Meningioma of unusual age of onset, location, histogenesis and histologic type is reported. The patient, 4 year-old girl, had an intradural spinal meningioma arising from lumbar nerve root with no dural attachement. The meningioma revealed glycogen-rich, clear cell type with extensive and blocky hyalinization of the stroma. The tumor shared common fibrous sheath with attached lumbar nerve, and nerve fibers were scattered within the tumor. Ultrastructurally, the tumor cells had abundant glycogen particles, intermediate filaments and intercellular desmosomes. Hyalinized material revealed large amianthoid collagen fibers.
Child ; Male ; Female ; Humans ; Meningioma

The amplification of c-myc oncogene was evaluated in 42 cases of ovarian carcinomas to correlate with clinical parameters. Using oligonucleotide primers, sequences from the c-myc exon-3 gene and from a control gene, tissue plasminogen activator (tPA), were amplified simultaneously by polymerase chain reaction (PCR). After the products of differential PCR (d-PCR) were electrophoresed, slot blot hybridization was performed, and hybridized with P32 dATP-labeled myc and tPA oligonucleotide probes and then autoradiographed. The signal intensities of the two products were quantitated by densitometry and the ratios of two products (c-myc/tPA) were measured. The ovarian carcinomas showed significantly increased amplification of c-myc oncogene Oligonucleoti compared to normal control group (p<0.05). 15 of 42 cases (35.7%) showed various degrees of the MYC gene amplification up to 27 folds in various histologic types of ovarian carcinomas. No significant differences of the MYC gene amplification according to histologic subtypes, tumor action) grades and clinical stages of ovarian carcinomas were present.
Densitometry ; DNA Primers ; Genes, myc ; Oligonucleotide Probes ; Oncogenes* ; Polymerase Chain Reaction* ; Tissue Plasminogen Activator

Limb-body wall malformation complex(LBWC), also know as the amniotic band syndrome, is a poorly defined, sporadic group of congenital anomaly characterized by a collection of protean fetal malformation, deformation and disruption. Accurate diagnosis is often difficult because of its variable presentation pattern and the absence of exactly same case. We report two autopsy cases. One revealed body wall, cardiac, and craniofacial anomalies with anencephaly, and amniotic adhesive band attached to craniofacial defect of the fetal part without evidence of amniotic rupture. The other exibited abdominal wall defect with omphalocele, visceral, postural, and limb anomalies together with neural tube defect in the lumbosacral region. The pathogenesis of this syndrome was discussed in detail.

Fibrosarcoma in newborns and infants, designated as congenital, infantile, or juvenile fibrosarcoma is an uncommon soft tissue neoplasm occurring most frequently during the first year of life. Infantile fibrosarcoma is associated with favorable clinical behavior that is markedly different from that of adult fibrosarcoma., We report a case of infantile fibrosarcoma occured in a 3-year-old boy presenting as a palpable mass in the left lower extremity since 3 months of life. Histologic findings of the tumor are similar to those of f ibrosarcomas in adult.
Infant ; Adult ; Male ; Female ; Infant, Newborn ; Humans

No abstract available.
Histiocytosis*

No abstract available.
Diagnosis* ; Hemophilia A* ; Molecular Biology* ; Polymorphism, Restriction Fragment Length*

Adenoid cystic carcinoma of the uterine cervix is a rare tumor accounting for less than 1% of all cervical adenocarcinoma. This tumor is characterized by aggressive biological behavior with frequent local recurrence or metastatic spread, postmenopausal onset, and occasional association with conventional squamous cell carcinoma. The cytologic diagnosis of adenoid cystic carcinoma in the uterine cervix is often difficult because of negative smear due to intact overlying mucosa, cytologic findings mimicking endometrial cells, and masquerade as squamous cell carcinoma. Recently we have experienced a case of adenoid cystic carcinoma arising in the uterine cervix, which was identified on the routine Papanicolaou smear and was histologically confirmed by the consequent biopsy. The smear showed abundant cellularity composed of relatively uniform cells. The tumor cells were arranged in small clusters, acini, naked cells, and loose sheets with abortive cribriform pattern. There were scattered globoid basement membrane-like materials and tumor diathesis. The nuclei were pleomorphic and showed hyperchromatic and coarsely granular choromatin with inconspicuous nucleoli. The punch biopsy of the uterine cervix showed typical histologic findings of adenoid cystic carcinoma characterized by tumor nests composed of hyperchromatic uniform basaloid cells, cribriform pattern, and cylindrical hyaline bodies.
Adenocarcinoma ; Adenoids* ; Biopsy ; Carcinoma, Adenoid Cystic* ; Carcinoma, Squamous Cell ; Cervix Uteri* ; Diagnosis ; Disease Susceptibility ; Female ; Hyalin ; Mucous Membrane ; Papanicolaou Test ; Recurrence

No abstract available.
Carcinoid Tumor* ; Lymph Nodes* ; Neoplasm Metastasis*

Garcin's syndrome is defined as showing total unilateral cranial nerve palsies (at least more than 7 ipsilateral cranial nerve palsies), without either sensory or motor long tract disturbance, and without intracranial hypertension. The present report describes an 8-year-old child who had swelling on the right postauricular area and ptosis on the same side which developed before 2 months ago. Neurologic examination disclosed complete unilateral cranial nerve palsies on the right side. Brain MRI revealed a bulky hypervascular well-enhancing solid tumor, involving the right temporal bone, middle cranial fossa, cerebellopontine angle, infratemporal and pterygopalatine fossae, and parapharyngeal space. A biopsy was performed, and its finding showed a chondrosarcoma.
Biopsy ; Brain ; Cerebellopontine Angle ; Child ; Chondrosarcoma* ; Cranial Fossa, Middle ; Cranial Nerve Diseases ; Cranial Nerves ; Humans ; Intracranial Hypertension ; Magnetic Resonance Imaging ; Neurologic Examination ; Pterygopalatine Fossa ; Temporal Bone

Inflammatory and ischemic bowel disease commonly involve a longer segment than a maligant lesion. The characteristic findings of inflammatory bowel disease are mucosal nodular hypertrophy and luminal narrowing. It has recently been reported, however, that the features of mucinous adenocarcinoma are similar to those of inflammatory bowel disease with long segmental involvement. After a brief review of the literature, we describe two cases of mucinous adenocarcinoma of the ascending colon associated with inflammatory disease. Because of long segmental involvement of the ascending colon, mucosal nodular hypertrophy and luminal narrowing, one of these was thought to be multiple villous adenoma with colitis, but was confirmed as mucinous adnocarcinoma. The other case, thought to be tuberculous enterocolitis, was comfirmed as tuberculous enterocolitis associated with mucinous adenocarcinoma.
Adenocarcinoma, Mucinous* ; Adenoma, Villous ; Colitis ; Colon, Ascending* ; Enterocolitis ; Hypertrophy ; Inflammatory Bowel Diseases ; Mucins* ; Phenobarbital