Pheochromocytoma is originated from chromaffin cell of sympathetic nervous system and associated with other disease, such as neurofibromatosis, duodenal carcinoid, medullary thyroid cancer and parathyroid adenoma. Especially, pheochromocytoma is developed more than 50% in neurofibromatosis associated with hypertension. In such cases, several clinical features documented as more frequent bilateral phochromocytoma, more associated with other neuroendocrine tumors and thus more poor prognosis. We can observe the sustained hypertension despite of surgical resection of tumors in pheochromocytoma cases. One of the possible reason of post operative sustained hypertension is the pheochromocytoma originated from minor organ of Zukerkandl that was not resected during operation. Untreated or delayed treated cases with pheochromocytoma were often expired by complication of hypertension such as cerebrovascular hemorrhage, myocardial infarction, etc. Thus, in neurofibromatosis with hypertension, screening of pheochromocytorna is very important for the early detection of tumor and more favorable prognosis. Recently, We experienced a case of neurofibromatosis associated with bilateral pheochromocy-toma expired by cerebral hemorrhage during operation, so we report the case with literature review.
Carcinoid Tumor ; Cerebral Hemorrhage ; Chromaffin Cells ; Hemorrhage ; Hypertension ; Mass Screening ; Myocardial Infarction ; Neuroendocrine Tumors ; Neurofibromatoses* ; Parathyroid Neoplasms ; Pheochromocytoma* ; Prognosis ; Sympathetic Nervous System ; Thyroid Neoplasms

BACKGROUND: Trisomy 16 in mice is considered to be an animal model for Down's syndrome in human. We studied the morphologic characteristics of the heart and the edema, and their significance to the teratogenesis in this animal model. METHODS: A total of 30 dams were sacrificed to bear 125 (61.9%) normal fetuses 35 (17.3%) abnormal fetuses and 42 (20.8%) resorptions. Cytogenetic study and morphological examination were performed using microdissection agar-mount sectioning histologic examination and scanning electron microscope. RESULTS: The crown-rump length was significantly shorter in abnormal (trisomic fetuses) than the normal (eusomic) fetuses. Trisomic fetuses showed massive edema at the back from the vertex to the lumbar area. Four-chamber view section of the agar-mount and histologic section showed a common atrioventricular valve bridging the left and the right atrioventricular junction. Scanning electron microscopic examination on atrioventricular valves showed three types of atrioventricular valves:five cases with common atrioventricular orifice, three cases with partitioned atrioventricular orifice and two cases with atresia of the right atrioventricular orifice. CONCLUSION: This study confirmed the presence of massive edema and cardiac anomalies in the mouse with trisomy 16. But there was morphologic diversity of cardiac anomaly in this model.
Animals ; Crown-Rump Length ; Cytogenetics ; Down Syndrome ; Edema* ; Endocardial Cushion Defects ; Fetus* ; Heart ; Humans ; Hydrops Fetalis ; Mice ; Microdissection ; Models, Animal ; Pathology* ; Teratogenesis ; Trisomy*

Cellular schwaninoma is a variant of schwannoma, which is characterized by predominance of cellular Antoni A area, presence of mitotic activity, nuclear hyperchromasia, pleomorphism, and absence of Verocay body. These pathologic features often prompted a misdiagnosis of malignancy. However, the clinical outcome has indicated the benignity of the tumor. We have experienced a case of cellular schwannoma arising from right facial nerve with right hemifacial weakness and erosion of mastoid process. Grossly, it was a 3.5 x 3 cm sized and relatively well encapsulated mass with yellowish, friable cut surface. Microscopically, cellular growth with moderate cellular pleomorphism and some mitotic activity (5/40 HPFS, up to 2/HPF) were noted. Immunostaining for S-100 protein showed diffuse strong positive reaction.
Diagnostic Errors ; Facial Nerve* ; Mastoid ; Neurilemmoma* ; S100 Proteins

BACKGROUND: Cancer registries are fundamental for cancer control and multicenter collaborative research. However, there have been discrepancies among pathologists in classifying cancer and assigning the codes according to the International Classification of Disease Oncology 3 (ICD-O3). To improve the quality of cancer registries as well as to prevent the conflict with medical insurance compensation, a guideline for the coding of cancer is mandatory. METHODS AND RESULTS: Funded by the Management Center for Health Promotion, 40 members of the Gastrointestinal Pathology Study Group and the Cancer Registration Committee of the Korean Society of Pathologists participated in the 1st workshop for gastrointestinal tumor registration. The subjects of gastric epithelial tumor, intramucosal carcinoma of the colon, carcinoid tumor, gastrointestinal stromal tumor and appendiceal mucinous tumor were discussed to create a guideline. A survey to obtain consensus for the guideline proposed by the workshop was carried out by the members of the Korean Society of Pathologists and 240 members completed the questionnaire. CONCLUSION: Although there are some issues to be discussed further, such as coding of high grade dysplasia/adenoma and intramucosal carcinoma of stomach and colon, the members agreed upon most parts of the proposed guideline. Therefore, we suggest using the ICD-O3 coding guideline for gastrointestinal tumor.
Adenoma

Double primary cancer is defined as the case of primary malignant tumors, which must be arise in different sites and have a different histologic appearences. The number of reported cases of double primary malignant tumors has increased in recent and the occurrence of multiple primary cancers in the same individuals is one of the interesting subjects for investigation and research in relation to the carcinogenic mechanism and/or genetic factors. We have experienced a case with double primary malignant tumors of different site origins such as stomach adenocarcinoma and rectal adenocarcinoma, which were diagnosed by biopsies with gastro and colono fiberscopy. For its great rarity, we report this case with review of literatures.
Adenocarcinoma ; Biopsy ; Rectum* ; Stomach*

Intestinal tuberculosis has been known since antiquity. After about 1950's, effective antituberculous chemotherapy and an improved standard of living resulted in a steady decline in all forms of tuberculosis. However, intestinal tuberculosis has been reported with impressive frequency in developing countries including Korea. Disease affecting the duodenum is reported to be rare and isolated duodenal tuberculosis sparing the rest of the intestine is uncommon. We experienced a case of tuberculosis of duodenal bulb associated pulmonary tuberculosis. The diagnosis was made on the basis of the histological study of the endoscopic biopsy specimen. We have confirmed the healing of the duodenal lesion by the follow-up endoscopy after 9 months treatment of antituberculous medication.
Biopsy ; Developing Countries ; Diagnosis ; Drug Therapy ; Duodenum* ; Endoscopy ; Follow-Up Studies ; Intestines ; Korea ; Socioeconomic Factors ; Tuberculosis* ; Tuberculosis, Pulmonary*

Double primary cancer means that more than two cancers with different origin exist independently in an individual. The diagnosis of double primary cancer was determined by following criteria. Each of the tumors must present a definite picture of malignancy, and each must be distinct, and the probability of one being a metastasis of the other must be excluded. Small cell carcinoma has a distinct biological behavior such as, early invasion and metastasis, a rapid clinical course, and significant sensitivity to chemotherapy. Small cell carcinoma in the esophagus is relatively rare, and rarer when it is combined with other malignant disease. We have experienced a case of double primary cancer, a 70-year-old man with esophageal small cell carcinoma and gastric adenocarcinoma, which were diagnosed by gastrointestinal endoscopic biopsy. A review of the Korean medical literature failed to reveal any previously described case of esophageal small cell carcinoma with gastric adenocareinoma. We report this case with review of literatures.
Adenocarcinoma* ; Aged ; Biopsy ; Carcinoma, Small Cell* ; Diagnosis ; Drug Therapy ; Esophagus ; Humans ; Neoplasm Metastasis

PURPOSE: Isolated noncompaction of the ventricular myocardium(INVM) can present as heart failure or arrhythmias in a child. It is a rare disorder, characterized by prominent trabecular meshwork and deep intertrabecular recesses. We still know little about the diagnosis, symptoms, and clinical outcomes of INVM. METHODS: We included in our study 6 patients who showed ventricular noncompaction on echocardiography. Patients were diagnosed as INVM were excessively prominent trabeculations with deep intertrabecular recesses were found on echocardiography. Patients who had other complex heart lesions such as pulmonary atresia with intact ventricular septum in addition to ventricular noncompaction, were excluded. RESULTS: Age at presentation ranged from 1 day 7 years, with follow up being as long as 6 years. Symptoms at initial presentation were heart murmur, paroxysmal supraventricular tachycardia, cyanosis, feeding intolerance, ventricular tachycardia, and cardiomegaly at fetal screening. Prominent trabeculations and intertrabecular recesses were observed at left ventricular apex in all six patients. All patients were alive at last follow-up. One patient showed WPW syndrome on electrocardiography. Echocardiography revealed decreased systolic function in 4 patients, and decreased systolic and diastolic function in 1 patient. One patient is currently asymptomatic. CONCLUSION: Six patients were diagnosed with INVM with various symptoms at initial presentation. Echocardiography is the most important tool in the diagnosis of INVM due to its morphological characteristics. INVM can rarely be the cause of long term systolic dysfunction, and early detection by echocardiographic screening may be beneficial.
Arrhythmias, Cardiac ; Cardiomegaly ; Child ; Cyanosis ; Diagnosis ; Echocardiography ; Electrocardiography ; Follow-Up Studies ; Heart ; Heart Failure ; Heart Murmurs ; Humans ; Isolated Noncompaction of the Ventricular Myocardium* ; Mass Screening ; Pulmonary Atresia ; Tachycardia, Supraventricular ; Tachycardia, Ventricular ; Trabecular Meshwork ; Ventricular Septum ; Wolff-Parkinson-White Syndrome

Malignant transformation develops in a little less than 2% of mature cystic teratomas. A wide variety of malignant tumors may arise within benign mature cystic teratomas, and the most common of these is squamous cell carcinoma, which account for 75~85%. In general, the tumors are in an advanced stage and the prognosis is poor as most patients die within a year. However, when the tumor is confined to the ovary, they have a good prognosis and the 5-year survival rate is 63~83%. We experienced three cases of squamous cell carcinoma arising in mature cystic teratoma. Two of the carcinomas occurred in postmenopausal women: 58-(case 1) and 66-(case 2) year-old, and were confined to the ovaries. They were alive 37 months and 18 months after the operation, respectively. The third case was a 45-year-old premenopausal woman who had an extraovarian extension of the tumor and early recurrence within two months. Histologically, cases 1 and 3 were conventional well to moderately differentiated squamous cell carcinomas and case 2 showed a well-differentiated squamous cell carcinoma with exuberant proliferating trichilemmal tumor-like areas.
Carcinoma, Squamous Cell* ; Female ; Humans ; Middle Aged ; Ovary* ; Prognosis ; Recurrence ; Survival Rate ; Teratoma*

The adrenocartical carcinoma is a rare tumor with an estimated incidence of 1 case per 1,700,000 population. Despite its rarity, a large number of investigators have studied this neoplasm for the following two reasons. The First is the occasional difficulty of differentiation between careinoma and adenoma at the time of initial surgery, even by histopathologic examination. The other is its unique feature of corticosteroidogenesis. Steroid metabolism of adrenocortieal carcinoma is characterized by its low efficiency of steroid production due to deficiency of steroidogenic enzyme. The deficieney of 11 B-hydroxylase has been indieated in case of adrenoeortical carcinoma by several investigators. In this study, the serum level of cortisol was within normal range, but the serum level of 11-deoxycortisol was 50 times higher than normal. After the removal of tumor, the serum level of ll-deoxycortisol was markedly decm. In conclusion, the results from the this case suggest that measurement of serum 11-deoxy- cortisol may be a useful tool in the diagnosis and follow-up of adrenocortical carcinoma.
Adenoma ; Adrenocortical Carcinoma* ; Cortodoxone ; Diagnosis ; Follow-Up Studies ; Humans ; Hydrocortisone ; Incidence ; Metabolism ; Reference Values ; Research Personnel