Biomedical subjects
Publications and source records attributed to J Bjornsson.
In two patients, hematopoietic cells were found on cytologic study of fine-needle aspirates from the thyroid gland. The cells were thought to have been aspirated from ossified laryngeal tissues and to have no significance for hematologic disorders in these two patients.
Among 80 cases with a clinical diagnosis of primary (unexplained) pulmonary hypertension, 45 (56%) had thromboembolic disease and 22 (28%) had plexogenic arteriopathy; the remaining 13 (16%) had pulmonary veno-occlusive disease, primary medial hypertrophy, primary pulmonary arteritis, or changes consistent with pulmonary venous hypertension. The mean age was 16 years for primary pulmonary arteritis, 21 to 34 years for plexogenic pulmonary arteriopathy, primary medial hypertrophy, and pulmonary veno-occlusive disease, and 41 and 45 years for thromboembolic disease and pulmonary venous hypertension, respectively. In all forms except pulmonary veno-occlusive disease and apparent pulmonary venous hypertension, female patients were involved twice as often as male patients. With the exception of apparent pulmonary venous hypertension, patients with plexogenic pulmonary arteriopathy had the longest survival (63 months). Sudden death, however, occurred most frequently in patients with plexogenic disease (45%) and occurred 2.5 times as often in this group as in patients with thromboembolic disease. Among our 80 cases, the most frequent histopathologic lesions were medial hypertrophy, intimal proliferation and fibrosis, fibrinoid degeneration and necrosis, and thrombosis. Thrombi were commonly observed and may have developed in situ or by embolization; they were often rich in platelets when they occurred in small pulmonary vessels. These histologic features may form the morphologic substrate for elevated pulmonary vascular resistance, and their recognition may provide the rationale for possible intervention with pulmonary vasodilators, anticoagulants, or platelet inhibitors.
Eight cases of Paget's disease of genital mucosa with malignancy of the lower urinary tract are described. In five it was apparent that there was concurrence of two separate malignancies. In one patient with long-standing Paget's disease of the genital mucosa, carcinoma of the urethra and bladder developed subsequently in continuity with the genital lesion; the two lesions were indistinguishable histologically, suggesting extension of the Paget's disease into urothelium. In two patients with bladder malignancy, there was histologic evidence of outward pagetoid extension of this process along urothelium and onto the genital mucosa. The significance of genital Paget's disease is discussed in the light of these findings, and the possible origins of Paget cells within the epidermis are reviewed.
Seventy cases of histologically verified intracranial germ cell tumor were reviewed: 43 germinomas, 16 immature teratomas, seven mature teratomas, two embryonal carcinomas, one choriocarcinoma, and one yolk sac tumor. The male-to-female ratio was 2.6:1. The average age was 19 years in patients with germinoma, 11 years in patients with immature teratoma, and 17 years in patients with mature teratoma. Duration of symptoms averaged 19 months for germinoma, three months for immature teratoma, and 11 months for mature teratoma. Sixty-six lesions were located in the midline. Fifty-eight percent of the germinomas arose anterior to the pineal gland, whereas 29% of the immature and 14% of the mature teratomas were located anteriorly. The histologic appearance of the germinomas was indistinguishable from that of the usual testicular seminoma. The immature teratomas contained tissue from all three germ layers and exhibited morphologic features of fetal tissue. Of 14 immature teratomas, seven contained, in addition, foci of other malignant germ cell elements; thus, there were two teratocarcinomas, two lesions with germinoma and immature teratoma, two lesions with extensive rhabdomyoblastic differentiation in an immature teratoma, and one lesion with both germinoma and embryonal carcinoma in addition to immature teratoma. The seven mature teratomas consisted of fully differentiated epithelial and mesenchymal tissues. In 23 cases, immunoperoxidase stains for human chorionic gonadotropin (HCG), alpha-fetoprotein (AFP), and carcino-embryonic antigen (CEA) revealed patterns which, with minor exceptions, were essentially identical to those found in genital germ cell lesions. Survival was longest for patients with germinomas. In classifying germ cell tumors of the central nervous system, the World Health Organization's (WHO) classification of testicular germ cell tumors is preferable to its present classification of intracranial germ cell tumors.
The symptoms of progressive dyspnea, hemoptysis, spontaneous pneumothorax, chylous effusions, and cough in conjunction with ventilatory obstruction and abnormal gas exchange in a young woman should prompt the diagnosis of pulmonary lymphangiomyomatosis. Cytosol steroid-receptor assays and postmortem studies were conducted in an extensive investigation of a case of this disease. A biopsy specimen of the lung disclosed evidence of nuclear translocation of [3H]progesterone and the presence of a cytosolic receptor for progesterone, an indication that this disease could be treated effectively with progestin.
Clear cell chondrosarcoma of bone, a low-grade malignant tumor, frequently presents diagnostic difficulties. Its clinical, roentgenographic, and pathologic characteristics separate it from conventional chondrosarcoma and, more importantly, from several benign bone tumors with which it is often confused. This report analyzes 47 cases seen at the Mayo Clinic and in consultation. The lesion is more common in males than females (2.6:1) and has a predilection for the end of long bones, particularly the proximal femur. The age range is wide; most patients are in the third and fourth decades of life. Roentgenographically, the lesion most often is purely lytic and slightly expansile, with a sharp margin between the tumor and the adjacent normal bone. Pathologically, clear cells arranged in an indistinct lobular pattern characterize the tumor. Frequently, areas mimicking other primary bone tumors, benign and malignant, are present, often to such an extent as to obscure the true identity of the process. The overall mortality in the present series was 15%. En bloc resection, including a margin of normal bone and soft tissue, appears to be the treatment of choice.
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A 10-year-old girl had a primary choriocarcinoma of the posterior third ventricle. Craniotomy a few hours before death did not yield any tumor tissue. At autopsy, an extensively hemorrhagic tumor abutted the pineal gland. Immunostains were positive for beta-human chorionic gonadotropin (beta-HCG) but were negative for alpha-fetoprotein and carcinoembryonic antigen. The presence of beta-HCG in serum or cerebrospinal fluid may be used as a diagnostic marker and monitor of therapy. HCG is, however, not a unique marker for trophoblastic neoplasms, as a significant number of intracranial germinomas contain cells that are beta-HCG positive. Because of the rarity of primary extragenital choriocarcinomas and the much more common occurrence of metastases of genital choriocarcinomas, it is doubtful whether any investigation less than detailed autopsy can prove the extragenital origin of the tumor.
The application of positional cloning to the study of families with inherited renal neoplasms has allowed the identification of genes which are also important in the pathogenesis of sporadic tumors and has advanced the understanding of the mechanisms underlying renal carcinogenesis. This review provides a discussion of these mechanisms as well as the diagnosis and management of the inherited renal neoplasms.