source records attributed to J M Mirra.

At least 73 records · Page 4Linked to original sources

Histologic features relating to prognosis in synovial sarcoma.

Although the American Joint Commission has classified all synovial sarcomas as "high grade," histologic subtypes can be identified. By histologically subclassifying synovial sarcoma tumors according to percent glandularity and mitotic rates, the authors were able to define high-risk and low-risk patients. Charts and original pathologic slides were reviewed on 45 synovial sarcoma patients. With a 41-month median follow-up, the low-risk patients showed 100% survival, whereas the high-risk patients showed 37% survival.

Actuarial Analysis↗

Pathogenesis of Paget's disease based on viral etiology.

It has been slightly over 100 years since Sir James Paget's classic descriptions of "osteitis deformans" first appeared. He had described the mid- to late stages of patients with the chronic, debilitating, rare, and polyostotic forms of the disease. It is now known that the milder forms of the disease are quite common particularly in those of Anglo-Saxon ancestry. He believed the condition to be a chronic inflammation of unknown etiology because of its asymmetrical skeletal distribution, chronicity, and the gross appearance of the bones. With regard to the possible etiology of Paget's disease of bone, nothing worthy of note had been discovered until 1974 when viral-like inclusions were reported within the osteoclasts of all Paget's disease patients. In the ensuing decade, a great deal more circumstantial evidence from electron microscopic and immunologic studies supports the view that Paget's disease represents a slow virus infection. This article deals with the possible to probable viral etiology of Paget's disease with respect to its pathogenesis and its potential for eventual eradication. For many years Paget's disease was considered a disease almost exclusively confined to adulthood. Evidence now suggests that "familial chronic hyperphosphatasemia" represents the childhood form of Paget's disease.

Adult↗

Ischemic necrosis of the femoral head. Correlation of magnetic resonance imaging and histologic sections.

Magnetic resonance (MR) images were correlated with matched histologic sections of a resected ischemic necrotic femoral head and neck. Preoperative radiographs had revealed Stage 3 ischemic necrosis. Preoperative MR images disclosed foci of abnormally low signal intensity alternating with normal-appearing foci of high signal intensity. Surgical resection of the femoral head and neck was followed by MR imaging and pathologic sectioning, fixation, and staining, enabling the individual sections to be compared with the postoperative MR slices. A subchondral focus of diminished signal intensity was shown to represent saponified fat and consisted of a transudate of proteinaceous material with probable calcifications. The subjacent region of normal, high signal intensity represented "mummified" fat. The next lower stratum of diminished signal intensity was composed of fibrous and vascular tissues and histiocytic infiltrates that had extensively or completely replaced the fatty marrow.

Adult↗

Fine-needle aspiration of primary soft-tissue lesions.

This study presents the cytologic findings of fine-needle aspiration (FNA) in a series of 136 primary soft-tissue tumors. The cytologic appearances of some rare mesenchymal lesions are presented, including the first (to our knowledge) published descriptions of fibrous hamartoma of infancy and myositis ossificans. Fine-needle aspiration showed high diagnostic sensitivity (95%) and specificity (95%) for the determination of malignancy, approaching that obtained with frozen-section interpretation. Problems encountered with the diagnosis of mesenchymal lesions, including nodular fasciitis, Kaposi's sarcoma, and spindle cell lipoma, are discussed. Based on these findings, recommendations for aspiration cytology of soft-tissue tumors are presented.

Adolescent↗

Myxoid malignant melanoma. A previously undescribed histologic pattern noted in metastatic lesions and a report of four cases.

Four patients are presented with malignant melanoma in which the stroma showed a prominent myxoid change. Since this peculiar feature had not been previously reported, metastatic lesions with this change caused considerable diagnostic confusion with other more commonly encountered myxoid tumors. On the basis of light microscopy, special stains, and electron microscopy, we demonstrated the melanomatous nature of the metastases and that the myxoid matrix is derived from reactive stromal cells rather than from the melanoma cells themselves. Three of the four patients had a history of primary melanoma; the longest interval between the primary lesion and the metastasis was 24 years.

Adult↗

Radiology and pathology of malignant fibrous histiocytomas of the soft tissues: a report of ten cases.

Malignant fibrous histiocytomas (MFH) are believed to originate from histiocytes and are composed of malignant cells with spindle or round shapes. We evaluated ten MFH of the soft tissues by plain roentgenograms, computed tomography (CT), and angiography and subdivided them into four grades of anaplasia and five predominant histologic variants. The variants of MFH demonstrated different vascular patterns. The extension of the lesions could be determined by CT and angiography. CT is the method of choice in the assessment of size and extent of MFH of the soft tissues. When intra-arterial chemotherapy is indicated the angiograms obtained at each catheter placement may substitute CT in follow-up studies obviating additional diagnostic procedures.

Adult↗

Rheumatoid bursitis extending into the clavicle and to the skin surface.

A woman with rheumatoid arthritis developed persistent sterile drainage from a cutaneous fistula after biopsy of an inflamed supraclavicular mass. Radiographs showed several cavities in the underlying clavicle. Inability to culture a pathogen and failure of the fistula to heal despite empirical courses of antibiotic therapy led to surgical intervention. The final diagnosis, based on careful histological analysis by special staining techniques, was rheumatoid bursitis extending into the clavicle and to the skin surface.

Adult↗

The UCLA experience in limb salvage surgery for malignant tumors.

Between December 1980 and January 1985, 95 patients with 98 malignant bone tumors were treated at UCLA by a multidisciplinary approach. Seventy-eight patients with 81 lesions underwent primary limb salvage procedures utilizing 66 custom endoprostheses and, in 15 instances, soft tissue reconstruction alone. Sixty-three of 78 (80.7%) underwent successful limb salvage without complication. The 22 local complications in 15 patients (19.2%) were all successfully managed either by surgical or nonoperative techniques salvaging all 15 extremities at risk. Seventeen patients underwent primary amputation for local control of their tumor. The local recurrence rate of 6.4% in the limb salvage group is comparable to the 5.8% found in the amputation group. The selection of patients for limb salvage did not adversely prejudice these patients in terms of rates of progression or ultimate survivorship. The cosmetic and functional results of limb sparing surgery is felt to be at least comparable with that obtained by primary amputation and the use of external prostheses.

Adolescent↗

A new histologic approach to the differentiation of enchondroma and chondrosarcoma of the bones. A clinicopathologic analysis of 51 cases.

Fifty-one cases of central, hyalin cartilage tumors of the long and flat bones were analyzed. Although Grade 2 and 3 chondrosarcomas could be diagnosed on the basis of cytologic features alone, low-grade chondrosarcoma could not be adequately differentiated from pure, benign enchondroma(s) by cytology alone. The tumors can be distinguished by a new histologic approach based on tissue patterns. The crucial enchondroma patterns consist of multiple nodules of hyalin cartilage separated by normal marrow in conjunction with partial to complete encompassing plates of lamellar bone that conform to the irregular shapes of the cartilage nodules. The chondrosarcomatous patterns consist of a single confluent mass of cartilage, which commonly permeates the marrow, "trapping" host lamellar bone on all sides, and which forms bands of fibrosis between the confluent peripheral cartilage lobules. Other less common patterns included cartilagenous infiltration of the Haversian systems or marrow fat and/or the development of a soft tissue mass. A central secondary chondrosarcoma is defined as one that shows the combination of both the enchondromatous and chondrosarcomatous patterns. All 18 of the pure enchondromas diagnosed by the methods proposed in this article behaved with strict benignity (i.e., without evidence of recurrence or metastasis with an average follow-up period of 7.2 years). The 33 primary and secondary chondrosarcomas diagnosed using the described patterns behaved with the predicted frequency of recurrence, metastasis, and patient demise.

Adolescent↗

Myxoid angioblastomatosis of bones. A case report of a rare, multifocal entity with light, ultramicroscopic, and immunopathologic correlation.

An example of multicentric, skeletal, myxoid angioblastomas in a Japanese woman is reported. The disease was symptomatic at age 12 years and was characterized by slowly progressive, multiple, lytic bone defects. In addition the patient had juvenile hypertension, and, at age 20 years, had focal brain infarction. The primitive vascular nature of the process was supported by the following observations: occasional erythrocytes within cytoplasmic lumina and capillary-like cellular tubes; Weibel-Palade bodies, numerous pinocytotic vesicles, prominent microvilli, elaborate intercellular contacts, desmosomes, and numerous arrays of fine intracytoplasmic filaments by electron microscopy; and, in addition, Factor VIII positivity. The clinical findings in this case are more consistent with a multicentric, rather than a metastatic process. The name myxoid angioblastomatosis of bones is appropriate.

Adult↗

Synovial sarcoma with squamous differentiation of its mesenchymal glandular elements. A case report with light-microscopic, ultramicroscopic, and immunologic correlation.

A 25-year-old male patient with a biphasic synovial sarcoma of his right flank is reported. The tumor was not only richly glandular but also showed a rare finding in association with a sarcoma, namely, squamous differentiation. Light microscopy and electron microscopy demonstrated the classical features of a biphasic synovial sarcoma except for foci of squamous change. Antibody stain directed against keratin demonstrated this substance in both the glandular and squamous cells but not in the stromal elements. Therefore, the finding of squamous differentiation in association with a malignant, deep soft-tissue tumor must include in the differential diagnosis not only carcinomas but rare sarcomas as well, including the biphasic synovial sarcoma and the glandulosquamous variant of malignant schwanomma.

Adenocarcinoma↗