We report a case of aggressive angiomyxoma in ischiorectal fossa of a 39-year-old women. The tumor is characterized by relatively large size(13 x 11 cm), grossly gelatinous appearance and locally infiltrative nature. Microscopically, it consists of many variable sized blood vessels and spindle or stellate cells widely separated in myxoid or collagenous stroma. Immunohistochemical stains reveal that the tumor cells are strongly positive for actin and desmin. Electron microscopic findings are that of a few cells dispersed in abundant intercellular substance and collagen bundles. These cells form irregular cytoplasmic process without basal lamina and contain endoplasmic reticulum having cistern.
Female ; Humans

Pigmented(melanotic) schwannoma is a very rare variant of schwannoma that characteristically has massive cytoplasmic melanin. Since it was described in 1946 by Bjorneboe, about 44 cases have been reported in the English literature. It has a relatively benign clinical course, but the cases arising in the cranial nerve and sympathetic chain show aggressive behavior with malignant potential. We herein report a typical case of pigmented schwannoma with light microscopic findings. The results of immunohistochemical and electronmicroscopic study are also presented. The patient was a 30 year-old Korean male who had a mass in his posterior neck for 10 years that recently.began to induce neurologic manifestations. The tumor, which was mainly in the extramedullary intradural space of the cervical canal extending to the extradural space and soft tissues of the neck, was relatively well defined and composed of black solid tissue. Microscopic, densely pigmented spindle cells forming fascicles, nuclear palisading, whorling and polygonal cells with vacuolated or clear cytoplasm were characteristic. Immunohistochemically, the tumor cells were positive for vimentin, S-100 protein, and HMB-45. Electron microscopic study revealed that the tumor cells have interdigitating cytoplasmic processes containing varying stages of melanosomes with a very focal basal lamina and Luse body like collagen bundles.
Male ; Humans

We report three cases of neuroendocrine tumors of the lung characterized by large pleomorphic cell with frequent mitosis, which show neuroendocrine differentiation by both light microscopy or electron microscopy and iminunohistochemistry. These tumors have been categorized as large cell neuroendocrine carcinoma by Travis et al.(1991) in contrast with non-small cell lung cancer with neuroendocrine differentiation. In the latter, neuroendocrine differentiation is not evident by light microscopy and must be demonstrated by imunohistochemstry or by electron microscopy. The prognosis of large cell neuroendocrine carcinoma, together with non-small cell lung cancer with neuroendocrine differentiation, appears to be worse than cancer without neuroendocrine differentiation and intermediate between atypical carcinoid and small cell lung cancer. Larger numbers of patients will be needed to demonstrate significant differences in survival.
Lung Neoplasms

Fifteen cases of papillary cystic tumor of the pancreas (PCTP) were studied (14 female patients, one male patient; mean age: 23.5 years). Most tumors developed in the head of the pancreas as a well circumscribed large mass. The tumor had a mean diameter of 6.7 cm(range; 2 to 15 cm). Histopathologically abundant delicate papillary fragments, monomorphic tumor cells and degenerative changes of the solid area of the tumor were characteristic. All but two cases had completely circumscribed capsules. Two cases had duodenal invasion; one of all cases had cul de sac metastasis. Compared with 12 non-aggressive tumors, the aggressive cases had larger tumor size (more than 9 cm) with a thicker capsule (more than 2 mm). In studies to investigate the prognostic index using nucleolar organizing region (NOR), proliferating cell nuclear antigen (PCNA) and flow cytometry as well as nuclear grade and mitotic index, we could not find the useful parameter to detect the malignant potential of PCTP. In the flow cytometric analysis of cellular DNA contents, two invasive cases and the only one case of the male patient among the non-aggressive group were aneuploid. In conclusion, although it is hard to predict the prognosis by microscopic findings only, those with a thick capsule and aneuploidy tend to be related to malignant potential.
Adolescent ; Adult ; Cell Division/physiology ; Cystadenoma, Papillary/*chemistry/*pathology ; Female ; Flow Cytometry ; Human ; Immunohistochemistry ; Male ; Nucleolus Organizer Region/chemistry ; Pancreatic Cyst/*chemistry/*pathology ; Pancreatic Neoplasms/*chemistry/*pathology ; Predictive Value of Tests ; Prognosis ; Proliferating Cell Nuclear Antigen/analysis ; Silver Staining ; Support, Non-U.S. Gov't

Based on the rDNA ITS sequences data, specific primer set for PCR detection of wood-decaying fungus Phellinus linteus was designed. The length of PCR products using designed primer set(SHF and SHR) was about 540 bp. Among 11 species, 17 isolates of Phellinus spp. including Phellinus linteus, P. pomaceus, P. spiculosus, P. baumi, P. pini, P. igniarius, P. gilvus, P. biscuspidatus, P. weirii, P. johnsonianus, P. robutus, and P. igniarius, seven isolates of Phellinus linteus showed about 540 bp-sized single band. This molecular technique could offer a useful tool for detecting and identifying Phellinus linteus.
DNA, Ribosomal* ; Fungi* ; Polymerase Chain Reaction

Purpose: The aim of this study was to investigate the outcomes of endodontic treatment of mandibular second molar with C-shaped canal in elderly patients and related factors affecting the survival of the tooth. Materials and Methods: From 2010 to 2015, the survival rate following endodontic treatment was evaluated in elderly patients over 60 years who visited the Veterans Health Service Medical Center for endodontic treatment. The presence of C-shaped canals was confirmed using clinical records and radiographic features. Patientʼs age, sex, systemic diseases, tooth location, vitality, signs and symptoms, fractures, caries, apical radiolucency, canal shaping methods, sealer leakage, filling voids, and restoration of prosthesis were included in the analyses as confounding variables. The survival rate of teeth was analyzed using Kaplan–Meire analysis and the relationship between the survival rate and variables was analyzed using Simple and Multiple Cox regression analysis.Result: In total, 107 teeth in elderly patients had C-shaped canal. The survival rate of teeth that received endodontic treatment was 63.70%. None of the factors investigated significantly influenced the survival rate (P>0.05). Conclusion In elderly patients with C-shaped canal, the survival rate after root canal treatment was not significantly different from that of other mandibular molars.

X-linked juvenile retinoschisis (XLRS) is characterized by the progressive loss of visual acuity and vitreous hemorrhage. XLRS is caused by a mutation of retinoschisin 1 (RS1) gene at Xp22.13. In the current report, a 2-year-old Korean patient with XLRS was described. The germline deletion of exon 1 was identified in the RS1 gene. Considering X-linked inheritance pattern, validation of a carrier state of a patient's mother is important for the genetic counseling of other family members and for the future reproductive plan. To confirm the carrier state of his mother, the multiplex ligation-dependent probe amplification analysis was done using peripheral leukocytes and found the heterozygous deletion of exon 1 in his mother.
Carrier State ; Child, Preschool ; Exons ; Genes, X-Linked ; Genetic Counseling ; Humans ; Leukocytes ; Mothers ; Multiplex Polymerase Chain Reaction ; Retinoschisis* ; Visual Acuity ; Vitreous Hemorrhage

The records of 63 high-risk neuroblastoma patients with bone marrow (BM) tumors at diagnosis were retrospectively reviewed. All patients received nine cycles of induction chemotherapy followed by tandem high-dose chemotherapy and autologous stem cell transplantation (HDCT/auto-SCT). Follow-up BM examination was performed every three cycles during induction chemotherapy and every three months for one year after the second HDCT/auto-SCT. BM tumor cells persisted in 48.4%, 37.7%, 23.3%, and 20.4% of patients after three, six, and nine cycles of induction chemotherapy and three months after the second HDCT/auto-SCT, respectively. There was no difference in progression-free survival (PFS) rate between patients with persistent BM tumor and those without during the induction treatment. However, after tandem HDCT/auto-SCT, the PFS rate was worse in patients with persistent BM tumor than in those without (probability of 5-yr PFS 14.7% +/- 13.4% vs. 64.2% +/- 8.3%, P = 0.009). Persistent BM tumor during induction treatment is not associated with a worse prognosis when intensive tandem HDCT/auto-SCT is given as consolidation treatment. However, persistent BM tumor after tandem HDCT/auto-SCT is associated with a worse prognosis. Therefore, further treatment might be needed in patients with persistent BM tumor after tandem HDCT/auto-SCT.
Adolescent ; Antineoplastic Combined Chemotherapy Protocols/administration & dosage ; Bone Marrow Neoplasms/pathology/*secondary/*therapy ; Child ; Child, Preschool ; Combined Modality Therapy/methods ; Female ; Humans ; Induction Chemotherapy/methods ; Infant ; Infant, Newborn ; Male ; Neoplasms, Multiple Primary/pathology/*therapy ; Neuroblastoma/*pathology/*therapy ; Prognosis ; Retrospective Studies ; Risk Factors ; Stem Cell Transplantation/*methods ; Treatment Outcome ; Young Adult

Duchenne and Becker muscular dystrophies (DMD and BMD, respectively) are X-linked neuromuscular disorders characterized by progressive muscle weakness and severe skeletal muscle degeneration. BMD is a milder form with a later onset. Patients with BMD tend to survive much longer than those with DMD. The differentiation between DMD and BMD is important in the genetic counseling of affected patients and their families. Since muscle biopsies are invasive procedures, the differential diagnosis of BMD and DMD is often dependent on the mutation identified in the DMD gene in affected patients. However, when a novel DMD mutation is identified, the differential diagnosis should be based on muscle biopsy findings with other clinical findings. Here we describe two Korean patients with BMD confirmed by muscle biopsy and genetic testing. Two novel exonic deletions in the DMD gene were identified.
Biopsy ; Diagnosis, Differential ; Exons ; Genetic Counseling ; Genetic Testing ; Humans ; Muscle Weakness ; Muscle, Skeletal ; Muscular Dystrophies* ; Muscular Dystrophy, Duchenne*

Fetus in fetu is a poorly understood and rare congenital malfomation. This is a rare form of monozygotic twin that asymmetric twin becomes internalized in the other twin thus acting endoparasitically. Fetus in fetu can be distinguised from teratoma, because of vertebral column, skeletal axis, and well-differentiated internal organs. We present the findings in the two cases of fetus in fetu that were diagnosed with prenatal ultrasonography. After birth, we removed fetus-like structures and confirmed by pathologic examination. Fetus-like structures were consisted of vertebral column, extremities, and other well-developed internal organs.
Axis, Cervical Vertebra ; Extremities ; Fetus* ; Humans ; Parturition ; Spine ; Teratoma ; Twins, Monozygotic ; Ultrasonography, Prenatal*