BACKGROUND: Endocrine pancreas tumor is a rare disease which incidence is less than 2% of all pancreatic tumors. But it comprises various types of tumor and usually secretes several hormones from one type of tumor although the patient with this tumor complains of sole symptom associated with only one hormone. The mechanism and clinical significance of multiple hormone secretion in the endocrine pancreas tumom are not yet clearly defined. METHODS: We analyzed retrospectively the clinicopathologic features of 20 cases which were operated at Seoul National University Hospital during the period between February 1989 and May 1998. RESULTS: The most common tumor was insulinoma (13 cases) and the second most common tumor was nonfunctioning tumor (6 cases). There was one case of somatostatinoma. Most of the patients with insulinoma complained of neuroglycopenic symptoms. There were 9 cases (45.0%) in which the tumors secreted more than two kinds of hormones, 7 cases in insulinoma, 2 cases in nonfunctioning tumors. Whether the tumor secreted multiple hormones was detected by the method of immunohistochemical staining. Though the tumors secreted more than two kinds of hormones, the patients with the tumors complained of symptoms which were associated with the cell type most strongly stained by immunohistochemical method. Whether or not the tumors secreted multiple hormones was not associated with the pathologic features such as tumor size, histologic patterns of the tumor, status of tumor cell differentiation and malignancy. CONCLUSION: From this results, we suggest that endocrine tumors of the pancreas secreted multiple hormones not by the mechanism of dedifferentiation from already differentiated endocrine cells but by the mechanism of neogenesis of multipotent islet stem cells. Since the relationship between the function of multiple hormone secretion in the endocrine pancreas tumors and islet stem cell would be significant, further study should be needed to find out the function of stem cells and application of stem cells to clinical use.
Cell Differentiation ; Endocrine Cells ; Humans ; Incidence ; Insulinoma ; Islets of Langerhans ; Pancreas* ; Rare Diseases ; Retrospective Studies ; Seoul ; Somatostatinoma ; Stem Cells

BACKGROUND: The activation of phospholipase C(PLC) is one of the early cellular events in various growth process, including malignant transformation. PLC-gamma1 is activated through direct interaction with growth factor receptor tyrosine kinase. MATERIAL AND METHODS: Using immunoblot assay, we evaluated overexpression of PLC-gamma1 expression in twenty human breast cancer tissues. It was also determined whether there was any connection between other prognostic factors(numbers of metastatic axillary nodes, nuclear and histological grade, c-erbB2, p53 and E-cadherin) and the overexpression of PLC-gamma1 protein. RESULTS: Seventeen of 20 breast cancer tissues showed overexpression of PLC-gamma1, which was corresponded to that seen on the immunohistochemistry( kappa= 0.8275, p = 0.003). Of 3 tumor markers, immunohistochemically determined, positive expression of E-cadherin only was associated with PLC-gamma1 protein overexpression in a range of statistical significance (p=0.045, kappa=0.607). CONCLUSION: PLC-gamma1 overexpression might be pathogenic trigger involved in breast cancer and the relationship between expression of E-cadherin and PLC-gamma1 would require further elucidation.
Breast Neoplasms ; Cadherins ; Humans ; Phospholipases* ; Protein-Tyrosine Kinases ; Biomarkers, Tumor

A 38-year old female was admitted to our hospital for further evaluation of an incidentally found cardiac murmur. She had been in a hypertensive state for 5 years but had taken antihypertensive drugs intermittently on her own. Her history revealed that she had become amenorrheic for 7 months and had, had headaches for a few months. Physical examination revealed central obesity and a moon face, but no hirsuitism. When she first visited our hospital, her blood pressure was 260/170 mmHg and grade 2 systolic murmur was audible along the left sternal border. Laboratory studies revealed high levels of 24-hour urine-free cortisol and plasma aldosterone, but a very low level of plasma adrenocorticotropic hormone and plasma renin activity. A low-dose and a high-dose dexamethasone suppression tests, adrenal venous sampling, inferior petrosal sinus sampling, and a renin stimulation test were performed. Bilateral adrenal masses were found on computerized tomographic scanning and magnetic resonance imaging but there was no abnormality of the pituitary gland. The uncontrollable blood pressure and the elevated 24-hour urine-free cortisol and plasma aldosterone levels were corrected by a right-total and left-subtotal adrenalectomy. Pathologic findings were bilateral adrenal cortical adenomas of different cell types. These findings indicate that the adrenal cortical adenomas were tumors that functioned differently, causing Cushing's syndrome and primary aldosteronism in the same patient. A review of, the literature published in English showed that this is the first reported case, of bilateral adrenal adenomas functioning differently.
Adenoma ; Adrenalectomy ; Adrenocortical Adenoma* ; Adrenocorticotropic Hormone ; Adult ; Aldosterone ; Antihypertensive Agents ; Blood Pressure ; Cushing Syndrome* ; Dexamethasone ; Female ; Headache ; Heart Murmurs ; Humans ; Hydrocortisone ; Hyperaldosteronism* ; Magnetic Resonance Imaging ; Obesity, Abdominal ; Petrosal Sinus Sampling ; Physical Examination ; Pituitary Gland ; Plasma ; Renin ; Systolic Murmurs

PURPOSE: Infiltrating lobular carcinoma of the breast was known to have a high incidence of multicentricity and bilaterality in patients. We analyzed the clinical features of infiltrating lobular breast cancer. MATERIALS AND METHODS: We studied 29 patients with infiltrating lobular carcinoma of the breast, from Jan. 1980 to Mar. 1997 in the Department of Surgery, Seoul National University Hospital. RESULTS: The age of the patients ranged from 32 to 71 years with an average of 45.2 years. The main complaining symptom was a painless mass. The diameter of the tumor ranged from 0.7 to 7 cm with a mean size of 2.8 cm. The axillary lymph node was positive for malignancy in 12 cases. The number of multicentric breast cancers were 7 cases (24.1%), and the number of bilateral breast cancers were 3 cases (10.3%). The estrogen receptor was positive in 66.7%, and the progesterone receptor was positive in 75% of the cases. There were one local recurrence and three distant metastases during a mean follow-up period of 4.5 years. The 5-year survival rate and 5-year disease free survival rate were 89.6% and 88.6%, respectively. CONCLUSION: Infiltrating lobular breast cancers are at greater risk of developing multicentricity and bilaterality than nonlobular breast cancers. Careful program of frequent follow-up examinations and thorough histopathological studies are needed for patients with infiltrating lobular breast cancers.
Breast Neoplasms* ; Breast* ; Carcinoma, Lobular ; Disease-Free Survival ; Estrogens ; Follow-Up Studies ; Humans ; Incidence ; Lymph Nodes ; Neoplasm Metastasis ; Receptors, Progesterone ; Recurrence ; Seoul ; Survival Rate

PURPOSE: There are no specific clinical and histopathologic characteristics of malignant pheochromocytoma and the optimal treatment modality has not been established yet. We analyzed the clinical and histopathologic features of malignant pheochromocytoma and treatment results. MATERIALS AND METHODS: We reviewed the clinical records of 10 patients with malignant pheochromocytoma diagnosed at Seoul National University Hospital from March 1987 to June 1998. RESULTS: Nine of 10 (90%) patients had functional tumors. The biochemical laboratory findings showed elevated 24-hour urine VMA level in nine patients available. The median size of the tumors was 11x11 cm. Six of 10 (60%) patients were initially diagnosed as malignant tumors because of direct invasions to adjacent tissues or distant metastases. On the other hand, remaining 4 patients were initially diagnosed as benign, but the distant metastases developed metachronously after resection of the primary lesion. The median duration between the initial operation and the detection of metastases was 57 months (range: 47~72 months) in these patients. The liver was the most common site of metastases (60%). With regards to the histopathological features, most of the tumors (87.5%) showed capsulation, necrosis and hemorrhage. The findings of lymphatic invasion, angio-invasion, and mitosis were found in 62.5% of the cases. All but 2 patients were initially treated with radical operation for the primary lesions. The disease recurrences or metastases occurred in 7 out of 10 patients. Of these, 4 patients were treated with chemotherapy or interferon- a after recurrences. Overall, the median survival for all patients was 82 months (range: 37~143 months). Two patients is alive and only one patient is alive without recurrence. CONCLUSION: The careful follow-up for at least 5 years and the aggressive multi-disciplinary therapy may be needed for the diagnosis and the management of malignant pheochromocytoma.
Diagnosis ; Drug Therapy ; Follow-Up Studies ; Hand ; Hemorrhage ; Humans ; Liver ; Mitosis ; Necrosis ; Neoplasm Metastasis ; Pheochromocytoma* ; Recurrence ; Seoul

PURPOSE: H rthle cell neoplasm of the thyroid gland is a rare disease. The reported cases in Korea are not so many; thus, there has been a lack of common therapeutic and prognostic guidance. METHODS: The cases of 15 patients with a H rthle cell neoplasm of the thyroid gland, hospitalized at Seoul National University Hospital from Nov. 1981 to Feb. 1998, were retrospectively reviewed. RESULTS: There were 4 males and 11 females. The ages varied from 23 to 69 years old (mean: 46.3 years old). Among them, 13 had neck masses with a mean size of 3.3 cm (1.2-6.0 cm), and the mean duration of the symptom was 4 years (5 months-8 years). Of the other two, one had laryngeal discomfort for 2 months, and the other one was diagnosed through a fine needle aspiration follow-up. One of the 15 patients had hyperthyroidism whereas the others had euthyroidism. The thyroid scans for 10 of them showed cold nodules. The operations consisted of a unilateral lobectomy in 2 cases, a unilateral lobectomy and isth mectomy in 11 cases, a unilateral lobectomy, isthmectomy and contralateral partial lobectomy in 1 case, and a total thyroidectomy and neck lymph node dissection in the 1 case that was combined with a papillary carcinoma. Thirteen patients (86.7%) had a H rthle cell adenoma, and two of them (13.3%) had a H rthle cell carcinoma. The mean duration of follow-up was 33 months (range 1 month-12 years), and no recurrence or death occurred during the follow-up period. CONCLUSIONS: The incidence of H rthle cell neoplasms is higher in women and the sex ratio is 1:2.8. The incidence of H rthle cell carcinomas among H rthle cell neoplasms is 13.3% (2/15). We found no specific clinical features for differentiating a H rthle cell adenoma from a H rthle cell carcinoma and no factors for adequate operative methods and prognosis due to the rarity of the condition and the short duration of the follow-up. Because of the low incidence of H rthle cell neoplasms and our limited experience, not only co-research of centers but also pathologic research is needed.
Adenoma ; Aged ; Biopsy, Fine-Needle ; Carcinoma, Papillary ; Female ; Follow-Up Studies ; Humans ; Hyperthyroidism ; Incidence ; Korea ; Lymph Node Excision ; Male ; Neck ; Prognosis ; Rare Diseases ; Recurrence ; Retrospective Studies ; Seoul ; Sex Ratio ; Thyroid Gland* ; Thyroidectomy

BACKGROUND: A papillary carcinoma in the breast is a rare carninoma with a predominantly papillary growth patttern. Its incidence is said to be 1% to 2% of all breast carcinomas in women. Because of its rarity, little is known about its histopathological features. This study was undertake to analyze the histopathological characteristics of papillary breast cancer. METHODS: During the 17 years from January 1980 through December 1996, among 2166 primary breast cancer patients, 26 patients, who had been diagnosed with papillary breast cancer pathologically at the Department of Surgery, Seoul National University Hospital, were elegible for this study. The histopathological characteristics of the tumors in these patients, including immunohistochemically stained tumor marker-e.g., bcl2, c-erbB2, cathepsin D, and p53-were compared to those of patients with an invasive ductal carcinoma, NOS (IDC). RESULTS: The mean age of the patients was 47 years old, ranging from 28 to 74 years. There was no statistically significant difference in the primary tumor size between the papillary cancer and the IDC (2.62 cm vs 3.33 cm, p=0.127). Twenty-four of the papillary cancer patients had no metastatic axillary node, and metastatic nodes were present in the papillary cancer than in the IDC, with statistical significance (0.27+/-0.19 vs 3.43+/-0.08, p=0.023). Papillary cancer seemed to have more frequent ER and PgR (60%, 77.8%) than IDC (44.1%, 56.1%), but there was no significance to these results (p=0.351, 0.309). In terms of tumor markers, the positivity of bcl2, c-erbB2, cathepsin D, and p53 in papillary cancer were 42.9%, 100%, 85.5%, and 66.7%, respectively. Of all significance, immunohistochemically determined tumor markers, c-erbB2 expression was observed with statistical significance, more frequently in papillary cancer (100% vs 68.5%, p=0.044). Patients with papillary cancer had an overall 10-year survival rate of 83.3%. CONCLUSION:Papillary breast cancer had fewer metastatic axillary node, but with little statistical significance for a more favorable outcome. The relation between c-erbB2 expression and papillary cancer seems to be interesting and needs to be further elucidated.
Biomarkers, Tumor ; Breast Neoplasms ; Breast* ; Carcinoma, Ductal ; Carcinoma, Papillary ; Cathepsin D ; Female ; Humans ; Incidence ; Middle Aged ; Seoul ; Survival Rate

PURPOSE: Papillary thyroid microcarcinomas (PTMCs) have the same histological features as papillary thyroid cancer, but they are 1.0 cm or less in diameter. They can metastasize to the regional lymph nodes and distant sites, but its ability to cause significant morbidity and mortality has been questioned. Because of this reason, the extent of thyroid tumor resection remains an issue of controversy. This study is aimed at identifying the statistically significant factors that are associated with recurrence and we also wanted to devise an appropriate surgical treatment plan for PTMC patients. METHODS: The retrospective review (350 cases, 1990.1~2004. 11) was obtained from Seoul National University Hospital (SNUH). The mean age at the time of diagnosis was 46.5± 11.0 (yrs) (range: 12~75). The mean overall length of follow- up was 37.70± 36.03 months (range: 1~169). The PTMCs were treated with total and subtotal thyroidectomy or lobectomy. The invasiveness and lymph node metastasis (LNM) from 350 PTMCs were analyzed according to the size, multiplicity, bilaterality of the tumor and the perithyroidal invasion. Fishers exact test and the exact logistic regression test were used for the stratified analysis. RESULTS: 350 of the 2187 papillary carcinoma were PTMCs. There were 296 females (84.6%) and 54 males (15.4%) in the study. Invasion into the perithyroidal tissue was common (128/336, 38.1%). There were 68 patients with LNM among the 312 total patients (21.7%). The group with either perithyroidal invasion or LNM showed a significantly higher recurrence rate than those group having neither one (4.8% vs. 10.9%, 4.5% vs. 19.1%, respectively). Even for tumor smaller than 1 cm, a larger-sized tumor resulted in a poorer prognosis. CONCLUSION: PTMC is an early stage carcinoma with the capability of tissue invasion, lymph node metastasis and multiplicity. Based on this study, total thyroidectomy is recommended for significant portion of the PTMCs. Furthermore, more careful imaging studies (such as neck ultrasonography or neck CT scan) are needed to detect contralateral lesions or neck lymph node metastasis.
Carcinoma, Papillary ; Diagnosis ; Female ; Humans ; Logistic Models ; Lymph Nodes ; Male ; Mortality ; Neck ; Neoplasm Metastasis ; Prognosis ; Recurrence ; Retrospective Studies ; Seoul ; Thyroid Gland* ; Thyroid Neoplasms ; Thyroidectomy ; Ultrasonography

PURPOSE: Histopathological classification of invasive breast carcinoma with its earliest phases is fraught with pitfalls. We were willing to clarify the biology and clinicopathological features of microinvasive carcinoma which is not fully understood in comparison with those of in situ cancer. Particular attention is paid to identifying the novel markers which can be representative of the microinvasive carcinoma. METHODS: From January 1986 to December 1996, a total of 72 microinvasive carcinomas, defined as in situ carcinomas with invasion present in less than 10% of the histological section, were found out. Their paraffin blocks were chosen for immunohistochemical staining against four molecules. RESULTS: Microinvasive carcinoma was greater in primary tumor size (2.66?0.17cm vs 2.21?0.19cm, P=0.045) and metastatic axillary nodes (0.21?0.25 vs 0.06?0.16, P=0.019) than DCIS. In terms of nuclear grade(P=0.198) and comedo type(P=0.562), there was no statistical significance between microinvasive carcinoma and DCIS. Among three primary tumor features(size, comedo component, and nuclear grade), the tumor size> or =2.5cm had marginal significance affecting the incidence of axillary node metastasis in microinvasive carcinoma(P=0.081). Of investigational prognostic factors, determined by immunohistochemical staining, p53 expression was observed more frequently in microinvasive disease entity from in situ to invasive from than DCIS(P=0.031). CONCLUSION: Microinvasive carcinoma is thought to be transitional disease entity from in situ to invasive form. The microinvasive carcinoma of 2.5cm could be indication for axillary node dissection. In addition, p53 mutation might play a important biological role in progression from noninvasive to invasive form and these results provide further evidence that p53 mutation could have potential use as a molecular marker.
Biology ; Breast Neoplasms ; Carcinoma, Intraductal, Noninfiltrating ; Classification ; Incidence ; Neoplasm Metastasis ; Paraffin

PURPOSE: A medullary carcinoma of the breast(MC) is a well-circumscribes tumor composed of poorly differentiated cells growing in a syncytium with an accompanying stroma. However, the prognosis of a MC is considered as more favorable than that of an infiltrating ductal carcinoma (IDC). In the present study, we characterized MC in terms of prognosis by comparing an MC group with an IDC control group. We described the distribution of other clinicopathological characteristics, as well as the prevalence and the prognostic importance of generally well known risk factors, for breast cancer and compared the result. MATERIALS AND METHODS: Clinical data from hospital records and pathological materials were available from 60 patients with tumors that had been initially diagnosed from Jan. 1981 to Dec. 1999 at the Department of Surgery in Seoul National University Hospital as having a MC. We analyzed the survival and the prognostic factors of those patients and compared the results with those for an IDC control groep. RESULTS: The 60 cases of MC showed more risk factors, such as young age, high nuclear grade, poor histologic grade, negative hormone receptors, p53 overexpression, c-erb-B2 expression, and high proliferative index(ki 67), than the IDC cases did. However, the prognosis of MC was better than that of IDC. Most of the risk factors were of highly significant prognostic importance in the IDC control group. In the MC group, only lymph-node status and young age were significantly important for disease-free survival. CONCLUSION: We found MC to be biologically unique, and patients with MC have a better prognosis than those with IDC. We propose that MC patients with axillary lymph-node metastasis and young age should be considered as a high-risk group for recurrence.
Breast Neoplasms ; Breast* ; Carcinoma, Ductal* ; Carcinoma, Medullary ; Disease-Free Survival ; Giant Cells ; Hospital Records ; Humans ; Neoplasm Metastasis ; Prevalence ; Prognosis ; Recurrence ; Risk Factors ; Seoul