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Glycosaminoglycans of pleural mesothelioma: a possible biochemical variant containing chondroitin sulfate.

Glycosaminoglycans of a malignant pleural mesothelioma have been characterized histochemically and biochemically and compared with those of normal lung, pleural plaque, lung carcinoma, and other connective tissue neoplasms. Chondroitin sulfate constituted the major glycosaminoglycan (approximately 80% of total) present in the pleural mesothelioma while hyaluronic acid was present in only trace amounts (approximately 3% of total). In particular chondroitin 6-sulfate was the predominant isomer, constituting 80% of the total chondroitin sulfate. Control tissue exhibited different proportions of glycosaminoglycans and none of them contained as high an absolute concentration of chondroitin sulfate as the mesothelioma. These findings differ from previous reports demonstrating increased concentration of hyaluronic acid in mesothelioma and suggest the possible existence of a biochemically different form of this neoplasm.

Aged↗

The World Health Organization's histologic classification of bone tumors. A commentary on the second edition.

BACKGROUND: The World Health Organization's histologic classification of bone tumors was revised in 1993. METHODS: The first edition was reviewed by an international panel of pathologists from nine countries and modified to incorporate advances made in the twenty years since it appeared. RESULTS: The framework and concept of the classification remain the same (i.e., based on histologic criteria in terms of differentiation shown by the tumor cells via conventional light microscopy supplemented by immunohistochemistry). New entities not described in the first edition include, for example, benign and malignant fibrous histiocytomas, well differentiated osteosarcoma, round-cell osteosarcoma, clear cell chondrosarcoma, primitive neuroectodermal tumor of bone, osteofibrous dysplasia, and giant cell reparative granuloma. CONCLUSION: The revised and expanded classification reflects advances in our knowledge, but is similar in concept and framework to the original version to allow comparisons between data collected in the past and future.

Humans↗

Evaluation of Epstein-Barr virus-associated nuclear antigen with various human cell lines.

Critical evaluation of the anti-complement immunofluorescence (ACIF) test for Epstein-Barr virus-associated nuclear antigen (EBNA) in known EBV-associated cell lines was carried out. The ACIF procedure, in which the cell smears were thoroughly air-dried and fixed with carbon tetrachloride (for 15 min at room termperature), provided reproducible staining results with minimum variations. The fixed smears coulb be stored for at least 5 weeks at minus 80 degrees C without loss of reactivity. On the basis of the above evaluation, a total of 59 human cell lines, which had unknown or uncertain association with EBV, were examined for the presence or absence of EBNA by the ACIF procedure. All of the cell lines tested could be judged positive or negative for EBNA. All of 21 lines growing as monolayer or in a mixed state of monolayer and suspension, were negative. Of 38 lines grown in suspension, 36 lymphoblastoid lines were positive but the remaining 2 lines (seemingly not lymphoblastoid) were negative.

Animals↗

Ultrasonic effects on mammalian multicellular tumor spheroids.

Mammalian multicellular tumor spheroids (MTS), grown in liquid culture medium, 0.23 to 0.32 mm in diameter and consisting of 4,500 to 12,100 cells, were exposed to three ultrasonic machines: a clinical diagnostic device with an emitted intensity of 13 mW/cm2, a therapeutic machine with emitted intensities of 1 to 3.5 W/cm2, and a laboratory emitting 12 to 50 W/cm2. (All intensities are temporal and spatial average.) Four measures of ultrasonic effect on MTS were made: decrease in diameter after treatment, damage to subsequent growth outright kill, and number of cells detached from the MTS. There was no loss in MTS size from diagnostic or therapeutic exposures. None of the exposures from any of the three machines caused any damage to subsequent growth or survival. There was no significant detachment of cells from the MTS by any of the diagnostic exposures (at 13 mW/cm2, out of 40 minutes). With the laboratory machine, a significant number of cells were detached, about 0.02% of the MTS (approximately one cell per MTS) per W/cm2 in a ten-minute exposure. Cells detached by therapeutic exposure increased linearly with exposure. On the average, 1 W/cm2 detaches about 0.5 cells per minute, per MTS, or about 0.006% of the cells in the MTS.

Animals↗

Brachyury, a crucial regulator of notochordal development, is a novel biomarker for chordomas.

Chordomas are malignant tumours that occur along the spine and are thought to derive from notochordal remnants. There is significant morphological variability between and within chordomas, with some showing prominent areas of chondroid differentiation. Our microarray data from a broad range of connective tissue neoplasms indicate that, at the transcriptional level, chordomas resemble cartilaginous neoplasms. Here we show that chordomas express many genes known to be involved in cartilage development, but they also uniquely express genes distinguishing them from chondroid neoplasms. The brachyury transcription factor, known to be involved in notochordal development, is only expressed by chordomas. Using a polyclonal antibody, we show that brachyury is expressed in the embryonic notochord and in all 53 chordomas analysed, labelling both chondroid and chordoid areas of these tumours. In contrast, the protein was not detected in over 300 neoplasms, including 163 chondroid tumours. Brachyury was not detected in the nucleus pulposus, arguing against the hypothesis that this tissue derives directly from the notochord. These data provide compelling evidence that chordomas derive from notochord and demonstrate that brachyury is a specific marker for the notochord and notochord-derived tumours.

Biomarkers, Tumor↗

Retrorectal tumors. Mayo Clinic experience, 1960-1979.

One hundred twenty patients with primary retrorectal tumors (79 congenital, 14 neurogenic, 13 osseous, and 14 miscellaneous) had their initial treatment at the Mayo Clinic from 1960 to 1979. The mean age was 43 years (100 patients were adults). Female predominance was associated with congenital cysts (15:1) and male predominance with chordomas (5:1). Forty-three percent of the patients had malignant lesions. No dermoid cysts were found in this series. Diagnosis was made by digital examination or sacral radiographs in all patients. Computed tomography scan was the most important diagnostic method; the rate of positive findings was 100 percent in 20 patients. Approach to the tumor was posterior in 79 of 102 patients in whom resection was possible. Ten of 66 patients with benign tumors had recurrence. The five-year survival rate for patients with chordomas was 75 percent and for patients with other malignant lesions was 17 percent. Because preoperative biopsy can cause tumor spread, abscess, fecal fistula, or meningitis, it should not be performed if tumors are potentially resectable. Whenever possible, total resection should be done.

Adolescent↗

[Hereditary prolidase deficiency. Contribution to differential therapy refractory leg ulcer diagnosis].

Leg ulcers may be caused by many different diseases. Most frequently, they are due to vasculopathies, to a lesser extent to metabolic, neuropathic or hematologic diseases. Neoplasms, connective tissue diseases, infections, trauma, and panniculitis should also be included in the differential diagnosis. A 38-year-old Caucasian female patient with hereditary prolidase deficiency developed progressive and very painful leg ulcers. The ulcers first appeared in childhood and did not respond to various treatments. Additional features of prolidase deficiency included mental retardation, short stature, extensive dental caries, and multiple malar teleangiectases. Hereditary prolidase deficiency is a very rare autosomal recessive disease. It is caused by heterogeneous mutations of the prolidase gene and affects many aspects of protein metabolism. Ion exchange chromatography and high voltage electrophoresis of urine can prove the suspected diagnosis. So far, there is no efficient therapy for hereditary prolidase deficiency. All reported treatment attempts have ended in failure.

Adult↗

Smooth muscle tumours of the larynx.

Two cases of laryngeal smooth muscle tumours are reported: one a benign leiomyoma, the other a malignant leiomyosarcoma. These tumours may present diagnostic difficulties and immunocytochemistry is helpful in distinguishing smooth muscle tumours from other connective tissue neoplasms and spindle cell squamous carcinoma. Primary treatment in both cases should be surgical resection with small (T1, T2) malignant tumours suitable for partial laryngectomy. Postoperative radiotherapy may have a role in allowing a more limited surgical resection.

Adult↗