Neurofibromatosis type 1 (NF1) is an autosomal dominant disorder that occurs once in 3,000 live births. Patients with NF1 are prone to develop malignancies, particularly neural tumors, that is, malignant schwannoma and glioma in adults. For non-neural tumors, the NF1 incidence is increased in rhabdomyosarcomas, leukemia, malignant melanomas, breast cancer, lung cancer, thyroid cancer, and other organ cancers. The NF1 gene seems to increase the risk for carcinogenesis. A case of NF1 associated with metachronous cancers, such as breast cancer (1991) and small-bowel leiomyosarcoma (1997), in the same patient is reported. Investigation of the patient's family revealed four further cases (offsprings) of multiple neurofibromatosis. The relevant literature on the subject is reviewed.
Adult ; Breast Neoplasms ; Carcinogenesis ; Genes, Neurofibromatosis 1 ; Glioma ; Humans ; Incidence ; Leiomyosarcoma ; Leukemia ; Live Birth ; Lung Neoplasms ; Melanoma ; Neurilemmoma ; Neurofibromatoses ; Neurofibromatosis 1* ; Rhabdomyosarcoma ; Thyroid Neoplasms

The purpose of this study was to set the guidelines for selection of patients to do early surgery in ruptured intracranial aneurysm. We assessed 706 patients with single rupture and without large hematoma, who underwent aneurysm surgery from 1985 to 1995. The male and female ratio was 1:1.5. Among the 706 patients, early surgery was performed in 214 cases. The results of early surgery were good in 193 cases(90.2%), fair in 13 cases(6.0%), poor in 1 case(0.5%) and dead in 7 cases(3.3%). The rate of dead outcome in the early surgery group was higher compared to other timing groups. The Fisher group 1, 2 and 3 reveale good outcome in early surgery group;92.6%, 96.3%, 88.8% respectively. The incidence of delayed ischemic deficits(DID) of early surgery group was same as other groups. However, in Fisher group 3, the incidence of DID was significantly low, 32.5%, in early surgery group. It is suggested that the criteria of selection of early surgery in patients with ruptured intracranial aneurysm would include as follows:1) patients with good clinical grade, 2) poor grade patients with marked irritability, acute hydrocephalus, and poorly controlled hypertension, 3) none-complex aneurysm requiring less brain retraction, dissection and brief temporary clipping, 4) age under 60 or over 60 with good physical status, and 5) Fisher group 3 requiring cisternal larvage and anticipated triple-H therapy.
Aneurysm ; Brain ; Female ; Hematoma ; Humans ; Hydrocephalus ; Hypertension ; Incidence ; Intracranial Aneurysm* ; Male ; Patient Selection* ; Rupture

Parotid sialolithiasis accounts for 10 to 20% of all cases of sialolithiasis that involves the head and neck and presents management challenges for a variety of reasons. In this report, we present a rare case of removing a double stone in Stensen's duct by anexternal approach (open parotid sialolithotomy).
Head ; Neck ; Salivary Duct Calculi ; Salivary Ducts ; Salivary Gland Calculi

PURPOSE: Revascularization is a useful method for treating Kienbock's disease. The aim of this study was to evaluate the use of the second dorsal metacarpal vascular pedicle to treat Kienbock's disease. MATERIALS AND METHODS: A retrospective study was carried out on 9 patients who had undergone vascular pedicle graft for Kienbock's disease between 1999 and 2003. The mean follow up period was 35 months. At the time of surgery, 2 patients were graded as stage II, 6 as IIIa and 1 as IIIb. The ulnar variance was neutral in all cases. A vascular pedicle graft using second dorsal metacarpal vessel were performed and temporary scaphotrapeziotrapezoid (STT) fixation were applied for 6 months. RESULTS: Wrist motion except for the radial deviation and grip strength had improved significantly. The patients returned to work after an average of 22 weeks. Resorption of the sclerosis was observed in 7 cases, but lunate collapse was observed in the simple radiograph in 6 cases. According to Lichtman's criteria, 7 cases were satisfactory but 2 cases with a relative old age were unsatisfactory. According to Nakamura's classification, 5 cases were good, 3 cases were fair and 1 case was poor. CONCLUSION: In Kienbock's disease with neutral ulnar variance, a revascularization procedure of vascular pedicle graft with temporary STT fixation is a good treatment method but the patient's age should be considered when determining the revascularization procedure. A prospective study will be needed to determine the optimal duration of temporary STT fixation.
Classification ; Follow-Up Studies ; Hand Strength ; Humans ; Osteonecrosis* ; Retrospective Studies ; Sclerosis ; Transplants* ; Wrist

It was sometimes difficult to differentiate between acute-onset chronic inflammatory demyelinating polyneuropathy (A-CIDP) and subacute inflammatory demyelinating polyneuropathy (SIDP). The CNS involvement of these polyneuropathies has rarely reported in the literature. We present the case of a 42-year-old man who developed rapidly developing inflammatory demyelinating polyneuropathy followed by right optic neuritis. This case showed progressive motor weakness and sensory dysfunction with time to nadir at 8 weeks, demyelination in nerve conduction study, no other etiology of neuropathy, no relapse during follow-up of 18 months, good response to steroid and complete recovery which favor SIDP more than A-CIDP. We experienced the case of SIDP associated with optic neuritis.
Demyelinating Diseases ; Follow-Up Studies ; Neural Conduction ; Optic Neuritis ; Polyneuropathies ; Recurrence

BACKGROUND AND OBJECTIVES: This study aimed to gather three-dimensional data to detect fish bones as an esophageal foreign body model and to assess the possibility that this method could be applied for cases of real fish bone foreign body (FBFB). MATERIALS AND METHOD: Fish bones from two species were collected and sizes were measured. Pork meat loaf was placed flat into a plastic box containing plaster cast and bones were laid over the meat layer. Another layer of meat was placed on the bones, then another layer of plaster cast, and the final layer of meat were placed. The meat-bone sandwich was regarded as a fish bone foreign body model of esophagus. The model was imaged using computed tomography (CT) scan and the CT data were reconstructed three-dimensionally making multi-planar reconstruction, maximal intensity projection, and volume rendering images. We tried to find tools to detect the shape and lying position of the FBFB model. The above tools were applied to 3 FBFB cases to verify effectiveness of the tools. RESULTS: Multi-planar reconstruction, maximal intensity projection, and volume rendering images were reconstructed easily. After single or more processing, all of the bones could be detected. The shape and lying positions could be detected using tools made from FBFB models. CONCLUSION: Reconstructed images of CT data readily enabled the detection of fish bone in the esophageal model and real cases.
Casts, Surgical ; Deception ; Esophagus ; Foreign Bodies* ; Meat ; Plastics

PURPOSE: We wanted to determine the incidence of HGC (hereditary gastric cancer) in Korean under the minimal criteria of ICG-HGC (International Collaborative Group on Hereditary Gastric Cancer). MATERIALS AND METHODS: Tumor registry abstracts of 1752 patients who underwent operations for gastric cancer during the time period 1996 to 1998 in the Department of Surgery at Seoul National University College of Medicine were examined. Based on their family histories, candidate HGCs were identified. Their detailed family histories including diagnosis of cancer, age at diagnosis, and dates of birth and death were obtained from interviews by phone. Another study was performed on 195 patients with gastric cancer who admitted for operations in the same department during the time period April, 1999 to June, 1999. Their detailed family histories were also obtained from probands or nearest relatives during admission. Pedigree studies of documented families were conducted. Minimal Criteria of ICG-HGC we used for present study are as followings: At least three relatives with histologically verified gastric cancer; one of them should be a first-degree relative to the other two. At least two successive generations should be affected. In one of the relatives, gastric cancer should be diagnosed under 45 years of age. Suspected HGC fullfills only two of the above three criteria. HNPCC, FAP and Li-Fraumeni syndromes should be excluded. RESULTS: A total of 12 HGCs were identified in this study. In recent 3 years, during the time period 1996 to 1998, the incidence of true and suspected HGC accounted for 6 (0.3%) and 44 probands (2.5%) among 1752 patients (in 1996, 0.4% and 3.2%; in 1997, 0.3% and 1.8%; in 1998, 0.3% and 2.8%) respectively. In contrast, during the time period April, 1999 to June, 1999, the incidence of true and suspected HGC increased up to 3.1% (6 probands) and 11.3% (22 probands), respectively, out of 195 patients (in April, 1999, 0% and 11.7%; in May, 1999, 4% and 14.7%; in June, 1999, 5% and 6.7%). There was no difference in terms of the incidence even if the third criterion of age at diagnosis among Minimal Criteria of ICG-HGC was modified from 'under 45 years of age' to 50. Mean ages of 12 probands (46.3 8.8) were statistically younger than those of control gastric cancer patients (54.2 11.5) retrieved from database of Department of Surgery at Seoul National University College of Medicine. CONCLUSION: In the present study, the incidences of HGC were remarkably altered in accordance with study methods. Retrospective reviews of medical records revealed to be ineffective for this kind of study since their informations were often incomplete and some suspected patients were lost during follow-up. According to the Minimal Criteria of ICG-HGC, the incidence of true and suspected HGC was 3.1% (6 probands) and 11.3% (22 probands), respectively, out of 195 gastric cancer patients. We propose that families who meet the Minimal Criteria of ICG-HGC should be prospectively found in order to determine the exact incidence of HGC in Korean.
Diagnosis ; Family Characteristics ; Follow-Up Studies ; Humans ; Incidence* ; Korea ; Medical Records ; Parturition ; Pedigree ; Retrospective Studies ; Seoul ; Stomach Neoplasms*

BACKGROUND: Bulla is an air-filled space within the lung parenchyma resulting from deterioration of the alveolar tissue. Molecular mechanism of the formation of the bulla is not well described. Fibroblast growth factor(FGF)-7, bone morphogenetic protein(BMP) receptor, and transforming growth factor(TGF)-beta receptor are known to have a stimulatory or inhibitory role in the lung formation. We investigated to see if these growth factor or cytokine receptors are involved in the bulla formation by immunohistochemical staining of bullous lung tissues from patients with primary spontaneous pneumothorax. MATERIAL AND METHOD: Bullous lung tissues were obtained from 31 patients with primary spontaneous pneumothorax, including 30 males and 1 female from 15 to 39 years old. The bullous tissues were obtained by video-thoracoscopic surgery and/or mini-thoracotomy and fixed in formalin. Blocks of the specimens were embedded with paraffin and cut into 5~6 micrometer thick slices. The sections were deparaffinized and hydrated and then incubated with primary antibodies against FGF-7, BMP-RII, or TGF-RII. RESULT: Of the 31 patients, 24 were TGF-RII positive including 18 strong and 6 weak positives. Observation with high magnification showed that strong immunostaining was detected in the boundary region between bullous and normal lung tissues. In contrast, all of the sections were negative with FGF-7 or BMP-RII antibodies. CONCLUSION: These results suggest that overexpression of TGF-beta RII may be involved in the formation of bulla, although further molecular studies are needed to find out more detailed molecular mechanisms.
Adult ; Antibodies ; Female ; Fibroblasts ; Formaldehyde ; Humans ; Lung ; Male ; Paraffin ; Pneumothorax* ; Receptors, Cytokine ; Transforming Growth Factor beta

PURPOSE: Adenomatous polyps in the stomach are rather uncommon, however are related to a malignant transformation. So, in such cases it is important to predict cancer risk and to select an appropriate surgical method. The aim of this study is to examine clinicopathologic factor for predicting malignancy and determine appropriate surgical management of gastric adenomatous polyps. METHODS: A total 41 patients who underwent surgery from 1996 to 1999 for gastric adenomatous polyps at the Department of Surgery, Seoul National University Hospital were included this study. All patients had undergone preoperative endoscopic biopsy and histology was confirmed. Authors analyzed the clinicopathologic factors associated with malignancy and the outcome of surgical treatment. RESULTS: Of the 41 patients, 17 patients had an adenomatous polyp alone, 24 patients had an adenomatous polyp associated with malignancy. The location, number, size and morphology of the polyp were not associated with malignancy. Only cellular atypism in the preoperative biopsy was significantly associated with malignancy. 31 patients underwent subtotal gastrectomy whereas 10 patients wedge resection. All lymph nodes resected in patients with adenocarcinoma were negative. Recurrence of polyp or adenocarcinoma had not occurred in any patients after follow-up of mean 22 months. CONCLUSION: Cellular atypism detected in preoperative histology was associated with malignancy in gastric adenomatous polyp. Lymph node metastasis was negative in patients with malignancy. Our findings support the wedge resection withsafe margin as being appropriate in surgical management of gastric adenomatous polyps.
Adenocarcinoma ; Adenomatous Polyps* ; Biopsy ; Follow-Up Studies ; Gastrectomy ; Humans ; Lymph Nodes ; Neoplasm Metastasis ; Polyps ; Recurrence ; Seoul ; Stomach

Purpose: The vascular clipping system (VCS) is beneficial as it is simple and easy to apply for microvascular suturing. Arteriovenous fistula (AVF) creation is a very basic standard technique of microvascular surgery. In this study the VCS and the conventional suture methods were compared in a rabbit model using the carotid artery and vein to create an AVF. Methods: There were 28 rabbits assigned equally into 2 groups using the AVF creation method (conventional suturing or the VCS procedure). Histopathology was performed on fixed samples. The procedure time of the 2 methods and changes in histopathology of tissue samples after surgery were compared. Results: The VCS procedure showed a lower degree of fibrosis and hyperplasia histologically compared with the conventional suture method. The VCS was quicker to perform and no significant anastomosis stricture was observed. Conclusion In a rabbit model of AVF, the VCS has benefits over the conventional suture method. The VCS provides comparable patency rates, produces fewer side effects such as fibrosis and hyperplasia, and takes less operation time than suturing. The VCS is expected to be useful for cases where renal patients need periodic hemodialysis and thus repetitive access to a vessel.