A case of Juvenile Granulosa Cell Tumor (JGCT) of the ovary with unusual pleomorphic histologic and malignant biologic behaviour is described. The tumor occurred in a 10-year-old girl and was associated with clinical features of isosexual pseudoprecosity and a marked elevation of serum estradiol. The mass manifested initially in the right ovary and subsequently involved the contralateral ovary. A multi-organ metastasis developed during a 6-month-interval despite chemotherapy. She received two operations at 6-month interval, and tissues were obtained from the tumor mass. A marked histologic difference was observed between these two samples. The second biopsy showed profound cellular pleomorphism with numerous multinucleated tumor giant cell formation and hyaline bodies. The differential diagnosis from germ cell tumor and the possible factors for the pleomorphism are discussed.
Diagnosis, Differential ; Biopsy ; Neoplasm Metastasis

An autopsy case of holoacardius hemisomus acephalus is reported. She weighed 2,190 gm and the height was 38 cm. The head and upper extremities were absent, while the vertebrae and lower extremities were relatively well developed, but severely edematous. The heart, lungs, stomach, liver, spleen, and pancreas were missing, but the lower abdominal organs including kidneys, adrenal, urinary bladder, and genital organs were present. The intestine was blind-ended at jejunal level but opened into a normal anus. The umbilical cord had two arteries and one vein.

BACKGROUND: Sevoflurane has been reported to potentiate the effect of rocuronium. We compared the effect of sevoflurane and propofol on intubating conditions and their corresponding hemodynamic changes, and also compared the intubation conditions at different intubation timing using the rocuronium. METHODS: Sixty patients were randomized into four groups: group P (P-60, P-90) received 10 mg/kg/hr of propofol after 2 mg/kg of bolus injection; group S (S-60, S-90) were inhaled with 8 vol% sevoflurane after injection of 50 mg of thiopental sodium. Intubation was attempted at either 60 seconds (P-60, S-60) or 90 seconds (P-90, S-90) after the rocuronium administration. Intubation conditions were assessed and evaluated as excellent, good, poor and inadequate. Single twitch responses were measured at every 30 seconds after rocuronium administration. Blood pressure and heart rate were measured before induction, before intubation and immediately after intubation. RESULTS: Excellent and good intubation conditions were obtained in 60% (n = 9) of group P-60, 73% (n = 11) of group S-60, 92% (n = 14) of group P-90 and 100% (n = 15) of group S-90. Single twitch responses were fewer in group P-90 and S-90 compared to those in group P-60 and S-60 (P < 0.01). Blood pressure and heart rate after intubation were elevated in all four groups. CONCLUSIONS: Intubation conditions were not significantly different between inhalation of sevoflurane and intravenous administration of propofol when using rocuronium. Prolonged interval between rocuronium administration and intubation resulted in improved intubation conditions.
Administration, Intravenous ; Blood Pressure ; Heart Rate ; Hemodynamics ; Humans ; Inhalation ; Intubation* ; Propofol ; Thiopental

Although shock in sepsis is usually managed successfully by conventional medical treatment, a subset of cases do not respond and may require salvage therapies such as veno-arterial extracorporeal membrane oxygenation (VA ECMO) support as well as an attempt to remove endotoxins. However, there are limited reports of attempts to remove endotoxins in patients with septic shock on VA ECMO support. We recently experienced a case of septic shock with severe myocardial injury whose hemodynamic improvement was unsatisfactory despite extracorporeal membrane oxygenation (ECMO) support. Since the cause of sepsis was acute pyelonephritis and blood cultures grew gram-negative bacilli, we additionally applied polymyxin B direct hemoperfusion (PMX-DHP) to the ECMO circuit and were able to successfully taper off vasopressors and wean off ECMO support. To the best of our knowledge, this is the first adult case in which PMX-DHP in addition to ECMO support was successfully utilized in a patient with septic shock. This case indicates that additional PMX-DHP therapy may be beneficial and technically feasible in patients with septic shock with severe myocardial injury refractory to ECMO support.
Adult ; Cardiomyopathies* ; Endotoxins ; Extracorporeal Membrane Oxygenation ; Hemodynamics ; Hemoperfusion* ; Humans ; Membranes* ; Oxygen* ; Polymyxin B* ; Polymyxins* ; Pyelonephritis ; Salvage Therapy ; Sepsis ; Shock ; Shock, Septic*

The development of colorectai cancer following pelvic irradiation has been a much less common event. A 58-years-old woman presented with tenesmus for 5 months. She had a stage IIb, carcinoma of the cervix for which she underwent radiation therapy in 27 years before. Colonscopic findings revealed polypoid mass and irregular ulceration on the rectum and proctitis associated with stricture that was compatible with her previous history of radiation. Histoyathological examination of the polypectomy specimen disclosed adenocarcinoma. So we reported a case of radiation induced carcinoma of reotum which might be the first case in Korean literature. It therefore, behooves the clinician who follows patients whose carcinoma of the cervix or corpus uteri has been cured by radiation therapy to be vigilant for the subsequent development of colon and rectal cancer.
Adenocarcinoma* ; Cervix Uteri ; Colon ; Constriction, Pathologic ; Female ; Humans ; Proctitis ; Rectal Neoplasms ; Rectum ; Ulcer ; Uterus

Achondrogenesis is a lethal form of congenital chondrodystrophy characterized by extreme micromelia. We describe a case of achondrogenesis type II (Langer-Saldino achondrogenesis) detected by prenatal ultrasonography at 20-week gestation. A dwarfed fetus with large head, short neck and chest, prominent abdomen and short limbs was terminated transvaginally. Radiologic and histopathologic examination revealed features of mild form of achondrogenesis type II. Although the case had no known risk factor and the phenotypic abnormality was mild, modern development in prenatal screening made the early detection possible.
Abortion, Induced ; Achondroplasia/ultrasonography ; Achondroplasia/pathology* ; Bone and Bones/pathology ; Case Report ; Female ; Fetal Diseases/ultrasonography ; Fetal Diseases/pathology* ; Human ; Pregnancy ; Ultrasonography, Prenatal

This study aimed to investigate cognitive impairment and psychological distress of critically ill patients at discharge from intensive care unit (ICU). This study included 30 critically ill patients who had neither pre-existing dementia nor ongoing delirium. At ICU discharge, they performed a screening test for cognitive impairment (Mini-Cog test) and completed questionnaires for depression (Patient Health Questionnaire-2, PHQ-2) and for 4 stressful experiences during ICU stay including nightmares, severe anxiety or panic, severe pain, and trouble to breathe or feeling of suffocation (Post-Traumatic Stress Syndrome 14-Question Inventory, PTSS-14 Part A). Thirteen patients (43.3%) screened positive for cognitive impairment and 18 patients (60.0%) exhibited depressive symptoms. Twenty three patients (76.7%) recollected one or more stressful in-ICU experiences. Female patients (88.9%) was more likely to feel depressed at ICU discharge, compared to male patients (47.6%) (χ2=4.47, p=0.03). To the best of our knowledge, this is the first report on cognitive and psychological outcomes of ICU survivors in Korea. In this study, we observed that a considerable number of critically ill patients had experienced cognitive impairment or psychological distress at ICU discharge.
Anxiety ; Asphyxia ; Cognition Disorders* ; Critical Care* ; Critical Illness ; Delirium ; Dementia ; Depression ; Dreams ; Female ; Humans ; Intensive Care Units* ; Korea ; Male ; Mass Screening ; Panic ; Survivors

Colonic arteriovenous malformation (AVM) is one of the causes of lower gastrointestinal bleeding. Unlike small vascular ectasia or angiodysplasia, colonic AVM tends to be solitary, large in size, and identified endoscopically as flat or elevated bright red lesion. Herein, we report a case of non-solitary and small cecal AVMs which were removed by endoscopic biopsy. A 66-yr-old woman was referred for routine gastrointestinal cancer screening. She was suffering from diabetes, hypertension, end-stage renal disease, and anemia of chronic disease. On colonoscopic finding, three semi-pedunculated polyps, less than 5 mm in size, were noticed near to the appendiceal orifice. Since the lesions revealed normal-looking epithelium with converging folds on the cecal base, lesions were diagnosed as inflammatory polyps on gross finding. Three biopsies were taken from each lesion. Bleeding from the biopsied site ceased spontaneously. Histopathologic evaluation demonstrated intramucosal hemorrhage and dilated submucosal vessels which were consistent with polypoid colonic AVMs.
Aged ; Arteriovenous Malformations/*diagnosis/pathology/surgery ; Biopsy ; Cecum/blood supply/pathology ; Colon/*blood supply/pathology ; Colonic Polyps/pathology/*surgery ; Colonoscopy ; Female ; Humans

PURPOSE: To report one case of atypical Miller Fisher syndrome with spontaneous recovery. METHODS: A 44-year-old woman was visited our clinic complaining of diplopia for 2 days. She has irregular medication history for thyroid dysfunction, and there was no antecedent respiratory or gastrointestinal infection. The best corrected visual acuity of both eyes was 20/20. She showed bilateral proptosis and limitation of abduction and supraduction, mild blepharoptosis of left eye and esotropia of 30 prism diopter (PD). RESULTS: Thyroid fuction test, orbital CT and electromyography were normal. Cerebrospinal fluid (CSF) analysis revealed albuminocytologic dissociation. Steroid pulse therapy was started promptly, but there was no improvement. Extraocular movement and diplopia gradually improved after 4 weeks of onset. and spontaneously recovered completely after 2 months. CONCLUSIONS: We reported a case of atypical Miller Fisher syndrome that completely and spontaneously recovered within 2 months., and represented normal findings by every examination such as orbital CT, thyroid function test, electromyography and CSF analysis.
Adult ; Blepharoptosis ; Cerebrospinal Fluid ; Diplopia ; Electromyography ; Esotropia ; Exophthalmos ; Female ; Humans ; Miller Fisher Syndrome ; Ophthalmoplegia ; Orbit ; Thyroid Function Tests ; Thyroid Gland ; Visual Acuity

We report 2 cases of minimal deviation adenocarcinoma of the cervix and tumorlets of sex cord tumor with annular tubules (SCTATs) of the ovaries, accompanied by Peutz-Jeghers syndrome. Case 1 is a 36-year-old woman and case 2 is a 35-year-old woman. Grossly, the cervix of both cases showed markedly barrel shaped enlargement with an infiltrating tumor. Microscopically, well-differentiated atypical glands were infiltrating into the entire thickness of the cervix. The ovarian masses in case 1 were diagnosed as metastatic carcinoma in mucinous cystadenoma with tumorlets of SCTATs of the ovaries. Multiple scattered tumorlets of SCTATs were also found in the ovary of case 2. By direct DNA sequencing analysis, a frame shift mutation of the STK11/LKB1 gene was identified in case 1. Case 1 represented the more aggressive clinical course, and although the patient received additional combined chemo-radiation therapy, she expired 1 year later. In general, mutation of the STK11/LKB1 gene is associated with poor clinical outcome in malignant tumors accompanied by Peutz-Jeghers syndrome.
Adenocarcinoma ; Cervix Uteri ; Cystadenoma, Mucinous ; Female ; Frameshift Mutation ; Humans ; Ovary ; Peutz-Jeghers Syndrome ; Sequence Analysis, DNA