Erdheim Chester disease (ECD) is very rare non-Langerhans cell histiocytosis (LCH) which occurs in the skeletal system and multiple organs. As it is progressive, sometimes it causes fatal results. However, it is often misdiagnosed as LCH or multiple bone metastasis and, thus, is very difficult to diagnose. In Korea, only 10 cases were first reported in 1999. In particular, there have been a few orthopedic approaches or reports in English-speaking literatures, and no report has been issued in Korea. The authors performed bone biopsy in patients with knee and lower extremity pain who were referred for the integrated treatment. We attempts to report this diagnosis experience with literature review.
Biopsy ; Erdheim-Chester Disease ; Histiocytosis ; Histiocytosis, Langerhans-Cell ; Humans ; Knee ; Korea ; Lower Extremity ; Neoplasm Metastasis ; Orthopedics

No abstract available.
Leydig Cell Tumor*

McCune-Albright syndrome, which consists of cafe-au lait spots, multiple fibrous dysplasia, sexual precocity and endocrinal dysfunction such as pituitary, thyroid and adrenal aberrations, has been increasingly recognized recently. The etiology is now suggested that activating mutations on the gene that encodes the alpha subunit of stimulatory G protein cause McCune-Albright syndrome. The child presented with sexual precocity and elevated estrogen level was still persisted after left oophorectomy and right partial oophorectomy due to misdiagnosed as ovarian granulosa-theca cell tumor. Whole body bone scan revealed abnormal radiotracer uptake on left facial bone and left proximal femur. Facial bone MRI showed marked thickening of facial bone from fibrous dysplasia. And the patient had cafe-au-lait pigmentations on neck and buttock area. Other endocrine function was normal. Pathologic finding of ovary was finally confirmed with cystic follicles. Skull biopsy revealed fibrous dysplasia. And she was finally diagnosed McCune-Albright syndrome.
Biopsy ; Buttocks ; Cafe-au-Lait Spots ; Child ; Estrogens ; Facial Bones ; Female ; Femur ; Fibrous Dysplasia, Polyostotic* ; GTP-Binding Proteins ; Humans ; Magnetic Resonance Imaging ; Neck ; Ovariectomy ; Ovary ; Pigmentation ; Skull ; Thyroid Gland

No abstract available.
Krukenberg Tumor*

Adventitial cystic disease (ACD) of the veins is a rare vascular disease. Most cases of venous ACD are located adjacent to the joint area, such as the common femoral, external iliac, and popliteal veins. To the best of our knowledge, 67 cases of venous ACD have been reported, and ACD of the superficial femoral vein (SFV) has never been reported. Herein, we report the case of a 57-year-old male who presented with swelling and discomfort in the left leg. Computed tomography venography revealed multiple cystic lesions in the left distal SFV. The patient underwent cyst excision, which relieved the compression in the vein, although mild stenosis prevailed in the SFV. After a week, thrombosis developed in the popliteal vein. The thrombosis resolved after three months of anticoagulant therapy, and the patient showed no recurrence of ACD during three years of follow-up.

Adventitial cystic disease (ACD) of the veins is a rare vascular disease. Most cases of venous ACD are located adjacent to the joint area, such as the common femoral, external iliac, and popliteal veins. To the best of our knowledge, 67 cases of venous ACD have been reported, and ACD of the superficial femoral vein (SFV) has never been reported. Herein, we report the case of a 57-year-old male who presented with swelling and discomfort in the left leg. Computed tomography venography revealed multiple cystic lesions in the left distal SFV. The patient underwent cyst excision, which relieved the compression in the vein, although mild stenosis prevailed in the SFV. After a week, thrombosis developed in the popliteal vein. The thrombosis resolved after three months of anticoagulant therapy, and the patient showed no recurrence of ACD during three years of follow-up.

OBJECTIVE: Our purpose was to compare the pregnancy outcomes of nulliparous women aged 40 years and older with those of nulliparous women under 35 years of age. METHODS: From January 1989 to December 1998 total 57,563 deliveries were seen in Gachon Gil Medical Center. Among them we experienced 59 cases of nulliparas at 40 years and older. These women were compared with 188 young nulliparas under 35 years of age as the control group. The statistical analysis was performed using Chi-square tests, and statistical significance was defined as p<0.05. RESULTS: The incidence rate of elderly nulliparas aged 40 years and older was increased from 0.04% in 1989 to 0.30% in 1998. The age distribution was from 40 years to 45 years. The gravidity of eldery nulliparas was 2.2 in comparison with 1.6 in control group. The incidence of uterine myoma, gestational DM, IUGR and oligohydramnios was significantly high rate in elderly nulliparas. There was significantly high rate of cesarean section in elderly nulliparas(88.1%) in comparison with control group(40.4%). The reasons of cesarean section were her demand(39.0%), CPD(15.3%) and breech presentation(13.6%) in decreasing order. The preterm delivery rate was 10.2% in elderly nulliparas in comparison with 4.3% in the control group. Placenta accreta and uterine atony were significantly high in elderly nulliparas and mean estimated blood loss was also high. There was no difference in 5-minute Apgar score between both group, but there were more cases of neonatal intensive care unit admission in neonates of elderly nulliparas(6.8% vs 0%). CONCLUSION: The incidence of elderly nulliparas is continuously increasing. The elderly nulliparas and their babies are at greater risk than young women. Therefore all elderly nulliparas aged 40 years and older can be regarded as high risk patients and they must be managed with careful attention.
Age Distribution ; Aged ; Apgar Score ; Cesarean Section ; Female ; Fetal Growth Retardation ; Gravidity ; Humans ; Incidence ; Infant, Newborn ; Intensive Care, Neonatal ; Leiomyoma ; Oligohydramnios ; Placenta Accreta ; Pregnancy ; Pregnancy Outcome ; Uterine Inertia

Apert syndrome is an uncommon congenital disorder characterized by malformation of the skull, most often acrocephaly or oxycephaly, in association with symmetrical syndactyly of both hands and feet. It is due to a disturbance in the growth of bone and soft tissue, affecting principally the head, hands, and feet. Recently we experienced a typical Apert syndrome expressed only in one neonate of dizygotic twin.
Acrocephalosyndactylia* ; Congenital, Hereditary, and Neonatal Diseases and Abnormalities ; Craniosynostoses ; Foot ; Hand ; Head ; Humans ; Infant, Newborn* ; Skull ; Syndactyly ; Twins, Dizygotic*

Progressive multifocal leukoencephalopathy (PML) is a demyelinating disease resulting from infection of oligodendrocytes with JC virus. PML was a rare disease, but nowadays not uncommon as AIDS prevailed. Histopathologic features of the affected lesion shows infiltrations of foamy macrophages and hyperchromatic, pleomorphic, reactive astrocytes that may raise the suspicion of a brain tumor. We recently met with 3 cases of PML. Two of the patients had AIDS and the other had been treated for lymphoma. All cases were diagnosed by histopathologic examination in stereotactic brain biopsies.
Acquired Immunodeficiency Syndrome ; Astrocytes ; Biopsy ; Brain ; Brain Neoplasms ; Demyelinating Diseases ; Humans ; Immunocompromised Host* ; JC Virus ; Leukoencephalopathy, Progressive Multifocal* ; Lymphoma ; Macrophages ; Oligodendroglia ; Rare Diseases

No abstract available.
Humans*