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

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: 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*

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*

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

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*

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

PURPOSE:Urine examination for microalbuminuria during the clinical silent years can help to identify those patients most likely to progress to overt nephropathy. The normalization of the AER(Albumin Excretion Rate) by alteration of glomerular hemodynamics may be the key to preventing or delaying overt nephropathy. The prognostic and therapeutic implications have led to attempts to identify patients with microalbuminuria as early as possible.Because diagnosis of microalbuminuria has required a 12 or 24 hour urine collection,there has been interested in developing tests that might serve as a first-stage screen for microalbuminuria. Most reports have dealt with the usefulness of either the albumin concentration or the albumin/creatinine ratio in a timed or randomly collected urine specimen. Exercise induced albumin excretion is increased compared to resting and could unmask latent glomerular damage. The purpose of this study is to evaluate diagostic significance of urinary albumin measurement after exercise in children with insulin dependent diabetes. METHODS:Fifty-seven patients with IDDM collected their overnight urine from 22:00 to 6:00 in following morning. Exercised method for urine examination was 100M running and collected 2 hour urine after exercise. The AER and Albumin/ creatinine ratio(ACR) were measured in the urine and compared with before and after exercise. RESULTS:Mean ages of diabetic children was 13.7 yrs old and duration of diabetes was 4.1+/-2.7yrs, HbA1c 8.6+/-1.9%,Systolic BP 107+/-10mmHg and Diastolic BP 67+/-8mmHg. The exercise induced AER was increased compared to before exercise(10.3+/-10.5 vs 18.1+/-16.0 mg/24hr). The exercise induced ACR also increased too(1.4+/-1.5 mg/g.Cr vs 4.1+/-3.6mg/g.Cr)(P<0.01). Two(3.5%) children with IDDM was positive for microalbuminuria in terms of AER before exercise and 8 children(14%) after exercise. No one positive for microalbuminuria in terms of Alb/cr before and after exercise. The charateristics of 8 children with microalbuminuria after exercise was no specific difference between positive and negative microalbuminuria. There was specific correlation between microalbuminuria and HbA1c(P<0.05) but sex, ages, duration of diabetes & blood pressure were not correlated with incidence of microalbuminuria. CONCLUSION: The mean timed urinary albumin excretion after exercise was significantly higher than before exercise. Measurement of timed urinary albumin excretion after exercise will be helpful for detecting microalbuminuria earlier.
Blood Pressure ; Child* ; Creatinine ; Diabetes Complications ; Diabetes Mellitus, Type 1 ; Diabetic Nephropathies ; Diagnosis ; Hemodynamics ; Humans ; Incidence ; Insulin ; Running

Pulmonary blastoma is a rare type of malignant lung tumor comprised of epithelial and mesenchymal elements reminiscent of fetal lung. Mesenchymal element may show various patterns of differentiation toward mature tissue, such as cartilage, smooth muscle, and bone. Rhabdomyoblastic differentiation in pulmonary blastoma is quite rare. Our case of pulmonary blastoma with rhabdomyoblastic differentiation documented by electron microscopy and immunohistochemistry. Usually it has been treated with surgery, but both chemotherapy and radiotherapy have been used against the metastatic disease, and as the adjuvant setting. A 28/12-year-old girl is described who was presented with pulmonary blastoma. She underwent surgical excision of the tumor, followed by the T2protocol, a 8 cycles of combination chemotherapy consisting of actinomycin-D, adriamycin, vincristine and cyclophosphamide with the radiation therapy to the left chest cage(180cGyx11 times). The patient has been off therapy without any evidence of relapse for 24 months. We report a case of pulmonary blastoma with brief review of related literatures.
Cartilage ; Cyclophosphamide ; Doxorubicin ; Drug Therapy ; Drug Therapy, Combination ; Female ; Humans ; Immunohistochemistry ; Lung ; Microscopy, Electron ; Muscle, Smooth ; Pulmonary Blastoma* ; Radiotherapy ; Recurrence ; Thorax ; Vincristine