Osteoma is the most common benign tumor of the cranium, and the frontal sinus is its most frequent location in the cranium. A 39-year-old woman presented with left exophthalmos was found to have orbital and intracranial extension of a giant osteoma involving anterior cranial fossa. The etiology, presenting features, diagnosis and treatment of this tumor are reviewed.
Adult ; Cranial Fossa, Anterior* ; Diagnosis ; Exophthalmos ; Female ; Frontal Sinus ; Humans ; Orbit ; Osteoma* ; Skull

Traditionally, it has been emphasized that hemolytic disease was the primary cause of gallstones (cholelithiasis) in most young patients. In recent years, gallstones and common bile duct calculi have been increasingly diagnosed in infants and children, unrelated to hemolytic diseases. On the matter, the World Health Organization (WHO) classified intraepithelial neoplasia (dysplasia) of gall bladder as one of the precursor lesions of invasive cancer. The following article describes the case of a 2-year-old girl who had a laparoscopic cholecystectomy due to cholelithiasis and the pathologic diagnosis was chronic cholecystitis with diffuse, mild mucosal dysplasia.
Child ; Cholecystectomy, Laparoscopic ; Cholecystitis ; Cholelithiasis ; Gallbladder ; Gallstones ; Humans ; Infant ; Preschool Child ; Urinary Bladder ; World Health Organization

No abstract available.
Humans ; Kyphoplasty

Myeloid sarcoma is a solid, extramedullary tumor composed of leukemic myeloblasts or immature myeloid cells. Intraparenchymal myeloid sarcoma without the involvement of the skull or meninges is extremely rare. Here, we present the case of a 49-year-old man who developed intraparenchymal myeloid sarcoma on the left cerebellum after allogeneic bone marrow transplantation (BMT). He received radiotherapy after complete removal of intraparenchymal myeloid sarcoma, but he was diagnosed spinal myeloid sarcoma three month later. Nine months after the operation, new intracranial and spinal myeloid sarcoma were diagnosed and the patient's condition had been worsened rapidly. Although the spinal myeloid sarcoma was not histologically diagnosed, this report provides valuable insights into the clinical course of progression of intraparenchymal myeloid sarcoma.
Bone Marrow Transplantation ; Cerebellum ; Granulocyte Precursor Cells ; Humans ; Leukemia, Myeloid, Acute ; Meninges ; Middle Aged ; Myeloid Cells ; Sarcoma, Myeloid ; Skull

The authors present a case of 68-year-old woman who underwent resection of a metastatic adenocarcinoma in the left parietooccipital area. The intraoperative course was uneventful; however, after closure of the scalp incision, increased bleeding from the suture line was noted. A computerized tomography scan that was performed immediately after operation revealed acute epidural hemorrhage with mass effect under the bone flap. The patient developed disseminated intravascular coagulation and immediate re-exploration was performed. This patient was successfully treated owing to early recognition of the condition and immediate treatment with transfusion. Neurosurgeons should be alert that hypercoagulabe state is common in cancer patients and consumptive coagulopathy can occur after resection of metastatic brain tumor.
Adenocarcinoma ; Aged ; Blood Transfusion ; Brain ; Brain Neoplasms ; Disseminated Intravascular Coagulation ; Female ; Hemorrhage ; Hemostasis ; Humans ; Scalp ; Sutures

Pilocytic astrocytoma (PA) is a World Health Organization (WHO) grade I tumor typically affecting children and young adults. In general, surgical resection for PA is thought to be curative, and tumor recurrence or malignant transformation is relatively rare. Herein, we report an extremely rare case of a malignant transformation of PA. Five years prior, the patient underwent subtotal resection for PA in the left suprasellar area and left side brain parenchyma of the third ventricle, followed by gamma knife radiosurgery for remnants of the tumor. Four years after surgery, recurrent PA developed, and surgery was performed through an anterior interhemispheric transcallosal approach. One year after surgery, a rapidly growing tumor in the left basal ganglia and hypothalamus was discovered, and subtotal removal was performed. The histological diagnosis was glioblastoma. To the best of our knowledge, malignant transformation of PA has not been reported in Korea. Through review of the literature, we found malignant transformation to be limited solely to patients treated with radiotherapy, which is an observation that should be validated in future studies.
Astrocytoma ; Basal Ganglia ; Brain ; Child ; Glioblastoma ; Humans ; Hypothalamus ; Korea ; Neurofibromatoses ; Neurofibromatosis 1 ; Radiosurgery ; Recurrence ; Third Ventricle ; World Health Organization ; Young Adult

Astroblastoma is a rarely diagnosed primary brain neoplasm whose histogenesis has been clarified recently. It occurs in children and young adults and presents as a well circumscribed, contrast-enhancing lesion in the cerebral hemisphere. We present a case of 20-year-old woman with an astroblastoma in the left temporal convexity that was treated with total tumor resection alone. We thought the mass was extra-axial neoplasm based on radiological findings of computed tomography and magnetic resonance imaging initially, but later, we obtained angiographic findings suggesting an intra-axial neoplasm. The patient is doing well even two years after surgery. The characteristic radiological and histopathological features of this case are described with a literature review. An astroblastoma should be included in the differential diagnosis of a superficially located tumor presenting with the findings of an extra-axial mass, especially in a young patient.
Brain Neoplasms ; Cerebrum ; Child ; Diagnosis, Differential ; Female ; Humans ; Magnetic Resonance Imaging ; Neoplasms, Neuroepithelial ; Young Adult

OBJECTIVE: The von Hippel-Lindau (VHL) disease is an autosomal dominant disorder caused by deletions or mutations in a tumor suppressor gene on human chromosome 3p25. We investigate the VHL germline mutations in a family with VHL disease to define the role of molecular genetic analysis in the management of such patients. And our data add to the diversity of VHL germ-line mutations and provide a better understanding of VHL disease in terms of both clinical management and molecular pathogenesis. METHODS: We have experienced two cases of VHL disease in a family. A 18-year-old male had multiple scattered hemangioblastomas in cerebellum and spinal cord, retinal angiomas and renal cyst. And his mother had a renal cell carcinoma in addition to a solid type hemangioblastoma in cerebellum. The genomic DNA was isolated from peripheral blood cell of family members and renal cell carcinoma of affected mother, and amplified the three exons (acidic domain: aa 1-63, beta domain: aa 64-153, alpha domain: aa 154-213) of VHL suppressor gene to verify the sequences mutations. RESULTS: The data showed that the family with VHL disease does not have any notable mutation in three exons of VHL tumor suppressor gene in both peripheral blood and renal cell carcinoma. CONCLUSION: The failure to detect germline VHL mutations in family with VHL disease may indicate the presence of somatic mosaicism or additional susceptibility genes. Further studies are required to elucidate the mechanism how the VHL disease is induced without any notable mutational inactivation.
Adolescent ; Blood Cells ; Carcinoma, Renal Cell ; Cerebellum ; Chromosomes, Human ; DNA ; Exons ; Genes, Suppressor ; Genes, Tumor Suppressor ; Germ-Line Mutation ; Hemangioblastoma ; Hemangioma ; Humans ; Male ; Molecular Biology ; Mosaicism ; Mothers ; Retinaldehyde ; Spinal Cord ; von Hippel-Lindau Disease*

Primary aldosteronism, is defined as a group of disorders characterized by the excess of aldosteron, with suppressed rennin activity, resulting in hypertension and hypokalemia. In most cases, primary aldosteronism is sporadic due to a unilateral adrenal adenoma or bilateral adrenal hyperplasia. Familial hyperaldosteronism is a rare cause of primary aldosteronism and its prevalence has not been established well. We describe two cases of primary aldosteronism in a family involving a sister and brother due to an aldosterone producing adenoma in the left adrenal gland. Their hypokalemia and hypertension were cured by complete resection of the adrenal adenoma. Genetic analyses could not be done because of patients' rejection.
Adenoma ; Adrenal Glands ; Adrenocortical Adenoma ; Aldosterone ; Chymosin ; Humans ; Hyperaldosteronism ; Hyperplasia ; Hypertension ; Hypokalemia ; Prevalence ; Rejection (Psychology) ; Siblings

PURPOSE: To evaluate the effect of periosteal fixation in patients with large-angle paralytic strabismus that was not corrected through conventional strabismus surgery. METHODS: Four eyes of three patients with large-angle paralytic strabismus who underwent periosteal fixation from June 2014 to August 2014 were examined. All patients presented with exotropia > 50 prism diopters (PD). Two of them showed exotropia caused by chronic complete oculomotor nerve palsy; the other two showed exotropia caused by medial rectus muscle injury during endoscopic sinus surgery. RESULTS: The mean preoperative exodeviation using the Krimsky test was 58 ± 29 PD. The postoperative values were 6.5 ± 9.4 PD at 1 week, and 11.25 ± 2.5 PD at 6 months. The mean surgical effect of exodeviation was 43.75 ± 21.36 PD. CONCLUSIONS: Periosteal fixation is an effective surgery for the management of paralytic strabismus that was not corrected through conventional strabismus surgery.
Exotropia ; Humans ; Oculomotor Nerve Diseases ; Strabismus