Delayed bleeding after gamma knife surgery for meningioma.

We report the occurrence of haemorrhage in a meningioma after gamma knife surgery.A 52-year-old woman had undergone gamma knife radiosurgery for a growing meningioma in the left tentorial hiatus three years earlier (A radiation dose of 15 Gy was administered to the margin, with a maximum dose of 30 Gy, Fig. 1a). The size of the mass decreased steadily, and central lucency was seen in the follow-up magnetic resonance images, a usual finding seen after gamma knife surgery (MRI, Fig. 1b). However, a MRI taken at the 30-month follow-up showed the tumour to be swollen, and peritumoural oedema had increased (Fig. 1c). Three years later, apoplectic symptoms occurred, and computed tomography revealed a peritumoural haemorrhage, with oedema (Fig. 1d). An emergency craniotomy was carried out, and the biopsy showed a transitional type of meningioma, with vasculopathy and necrosis. After operation she had a right hemiparesis and a visual defect.

Temporal lobe epilepsy caused by choroid plexus papilloma in the temporal horn.

Choroid plexus papilloma (CPP) arising in the temporal horn is rare in adult population, and to the best of our knowledge, there has been no report of such a case with temporal lobe epilepsy (TLE). The authors describe a unique case of a 27-year-old woman who was diagnosed as TLE and was found to have a CPP in the temporal horn. Choroid plexus papilloma of the temporal horn, even though rare, can be found in adult population and be causally related to temporal lobe epilepsy.

Sclerosing meningioma: immunohistochemical analysis of five cases.

Sclerosing meningioma is a rare morphologic subtype of meningioma and may be mistaken for atypical or malignant meningioma and astrocytoma or schwannoma because of marked collagen deposits and a sparse population of cells with little resemblance to meningothelial cells. Authors describe the histopathologic and immunophenotypic features of five cases of sclerosing meningioma. Histologically, all the cases consisted of paucicellular collagenous tissue containing spindle cells with or without small foci of meningothelial cell proliferation. The morphology and immunohistochemical profile of the spindle cells were different from those of conventional meningothelial cells. The meningothelial cells showed a typical immunoreactivity of conventional meningiomas, while the spindle cells displayed a strong expression of vimentin. The Ki-67 labelling index was uniformly low in all cases, and none of cases expressed p53 protein. In summary, the recognition of meningothelial cells in massively sclerotic lesions is helpful for a correct diagnosis. In the cases with a total absence of meningothelial cells, however, the vague collagenous whorls are more diagnostic rather than immunohistochemistry. Considering association with clear cell meningioma, prospective and retrospective long-term follow-up is necessary for deciding whether reminiscent clear cell meningiomas should be separated from sclerosing meningioma or not.

The significance of gemistocytes in astrocytoma.

BACKGROUND: A retrospective clinical analysis of astrocytomas which contained a significant proportion of gemistocytes was carried out in order to evaluate their effect on prognosis, and other factors influencing prognosis. METHOD: From 253 consecutive cases of astrocytic tumours in adults, 25 were selected who had more than 20% gemistocytes in every high-power field examined. 9 of these had anaplasia, the remainder did not. They were divided into two groups according to the proportion of gemistocytes; group A, contained more than 60% gemistocytes, and group B, had between 20 and 60% gemistocytes. TUNEL and immunohistochemical staining for PCNA, p53, Ki-67, bcl-2 were performed in the 20 available cases. FINDINGS: The median follow-up period was 46 months. There were 14 recurrences, with a median time to recurrence of 15 months. Thirteen repeat operations were performed in nine cases, and two cases showed recurring malignant transformation. The overall median survival time following diagnosis was 73 months and the 5-year survival rate was 52%. There were no significant differences in median survival between groups A and B with different proportions of gemistocytes. On the other hand the median survival of the gemistocytic astrocytomas with anaplasia was 25 months, compared with 158 months for those without anaplasia (p=0.0005). The significant impact of anaplasia on survival persisted in both groups. There were no significant differences in immunohistochemical staining between the two groups, with the exception of staining for Ki-67 (means of the two groups: group A 1.40; group B 2.50). CONCLUSIONS: It is suggested that the proportion of gemistocytes does not itself affect prognosis.

Cerebral germinoma with hemiatrophy of the brain: report of three cases.

BACKGROUND: The authors report three cases of cerebral germinoma that occurred in young adults with unusual presentation. METHOD: All three patients presented with hemiparesis and were treated at Seoul National University. A histological diagnosis of germinoma was made by a stereotactic biopsy in all three cases. FINDINGS: Magnetic resonance (MR) images showed that their tumors were located in the internal capsule and thalamus, and were associated with ipsilateral cerebral hemisphere and brain stem atrophy. The hemiparesis slowly progressed and this was accompanied by a haemorrhagic cyst in each patient. INTERPRETATION: Clinical diagnosis was not easy because of the unusual clinical presentations and atypical MR imaging findings. It is suggested that cerebral germinoma should be included in the differential diagnosis of a haemorrhagic mass which is associated with cerebral atrophy in the thalamus, basal ganglia, or internal capsule, especially in adolescents or young adults.

Childhood meningiomas associated with meningioangiomatosis: report of five cases and literature review.

Meningioangiomatosis is a unique, rare hamartomatous lesion. Meningiomas arising in the background of meningioangiomatosis are rare conditions which pathologically and radiologically mimic invasive meningiomas, but have a benign clinical course in children and young adults. In this study, five such cases are reported. To our knowledge, this is the largest reported collection of meningiomas associated with meningioangiomatosis. Less immunoreactivity for progesterone receptor and high Ki-67 labelling index are generally known to be associated with invasive meningiomas. However, high expression of progesterone receptor and low Ki-67 labelling index in the present cases supports the idea that brain invasion is not an indicator of malignancy but an independent finding associated with meningiomas which have arisen from meningioangiomatosis. We emphasize the good prognosis of such tumours and discuss pathogenesis of meningiomas with meningioangiomatosis.

Childhood meningioma: unusual location, atypical radiological findings, and favorable treatment outcome.

OBJECTS: To investigate the characteristics of childhood meningioma, especially, locations, radiological findings, pathological features (including proliferative potential) and outcome, 11 children with meningiomas were retrospectively analyzed. RESULTS: Unusual location, large size, frequent calcification, and cyst formation were characteristic radiological findings. Gross total resection was achieved in 8 patients, and there was recurrence in 2. Gamma knife radiosurgery was performed on residual and recurrent tumors. MIB-1 indices tended to be high in large tumors. Nine patients had a Karnofsky Performance Scale of more than 70 during the follow-up period of 10 months to 19.5 years. Surgical treatment rendered 4 of 5 epileptic patients seizure free. The childhood meningiomas examined had unusual locations, atypical radiological findings, and various proliferative potentials. CONCLUSIONS: Complete resection is the treatment of choice. Gamma knife radiosurgery can be a good alternative for residual tumors and small recurrent tumors. The outcome of childhood meningiomas is good after surgery.

Radical excision of pediatric craniopharyngioma: recurrence pattern and prognostic factors.

The purpose of our study was to investigate the pattern of recurrence and the prognostic factors for recurrence of pediatric craniopharyngiomas after radical excision. A series of 36 patients with craniopharyngiomas (21 boys and 15 girls; age range 1-15 years; mean 7.3 years) were reviewed. All patients had undergone radical excision without radiotherapy. The mean follow-up period was 52 months (range 1-149 months). Tumors recurred in 14 patients within 83 months (mean 31.4 months). The overall 5-year recurrence-free survival rate was 55%. Regular neuroimaging follow-up detected tumor recurrence while the lesions were still small before symptoms developed (P<0.05). At the first surgical procedure, the optic nerve/chiasm (n=23) was the most common adhesion site. The most frequent sites of recurrence were the optic nerve/chiasm (n=6) and the pituitary fossa (n=6). Tumor location was the single significant clinical predictor of recurrence. The 5-year recurrence-free survival rate was 39% for those who had an intrasellar tumor component and 81% for those who did not (P<0.05). The Ki-67 labeling indices (LIs) of primary tumors did not have prognostic value for recurrence. Recurrent tumors tended to have higher Ki-67 LIs than their primary counterparts. On the basis of this study, we concluded that craniopharyngiomas with intrasellar components should be followed cautiously and the necessity for regular follow-up should be emphasized, even when the tumor is "totally" resected.

Cerebellopontine angle ganglioglioma: MR findings.

We present a case of cerebellopontine (CP) angle ganglioglioma in a young child with developmental delay and no trigeminal nerve symptoms. MR imaging demonstrated a mass of homogeneous low signal intensity in the left CP angle on T1-weighted images with no enhancement with gadolinium, and of relatively homogeneous high signal intensity on T2-weighted images.

Umbilical arteritis and phlebitis mark different stages of the fetal inflammatory response.

OBJECTIVE: Funisitis, the inflammation of the umbilical cord determined by histologic examination of the placenta, is evidence of a fetal inflammatory response. The inflammatory process may involve the umbilical vein (phlebitis) and one or both umbilical arteries (arteritis) and extend into the Wharton's jelly. This study was conducted to examine whether the pattern of inflammation of the umbilical cord correlates with a biochemical marker of systemic fetal inflammation (umbilical cord plasma interleukin-6) and an adverse neonatal outcome. STUDY DESIGN: This cohort study included 636 cases of preterm delivery (<36 weeks) with or without inflammation of the umbilical cord. Umbilical cord blood was collected at the time of delivery. The aim of pathologic examination was to characterize the extent of umbilical cord inflammation and the involvement of the vein (phlebitis), the involvement of one or both arteries (arteritis), and the presence of inflammation of the Wharton's jelly. Umbilical cord plasma interleukin-6 concentrations were assayed by a sensitive and specific immunoassay. RESULTS: Neonates with umbilical arteritis had a significantly higher median concentration of cord plasma interleukin-6 (median, 111 pg/mL; range, 0.1-19,230 pg/mL) than those without umbilical arteritis (median, 22.5 pg/mL; range, 0.9-511.6 pg/mL; P <.05). Also, severe neonatal morbidity occurred more frequently in infants with arteritis than in those without arteritis (74% vs 50%; P <.05). And finally, the most severe form of inflammation, which involves both arteries, vein, and Wharton's jelly, was associated with the highest median concentration of plasma interleukin-6 observed in this study (median, 182.6 pg/mL; range, 0.1-7,400 pg/mL), whereas inflammation limited to the vein (phlebitis) was associated with a lower concentration of cord plasma interleukin-6 (median, 29.1 pg/mL; range, 0.9-511.6 pg/mL; P <.05). CONCLUSION: Neonates whose placenta demonstrates umbilical arteritis have higher concentrations of umbilical cord plasma interleukin-6 and higher rates of adverse outcome than those without umbilical arteritis.

Biological behavior and tumorigenesis of subependymal giant cell astrocytomas.

In spite of the benign nature of subependymal giant cell astrocytomas (SEGAs), some show massive hemorrhage, rapid growth, and tumor recurrence. This led us to investigate the biological behavior, cell dynamics, and tumorigenesis of SEGAs. All patients (4 men and 3 women; age range, 6-27 years; mean, 13.6 years) had features of tuberous sclerosis complex and obstructive hydrocephalus. One patient had intratumoral bleeding. In two patients, sequential neuroimaging showed a subependymal nodule growing to become a SEGA. All underwent surgical resection without radiation therapy. One tumor recurred and was treated surgically. There were no postoperative deaths. The presence of cytologic atypia, mitoses and vascular proliferation had no implication in terms of the clinical course. MIB-1 labeling indices were low (mean, 0.9), indicating low proliferative potential. Unexpectedly, bcl-2 staining was sparse and bax staining predominated in majority of cases. However, the mean value of terminal deoxynucleotidyl transferase-mediated dUTP-biotin nick end labeling index was low. Immunohistochemically, tumors were positive for both glial and neuronal markers. In the majority of our cases, the expression of p53 was low. Only one tumor was focally positive for tuberin. SEGAs have low proliferative potential and apoptotic activity, and exhibit features of mixed glial-neuronal differentiation. In contrast to p53, tuberin is suggested to be the tumor suppressor in this tumor.

Prenatal development of the human mandible.

In an effort to better understand the interrelationship of the growth and development pattern of the mandible and condyle, a sequential growth pattern of human mandibles in 38 embryos and 111 fetuses were examined by serial histological sections and soft X-ray views. The basic growth pattern of the mandibular body and condyle appeared in week 7 of fertilization. Histologically, the embryonal mandible originated from primary intramembranous ossification in the fibrous mesenchymal tissue around the Meckel cartilage. From this initial ossification, the ramifying trabecular bones developed forward, backward and upward, to form the symphysis, mandibular body, and coronoid process, respectively. We named this initial ossification site of embryonal mandible as the mandibular primary growth center (MdPGC). During week 8 of fertilization, the trabecular bone of the mandibular body grew rapidly to form muscular attachments to the masseter, temporalis, and pterygoid muscles. The mandible was then rapidly separated from the Meckel cartilage and formed a condyle blastema at the posterior end of linear mandibular trabeculae. The condyle blastema, attached to the upper part of pterygoid muscle, grew backward and upward and concurrent endochondral ossification resulted in the formation of the condyle. From week 14 of fertilization, the growth of conical structure of condyle became apparent on histological and radiological examinations. The mandibular body showed a conspicuous radiating trabecular growth pattern centered at the MdPGC, located around the apical area of deciduous first molar. The condyle growth showed characteristic conical structure and abundant hematopoietic tissue in the marrow. The growth of the proximal end of condyle was also approximated to the MdPGC on radiograms. Taken together, we hypothesized that the MdPGC has an important morphogenetic affect for the development of the human mandible, providing a growth center for the trabecular bone of mandibular body and also indicating the initial growth of endochondral ossification of the condyle.

Fatal submassive hepatic necrosis associated with tyrosine-methionine-aspartate-aspartate-motif mutation of hepatitis B virus after long-term lamivudine therapy.

We present a case of infection with lamivudine-resistant mutant hepatitis B virus (HBV) that fatally exacerbated hepatitis following the emergence of HBV with mutations in the tyrosine-methionine-aspartate-aspartate (YMDD) motif in an immunocompetent patient who was receiving long-term lamivudine therapy. Restriction fragment length polymorphism analysis showed that the YMDD-motif mutant was the predominant form of circulating HBV at the time of the fatal exacerbation, and a necropsy specimen of the liver revealed submassive hepatic necrosis without steatosis.

Acute funisitis of preterm but not term placentas is associated with severe fetal inflammatory response.

Acute funisitis, whose basic pathologic feature is umbilical vasculitis, constitutes a type of fetal inflammatory response to intrauterine infection. In the present study, a comparative analysis was performed between the clinicopathologic profiles of acute funisitis in term and preterm placentas along with measurement of fetal plasma interleukin 6 (IL-6) levels by specific immunoassay to assess the different biologic implications for the fetus. Acute funisitis in preterm placentas showed a significantly higher incidence of umbilical arteritis (P <.000001), higher fetal plasma IL-6 level (P <.0001), and higher prevalence of major perinatal morbidities (P <.0001). To assess the possible variation in fetal cell response to infectious agents according to gestational age, amnion cells and placental villous tissues obtained at different gestational ages were treated with bacterial lipopolysaccharides, and the IL-6 level of the culture media was assayed. Amnion cells and placental villous tissues from preterm placenta showed a more pronounced cytokine response than those from term placenta. The findings of this study indicate that the clinicopathologic significance of acute funisitis in term placentas is different from that of preterm placentas. Furthermore, they indicate that the robust inflammatory response of the fetus associated with elevated fetal plasma IL-6 level may reflect the biologic needs of the premature fetus to escape from the hostile intrauterine environment.

CD99 immunoreactivity in ependymoma.

The expression of CD99 in normal ependymal cells and ependymoma has been reported. However, only limited numbers of tumors have been studied, and the pattern of CD99 expression has not been described. The authors' purpose was to investigate CD99 immunoreactivity in ependymoma and its use for differential diagnosis. Twenty-five ependymomas were immunostained with antibody directed at CD99. The result of immunostaining of ependymomas was compared with 63 nonependymal tumors that histologically resemble ependymal neoplasms. The nonependymal tumors included 19 astrocytic tumors, 6 oligodendroglial tumors, 8 choroid plexus neoplasms, 2 central neurocytomas, 5 medulloblastomas, 10 primitive neuroectodermal tumors (PNET), and 13 pituitary adenomas. All ependymomas showed strong expression of CD99 in membranous pattern with intracytoplasmic or intercellular dots (ICDs). The expression pattern of CD99 was not correlated with histologic type or grade of ependymomas. Among 63 nonependymal tumors, 11 (17.5%) showed incomplete membrane staining for CD99; diffuse in 4 PNETs and focal in 5 choroid plexus neoplasms (3 papillomas and 2 carcinomas) and one each of pituitary adenoma and oligodendroglioma. The ICD was not found in nonependymal tumors except a case of choroid plexus papilloma. However, membrane staining or ICD for CD99 was not distinctive in nonependymal tumors. In conclusion, the characteristic pattern of anti-CD99 antibody, i.e., diffuse strong membranous immunostaining with ICDs, is useful in distinguishing ependymomas from the central nervous system tumors that histologically mimic ependymoma.

Congenital monoblastic leukemia with 9;11 translocation in monozygotic twins : a case report.

We report an autopsy case of congenital monoblastic leukemia that developed in monozygotic twins. The twin presented with progressive hepatosplenomegaly at 4 weeks after birth. One twin died of massive bleeding and hypovolemic shock before the treatment started. At autopsy, the liver was diffusely enlarged and showed a diffuse whitish discoloration except for the subcapsular and perivenular areas. Microscopic examination disclosed infiltration of histiocyte-like atypical cells along the sinusoids and portal areas of the liver. Spleen, lymph nodes and choroid plexus were also infiltrated by the tumor cells. However, bone marrow involvement of the tumor was minimal although multifocal. On immunohistochemical staining, these atypical cells were reactive for CD68 (PGM-1) and lysozyme, suggesting that the tumor cells might have been derived from mono- histiocyte. Cytogenetic study revealed 9;11 translocation, which is frequently associated with acute monoblastic leukemia. To the best of our knowledge, this is the first report of congenital monoblastic leukemia of monozygotic twins in Korea.

Gangliocytoma of the spinal cord: a case report.

We present a case of intramedullary spinal gangliocytoma in a 7-year-old girl who presented with scoliosis and progressive weakness of both legs. The tumour involved the whole spinal cord and medulla oblongata and was composed of inner cystic and outer solid components. On MRI, the solid portion of the lesion showed strong enhancement at the thoracolumbar level and mild enhancement at the cervical and medullary levels. Histological examination of the surgical specimen showed neoplastic ganglion cells arranged irregularly in benign normocellular glial background, which made a diagnosis of gangliocytoma.

A histomorphometric study of corneal endothelial cells in normal human fetuses.

The purpose of this study was to investigate the histomorphometric change in the normal development of human fetal corneal endothelial cells. Eighty one human fetal corneas, ranging from 12 to 40 weeks of gestation, were examined. For determination of gross parameters, corneal diameter and height were measured. Then the corneal endothelium including Descemet's membrane was stained with hematoxylin-eosin using a flat preparation method. In addition to histologic examination under the light microscope, computer-assisted image analysis was performed to determine the cell area, coefficient of variation in cell area and cell density, in both central and peripheral cornea, from each specimen. Total cell count per cornea was obtained by multiplying endothelial cell density by corneal surface area. Linear and nonlinear regression analysis of gestational age and each parameter were used to model corneal endothelial development during the prenatal period. Fetal cornea grows rapidly throughout the prenatal period. During the same period, mean cell area and total cell count also increases gradually, but there is a steep increase in the total cell count in the early period and of the cell area in the late period. The mean cell density decreases rapidly from 16 015 to 6167 cell x mm(-2). There was no significant difference in all parameters except cell density, between the central and peripheral cornea and the difference in cell density was only 2%. In the early prenatal period, there is a rapid increase of total cell count by mitosis, whereas in the late period enlarged endothelial cells cover the rapidly widening inner corneal surface without a significant change in the total cell count.