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Biomedical subjects

G Chejfec

Publications and source records attributed to G Chejfec.

At least 73 records · Page 4Linked to original sources
PubMed1984

Adult nesidiodysplasia.

Subtotal pancreatectomy specimens from two adults with hyperinsulinemic hypoglycemia and one adult with watery diarrhea syndrome were investigated. All three specimens were originally diagnosed as "nesidioblastosis"; none had a neoplasm, and all patients were cured of their endocrine dysfunction by the surgical procedure. Tissue samples were studied by light microscopy and light microscopic immunohistochemistry for serotonin, ACTH, bombesin, calcitonin, gastrin, glucagon, insulin, HPP, somatostatin, and VIP. The results were compared with those obtained from the parallel study of ten adult pancreata obtained at autopsy from patients without pancreatic disease or endocrine dysfunction. The total endocrine cell mass was not notably greater in the pancreata from patients with endocrine dysfunction than in the controls. Both groups had an estimated 95% of the endocrine cell population organized in islets while the remaining 5% was irregularly dispersed amidst the exocrine ducts and acini. Findings more conspicuous in patients with endocrine dysfunction but not absent in the controls included: large islets, islets with irregular contours and ragged edges, and large individual islet cells with abundant cytoplasm and bizarre nuclei. Immunoreactivity for insulin, glucagon, and somatostatin was demonstrated in all cases; the ratio among these hormones in the patients with endocrine dysfunction and in the controls was similar. In the patients with hyperinsulinemic hypoglycemia, step sections sequentially stained for insulin and somatostatin showed a close topographic relationship between these cell types. In the patient with the watery diarrhea syndrome, VIP immunoreactive cells were easily identified admixed with exocrine components; they were only rarely seen within islets. We suggest that the hyperinsulinemic hypoglycemia in these adults was not due to simple quantitative abnormalities in total endocrine cell mass nor to its maldistribution nor to lack of topographic proximity between insulin and somatostatin cells. We speculate that the syndrome may be based on still unknown derangements of insulin secretion, release, and/or its degradation. In the case of the watery diarrhea syndrome, the readily identifiable VIP immunoreactive cells represent the emergence of a functional expression regarded as ectopic for the endocrine cells of the pancreas. We conclude that nesidioblastosis per se cannot be viewed as the structural basis of these endocrine dysfunctions since many of its features were present in control pancreata lacking any such association.(ABSTRACT TRUNCATED AT 400 WORDS)

Adult↗
PubMed1984

Parathyroid carcinoma: biochemical and pathologic response to DTIC.

Parathyroid carcinoma is a rare cause of hyperparathyroidism. Cure results from successful en bloc resection. However, because of its rarity, the malignant nature may not be appreciated at the initial operative procedure and as a result, definitive resection may not be accomplished. However, even with extensive en bloc resections, local recurrences do occur and patients die of metabolic derangements associated with hypercalcemia. Thus in addition to operative intervention, palliative chemotherapy may be required to control the hypercalcemia. Radiotherapy has been unsuccessful. A single case of nonfunctioning parathyroid carcinoma responding to treatment with methotrexate, Adriamycin, cyclophosphamide, and CCNU has been reported. We report a case of recurrent functioning parathyroid carcinoma treated with dacarbazine (DTIC) in which biochemical and pathologic evidence of at least a partial response was seen. The patient, a 33-year-old woman, had undergone five previous neck explorations during a 26-month period for aggressive locally recurrent disease. Before DTIC therapy the intact parathyroid hormone (PTH) level was 1032 pg Eq/ml (normal 163 to 347 pg Eq/ml) and the serum calcium level was 16.8 mg/dl (normal 8.8 to 10.0 mg/dl). After a course of DTIC there was a marked improvement in her clinical status and biochemical parameters (intact PTH 545 pg Eq/ml; serum calcium 11.8 mg/dl). For 2 months her condition stabilized, with PTH levels between 700 and 760 pg Eq/ml and serum calcium levels between 10.2 and 16.0 mg/dl. With a slowly progressive rise in biochemical parameters a second course of DTIC was initiated and a marked drop in serum calcium levels (5.7 mg/dl) occurred, but PTH levels remained unchanged. A progressive course of septicemia, malnutrition, and disseminated intravascular clotting ultimately lead to her death 4 weeks later. At autopsy examination the tumor was confined to the neck. Grossly and microscopically there was extensive central as well as peripheral necrosis of the tumor, which was thought to be the result of the cytotoxic effect of DTIC. From this experience and because of the grim prognosis in patients with recurring parathyroid carcinoma, it may be that aggressive use of chemotherapy with DTIC early in the course of treatment should be considered.

Adult↗
PubMed1983

Linitis plastica carcinoma of the esophagus.

Two patients, 60 and 62 years old, were admitted with progressive dysphagia and weight loss of 8 to 12 weeks duration. Radiographic studies revealed an area of stenosis in the lower third of the esophagus in both cases. Endoscopic and biopsy findings established the diagnosis of a neoplasm, with malignant signet ring cells infiltrating the mucosa. Partial esophagogastrectomy in both cases disclosed an infiltrative adenocarcinoma of the lower esophagus, without involvement of the gastroesophageal junction. One of the patients had metastases to regional lymph nodes; the other patient has no clinical evidence of recurrence 14 months after surgery. Endoscopic, radiologic, and pathologic findings are consistent with the diagnosis of linitis plastica of the esophagus.

Adenocarcinoma↗
PubMed1983

A serotonin and beta HCG-producing islet cell carcinoma associated with focal nodular hyperplasia of the liver.

A 48-year-old woman is described with a pancreatic islet cell carcinoma that had no clinical evidence of hormone production. This neoplasm was removed by radical distal pancreatectomy. Immunohistochemical and extraction studies performed on the tumor tissue revealed that it was producing serotonin and beta HCG. Circulating levels of these hormones were elevated in preoperative serum samples that had been frozen. Beta HCG, a known tumor marker, has remained normal following operation, and this mirrors her clinical course since she has no evidence of disease 1.5 yr after operation. This patient also had focal nodular hyperplasia of the liver. It is possible that the trophoblastic hormone, beta HCG, which was being produced in excess by the islet tumor, may have directly or indirectly caused or facilitated the development of focal nodular hyperplasia.

Adenoma, Islet Cell↗
PubMed1983

Neuropathy accompanying IgM lambda monoclonal gammopathy.

A set of observations made on a patient with IgM lambda monoclonal gammopathy and neuropathy implicate humoral immunity in the pathogenesis of the neuropathy. A sural nerve biopsy from the patient showed a characteristic increase in the width of the intraperiod lines. Deposits of mu-heavy chains and lambda-light chains were found in myelin sheaths of the nerve biopsy. Immunohistochemically, it was demonstrated that mu-heavy chains and lambda-light chains from the patient's serum bound to myelin sheaths of normal peripheral nerves and to a lesser extent to myelin sheaths in the central nervous system (CNS). By immunoblots it was demonstrated that mu-heavy chains and lambda-light chains from the patient's serum bound to myelin associated glycoprotein but to no other antigens from the peripheral and central nervous systems. gamma and alpha heavy chains and chi light chains from the patient's serum were also shown to bind to myelin-associated glycoprotein but not as distinctly as the mu and lambda chains. It is postulated that the monoclonal gammopathy may have arisen on the background of polyclonal autoimmune attack directed against myelin-associated glycoprotein.

Biopsy↗
PubMed1983

"Myeloma lung"--a previously unreported complication of multiple myeloma.

A 52-year-old man with known lambda light-chain multiple myeloma developed chronic renal failure, which was proven by renal biopsy to be the result of "myeloma kidney." Terminally, disseminated pulmonary infiltrates developed. Postmortem examination showed the infiltrates to consist of neoplastic plasma cells with crystalline casts, strikingly similar to those found in the kidneys. The term "myeloma lung" is proposed to describe this unusual and heretofore unreported complication of multiple myeloma.

Crystallization↗
PubMed1983

von Hippel angiomatosis. A light, electron microscopic, and immunoperoxidase characterization.

A 25-year-old Iranian man had undergone eye wall resection of a large von Hippel angioma to alleviate an exaggerated macular response, affording study by light and electron microscopy and immunohistochemistry before the obfuscatory effects of long-standing exudative retinal detachment, gliosis, or iatrogenic ablation supervened. We used this vantage point to assess the interrelation between the component endothelial cells, pericytes, and stromal foam cells. On the basis of staining with glial fibrillary acidic protein, factor VIII, the C3 fraction of complement, fibrinogen, and lysozyme, it is unlikely that stromal foam cells derive from glial precursors, but may represent degenerating cells, perhaps arising from a common vasoformative stem cell under hypoxic stress.

Adult↗
PubMed1983

"Nonfunctioning" islet cell carcinoma of the pancreas.

Although most pancreatic islet cell tumors are associated with clinically evident hormone hypersecretion, a small group have no obvious signs or symptoms of excess endocrine activity and are termed "nonfunctioning." The clinical course of eight patients with "nonfunctioning" islet cell carcinoma seen during an eight-year period was reviewed. The six men and two women ranged in age from 36 to 68 years (mean--52). The initial complaint in six was a palpable abdominal mass associated with pain, steatorrhea, or jaundice. Two patients presented with abdominal pain that was initially thought to be of biliary tract origin, and the tumor was discovered at operation. Two patients underwent radical distal pancreatectomy and have no gross evidence of residual or recurrent tumor one and two years later. Five had a biopsy and biliary diversion; three of these also had a gastrojejunostomy. Five were given postoperative 5-fluorouracil and streptozotocin chemotherapy. One developed renal dysfunction and was switched to dimethyltriazenoimidazole carboxamide (DTIC) chemotherapy. Three patients are alive four, six, and eight years, respectively, after diagnosis. Two expired two and a half and three years after diagnosis. One patient had only biopsy of peripancreatic nodes, and he expired in one year. "Nonfunctioning" islet cell carcinoma presents with symptoms related to the mass effects of the tumor. An aggressive therapeutic approach utilizing surgery and chemotherapy is advocated for these slow growing neoplasms.

Adenoma, Islet Cell↗
PubMed1983

Effects of radiation on parafollicular C cells of the thyroid gland.

While radiation has well-recognized effects on follicular cells of the thyroid gland, those on parafollicular C cells are not yet established. Low-dose radiation that has been proved to be nonablative and carcinogenic to follicular cells was administered to 8-week-old Long-Evans rats to study the changes in C cell number and function. Circulating calcitonin levels were significantly reduced in animals that had undergone radiation at age 24 months. Mean calcitonin values were 0.66 (+/- 0.20) ng/ml and 1.64 (+/- 0.59) ng/ml for control males and females compared with 0.14 (+/- 0.06) ng/ml and 0.11 (+/- 0.01) ng/ml for males (P less than 0.05) and females (P less than 0.001) that had undergone radiation, respectively. These levels correlated well with C cell population density in thyroid glands in the control group and in the group that had undergone radiation as evidenced by light microscopy. Routine hematoxylin and eosin staining showed C cell hyperplasia in 77% of control animals of both sexes compared with 4% in animals that had undergone radiation (P less than 0.005). Immunoperoxidase staining with an anticalcitonin antibody showed virtual absence of C cells in most animals that had undergone radiation compared with diffusely scattered cells in animals in the control group. Medullary carcinomas occurred in 14% of animals in the control group compared with 3% of animals that had undergone radiation (P less than 0.05). These data indicate that the radiation dosage that is carcinogenic to the follicular epithelium causes lethal injury to C cells and thus suggest that C cells are more sensitive to radiation than are follicular cells. This increased sensitivity could explain the virtual absence of C cells, decrease in calcitonin levels, and reduced numbers of medullary carcinomas in the animals that had undergone radiation.

Animals↗
PubMed1980

Neuroendocrinoma of the jejunum: electron microscopic and biochemical analysis.

A 56-year-old woman presented with a sudden, severe hemorrhage per rectum. Angiography localized a jejunal tumor, which was excised. Light microscopy suggested a neuroendocrine tumor, but neither a smooth muscle tumor nor a lymphoma could be excluded. Electron microscopy showed dense cored, single membrane bound secretory granules 150--220 nm in diameter; myofilaments were not observed. Biochemical analysis of tumor tissue yielded considerable amounts of catecholamines. VMA, 5-HIAA, and metanephrines. These combined ultrastructural and biochemical observations establish the diagnosis of neuroendocrine tumor; however, in this case neither type of information is sufficiently specific to define the tumor as either a paraganglioma or a carcinoid. Although paraganglia and mucosal endocrine cells in the GI are currently thought to constitute distinct cell types, they share numerous structural and functional properties, and they are both thought to be part of the APUD cell system. These parallels and similarities are shared by the neoplasms derived from them which often display features of both. In the absence of specific granule types or specific substances isolated from tumor tissue, only the application of specific immunocytochemistry techniques may allow the precise "functional" classification of such tumors.

APUD Cells↗