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G Mendelsohn

Publications and source records attributed to G Mendelsohn.

At least 19 recordsLinked to original sources

Adenoid cystic carcinoma of the breast.

Adenoid cystic carcinoma of the breast is a rare neoplasm, with only 140 cases having been reported to date. Data on 123 of these cases are reviewed herein and another case is presented in detail. Several features distinguish this type of breast cancer from more typical histologic types and suggest that it may have a unique tumor behavior. The prognosis appears to be favorable and the incidence of axillary lymph node involvement is lower. Distant metastases are uncommon, but they tend to occur without prior lymph node involvement. This lack of prognostic significance for negative axillary lymph nodes underscores the need for other prognostic markers in this disease and suggests that axillary dissection can be eliminated in most cases. Similarities to typical breast cancer include the incidence of local recurrence and the lack of effect of surgical treatment on distant metastases and overall survival. These data suggest that breast-conserving treatment may be applicable to adenoid cystic carcinoma.

Breast Neoplasms

Somatostatin-producing neuroendocrine tumor of the ampulla (ampullary somatostatinoma). Evidence of prosomatostatin production.

Two cases of somatostatin-producing ampullary neuroendocrine tumors (somatostatinoma) are reported. The authors have characterized their immunoreactivity using antibodies specific for the amino- and carboxyl-terminal portions of prosomatostatin, the precursor of somatostatin in the normal synthetic pathway. Cytoplasmic staining was found using each of these two antibodies in the tumor cells of both ampullary somatostatinomas as well as in the cytoplasm of cells in the hypothalamus, crypt cells of the duodenal mucosa, mucosal cells of the biliary tract, D cells of the pancreatic islets, and parafollicular cells of fetal thyroid. These studies suggest that the synthesis of somatostatin in ampullary somatostatinomas occurs through the normal pathway from the precursor prosomatostatin.

Ampulla of Vater

Report of a case of localized Castleman's disease with progression to malignant lymphoma.

Patients with multicentric Castleman's disease have an increased risk of developing non-Hodgkin's lymphoma. However, development of lymphoma in the localized form of Castleman's disease has not been previously reported. This case study describes a patient with localized Castleman's disease, hyaline vascular type, whose course was complicated by follicular non-Hodgkin's lymphoma.

Adolescent

Infective endocarditis during the first decade of life. An autopsy review of 33 cases.

Twenty-six cases of infective endocarditis seen at autopsy during the first decade of life, between 1911 and 1944, are compared with seven fatal cases between 1944 and 1977. The incidence of infective endocarditis at autopsy in this age-group has decreased since 1944 (0.60% to 0.23%). Before 1944, Gram-positive cocci were most frequently responsible. Rheumatic heart disease (31%) more often predisposed to infective endocarditis than did congenital heart disease and left-sided valves were most frequently involved. Peripheral septic foci and penumonia were the most frequent sources of infection. The introduction of penicillin and advances in cardiology and cardiovascular surgery have had considerable impact of the spectrum and evolution of the diseases. Fungi and uncommon opportunistic bacteria are now frequently encountered. Rheumatic heart disease rarely predisposes to infective endocarditis in this age-group, congenital heart disease being the major underlying disease.

Age Factors

Levels of histaminase and L-DOPA decarboxylase activity in the transition from C-cell hyperplasia to familial medullary thyroid carcinoma.

The distribution of calcitonin, L-dopa decarboxylase, and histaminase is studied in sectioned total thyroid glands from patients with different stages of familial medullary thyroid carcinoma. In 5 glands with gross carcinoma and in 3 with early microscopic carcinoma the distribution of all three parameters positively correlates (p less than .01 for each correlation). In contrast, in 6 glands with C-cell hyperplasia only the distribution of calcitonin and L-dopa decarboxylase correlates (r = 0.64, p less than .01) while those for histaminase vs. calcitonin (r = .17, p = N.S.) and histaminase vs. dopa decarboxylase (r = .03, p = N.S.) do not. In the glands with microscopic carcinoma the peak levels of histaminase occur in the areas of disease as defined by immunohistochemical staining of calcitonin; mean histaminase activity is the only one of the three parameters measured that distinguishes between C-cell hyperplasia and microscopic carcinoma (p less than .005). Immunohistochemical staining of histaminase shows positive cells in glands with gross and microscopic carcinoma, but in none of the glands with hyperplasia alone. Histaminase is thus found in high amounts in some malignant C-cells only and may be a useful marker to distinguish between hyperplasia and malignancy in thyroids with early C-cell proliferative disorders.

Amine Oxidase (Copper-Containing)

Distribution of beta-endorphin immunoreactivity in normal human pituitary.

Recent immunohistochemical demonstration of calcitonin in rat pituitary has suggested that calcitonin, in addition to ACTH, endorphins, lipotropins, and melanocyte-stimulating hormones might be derived from a 31,000-dalton glycoprotein percursor molecule. This immunoperoxidase study demonstrates a similar distribution for beta-endorphin and ACTH immunoreactivity in human pituitary; however, the two peptides are not necessarily present in the same cells at all times. Calcitonin could not be demonstrated in human pituitary under conditions suitable for demonstration of the peptide in thyroid C cells. Weakly positive immunostaining could be obtained only with much increase in antiserum concentration and length of incubation, and higher concentrations of calcitonin were needed to abolish staining in preabsorption studies. It thus appears that the immunoreactive calcitonin in human pituitary differs from that in thyroid C cells. Likewise, we could not demonstrate immunoreactive endorphin in any developmental stage of medullary thyroid carcinoma. Our study suggests that caution should be applied in considering a physiologic role for calcitonin in the pituitary and in postulating a common peptide origin for endorphin and calcitonin in humans.

Adrenocorticotropic Hormone

Vasoactive intestinal peptide and its relationship to ganglion cell differentiation in neuroblastic tumors.

Immunohistochemical studies have demonstrated that immunoreactive vasoactive intestinal peptide is present in, and restricted to, the differentiating and mature ganglion cells in a variety of normal and neoplastic neural tissues. In a composite pheochromocytoma-ganglioneuroma (associated with the syndrome of watery diarrhea, hypokalemia, and hypochlorhydria), five ganglioneuroblastomas, five ganglioneuromas (two of which were associated with diarrheal syndromes), an unusual mixed neuroblastoma-ganglioneuroma, and four normal sympathetic ganglia, vasoactive intestinal peptide was present in differentiating and mature ganglion cells. The peptide was also demonstrated in isolated ganglion cells in two pheochromocytomas but was not present in pheochromocytes, Schwann cells, or undifferentiated neuroblastic cells in the neuroblastomas and ganglioneuroblastomas. These studies indicate that the presence and presumably the production of vasoactive intestinal peptide thus reflect a particular line of neuroblastic differentiation and are not merely a reflection of common derivation of these tissues. Our identification of vasoactive intestinal peptide in neurogenic tumors associated with diarrhea supports the contention that the peptide might be an important diarrheogenic factor in these tumors.

Adrenal Gland Neoplasms

Ileal adenomas after colectomy in nine patients with adenomatous polyposis coli/Gardner's syndrome.

Adenomas of the ileal mucosa are an uncommon finding in adenomatous polyposis coli/Gardner's syndrome. We report 9 patients who were found to have ileal adenomas 1 yr 11 mo to 25 yr 11 mo after colectomy. The ileal adenomas occurred proximal to an ileorectal anastomosis in 7 patients, both proximal to a cecoproctostomy and later proximal to an ileostomy in 1 patient, and in an ileostomy in 1 patient. The cases confirm that ileal mucosa is susceptible to adenoma formation in adenomatous polyposis coli/Gardner's syndrome and thus provide additional evidence that adenomas are not confined to the colon in these patients. In addition, the findings in the cases suggest that the ileal adenomas developed after colectomy. Thus, our cases suggest that the small intestine should be studied for the presence of adenomas after colectomy with either ileoproctostomy or ileostomy, as well as before resection. On the other hand, aggressive management does not seem warranted as carcinoma of the jejunum and ileum has been reported only rarely.

Adult

Variable content of histaminase, L-dopa decarboxylase and calcitonin in small-cell carcinoma of the lung. Biologic and clinical implications.

To ascertain whether the content of endocrine markers is constant in small-cell carcinoma of the lung, levels of three markers of medullary thyroid carcinoma were studied in this tumor. Histaminase was increased in six of six primary tumors (three to 14,000 times), L-dopa decarboxylase in four of six (six to 30 times), and calcitonin in one of one (eight times) over levels in adjacent lung. Marker levels in mediastinal metastases reflected those in primary tumors in four of five patients. However, in four of seven, multiple hepatic metastases contained low to absent levels despite simultaneously high values in chest lesions. Immunohistochemical studies of histaminase revealed that within each primary tumor different cells contained different amounts of the enzyme. Since marker content varied between tumor cells, between primary tumors and between metastases in individual patients we conclude that circulating levels of these three markers cannot be expected necessarily to mirror tumor burden in patients with small-cell lung tumors.

APUD Cells

Supravalvular aortic stenosis with parafollicular cell (C-cell) hyperplasia.

Autopsy examination of a patient with well-documented supravalvular aortic stenosis and other characteristic features of the idiopathic infantile hypercalcemia syndrome revealed previously unreported hyperplasia of parafollicular cells (C cells). Immunohistochemical analysis demonstrated up to 30 calcitonin-containing cells per high power field, whereas normal glands contain only 4 to 10 cells per low power field in areas of highest concentration. The parathyroid glands were found to be normal both grossly and microscopically, whereas the bones showed thickened trabeculas, normal Haversian canals and no apparent increase in osteoblast or osteoclast activity, suggesting normal parathyroid hormone, but increased thyrocalcitonin activity. We suggest that C-cell hyperplasia has occurred in response to a persistent, rather than transient, elevation in serum calcium levels and that thyrocalcitonin function is augmented, rather than impaired in this disorder. The primary biochemical defect promoting hypercalcemia remains to be clarified, as well as the role, if any, such a defect plays in producing significant pathology in the central nervous system and in the cardiovascular, renal and skeletal systems.

Adult

Dysgenetic male pseudohermaphroditism.

Dysgenetic male pseudohermaphroditism is a disorder of sexual differentiation in which patients present with bilateral dysgenetic testes, persistent müllerian structures, cryptorchidism and inadequate virilization. There were 10 patients with this syndrome seen at our hospital between 1956 and 1976. In this series, there was a 30 per cent incidence of testicular tumors. Because patients with dysgenetic male pseudohermaphroditism have a high incidence of testicular tumors this disorder, which is actually a variant of mixed gonadal dysgenesis, must be distinguished from the other forms of male pseudohermaphroditism.

Adult

Pheochromocytomas.

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Adrenal Gland Neoplasms

Congenital cystic adenomatoid malformation of the lung.

Ultrastructural study of a congenital cystic adenomatoid malformation of the lung revealed a persistence of type 2 pneumocytes lining the alveolar and gland-like spaces. The larger cystic areas were lined by normal bronchiolar-type epithelium and were surrounded by a few smooth muscle cells. The interstitium contained undifferentiated mesenchymal cells. No cartilage or bronchial glands were present. These features suggest that this lesion is developmental in nature, with a failure of maturation resulting from an insult occurring early in embryonic life, prior to the onset of cartilaginous differentiation within the lungs.

Epithelium

Cardiovascular manifestations of Pseudoxanthoma elasticum.

Autopsies were performed in three cases of pseudoxanthoma elasticum (PXE) to evaluate the cardiovascular changes. The endocardial lesion characterized by intimal fibroelastotic thickening and disorganization, fragmentation, and calcification of elastic fibers in the deeper endocardial layers is unique histologically. Severe atherosclerosis was present in all cases and resembled that encountered routinely. Fragmentation and degeneration of the elastic laminae of muscular arteries was followed by vascular calcification that could not be distinguished morphologically from Mönckeberg's arteriosclerosis. All three cases showed striking initimal fibroelastotic thickening, particularly in intrarenal arteries, resembling that seen in hypertension, although only one of the subjects was hypertensive. It would appear that the metabolic defect in PXE predisposes to the premature onset and accelerated development of commonly encountered vascular aging processes, and that the endocardial lesion is the only specific cardiovascular manifestation of the disease.

Aged