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: 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: 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

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

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

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: Primary aldosteronism due to an adrenal cortical adenoma is a surgically curable disease. However, hypertension is known to persist postoperatively in many patients. The aim of this study was to determine the factors influencing the long-term outcome of blood pressure after an adrenalectomy for a primary aldosteronism and to evaluate the changing pattern of renin and aldosterone. METHODS: Forty-two cases of primary aldosteronism, which were operated on and followed up at the Department of Surgery, Seoul National University Hospital from January 1986 to June 2001 were included in this study. The subjects were classified into a normotensive group and a hypertensive group and the two groups were compared according to the clinical, biochemical and pathological parameters. RESULTS: After surgery, the aldosterone concentration was decreased and the plasma renin activity was increased. During a mean follow-up period of 28 months, 31 patients (73.8%) had a normal blood pressure without an antihypertensive treatment. The significant risk factors for persistent hypertension were a family history of hypertension, a long duration of preoperative hypertension, a poor response of preoperative spironolactone. The hypertensive group had a higher level of postoperative plasma renin activity and an aldosterone concentration in the long-term follow-up period after surgery. CONCLUSION: A family history of hypertension, the duration of hypertension and the response to spironolactone were factors influencing persistent hypertension after surgery for a primary aldosteronism. A high level of plasma renin activity and aldosterone during the follow-up period is related to the persistent hypertension. Therefore, early detection and surgery for a primary aldosteronism would reduce the preoperative cardiovascular changes and improve the postoperative outcome.
Adrenalectomy ; Adrenocortical Adenoma ; Aldosterone ; Blood Pressure ; Follow-Up Studies ; Humans ; Hyperaldosteronism* ; Hypertension ; Plasma ; Renin ; Risk Factors ; Seoul ; Spironolactone

PURPOSE: To determine the clinical feature, surgical management of primary and recurrent disease, predictive factors for outcome, and impact of multimodality therapy in retroperitoneal sarcoma. MATERIALS AND METHODS: 60 patients were confirmed pathologically as soft-tissue sarcoma of the retroperitoneum by operation or needle biopsy in Seoul National University Hospital from 1983 to 1995. A retrospective analysis was performed. RESULTS: The abdominal mass was common presenting symptom. Histologically liposarcomas(25%) and leiomyosarcomas(23.3%) were most common, and MFHs(11.7%) and malignant schwannomas(11.7%) followed. The overall 5 year survival rate was 54.6%. Complete resection was possible in 51.7% of patients and strongly predicts outcome (<0.0001). These patients had a median survival of 130 months compared to 20 months for those undergoing partial resection and 9 months for those with unresectable tumors. 11(35%) of completely resected patients have had local recurrence. These patients underwent reoperation when feasible. Complete resection of recurrent disease was performed in 10 patients(90%), with a 42 months median survival time after reoperation. Resection of adjacent organ was performed in 19 patients. 14 of these were completely resected, and showed 100% of 5 year survival rate. Tumor grade was not a significant predictor of outcome. Gender, histologic type, encapsulation, stage, resectability, combined resection were significant prognostic factors by univariate analysis. But resectability was only independent prognostic factor on mutivariate analysis. Radiation therapy and chemotherapy could not be shown to have significant impact on survival. CONCLUSION: Complete resection is the most important prognostic factor of retroperitoneal sarcoma. Extensive and aggressive surgery must be considered including resection of adjacent organs. Multiple resection seems to improve survival in recunent cases.
Biopsy, Needle ; Drug Therapy ; Humans ; Recurrence ; Reoperation ; Retrospective Studies ; Sarcoma* ; Seoul ; Survival Rate

Substernal extension of a goiter into the thoracic inlet endows a generally benign neck mass with morbid potential. The reported incidence varies between 1% and 15% of all thyroidectomies performed. Whether all patients with a substernal goiter should undergo an operation or whether the operation should be performed selectively remains controversial. From May 1989 to March 1996, 10 patients underwent thyroidectomies for substernal goiters, and those cases of resected substernal goiters have been reviewed to access the symptoms and signs that brought patients to surgery: the size and the position of goiter, the preoperative work-up, the risk associated with the operation, and the histopathologic state of the goiter. There were 3 male and 7 female patients, and their ages ranged from 40 to 68 years. The chief complaints were cervical mass (4), dyspnea (2), facial edema (1) and chest pain (1). No symtomatic cases (2) were also found. The average mass size was 8.4 cm (5-14 cm) and the average weight was 109 gm (41-350 gm). Although chest film was the most used, computed tomography was by far the most useful study. Thyroid scans often failed to show the substernal goiter. Fine-needle aspiration was not helpful because of inaccessibility. In the majority of the patients (7 cases), the substernal goiters were removed by a cervical incision. Three cases of goiters located deep to the carina level required a combined cervical and sternotomy approach or thoracotomy. In the cervical incision group, the complications were transient hypocalcemia (1) and unilateral recurrent laryngeal nerve injury (1). In the combined cervical and sternotomy approach or thoracotomy, unilateral recurrent laryngeal nerve injury (1) and bilateral recurrent laryngeal nerve injury (1) and mediastinitis (1) were the complications. An occult papillary carcinoma, which was not identified preoperatively, was found in one case. Removal was almost always accomplished via cervical incision and with low morbidity and no deaths. Also, the substernal goiters revealed unusual symptoms and signs, such as dyspnea, facial edema and chest pain, compared to usual thyoid goiters and were relatively bigger in size. In conclusion, most substernal goiter above the carinal level could be removed by cervical incision with a low rate of complication. The threat of compression, the substantial chance of malignancy, and the safety of resection mean that the presence of a substernal goiter is an indication for surgery.
Bays ; Biopsy, Fine-Needle ; Carcinoma, Papillary ; Chest Pain ; Dyspnea ; Edema ; Female ; Goiter ; Goiter, Substernal* ; Humans ; Hypocalcemia ; Incidence ; Male ; Mediastinitis ; Neck ; Recurrent Laryngeal Nerve Injuries ; Sternotomy ; Thoracotomy ; Thorax ; Thyroid Gland ; Thyroidectomy