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Biomedical subjects

R C Hickey

Publications and source records attributed to R C Hickey.

At least 37 records · Page 2Linked to original sources

Multiple endocrine syndrome type I. Clinical, laboratory findings, and management in five families.

The clinical features of 20 patients from five families with multiple endocrine neoplasia syndrome type I (MEN-I) were studied. Nineteen patients (95%) had hyperparathyroidism. Five patients who had a diagnosis during surgery of adenoma and who had fewer than 3.5 glands removed had recurrence of hypercalcemia after surgery. Fourteen patients (70%) had pancreatic islet cell tumors. All had one or more elevated serum polypeptide hormones, and six had symptoms related to the hormones produced. Multiple pancreatic tumors were identified in the nine patients who underwent surgery. Three patients who died had a mean survival of 6.3 +/- 2.9 years. Eight patients had pituitary tumors; seven had macroadenomas. Of the eight patients with pituitary tumors, seven had high serum prolactin and responded to bromocriptine therapy, whereas the eighth patient had acromegaly treated with radiotherapy. It was concluded that hypercalcemia due to hyperparathyroidism in MEN-I syndrome patients should be managed by a resection of four glands and transplantation of one half gland into the forearm because none of the patients has shown evidence of a recurrence, and serum calcium levels have been normal. Pancreatic tumors, which are usually multiple, may be asymptomatic. Patients with these tumors usually have long survival rates, even with distant metastasis. Total pancreatectomy may be the method of choice, especially in patients with gastrinoma caused by the diffuse nature of the disease. Long-term follow-up is needed, however, with more patients. Pituitary tumors are primarily prolactin-producing tumors, and medical treatment is the method of choice.

Adenoma, Islet Cell↗

Adrenal cortical carcinoma.

The records of 110 patients (58 females and 52 males) with histologically proven adrenal cortical carcinoma were studied. Fifty-six of 110 (50.9%) patients had abdominal symptoms and 33 (30.0%) had a palpable abdominal mass. Surgery for localized and regional disease was associated with a disease-free survival time of at least 2 years in 28 of the 50 (56%) patients. Abdominal radiotherapy, systemic chemotherapy, and 1,1 dichloro-2 (O-chlorophenyl)-2 (P-chlorophenyl) ethane (OPDDD) were effective in three of 19 (15.8%) patients, three of 31 (9.7%) patients, and 21 of 72 (29.2%) patients, respectively. Combined radiotherapy and OPDDD did not confer any additional benefit over OPDDD alone. Six of seven patients who received OPDDD as adjuvant treatment are still alive at 1 to 4 years. Distant metastases occurred most commonly in the lungs, liver, peritoneum, lymph nodes, and bones. Only 23% of the patients survived 5 years or more. We conclude that early diagnosis and complete surgical excision offer the best prospects for long-term survival and that the efficacy of adjuvant OPDDD needs to be evaluated further.

Adolescent↗

Current results of treatment for distant metastatic well-differentiated thyroid carcinoma.

Between 1960 and 1986, 37 patients were treated at M. D. Anderson Hospital and Tumor Institute, Houston, Tex, for metastases beyond the thyrocervical region arising from well-differentiated thyroid carcinomas. Patient, tumor, and treatment factors were analyzed in this group for their influence on survival. No specific factors achieved statistical significance; however, patients having more than one site of metastasis and patients whose metastases arose less than 5 years after treatment of the primary tumor tended to have shorter overall survival times. More than one modality of treatment was used in the majority of patients. Complete surgical resection of metastases offered the best chance for prolonged survival and palliation. With long survival times being the rule, several treatment modalities are often required to provide continuous palliation.

Adenocarcinoma↗

Necrotizing gas-forming infections in cancer patients.

Necrotizing gas-forming infections in cancer patients present some unique characteristics, such as nontraumatic, spontaneous clostridial gangrene and gangrene involving an ischemic tumor mass. These infections can be rapidly progressive and uniformly fatal without surgical debridement. We review ten cases of gas gangrene seen during an 18-year period. Four were caused by Clostridium species and six by other organisms. Neutropenia was present in seven patients. During the last nine years, Clostridium septicemia occurred in 54 patients; in only two of those patients did gas gangrene ensue.

Adolescent↗

Diagnosis, management, and pathogenetic studies in medullary thyroid carcinoma syndrome.

A retrospective study of 224 patients with medullary thyroid carcinoma (MTC) diagnosed between 1963 and 1988 was performed to 1) establish the diagnosis of MTC in early childhood, 2) establish the role of prophylactic regional lymphadenectomy in patients with MTC, 3) study the effect of chemotherapy on MTC patients with metastatic disease, 4) study the effect of somatostatin analog 201-995 (Sandoz Pharmaceuticals) on the frequency of diarrhea in MTC, and 5) locate the common region(s) of gene deletion on chromosome 1 and examine the loss of heterozygosity on chromosome 10 in tumors. Our data indicated that a progressive rise of serum calcitonin in early childhood (rather than the expected fall with age seen in normal subjects) is diagnostic of MTC. No differences in clinical course of prognosis were observed between patients with MTC localized to the thyroid who had prophylactic neck node dissection and those who did not. Conventional chemotherapy had no significant benefit in the treatment of patients with metastatic disease. The somatostatin analog was found to be an effective drug in the treatment of diarrhea associated with MTC. Allelic losses were frequently found in MTCs and pheochromocytomas, and the loss of DNA sequences in these tumors appeared to involve the distal third of the short arm of chromosome 1, with a common breakpoint at 1p32.

Adrenal Gland Neoplasms↗

Medullary thyroid carcinoma: prognosis of familial versus nonfamilial disease and the role of radiotherapy.

A retrospective study of 202 patients with medullary thyroid carcinoma (MTC) diagnosed between 1943 and 1987 was done to compare the prognosis of patients with sporadic disease and those with the familial form of multiple endocrine neoplasia type II (MEN-II) and to study the effect of radiotherapy. The relationship between serum calcitonin (iCT), carcinoembryonic antigen (CEA), and calcitonin gene-related peptide (CGRP) to the extent of disease and prognosis was also studied. Patients with MEN-II had significantly longer survival rates than did patients with the sporadic variety (P less than 0.005), but most patients with sporadic disease were older and had more advanced disease. No differences in survival rates were found when patients from these two groups were matched for age and extent of disease (P greater than 0.7). When patients who received radiotherapy were matched for age, extent of disease, and surgery with patients who had had no radiotherapy, the latter group was found to live significantly longer (P less than 0.05). Our studies suggested (1) that the apparently poor prognosis of patients with sporadic MTC may be related to the patients' older age at detection rather than to inherent differences in the two forms of disease, (2) radiotherapy has little effect on MTC, (3) calcitonin gene-related peptide measurement was not helpful in determining the extent of disease or prognosis, (4) patients with liver metastasis had the highest iCT levels, (5) patients with bone metastasis had the highest CEA levels, and (6) patients with diarrhea had poor prognosis and had high iCT levels.

Adolescent↗

Diagnosis, localization, and management of pheochromocytoma. Pitfalls and follow-up in 41 patients.

Forty-one patients who had pheochromocytoma are described. These patients represent the experience of the authors over the last 19 years. The diagnoses, investigations, treatments, and pitfalls of this study and the management of these patients are described. The most sensitive screening test was the urinary measurement of catecholamines, vanillylmandelic acid (VMA), and metanephrines. The most useful localizing procedure was the metaiodobenzylguanidine (MIBG) scintigraphy and computed tomography (CT). After careful alpha and beta adrenergic blockade in 32 patients, no complications occurred during or after tumor resection. When this procedure was ignored even in patients who had normal blood pressure before surgery, severe cardiovascular complications occurred and two patients died. In familial pheochromocytoma, bilateral adrenalectomy with preservation of normal adrenal cortical tissue when possible may be the method of choice, but careful follow-up is warranted.

Adolescent↗

Alveolar soft part sarcoma. A cytologic and immunohistochemical study.

Alveolar soft part sarcoma is an uncommon soft tissue tumor that has seldom been studied by cytologic methods. The cytomorphologic features of two cases of this sarcoma are described. To enhance diagnostic accuracy in a suspected alveolar soft part sarcoma, the authors present the differential diagnosis and the application of immunocytochemical procedures to cytologic specimens.

Adult↗

Hürthle cell tumors of the thyroid. A flow cytometric DNA analysis.

Twenty-five Hürthle cell tumors of the thyroid gland, histopathologically classified into three groups--adenomas, carcinomas, and indeterminant--have been studied by DNA flow cytometry using archived, paraffin-embedded tissues. Tumor ploidy characteristics were correlated with patient follow-up and survival with the conclusions that (1) nuclear DNA ploidy alone does not distinguish benign from malignant Hürthle cell tumors; (2) diploid DNA Hürthle cell carcinomas behave far less aggressively than aneuploid Hürthle cell carcinomas; and (3) all patients with aneuploid carcinomas died of their disease or are alive with persistent carcinoma.

Adenoma↗

Comparison of HMB-45 monoclonal antibody and S-100 protein in the immunohistochemical diagnosis of melanoma.

The value of HMB-45 mouse monoclonal antibody in the immunohistochemical diagnosis of melanoma was compared with that of two antibodies to S-100 protein. Tissue from 32 (91.4%) of the 35 melanomas studied reacted with HMB-45, however, none of the 98 nonmelanoma tumors stained with this antibody. In contrast, 31 (88.5%) melanomas and 24 (24.4%) of the nonmelanoma neoplasms expressed S-100 protein. These results indicated that HMB-45 monoclonal antibody has a higher specificity for melanoma cells than did S-100 protein and that, because of its reactivity in routinely processed tissues, it may be helpful in surgical pathology.

Antibodies, Monoclonal↗

Medullary thyroid carcinoma: prognosis of familial versus sporadic disease and the role of radiotherapy.

A retrospective study of 202 patients with medullary thyroid carcinoma (MTC) diagnosed between 1943 and 1987 was done to compare the prognosis of patients with sporadic disease and those with the familial form of multiple endocrine neoplasia type II and to study the effect of radiotherapy. Patients with multiple endocrine neoplasia type II had a significantly higher survival rate than did patients with the sporadic variety (P less than 0.005), but most patients with sporadic tumors were older and had more advanced disease. No differences in survival rates were found when patients from these 2 groups were matched for age and involvement of the thyroid gland only (P greater than 0.3), involvement of the thyroid gland plus cervical nodes (P greater than 0.3), and involvement of the thyroid gland, cervical nodes, and soft tissue (P greater than 0.7). When patients with MTC who received radiotherapy were matched for age, extent of disease, and surgery with patients who had had no radiotherapy, the latter group was found to live significantly longer (P less than 0.05). We conclude that 1) the apparently poor prognosis of patients with the sporadic variety of MTC may be related to the patients' older age at detection rather than to inherent differences in the two forms of disease, and 2) radiotherapy has little effect on MTC.

Adult↗

Genetic instability in fibroblasts of patients with thyroid cancers (TC).

In cultured fibroblasts initiated from fresh tumor biopsies of 11 patients with thyroid tumors, 5 medullary carcinoma of the thyroid (MCT) and 6 papillary follicular, the chromosomes are relatively unstable when compared with those from control fibroblast cultures. Tumor-derived fibroblast samples showed a mean number of cells in metaphase with chromosome abnormalities at a frequency of 9.1% and a range of 6-13%, whereas 5 controls exhibited a mean frequency of 4% and a range of 3-5%. Our results indicate that genetic instability in thyroid cancer patients is present not only in peripheral blood cultures, as reported earlier, but in their fibroblast cultures as well.

Cells, Cultured↗

A comparison of thyroid carcinoma in those who have and have not had head and neck irradiation in childhood.

Among 1324 patients with well differentiated (papillary, follicular, Hurthle cell) thyroid carcinoma treated at the University of Texas M. D. Anderson Hospital and Tumor Institute between January 1950 and July 1984, 125 had a history of external irradiation to the head and neck region during childhood. This study was a comparison of the characteristics and disease course of thyroid carcinoma in these patients with those of patients who had not received such irradiation. Each irradiated patient was matched to 3 nonirradiated patients by age, sex, and extent of disease. The groups had similar distributions of histological type of lesion, surgical procedures, and number of patients who received radioactive iodine as part of their initial treatment. The two groups' recurrence and mortality rates were similar as well, although patients who had head and neck irradiation as children more often presented with thyroid cancer not limited to the thyroid gland and bilateral thyroid lobe involvement (P less than 0.005). The data indicate that patients who received head and neck irradiation have, at the time of diagnosis, more extensive thyroid carcinoma. However, the prognosis of well differentiated thyroid carcinoma in patients who have a history of head and neck irradiation is similar to that in patients without such a history.

Adult↗

Medullary carcinoma of the thyroid: differentiating the types and current management.

Medullary Ca of the thyroid (MCT) occurs in sporadic and familial form. When familial MCT is associated with pheochromocytoma and parathyroid adenoma or hyperplasia it is called multiple endocrine syndrome type IIa (MEN type IIa). When the syndrome is associated with mucosal neuromata or marfanoid habitus it is called MEN type IIb or type III. When familial MCT is not associated with the above tumors, it is called type IV. Measurement of serum calcitonin is the diagnostic method of choice. CEA is a better prognostic marker than calcitonin. When MCT is confined to the thyroid, total thyroidectomy is recommended. If lymph nodes are involved, lymph node neck dissection is required. I131 therapy and radiotherapy are ineffective. Optimal chemotherapy has not yet been established.

Biopsy, Needle↗

Pheochromocytoma.

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Adrenal Gland Neoplasms↗