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Sample records for chordate anterior neuroectodermal

  1. Peripheral primitive neuroectodermal tumor associated with the anterior mandible: a case report and review of the literature.

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    Votta, Timothy J; Fantuzzo, Joseph J; Boyd, Barry C

    2005-11-01

    Neuroectodermal tumors may arise in many places throughout the body including the diverse tissues of the head and neck. The primitive neuroectodermal tumor is a predominately neural, nonepithelial neoplasm similar to Ewing sarcoma. This article describes an 18-year-old female patient with a highly malignant peripheral primitive neuroectodermal tumor located in the soft tissue anterior to the mandibular symphysis. The clinical and radiographic presentation as well as the histopathology and immunohistochemistry of this rare entity is discussed. A review of the literature with respect to this tumor, as well as the current management of this tumor, is presented.

  2. Reconstruction of anterior maxillary defect with buccal pad fat after excision of melanotic neuroectodermal tumor of infancy

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    Kumar, Dinesh; Rattan, Vidya; Rai, Sachin; Yadav, Shikha; Sahu, Gyana Ranjan

    2015-01-01

    This paper highlights a rare case of melanotic neuroectodermal tumor of infancy involving the anterior maxilla in a 3-month-old infant. The tumor was excised completely, and the defect was reconstructed with a bilateral buccal pad of fat. The patient has been followed for 2 years without any evidence of recurrence. We propose that for similar anterior maxillary defects in infants and children, a buccal pad of fat can be utilized as an appropriate pedicled flap for coverage after tumor resection. PMID:26981478

  3. ACAM, a novel member of the neural IgCAM family, mediates anterior neural tube closure in a primitive chordate.

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    Morales Diaz, Heidi; Mejares, Emil; Newman-Smith, Erin; Smith, William C

    2016-01-01

    The neural IgCAM family of cell adhesion molecules, which includes NCAM and related molecules, has evolved via gene duplication and alternative splicing to allow for a wide range of isoforms with distinct functions and homophilic binding properties. A search for neural IgCAMs in ascidians (Ciona intestinalis, Ciona savignyi, and Phallusia mammillata) has identified a novel set of truncated family members that, unlike the known members, lack fibronectin III domains and consist of only repeated Ig domains. Within the tunicates this form appears to be unique to the ascidians, and it was designated ACAM, for Ascidian Cell Adhesion Molecule. In C. intestinalis ACAM is expressed in the developing neural plate and neural tube, with strongest expression in the anterior sensory vesicle precursor. Unlike the two other conventional neural IgCAMs in C. intestinalis, which are expressed maternally and throughout the morula and blastula stages, ACAM expression initiates at the gastrula stage. Moreover, C. intestinalis ACAM is a target of the homeodomain transcription factor OTX, which plays an essential role in the development of the anterior central nervous system. Morpholino (MO) knockdown shows that ACAM is required for neural tube closure. In MO-injected embryos neural tube closure was normal caudally, but the anterior neuropore remained open. A similar phenotype was seen with overexpression of a secreted version of ACAM. The presence of ACAM in ascidians highlights the diversity of this gene family in morphogenesis and neurodevelopment.

  4. Dorsoventral patterning in hemichordates: insights into early chordate evolution.

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    Christopher J Lowe

    2006-09-01

    Full Text Available We have compared the dorsoventral development of hemichordates and chordates to deduce the organization of their common ancestor, and hence to identify the evolutionary modifications of the chordate body axis after the lineages split. In the hemichordate embryo, genes encoding bone morphogenetic proteins (Bmp 2/4 and 5/8, as well as several genes for modulators of Bmp activity, are expressed in a thin stripe of ectoderm on one midline, historically called "dorsal." On the opposite midline, the genes encoding Chordin and Anti-dorsalizing morphogenetic protein (Admp are expressed. Thus, we find a Bmp-Chordin developmental axis preceding and underlying the anatomical dorsoventral axis of hemichordates, adding to the evidence from Drosophila and chordates that this axis may be at least as ancient as the first bilateral animals. Numerous genes encoding transcription factors and signaling ligands are expressed in the three germ layers of hemichordate embryos in distinct dorsoventral domains, such as pox neuro, pituitary homeobox, distalless, and tbx2/3 on the Bmp side and netrin, mnx, mox, and single-minded on the Chordin-Admp side. When we expose the embryo to excess Bmp protein, or when we deplete endogenous Bmp by small interfering RNA injections, these expression domains expand or contract, reflecting their activation or repression by Bmp, and the embryos develop as dorsalized or ventralized limit forms. Dorsoventral patterning is independent of anterior/posterior patterning, as in Drosophila but not chordates. Unlike both chordates and Drosophila, neural gene expression in hemichordates is not repressed by high Bmp levels, consistent with their development of a diffuse rather than centralized nervous system. We suggest that the common ancestor of hemichordates and chordates did not use its Bmp-Chordin axis to segregate epidermal and neural ectoderm but to pattern many other dorsoventral aspects of the germ layers, including neural cell fates

  5. Renal primitive neuroectodermal tumors.

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    Bartholow, Tanner; Parwani, Anil

    2012-06-01

    Primitive neuroectodermal tumors exist as a part of the Ewing sarcoma/primitive neuroectodermal tumor family. These tumors most commonly arise in the chest wall and paraspinal regions; cases with a renal origin are rare entities, but have become increasingly reported in recent years. Although such cases occur across a wide age distribution, the average age for a patient with a renal primitive neuroectodermal tumor is the mid- to late 20s, with both males and females susceptible. Histologically, these tumors are characterized by pseudorosettes. Immunohistochemically, CD99 is an important diagnostic marker. Clinically, these are aggressive tumors, with an average 5-year disease-free survival rate of only 45% to 55%. Given that renal primitive neuroectodermal tumor bears many similarities to other renal tumors, it is important to review the histologic features, immunostaining profile, and genetic abnormalities that can be used for its correct diagnosis.

  6. Uterine primitive neuroectodermal tumor.

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    Aminimoghaddam, Soheila; Seifirad, Soroush; Abbasi Dezfouli, Golbahar; Abbasi, Neda; Zare Mehrjardi, Ali; Razavi, Seyed Mohsen; Mahmoudzadeh, Fatemeh

    2015-04-01

    Primitive neuroectodermal tumors are fairly rare in uterus. A case of uterine body primitive neuroectodermal tumor in a 32-year-old Iranian woman is presented. The patient was admitted with abdominal pain and fever and underwent emergency exploratory surgery with total abdominal hysterectomy, bilateral salpingo-oophorectomy, and pelvic lymph node dissection. Posterior wall of the uterus was necrotic and ruptured and a huge tumor disrupted the uterine body. The tumor was strongly positive for CD99, NSE, and chromogranin; No reaction was seen for CD10, CD45 and myogenin. To the best of our knowledge, this is the first report of an uterine body primitive neuroectodermal tumor and the second report of uterine primitive neuroectodermal tumor from Iran.

  7. Primitive Neuroectodermal Tumor of Mediastinum in an Adult: A Case Report

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    Sung, Young Jae; Kim, Jeung Sook [Dongguk University Ilsan Hospital, Goyang (Korea, Republic of)

    2009-11-15

    A peripheral primitive neuroectodermal tumor (PNET) is a rare and aggressive malignant tumor, which most frequently occurs in children and young adults. The most well described site of origin is the chest wall. We report the case of a primitive neuroectodermal tumor in the anterior mediastinum with unusual CT findings.

  8. Five longitudes in chordate body.

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    Kuznetsov, Alexander N

    2012-01-01

    The evolutionary scenario of chordate origin and body plan formation remains controversial. The existing theories explain very few chordate features besides the dorsal position of their central nervous system. An update of the morphological background can help to interpret new paleontological findings and to pose new molecular targets for further endeavor into the origin of chordates. In the present study, previously underestimated features of vertebrates are generalized, indicative of the presence in the evolution of chordates of a pentaradiate stage, commonly regarded as unique to echinoderms. Five longitudinal lines are distinguished in the trunk of chordates, comprising basic blueprint for ectoderm-mesoderm interactions. All the five can develop outgrowths, such as fin-folds, spines, scutes, limbs and nipples, or invaginations, such as neural tube and lateral line canals, and are regarded as representatives of the five radii of the ancestral pentaradiality. The long-standing theory of dorsoventral inversion at the bilateral evolutionary stage is discouraged. Instead, the hypothetical ancestral subphylum Pentachordata is introduced, characterized by five neural tubes and five muscular notochords. The morpho-functional analysis allows to restore pentachordates as sessile, but active sediment-feeders. Two problematic Precambrian and Lower Cambrian fossils are ascribed to pentachordates. An idea of experimental verification of the Pentachordata hypothesis is proposed.

  9. Primitive neuroectodermal tumor: CT findings

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    Kim, Chang Soo; Eun, Tchoong Kie; Cha, Seong Sook; Han, Sang Suk; Chung, Duck Hwan [Inje College, Paik Hospital, Busan (Korea, Republic of)

    1987-04-15

    Primitive neuroectodermal tumor is a neoplasm of young individuals that occurs predominantly in the supratentorial compartment. Authors experienced three cases of primitive neuroectodermal tumor. On the brain CT scans, all three cases reveal the large, irregular, hyperdense mass lesions with calcifications and cystic or necrotic areas, and show dense heterogeneous contrast enhancement.

  10. Thoracic Primitive Neuroectodermal Tumor: An Unusual Case and Literature Review

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    Kubra Erol Kalkan

    2013-01-01

    Full Text Available We describe herein a rare case of a primary primitive neuroectodermal tumor (PNET in the mediastinum of a 75-year-old man. Grossly, the tumor was located in the left upper anterior mediastinum. Transcutaneous fine-needle biopsy (TCNB revealed small round-cell proliferation. The expression immunohistochemical analysis was confirmed the diagnosis of PNET. He was successfully treated with chemotherapy and is alive with no sign of recurrence for 17 months after the diagnosis.

  11. Vertebrate-like regeneration in the invertebrate chordate amphioxus.

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    Somorjai, Ildikó M L; Somorjai, Rajmund L; Garcia-Fernàndez, Jordi; Escrivà, Hector

    2012-01-10

    An important question in biology is why some animals are able to regenerate, whereas others are not. The basal chordate amphioxus is uniquely positioned to address the evolution of regeneration. We report here the high regeneration potential of the European amphioxus Branchiostoma lanceolatum. Adults regenerate both anterior and posterior structures, including neural tube, notochord, fin, and muscle. Development of a classifier based on tail regeneration profiles predicts the assignment of young and old adults to their own class with >94% accuracy. The process involves loss of differentiated characteristics, formation of an msx-expressing blastema, and neurogenesis. Moreover, regeneration is linked to the activation of satellite-like Pax3/7 progenitor cells, the extent of which declines with size and age. Our results provide a framework for understanding the evolution and diversity of regeneration mechanisms in vertebrates.

  12. Neuroradiology of primitive neuroectodermal tumors

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    Hinshaw, D.B. Jr.; Thompson, J.R.; Hasso, A.N.; Ashwal, S.

    1983-05-01

    The neuroradiological findings in four cases of primitive neuroectodermal tumor of the cerebrum are described. These highly malignant neoplasms of childhood present as large, enhancing cerebral masses with extensive neovascularity. Cerebrospinal fluid seeding is common and distant extraneural metastases may occur.

  13. Primary renal primitive neuroectodermal tumor.

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    Goel, V; Talwar, V; Dodagoudar, C; Singh, S; Sharma, A; Patnaik, N

    2015-01-01

    Primitive Neuroectodermal Tumor of the kidney is a rare entity. Very few cases of primary renal PNET have been reported to date. Most literature about rPNET is isolated case reports. We report a case of rPNET in a 39-year-old male with a pre-operative diagnosis of renal cell carcinoma with renal vein thrombosis. The patient underwent radical nephrectomy with thrombolectomy, and histopathological examination revealed a highly aggressive tumor composed of monotonous sheets of round cells. Tumor cells were positive for CD 99 and FLI-1, hence confirming the diagnosis of Primitive Neuroectodermal Tumor. Post-surgery, patient was given VAC/IE-based adjuvant chemotherapy. In view of highly aggressive nature of this tumor, prompt diagnosis and imparting effective chemotherapy regimen to the patient is required, and it is important to differentiate PNET from other small round-cell tumors because of different therapeutic approach.

  14. Hemichordates and the Origin of Chordates

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    Gerhart, John; Kirschner, Marc; Lowe, Chris

    2002-01-01

    At the start of the period of the NASA grant three years ago, we had no information on the organization and development of the body axis of the hemichordate, Saccoglossus kowalevskii. Now we have substantial findings about the anteroposterior axis and dorsoventral axis, and based on this information, we have new insights about the origin of chordates from ancestral deuterostomes. We found ways to obtain and preserve large numbers of embryos and hatched juveniles. We can now collect about 40,000 embryos in the month of September, the time of S. kowalevskii spawning at Woods Hole. Excellent cDNA libraries were prepared from three developmental stages. From these libraries, we directly isolated about 30 gene ortholog sequences by screening and pcr techniques, all of these sequences of interest in the inquiry about the animal's organization and development. We also performed a mid-sized EST project (60,000 randomly picked clones, many of these arrayed). About half of these have been analyzed so far by blastx and are suitable for direct use of clones. We have obtained about 50 interesting sequences from this set. The rest still await analysis. Thus, at this time we have isolated orthologs of 80 genes that are known to be expressed in chordates in conserved domains and known to have interesting roles in chordate organization and development. The orthology of the S. kowalevskii sequences has been verified by neighbor joining and parsimony methods, with bootstrap estimates of validity. The S. kowalevskii sequences cluster with other deuterostome sequences, namely, other hemichordates, echinoderms, ascidians, amphioxus, or vertebrates, depending on what sequences are available in the database for comparison. We have used these sequences to do high quality in situ hybridization on S. kowalevskii embryos, and the results can be divided into three sections-those concerning the anteroposterior axis of S. kowalevskii in comparison to the same axis of chordates, those concerning

  15. Thoracoabdominal peripheral primitive neuroectodermal tumors in childhood: radiological features

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    Schulman, H.; Laufer, L. [University Medical Center, Beer-Sheva (Israel). Dept. of Radiology; Newman-Heinman, N.; Kurtzbart, E. [Soroka Univ. Medical Center, Beer-Sheva (Israel). Dept. of Pediatrics; Maor, E.; Zirkin, H. [Soroka Univ. Medical Center, Beer-Sheva (Israel). Dept. of Pathology

    2000-10-01

    Peripheral primitive neuroectodermal tumors (PNET) are extremely uncommon, malignant neoplasms affecting mostly children and young adults. We retrospectively reviewed the clinical data and radiological studies of four such cases. All cases were pathologically proven. Plain films, US, and CT scans were used. The youngest child had a huge pelvic tumor and two adolescents each had a chest wall (Askin) tumor. The fourth patient had a most unusual location of the PNET in the anterior mediastinum. The CT findings are emphasized. We emphasize that the markedly abnormal CT findings are not specific for PNET. (orig.)

  16. New Early Cambrian Chordates from Haikou, Kunming

    Institute of Scientific and Technical Information of China (English)

    2001-01-01

    Two new chordates, Cathaymyrus haikouensis Luo et Hu sp. nov. andZhongxiniscus intermedius Luo et Hu gen. et sp. nov. are studied in this paper. Both display numerous S-shaped myomeres on their trunk. C. haikouensis shows a long and slim body similar to that of Cathaymyrus diadexus Shu et al., Zhongxiniscus approaches to Myllokunmingia and Haikouichthvs in the dorsal fin, but differs in the myomeres. Zhongxiniscus may be the intermediate form between Ca thaymyrus and Myllokunmingia and Haikouichthys.

  17. Melanotic neuroectodermal tumour of the pineal region

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    Gorhan, C.; Soto-Ares, G.; Pruvo, J.P. [Dept. of Neuroradiology, Hopital Roger Salengro, CHRU Lille, Lille (France); Ruchoux, M.M. [Dept. of Neuropathology, Hopital Roger Salengro, CHRU Lille (France); Blond, S. [Dept. of Neurosurgery, Hopital Roger Salengro, CHRU Lille (France)

    2001-11-01

    We describe CT and MR findings in a 23-month-old infant with a melanotic neuroectodermal tumour of the pineal gland. The tumour has been stereotactically biopsied and surgically resected. The pathological diagnosis was made on the resected piece. Embryology of the pineal gland and the histology of melanotic neuroectodermal tumour of infancy are discussed. (orig.)

  18. Evolution of the Role of RA and FGF Signals in the Control of Somitogenesis in Chordates.

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    Bertrand, Stéphanie; Aldea, Daniel; Oulion, Silvan; Subirana, Lucie; de Lera, Angel R; Somorjai, Ildiko; Escriva, Hector

    2015-01-01

    During vertebrate development, the paraxial mesoderm becomes segmented, forming somites that will give rise to dermis, axial skeleton and skeletal muscles. Although recently challenged, the "clock and wavefront" model for somitogenesis explains how interactions between several cell-cell communication pathways, including the FGF, RA, Wnt and Notch signals, control the formation of these bilateral symmetric blocks. In the cephalochordate amphioxus, which belongs to the chordate phylum together with tunicates and vertebrates, the dorsal paraxial mesendoderm also periodically forms somites, although this process is asymmetric and extends along the whole body. It has been previously shown that the formation of the most anterior somites in amphioxus is dependent upon FGF signalling. However, the signals controlling somitogenesis during posterior elongation in amphioxus are still unknown. Here we show that, contrary to vertebrates, RA and FGF signals act independently during posterior elongation and that they are not mandatory for posterior somites to form. Moreover, we show that RA is not able to buffer the left/right asymmetry machinery that is controlled through the asymmetric expression of Nodal pathway actors. Our results give new insights into the evolution of the somitogenesis process in chordates. They suggest that RA and FGF pathways have acquired specific functions in the control of somitogenesis in vertebrates. We propose that the "clock and wavefront" system was selected specifically in vertebrates in parallel to the development of more complex somite-derived structures but that it was not required for somitogenesis in the ancestor of chordates.

  19. The evolution of dopamine systems in chordates

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    Kei eYamamoto

    2011-03-01

    Full Text Available Dopamine (DA neurotransmission in the central nervous system (CNS is found throughout chordates, and its emergence predates the divergence of chordates. Many of the molecular components of DA systems, such as biosynthetic enzymes, transporters and receptors, are shared with those of other monoamine systems, suggesting the common origin of these systems. In the mammalian CNS, the DA neurotransmitter systems are diversified and serve for visual and olfactory perception, sensory-motor programming, motivation, memory, emotion, and endocrine regulations. Some of the functions are conserved among different vertebrate groups, while others are not, and this is reflected in the anatomical aspects of DA systems in the forebrain and midbrain. Recent findings concerning a second tyrosine hydroxylase gene (TH2 revealed new populations of DA synthesizing cells, as evidenced in the periventricular hypothalamic zones of teleost fish. It is likely that the ancestor of vertebrates possessed TH2 DA-synthesizing cells, and the TH2 gene has been lost secondarily in placental mammals. All the vertebrates possess DA cells in the olfactory bulb, retina and in the diencephalon. Midbrain DA cells are abundant in amniotes while absent in some groups, e.g. teleosts. Studies of protochordate DA cells suggest that the diencephalic DA cells were present before the divergence of the chordate lineage. In contrast, the midbrain cell populations have probably emerged in the vertebrate lineage following the development of the midbrain-hindbrain boundary. The functional flexibility of the DA systems, and the evolvability provided by duplication of the corresponding genes permitted a large diversification of these systems. These features were instrumental in the adaptation of brain functions to the very variable way of life of vertebrates.

  20. The evolution of dopamine systems in chordates.

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    Yamamoto, Kei; Vernier, Philippe

    2011-01-01

    Dopamine (DA) neurotransmission in the central nervous system (CNS) is found throughout chordates, and its emergence predates the divergence of chordates. Many of the molecular components of DA systems, such as biosynthetic enzymes, transporters, and receptors, are shared with those of other monoamine systems, suggesting the common origin of these systems. In the mammalian CNS, the DA neurotransmitter systems are diversified and serve for visual and olfactory perception, sensory-motor programming, motivation, memory, emotion, and endocrine regulations. Some of the functions are conserved among different vertebrate groups, while others are not, and this is reflected in the anatomical aspects of DA systems in the forebrain and midbrain. Recent findings concerning a second tyrosine hydroxylase gene (TH2) revealed new populations of DA-synthesizing cells, as evidenced in the periventricular hypothalamic zones of teleost fish. It is likely that the ancestor of vertebrates possessed TH2 DA-synthesizing cells, and the TH2 gene has been lost secondarily in placental mammals. All the vertebrates possess DA cells in the olfactory bulb, retina, and in the diencephalon. Midbrain DA cells are abundant in amniotes while absent in some groups, e.g., teleosts. Studies of protochordate DA cells suggest that the diencephalic DA cells were present before the divergence of the chordate lineage. In contrast, the midbrain cell populations have probably emerged in the vertebrate lineage following the development of the midbrain-hindbrain boundary. The functional flexibility of the DA systems, and the evolvability provided by duplication of the corresponding genes permitted a large diversification of these systems. These features were instrumental in the adaptation of brain functions to the very variable way of life of vertebrates.

  1. [Primitive neuroectodermal tumor of the vagus nerve].

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    Pegbessou, E; Diom, E S; Ndiaye, M; Dieng, P A; Nao, E E M; Thiam, A; Diouf, M S; Boube, D; Ndiaye, C; Kossinda, F; Tall, A; Diallo, B K; Ndiaye, I C; Diouf, R; Diop, E M

    2013-12-01

    Primitive neuroectodermal tumors are a rare type of malignant neuroectodermal tumor that is very aggressive. Cervicofacial location is rare, even exceptional. We report a case of a 4-month-old male infant, referred from the pediatric clinic for severe supralaryngeal dyspnea, a firm mass under the left mandibular angle, mobile and extended to the parotid area, painful, with a curve of the left side wall of the oropharynx. Cervical computed tomodensitometry showed a well-limited mass in the carotid area, enhanced by the contrast product. A vascularized mass, which had developed at the expense of the vagus nerve, was removed surgically. Histology found a primitive neuroectodermal tumor.

  2. Primary primitive neuroectodermal tumor of the orbit

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    Das Dipankar

    2009-01-01

    Full Text Available Primitive neuroectodermal tumor (PNET is a small round cell malignant tumor of neuroectodermal origin. Most of the PNETs occur in the central nervous system (CNS. PNETs recognized outside of CNS are diagnosed as peripheral PNET (pPNET. This tumor which expresses MIC-2 gene (CD99 seems to be least aggressive after complete tumor resection. We describe a rare case of PNET in a young girl.

  3. Melanotic neuroectodermal tumor of infancy. MR findings and a review of the literature

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    Atkinson, G.O. Jr.; Davis, P.C.; Patrick, L.E.; Winn, K.J.; Ball, T.I.; Wyly, J.B. (Emory Univ., Atlanta, GA (USA). Dept. of Radiology)

    1989-11-01

    Melanotic neuroectodermal tumor of infancy is an uncommon neoplasm occurring primarily in the child one year or less in age. Difficulty in deciding the cellular origin of this tumor has led to numerous names, including congenital melanocarcinoma, melanotic epithelial odontoma, melanotic ameloblastoma, and retinal anlage tumor, to list a few. Electron microscopy and histochemical studies, however, have now established the neural crest the most likely origin. The most frequent site of occurrence is the maxilla followed by the skull, the brain and the mandible. The genital organs are the most frequent extracranial site. Within the skull, there is a predilection for the anterior fontanel. The following is a case report of a young child with melanotic neuroectodermal tumor of infancy arising at the anterior fontanel. Included is a discussion of magnetic resonance (MR) findings, which to our knowledge, have not been previously reported in this tumor. (orig.).

  4. The amphioxus genome and the evolution of the chordate karyotype

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    Putnam, Nicholas H.; Butts, Thomas; Ferrier, David E.K.; Furlong, Rebecca F.; Hellsten, Uffe; Kawashima, Takeshi; Robinson-Rechavi, Marc; Shoguchi, Eiichi; Terry, Astrid; Yu, Jr-Kai; Benito-Gutierrez, Elia; Dubchak, Inna; Garcia-Fernandez, Jordi; Gibson-Brown, Jeremy J.; Grigoriev, Igor V.; Horton, Amy C.; de Jong, Pieter J.; Jurka, Jerzy; Kapitonov, Vladimir; Kohara, Yuji; Kuroki, Yoko; Lindquist, Erika; Lucas, Susan; Osoegawa, Kazutoyo; Pennacchio, Len A.; Salamov, Asaf A.; Satou, Yutaka; Sauka-Spengler, Tatjana; Schmutz[, Jeremy; Shin-I, Tadasu; Toyoda, Atsushi; Bronner-Fraser, Marianne; Fujiyama, Asao; Holland, Linda Z.; Holland, Peter W. H.; Satoh, Nori; Rokhsar, Daniel S.

    2008-04-01

    Lancelets ('amphioxus') are the modern survivors of an ancient chordate lineage with a fossil record dating back to the Cambrian. We describe the structure and gene content of the highly polymorphic {approx}520 million base pair genome of the Florida lancelet Branchiostoma floridae, and analyze it in the context of chordate evolution. Whole genome comparisons illuminate the murky relationships among the three chordate groups (tunicates, lancelets, and vertebrates), and allow reconstruction of not only the gene complement of the last common chordate ancestor, but also a partial reconstruction of its genomic organization, as well as a description of two genome-wide duplications and subsequent reorganizations in the vertebrate lineage. These genome-scale events shaped the vertebrate genome and provided additional genetic variation for exploitation during vertebrate evolution.

  5. Primitive neuroectodermal tumor of the heart.

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    Nwaejike, Nnamdi; Rassl, Doris; Ford, Hugo; Large, Stephen R

    2012-02-01

    We present a case of primitive neuroectodermal tumor of the left atrium with involvement of the coronary sinus. The initial presentation was of cardiac tamponade resulting from the size of the tumor. There was no evidence of tumor elsewhere, and after complete resection and without adjuvant chemotherapy the patient is well at 2-year follow-up. There has been no evidence of tumor recurrence. This is a rare reported case of resection of a cardiac primitive neuroectodermal tumor without adjuvant chemotherapy. Other cases in the literature have been treated by orthoptic transplantation and resection with chemotherapy.

  6. Primary primitive neuroectodermal tumor of spinal cord

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    Ashutosh Das Sharma

    2016-01-01

    Full Text Available Primarily spinal primitive neuroectodermal tumors are rare neoplasm. A 28-year-old female presented with complaints of pain in lower back, radiating to both lower limbs. Magnetic resonance imaging scan of the lumbosacral spine showed an intradural extramedullary space-occupying lesion. The patient underwent L2–L5 laminectomy with excision of the lesion. Histopathology and immunohistochemistry reports confirmed the diagnosis of primitive neuroectodermal tumor while a thorough metastatic workup ruled out secondary to the spinal cord. The patient developed recurrence at local site within a month after surgery, even before the adjuvant treatment could be started. She is being treated with chemotherapy (human resources protocol.

  7. Cutaneous metastasis of primitive neuroectodermal lung tumor.

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    Garcia Romero, Diana; Hilara Sanchez, Yolanda; Perez Alvarez, Javier; Ramirez Garcia, Jose Ramon; De Pable Martin, Maria Pilar

    2013-06-15

    Primary sarcomas of the chest are rare. Although primitive neuroectodermal tumor (PNET) usually develops in the chest wall, it has been described as a primary pulmonary tumor. We present an unusual case of PNET arising in the lung of an 89-year-old man.

  8. Primitive neuroectodermal tumor of the kidney

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    Aatif Rashid

    2012-01-01

    Full Text Available Primitive neuroectodermal tumor of the kidney is a rare entity. Only a few cases have been reported in the literature and they revealed a variable presentation and an aggressive behavior. Most commonly it is seen in the relatively young population. The diagnosis is usually made at histopathology.

  9. Retinoic acid signaling and the evolution of chordates

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    2006-04-01

    Full Text Available In chordates, which comprise urochordates, cephalochordates and vertebrates, the vitamin A-derived morphogen retinoic acid (RA has a pivotal role during development. Altering levels of endogenous RA signaling during early embryology leads to severe malformations, mainly due to incorrect positional codes specifying the embryonic anteroposterior body axis. In this review, we present our current understanding of the RA signaling pathway and its roles during chordate development. In particular, we focus on the conserved roles of RA and its downstream mediators, the Hox genes, in conveying positional patterning information to different embryonic tissues, such as the endoderm and the central nervous system. We find that some of the control mechanisms governing RA-mediated patterning are well conserved between vertebrates and invertebrate chordates, such as the cephalochordate amphioxus. In contrast, outside the chordates, evidence for roles of RA signaling is scarce and the evolutionary origin of the RA pathway itself thus remains elusive. In sum, to fully understand the evolutionary history of the RA pathway, future research should focus on identification and study of components of the RA signaling cascade in non-chordate deuterostomes (such as hemichordates and echinoderms and other invertebrates, such as insects, mollusks and cnidarians.

  10. Peripheral Primitive Neuroectodermal Tumor of the Pelvis

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    Zohreh Yousefi

    2014-01-01

    Full Text Available The primitive neuroectodermal tumor (PNET belongs to a group of highly malignant tumors and is composed of small round cells of a neuroectodermal origin. Categorized in the same tumor family as Ewing sarcoma, the PNET is most likely to occur in bones and soft tissues. However, a small number of PNET cases arising in the pelvis have been reported as well. We present three cases of pelvic PNET: two cases in the ovary and one case in the broad ligament. The PNET often exhibits aggressive clinical behavior with worse outcomes than other small round cell tumors. The significant prognostic factors of the PNET include site of tumor, volume of neoplasm, and presence of metastasis. The treatment protocol is multimodal and includes local surgical treatment followed by chemotherapy. We herein describe three PNET cases as a rare entity in the pelvis. Pelvic PNETs should be included in the differential diagnosis of pelvic masses.

  11. Peripheral primitive neuroectodermal tumor in masseter muscle.

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    Yazc, Haşmet; Yiğit, Barş; Doğan, Sedat; Sunter, Ahmet Volkan; Behzatoğlu, Kemal

    2013-05-01

    Primitive neuroectodermal tumor is a member of malignant small round cell tumors. These tumors especially originate from the central and autonomous nervous system. However, these tumors may be originated from peripheral tissues and are called peripheral primitive neuroectodermal tumor. A 14-year-old girl attended to the Ear Nose Throat Clinic with the complaint of progressive painless swelling mass for 2 months on the right side of the face. Neck magnetic resonance imaging showed 3.5 × 2.5 × 2-cm isointense mass on T1 and hyperintense on T2 sequences. There was no pathological lymphadenopathy on computed tomographic scan. As a result of mandibular cortical invasion seen on computed tomographic scan, radical surgical excision was decided as surgical treatment. Total parotidectomy with preserving facial nerve and partial mandibulectomy with a 2-cm margin of safety were done, and reconstruction plaque applied to the mandible. Two lymph nodes were seen at the submandibular region. For this reason, prophylactic supraomohyoid neck dissection had also been performed. Pathological assessment proved the diagnosis of PNET, and chemoradiotherapy was planned for the patient.To our knowledge, this is the second reported case in literature. In this present case, peripheral neuroectodermal tumor in the masseter muscle and its diagnosis and treatment process were reported with literature review.

  12. Uterine primitive neuroectodermal tumor with adenosarcoma: a case report

    Directory of Open Access Journals (Sweden)

    Chawla Indu

    2010-06-01

    Full Text Available Abstract Introduction Primitive neuroectodermal tumor of the uterus is extremely rare. They occur as either pure primitive neuroectodermal tumors or admixed with neoplasms of mullerian origin. Case presentation A case of uterine primitive neuroectodermal tumor with adenosarcoma in a 50-year-old Asian Indian woman is presented. Histologically, the neoplasm displayed perivascular pseudorosettes and occasional Homer-Wright rosettes. A strong positivity for neuronspecific enolase and synaptophysin was noted, while chromogranin and CD99 were negative. Merging imperceptibly with the neuroectodermal components were the areas of adenosarcoma. Conclusion To the best of our knowledge, this report represents the second case of a uterine primitive neuroectodermal tumor with an admixed adenosarcoma.

  13. Developmental Control of Cell-Cycle Compensation Provides a Switch for Patterned Mitosis at the Onset of Chordate Neurulation.

    Science.gov (United States)

    Ogura, Yosuke; Sasakura, Yasunori

    2016-04-18

    During neurulation of chordate ascidians, the 11th mitotic division within the epidermal layer shows a posterior-to-anterior wave that is precisely coordinated with the unidirectional progression of the morphogenetic movement. Here we show that the first sign of this patterned mitosis is an asynchronous anterior-to-posterior S-phase length and that mitotic synchrony is reestablished by a compensatory asynchronous G2-phase length. Live imaging combined with genetic experiments demonstrated that compensatory G2-phase regulation requires transcriptional activation of the G2/M regulator cdc25 by the patterning genes GATA and AP-2. The downregulation of GATA and AP-2 at the onset of neurulation leads to loss of compensatory G2-phase regulation and promotes the transition to patterned mitosis. We propose that such developmentally regulated cell-cycle compensation provides an abrupt switch to spatially patterned mitosis in order to achieve the coordination between mitotic timing and morphogenesis.

  14. Anteroposterior patterning in hemichordates and the origins of the chordate nervous system

    Science.gov (United States)

    Lowe, Christopher J.; Wu, Mike; Salic, Adrian; Evans, Louise; Lander, Eric; Stange-Thomann, Nicole; Gruber, Christian E.; Gerhart, John; Kirschner, Marc

    2003-01-01

    The chordate central nervous system has been hypothesized to originate from either a dorsal centralized, or a ventral centralized, or a noncentralized nervous system of a deuterostome ancestor. In an effort to resolve these issues, we examined the hemichordate Saccoglossus kowalevskii and studied the expression of orthologs of genes that are involved in patterning the chordate central nervous system. All 22 orthologs studied are expressed in the ectoderm in an anteroposterior arrangement nearly identical to that found in chordates. Domain topography is conserved between hemichordates and chordates despite the fact that hemichordates have a diffuse nerve net, whereas chordates have a centralized system. We propose that the deuterostome ancestor may have had a diffuse nervous system, which was later centralized during the evolution of the chordate lineage.

  15. Evolution of the reproductive endocrine system in chordates.

    Science.gov (United States)

    Kubokawa, Kaoru; Tando, Yukiko; Roy, Sonali

    2010-07-01

    The cephalochordate, amphioxus, is phylogenetically placed at the most primitive position in the chordate clade. Despite many studies on the endocrine system of amphioxus, definitive evidence has not been reported for the presence an endocrine system comparable to the pituitary-gonadal axis, which is important in the regulation of reproduction in vertebrates. Recent genome analyses in the amphioxus, Branchiostoma floridae, showed that it does not have any pituitary hormone genes except the thyrostimulin gene. Thyrostimulin is a heterodimeric glycoprotein hormone consisting of α and β subunits, and is present in various organs of vertebrates. Analyses of a phylogenetic tree and a synteny suggest that amphioxus' thyrostimulin is an ancestral type of the glycoprotein hormones in chordates. In addition, genes for sex steroidogenic enzymes belonging to the CYP family were found in the genome sequences. The conversion pathway of sex steroids from cholesterol to estrogen, androgen, and major sex steroids was also identified in the gonads of amphioxus in vitro. Furthermore, we demonstrated the expression of genes encoding thyrostimulin and sex steroidogenic enzymes by an in situ hybridization technique. Here, we discuss the evolution of hormones and reproductive functions in the neuroendocrine control system of chordates.

  16. Primary primitive neuroectodermal tumor of the cervix

    Directory of Open Access Journals (Sweden)

    Li B

    2013-06-01

    Full Text Available Bo Li,1 Ling Ouyang,1 Xue Han,1 Yang Zhou,1 Xin Tong,1 Shulang Zhang,1 Qingfu Zhang21Department of Obstetrics and Gynecology, Shengjing Hospital of China Medical University, 2Department of Pathology, the First Affiliated Hospital and College of Basic Medical Sciences, China Medical University, Shenyang, People’s Republic of ChinaAbstract: Primary primitive neuroectodermal tumors (PNETs are rare and high-grade malignant tumors that mostly occur in children and young adults. The most common sites are the trunk, limbs, and retroperitoneum. Herein, we present a case of a PNET involving the cervix uteri in a 27-year-old woman. The lesion showed characteristic histologic features of a PNET and was positive for the immunohistochemical markers cluster of differentiation (CD 99, vimentin, neuron-specific enolase, neural cell adhesion molecule 1 (CD56, and CD117 (c-kit, further defining the tumor while helping to confirm PNET. The clinical Stage IIIB tumor was treated with chemotherapy and radiotherapy.Keywords: primitive neuroectodermal tumor, PNET, cervical neoplasm, immunohistochemistry

  17. Primitive neuroectodermal tumor of the posterior mandible: A case report

    Directory of Open Access Journals (Sweden)

    Monal B. Yuwanati

    2013-03-01

    Full Text Available Primitive neuroectodermal tumor is a term used to describea category of neoplasms of neuroectodermal originwith variable cell differentiation. These tumors arise inmany places throughout the body. The chest wall, larynx,abdomen, and pelvis being the most common primarysites; in rare cases, they occur in the head and neck area.Only 3% of cases have been reported within the skull andjaws. In this report, a rare case of primitive neuroectodermaltumor in the left side posterior mandible in a youngadult is described. J Clin Exp Invest 2013; 4 (1: 101-104Key words: Neuroectodermal tumors, primitive, peripheral,oral cavity, mandible

  18. Malignant melanotic neuroectodermal tumour of infancy affecting the occipital squama.

    Directory of Open Access Journals (Sweden)

    Patankar T

    1998-07-01

    Full Text Available An unusual case of a melanotic neuroectodermal tumour of the occipital squama, which underwent malignant transformation in a nine-month-old infant is reported and pertinent literature reviewed.

  19. Peripheral Primitive Neuroectodermal Tumor of the Stomach: A Case Report

    Energy Technology Data Exchange (ETDEWEB)

    Park, Woon Ju; Cho, June Sik; Shin, Kyung Sook; Jeong, Hyung Yong; Noh; Seung Moo; Song, Kyu Sang [Chungnam University Hospital, Daejeon (Korea, Republic of)

    2010-06-15

    Peripheral primitive neuroectodermal tumors (peripheral PNETs) are very rare and highly aggressive soft tissue malignancies originating from the neural crest. To the best of our knowledge, only a few cases of peripheral PNETs of the stomach have been reported in the literature. We report a case of large peripheral primitive neuroectodermal tumor of the stomach with MDCT findings in a 22-year-old man presenting epigastric pain and vomiting

  20. Primary spinal primitive neuroectodermal tumor on MR imaging.

    Science.gov (United States)

    Thoriya, Prashant J; Watal, Pankaj; Bahri, Nandini U; Rathod, Ketan

    2015-01-01

    Neoplasms in the region of filum terminale are not uncommon. Myxopapillary ependymoma is the commonest tumor at this location. The differentials reported for this entity are nerve sheath tumor, meningioma, paraganglioma, intradural metastases, lymphoma, other varieties of ependymoma, subependymoma, astrocytoma, ganglioglioma, hemangioblastoma, and primitive neuroectodermal tumor (PNET). PNET may very rarely present as an intradural thoracolumbar mass. We present pre- and post-therapy magnetic resonance imaging (MRI) features of a patient with proven primary spinal primitive neuroectodermal tumor (PSPNET) of peripheral subtype.

  1. FOETAL ULTRASOUND - NEUROECTODERMAL ANOMALIES IN RURAL PREGNANT WOMEN

    Directory of Open Access Journals (Sweden)

    Mala Venkata

    2016-06-01

    Full Text Available BACKGROUND A prospective clinical study to know the various types of congenital Neuroectodermal Anomalies on obstetric Ultrasound, in rural pregnant women. To reduce the maternal morbidity and mortality by early detection of these Congenital Neuroectodermal Anomalies. To calculate the incidence and prevalence of different types of Congenital Neuroectodermal Anomalies, in these rural pregnant women. To assist the obstetrician in taking decisions regarding the termination or continuation of the pregnancy in relation to the type of malformation and its prognosis. METHODS A prospective clinical study of Congenital Neuroectodermal Anomalies in 22,000 rural pregnant women coming to the Santhiram Medical College, Radiology Department for a routine obstetric scan. 44 cases of neuroectodermal anomalies were detected out of the 22000 cases, within an incidence of 2 per 1000 cases. Approximately 1 in every 500 cases showed an anomaly. RESULTS The most common lesions detected were hydrocephalus, and spina bifida followed by anencephaly. Association of these lesions with consanguinity, previous history of similar anomaly and intake of iron and folic acid tablets was noted. CONCLUSION Ultrasound is an excellent modality for the diagnosis and characterisation of the neuroectodermal anomalies. Its multiplanar imaging property along with real time image visualisation make it an excellent tool for the diagnosis and characterisation of these anomalies

  2. Facts and fancies about early fossil chordates and vertebrates.

    Science.gov (United States)

    Janvier, Philippe

    2015-04-23

    The interrelationships between major living vertebrate, and even chordate, groups are now reasonably well resolved thanks to a large amount of generally congruent data derived from molecular sequences, anatomy and physiology. But fossils provide unexpected combinations of characters that help us to understand how the anatomy of modern groups was progressively shaped over millions of years. The dawn of vertebrates is documented by fossils that are preserved as either soft-tissue imprints, or minute skeletal fragments, and it is sometimes difficult for palaeontologists to tell which of them are reliable vertebrate remains and which merely reflect our idea of an ancestral vertebrate.

  3. Case Report: Intracranial peripheral primitive neuroectodermal tumor - Ewing′s sarcoma of dura with transcalvarial-subgaleal extension: An unusual radiological presentation

    Directory of Open Access Journals (Sweden)

    Bano Shahina

    2009-01-01

    Full Text Available The occurrence of the intracranial, peripheral, primitive, neuroectodermal tumor, Ewing′s sarcoma (pPNET-ES of the dura, is very rare. Immunophenotypical as well as genetic analyses play key roles in its diagnosis and differentiation from central PNET. We describe here the CT scan and MRI findings of an interesting case of intracranial pPNET-ES arising from the anterior falx cerebri with a trancalvarial-subgaleal extension.

  4. Gridded genomic libraries of different chordate species: a reference library system for basic and comparative genetic studies of chordate genomes.

    Science.gov (United States)

    Burgtorf, C; Welzel, K; Hasenbank, R; Zehetner, G; Weis, S; Lehrach, H

    1998-09-01

    The use of genomic libraries maintained in arrayed format is becoming a more and more popular tool for the analysis of molecular evolution and comparative molecular development. Being able to use already existing reference libraries considerably reduces the work load, and if results are made publicly available, it will facilitate in silica experiments in the future. Here we describe the construction and preliminary characterization of six cosmid libraries of different chordate species, Ciona intestinalis (Hemichordate), Branchiostoma floridae (Cephalochordate), Lampetra fluviatilis (Cyclostoma), Xiphophorus maculatus, and Danio rerio (Osteichthyes) in Lawrist7 and Fugu rubripes in Lawrist4.

  5. Primitive neuroectodermal tumor of the orbit in a 5-year-old girl with microphthalmia

    DEFF Research Database (Denmark)

    Alyahya, Ghassan Ayish Jabur; Heegaard, Steffen; Fledelius, Hans C.;

    2000-01-01

    ophthalmology, primitive neuroectodermal tumor (PNET), Ewing's sarcoma, small round-cell tumors, retinoblastoma, medulloepithelioma, microphthalmia, orbitotomy......ophthalmology, primitive neuroectodermal tumor (PNET), Ewing's sarcoma, small round-cell tumors, retinoblastoma, medulloepithelioma, microphthalmia, orbitotomy...

  6. Renal Primitive Neuroectodermal Tumor: A Case Report.

    Science.gov (United States)

    Yang, Cheng; Xu, Hanjiang; Zhou, Jun; Hao, Zongyao; Wang, Jianzhong; Lin, Changmin; Zhang, Li; Zhu, Xia; Liang, Chaozhao

    2015-12-01

    Primitive neuroectodermal tumor (PNET) is a malignant small round cell tumor and typically arises from bone or soft tissue in adolescents and young adults. Renal PNET is extraordinarily rare and exhibits highly aggressive biological behavior with poor prognosis.We present here a new case of renal PNET in a 31-year-old female. The patients were referred to our hospital because of left flank pain with nausea and vomiting for 1 week. A computed tomography scan revealed a 14.7 × 12.7 cm well-defined, unevenly mass lesion with both solid and cystic components and the tumor was not enhanced uniformly.A preoperative diagnosis of cystic renal cell carcinoma and urinary tract infection was made. The patient undergone anti-inflammatory therapy followed by a left radical nephrectomy. Taken with morphological pattern and immunohistochemical markers, a diagnosis of renal PNET was made. Two cycles of combined chemotherapy were executed. At the 14-month follow-up, no evidence of metastasis or recurrence was indicated.This case reminds clinicians that for adolescents and young adults with a suspicious renal mass, a diagnosis of renal PNET should be always considered. An initial surgery followed by radiotherapy and chemotherapy is suggested for the therapeutic management.

  7. Intramedullary spinal cord primitive neuroectodermal tumor presenting with hydrocephalus.

    Science.gov (United States)

    Alexiou, George A; Siozos, George; Stefanaki, Kalliopi; Moschovi, Maria; Prodromou, Neofytos

    2013-02-01

    Spinal primitive neuroectodermal tumors are exceedingly rare. Herewith, we present the first case of an intramedullary spinal cord tumor associated with hydrocephalus in a 2-month-old boy that presented with left hemiparesis. The patient had been diagnosed on prenatal ultrasound with enlarged ventricular system. At his current admission, a brain magnetic resonance imaging (MRI) revealed hydrocephalus and an intramedullary lesion extending from the second cervical to the first thoracic vertebrae. Dissemination of the tumor was revealed intracranially and in the spinal canal. After a ventriculoperitoneal shunt placement a radical resection of the tumor was performed, however some small tumor remnants could not be safely removed. Postoperative there was no neurologic deterioration. The tumor was diagnosed as a central nervous system primitive neuroectodermal tumor (World Health Organization grade IV). Spinal intramedullary primitive neuroectodermal tumors are extremely rare. In such rare tumors, multiinstitutional studies are needed for treatment guidelines to be established.

  8. Early Chordate Origins of the Vertebrate Second Heart Field

    Science.gov (United States)

    Stolfi, Alberto; Gainous, T. Blair; Young, John J.; Mori, Alessandro; Levine, Michael; Christiaen, Lionel

    2016-01-01

    The vertebrate heart is formed from diverse embryonic territories, including the first and second heart fields. The second heart field (SHF) gives rise to the right ventricle and outflow tract, yet its evolutionary origins are unclear. We found that heart progenitor cells of the simple chordate Ciona intestinalis also generate precursors of the atrial siphon muscles (ASMs). These precursors express Islet and Tbx1/10, evocative of the splanchnic mesoderm that produces the lower jaw muscles and SHF of vertebrates. Evidence is presented that the transcription factor COE is a critical determinant of ASM fate. We propose that the last common ancestor of tunicates and vertebrates possessed multipotent cardiopharyngeal muscle precursors, and that their reallocation might have contributed to the emergence of the SHF. PMID:20671188

  9. Primitive chest wall neuroectodermal tumor in a pediatric patient.

    Science.gov (United States)

    Liu, Zhengcheng; Zou, Wei; Ma, Guodong; Pan, Yanqing

    2011-10-01

    A 13-year-old boy with a primitive neuroectodermal tumor of the chest wall is presented. After four cycles of chemotherapy, a computed tomography scan of his chest showed a larger mass invading the left upper lobe of the lung. He underwent resection of the left chest wall from the left fourth to sixth ribs, including the tumor, combined with left upper lobectomy and lymph node dissection. A diagnosis of primitive neuroectodermal tumor was confirmed histopathologically and immunohistochemically. After surgery, four cycles of chemotherapy with ifosfamide and etoposide were given. One year after treatment, the patient is currently doing well without evidence of recurrence.

  10. Endobronchial Primitive Neuroectodermal Tumor With Pneumothorax Ex Vacuo.

    Science.gov (United States)

    Han, Wongyeong; Huh, Dongmyung; Kim, Byoungho; Kwak, Eunkyoung; Lee, Sunah

    2015-10-01

    We experienced a rare case of an endobronchial primitive neuroectodermal tumor of the left main bronchus. Initially we suspected pneumothorax caused by a collapsed left upper lobe and an air-entrapped lower lobe. After tube thoracostomy, the pneumothorax persisted without air leakage. A tumor was detected at the left main bronchus on computed tomography and bronchoscopy, and diagnosed pathologically as small cell lung cancer. Under the presumed diagnosis of limited-stage small cell lung cancer, we performed a left pneumonectomy. The tumor was eventually identified pathologically as a primitive neuroectodermal tumor. Although adjuvant chemoradiotherapy was not performed, no recurrence was observed.

  11. Childhood pheochromocytoma in a survivor of central primitive neuroectodermal tumor.

    Science.gov (United States)

    Nakano, Yoshiko; Fujimaru, Rika; Ishii, Keiichi; Sakamoto, Hiroaki; Inoue, Takeshi; Sako, Masahiro; Yamada, Hiroshi

    2013-08-01

    Pheochromocytoma and central nervous system primitive neuroectodermal tumor are both neural crest-derived tumors. The former is usually benign and develops mainly in adulthood and the latter brain tumor mainly occurs in childhood and has a poor prognosis. We report a case of a 15-year-old boy who developed pheochromocytoma after more than 10 years of complete remission of central primitive neuroectodermal tumor. Thus far, there have been no reports of childhood cancer survivors who developed pheochromocytoma. This quite rare occurrence of two tumors in a single patient may imply some unidentified linkage or common genetic background.

  12. Radiotherapy of supratentorial primitive neuroectodermal tumor

    Energy Technology Data Exchange (ETDEWEB)

    Kim, D. Y. [Sung Kyun Kwan Univ., Seoul (Korea, Republic of). Coll. of Medicine; Kim, I. H.; Yoo, H. J. [Seoul National Univ., Seoul (Korea, Republic of). Coll. of Medicine; Cho, Y. K. [Inha Univ., Inchon (Korea, Republic of). Coll. of Medicine

    1997-03-01

    To evaluate the efficacy of combined treatment of surgery and chemoradiotherapy for supratentorial primitive neuroectodermal tumors (SPNET) and obtain the prognostic factors and complications. The age of 18 patients ranged from 1 to 27 years (median = 5 years). There were 12 males and 6 females. The extents of surgery were gross total (n=9), subtotal (n=8), biopsy only (n=1). Craniospinal radiotherapy was delivered to all the patients except 2 patients who were treated only with the whole brain and primary lesion. Radiation dose were 3120 - 5800 cGy (median=5460) to primary mass, 1500 - 4200 cGy (median = 3600 cGy) to the whole brain and 1320 - 3600 cGy (median = 2400 cGy) to the spinal axis. Chemotherapy was done in 13 patients. Median follow-up period was 45 months ranged from 1 to 89 months. Patterns of failure were as follows; local recurrence (1), multiple intracranial recurrence (2), spinal seeding (3), craniospinal seeding (2) and multiple bone metastasis (1). Two of two patients who did not received craniospinal radiotherapy failed at spinal are. All the relapsed cases died at 1 to 13 months after diagnosis of progression. The 2- and 5-year overall survival rates were 61% and 49%, respectively. The age, sex, tumor location did not influence the survival bu aggressive resection with combined chemotherapy showed better outcome. Among 9 survivors, complications were detected as radiation necrosis (n=1), hypopituitarism (n=2), cognitive defect (n=1), memory deficit (n=1), growth retardation (n=1). To improve the results of treatment of SPENT, maximal surgical resection followed by radiation therapy and chemotherapy is necessary. The extended radiation field including craniospinal axis may reduce the recurrence in spinal axis. (author).

  13. Primary Primitive Neuroectodermal Tumor of the Breast: a Case Report

    Energy Technology Data Exchange (ETDEWEB)

    Ko, Kyung Ran; Kim, Eun Ah; Kwon, Young Mee [Hospital and Research Institute, National Cancer Center, Goyang (Korea, Republic of); Lee, Eun Sook [Korea University, Seoul (Korea, Republic of)

    2009-08-15

    Primary primitive neuroectodermal tumors (PNET) are rare malignant tumors, affecting mostly children and adolescents. Only three cases of primary breast PNETs have been reported in the medical literature, with none in Korea. We present a case of a primary PNET of the breast in a 33-year-old woman, with imaging and immunohistopathology findings.

  14. Primary extraskeletal Ewing's sarcoma/primitive neuroectodermal tumor of breast

    Directory of Open Access Journals (Sweden)

    Smita Srivastava

    2016-01-01

    Full Text Available Extraskeletal Ewing's sarcoma (EES is a rare soft tissue tumor that is morphologically indistinguishable from skeletal ES. We report a case of a 25-year-old female with recurrent EES/primitive neuroectodermal tumor of right breast with imaging findings on mammogram, ultrasound, magnetic resonance imaging breast, and positron emission tomography–computed tomography.

  15. Cervicoplastia anterior Anterior cervicoplasty

    Directory of Open Access Journals (Sweden)

    Lucas Gomes Patrocínio

    2004-10-01

    Full Text Available Muitos pacientes buscam correção estética da frouxidão da pele do pescoço, depósito de gordura na região submentoneana ou bandas de platisma. Em grande parte dos casos a ação medial, via cervicoplastia anterior é necessária. OBJETIVO: Demonstrar a casuística e avaliar os resultados e complicações com a técnica de cervicoplastia anterior no Serviço de Otorrinolaringologia da Universidade Federal de Uberlândia. FORMA DE ESTUDO: Relato de série. PACIENTES E MÉTODOS: Quarenta e dois pacientes, entre 39 e 65 anos de idade, sendo 40 (95,2% do sexo feminino e 2 (4,8% do masculino, foram submetidos a cervicoplastia anterior. Retrospectivamente foram avaliados resultados e complicações. RESULTADOS: Destes, 34 apresentaram resultados satisfatórios, 4 apresentaram déficit estético notado somente pelo cirurgião, 3 apresentaram déficit estético notado somente pelo paciente e 1 apresentou déficit estético necessitando cirurgia revisional. Ao estudo fotográfico, todos os pacientes apresentaram melhora do perfil cervical, redução das bandas de platisma e da frouxidão da pele, estabilização da musculatura cervical e acentuação do ângulo cervicomental, em graus variados. Houve complicação em 2 casos (discreto serohematoma e cicatriz um pouco alargada. CONCLUSÃO: A cervicoplastia, associada ou não à tração lateral pela ritidoplastia, é uma técnica que produz resultados satisfatórios na grande maioria dos casos.Many patients look for aesthetic correction of the laxity of neck skin, submandibular fat deposit or platisma bands. In a large part of the cases, medial action, through anterior cervicoplasty is necessary. AIM: To demonstrate the casuistic and to evaluate the results and complications with anterior cervicoplasty technique in the Otorhinolaryngology Service of the Federal University of Uberlândia. STUDY DESIGN: Serie report. PATIENTS AND METHODS: Forty-two patients, between 39 and 65 years of age, being 40 (95

  16. The genome sequence of the colonial chordate, Botryllus schlosseri

    Science.gov (United States)

    Voskoboynik, Ayelet; Neff, Norma F; Sahoo, Debashis; Newman, Aaron M; Pushkarev, Dmitry; Koh, Winston; Passarelli, Benedetto; Fan, H Christina; Mantalas, Gary L; Palmeri, Karla J; Ishizuka, Katherine J; Gissi, Carmela; Griggio, Francesca; Ben-Shlomo, Rachel; Corey, Daniel M; Penland, Lolita; White, Richard A; Weissman, Irving L; Quake, Stephen R

    2013-01-01

    Botryllus schlosseri is a colonial urochordate that follows the chordate plan of development following sexual reproduction, but invokes a stem cell-mediated budding program during subsequent rounds of asexual reproduction. As urochordates are considered to be the closest living invertebrate relatives of vertebrates, they are ideal subjects for whole genome sequence analyses. Using a novel method for high-throughput sequencing of eukaryotic genomes, we sequenced and assembled 580 Mbp of the B. schlosseri genome. The genome assembly is comprised of nearly 14,000 intron-containing predicted genes, and 13,500 intron-less predicted genes, 40% of which could be confidently parceled into 13 (of 16 haploid) chromosomes. A comparison of homologous genes between B. schlosseri and other diverse taxonomic groups revealed genomic events underlying the evolution of vertebrates and lymphoid-mediated immunity. The B. schlosseri genome is a community resource for studying alternative modes of reproduction, natural transplantation reactions, and stem cell-mediated regeneration. DOI: http://dx.doi.org/10.7554/eLife.00569.001 PMID:23840927

  17. Neoplastic meningitis as the presentation of occult primitive neuroectodermal tumors.

    Science.gov (United States)

    Jennings, M T; Slatkin, N; D'Angelo, M; Ketonen, L; Johnson, M D; Rosenblum, M; Creasy, J; Tulipan, N; Walker, R

    1993-10-01

    Seven children and young adults initially presented with subacute meningitis and/or increased intracranial pressure. The diagnosis of neoplastic meningitis secondary to a primitive neuroectodermal neoplasm was delayed by the absence of an obvious primary tumor. The neuroradiologic appearance was that of a basimeningeal infiltrative process, complicated by communicating hydrocephalus or "pseudotumor cerebri." Myelography was important in the diagnosis of disseminated meningeal malignancy in four cases. Cerebrospinal fluid cytologic diagnosis was insensitive but ultimately confirmed in five cases. All seven patients experienced progressive disease despite neuraxis radiotherapy and intensive chemotherapy; six have died. Systemic dissemination to bone and/or peritoneum occurred in three patients while on therapy. In two, a primary parenchymal brain or spinal cord tumor could not be identified at postmortem examination. The presentation of a primitive neuroectodermal tumor as subacute meningitis without an evident primary tumor heralds an aggressive and refractory neoplasm.

  18. Primary renal primitive neuroectodermal tumor: A rare presentation

    Directory of Open Access Journals (Sweden)

    V Goel

    2015-01-01

    Full Text Available Primitive Neuroectodermal Tumor of the kidney is a rare entity. Very few cases of primary renal PNET have been reported to date. Most literature about rPNET is isolated case reports. We report a case of rPNET in a 39-year-old male with a pre-operative diagnosis of renal cell carcinoma with renal vein thrombosis. The patient underwent radical nephrectomy with thrombolectomy, and histopathological examination revealed a highly aggressive tumor composed of monotonous sheets of round cells. Tumor cells were positive for CD 99 and FLI-1, hence confirming the diagnosis of Primitive Neuroectodermal Tumor. Post-surgery, patient was given VAC/IE-based adjuvant chemotherapy. In view of highly aggressive nature of this tumor, prompt diagnosis and imparting effective chemotherapy regimen to the patient is required, and it is important to differentiate PNET from other small round-cell tumors because of different therapeutic approach.

  19. Primitive neuroectodermal tumor/Ewing sarcoma of the retina.

    Science.gov (United States)

    Grossniklaus, Hans E; Shehata, Bahig; Sorensen, Poul; Bergstrom, Chris; Hubbard, G Baker

    2012-07-01

    An 11-year-old boy underwent enucleation of his left eye for an intraocular tumor. Examination showed a small, round blue cell tumor arising in the peripheral retina near the ciliary body. Immunohistochemical stain results were positive for neuron-specific enolase, synaptophysin, cluster of differentiation 99 (CD99), Friend leukemia integration 1, and CD56. Ultrastructural findings included occasional intracytoplasmic dense core granules. Polymerase chain reaction of the tumor showed a Ewing sarcoma/Friend leukemia integration gene fusion product. The tumor was classified as a primitive neuroectodermal tumor/Ewing sarcoma of the retina and should be distinguished from retinoblastoma. To our knowledge, this is the first case of primary primitive neuroectodermal tumor of the retina.

  20. Symmetry breaking and convergent extension in early chordate development.

    Science.gov (United States)

    Schiffmann, Yoram

    2006-10-01

    The initiation of axis, polarity, cell differentiation, and gastrulation in the very early chordate development is due to the breaking of radial symmetry. It is believed that this occurs by an external signal. We suggest instead spontaneous symmetry breaking through the agency of the Turing-Child field. Increased size or decreased diffusivity, both brought about by mitotic activity, cause the spontaneous loss of stability of the homogeneous state and the evolution of the metabolic pattern during development. The polar metabolic pattern is the cause of polar gene expression, polar morphogenesis (gastrulation), and polar mitotic activity. The Turing-Child theory explains not only the spontaneous formation of the invagination in gastrulation but also the coherent cell movement observed in convergence and extension during gastrulation and neurulation. The theory is demonstrated with respect to experimental observations on the early development of fish, amphibian, and the chick. The theory can explain a multitude of experimental details. For example, it explains the splayed polar progression of reduction in the fish blastoderm. Reduction starts on that side of the blastoderm margin, which will initiate invagination several hours later. It progresses toward the blastoderm center and somewhat laterally from this future "dorsal lip". This is precisely as predicted by a Turing-Child system in a circle. And for a fish like zebrafish with a blastoderm that is slightly oval, reduction is observed to progress along the long axis of the ellipse, which is what Turing-Child theory predicts. In general the shape and the chemical nature of the experimental patterns are the same as predicted by the Turing couple (cAMP, ATP). Embryological polarity and convergent extension are based on polar eigenfunction and saddle-shaped eigenfunction, respectively.

  1. Parallel evolution of chordate cis-regulatory code for development.

    Directory of Open Access Journals (Sweden)

    Laura Doglio

    2013-11-01

    Full Text Available Urochordates are the closest relatives of vertebrates and at the larval stage, possess a characteristic bilateral chordate body plan. In vertebrates, the genes that orchestrate embryonic patterning are in part regulated by highly conserved non-coding elements (CNEs, yet these elements have not been identified in urochordate genomes. Consequently the evolution of the cis-regulatory code for urochordate development remains largely uncharacterised. Here, we use genome-wide comparisons between C. intestinalis and C. savignyi to identify putative urochordate cis-regulatory sequences. Ciona conserved non-coding elements (ciCNEs are associated with largely the same key regulatory genes as vertebrate CNEs. Furthermore, some of the tested ciCNEs are able to activate reporter gene expression in both zebrafish and Ciona embryos, in a pattern that at least partially overlaps that of the gene they associate with, despite the absence of sequence identity. We also show that the ability of a ciCNE to up-regulate gene expression in vertebrate embryos can in some cases be localised to short sub-sequences, suggesting that functional cross-talk may be defined by small regions of ancestral regulatory logic, although functional sub-sequences may also be dispersed across the whole element. We conclude that the structure and organisation of cis-regulatory modules is very different between vertebrates and urochordates, reflecting their separate evolutionary histories. However, functional cross-talk still exists because the same repertoire of transcription factors has likely guided their parallel evolution, exploiting similar sets of binding sites but in different combinations.

  2. Evolution of a core gene network for skeletogenesis in chordates.

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    Jochen Hecht

    2008-03-01

    Full Text Available The skeleton is one of the most important features for the reconstruction of vertebrate phylogeny but few data are available to understand its molecular origin. In mammals the Runt genes are central regulators of skeletogenesis. Runx2 was shown to be essential for osteoblast differentiation, tooth development, and bone formation. Both Runx2 and Runx3 are essential for chondrocyte maturation. Furthermore, Runx2 directly regulates Indian hedgehog expression, a master coordinator of skeletal development. To clarify the correlation of Runt gene evolution and the emergence of cartilage and bone in vertebrates, we cloned the Runt genes from hagfish as representative of jawless fish (MgRunxA, MgRunxB and from dogfish as representative of jawed cartilaginous fish (ScRunx1-3. According to our phylogenetic reconstruction the stem species of chordates harboured a single Runt gene and thereafter Runt locus duplications occurred during early vertebrate evolution. All newly isolated Runt genes were expressed in cartilage according to quantitative PCR. In situ hybridisation confirmed high MgRunxA expression in hard cartilage of hagfish. In dogfish ScRunx2 and ScRunx3 were expressed in embryonal cartilage whereas all three Runt genes were detected in teeth and placoid scales. In cephalochordates (lancelets Runt, Hedgehog and SoxE were strongly expressed in the gill bars and expression of Runt and Hedgehog was found in endo- as well as ectodermal cells. Furthermore we demonstrate that the lancelet Runt protein binds to Runt binding sites in the lancelet Hedgehog promoter and regulates its activity. Together, these results suggest that Runt and Hedgehog were part of a core gene network for cartilage formation, which was already active in the gill bars of the common ancestor of cephalochordates and vertebrates and diversified after Runt duplications had occurred during vertebrate evolution. The similarities in expression patterns of Runt genes support the view

  3. Primary intraspinal extradural primitive neuroectodermal tumor: A rare case

    Science.gov (United States)

    Rege, Shrikant V.; Tadghare, Jitendra; Patil, Harshad; Narayan, Sharadendu

    2016-01-01

    Primitive neuroectodermal tumors (PNETs) are aggressive childhood malignancies and are difficult to treat. Primary intraspinal PNETs are rare. These patients have poor prognosis with short survival time even after surgery and chemoradiation. As there are no standard guidelines exist for the management of these tumors, a multidisciplinary approach has been employed with varying success. According to the review of literature, only few cases of primary intraspinal extradural PNETs have been reported. Herein, author has described a case of intraspinal, extradural PNET. PMID:28217164

  4. Intracranial Ewings Sarcoma/peripheral primitive neuroectodermal tumor

    Directory of Open Access Journals (Sweden)

    R Amita

    2014-01-01

    Full Text Available Central nervous system primitive neuroectodermal tumours (CNS PNET are aggressive embryonal tumours composed of undifferentiated or poorly differentiated neuroepithelial cells seen in the pediatric age group. This is rare and only a handful of cases of ES/pPNET in CNS are reported. We report such a case in a 3 year old child. Reporting of more such cases is needed to better define these rare tumours of the dura.

  5. MOLECULAR (RE-)CLASSIFICATION OF CNS-PRIMITIVE NEUROECTODERMAL TUMORS

    OpenAIRE

    Kool, Marcel; Sturm, Dominik; Northcott, Paul A.; Jones, David T. W.; Korshunov, Andrey; Lichter, Peter; Pfister, Stefan

    2014-01-01

    BACKGROUND: According to the current WHO classification of CNS tumors, childhood CNS primitive neuro-ectodermal tumors (CNS-PNETs; WHO °IV) are poorly differentiated embryonal tumors with early onset and aggressive clinical behavior. Histological diagnosis can be complicated by morphological heterogeneity and divergent differentiation. Recent studies suggest the existence of molecular subgroups of CNS-PNETs sharing biological characteristics with other childhood CNS tumors. Here, we aimed at ...

  6. Mohammad Shakeel et al 694 Melanotic Neuroectodermal Tumor of Infancy

    OpenAIRE

    Mohammad Shakeel; Munaza Shafi; Mohammad Imran; Mudasar Ahad

    2016-01-01

    Melanotic neuroec todermal tumor of infancy is a rare congenital neoplasm involving the head and neck region in young patients. A case of melanotic neuroectodermal tumor of infancy is pre - sented. This tumor occurred in left maxillary alveolar ridge in a 5-month-old female. The present case showed an increased urinary level of vanillylmandelic acid, confirming that the tumor is originated from neural crest. Clinical assessment, histologic diagnosis and labora...

  7. Primitive neuroectodermal tumor of the liver: a case report.

    Science.gov (United States)

    Mani, Siddhartha; Dutta, Deep; De, Binay K

    2010-03-01

    Ewing sarcoma/primitive neuroectodermal tumor is a rare tumor of soft tissues of thoraco-pulmonary regions, pelvis and lower extremities. Involvement of visceral organs by primitive neuroectodermal tumor is even rarer, with the kidney being the most commonly involved organ. Involvement of the liver has been reported in the form of metastasis from other primary sources presenting as liver abscess. We report a 20-year-old lady presenting with massive hepatomegaly, with computed tomography scan evidence of diffuse hepatomegaly and a normal porta and intrahepatic biliary radicles. She subsequently underwent ultrasonography-guided true-cut needle biopsy of the liver. Histopathology of the liver revealed nests of small round blue tumor cells in the background of hepatocytes infiltrating the liver, which expressed Mic-2 and Fli-1, and were negative for cytokeratin, desmin, hepatocyte-specific antigen (OCHIE5), synaptophysin, chromogranin A and CD-20. Immunohistochemistry revealed CD-99-positive. Extensive search regarding any possible different site of involvement by the tumor was negative. The patient responded to a combination therapy of vincristine, adriamycin and cyclophosphamide alternating with ifosfamide and etoposide 3 weekly over 43 weeks and has been doing well even after 1 year of diagnosis. The clinical presentation, the macroscopic aspect, together with the histological pattern, the cytological characteristic and the cellular immunophenotype lead to the diagnosis of primary primitive neuroectodermal tumor of the liver which responded well to combination chemotherapy.

  8. Distinctive expression patterns of Hedgehog pathway genes in the Ciona intestinalis larva: implications for a role of Hedgehog signaling in postembryonic development and chordate evolution.

    Science.gov (United States)

    Islam, A F M Tariqul; Moly, Pricila Khan; Miyamoto, Yuki; Kusakabe, Takehiro G

    2010-02-01

    Members of the Hedgehog (Hh) family are soluble ligands that orchestrate a wide spectrum of developmental processes ranging from left-right axis determination of the embryo to tissue patterning and organogenesis. Tunicates, including ascidians, are the closest relatives of vertebrates, and elucidation of Hh signaling in ascidians should provide an important clue towards better understanding the role of this pathway in development. In previous studies, expression patterns of genes encoding Hh and its downstream factor Gli have been examined up to the tailbud stage in the ascidian embryo, but their expression in the larva has not been reported. Here we show the spatial expression patterns of hedgehog (Ci-hh1, Ci-hh2), patched (Ci-ptc), smoothened (Ci-smo), and Gli (Ci-Gli) orthologs in larvae of the ascidian Ciona intestinalis. The expression patterns of Ci-hh2 and Ci-Gli dramatically change during the period between the late tailbud embryo and the swimming larva. At the larval stage, expression of Ci-Gli was found in a central part of the endoderm and in the visceral ganglion, while Ci-hh2 was expressed in two discrete endodermal regions, anteriorly and posteriorly adjacent to the cells expressing Gli. The expression patterns of these genes suggest that the Hh ligand controls postembryonic development of the endoderm and the central nervous system. Expression of a gene encoding Hh in the anterior and/or pharyngeal endoderm is probably an ancient chordate character; diversification of regulation and targets of the Hh signaling in this region may have played a major role in the evolution of chordate body structures.

  9. Primitive neuroectodermal tumor of the kidney in a young male: Case report and review of literature.

    Science.gov (United States)

    Patnaik, Nivedita; Mishra, Kiran; Saini, Pradeep; Agarwal, Nitin

    2015-01-01

    Primitive neuroectodermal tumor of the kidney is a rare tumor. A total of approximately 79 primary renal cases have been reported to date. Primitive neuroectodermal tumors occur preferentially in the soft-tissues of the paravertebral region and chest wall, less frequently in extremities, with a slight male predominance. We report a case of primitive neuroectodermal tumor of the kidney in a 17-year-old male with a pre-operative diagnosis of renal cell carcinoma-stage 4. The patient underwent radical nephrectomy and histopathological examination revealed a highly aggressive tumor of monotonous sheets of round cells with focal areas of rosette formations and high mitotic rate with Ki67 index of 25-30%. Tumor cells were positive for CD 99 confirming the diagnosis of primitive neuroectodermal tumor. Primitive neuroectodermal tumor of the kidney needs to be kept in mind as a differential diagnosis in young adults presenting with a large kidney mass.

  10. Evolutionary patterns of RNA-based duplication in non-mammalian chordates.

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    Ming Chen

    Full Text Available The role of RNA-based duplication, or retroposition, in the evolution of new gene functions in mammals, plants, and Drosophila has been widely reported. However, little is known about RNA-based duplication in non-mammalian chordates. In this study, we screened ten non-mammalian chordate genomes for retrocopies and investigated their evolutionary patterns. We identified numerous retrocopies in these species. Examination of the age distribution of these retrocopies revealed no burst of young retrocopies in ancient chordate species. Upon comparing these non-mammalian chordate species to the mammalian species, we observed that a larger fraction of the non-mammalian retrocopies was under strong evolutionary constraints than mammalian retrocopies are, as evidenced by signals of purifying selection and expression profiles. For the Western clawed frog, Medaka, and Sea squirt, many retrogenes have evolved gonad and brain expression patterns, similar to what was observed in human. Testing of retrogene movement in the Medaka genome, where the nascent sex chrosomes have been well assembled, did not reveal any significant gene movement. Taken together, our analyses demonstrate that RNA-based duplication generates many functional genes and can make a significant contribution to the evolution of non-mammalian genomes.

  11. Primitive neuroectodermal tumor of the kidney: a case report

    Energy Technology Data Exchange (ETDEWEB)

    Kim, See Hyung; Cho, Jae Ho; Chang, Jay Chun; Park, Bok Hawn [School of Medicine, Yeungnam Univ., Daegu (Korea, Republic of)

    2002-07-01

    Primitive neuroectodermal tumor (PNET) is relatively uncommon, arising outside the central nervous system. Very rarely, it occurs within the urinary system. A 55-year-old woman presented with gross hematuria and right flank pain which had begun two months earlier. A well-marginated, low-density mass containing high-density portions representing hemorrhage was seen in the right kidney at pre-enhanced CT; contrast enhancement was not prominent. As both T1- and T2- weighted MR imaging, a multilocular cystic mass with high signal intensity portions representing hemorrhage was observed. Contrast enhancement was absent. We report the radiologic findings in the case of renal PNET.

  12. An Incidentaloma: Primitive Neuroectodermal Tumor of the Thymus

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    Suzanne M. Smith

    2011-01-01

    Full Text Available After presenting for a routine screening exam, and 57-year-old man was diagnosed with an incidentaloma—a primitive neuroectodermal tumor (PNET of the thymus. A member of the Ewing sarcoma family of tumors, a PNET is typically regarded as a malignancy of childhood and adolescence, usually occurring in the central nervous system. In the case at hand, our patient had an extremely unusual presentation, given his age and tumor location. Initial presentation is the only predictor for long-term survival. Current treatment recommendations advocate complete surgical resection whenever possible, radiation therapy, and adjuvant versus neoadjuvant chemotherapy.

  13. Primary intraspinal extradural primitive neuroectodermal tumor: A rare case

    Directory of Open Access Journals (Sweden)

    Shrikant V Rege

    2016-01-01

    Full Text Available Primitive neuroectodermal tumors (PNETs are aggressive childhood malignancies and are difficult to treat. Primary intraspinal PNETs are rare. These patients have poor prognosis with short survival time even after surgery and chemoradiation. As there are no standard guidelines exist for the management of these tumors, a multidisciplinary approach has been employed with varying success. According to the review of literature, only few cases of primary intraspinal extradural PNETs have been reported. Herein, author has described a case of intraspinal, extradural PNET.

  14. Primitive neuroectodermal tumor of lumbar spine: case report

    Institute of Scientific and Technical Information of China (English)

    HE Shi-sheng; ZHAO Jie; HAN Kai-wei; HOU Tie-sheng; Nazakat Hussain; ZHANG Shun-ming

    2007-01-01

    @@ Primitive neuroectodermal tumors (PNETs), derived from the primitive neural crest, are highly malignant and mainly exist in the central nervous system (CNS),chest wall, lower extremities, trunk, kidney, and orbit but rarely in the spine. Though multidisciplinary treatments have been well established as the standard therapy for intracranial PNETs, little is known about the optimal treatment strategy and therapeutic results for intraspinal PNETs. The following report illustrates the operative and non-operative management of an extradural PNET at the level of L5 in a young girl.

  15. Supratentorial primitive neuroectodermal tumor presenting with intracranial hemorrhage in adult

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    Bashar Abuzayed

    2014-01-01

    Full Text Available A 24-year-old female patient presented with complaints of nausea, vomiting and of loss of consciousness lasted for 15 minutes with left sided weakness. Neuroradiological evaluation revealed a hemorrhagic mass lesion in the right frontal lobe. The patient was operated and intraoperative findings showed a cortical-subcortical hematoma including hemorrhagic and disrupted tissue with a pathologic purple tissue on the periphery of the hematoma. Postoperative course was uneventful and postoperative histopathological examination revealed primitive neuroectodermal tumor. The patient was then referred to medical and radiation oncology clinics for further evaluation and treatment.

  16. Modeling RNA polymerase interaction in mitochondria of chordates

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    Lyubetsky Vassily A

    2012-08-01

    selected genes only relative RNA concentrations have been experimentally determined. Conversely, these characteristics and absolute transcription levels can be obtained using relative RNA concentrations and RNA half-lives known from various experimental studies. In this case, the “inverse problem” is solved with multi-objective optimization. Conclusions In this study, we demonstrate that our model accurately reproduces all relevant experimental data available for plant plastids, as well as the mitochondria of chordates. Using experimental data, the model is applied to estimate binding intensities of phage-type RNA polymerases to their promoters as well as predicting terminator characteristics, including polarization. In addition, one can predict characteristics of phage-type RNA polymerases and the transcription process that are difficult to measure directly, e.g., the association between the promoter’s nucleotide composition and the intensity of polymerase binding. To illustrate the application of our model in functional predictions, we propose a possible mechanism for MELAS syndrome development in human involving a decrease of Phe-tRNA, Val-tRNA and rRNA concentrations in the cell. In addition, we describe how changes in methylation patterns of the mTERF binding site and three promoters in hypothyroid rat correlate with changes in intensities of the mTERF binding and transcription initiations. Finally, we introduce an auxiliary model to describe the interaction between polysomal mRNA and ribonucleases.

  17. Peripheral primitive neuroectodermal tumors:a rare case report

    Institute of Scientific and Technical Information of China (English)

    Kun Yan; Yanyan Ge; Youhong Ding; Shiguang Yan; Xiangyang Li

    2014-01-01

    We-aimed-to-explore-the-diagnosis-and-treatment-of-peripheral-primitive-neuroectodermal-tumors-(pPNETs).-We-retrospectively-analyzed-the-diagnosis-and-treatment-process-of-a-patient-who-was-diagnosed-with-pPNETs-by-pathology.-This-case-was-a-man-with-soft-masses-arising-from-the-left-chest-wal-near-the-armpit-and-left-supraclavicular-of-a-47-year-old-man.-The-patient-mainly-presented-with-the-masses-which-increasing-gradual-y-with-obvious-pain.-Needle-biopsy-showed-that-they-were-both-metastatic-adenocarcinoma.-Ultrasonography-B-revealed-blood-flow-of-these-two-low-density-placeholders-can-be-seen-in-the-signal,-not-oppression-axil-ary-and-vein.-Radical-resection-of-the-masses-were-performed.-Histopathologic-study-and-immunohistochemistry-(IHC)-confirmed-the-masses-to-be-peripheral-primitive-neuroectodermal-tumors.-pPNETs-is-a-rare-malignant-smal-round-cel-tumor.-CT-and-MRI-examination-can-estimate-the-resectability-of-the-tumor;Ultrasound-B-can-make-sure-its-inside-blood-supply-and-the-positional-relationship-between-the-mass-and-the-surrounding-vasculature.-The-diagnosis-of-pPNETs-is-based-primarily-on-histopathologic-study-and-IHC,-especial-y-those-with-the-characteristics-of-the-Homer-Wright-and-neuroendocrine-markers.-Radical-resection-of-the-tumor-is-the-most-ef-ective-therapeutic-method.-The-ef-ect-of-adjuvant-chemo-radiation-is-worth-af-irmation.-Autologous-stem-cel-rescue-besides-adjuvant-chemotherapy-has-been-associated-with-prolonged-survival.

  18. Medulloblastomas and central nervous system primitive neuroectodermal tumors.

    Science.gov (United States)

    McLean, Thomas W

    2003-12-01

    Significant advances in the treatment of medulloblastoma and primitive neuroectodermal tumors have been made in the past three decades. Maximal surgical resection is a mainstay of therapy. However, unlike many other central nervous system neoplasms, medulloblastoma and primitive neuroectodermal tumors are radiation and chemotherapy responsive. Despite this response, the prognosis for patients with these tumors remains variable and is relatively poor in infants and patients with metastatic disease. These tumors most commonly arise in children, thus most clinical trials emphasize the reduction of long-term sequelae, in addition to improving survival. All newly diagnosed patients who are eligible should be offered participation in a clinical trial. If a patient is ineligible or declines consent/assent for a clinical trial, the best current treatment approach is surgical resection, followed by radiation therapy (except for children younger than 3 years) with weekly vincristine. For high-risk patients, 36 Gy of craniospinal irradiation should be delivered plus a boost of 19.8 Gy to the posterior fossa/primary tumor bed and sites of bulk metastatic disease. For average-risk patients, the craniospinal irradiation dose may be lowered to 23.4 Gy plus 32.4 Gy to the posterior fossa/tumor bed. After radiation therapy, intensive multimodal chemotherapy should be used for all patients.

  19. The Middle Cambrian fossil Pikaia and the evolution of chordate swimming

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    Lacalli Thurston

    2012-07-01

    Full Text Available Abstract Conway Morris and Caron (2012 have recently published an account of virtually all the available information on Pikaia gracilens, a well-known Cambrian fossil and supposed basal chordate, and propose on this basis some new ideas about Pikaia’s anatomy and evolutionary significance. Chief among its chordate-like features are the putative myomeres, a regular series of vertical bands that extends the length of the body. These differ from the myomeres of living chordates in that boundaries between them (the myosepta are gently curved, with minimal overlap, whereas amphioxus and vertebrates have strongly overlapping V- and W-shaped myomeres. The implication, on biomechanical grounds, is that myomeres in Pikaia exerted much less tension on the myosepta, so the animal would have been incapable of swimming as rapidly as living chordates operating in the fast-twitch mode used for escape and attack. Pikaia either lacked the fast-twitch fibers necessary for such speeds, having instead only slow-twitch fibers, or it had an ancestral fiber type with functional capabilities more like modern slow fibers than fast ones. The first option is supported by the sequence of development in zebrafish, where both myoseptum formation and fast fiber deployment show a dependence on slow fibers, which develop first. For Pikaia, the absence of fast fibers has both behavioral and anatomical implications, which are discussed. Among the latter is the possibility that a notochord may not have been needed as a primary stiffening device if other structures (for example, the dorsal organ could perform that role.

  20. AmphiFoxQ2, a novel winged helix/forkhead gene, exclusively marks the anterior end of the amphioxus embryo

    Science.gov (United States)

    Yu, Jr-Kai; Holland, Nicholas D.; Holland, Linda Z.

    2003-01-01

    A full-length FoxQ-related gene (AmphiFoxQ2) was isolated from amphioxus. Expression is first detectable in the animal/anterior hemisphere at the mid blastula stage. The midpoint of this expression domain coincides with the anterior pole of the embryo and is offset dorsally by about 20 degrees from the animal pole. During the gastrula stage, expression is limited to the anterior ectoderm. By the early neurula stage, expression remains in the anterior ectoderm and also appears in the adjacent anterior mesendoderm. By the early larval stages, expression is detectable in the anteriormost ectoderm and in the rostral tip of the notochord. AmphiFoxQ2 is never expressed anywhere except at the anterior tip of amphioxus embryos and larvae. This is the first gene known that exclusively marks the anterior pole of chordate embryos. It may, therefore, play an important role in establishing and/or maintaining the anterior/posterior axis.

  1. Primitive neuroectodermal tumor presenting as a delayed sequela to cranial irradiation and intrathecal methotrexate

    Energy Technology Data Exchange (ETDEWEB)

    Barasch, E.S.; Altieri, D.; Decker, R.E.; Ahmed, S.; Lin, J.

    1988-11-01

    A patient developed a primitive neuroectodermal tumor (PNET) many years after therapeutic cerebral radiation and methotrexate treatment for leukemia. The differential radiologic and histologic diagnoses, as well as the possible co-oncogenic effects of radiation and methotrexate, are evaluated.

  2. Primitive Neuroectodermal Tumor with Glioblastoma Multiforme Components in an Adult: A Collision Tumor.

    Science.gov (United States)

    Forbes, Victoria; Vredenburgh, James

    2016-01-11

    We report a rare case of a central nervous system collision tumor in a 40-year-old woman. Histopathological examination of her large temporal tumor revealed two different components making up the tumor tissue. The predominant component of the tumor was found to be a primitive neuroectodermal tumor. The other component was glioblastoma multiforme. Both of these tumors carry a poor prognosis, and primitive neuroectodermal tumors are extremely uncommon in adults. Central nervous system neoplasms with the combined features of both primitive neuroectodermal tumor and malignant glioma are very rare and represent a diagnostic and treatment predicament. The patient underwent surgical resection, radiation therapy, and chemotherapy targeting both the primitive neuroectodermal tumor and glioblastoma. Our patient has been fortunate in not showing any sign of recurrence and will celebrate the third anniversary since her diagnosis this January.

  3. Supratentorial primitive neuroectodermal tumor in an adult: a case report and review of the literature

    Directory of Open Access Journals (Sweden)

    Lawandy Shokry

    2012-10-01

    Full Text Available Abstract Introduction Supratentorial primitive neuroectodermal tumors predominantly occur in children, and are rare in the adult population. Less than 100 cases of supratentorial primitive neuroectodermal tumor have been reported in adults internationally. Our case study reports this rare incident. Case presentation A 22-year-old Hispanic man presented with headaches, blurry vision, diplopia, intermittent vomiting, and grossly decreased vision. A magnetic resonance image showed a left posterior parietal heterogeneously enhancing mass measuring 4.2cm × 7.2cm × 7.0cm. After craniotomy for resection and decompression, the mass was histologically revealed to be a supratentorial primitive neuroectodermal tumor. Standardized immunohistochemical studies for this mass were carried out. Conclusion We have concluded that immunohistochemical and genetic workup should be included in the standardized pathological workup for primitive neuroectodermal tumors in order to provide more prognostic information. Based on our current literature review, we propose an immunohistochemical panel.

  4. Melanotic neuroectodermal tumor of infancy: A rare case report

    Directory of Open Access Journals (Sweden)

    E Rajendra Reddy

    2013-01-01

    Full Text Available Melanotic neuroectodermal tumor of infancy (MNTI is a relatively uncommon osteolytic-pigmented neoplasm that primarily affects the jaws of infants. The early onset and its rapid disfiguring spread necessitate early diagnosis. A 4-month-old male child reported with the complaint of swelling in the right back tooth region of the upper jaw, which rapidly increased in size causing disfigurement of the face. Radiographic examination showed a diffuse osteolytic radiolucent lesion in the right maxilla and displacement and dysmorphic changes in the developing primary tooth buds. Wide surgical excision was performed under general anesthesia. Histopathological report revealed characteristic large pigmented epitheloid cells (melanocyte like cells. The biphasic tumor cell population arranged in a background of fibrous connective tissue stroma is suggestive of MNTI involving the cancellous bone. Early diagnosis and management of such aggressive tumors precludes significant morbidity of the patient.

  5. [Primitive neuroectodermal tumor of kidney : a case report].

    Science.gov (United States)

    Tsutsumi, Naofumi; Sumiyoshi, Takayuki; Okamura, Motohiro; Nakashima, Yoshiharu; Iwamura, Hiroshi; Mitsumori, Kenji; Nishimura, Kazuo; Shintaku, Masayuki; Koyama, Takashi; Matsui, Yusuke; Watanabe, Mitsumasa

    2013-06-01

    A 16-year-old man was referred to our hospital for asymptomatic gross hematuria. The findings of abdominal ultrasonography were normal. A month later, gross hematuria disappeared, and he was not followed after that. A month later, the patient was taken to our hospital in an ambulance for severe back pain and recurring gross hematuria. Computed tomography (CT) revealed a large right renal tumor with tumor thrombus penetrating inside the inferior vena cava. The patient underwent radical nephrectomy and embolectomy. The pathological diagnosis of the tumor was diagnosed as primitive neuroectodermal tumor (PNET) of kidney by immunostaining and gene analysis. We started adjuvant chemotherapy soon after the operation. However, at 10 months after, multiple pulmonary metastases were detected. The patient was treated with salvage chemotherapy, surgery and irradiation therapy as combined modality therapy. Nevertheless, he died 18 months after the diagnosis.

  6. Primitive Neuroectodermal Tumor of the Stomach: A Case Report.

    Science.gov (United States)

    Song, Min Jeong; An, Soyeon; Lee, Seung Soo; Kim, Beom Su; Kim, Jihun

    2016-09-01

    Ewing sarcoma/primitive neuroectodermal tumor (ES/PNET) is a highly aggressive small round cell tumor that mainly occurs in the bone or soft tissue of children or young adults but is extremely rare in the stomach. A 55-year-old man presented with melena and anemia. On endoscopy, an ulcerofungating mass was observed in the high body and total gastrectomy was performed. Histologically, the mass consisted of small round cells with scanty cytoplasm and inconspicuous nucleoli. They often formed perivascular pseudorosettes and multinucleated giant cells were frequently observed. The tumor cells strongly expressed CD99, FLI1, and chromogranin and weakly expressed synaptophysin and CD56. EWS-FLI1 fusion transcript was confirmed by reverse transcription-polymerase chain reaction. ES/PNET is frequently misdiagnosed because of its similarity with small cell carcinoma. Although gastric ES/PNET is very rare, it should be included in differential diagnoses of small round cell tumor in the stomach.

  7. Paravaginal peripheral primitive neuroectodermal tumor: A rare tumor

    Directory of Open Access Journals (Sweden)

    Samrat Dutta

    2011-01-01

    Full Text Available Peripheral primitive neuroectodermal tumor (PNET of the female genital tract, particularly those in the vaginal and paravaginal region, are extremely rare. A 36-year-old woman presented with clinical features similar to that of a case of cervical fibroid. It was only after surgery that the histopathology suggested it to be a malignant round cell tumour and was CD99 positive. She underwent adjuvant chemotherapy with the Ifosfamide and Etoposide alternating with Vincristine, Doxorubicin, and Cyclophosphamide regime and radical radiotherapy. She is disease free at 12 months of follow-up. The importance of immunostaining and adequate histopathology report lies in the fact that the correct diagnosis thus achieved enabled us to manage a rare case of paravaginal PNET with a multimodality approach.

  8. Heterogeneity of extraparenchymal primitive neuroectodermal tumors within the craniospinal axis.

    Directory of Open Access Journals (Sweden)

    M Debiec-Rychter

    2005-02-01

    Full Text Available Four cases of primitive neuroectodermal tumors (PNETs with unusual localization (three intraspinal extramedullary and one pontocerebellar are reviewed. Histologically, they were small round blue cell tumors with diverse patterns. Immunohistochemically, all tumors were positive for at least two neuronal markers, two cases were Mic-2 positive and one showed glial differentiation. The paraffin-embedded tumor specimens were examined by interphase FISH using dual-color probes specific for EWS, HER-2 and BCR loci. Molecular cytogenetic study revealed the presence of EWS rearrangement in two cases and the presence of i(17q in one tumor. Three tumors exhibited 22 disomy and one was 22 polyploid. Extraparenchymal PNETs within craniospinal axis are heterogeneous from the clinical, histological, immunohistochemical and molecular point of view. These PNETs can be of a central or peripheral type. Multidisciplinary approach is of a basic importance in differential diagnosis of such cases.

  9. Sequential antagonism of early and late Wnt-signaling by zebrafish colgate promotes dorsal and anterior fates.

    Science.gov (United States)

    Nambiar, Roopa M; Henion, Paul D

    2004-03-01

    The establishment of the vertebrate body plan involves patterning of the ectoderm, mesoderm, and endoderm along the dorsoventral and antero-posterior axes. Interactions among numerous signaling molecules from several multigene families, including Wnts, have been implicated in regulating these processes. Here we provide evidence that the zebrafish colgate(b382) (col) mutation results in increased Wnt signaling that leads to defects in dorsal and anterior development. col mutants display early defects in dorsoventral patterning manifested by a decrease in the expression of dorsal shield-specific markers and ectopic expression of ventrolaterally expressed genes during gastrulation. In addition to these early patterning defects, col mutants display a striking regional posteriorization within the neuroectoderm, resulting in a reduction in anterior fates and an expansion of posterior fates within the forebrain and midbrain-hindbrain regions. We are able to correlate these phenotypes to the overactivation of Wnt signaling in col mutants. The early dorsal and anterior patterning phenotypes of the col mutant embryos are selectively rescued by inactivation of Wnt8 function by morpholino translational interference. In contrast, the regionalized neuroectoderm posterioriorization phenotype is selectively rescued by morpholino-mediated inactivation of Wnt8b. These results suggest that col-mediated antagonism of early and late Wnt-signaling activity during gastrulation is normally required sequentially for both early dorsoventral patterning and the specification and patterning of regional fates within the anterior neuroectoderm.

  10. Adult thoracic primitive neuroectodermal tumor - clinical, radiological and pathological features; Tumor neuroectodermico primitivo toracico em adulto

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    Setubal, Roger; Santos, Leynalze Lins Ramos; Georges, Silvana Habib; Souza, Ricardo Pires de [Hospital Heliopolis, Sao Paulo, SP (Brazil). Servico de Diagnostico por Imagem; Menezes, Yara de [Hospital heliopolis, Sao Paulo, SP (Brazil). Servico de Patologia; Gomes, Marcio Rogerio Alcala; Mayo, Suzete Varela; Leiro, Luis Carlos Filgueira [Hospital Heliopolis, Sao Paulo, SP (Brazil). Servico de Pneumologia e Cirurgia Toracica

    1997-03-01

    Precise diagnosis of small round cell tumors, which include the thoracic primitive neuroectodermal tumor, is often a challenge to the clinical, oncologist, radiologist and pathologist. Different terms have been used to designate these tumors, depending on their location and extent of neural differentiation. With the progress of immuno-histochemical and cytogenetic diagnosis method, some of this tumors are now classified a unique clinical entity with several locations and mode of presentation. Therefore, tumors-like peripheral neuro epithelioma, Askin`s tumor, adult neuroblastoma, and primitive neuroectodermal tumor are now named peripheral primitive neuroectodermal tumor. The most common location of peripheral primitive neuroectodermal tumor is the thorax, and the mean age are close of the adolescence. This study reports the clinical, radiological and pathological features of 5 adults (3 men and 2 women) seen at the Hospital Heliopolis, Sao Paulo, SP Brazil, between 1991 and 1995 (5 years), with diagnosis of peripheral primitive neuroectodermal tumor made by immuno-histochemical and pathological studies. The mean age of the patients was 34 years, ranged between 17 and 57 years, and the most common location was the chest wall (n=3), followed by the posterior mediastinum (n=2). All the patients were symptomatic and undergone plain films and computed tomography. (author) 12 refs., 5 figs.

  11. CT and MR findings of primitive neuroectodermal tumor

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    Kook, Shin Ho; Kim, In One; Chang, Kee Hyun; Han, Moon Hee; Cho, Byung Kyu [Seoul National University Collge of Medicine, Seoul (Korea, Republic of)

    1991-07-15

    Cerebral primitive neuroectodermal tumor (PNET), consisting of undifferentiated primitive cells, is a neoplasm of children and young adults that occurs predominantly in the supratentorial compartment. In this report, we retrospectively reviewed and analyzed 18 CT and 6 MR findings in 18 patients with pathologically-proven PNET to discover the characteristic findings, if may. The most characteristic feature of the PNETs was a well-defined multilobular oval or round large mass with components of peripheral cystic change or calcification in the cerebral hemisphere, especially in the parietal lobe. Usually there was only minimal surrounding edema. The CT density of the tumor was iso - or slightly high density with homogeneous contrast enhancement in the solid portion and low density in the cystic area. MR findings demonstrated iso - signal intensity on both T1 - weighted (T1WI) and T2-weighted (T2WI) images and dense enhancement in the solid element. The cystic portion revealed low intensity on T1WI and high intensity on T2WI.

  12. Primitive neuroectodermal tumor of adrenal: clinical presentation and outcomes.

    Science.gov (United States)

    Dutta, Deep; Shivaprasad, K S; Das, Ram Narayan; Ghosh, Sujoy; Chowdhury, Subhankar

    2013-01-01

    Primitive neuroectodermal tumor (PNET) of adrenal is an extremely rare tumor of neural crest origin. A nonfunctional left adrenal mass (14.6 × 10.5 × 10.0 cm) on computed tomography (CT) was detected in a 40-year-old lady with abdominal pain, swelling, and left pleural effusion. She underwent left adrenalectomy and left nephrectomy with retroperitoneal resection. Histopathology revealed sheets and nest of oval tumor cells with hyperchromatic nuclei, prominent nucleoli, scanty cytoplasm, brisk mitotic activity, necrosis, lymphovascular invasion, capsular invasion, and extension to the surrounding muscles; staining positive for Mic-2 (CD-99 antigen), vimentin, synaptophysin, and Melan-A. Thoracocentesis, pleural fluid study, and pleural biopsy did not show metastasis. She responded well to vincristine, adriamycin, and cyclophosphamide followed by ifosfamide and etoposide (IE). This is the first report of adrenal peripheral PNET (pPNET) from India. This report intends to highlight that pPNET should be suspected in a patient presenting with huge nonfunctional adrenal mass which may be confused with adrenocortical carcinoma.

  13. Primitive Neuroectodermal Tumor of the Liver: A Case Report

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    Eduardo Cambruzzi

    2011-01-01

    Full Text Available Primary liver sarcomas represent a rare group of neoplasias, with angiosarcoma being the most common histological type. Primitive neuroectodermal tumor (PNET represents a high malignant neoplasia that usually affects the central nervous system and soft tissues. An 18-year-old male patient was admitted with clinical complains of pain in the right upper abdominal quadrant. The clinical evaluation revealed a solid mass in the right hepatic lobe. On the gross examination of the resected liver specimen, the right lobe of the liver was replaced by a yellow-red solid mass measuring 21 cm in its largest dimension. On the histopathology, a tumor composed of small round blue cells with little cytoplasm and round nuclei was identified. The lesion revealed positive immunoexpression for vimentin and CD99 and negative immunostaining for desmin, CD45, cytokeratin, and neuroblastoma protein, suggesting, then, the diagnosis of PNET. Although it is an unusual tumor, it should be considered in the differential diagnosis of liver masses, especially in young patients.

  14. Primary Ewing Sarcoma/Primitive Neuroectodermal Tumor of the Stomach

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    Safi Khuri

    2016-11-01

    Full Text Available Ewing sarcoma/primitive neuroectodermal tumor (ES/PNET is a tumor of small round cells arising in skeletal tissues. These tumors rarely arise in the stomach. We present a 31-year-old healthy female patient who was admitted to our surgical ward due to upper gastrointestinal hemorrhage. Upper endoscopy revealed a large ulcerated bleeding mass originating from the lesser curvature. Biopsy revealed tumor cell immunoreactivity positive for CD99, vimentin, and Ki67 (an index of proliferation. These findings were compatible with gastric ES/PNET. The fluorescence in situ hybridization analysis result for the EWSR1 gene rearrangement (11: 22 translocation was positive. The patient refused neoadjuvant treatment and thus underwent an operation during which a mass at the lesser curvature of the stomach was found. The mass was adhering to the pancreatic tail and to the mesentery of the transverse and descending colon. Total gastrectomy, distal pancreatectomy, splenectomy, and left adrenalectomy were done. The patient refused adjuvant treatment. She is free of disease 3 years after surgery.

  15. Primitive neuroectodermal tumor of adrenal: Clinical presentation and outcomes

    Directory of Open Access Journals (Sweden)

    Deep Dutta

    2013-01-01

    Full Text Available Primitive neuroectodermal tumor (PNET of adrenal is an extremely rare tumor of neural crest origin. A nonfunctional left adrenal mass (14.6 × 10.5 × 10.0 cm on computed tomography (CT was detected in a 40-year-old lady with abdominal pain, swelling, and left pleural effusion. She underwent left adrenalectomy and left nephrectomy with retroperitoneal resection. Histopathology revealed sheets and nest of oval tumor cells with hyperchromatic nuclei, prominent nucleoli, scanty cytoplasm, brisk mitotic activity, necrosis, lymphovascular invasion, capsular invasion, and extension to the surrounding muscles; staining positive for Mic-2 (CD-99 antigen, vimentin, synaptophysin, and Melan-A. Thoracocentesis, pleural fluid study, and pleural biopsy did not show metastasis. She responded well to vincristine, adriamycin, and cyclophosphamide followed by ifosfamide and etoposide (IE. This is the first report of adrenal peripheral PNET (pPNET from India. This report intends to highlight that pPNET should be suspected in a patient presenting with huge nonfunctional adrenal mass which may be confused with adrenocortical carcinoma.

  16. Primary Ewing Sarcoma/Primitive Neuroectodermal Tumor of the Stomach

    Science.gov (United States)

    Khuri, Safi; Gilshtein, Hayim; Sayidaa, Sa'd; Bishara, Bishara; Kluger, Yoram

    2016-01-01

    Ewing sarcoma/primitive neuroectodermal tumor (ES/PNET) is a tumor of small round cells arising in skeletal tissues. These tumors rarely arise in the stomach. We present a 31-year-old healthy female patient who was admitted to our surgical ward due to upper gastrointestinal hemorrhage. Upper endoscopy revealed a large ulcerated bleeding mass originating from the lesser curvature. Biopsy revealed tumor cell immunoreactivity positive for CD99, vimentin, and Ki67 (an index of proliferation). These findings were compatible with gastric ES/PNET. The fluorescence in situ hybridization analysis result for the EWSR1 gene rearrangement (11: 22 translocation) was positive. The patient refused neoadjuvant treatment and thus underwent an operation during which a mass at the lesser curvature of the stomach was found. The mass was adhering to the pancreatic tail and to the mesentery of the transverse and descending colon. Total gastrectomy, distal pancreatectomy, splenectomy, and left adrenalectomy were done. The patient refused adjuvant treatment. She is free of disease 3 years after surgery. PMID:27920700

  17. [Case of primitive neuroectodermal tumor of the kidney].

    Science.gov (United States)

    Ueno, Yoko; Ishikawa, Masakuni; Tsuruta, Takashi; Wajiki, Masahisa

    2008-03-01

    A 28-year-old woman presented with right flank pain. A large, firm, fixed mass was palpable in the right side of the abdomen. Computed tomography revealed a solid mass of the right kidney with extension into the renal vein and inferior vena cava. The patient underwent right radical nephrectomy with en bloc resection of the inferior vena cava containing tumor thrombus and right adrenalectomy. Histologically the tumor consisted of small tumor cells with rosette formation. Immunohistochemical staining was positive for CD99 and NSE. Analysis with polymerase chain reaction (PCR) demonstrated the EWS/FLI1 fusion products resulting from a chromosomal translocation. These findings were consistent with primary renal primitive neuroectodermal tumor (PNET). Two months after surgery, multiple lung, liver and lymph node metastases were found. The patient received 2 cycles of chemotherapy with cisplatin, ifosfamide, etoposide, resulting in a partial remission. She subsequently received 1 cycle chemotherapy with paclitaxel and carboplatin, resulting in no response. The metastatic lung and liver diseases progressed and she died 5 months after diagnosis.

  18. Melanotic neuroectodermal tumour of infancy: CT and MR findings

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    Haque, Saira; Sebire, Neil; McHugh, Kieran [Great Ormond Street Hospital, Department of Radiology, London (United Kingdom); McCarville, Mary Beth [St. Jude Children' s Research Hospital, Memphis, TN (United States)

    2012-06-15

    Melanotic neuroectodermal tumour of infancy (MNTI) is a rare neoplasm of neural crest origin. To describe three further cases of MNTI, with emphasis on CT and MRI findings. Data for children with histologically confirmed MNTI following biopsy or surgery were retrieved. Three children with available imaging at the time of diagnosis were included in the study. All three children had primary tumour in the head and neck region: one in the maxilla, one in the occipital bone (extra-axial but with intracranial extension) and one with an unusual tumour growing exophytically from the subcutaneous tissues adjacent to the occipital bone. All tumours were iso/hypointense both on T1- and T2-weighted MRI, and showed marked contrast enhancement in their non-ossified components. CT allowed identification of bone destruction and remodelling. Our findings are consistent with previously reported cases of MNTI regarding age at presentation and location in the head and neck region. Our MR findings did not demonstrate the typical pattern of T1-shortening expected from melanin deposition. (orig.)

  19. Malignant neuroectodermal tumor with melanocytic and rhabdomyoblastic differentiation

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    Brian P. Rubin

    2009-12-01

    Full Text Available Malignant melanoma can metastasize widely and vary significantly in its histological appearance; it rarely presents as a deep-seated mass without an obvious primary site elsewhere. Malignant peripheral nerve sheath tumor (MPNST is a high-grade sarcoma characterized by conventional and epithelioid subtypes. MPNST can demonstrate heterologous differentiation, usually in the form of osteosarcomatous, chondrosarcomatous, or rhabdo-myosarcomatous differentiation. MPNST does not harbor true melanocytic differentiation, although epithelioid MPNST typically is diffusely S-100 protein positive and superficially can resemble malignant melanoma. An unusual intra-abdominal mass was recently encountered with features of both melanoma and conventional or epithelioid MPNST containing a fascicular spindle cell component, an epithelioid component with melanocytic differentiation, as well as a rhabdomyosarcomatous component. The terminology “malignant neuroectodermal tumor with melanocytic and rhabdomyoblastic differentiation” is proposed to describe this neoplasm, reflecting the unusual concomittant lines of differentiation as well as offering a possible rationale for nosologically challenging aspects of this neoplasm.

  20. Primitive Neuroectodermal Tumor (PNET of the kidney: a case report

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    Selli Cesare

    2004-01-01

    Full Text Available Abstract Background A case of Primitive Neuroectodermal Tumor (PNET of the kidney in a 27-year-old woman is presented. Few cases are reported in the literature with a variable, nonspecific presentation and an aggressive behaviour. In our case, a radical nephrectomy with lymphadenectomy was performed and there was no residual or recurrent tumour at 24-month follow-up. Methods The surgical specimens were formalin-fixed and paraffin embedded. The sections were stained with routinary H&E. Immunohistochemistry was performed. Results The immunohistochemical evaluation revealed a diffuse CD99 positivity in the cytoplasm of the neoplastic cells. Pankeratin, cytokeratin AE1/AE3, vimentin, desmin, S100, cromogranin were negative. The clinical presentation and the macroscopic aspect, together with the histological pattern, the cytological characteristic and the cellular immunophenotype addressed the diagnosis towards primary PNET of kidney. Conclusions Since sometimes it is difficult to discriminate between PNET and Ewing's tumour, we reviewed the difficulties in differential diagnosis. These tumors have a common precursor but the stage of differentiation in which it is blocked is probably different. This could also explain their different biological behaviour and prognosis.

  1. SOXE neofunctionalization and elaboration of the neural crest during chordate evolution

    Science.gov (United States)

    Tai, Andrew; Cheung, Martin; Huang, Yong-Heng; Jauch, Ralf; Bronner, Marianne E.; Cheah, Kathryn S. E.

    2016-01-01

    During chordate evolution, two genome-wide duplications facilitated acquisition of vertebrate traits, including emergence of neural crest cells (NCCs), in which neofunctionalization of the duplicated genes are thought to have facilitated development of craniofacial structures and the peripheral nervous system. How these duplicated genes evolve and acquire the ability to specify NC and their derivatives are largely unknown. Vertebrate SoxE paralogues, most notably Sox9/10, are essential for NC induction, delamination and lineage specification. In contrast, the basal chordate, amphioxus, has a single SoxE gene and lacks NC-like cells. Here, we test the hypothesis that duplication and divergence of an ancestral SoxE gene may have facilitated elaboration of NC lineages. By using an in vivo expression assay to compare effects of AmphiSoxE and vertebrate Sox9 on NC development, we demonstrate that all SOXE proteins possess similar DNA binding and homodimerization properties and can induce NCCs. However, AmphiSOXE is less efficient than SOX9 in transactivation activity and in the ability to preferentially promote glial over neuronal fate, a difference that lies within the combined properties of amino terminal and transactivation domains. We propose that acquisition of AmphiSoxE expression in the neural plate border led to NCC emergence while duplication and divergence produced advantageous mutations in vertebrate homologues, promoting elaboration of NC traits. PMID:27734831

  2. Ontology for the asexual development and anatomy of the colonial chordate Botryllus schlosseri.

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    Lucia Manni

    Full Text Available Ontologies provide an important resource to integrate information. For developmental biology and comparative anatomy studies, ontologies of a species are used to formalize and annotate data that are related to anatomical structures, their lineage and timing of development. Here, we have constructed the first ontology for anatomy and asexual development (blastogenesis of a bilaterian, the colonial tunicate Botryllus schlosseri. Tunicates, like Botryllus schlosseri, are non-vertebrates and the only chordate taxon species that reproduce both sexually and asexually. Their tadpole larval stage possesses structures characteristic of all chordates, i.e. a notochord, a dorsal neural tube, and gill slits. Larvae settle and metamorphose into individuals that are either solitary or colonial. The latter reproduce both sexually and asexually and these two reproductive modes lead to essentially the same adult body plan. The Botryllus schlosseri Ontology of Development and Anatomy (BODA will facilitate the comparison between both types of development. BODA uses the rules defined by the Open Biomedical Ontologies Foundry. It is based on studies that investigate the anatomy, blastogenesis and regeneration of this organism. BODA features allow the users to easily search and identify anatomical structures in the colony, to define the developmental stage, and to follow the morphogenetic events of a tissue and/or organ of interest throughout asexual development. We invite the scientific community to use this resource as a reference for the anatomy and developmental ontology of B. schlosseri and encourage recommendations for updates and improvements.

  3. A conserved non-reproductive GnRH system in chordates.

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    Takehiro G Kusakabe

    Full Text Available Gonadotropin-releasing hormone (GnRH is a neuroendocrine peptide that plays a central role in the vertebrate hypothalamo-pituitary axis. The roles of GnRH in the control of vertebrate reproductive functions have been established, while its non-reproductive function has been suggested but less well understood. Here we show that the tunicate Ciona intestinalis has in its non-reproductive larval stage a prominent GnRH system spanning the entire length of the nervous system. Tunicate GnRH receptors are phylogenetically closest to vertebrate GnRH receptors, yet functional analysis of the receptors revealed that these simple chordates have evolved a unique GnRH system with multiple ligands and receptor heterodimerization enabling complex regulation. One of the gnrh genes is conspicuously expressed in the motor ganglion and nerve cord, which are homologous structures to the hindbrain and spinal cord of vertebrates. Correspondingly, GnRH receptor genes were found to be expressed in the tail muscle and notochord of embryos, both of which are phylotypic axial structures along the nerve cord. Our findings suggest a novel non-reproductive role of GnRH in tunicates. Furthermore, we present evidence that GnRH-producing cells are present in the hindbrain and spinal cord of the medaka, Oryzias latipes, thereby suggesting the deep evolutionary origin of a non-reproductive GnRH system in chordates.

  4. Evolutionary history of chordate PAX genes: dynamics of change in a complex gene family.

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    Vanessa Rodrigues Paixão-Côrtes

    Full Text Available Paired box (PAX genes are transcription factors that play important roles in embryonic development. Although the PAX gene family occurs in animals only, it is widely distributed. Among the vertebrates, its 9 genes appear to be the product of complete duplication of an original set of 4 genes, followed by an additional partial duplication. Although some studies of PAX genes have been conducted, no comprehensive survey of these genes across the entire taxonomic unit has yet been attempted. In this study, we conducted a detailed comparison of PAX sequences from 188 chordates, which revealed restricted variation. The absence of PAX4 and PAX8 among some species of reptiles and birds was notable; however, all 9 genes were present in all 74 mammalian genomes investigated. A search for signatures of selection indicated that all genes are subject to purifying selection, with a possible constraint relaxation in PAX4, PAX7, and PAX8. This result indicates asymmetric evolution of PAX family genes, which can be associated with the emergence of adaptive novelties in the chordate evolutionary trajectory.

  5. Ovarian malignant mixed mullerian tumor with primitive neuroectodermal differentiation: case report with review of the literature.

    Science.gov (United States)

    Nasser, Haitham; Morris, Robert T; Fathallah, Lamia

    2011-03-15

    Ovarian malignant mixed mullarian tumor (OMMMT) is a rare and aggressive tumor of the female genital tract, occurring mainly in elderly women. Stage of disease is the most important predictor for survival with no prognostic effect, yet, of heterologous elements. Rare case reports described the peculiar presence of primitive neuroectodermal tissue among other heterologous elements in these tumors. Attractive designations, such as teratoid carcinosarcoma, were set by some authors to describe this subset of lesions, where it was considered a primary neuroectodermal tumor capable of multilineage differentiation. We here report a case of OMMMT in an elderly woman with focal primitive neuroectodermal differentiation as the sole heterologous element, and review the controversy on this topic in the literature.

  6. Spontaneous cerebellar primitive neuroectodermal tumor in a juvenile cynomolgus monkey (Macaca fascicularis).

    Science.gov (United States)

    Mukaratirwa, Sydney; Rogerson, Petrina; Blanco, Ana L; Naylor, Stuart W; Bradley, Alys

    2012-08-01

    A neoplastic mass compressing the left cerebellar hemisphere and hindbrain was observed at trimming in a 3½-year-old male cynomolgus monkey from a control dose group. Microscopically, the neoplastic mass was nonencapsulated, invasive, and showed two morphological patterns. The predominant area consisted of densely packed undifferentiated, polygonal to spindle cells arranged in vague sheets supported by a scant fibrovascular stroma. The other area was less cellular and composed of round neoplastic cells separated by eosinophilic fibrillar material. Immunohistochemical staining for vimentin, synaptophysin, glial fibrillary acidic protein, neuron-specific enolase, neurofilament, and S-100 confirmed the presence of primitive undifferentiated neuroectodermal cells and some cells with neuronal or glial differentiation. On the basis of histopathology and immunohistochemical findings, a diagnosis of cerebellar primitive neuroectodermal tumor with neuronal and glial differentiation was made. Primitive neuroectodermal tumors are rare in animals including nonhuman primates; this is the first published report in this species.

  7. Malignant mediastinal neuroectodermal tumor. Case report; Tumeur neuroectodermique mediastinale. A propos d`un cas

    Energy Technology Data Exchange (ETDEWEB)

    Leconte, I.; Petit, E.; Sauvaget, J.; Rebischung, J.L.; Kleinmann, P.; Marichez, M. [Hopital Saint-Joseph, 75 - Paris (France)

    1997-09-01

    Peripheral malignant neuroectodermal tumors are rare and aggressive small-cell tumors seen predominantly in children and young adults. Mediastinal location is very uncommon. A Mediastinal primitive neuroectodermal tumor was diagnosed in a 27-year-old man. Despite treatment combining surgery, radiotherapy and chemotherapy, disease recurred locally along with lungs, spinal and epidural metastases, leading to death. CT and MRI examinations show heterogenous masses with necrosis, hemorrhage and intense enhancement after enhancement after Gadolinium injection. The CT and MRI findings are not specific and the diagnosis is based on pathologic, immunohistochemical and electron microscopic features. Although uncommon, peripheral neuroectodermal tumor should be considered in the differential diagnosis of posterior mediastinal masses in children and young adults. (authors).

  8. Primitive neuroectodermal tumors in the posterior fossa: excluding medulloblastoma based on pathology

    Energy Technology Data Exchange (ETDEWEB)

    Suh, Kyung Jin; Kim, Tae Hun; Kim, Yong Joo; Kang, Duk Sik; Shon, Youn Kyung [College of Medicine, Kyungpook National University, Daegu (Korea, Republic of); Kim, Sun Young [College of Medicine, Youngnam University, Daegu (Korea, Republic of)

    1992-01-15

    Four children, with histopathologically confirmed posterior fossa primitive neuroectodermal tumors, were examined by plain radiography, computed tomography (CT) and cerebral angiography. The homogeneously well enhanced solid mass in the midline of the posterior fossa and hydrocephalus of various degree were seen on all CT scans. One case had calcifications and another case had low density areas in the tumor mass. Three cerebral angiograms showed vascular displacement without tumor vascularities. Unfortunately, these CT and angiography findings are not specific for primitive neuroectodermal tumors, as similar findings are seen in medulloblastoma, ependymoma and other tumors. Therefore, we think that the primitive neuroectodermal tumors should be included in the differential diagnosis when a well enhanced solid mass in the midline posterior fossa is seen on CT scan in children.

  9. Multimodality treatment approach in management of primary peripheral primitive neuroectodermal tumor of the orbit

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    Kim Usha

    2009-01-01

    Full Text Available Primitive neuroectodermal tumor is a small round cell malignancy which rarely involves the orbit. We report a case of a two-year old male child presenting as unilateral eccentric proptosis with extraconal and intraconal mass, diagnosed as primary peripheral primitive neuroectodermal tumor (pPPNET on histopathology and immunohistochemistry. There is no defined consensus in the management of these tumors due to its rare presentation. We describe its distinguishing features with emphasis on multimodal and aggressive treatment approach which ensures appropriate management of these cases.

  10. Diverse ETS transcription factors mediate FGF signaling in the Ciona anterior neural plate.

    Science.gov (United States)

    Gainous, T Blair; Wagner, Eileen; Levine, Michael

    2015-03-15

    The ascidian Ciona intestinalis is a marine invertebrate belonging to the sister group of the vertebrates, the tunicates. Its compact genome and simple, experimentally tractable embryos make Ciona well-suited for the study of cell-fate specification in chordates. Tunicate larvae possess a characteristic chordate body plan, and many developmental pathways are conserved between tunicates and vertebrates. Previous studies have shown that FGF signals are essential for neural induction and patterning at sequential steps of Ciona embryogenesis. Here we show that two different ETS family transcription factors, Ets1/2 and Elk1/3/4, have partially redundant activities in the anterior neural plate of gastrulating embryos. Whereas Ets1/2 promotes pigment cell formation in lateral lineages, both Ets1/2 and Elk1/3/4 are involved in the activation of Myt1L in medial lineages and the restriction of Six3/6 expression to the anterior-most regions of the neural tube. We also provide evidence that photoreceptor cells arise from posterior regions of the presumptive sensory vesicle, and do not depend on FGF signaling. Cells previously identified as photoreceptor progenitors instead form ependymal cells and neurons of the larval brain. Our results extend recent findings on FGF-dependent patterning of anterior-posterior compartments in the Ciona central nervous system.

  11. Localization of CiCBR in the invertebrate chordate Ciona intestinalis: evidence of an ancient role for cannabinoid receptors as axonal regulators of neuronal signalling.

    Science.gov (United States)

    Egertová, Michaela; Elphick, Maurice R

    2007-06-01

    CiCBR is a G-protein-coupled receptor in the sea-squirt Ciona intestinalis and the first ortholog of vertebrate CB(1) and CB(2) cannabinoid receptors to be identified in an invertebrate (Elphick et al. [2003] Gene 302:95-101). Here we have used Western blotting and immunocytochemistry to examine expression of CiCBR in adult Ciona, employing novel antibodies to the C-terminal tail of CiCBR. Consistent with the expected mass for CiCBR, a approximately 47-kDa band was detected in Ciona membranes, and immunocytochemical analysis of serial sections of Ciona revealed intense immunoreactivity in the cerebral ganglion localised in a dense meshwork of fibers in the neuropile. Accordingly, Western blot analysis of neural complex homogenates revealed the presence of a approximately 47-kDa band. CiCBR immunoreactivity was also observed in axons exiting the ganglion in the anterior and posterior nerves, and analysis of whole-mount preparations revealed that these axons project over the interior surface of the oral and atrial siphons. Isolated CiCBR-immunoreactive axons not associated with the anterior and posterior nerves were observed projecting through the cortical layer of the cerebral ganglion. Central and peripheral CiCBR-immunoreactive fibers were studded with intensely stained varicosities, indicative of a role for CiCBR in regulation of axonal release of neurotransmitters, neuromodulators, or neurohormones. Collectively, our data suggest that the well-established role that the CB(1) receptor has as an axonal regulator of neurotransmitter release in mammals may have originated with ancestral-type cannabinoid receptors in invertebrate chordates before the emergence of CB(1)- and CB(2)-type receptors in vertebrates.

  12. Tunicate mitogenomics and phylogenetics: peculiarities of the Herdmania momus mitochondrial genome and support for the new chordate phylogeny

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    Loya Yossi

    2009-11-01

    Full Text Available Abstract Background Tunicates represent a key metazoan group as the sister-group of vertebrates within chordates. The six complete mitochondrial genomes available so far for tunicates have revealed distinctive features. Extensive gene rearrangements and particularly high evolutionary rates have been evidenced with regard to other chordates. This peculiar evolutionary dynamics has hampered the reconstruction of tunicate phylogenetic relationships within chordates based on mitogenomic data. Results In order to further understand the atypical evolutionary dynamics of the mitochondrial genome of tunicates, we determined the complete sequence of the solitary ascidian Herdmania momus. This genome from a stolidobranch ascidian presents the typical tunicate gene content with 13 protein-coding genes, 2 rRNAs and 24 tRNAs which are all encoded on the same strand. However, it also presents a novel gene arrangement, highlighting the extreme plasticity of gene order observed in tunicate mitochondrial genomes. Probabilistic phylogenetic inferences were conducted on the concatenation of the 13 mitochondrial protein-coding genes from representatives of major metazoan phyla. We show that whereas standard homogeneous amino acid models support an artefactual sister position of tunicates relative to all other bilaterians, the CAT and CAT+BP site- and time-heterogeneous mixture models place tunicates as the sister-group of vertebrates within monophyletic chordates. Moreover, the reference phylogeny indicates that tunicate mitochondrial genomes have experienced a drastic acceleration in their evolutionary rate that equally affects protein-coding and ribosomal-RNA genes. Conclusion This is the first mitogenomic study supporting the new chordate phylogeny revealed by recent phylogenomic analyses. It illustrates the beneficial effects of an increased taxon sampling coupled with the use of more realistic amino acid substitution models for the reconstruction of animal

  13. The MRI findings of meningeal peripheral-type primitive neuroectodermal tumors

    Institute of Scientific and Technical Information of China (English)

    李文一

    2014-01-01

    Objective To analyze the MRI features of Ewing sarcoma/peripheral primitive neuroectodermal tumors(pPNETs)arising from the meninges.Methods The MRI imaging of 9 patients with Ewing sarcoma/pPNET were reviewed retrospectively,and imaging features and pathological characteristics were analyzed.Results The age of most patients ranged from 10 to 20

  14. Primary primitive neuroectodermal tumor: An unusual cause of right ventricular intracavitary obstruction in a child

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    Thachil Ajit

    2008-01-01

    Full Text Available A six-year-old boy presented with a brief history suggestive of right heart failure. Investigations revealed a mass filling almost the entire right ventricle. Palliative resection of the mass was done. The operative specimen revealed a primary primitive neuroectodermal tumor of the heart, the first of its kind reported in the pediatric age group .

  15. Pediatric Primitive Neuroectodermal Tumors of the Central Nervous System Differentially Express Granzyme Inhibitors

    NARCIS (Netherlands)

    Vermeulen, Jeroen F; van Hecke, Wim; Spliet, Wim G M; Villacorta Hidalgo, José; Fisch, Paul; Broekhuizen, Roel; Bovenschen, Niels

    2016-01-01

    BACKGROUND: Central nervous system (CNS) primitive neuroectodermal tumors (PNETs) are malignant primary brain tumors that occur in young infants. Using current standard therapy, up to 80% of the children still dies from recurrent disease. Cellular immunotherapy might be key to improve overall surviv

  16. Primitive neuro-ectodermal tumor of the lung in an adult

    Directory of Open Access Journals (Sweden)

    Gaude G

    2009-01-01

    Full Text Available A rare case of a thoracic primitive neuro-ectodermal tumor in an adult is presented here. In this case, wide excision surgical excision followed by chemotherapy and radiotherapy were delivered. But due to the rapid aggressive progression of the tumor, which is the characteristic of disease, the patient died within four months after the diagnosis.

  17. Identification of novel biomarkers in pediatric primitive neuroectodermal tumors and ependymomas by proteome-wide analysis

    NARCIS (Netherlands)

    de Bont, Judith M.; den Boer, Monique L.; Kros, Johan M.; Passier, Monique M. C. J.; Reddinglus, Roel E.; Smitt, Peter A. E. Sillevis; Luider, Theo M.; Pieters, Rob

    2007-01-01

    The aim of this study was to identify aberrantly expressed proteins in pediatric primitive neuroectodermal tumors (PNETs) and ependymornas. Tumor tissue of 29 PNET and 12 ependymoma patients was subjected to 2-dimensional difference gel electrophoresis. Gel analysis resulted in 79 protein spots bein

  18. Primitive Neuroectodermal Tumors of Hand and Foot: Report of Two Cases

    OpenAIRE

    DEMİR, Pelin; Arikan, Murat; ÇİLEDAĞ, Nazan; AKTAŞ, ELIF; Güngör, Şafak; Arda, Kemal

    2009-01-01

    Primitive neuroectodermal tumor (PNET) of hand and foot is extremely rare. To our knowiedge, limited number of cases of typicat PNET has been reported, involving the hand orfoot. İn this report, we presented magnetic resonance imaging findings of a PNET case localized in the proximal phalanx of left foot and a case localized in distal phalanx of the right hand.

  19. Melanotic neuroectodermal tumor of infancy: Presentation of a case affecting the maxilla

    Directory of Open Access Journals (Sweden)

    Agarwal Pooja

    2010-01-01

    Full Text Available Melanotic neuroectodermal tumor of infancy is a rare, distinctive neoplasm of early infancy with rapid expansile growth and a high rate of recurrence. Most commonly, the lesion affects the maxilla of infants during the first year of life. One such case was diagnosed in the Department of Oral Pathology and Microbiology in Subharti Dental College, Meerut.

  20. Gut-spilling in chordates: evisceration in the tropical ascidian Polycarpa mytiligera.

    Science.gov (United States)

    Shenkar, Noa; Gordon, Tal

    2015-04-16

    The ejection of internal organs, i.e., evisceration, is a well-known phenomenon in sea-cucumbers. We report the ability of a member of the Chordate phyla, the tropical ascidian Polycarpa mytiligera, to eviscerate and regenerate its gut within 12 days, and to rebuild its branchial sac within 19 days. Evisceration occurred within 4-43 seconds of gentle mechanical pressure exerted on the tunic in 47% of the tested P. mytiligera. Individuals were able to discard up to 3/4 of their digestive tract via the incurrent siphon by rupture of the branchial sac in this area. Although chemical analysis revealed no significant levels of toxic compounds, the eviscerated guts were unpalatable to the triggerfish and pufferfish on which they were tested, suggesting evisceration as a defense mechanism. Given the close affinity of ascidians to vertebrates, the regeneration pathway of the viscera and branchial sac of ascidians suggests its potential beneficial application in soft tissue regeneration research.

  1. A glycine receptor is involved in the organization of swimming movements in an invertebrate chordate

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    Okamura Yasushi

    2010-01-01

    Full Text Available Abstract Background Rhythmic motor patterns for locomotion in vertebrates are generated in spinal cord neural networks known as spinal Central Pattern Generators (CPGs. A key element in pattern generation is the role of glycinergic synaptic transmission by interneurons that cross the cord midline and inhibit contralaterally-located excitatory neurons. The glycinergic inhibitory drive permits alternating and precisely timed motor output during locomotion such as walking or swimming. To understand better the evolution of this system we examined the physiology of the neural network controlling swimming in an invertebrate chordate relative of vertebrates, the ascidian larva Ciona intestinalis. Results A reduced preparation of the larva consisting of nerve cord and motor ganglion generates alternating swimming movements. Pharmacological and genetic manipulation of glycine receptors shows that they are implicated in the control of these locomotory movements. Morphological molecular techniques and heterologous expression experiments revealed that glycine receptors are inhibitory and are present on both motoneurones and locomotory muscle while putative glycinergic interneurons were identified in the nerve cord by labeling with an anti-glycine antibody. Conclusions In Ciona intestinalis, glycine receptors, glycinergic transmission and putative glycinergic interneurons, have a key role in coordinating swimming movements through a simple CPG that is present in the motor ganglion and nerve cord. Thus, the strong association between glycine receptors and vertebrate locomotory networks may now be extended to include the phylum chordata. The results suggest that the basic network for 'spinal-like' locomotion is likely to have existed in the common ancestor of extant chordates some 650 M years ago.

  2. The synapsin gene family in basal chordates: evolutionary perspectives in metazoans

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    De Bernardi Fiorenza

    2010-01-01

    Full Text Available Abstract Background Synapsins are neuronal phosphoproteins involved in several functions correlated with both neurotransmitter release and synaptogenesis. The comprehension of the basal role of the synapsin family is hampered in vertebrates by the existence of multiple synapsin genes. Therefore, studying homologous genes in basal chordates, devoid of genome duplication, could help to achieve a better understanding of the complex functions of these proteins. Results In this study we report the cloning and characterization of the Ciona intestinalis and amphioxus Branchiostoma floridae synapsin transcripts and the definition of their gene structure using available C. intestinalis and B. floridae genomic sequences. We demonstrate the occurrence, in both model organisms, of a single member of the synapsin gene family. Full-length synapsin genes were identified in the recently sequenced genomes of phylogenetically diverse metazoans. Comparative genome analysis reveals extensive conservation of the SYN locus in several metazoans. Moreover, developmental expression studies underline that synapsin is a neuronal-specific marker in basal chordates and is expressed in several cell types of PNS and in many, if not all, CNS neurons. Conclusion Our study demonstrates that synapsin genes are metazoan genes present in a single copy per genome, except for vertebrates. Moreover, we hypothesize that, during the evolution of synapsin proteins, new domains are added at different stages probably to cope up with the increased complexity in the nervous system organization. Finally, we demonstrate that protochordate synapsin is restricted to the post-mitotic phase of CNS development and thereby is a good marker of postmitotic neurons.

  3. Conservation and diversification of an ancestral chordate gene regulatory network for dorsoventral patterning.

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    Iryna Kozmikova

    Full Text Available Formation of a dorsoventral axis is a key event in the early development of most animal embryos. It is well established that bone morphogenetic proteins (Bmps and Wnts are key mediators of dorsoventral patterning in vertebrates. In the cephalochordate amphioxus, genes encoding Bmps and transcription factors downstream of Bmp signaling such as Vent are expressed in patterns reminiscent of those of their vertebrate orthologues. However, the key question is whether the conservation of expression patterns of network constituents implies conservation of functional network interactions, and if so, how an increased functional complexity can evolve. Using heterologous systems, namely by reporter gene assays in mammalian cell lines and by transgenesis in medaka fish, we have compared the gene regulatory network implicated in dorsoventral patterning of the basal chordate amphioxus and vertebrates. We found that Bmp but not canonical Wnt signaling regulates promoters of genes encoding homeodomain proteins AmphiVent1 and AmphiVent2. Furthermore, AmphiVent1 and AmphiVent2 promoters appear to be correctly regulated in the context of a vertebrate embryo. Finally, we show that AmphiVent1 is able to directly repress promoters of AmphiGoosecoid and AmphiChordin genes. Repression of genes encoding dorsal-specific signaling molecule Chordin and transcription factor Goosecoid by Xenopus and zebrafish Vent genes represents a key regulatory interaction during vertebrate axis formation. Our data indicate high evolutionary conservation of a core Bmp-triggered gene regulatory network for dorsoventral patterning in chordates and suggest that co-option of the canonical Wnt signaling pathway for dorsoventral patterning in vertebrates represents one of the innovations through which an increased morphological complexity of vertebrate embryo is achieved.

  4. Primary vaginal Ewing's sarcoma or primitive neuroectodermal tumor in a 17-year-old woman: a case report

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    Kurkure Purna

    2010-03-01

    Full Text Available Abstract Introduction Primary Ewing's sarcoma or primitive neuroectodermal tumor of the genital tract of women is uncommon. Rarer still is its occurrence in the vagina, with only five cases described so far. Out of these, only one case was confirmed using molecular analysis. Case presentation We present an extremely rare case of Ewing's sarcoma or primitive neuroectodermal tumor in a 17-year-old Indian girl. She presented with a vaginal mass that was initially diagnosed as a malignant round cell tumor. Immunohistochemistry showed diffuse positivity for vimentin, membranous positivity for MIC2, and positivity for BCL2 and FLI-1. On the other hand, she was negative for cytokeratin, epithelial membrane antigen, desmin, Myo D-1, myogenin and smooth muscle actin. A diagnosis of primitive neuroectodermal tumor was thus offered. Furthermore, a molecular analysis of our patient using reverse transcription-polymerase chain reaction technique showed positivity for t(11; 22 (q24; q12 (EWSR1-FLI1, thus confirming the diagnosis of a Ewing's sarcoma/primitive neuroectodermal tumor. Our patient was offered chemotherapy on Institutional protocol EFT 2001. Conclusion This is a rare case of primary vaginal Ewing's sarcoma or primitive neuroectodermal tumor, which was confirmed with molecular analysis, in the youngest patient known so far. This study reinforces the value of integrating morphological features with membranous MIC2 positivity, along with application of molecular techniques in objective identification of an Ewing's sarcoma or primitive neuroectodermal tumor at uncommon sites.

  5. Molecular signatures that are distinctive characteristics of the vertebrates and chordates and supporting a grouping of vertebrates with the tunicates.

    Science.gov (United States)

    Gupta, Radhey S

    2016-01-01

    Members of the phylum Chordata and the subphylum Vertebrata are presently distinguished solely on the basis of morphological characteristics. The relationship of the vertebrates to the two non-vertebrate chordate subphyla is also a subject of debate. Analyses of protein sequences have identified multiple conserved signature indels (CSIs) that are specific for Chordata or for Vertebrata. Five CSIs in 4 important proteins are specific for the Vertebrata, whereas two other CSIs are uniquely found in all sequenced chordate species including Ciona intestinalis and Oikapleura dioica (Tunicates) as well as Branchiostoma floridae (Cephalochordates). The shared presence of these molecular signatures by all vertebrates/chordate species, but in no other animal taxa, strongly indicates that the genetic changes represented by the identified CSIs diagnose monophyletic groups. Two other discovered CSIs are uniquely shared by different vertebrate species and by either one (Ciona intestinalis) or both tunicate (Ciona and Oikapleura) species, but they are not found in Branchiostoma or other animal species. Specific presence of these CSIs in different vertebrates and either one or both tunicate species provides strong independent evidence that the vertebrate species are more closely related to the urochordates (tunicates) than to the cephalochordates.

  6. Ion channel clustering at the axon initial segment and node of Ranvier evolved sequentially in early chordates.

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    Alexis S Hill

    2008-12-01

    Full Text Available In many mammalian neurons, dense clusters of ion channels at the axonal initial segment and nodes of Ranvier underlie action potential generation and rapid conduction. Axonal clustering of mammalian voltage-gated sodium and KCNQ (Kv7 potassium channels is based on linkage to the actin-spectrin cytoskeleton, which is mediated by the adaptor protein ankyrin-G. We identified key steps in the evolution of this axonal channel clustering. The anchor motif for sodium channel clustering evolved early in the chordate lineage before the divergence of the wormlike cephalochordate, amphioxus. Axons of the lamprey, a very primitive vertebrate, exhibited some invertebrate features (lack of myelin, use of giant diameter to hasten conduction, but possessed narrow initial segments bearing sodium channel clusters like in more recently evolved vertebrates. The KCNQ potassium channel anchor motif evolved after the divergence of lampreys from other vertebrates, in a common ancestor of shark and humans. Thus, clustering of voltage-gated sodium channels was a pivotal early innovation of the chordates. Sodium channel clusters at the axon initial segment serving the generation of action potentials evolved long before the node of Ranvier. KCNQ channels acquired anchors allowing their integration into pre-existing sodium channel complexes at about the same time that ancient vertebrates acquired myelin, saltatory conduction, and hinged jaws. The early chordate refinements in action potential mechanisms we have elucidated appear essential to the complex neural signaling, active behavior, and evolutionary success of vertebrates.

  7. A primary primitive neuroectodermal tumor of the central nervous system in a 51-year-old woman: a case report and literature review.

    Science.gov (United States)

    Ašmonienė, Virginija; Skiriutė, Daina; Gudinavičienė, Inga; Tamašauskas, Šarūnas; Skauminas, Kęstutis; Deltuva, Vytenis Pranas; Tamašauskas, Arimantas

    2011-01-01

    Primitive neuroectodermal tumors are a group of rare, aggressive, and highly malignant embryonal tumors of unknown etiology of the central and peripheral nervous systems. It is a term for a group of small round cell tumors thought to be derived from fetal neuroectodermal precursor cells. Primitive neuroectodermal tumor is usually described as a tumor of children younger than 15 years and is very rare in adults. The article presents a short literature review and a rare case of a primary primitive neuroectodermal tumor of the central nervous system diagnosed in a 51-year-old woman.

  8. Adult primary pulmonary primitive neuroectodermal tumor: molecular features and translational opportunities.

    Science.gov (United States)

    Andrei, Mirela; Cramer, Stewart F; Kramer, Zachary B; Zeidan, Amer; Faltas, Bishoy

    2013-02-01

    Primitive neuroectodermal tumors (PNET) arising directly from the lung are very rare but particularly aggressive neoplasms. We report a case of a 31-y-old man with primary pulmonary neuroectodermal tumor. We review the clinical as well as pathological features. As typical for these tumors, the diagnosis was initially delayed in our patient and prognosis was poor despite aggressive surgical resection, postoperative chemotherapy and local irradiation. Recent biological insights have revealed unique chromosomal translocations crucial to the pathogenesis of these tumors, most notably the EWS-FLI-1 translocation. We provide an overview of the molecular features of the Ewing Sarcoma Family of Tumors (ESFT) including PNET and their potential implications for therapeutic targeting.

  9. Primitive neuroectodermal tumor in a mixed germ cell tumor - A rare case report

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    Khushboo Dewan

    2015-01-01

    Full Text Available A rare case of testicular tumor in a 20-year-old male with Primitive Neuroectodermal Tumor (PNET was reported. Imaging studies showed a large heterogenous mass in the right scrotal sac and a large retroperitoneal mass with metastasis in the lung and liver. Serum alpha fetoprotein (AFP was markedly elevated with moderate increase in serum β-human chorionic gonadotropin (hCG levels. After orchidectomy, a histological diagnosis of mixed germ cell tumor-teratoma with primitive neuroectodermal, embryonal, and yolk sac components was made. Some scattered embryoid bodies representative of primitive germ cell tumor were also present. Morphological diversity including PNET prompted the authors to report this case as PNET points toward a poor prognosis.

  10. Successful penile reconstruction after multimodal therapy in patients with primitive neuroectodermal tumor originating from the penis.

    Science.gov (United States)

    Akino, Tomoshige; Shinohara, Nobuo; Hatanaka, Kanako; Kobayashi, Nozomi; Yamamoto, Yuhei; Nonomura, Katsuya

    2014-06-01

    We herein present an extremely rare case of primitive neuroectodermal tumor originating in the penis. A 16-year-old male adolescent presented with painful penile swelling. Pathological, immunohistochemical and cytogenetical examinations of the specimens obtained from total penectomy confirmed the diagnosis of primitive neuroectodermal tumor. After total penectomy, the patient received adjuvant chemotherapy with ifosfamide-based regimen for 48 weeks. As a series of therapies, the patient underwent penile reconstruction surgery after completing adjuvant chemotherapy. The patient has not shown any evidence of recurrence for the 7 years after penile reconstruction surgery, and voiding function is completely normal. A favorable outcome was observed by multimodal therapy including aggressive resection for local control, intensive adjuvant chemotherapy, and penile reconstruction with cosmetic and functional success. Similar therapeutic approaches might be selected for children with primary malignant tumors of the penis.

  11. Ewing Sarcoma/Primitive Neuroectodermal Tumor of the Kidney: A Rare and Lethal Entity.

    Science.gov (United States)

    Celli, Romulo; Cai, Guoping

    2016-03-01

    Ewing sarcoma/primitive neuroectodermal tumor represents a spectrum of undifferentiated tumors with similar biology that together represent the second most common sarcoma in the pediatric-young adult age range. Very rarely, this tumor presents as a primary neoplasm of the kidney. The clinical presentation of this tumor is not specific, and other renal tumors may present with a similar histologic appearance. Establishing the correct diagnosis is critical because renal Ewing sarcoma/primitive neuroectodermal tumor carries a strikingly dismal prognosis and thus dictates a specific treatment strategy. A low threshold for the use of ancillary molecular tests is recommended, particularly in diagnostically problematic cases. Important considerations with regards to morphology, immunohistochemistry, and molecular alterations will be reviewed here and should be taken into account before rendering this rare and lethal diagnosis.

  12. Melanotic Neuroectodermal Tumor of Infancy with Involvement of the Superior Sagittal Sinus.

    Science.gov (United States)

    Foster, Kimberly A; Choudhri, Asim; Lingo, Ryan; Boop, Frederick

    2017-01-01

    Melanotic neuroectodermal tumor of infancy (MNTI) is a rare lesion that typically manifests in the first year of life, most commonly involving the facial bones. We present 2 infants with MNTI involving the posterior skull with associated compression of the superior sagittal sinus (SSS). A review of the anatomical locations of MNTI is offered, and the implications of SSS involvement are described. This represents the first known description of MNTI with involvement of the posterior SSS.

  13. Primary Epidural Peripheral Primitive Neuroectodermal Tumor of the Lumbar Spine: A Case Report

    Energy Technology Data Exchange (ETDEWEB)

    Choi, Woo Jung; Lee, Seung Hun; Joo, Kyung Bin [Dept. of Radiology, Hanyang University Hospital, Seoul (Korea, Republic of); Paik, Seung Sam; Jun, Young Jin [Dept. of Pathology, Hanyang University Hospital, Seoul (Korea, Republic of); Lee, Young Ho [Dept. of Pediatrics, Hanyang University Hospital, Seoul (Korea, Republic of)

    2011-05-15

    A primitive neuroectodermal tumor (PNET) is a highly malignant tumor in children and young adults, and extremely rare in the spine. We report a case of a primary epidural peripheral PNET of the lumbar spine. The present extremely rare case of primary epidural peripheral PNET of the lumbar spine illustrates the unexpected occurrence and should be included in differential diagnoses for patients with spinal tumors.

  14. Rare Renal Incidentaloma in Pregnancy: An Unusual Primitive Neuroectodermal Tumor Presentation

    OpenAIRE

    2015-01-01

    Peripheral Primitive Neuroectodermal Tumors (PNETs) are rare lesions that arise from outside the central nervous system and normally do not affect the genitourinary system. Primary renal presentations are extremely rare but given their aggressive behavior and characteristic cytomorphologic and genetic features should be considered well-defined distinct clinical entities in order to distinguish them from other primary tumors featuring round cells in the kidney. We report one case of PNET invol...

  15. Peripheral primitive neuroectodermal tumor of the small bowel mesentery: Report of a case

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    Marić Helena

    2015-01-01

    Full Text Available Introduction. Primitive neuroectodermal tumor or Ewing’s sarcoma is a tumor of undifferentiated small round cells that arise from the soft tissues, and is believed to be of neural origin. It occurs most often in children, followed by adolescents and young adults. Case Outline. A case of a 24-year-old patient with ulcerostenosans Ewing’s sarcoma of the initial part of the small intestine is presented in our paper. Reviewing the literature and using as an example the case of a female patient with signs of sideropenic anemia caused by primitive neuroectodermal tumor of the small intestine, an attempt was made to clarify the etiology, clinical presentation, diagnosis and therapy with the aim of its rapid detection and treatment. Conclusion. Mesenteric primitive neuroectodermal tumor is a rare neoplasm in adults, while it usually occurs in children and young adults. Surgical resection of the lesions with the application of chemotherapy is the main form of treatment of patients suffering from this disease.

  16. Composite uterine neoplasm with embryonal rhabdomyosarcoma and primitive neuroectodermal tumor components: rhabdomyosarcoma with divergent differentiation, variant of primitive neuroectodermal tumor, or unique entity?

    Science.gov (United States)

    Cate, Frances; Bridge, Julia A; Crispens, Marta A; Keedy, Vicki L; Troutman, Ashley; Coffin, Cheryl M; Fadare, Oluwole

    2013-04-01

    Three cases of composite uterine neoplasms comprised of primitive neuroectodermal tumor (PNET) and rhabdomyosarcoma (RMS) have previously been described, including only one wherein the rhabdomyosarcomatous component was of the embryonal subtype. Whether such composite neoplasms are a variant of RMS, a variant of PNET, or a unique entity is unknown. We report the clinicopathologic, immunohistochemical, and molecular cytogenetic findings in a case of uterine embryonal RMS with coexisting PNET that was diagnosed in a 25-year-old female. The tumor broadly involved the cervix and corpus uteri and resulted in uterine inversion. The 2 distinct components each showed classic morphologic features, including cartilage in the RMS component. The unique combination of histologic, immunohistochemical and molecular findings in composite neoplasms of this type raises a question of whether they should be classified and treated as RMS, PNET, or a unique high-grade sarcoma. A variety of clinicopathologic arguments are presented that support the notion that the current neoplasm is an embryonal rhabdomyosarcoma with divergent neuroectodermal and cartilaginous differentiation.

  17. The central nervous system of sea cucumbers (Echinodermata: Holothuroidea shows positive immunostaining for a chordate glial secretion

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    Grondona Jesus M

    2009-06-01

    Full Text Available Abstract Background Echinoderms and chordates belong to the same monophyletic taxon, the Deuterostomia. In spite of significant differences in body plan organization, the two phyla may share more common traits than was thought previously. Of particular interest are the common features in the organization of the central nervous system. The present study employs two polyclonal antisera raised against bovine Reissner's substance (RS, a secretory product produced by glial cells of the subcomissural organ, to study RS-like immunoreactivity in the central nervous system of sea cucumbers. Results In the ectoneural division of the nervous system, both antisera recognize the content of secretory vacuoles in the apical cytoplasm of the radial glia-like cells of the neuroepithelium and in the flattened glial cells of the non-neural epineural roof epithelium. The secreted immunopositive material seems to form a thin layer covering the cell apices. There is no accumulation of the immunoreactive material on the apical surface of the hyponeural neuroepithelium or the hyponeural roof epithelium. Besides labelling the supporting cells and flattened glial cells of the epineural roof epithelium, both anti-RS antisera reveal a previously unknown putative glial cell type within the neural parenchyma of the holothurian nervous system. Conclusion Our results show that: a the glial cells of the holothurian tubular nervous system produce a material similar to Reissner's substance known to be synthesized by secretory glial cells in all chordates studied so far; b the nervous system of sea cucumbers shows a previously unrealized complexity of glial organization. Our findings also provide significant clues for interpretation of the evolution of the nervous system in the Deuterostomia. It is suggested that echinoderms and chordates might have inherited the RS-producing radial glial cell type from the central nervous system of their common ancestor, i.e., the last common

  18. CONGENITAL ANTERIOR TIBIOFEMURAL SUBLUXATION

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    A. Shahla

    2008-06-01

    Full Text Available Congenital anterior tibiofemoral subluxation is an extremely rare disorder. All reported cases accompanied by other abnormalities and syndromes. A 16-year-old high school girl referred to us with bilateral anterior tibiofemoral subluxation as the knees were extended and reduced at more than 30 degrees flexion. Deformities were due to tightness of the iliotibial band and biceps femuris muscles and corrected by surgical release. Associated disorders included bilateral anterior shoulders dislocation, short metacarpals and metatarsals, and right calcaneuvalgus deformity.

  19. Primitive neuroectodermal tumor of the uterine cervix diagnosed during pregnancy: a rare case with review of literature.

    Science.gov (United States)

    Khosla, Divya; Rai, Bhavana; Patel, Firuza D; Sreedharanunni, Sreejesh; Dey, Pranab; Sharma, Suresh C

    2014-03-01

    Primitive neuroectodermal tumors of the cervix are very rare. A 28-year-old pregnant woman presented with a cervical mass. The tumor was staged as IB2. The biopsy from tumor was suggestive of malignant small round cell tumor. She then underwent termination of pregnancy followed by radical hysterectomy. Based on morphologic and immunohistochemical profile, a diagnosis of peripheral primitive neuroectodermal tumor of the cervix was made. The patient received adjuvant chemotherapy and radiotherapy. The patient is alive and disease-free 33 months post-surgery. The present case highlights the importance of keeping primitive neuroectodermal tumors in the differential diagnosis of small cell neoplasms of the uterine cervix. Pregnancy should not be a barrier to early detection and treatment of this potentially aggressive tumor. The optimal treatment methods have not yet been established because of the rarity of the tumor.

  20. Early dural metastasis from a case of glioblastoma with primitive neuroectodermal differentiation: A case report and literature review.

    Science.gov (United States)

    Konar, Subhas K; Bir, Shyamal C; Maiti, Tanmoy K; Patra, Devi Prasad; DiPoto Brahmbhatt, Angela C; Jacobsohn, Jamie A; Nanda, Anil

    2017-01-01

    Glioblastoma with a primitive neuroectodermal (PNET) variant is a rare primary parenchymal tumor. Only a few cases of extraparenchymal metastasis are reported in world literature. Although the overall survival duration of glioblastoma multiforme (GBM) with primitive neuroectodermal tumor (PNET) variety may be prolonged in comparison to classical glioblastoma, the metastatic trend is completely different, and the prognosis is worse. We report an early dural metastasis of pure PNET component appearing in a case of primary glioblastoma with PNET variant. The lesson learned from this case is to look for early craniospinal metastasis in GBM patient with PNET component, even after completion of adjuvant radiochemotherapy.

  1. Peripheral primitive neuroectodermal tumor of the adrenal gland: A rare entity

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    Chandan Phukan

    2013-01-01

    Full Text Available Peripheral primitive neuroectodermal tumor (PNET is an uncommon tumor and the overall incidence is 1% of all sarcomas. PNET of the adrenal gland is an even rarer entity. A 37-year-old female was evaluated for an episode of loin pain. Ultrasonography showed a large heterogenous left adrenal mass with internal echogenic components. Computed tomography did not show any fat density within to suggest a myelolipoma. Biopsy suggested a poorly differentiated neoplasm with a possibility of PNET of the adrenal gland.

  2. Heavy Metal Bioaccumulation in an Atypical Primitive Neuroectodermal Tumor of the Abdominal Wall.

    Science.gov (United States)

    Roncati, Luca; Gatti, Antonietta Morena; Capitani, Federico; Barbolini, Giuseppe; Maiorana, Antonio; Palmieri, Beniamino

    2015-01-01

    Heavy metals are able to interfere with the function of vital cellular components. Besides in trace heavy metals, which are essential at low concentration for humans, there are heavy metals with a well-known toxic and oncogenic potential. In this study, for the first time in literature, we report the unique adulthood case of an atypical primitive neuroectodermal tumor of the abdominal wall, diagnosed by histology and immunohistochemistry, with the molecular hybridization support. The neoplasia occurred in a patient chronically exposed to a transdermal delivery of heavy metal salts (aluminum and bismuth), whose intracellular bioaccumulation has been revealed by elemental microanalysis.

  3. Intraspinal primitive neuroectodermal tumors: Report of four cases and review of the literature

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    Jingyu Chen

    2009-12-01

    Full Text Available Intraspinal primitive neuroectodermal tumors (PNETs are extremely rare and are aggressive with poor outcomes. Till date only 41 cases of intraspinal PNET have been reported. Here we report four new cases. Total excision of the tumor was done in three patients and partial excision in one patient. The unusual clinical features were vertebral metasis in one patient and short history of symptoms for four dasy. All had surgical excision, total excision in three patients and gross partial excision in one patient. Three patients were treated with craniospinal radiotherapy and chemotherapy. All the four patients are alive and asymptomatic at 6 to 25 months of followup.

  4. Cytokeratin-positive primitive neuroectodermal tumor of the prostate: Case report and review of literature

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    Ibrahim Al Haddabi

    2012-01-01

    Full Text Available Ewing′s sarcoma/primitive neuroectodermal tumor (ES/PNET of the prostate is extremely rare. Here, we report a case of ES/PNET of prostate in a 24-year-old man presenting with dysuria and pelvic discomfort. Computed tomography scan revealed a heterogeneous mass involving the prostate without evidence of distant metastases. Histologically, the tumor was composed of small round blue cells strongly and diffusely positive for CD99 and epithelial markers. Fluorescence in situ hybridization confirmed rearrangement of the Ewing′s sarcoma region on chromosome 22.

  5. Peripheral primitive neuroectodermal tumor of the kidney presenting with pulmonary tumor embolism: A case report

    Institute of Scientific and Technical Information of China (English)

    Sathya; Chinnaa; Chandan; J; Das; Sanjay; Sharma; Prabhjot; Singh; Amlesh; Seth; Suvendu; Purkait; Sandeep; R; Mathur

    2014-01-01

    Peripheral primitive neuroectodermal tumor(PNET) of the kidney is a rare, aggressive tumor known for its recurrence and metastatic potential. Despite the frequency of venous extension to the renal veins and inferior vena cava, pulmonary tumor embolism at the initial presentation is not common. We report a case of 22-year-old female with PNET of the kidney who presented with tumor embolism in the inferior vena cava(IVC) and bilateral pulmonary artery. The patient underwent surgical resection and histopathological analysis confirmed the presence of tumor within the IVC and pulmonary arteries. The patient received adjuvant chemotherapy and is currently doing well on follow-up.

  6. Peripheral primitive neuroectodermal tumor of the posterior mediastinum:A case report

    Institute of Scientific and Technical Information of China (English)

    Yu Liu; Weigang Zhao; Yusheng Shu

    2014-01-01

    Peripheral primitive neuroectodermal tumor (pPNET) is an extremely rare disease entity of malignant tumors belonging to the Ewing sarcoma family that usualy occurs in children and adolescents. We describe a 41-year-old female who presented with right upper abdominal pain. Surgical resection and biopsy revealed smal round-celltumor. Combined with immunohistochemical analysis, pPNET was diagnosed. No evidence of recurrence was noted at 18 months postoperatively. Even thought pPNET is a highly malignant tumor, Wide tumor-free resection and multi-agent chemotherapy can also obtain good clinical outcomes.

  7. Ascidians as excellent chordate models for studying the development of the nervous system during embryogenesis and metamorphosis.

    Science.gov (United States)

    Sasakura, Yasunori; Mita, Kaoru; Ogura, Yosuke; Horie, Takeo

    2012-04-01

    The swimming larvae of the chordate ascidians possess a dorsal hollowed central nervous system (CNS), which is homologous to that of vertebrates. Despite the homology, the ascidian CNS consists of a countable number of cells. The simple nervous system of ascidians provides an excellent experimental system to study the developmental mechanisms of the chordate nervous system. The neural fate of the cells consisting of the ascidian CNS is determined in both autonomous and non-autonomous fashion during the cleavage stage. The ascidian neural plate performs the morphogenetic movement of neural tube closure that resembles that in vertebrate neural tube formation. Following neurulation, the CNS is separated into five distinct regions, whose homology with the regions of vertebrate CNS has been discussed. Following their larval stage, ascidians undergo a metamorphosis and become sessile adults. The metamorphosis is completed quickly, and therefore the metamorphosis of ascidians is a good experimental system to observe the reorganization of the CNS during metamorphosis. A recent study has shown that the major parts of the larval CNS remain after the metamorphosis to form the adult CNS. In contrast to such a conserved manner of CNS reorganization, most larval neurons disappear during metamorphosis. The larval glial cells in the CNS are the major source for the formation of the adult CNS, and some of the glial cells produce adult neurons.

  8. Conservative multimodal management of a primitive neuroectodermal tumor of the thyroid

    Directory of Open Access Journals (Sweden)

    Juliette Haudebourg

    2013-04-01

    Full Text Available Primitive neuroectodermal tumors (PNET represent 1% of sarcomas. Head and neck peripheral PNETs have an intermediate prognosis between abdominopelvic disease and extremities. We here report the case of a 40-year old male who presented with primitive neuroectodermal tumor of the thyroid and was treated by multimodal treatment, including surgery, chemotherapy and intermediate dose radiotherapy. The patient is alive and fit with a functional larynx at 27 months. Multimodal treatments yield five-year survival rates of about 60%. Major drug regimens use vincristine, doxorubicin, ifosfamide or cyclophosphamide, dactinomycin and/or etoposide. Complete surgical excision is undertaken whenever possible to improve long-term survival. However, the relative radiosensitivity of tumors of the Ewing family, suggest multimodal treatment including adjuvant conformal radiotherapy in case of positive margins or poor response to chemotherapy rather than resection with 2-3 cm margins, which would imply laryngeal sacrifice for thyroid tumors. The role of expert rare tumor networks is crucial for optimal decision-making and management of such rare tumors on a case by case basis.

  9. A peripheral primitive neuroectodermal tumor in the larynx: A case report and literature review.

    Science.gov (United States)

    Ijichi, Kei; Tsuzuki, Toyonori; Adachi, Makoto; Murakami, Shingo

    2016-02-01

    Primitive neuroectodermal tumors (PNETs) are malignant tumors comprised of small round cells of neuroectodermal origin. Current evidence indicates that peripheral PNETs (pPNETs), which arise in the non-central nervous system, possess histological similarity to Ewing's sarcoma. Though the occurrence of pPNETs in the head and neck region is rare, these are aggressive malignant tumors, and long-term survival rates following diagnosis remain poor. The current report presents a case of pPNET and evaluates its significance with regard to previous studies. In the present case, a tumor was located in the larynx of the patient, and was diagnosed as pPNET. Immunohistochemical analysis indicated that tumor cells were positive for cluster of differentiation 99. The patient was treated with surgery, multiagent chemotherapy and radiotherapy. Five years subsequent to treatment, the patient had survived and demonstrated no evidence of disease recurrence. In existing literature concerning pPNET located outside the head and neck region, it is recommended that patients are treated with a combination of resection with a wide surgical margin, multiagent chemotherapy and radiotherapy. The present case report concluded that the combination of surgery, systematic chemotherapy and radiotherapy, offers an improved outcome for pPNET localized to the head and neck region, compared with any of these therapies alone.

  10. {sup 68}Ga-labelled peptides in the management of neuroectodermal tumours

    Energy Technology Data Exchange (ETDEWEB)

    Naji, Meeran [Maidstone and Tunbridge Wells NHS Trust, Departments of Nuclear Medicine and Radiology, Maidstone (United Kingdom); Al-Nahhas, Adil [Hammersmith Hospital, Imperial College NHS Trust, Department of Nuclear Medicine, London (United Kingdom)

    2012-02-15

    Neuroectodermal tumours arise from chromaffin cells and possess the ability to secrete catecholamines. They are generally rare and may occur in association with a variety of hereditary syndromes such as MEN-2A and 2B, neurofibromatosis type 1 and von Hippel-Lindau disease. The most common types are phaeochromocytoma arising from the adrenal medulla and paraganglioma of extra-adrenal origin. Phaeochromocytomas tend to be benign and are often associated with a gene mutation if the disease is bilateral, while paragangliomas are often malignant, have a more aggressive nature and tend to metastasize. There are no specific histological or immunohistochemical features that indicate the malignant potential and the diagnosis of malignancy can only be established by the presence of distant metastases. Therefore, imaging can play a vital role in the diagnosis, localization, staging and assessment of spread. Traditionally, this is achieved with a combination of cross-sectional (CT and MRI) and functional ({sup 123}I-MIBG or {sup 111}In-octreotide) imaging. However, these modalities are not adequate and achieve moderate sensitivity. The introduction of {sup 68}Ga-DOTA peptide in PET/CT imaging has led to improved receptor targeting and superb PET resolution, as well as accurate localization of lesions. The use of this technique in neuroectodermal tumours has been shown to be superior to all available modalities, but the available data are limited and larger studies are awaited to establish its role in the management of these tumours. (orig.)

  11. Anterior Cruciate Ligament (ACL) Injuries

    Science.gov (United States)

    ... Week of Healthy Breakfasts Shyness Anterior Cruciate Ligament (ACL) Injuries KidsHealth > For Teens > Anterior Cruciate Ligament (ACL) ... and Recovery Coping With an ACL Injury About ACL Injuries A torn anterior cruciate ligament (ACL) is ...

  12. A novel splice mutation in the TP53 gene associated with Leydig cell tumor and primitive neuroectodermal tumor

    DEFF Research Database (Denmark)

    Stecher, C.W.; Hasle, H.; Grønbæk, Kirsten

    2008-01-01

    A 20-month-old boy presented with precocious puberty due to a Leydig cell tumor, and at the age of 6 years with a primitive neuroectodermal brain-tumor (PNET). A novel splice site mutation of the TP53-gene, likely to be associated with a nonfunctional protein, was found in the proband, his father...

  13. Anterior cervical plating

    Directory of Open Access Journals (Sweden)

    Gonugunta V

    2005-01-01

    Full Text Available Although anterior cervical instrumentation was initially used in cervical trauma, because of obvious benefits, indications for its use have been expanded over time to degenerative cases as well as tumor and infection of the cervical spine. Along with a threefold increase in incidence of cervical fusion surgery, implant designs have evolved over the last three decades. Observation of graft subsidence and phenomenon of stress shielding led to the development of the new generation dynamic anterior cervical plating systems. Anterior cervical plating does not conclusively improve clinical outcome of the patients, but certainly enhances the efficacy of autograft and allograft fusion and lessens the rate of pseudoarthrosis and kyphosis after multilevel discectomy and fusions. A review of biomechanics, surgical technique, indications, complications and results of various anterior cervical plating systems is presented here to enable clinicians to select the appropriate construct design.

  14. Tumor neuroectodérmico pigmentado infantil: Reporte de un caso Neuroectodermal tumor pigmented children: A case report

    Directory of Open Access Journals (Sweden)

    A.J. Díaz Caballero

    2011-08-01

    Full Text Available El tumor neuroectodérmico pigmentado es un tumor benigno de crecimiento rápido e intensamente pigmentado del maxilar inferior (y en ocasiones de otros sitios, que consiste en una masa infiltrativa de células dispuestas según un patrón alveolar. Aparece casi exclusivamente en lactantes. Se presenta como una tumoración protuberante en un maxilar o la mandíbula principalmente en tejidos blandos; encontrándose excepcionalmente en el tracto intestinal, pelvis, retroperitoneo y riñón. Clínicamente se manifiesta como una tumoración localizada, con presencia o no de zonas pigmentadas. Estas neoplasias son raras y afectan mayormente niños, que por lo general son menores de un año y que pueden ser congénitos. Su localización en cerca de un 70% es la mandíbula; facio-cervical, en 90%, también se reportaron en localizaciones como: fontanela anterior, hueso temporal y duramadre, epidídimo, huesos largos, mediastino. Éstos son considerados como formas periféricas, ya que existen formas centrales que pueden afectar cerebro, cerebelo, glándula pineal. La clínica de estos casos puede simular a muchas otras patologías, dificultando su diagnostico ya que es una masa que protruye y deforma que rara vez ulcera. Las radiografías muestran una masa radiolúcida con capacidad de destrucción focal y desplazamiento de los dientes, por lo que se puede confundir con un ameloblastoma es por esto que es necesario un manejo adecuado con los exámenes histológicos necesarios como se hizo en el presente caso.The Pigmented neuroectodermal tumor is a benign tumor of intensely pigmented and rapid growth of the lower jaw (and in occasions of other sites [places], that consists of a mass of infiltrative cells arranged according to an alveolar boss. It (he, she appears almost exclusively in nursing. He (she appears as a protruding tumor in the jaw Maxillary one or principally in the soft (smooth fabrics; being exceptionally in the intestinal tract, pelvis

  15. Tipifarnib in Treating Young Patients With Recurrent or Progressive High-Grade Glioma, Medulloblastoma, Primitive Neuroectodermal Tumor, or Brain Stem Glioma

    Science.gov (United States)

    2013-10-07

    Childhood High-grade Cerebral Astrocytoma; Childhood Oligodendroglioma; Recurrent Childhood Brain Stem Glioma; Recurrent Childhood Cerebellar Astrocytoma; Recurrent Childhood Cerebral Astrocytoma; Recurrent Childhood Medulloblastoma; Recurrent Childhood Supratentorial Primitive Neuroectodermal Tumor; Recurrent Childhood Visual Pathway and Hypothalamic Glioma

  16. Primary Ewing's sarcoma/primitive neuroectodermal tumor (ES/PNET) of the penis: a report of an extraordinarily unusual site and a literature review of extraskeletal Ewing's sarcoma.

    Science.gov (United States)

    Ma, Zheng; Brimo, Fadi; Zeizafoun, Nebras

    2013-02-01

    Ewing's sarcomas/primitive neuroectodermal tumors (ES/PNETs) arise from a multipotent progenitor cell and are considered to be of neuroectodermal derivation. Most tumors commonly arise in the skeletal system, which are the classic ES/PNET and occasionally occur in the soft tissue of extraskeletal sites, which are named extraskeletal Ewing's sarcomas (EES/PNET). This study reports a case of a 28-year-old man with primary EES/PNET of the penis.

  17. Anaesthetic Management of a Neuroectodermal Tumor of Infancy: A Rare Case Report

    Science.gov (United States)

    Ubale, Pravin; Baldwa, Namita; Gujjar, Pinakin

    2017-01-01

    Melanotic neuroectodermal tumor of infancy is a relatively uncommon osteolytic-pigmented neoplasm that primarily affects the jaws of infants. We report a 5-month-old male child who presented with a swelling in the right upper tooth region of upper jaw in which we face difficult mask ventilation as well as difficult intubation. Wide surgical excision was performed under general anesthesia. The uneventful course of anesthesia in the presented case was due to the thorough systemic evaluation and careful anesthetic strategy. Patients of congenital epulis continue to pose challenge to anesthesiologist as a consequence of the potential difficult mask ventilation and intubation. We hereby present a case of congenital epulis repair using diode laser under general anesthesia.

  18. Congenital peripheral primitive neuroectodermal tumor: A case treated successfully with multimodality treatment

    Directory of Open Access Journals (Sweden)

    Shikha Goyal

    2014-12-01

    Full Text Available Neonatal tumors comprise less than two percent of childhood malignancies. Most are solid tumors, most common histologies being teratoma and neuroblastoma. We encountered a child who was detected to have a right arm mass on antenatal sonogram, which was diagnosed to be a primitive neuroectodermal tumor involving the triceps on fine needle aspiration cytology performed in the post-natal period. The child was successfully treated with multimodality treatment consisting of surgery, chemotherapy and radiotherapy. We also discuss briefly the problems associated with therapy in neonatal period. A review of all cases reported to have congenital Ewing’s sarcoma family of tumors is presented. Novel therapies are needed to improve efficacy and decrease the devastating side effects of treatment in this age group.

  19. A case report of adrenocortical carcinosarcoma with oncocytic and primitive neuroectodermal-like features.

    Science.gov (United States)

    Kao, Chia-Sui; Grignon, David J; Ulbright, Thomas M; Idrees, Muhammad T

    2013-09-01

    Adrenocortical carcinosarcomas are rare aggressive neoplasms; only a few have been reported to date, all with dismal prognosis. These were reported as having varying morphology. We have encountered a case of adrenal carcinosarcoma with an undifferentiated component bearing similarities to primitive neuroectodermal tumors and other areas of oncocytic differentiation. The 48-year-old woman patient presented with abdominal pain and unintended, excessive weight loss. Computed tomographic imaging revealed a tumor located adjacent to the liver and kidney necessitating a partial nephrectomy and hepatectomy. Histologically, the tumor exhibited malignant features. Melan-A, inhibin, calretinin, cytokeratin AE1/AE3, synaptophysin, and neuron-specific enolase were positive immunohistochemically. The patient developed metastasis within 2 months of surgery and is currently alive with disease after chemotherapy. Adrenal carcinosarcoma is a rare highly aggressive malignancy with a wide morphologic spectrum. Recognition of variant morphology and applying correct immunohistochemical studies will aid in reaching an accurate diagnosis.

  20. Retroperitoneal primitive neuroectodermal tumor in an adult: A rare case report and review of the literature

    Directory of Open Access Journals (Sweden)

    Kavita Mardi

    2016-01-01

    Full Text Available Primitive neuroectodermal tumor (PNET and Ewing's sarcoma (EWS are small round cell tumors occurring mainly in children and adolescents. Their occurrence in adults is rare. The abdominal cavity and retroperitoneal PNET/EWS are also relatively rare, grow rapidly in size, compressing surrounding organs/large vessels, and make surgical resection difficult. We report one such rare occurrence of a retroperitoneal PNET in 41-year-old male who presented with abdominal pain and constipation. Contrast enhanced computed tomography abdomen showed large lobulated necrotic hypodense enhancing lesion extending from epigastrium to hypogastrium and involving entire abdomen. Excision of retroperitoneal mass with omentectomy was done. Microscopic examination revealed a malignant small round cell tumor with homer wright rosettes and the tumor cells were positive for CD99.

  1. Peripheral primitive neuroectodermal tumor causing cauda equina syndrome with destruction of L5 vertebra

    Directory of Open Access Journals (Sweden)

    Dhatt Sarvdeep

    2010-01-01

    Full Text Available A 24-year-old male patient presented with cauda equina lesion symptoms. His clinicoradiological examination including X-rays, CT scan and MRI revealed destruction of L 5 vertebral body, pedicle and a mass extending to lateral recess and left intervertebral foramina causing pressure over the thecal sac. A CT guided FNAC was inconclusive. Open biopsy and hemilaminectomy of L 5 vertebra was performed. Histopathology and immunocytochemical analysis revealed it to be primitive neuroectodermal tumor. Patient was given chemotherapy and radiation therapy. His lower limb power improved by grade I post operatively and at 2 years follow-up bowel/bladder recovery was noticed. Patient died after 2.5 years of surgery because of pulmonary metastasis.

  2. Congenital peripheral primitive neuroectodermal tumor: a case treated successfully with multimodality treatment.

    Science.gov (United States)

    Goyal, Shikha; Biswas, Ahitagni; Gupta, Ruchika; Mohanti, Bidhu Kalyan

    2014-12-01

    Neonatal tumors comprise less than two percent of childhood malignancies. Most are solid tumors, most common histologies being teratoma and neuroblastoma. We encountered a child who was detected to have a right arm mass on antenatal sonogram, which was diagnosed to be a primitive neuroectodermal tumor involving the triceps on fine needle aspiration cytology performed in the post-natal period. The child was successfully treated with multimodality treatment consisting of surgery, chemotherapy and radiotherapy. We also discuss briefly the problems associated with therapy in neonatal period. A review of all cases reported to have congenital Ewing's sarcoma family of tumors is presented. Novel therapies are needed to improve efficacy and decrease the devastating side effects of treatment in this age group.

  3. [Primitive neuroectodermal tumour of soft tissue of the index finger in an adult. A case report].

    Science.gov (United States)

    Berrada, N; Bellarbi, S; El Mannouar, M; Errihani, H

    2012-12-01

    The primitive neuroectodermal tumours (PNET) of soft tissues belong to the Ewing's tumors family and affects particularly the child. The localization of the disease at the extremities is very rare within the adult population and raises the problem of differential diagnosis with others tumors of the soft tissues. We report the case of a 48-year-old patient with a localized tumor, at the level of the second right finger, of six months evolution. The biopsy showed the infiltrating nature of the tumour; and the diagnosis of (PNET) was confirmed after the histological and immunohistochemical study. The extension assessment was negative and the patient had an amputation of the second and third rays of the right hand. Four years afterwards, the patient showed no recurrence or metastases.

  4. Central type primitive neuroectodermal tumor/neuroblastoma of the uterus: a case report.

    Science.gov (United States)

    Shimada, Chisa; Todo, Yukiharu; Okamoto, Kazuhira; Akashi, Daisuke; Yamashiro, Katsushige; Hasegawa, Tadashi

    2014-10-01

    We encountered a 63-year-old woman who had a uterine tumor with peritoneal dissemination and para-aortic lymph node metastasis. Microscopic specimens of the tumor showed a small blue round-cell tumor. Immunohistochemistry showed cells to be negative for cytokeratin AE1/3, desmin, myogenin, CD10, CD34, and CD99, focal positive for vimentin, and positive for muscle-specific actin (HHF-35), neurofilament, synaptophysin and CD56. Fluorescence in situ hybridization revealed no split signal showing Ewing sarcoma breakpoint region 1 gene translocation. Deletion of 1p36 was identified in 30% of the tumor cells. These findings are thought to be equivalent to central type primitive neuroectodermal tumors/neuroblastoma. Cytoreductive debulking surgery followed by chemotherapy, including cyclophosphamide, vincristine and adriamycin, resulted in complete remission. She has no evidence of disease at 24 months after surgery.

  5. Solitary epidural brain metastasis of Neuroepithelioma (a Primitive Neuroectodermal Tumor: case report

    Directory of Open Access Journals (Sweden)

    Farnaz Farshidfar

    2008-08-01

    Full Text Available A 14 years old male was referred to Computerized tomography scan (CT of our hospital for evaluation of headache. The patient was known case of cervical soft tissue Primitive neuroectodermal tumor (PNET which has undergone surgery and radiotherapy 4 years ago. The CT scan showed large solitary extra axial, epidural lesion in right parietal region, with mass effect and bony involvement. Then surgery was done for him and the resultant biopsy was Neuroepithelioma. After diagnosis the patient has undergone chemotherapy and radiotherapy. He has no signs or symptoms of malignancy, and also follow up CT scan of the brain, chest, and abdomen were normal after two years of surgery. This is the first reported case of epidural metastasis of a head and neck PNET in an adolescent.

  6. BMPs regulate msx gene expression in the dorsal neuroectoderm of Drosophila and vertebrates by distinct mechanisms.

    Directory of Open Access Journals (Sweden)

    Francisco F Esteves

    2014-09-01

    Full Text Available In a broad variety of bilaterian species the trunk central nervous system (CNS derives from three primary rows of neuroblasts. The fates of these neural progenitor cells are determined in part by three conserved transcription factors: vnd/nkx2.2, ind/gsh and msh/msx in Drosophila melanogaster/vertebrates, which are expressed in corresponding non-overlapping patterns along the dorsal-ventral axis. While this conserved suite of "neural identity" gene expression strongly suggests a common ancestral origin for the patterning systems, it is unclear whether the original regulatory mechanisms establishing these patterns have been similarly conserved during evolution. In Drosophila, genetic evidence suggests that Bone Morphogenetic Proteins (BMPs act in a dosage-dependent fashion to repress expression of neural identity genes. BMPs also play a dose-dependent role in patterning the dorsal and lateral regions of the vertebrate CNS, however, the mechanism by which they achieve such patterning has not yet been clearly established. In this report, we examine the mechanisms by which BMPs act on cis-regulatory modules (CRMs that control localized expression of the Drosophila msh and zebrafish (Danio rerio msxB in the dorsal central nervous system (CNS. Our analysis suggests that BMPs act differently in these organisms to regulate similar patterns of gene expression in the neuroectoderm: repressing msh expression in Drosophila, while activating msxB expression in the zebrafish. These findings suggest that the mechanisms by which the BMP gradient patterns the dorsal neuroectoderm have reversed since the divergence of these two ancient lineages.

  7. Primitive neuroectodermal tumor of the zygomaticoorbital complex: a rare location and ways of surgical repair of the area

    Directory of Open Access Journals (Sweden)

    Ch. R. Ragimov

    2015-01-01

    Full Text Available Primitive neuroectodermal tumor in the zygomaticoorbital region is a rare neoplasm of the head and neck. Due to the necessity for wide radical excision of a primary tumor, there may be serious functional and cosmetic disorders that substantially affect quality of life in patients. Restoration of this region is one of the challenges of reconstructive surgery because of the specific features of the relief of bone structures. The paper describes a clinical case of the site of primitive neuroectodermal tumor in the zygomaticoorbital complex and a method for repairing postresectional defect and completely recovering the function of the organ of vision and aesthetic parameters of the face.

  8. Spinal primitive neuroectodermal tumor mimicking as chronic inflammatory demyelination polyneuropathy: a case report and review of literature.

    Science.gov (United States)

    Chan, Sophelia H S; Tsang, Dickson S F; Wong, Virginia C N; Chan, Godfrey C F

    2015-02-01

    We report a young boy who presented with progressive weakness of lower extremities associated with areflexia and abnormal electrophysiological findings initially suggestive of chronic inflammatory demyelinating polyneuropathy. Initial lumbosacral spinal magnetic resonance imaging (MRI) showed thickened descending spinal nerve roots only. Immunomodulating therapy was given but with limited clinical response. Repeated spine magnetic resonance imaging showed cauda equina and also new spinal cord extramedullary contrast enhancement. The initial extensive investigations including open biopsy did not point to any specific diagnosis. Only through pursuing a repeated biopsy, the diagnosis of the spinal peripheral primitive neuroectodermal tumor was confirmed. This case highlights the diagnostic challenges of the spinal peripheral primitive neuroectodermal tumor that could have an initial chronic inflammatory demyelinating polyneuropathy-like presentation. The literature review confirms that this is a rare condition and cauda equina origin has only been reported in adults and teenagers, and this is the first reported case in a young child.

  9. The posterior neural plate in axolotl gives rise to neural tube or turns anteriorly to form somites of the tail and posterior trunk.

    Science.gov (United States)

    Taniguchi, Yuka; Kurth, Thomas; Weiche, Susanne; Reichelt, Saskia; Tazaki, Akira; Perike, Srikanth; Kappert, Verena; Epperlein, Hans-Henning

    2017-02-15

    Classical grafting experiments in the Mexican axolotl had shown that the posterior neural plate of the neurula is no specified neuroectoderm but gives rise to somites of the tail and posterior trunk. The bipotentiality of this region with neuromesodermal progenitor cell populations was revealed more recently also in zebrafish, chick, and mouse. We reinvestigated the potency of the posterior plate in axolotl using grafts from transgenic embryos, immunohistochemistry, and in situ hybridization. The posterior plate is brachyury-positive except for its more anterior parts which express sox2. Between anterior and posterior regions of the posterior plate a small domain with sox2+ and bra+ cells exists. Lineage analysis of grafted GFP-labeled posterior plate tissue revealed that posterior GFP+ cells move from dorsal to ventral, form the posterior wall, turn anterior bilaterally, and join the gastrulated paraxial presomitic mesoderm. More anterior sox2+/GFP+ cells, however, are integrated into the developing spinal cord. Tail notochord is formed from axial mesoderm involuted already during gastrulation. Thus the posterior neural plate is a postgastrula source of paraxial mesoderm, which performs an anterior turn, a novel morphogenetic movement. More anterior plate cells, in contrast, do not turn anteriorly but become specified to form tail spinal cord.

  10. The Xenopus receptor tyrosine kinase Xror2 modulates morphogenetic movements of the axial mesoderm and neuroectoderm via Wnt signaling.

    Science.gov (United States)

    Hikasa, Hiroki; Shibata, Mikihito; Hiratani, Ichiro; Taira, Masanori

    2002-11-01

    The Spemann organizer plays a central role in neural induction, patterning of the neuroectoderm and mesoderm, and morphogenetic movements during early embryogenesis. By seeking genes whose expression is activated by the organizer-specific LIM homeobox gene Xlim-1 in Xenopus animal caps, we isolated the receptor tyrosine kinase Xror2. Xror2 is expressed initially in the dorsal marginal zone, then in the notochord and the neuroectoderm posterior to the midbrain-hindbrain boundary. mRNA injection experiments revealed that overexpression of Xror2 inhibits convergent extension of the dorsal mesoderm and neuroectoderm in whole embryos, as well as the elongation of animal caps treated with activin, whereas it does not appear to affect cell differentiation of neural tissue and notochord. Interestingly, mutant constructs in which the kinase domain was point-mutated or deleted (named Xror2-TM) also inhibited convergent extension, and did not counteract the wild-type, suggesting that the ectodomain of Xror2 per se has activities that may be modulated by the intracellular domain. In relation to Wnt signaling for planar cell polarity, we observed: (1) the Frizzled-like domain in the ectodomain is required for the activity of wild-type Xror2 and Xror2-TM; (2) co-expression of Xror2 with Xwnt11, Xfz7, or both, synergistically inhibits convergent extension in embryos; (3) inhibition of elongation by Xror2 in activin-treated animal caps is reversed by co-expression of a dominant negative form of Cdc42 that has been suggested to mediate the planar cell polarity pathway of Wnt; and (4) the ectodomain of Xror2 interacts with Xwnts in co-immunoprecipitation experiments. These results suggest that Xror2 cooperates with Wnts to regulate convergent extension of the axial mesoderm and neuroectoderm by modulating the planar cell polarity pathway of Wnt.

  11. Ewing sarcoma and primitive neuroectodermal tumor of the esophagus: report of a case and review of literature.

    Science.gov (United States)

    Johnson, Andrew D; Pambuccian, Stefan E; Andrade, Rafael S; Dolan, Michelle M; Aslan, Deniz L

    2010-10-01

    This study presents a case of Ewing sarcoma and primitive neuroectodermal tumor arising in the esophagus of a 44-year-old woman who presented with progressive dysphagia. Imaging studies demonstrated a polypoid lesion in the esophagus. The tumor was characterized by corded and pseudopapillary architecture, cytologic monotony, and low proliferative activity. Immunohistochemical stains were positive for CD99, neuron-specific enolase, vimentin, cyclin D1, p53, and FLI1 gene product. Fluorescence in situ hybridization demonstrated a 22q12 translocation, associated with primitive neuroectodermal tumor in the tumor cells, whereas reverse transcription polymerase chain reaction conformed expression of Ewing sarcoma/FLI1 fusion transcript in the patient's bone marrow aspirate. Although this is a rare site for this type of tumor to occur, primitive neuroectodermal tumor should be considered in the differential diagnosis of mesenchymal tumors of the esophagus. Genetic analysis is crucial to establish the diagnosis and can be successfully performed on formalin-fixed, paraffin-embedded material and hematopoietic tissue.

  12. Anterior crucate ligament (ACL) injury

    Science.gov (United States)

    ... An anterior cruciate ligament injury is the over-stretching or tearing of the anterior cruciate ligament (ACL) ... may be injured. This is a medical emergency. Prevention Use proper techniques when playing sports or exercising. ...

  13. Facetas em dentes anteriores

    OpenAIRE

    Veloso, Helena Rafaela Lourenço Martins

    2015-01-01

    Projeto de Pós-Graduação/Dissertação apresentado à Universidade Fernando Pessoa como parte dos requisitos para obtenção do grau de Mestre em Medicina Dentária A presente revisão bibliográfica aborda as facetas estéticas em dentes anteriores, pela crescente valorização de um sorriso esteticamente agradável, facto que faz com que as pessoas procurem cada vez mais alternativas de tratamento para melhorar a aparência do seu sorriso. Os dentes anteriores são decisivos na aparência estética e, c...

  14. Intradural anterior transpetrosal approach.

    Science.gov (United States)

    Ichimura, Shinya; Hori, Satoshi; Hecht, Nils; Czabanka, Marcus; Vajkoczy, Peter

    2016-10-01

    The standard anterior transpetrosal approach (ATPA) for petroclival lesions is fundamentally an epidural approach and has been practiced for many decades quite successfully. However, this approach has some disadvantages, such as epidural venous bleeding around foramen ovale. We describe here our experience with a modified technique for anterior petrosectomy via an intradural approach that overcomes these disadvantages. Five patients with petroclival lesions underwent surgery via the intradural ATPA. The intraoperative hallmarks are detailed, and surgical results are reported. Total removal of the lesions was achieved in two patients with petroclival meningioma and two patients with pontine cavernoma, whereas subtotal removal was achieved in one patient with petroclival meningioma without significant morbidity. No patient experienced cerebrospinal fluid leakage. The intradural approach is allowed to tailor the extent of anterior petrosectomy to the individually required exposure, and the surgical procedure appeared to be more straightforward than via the epidural route. Caveats encountered with the approach were the temporal basal veins that could be spared as well as identification of the petrous apex due to the lack of familial epidural landmarks. The risk of injury to the temporal bridging veins is higher in this approach than in the epidural approach. Intradural approach is recommended in patients with a large epidural venous route, such as sphenobasal and sphenopetrosal vein. Navigation via bone-window computed tomography is useful to identify the petrous apex.

  15. The effect of antibiotic exposure on eicosanoid generation from arachidonic acid and gene expression in a primitive chordate, Branchiostoma belcheri.

    Science.gov (United States)

    Yuan, Dongjuan; Pan, Minming; Zou, Qiuqiong; Chen, Chengyong; Chen, Shangwu; Xu, Anlong

    2015-01-01

    Chloramphenicol (Chl) is an effective antimicrobial agent widely used in veterinary medicine and commonly used in fish. Its use is restricted in the clinic because of adverse effects on the immune system and oxidative stress in mammals. However, the effects of Chl treatment on invertebrates remain unclear. Amphioxus, a basal chordate, is an ideal model to study the origin and evolution of the vertebrate immune system as it has a primary vertebrate-like arachidonic acid (AA) metabolic system. Here, we combined transcriptomic and lipidomic approaches to investigate the immune system and observe the oxygenated metabolites of AA to address the antibiotic effects on amphioxus. Tissue necrosis of the gill slits occurred in the Chl-treated amphioxus, but fewer epithelial cells were lost when treated with both Chl and ampicillin (Amp). The immune related pathways were dysregulated in both of the antibiotic treatment groups. The Chl alone treatment resulted in immunosuppression with down-regulation of the innate immune genes. In contrast, the Chl + Amp treatment resulted in immunostimulation to some extent, as shown by KEGG clustering. Furthermore, Chl induced a 3-fold reduction in the level of the eicosanoids, while the Chl + Amp treatment resulted in 1.7-fold increase of eicosanoid level. Thus in amphioxus, Amp might relieve the effects of the Chl-induced immune suppression and increase the level of eicosanoids from AA. Finally, the oxygenated metabolites from AA might be crucial to evaluate the effects of Chl treatment in animals.

  16. Ex vivo organ culture of human hair follicles: a model epithelial-neuroectodermal-mesenchymal interaction system.

    Science.gov (United States)

    Tobin, Desmond J

    2011-01-01

    The development of hair follicle organ culture techniques is a significant milestone in cutaneous biology research. The hair follicle, or more accurately the "pilo-sebaceous unit", encapsulates all the important physiologic processes found in the human body; controlled cell growth/death, interactions between cells of different histologic type, cell differentiation and migration, and hormone responsitivity to name a few. Thus, the value of the hair follicle as a model for biological scientific research goes way beyond its scope for cutaneous biology or dermatology alone. Indeed, the recent and dramatic upturn in interest in hair follicle biology has focused principally on the pursuit of two of biology's holy grails; post-embryonic morphogenesis and control of cyclical tissue activity. The hair follicle organ culture model, pioneered by Philpott and colleagues, ushered in an exceptionally accessible way to assess how cells of epithelial (e.g., keratinocytes), mesenchymal (e.g., fibroblasts), and neuroectodermal (e.g., melanocytes) origin interact in a three-dimensional manner. Moreover, this assay system allows us to assess how various natural and pharmacologic agents affect complex tissues for growth modulation. In this article, I focus on the culture of the human hair follicle mini-organ, discussing both the practical issues involved and some possible research applications of this assay.

  17. Huge primitive neuroectodermal tumor of the pancreas: Report of a case and review of the literature

    Institute of Scientific and Technical Information of China (English)

    Thilo Welsch; Gunhild Mechtersheimer; Sebastian Aulmann; Sascha A Mueller; Markus W Buechler; Jan Schmidt; Peter Kienle

    2006-01-01

    Primitive neuroectodermal tumor (PNET) of the pancreas is an extremely rare tumor that usually occurs in children or young adults. We report a case of a 33-year-old male patient with an 18 cm × 18 cm × 16 cm mass arising from the pancreatic body and tail with a oneday history of abdominal pain. Initial CT scan showed no signs of metastatic tumor spread. The tumor caused intrabdominal bleeding and the patient underwent primary tumor resection including partial gastrectomy, lett pancreatic resection and splenectomy. Diagnosis of PNET was confirmed by histology, immunohistochemistry and FISH analysis. All neoplastic cells were stained positive for MIC2-protein (CD99). Approximately one month after surgery, several liver metastases were observed and the patient underwent chemotherapy according to the EuroEwing protocol. Subsequent relaparotomy excluded any residual hepatic or extrahepatic abdominal metastases.Although PNET in the pancreas is an extremely rare entity, it should be considered in the diffential diagnosis of pancreatic masses, especially in young patients. This alarming case particularly illustrates that PNET in the pancreas although in an advanced stage can present with only a short history of mild symptoms.

  18. Primary Multiple Pulmonary Primitive Neuroectodermal Tumor: Case Report and Literature Review.

    Science.gov (United States)

    Dong, Ming; Liu, Jinghao; Song, Zuoqing; Li, Xin; Shi, Tao; Wang, Dan; Ren, Dian; Chen, Jun

    2015-07-01

    Primitive neuroectodermal tumors (PNETs) arising directly from the lung are extremely rare but particularly aggressive neoplasms. Although thoracic PNET usually develops on the chest wall, there have been reports of primary Ewing sarcoma/PNET of the lung.We present the case of a 16-year-old male with PNET diagnosed following histologic and immunohistochemical examination of a video-assisted thoracic surgical biopsy. As typically occurring for these tumors, the diagnosis was initially delayed in our patient and prognosis was poor despite aggressive surgical resection and postoperative chemotherapy. We attempted to explore the use of targeted pharmacotherapy through high-throughput sequencing in this case. We found that this treatment using Avastin and Cetuximab could provide a new therapeutic direction for PNET.PNENTs arising from the lung parenchyma without pleural or chest wall involvement are extremely rare. Although uncommon, if the pathological features are similar to Ewing sarcoma, PNENTs should be kept in mind, and the target therapy may be a potent treatment for this disease.

  19. A Case of Renal Primitive Neuroectodermal Tumor Confirmed by Fluorescence in situ Hybridization

    Directory of Open Access Journals (Sweden)

    Toshiki Etani

    2015-04-01

    Full Text Available Primitive neuroectodermal tumor (PNET is a member of the Ewing's sarcoma family of tumors (ESFT. We report a case of PNET in a 66-year-old male who presented with a large solid tumor within the parenchyma of the middle pole of the left kidney with metastases to the left adrenal gland and right ischium. A fine-needle biopsy was performed and showed a small round cell tumor. Results of immunohistochemical staining suggested this tumor belonged to ESFT. Preoperative VDC-IE (combined vincristine, doxorubicin and cyclophosphamide followed by another combination of ifosfamide and etoposide chemotherapy and left radical nephrectomy and adrenalectomy were performed. The histopathological findings of the resected tumor were similar to those in the biopsy specimen, but the results of AE1/AE3 were different. For the diagnosis, fluorescence in situ hybridization was performed. Split signals of the EWSR1 gene were detected, and transmission electron microscopy showed neuroendocrine granules and microtubules. The final diagnosis of this tumor was PNET of the kidney.

  20. Left inguinal lymphadenopathy as the solitary metastatic presentation of primitive neuroectodermal tumor of unknown origin

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    Tamojit Chaudhuri

    2013-01-01

    Full Text Available Primitive neuroectodermal tumor (PNET/ewing′s sarcoma is a rare neural crest tumor of central nervous system, thoracopulmonary regions, pelvis, and lower extremities. Visceral involvement by PNET is a rare phenomenon, with kidney being the most commonly involved organ. We report a 35-year-old Asian female presenting with left inguinal swelling, with computed tomography scan evidence of conglomerate lymph nodal mass in the left external iliac and inguinal region. A clinico-radiological diagnosis of lymphoproliferative disorder was made. She subsequently underwent excision biopsy. Histopathology of the biopsy specimen revealed completely effaced lymph nodal architecture, which was replaced by a tumor composed of nests of small, round, blue cells. On immunohistochemistry, the tumor cells were positive for CD99 and negative for CD3, CD20, leucocyte common antigen, epithelial membrane antigen, cytokeratin, desmin, vimentin, synaptophysin, and chromogranin A. Extensive search regarding any possible different site of involvement by the tumor was negative. The clinical presentation and histological, cytological, and immunohistochemical pattern, lead to the diagnosis of metastatic PNET of the left external iliac and inguinal lymph node with unknown primary origin. To the best of our knowledge, it is the first ever reported case of inguinal lymphadenopathy as the solitary metastatic presentation of PNET of unknown origin.

  1. MRI Features of Intracranial Primitive Neuroectodermal Tumors in Adults:Comparing with Histopathological Findings

    Institute of Scientific and Technical Information of China (English)

    石浩军; 孔祥泉; 徐海波; 徐丽莹; 刘定西

    2004-01-01

    Summary: The MRI appearances of 7 adult patients with pathologically proven intracranial primitive neuroectodermal tumors (PNET) were retrospectively analyzed. The MRI features were compared with findings in pathology and surgery. In this group, the tumor masses were most commonly found in the semisphere of cerebrum and in the vermis of cerebellum. They were relatively large and 4 were in lobulated shape. All of them had well-defined margins. MR images showed the tumors to be mildly or obviously hypointense on Ti-weighted images and hyperintense on T2-weighted images. Most masses had heterogeneous appearances with some cystic and necrotic areas. Intratumoral haemorrhage and focal calcification were occasionally seen. Mostly, there was no or only mild surrounding edema. Marked inhomogeneous contrast enhancement on MRI was seen in 6 cases except one. Two patients with multiple intracranial metastases were revealed on MR images. In this series, there was good correlation between MRI features and findings in pathology and surgery.These results showed that certain MRI features might suggest the diagnosis of intracranial PNET in adults. MRI is an effective technique to detect these tumors and is helpful to treatment planning and follow-up.

  2. Connexin 43 Is a Potential Prognostic Biomarker for Ewing Sarcoma/Primitive Neuroectodermal Tumor

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    Marilyn M. Bui

    2011-01-01

    Full Text Available Connexins (Cxs are building unit proteins of gap junctions (GJs that are prognostic markers in carcinomas. To investigate the role of Cx in Ewing sarcoma (EWS/primitive neuroectodermal tumor (PNET, we examined the expression of Cx43 and Cx26 in 36 EWS/PNETs and found (1 cytoplasmic Cx43 reactivity in 28/36 (78% cases. (2 Cx43 score was significantly correlated with overall survival (P=.025. The average scores for patients alive and dead at 3 years are 46.08 and 96.98 (P=.004 at 5 years are 46.06 and 96.42 (P=.002. (3 Metastasis had a significant effect on the overall survival (P=.003. (4 Cytoplasmic Cx26 reactivity was detected in 2 of 36 (6% patients who died with metastasis. Our results suggest a possible oncogenic and prognostic role for Cx43 and Cx26 in EWS/PNET. The lack of membranous immunoreactivity suggests that the effect of Cx in EWS/PNET is via a GJ function-independent mechanism.

  3. Primary primitive neuroectodermal tumor of kidney: A rare case report with diagnostic challenge

    Directory of Open Access Journals (Sweden)

    Sunita Kakkar

    2014-01-01

    Full Text Available Primary primitive neuroectodermal tumors (PNETs of the kidney are quite rare and can be mistaken for a wide variety of other small round blue cell tumors which includes rhabdomyosarcoma, Wilm′s tumor, carcinoid, neuroblastoma, clear cell sarcoma of the kidney, lymphoma etc. Renal Ewings/PNET can occur in the age group from 4 to 61 years. Approximately, 90% of Ewing sarcoma (ES/PNET have a specific t(11;22 which results in a chimeric EWS-FLI-1 fusion protein. Immunohistochemical for the carboxy-terminus of FLI-1 is sensitive and highly specific for the diagnosis of ES/PNET. Herein, we have an interesting presentation in a 23-year-old male who came with neck pain and progressive quadriparesis and was diagnosed as a case of poorly differentiated malignant tumor with a differential of lymphoma versus metastatic renal cell carcinoma. The patient′s condition deteriorated fast and he had a rapid downhill course. The final diagnosis of Ewings/PNET was confirmed at autopsy.

  4. Transcription factor KLF7 regulates differentiation of neuroectodermal and mesodermal cell lineages

    Energy Technology Data Exchange (ETDEWEB)

    Caiazzo, Massimiliano, E-mail: caiazzo@igb.cnr.it [Institute of Genetics and Biophysics ' A. Buzzati-Traverso,' CNR, 80131 Naples (Italy); Istituto di diagnosi e cura ' Hermitage Capodimonte,' 80131 Naples (Italy); Colucci-D' Amato, Luca, E-mail: luca.colucci@unina2.it [Institute of Genetics and Biophysics ' A. Buzzati-Traverso,' CNR, 80131 Naples (Italy); Dipartimento di Scienze della Vita, Seconda Universita di Napoli, 81100 Caserta (Italy); Esposito, Maria T., E-mail: maria_teresa.esposito@kcl.ac.uk [CEINGE Biotecnologie Avanzate, 80145 Naples (Italy); Parisi, Silvia, E-mail: parisi@ceinge.unina.it [CEINGE Biotecnologie Avanzate, 80145 Naples (Italy); Stifani, Stefano, E-mail: stefano.stifani@mcgill.ca [Centre for Neuronal Survival, Montreal Neurological Institute, McGill University, Montreal, Quebec, Canada H3A 2B4 (Canada); Ramirez, Francesco, E-mail: francesco.ramirez@mssm.edu [Department of Pharmacology and Systems Therapeutics, Mount Sinai School of Medicine, New York, NY 10029 (United States); Porzio, Umberto di, E-mail: diporzio@igb.cnr.it [Institute of Genetics and Biophysics ' A. Buzzati-Traverso,' CNR, 80131 Naples (Italy)

    2010-08-15

    Previous gene targeting studies in mice have implicated the nuclear protein Krueppel-like factor 7 (KLF7) in nervous system development while cell culture assays have documented its involvement in cell cycle regulation. By employing short hairpin RNA (shRNA)-mediated gene silencing, here we demonstrate that murine Klf7 gene expression is required for in vitro differentiation of neuroectodermal and mesodermal cells. Specifically, we show a correlation of Klf7 silencing with down-regulation of the neuronal marker microtubule-associated protein 2 (Map2) and the nerve growth factor (NGF) tyrosine kinase receptor A (TrkA) using the PC12 neuronal cell line. Similarly, KLF7 inactivation in Klf7-null mice decreases the expression of the neurogenic marker brain lipid-binding protein/fatty acid-binding protein 7 (BLBP/FABP7) in neural stem cells (NSCs). We also report that Klf7 silencing is detrimental to neuronal and cardiomyocytic differentiation of embryonic stem cells (ESCs), in addition to altering the adipogenic and osteogenic potential of mouse embryonic fibroblasts (MEFs). Finally, our results suggest that genes that are key for self-renewal of undifferentiated ESCs repress Klf7 expression in ESCs. Together with previous findings, these results provide evidence that KLF7 has a broad spectrum of regulatory functions, which reflect the discrete cellular and molecular contexts in which this transcription factor operates.

  5. Primary malignant neuroectodermal tumor of the ileum with predominantly uncommon pseudopapillary architecture.

    Science.gov (United States)

    Zhao, Zhihua; Zhang, Dandan; Li, Wencai; Zhang, Lan; Li, Zhen; Zhou, Jun

    2014-01-01

    A malignant gastrointestinal neuroectodermal tumor (GNET), a distinctive entity covering the characteristics of clear cell sarcoma (CCS) of gastrointestinal tract described recently, arising primarily in the ileum of a 33-year-old woman is reported. Histologically, the neoplasm involved the full thickness of the intestinal wall. Tumor cells, mainly displayed epithelioid or polygonal appearance with oval or round nuclei, arranged in strand, nested, and solid pattern with prominent pseudopapillary architecture instead of the familiar histological image with multinucleated osteoclast-like giant cells. They were positive for vimentin, S-100, synaptophysin, CD56 and CD99 protein, but negative for AE1/AE3, EMA, CEA, LCA, Desmin, CK7, CK20, Villin, CgA, CD117, Dog-1, GFAP, Melan-A, HMB-45, CD34, CR, WT1, D2-40. Fluorescence in situ hybridization (FISH) showed the presence of chromosomal translocation involving EWSR. The patients lived through a calm period after a tumor resection and 4 cycles of chemotherapy combining ifosfamide and epirubicin. This case demonstrates that GNET is a rare tumor in gastrointestinal tract, and furthermore, various misleading histological characteristics should been taken into consideration in the diagnosis.

  6. Anterior knee pain

    Energy Technology Data Exchange (ETDEWEB)

    LLopis, Eva [Hospital de la Ribera, Alzira, Valencia (Spain) and Carretera de Corbera km 1, 46600 Alzira Valencia (Spain)]. E-mail: ellopis@hospital-ribera.com; Padron, Mario [Clinica Cemtro, Ventisquero de la Condesa no. 42, 28035 Madrid (Spain)]. E-mail: mario.padron@clinicacemtro.com

    2007-04-15

    Anterior knee pain is a common complain in all ages athletes. It may be caused by a large variety of injuries. There is a continuum of diagnoses and most of the disorders are closely related. Repeated minor trauma and overuse play an important role for the development of lesions in Hoffa's pad, extensor mechanism, lateral and medial restrain structures or cartilage surface, however usually an increase or change of activity is referred. Although the direct relation of cartilage lesions, especially chondral, and pain is a subject of debate these lesions may be responsible of early osteoarthrosis and can determine athlete's prognosis. The anatomy and biomechanics of patellofemoral joint is complex and symptoms are often unspecific. Transient patellar dislocation has MR distinct features that provide evidence of prior dislocation and rules our complication. However, anterior knee pain more often is related to overuse and repeated minor trauma. Patella and quadriceps tendon have been also implicated in anterior knee pain, as well as lateral or medial restraint structures and Hoffa's pad. US and MR are excellent tools for the diagnosis of superficial tendons, the advantage of MR is that permits to rule out other sources of intraarticular derangements. Due to the complex anatomy and biomechanic of patellofemoral joint maltracking is not fully understood; plain films and CT allow the study of malalignment, new CT and MR kinematic studies have promising results but further studies are needed. Our purpose here is to describe how imaging techniques can be helpful in precisely defining the origin of the patient's complaint and thus improve understanding and management of these injuries.

  7. The anterior cingulate cortex

    Directory of Open Access Journals (Sweden)

    Pavlović D.M.

    2009-01-01

    Full Text Available The anterior cingulate cortex (ACC has a role in attention, analysis of sensory information, error recognition, problem solving, detection of novelty, behavior, emotions, social relations, cognitive control, and regulation of visceral functions. This area is active whenever the individual feels some emotions, solves a problem, or analyzes the pros and cons of an action (if it is a right decision. Analogous areas are also found in higher mammals, especially whales, and they contain spindle neurons that enable complex social interactions. Disturbance of ACC activity is found in dementias, schizophrenia, depression, the obsessive-compulsive syndrome, and other neuropsychiatric diseases.

  8. Primitive mediastinal neuroectodermal tumor with cervical extension: a case report; Tumor neuroectodermico primitivo mediastinico con extension cervical. A proposito de un caso

    Energy Technology Data Exchange (ETDEWEB)

    Marcuello, P.; Lopez, M. V.; Campo, L. del [Hospital Universitario de la Princesa. Madrid (Spain)

    2000-07-01

    Primitive neuroectodermal tumors (PNET) are rare lesions belonging to the Ewing's family of tumors. The major histological features are the presence of small, round cells and different degrees of neuroectodermal differentiation. In most cases, the radiological findings are nonspecific, and diagnosis requires the performance of electron microscopy and immunohistochemical studies. We present a case of PNET in a 29-year-old man with no personal medical history of interest, and review the literature on tumors of this type, which focuses very little on the radiological aspects. (Author) 12 refs.

  9. Multidisciplinary management of anterior diastemata

    DEFF Research Database (Denmark)

    Furuse, Adilson Yoshio; Herkrath, Fernando José; Franco, Eduardo Jacomino

    2007-01-01

    Anterior diastemata may compromise the harmony of a patient's smile. Consideration of etiologic factors, previous gingival conditioning, and individual treatment planning are essential in the proper management of anterior diastemata. An integrated orthodontic-restorative approach may enhance...... the aesthetic results when orthodontic therapy itself is not feasible. This article presents integrated orthodonticrestorative solutions of anterior diastemata, associated with the conditioning of the gingival tissue with composite resin, and discusses the most relevant aspects related to their etiology...

  10. A novel splice mutation in the TP53 gene associated with Leydig cell tumor and primitive neuroectodermal tumor

    DEFF Research Database (Denmark)

    Stecher, Chalotte Willemann; Grønbaek, Kirsten; Hasle, Henrik

    2008-01-01

    A 20-month-old boy presented with precocious puberty due to a Leydig cell tumor, and at the age of 6 years with a primitive neuroectodermal brain-tumor (PNET). A novel splice site mutation of the TP53-gene, likely to be associated with a nonfunctional protein, was found in the proband, his father...... and younger sister, but only the proband has so far developed malignancy. The clinical phenotype in the boy is suggestive of Li-Fraumeni syndrome, but the family does not strictly conform to the canonical definition. Udgivelsesdato: 2008-Mar...

  11. Primitive neuroectodermal tumor of the central nervous system with glial differentiation: a FISH study of an adult case.

    Science.gov (United States)

    Alameda, F; Lloreta, J; Ariza, A; Salido, M; Espinet, B; Baro, T; Garcia-Fructoso, G; Galito, E; Munne, A; Cruz Sanchez, F F; Sole, F; Serrano, S

    2007-01-01

    Primitive neuroectodermal tumors (PNETs) of the central nervous system (CNS), a rare occurrence in adults, may show glial differentiation and can be misinterpreted as pure astrocytic neoplasms. Few fluorescence in situ hybridization (FISH) studies have been carried out on these tumors; isochromosome 17q was found to be the major chromosomal abnormality. We present the case of an adult in which we performed a FISH study of both the glial and neuronal components. A complex array of FISH changes, not including an isochromosome 17q were identified.

  12. A case of primary mediastinal Ewing′s sarcoma /primitive neuroectodermal tumor presenting with initial compression of superior vena cava

    Directory of Open Access Journals (Sweden)

    Alessia Reali

    2013-01-01

    Full Text Available Ewing′s sarcomas and peripheral primitive neuroectodermal tumors (ES/PNETs are high grade malignant neoplasms. These malignancies are characterized by a chromosome 22 rearrangement, arise from bone or soft tissue, predominantly affect children and young adults, and are grouped in the Ewing family of tumors. Multimodality treatment programs are the treatment of choice. Primary localization of ES/PNET in the mediastinum is extremely rare. We describe a case of ES/PNET presenting as a mediastinal mass with tracheal compression and initial signs of superior vena cava in a 66-year-old woman.

  13. Primary Primitive Neuroectodermal Tumor of the Conus Medullaris in an Elderly Patient: A Case Report and Review of the Literature

    Directory of Open Access Journals (Sweden)

    H. Shimosawa

    2011-05-01

    Full Text Available Primary spinal primitive neuroectodermal tumors (PNETs are very rare conditions. Most of these tumors occur in children and young adults. A 63-year-old man with a primary spinal PNET in the conus medullaris from the L1 to L2 level is presented in this report. The optimal treatment of primary spinal PNETs is yet unknown. Surgical resection, radiation therapy, and chemotherapy have been advocated for the treatment of spinal PNET based on PNETs at other sites. However, the outcome is very poor. There are a few reports of cases with long-term survival and no recurrence. In these patients, en bloc resections were performed.

  14. Bilateral Ewing Sarcoma/Primitive Neuroectodermal Tumor of the Breast: A Very Rare Entity and Review of the Literature

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    N. Majid

    2013-01-01

    Full Text Available Peripheral primitive neuroectodermal tumors (PNET are rare malignant tumors, affecting mostly children and adolescents and have been described in breast in eight case reports only. In this paper, we present a case of bilateral mammary ES/PNET where distinction between primary and metastatic diseases was discussed through a literature review. The aim of this work is to demonstrate that although rare, the possibility of PNET should be kept in mind while evaluating a palpable breast abnormality in a young female.

  15. A case of fatal late vasospasm in a patient with a recurrent, supratentorial rhabdoid primitive neuroectodermal tumor: possible molecular implications.

    Science.gov (United States)

    Miscusi, Massimo; Martino, Luca De; Antonelli, Manila; Mangino, Giorgio; Ricciardi, Luca; Spinelli, Gianpaolo; Forcato, Stefano; Calogero, Antonella; Petrozza, Vincenzo; Ragona, Giuseppe; Raco, Antonino

    2014-01-01

    We report the case of a 44-year-old man who experienced a fatal and untreatable delayed vasospasm after resection of a recurrent temporal IV grade primitive neuroectodermal tumor (PNET). The histological analysis demonstrated a rare rhabdoid variant of the tumor with a diffuse myxoid degeneration; molecular investigations demonstrated an upregulation of IL-1β and IL-6 expression in the recurrence. We reviewed the pathophysiology of the vasospasm that occurs after tumors resection, and due to the rarity of case, we speculated on the possibility that specific histological and molecular features of the tumor could have contributed to the delayed and fatal complication.

  16. Antracyclin toxicity in a child with primitive neuroectodermal tumor of the chest wall with and brain metastasis.

    Science.gov (United States)

    Atas, Erman; Kesik, Vural

    2015-01-01

    Chemotherapy regimens, including doxorubicin used in primitive neuroectodermal tumor's (PNET) treatment can cause life-threatening disorders in cardiac functions. Follow-up of cardiac functions in the clinical course is very important during treatment with ejection fraction (EF) and shortening fraction (SF). However, sometimes the detection of cardiac failure with EF and SF cannot be possible. In this condition, we may need new evaluation test. Herein, we wanted to present a child with PNET of the chest wall suffered from antracycline toxicity and indicate that close monitoring of cardiac function could be important.

  17. Primitive neuroectodermal tumor of the kidney with inferior vena cava tumor thrombus during pregnancy response to sorafenib

    Institute of Scientific and Technical Information of China (English)

    WU Yun-jian; YANG Yu-ru; ZENG Hao; ZHU Yu-chun; CHEN Hui; HUANG Ying; WEI Qiang; CHEN Hui-jiao; XIE Xi; LI Xiang; ZHOU Qiao

    2010-01-01

    @@ Primitive neuroectodermal tumor (PNET) most often presents as a bone or soft tissue mass in the trunk or axial skeleton in adolescents and young adults.1 It is highly aggressive and rarely arises in the kidney.2 A combined therapy, consisting of surgical resection,chemotherapy and radiotherapy, is needed to treat this tumor but long-term survival remains poor.3,4 In the current study, we describe a case of 26-year-old woman (primigravida) who presented with a PNET of the right kidney with inferior vena cava (IVC) tumor thrombus.

  18. Primary Ewing's sarcoma/primitive neuroectodermal tumor of the urogenital tract in children

    Institute of Scientific and Technical Information of China (English)

    SONG Hong-cheng; SUN Ning; ZHANG Wei-ping; HUANG Cheng-ru

    2012-01-01

    Background Primary Ewing's sarcoma/primitive neuroectodermal tumor (ES/PNET) of urogenital tract is a rare condition with non-specific clinical presentations,which can make it difficult to diagnose.In this study,we summarize the clinical presentation,pathological features,therapeutic strategies,and prognosis of ES/PNET.Methods Clinical information on two cases of ES/PNET in the penis and ureter was analyzed,and relevant literature was reviewed.Results ES/PNET was confirmed pathologically,immunohistochemically and via molecular biology techniques in the penis (n=1) and ureter (n=1).In one case,a tumor was found at the base of penis,which had invaded the corpus cavernosum,and resulted in a massive enlargement of the penis.This tumor was initially diagnosed as an endocrine disorder.However,a confirmed diagnosis was made 11 months later when massive metastases in both lungs were noted.A tumor biopsy was performed to confirm the diagnosis,and chemotherapy with a CAV (cyclophosphamide+doxorubicin+vincristine) + IE (ifosfamide+ etoposide) regimen for 9 months was prescribed.In the second case,a child was admitted due to abdominal pain and a hydroureter in the right kidney,as determined by ultrasonography.A tumor was found in the right ureter at the level of iiiac vessels.Removal of the tumor and ureteral anastomosis were performed,and chemotherapy with CAV+IE for 8 months were prescribed.Both patients are currently being followed-up closely.Conclusions ES/PNET is a highly malignant tumor and has poor prognosis.Pre-operative diagnosis of ES/PNET of urogenital tract is difficult and largely depends on pathology,immunohistochemistry,and,if applicable,molecular biology.Comprehensive therapy may include surgery,chemotherapy and radiotherapy.

  19. Ewing sarcoma/primitive neuroectodermal tumor of the kidney: clinicopathologic analysis of 34 cases.

    Science.gov (United States)

    Karpate, Arti; Menon, Santosh; Basak, Ranjan; Yuvaraja, Thyavihalli B; Tongaonkar, Hemant B; Desai, Sangeeta B

    2012-08-01

    The present study describes the clinicopathologic analysis of 34 cases of Ewing sarcoma/primitive neuroectodermal tumor occurring in the kidney. The patients were 21 males and 13 females with an age range of 6 to 44 years. Clinically, patients presented with multiple symptoms including hematuria, pain, and/or lump in the abdomen. Nephrectomy was performed in most of the cases. Grossly, whole of the renal parenchyma was involved by a variegated tumor. Histologically, the tumor was composed of monomorphic, small, and round cells arranged in a variety of patterns. Rosettes, geographical areas of necrosis, and arborizing vascular pattern were the prominent histologic features. The nucleus was monomorphic and round. Anisonucleosis was also noted in some cases. The nucleus was mostly hyperchromatic. A mixture of hyperchromatic and powdery chromatin was noted in few cases. Immunohistochemically, MIC2 (CD99) was positive in 32 of 34 cases followed by neuron-specific enolase (9/12 cases), vimentin (8/14 cases), synaptophysin (1/8 cases), and S-100 protein (1/4 cases). Molecular analysis by reverse transcriptase-polymerase chain reaction that was carried out in 26 cases revealed presence of EWS-FLI-1 type 1 translocation in 12 cases, EWS-FLI-1 type 2 translocation in 10 cases, and both type 1 and type 2 EWS-FLI-1 translocation in 2 cases. Two cases did not demonstrate any translocation. Follow-up data were available for 17 of 34 cases. Local recurrence of the tumor was seen in 4 patients, and 10 patients were recorded to have distant metastasis in various organs, such as lung, bone, and lymph node, during the course of the disease.

  20. CT Features and Pathological Correlation of Primitive Neuroectodermal Tumor of the Kidney.

    Science.gov (United States)

    Dong, Junqiang; Xing, Jingjing; Limbu, Hangsha Hang; Yue, Songwei; Su, Lei; Zhang, Dandan; Gao, Jianbo

    2015-09-01

    The purpose of the study was to analyze the computed tomography (CT) findings of primitive neuroectodermal tumor (PNET) of the kidney and correlate them pathologically. Ten cases of pathologically confirmed renal PNET were collected and retrospectively reviewed. The CT features that were analyzed include tumor size, shape, margins, density, nature of enhancement, presence of thrombosis, and metastasis, etc. These parameters were correlated with pathological findings and combined with literature review. The median age of the patients was 30 years. CT images showed solitary, large, ill-defined, irregular, or lobulated heterogeneous mass. Invasive growth toward the renal cortex and pelvis with renal cortical interruptions were seen in eight cases with one case exhibiting invasion that extended beyond the renal capsule with soft tissue seen in the perirenal fat pace. The tumors were confined to the kidney contour with enlargement of kidney in six of the cases. Cystic changes with mural nodules were detected in three cases. Eight cases showed persistent moderate enhancement during the nephrographic phase. Irregular septum-like structures were seen in four cases. Thrombosis was detected in eight cases. Lymph node metastasis was detected in eight cases with bilateral lung metastasis in two and bone metastasis in one. Renal PNET is a rare highly aggressive disease affecting younger people. It should be considered as a strong differential when well confined, yet large tumors that cause enlargement of the kidney are seen and also when tumors expressing cystic changes along with mural nodules are seen. Although renal PNET has certain other characteristic CT features, pathological and immunohistochemistry report must also be sought for definitive diagnosis.

  1. Clinical outcomes of children and adults with central nervous system primitive neuroectodermal tumor.

    Science.gov (United States)

    Lester, Rachael A; Brown, Lindsay C; Eckel, Laurence J; Foote, Robert T; NageswaraRao, Amulya A; Buckner, Jan C; Parney, Ian F; Wetjen, Nicholas M; Laack, Nadia N

    2014-11-01

    Central nervous system primitive neuroectodermal tumors (CNS PNETs) predominantly occur in children and rarely in adults. Because of the rarity of this tumor, its outcomes and prognostic variables are not well characterized. The purpose of this study was to evaluate clinical outcomes and prognostic factors for children and adults with CNS PNET. The records of 26 patients (11 children and 15 adults) with CNS PNET from 1991 to 2011 were reviewed retrospectively. Disease-free survival (DFS) and overall survival (OS) were estimated with the Kaplan-Meier method, and relevant prognostic factors were analyzed. For the cohort, both the 5-year DFS and the OS were 46 %. For pediatric patients, the 5-year DFS was 78 %; for adult patients, it was 22 % (P = 0.004). Five-year OS for the pediatric and adult patients was 67 and 33 %, respectively (P = 0.07). With bivariate analysis including chemotherapy regimen (high dose vs. standard vs. nonstandard) or risk stratification (standard vs. high) and age, the increased risk of disease recurrence in adults persisted. A nonsignificant tendency toward poorer OS in adult patients relative to pediatric patients also persisted. High-dose chemotherapy with stem cell rescue was associated with a statistically significant improvement in OS and a tendency toward improved DFS, although the findings were mitigated when the effect of age was considered. Local recurrence was the primary pattern of treatment failure in both adults and children. Our results suggest that adult patients with CNS PNETs have inferior outcomes relative to the pediatric cohort. Further research is needed to improve outcomes for CNS PNET in populations of all ages.

  2. [Anterior cervical hypertrichosis: case report].

    Science.gov (United States)

    Orozco-Gutiérrez, Mario H; Sánchez-Corona, José; García-Ortiz, José E; Castañeda-Cisneros, Gema; Dávalos-Rodríguez, Nory O; Corona-Rivera, Jorge R; García-Cruz, Diana

    2016-10-01

    The non-syndromic anterior cervical hypertrichosis (OMIM N° 600457) is a genetic disorder characterized by a patch of hair at the level of the laryngeal prominence. We present a 12-year-old boy with anterior cervical hypertrichosis and mild generalized hypertrichosis. He has no neurological, ophthalmological or skeletal anomalies. The clinical follow up is 10 years.

  3. Inestabilidad Anterior de Hombro

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    Pablo David Flint Kuran

    2013-11-01

    Full Text Available In­tro­duc­ción La luxación recidivante de hombro es una patología frecuente en pacientes jóvenes, laboralmente activos. Existen numerosas técnicas quirúrgicas para la inestabilidad glenohumeral. La técnica de Bristow, discutida por no ser anatómica y por sus complicaciones, continúa vigente debido al bajo índice de reluxaciones. Los objetivos fueron determinar el índice de recidiva, alteraciones funcionales e índice de consolidación del injerto. Materiales­ y­ Métodos Se evaluaron 24 pacientes del sexo masculino, de entre 19 y 40 años, operados por luxación anterior recidivante de hombro según la técnica de Bristow, entre enero de 2003 y agosto de 2011. Se evaluó la tasa de reluxación, la función articular según el puntaje de Constant y el posicionamiento del injerto con respecto a la superficie articular con tomografía y radiografías para evaluar la consolidación del injerto. Se registraron las complicaciones quirúrgicas. Resultados ­Todos los pacientes eran hombres, con rango de edad de 19 a 40 años. La causa fue traumática en 24 pacientes. Dieciséis pacientes presentaron más de 3 episodios de luxación prequirúrgicos. Según la escala de Constant, 21 obtuvieron entre 96 y 100 puntos, y los restantes, entre 90 y 95 puntos. No hubo nuevos episodios de luxaciones. La tomografía mostró la consolidación en todos los casos. Un paciente tuvo una imagen osteolítica alrededor del tornillo, sin compromiso funcional del hombro. Conclusión La técnica de Bristow para tratar la luxación anterior recidivante de hombro provocó un bajo índice de complicaciones, con resultados funcionales entre excelentes y buenos. No hubo episodios de reluxación y se logró la consolidación del injerto óseo en todos los casos.

  4. Characterization of bbtTICAM from amphioxus suggests the emergence of a MyD88-independent pathway in basal chordates

    Institute of Scientific and Technical Information of China (English)

    ManyiYang; ShaochunYuan; Shengfeng Huang; Jun Li; Liqun Xu; Huiqing Huang; Xin Tao; Jian peng; Anlong Xu

    2011-01-01

    The MyD88-independent pathway,one of the two crucial TLR signaling routes,Is thought to be a vertebrate innovation.However,a novel Toll/interleukin-1 receptor (TIR) adaptor,designated bbtTICAM,which was identified in the basal chordate amphioxus,links this pathway to invertebrates.The protein architecture of bbtTICAM is similar to that of vertebrate TICAM1 (TIR-containing adaptor molecule-1,also known as TRIF),while phylogenetic analysis based on the TIR domain indicated that bbtTICAM is the oldest ortholog of vertebrate TICAMI and TICAM2(TIR-containing adaptor molecule-2,also known as TRAM).Similar to human TICAM1,bbtTICAM activates NF-κB in a MyD88-independent manner by interacting with receptor interacting protein (RIP) via its RHIM motif.Such activation requires bbtTICAM to form homodimers in endosomes,and it may be negatively regulated by amphioxus SARM (sterile a and armadillo motif-containing protein) and TRAF2.However,bbtTICAM did not induce the production of type I interferon.Thus,our study not only presents the ancestral features of vertebrate TICAM I and TICAM2,but also reveals the evolutionary origin of the MyD88-independent pathway from basal chordates,which will aid in understanding the development of the vertebrate TLR network.

  5. 外周性原始神经外胚层瘤的诊治体会%Diagnosis and treatment for primitive neuroectodermal tumors

    Institute of Scientific and Technical Information of China (English)

    魏剑锋; 欧阳宛炯; 庄雪瑜

    2011-01-01

    @@ 原始神经外胚层瘤(primitive neuroectodermal tumors,PNETs)临床上少见.现回顾性分析2000年4月-2009年6月笔者医院收治的4例PNETs的临床表现、病理学特点、治疗体会,报告如下:

  6. Primary peripheral primitive neuroectodermal tumor/Ewing's tumor of the testis in a 46-year-old man-differential diagnosis and review of the literature.

    Science.gov (United States)

    Heikaus, Sebastian; Schaefer, Karl-Ludwig; Eucker, Jan; Hogrebe, Esther; Danebrock, Raihanatou; Wai, Daniel H; Krenn, Veit; Gabbert, Helmut E; Poremba, Christopher

    2009-06-01

    Peripheral primitive neuroectodermal tumor/Ewing's tumors are rare bone and soft tissue malignancies with a highly aggressive clinical course and early metastases occurring at multiple peripheral sites. Here, we present for the first time a case of a 46-year-old man with a primary peripheral primitive neuroectodermal tumor/Ewing's tumor of the testis. The diagnosis of peripheral primitive neuroectodermal tumor/Ewing's tumor was established by histology, immunohistochemistry, and molecular pathology. The tumor revealed a rapid progress in 2 months' time. Therefore, the patient was included in the EURO-E.W.I.N.G.99 study and was placed on chemotherapy. However, the tumor progressed during ongoing therapy, and the patient died in March 2008. In conclusion, though being reported here for the first time, peripheral primitive neuroectodermal tumor/Ewing's tumors should be considered in the differential diagnosis of blue round cell tumors of the testis. A rapid and correct diagnosis of this entity is crucial for fast and accurate therapy, which is stressed by the fatal case presented here.

  7. Tissue elasticity regulated tumor gene expression: implication for diagnostic biomarkers of primitive neuroectodermal tumor.

    Directory of Open Access Journals (Sweden)

    Long T Vu

    Full Text Available The tumor microenvironment consists of both physical and chemical factors. Tissue elasticity is one physical factor contributing to the microenvironment of tumor cells. To test the importance of tissue elasticity in cell culture, primitive neuroectodermal tumor (PNET stem cells were cultured on soft polyacrylamide (PAA hydrogel plates that mimics the elasticity of brain tissue compared with PNET on standard polystyrene (PS plates. We report the molecular profiles of PNET grown on either PAA or PS.A whole-genome microarray profile of transcriptional expression between the two culture conditions was performed as a way to probe effects of substrate on cell behavior in culture. The results showed more genes downregulated on PAA compared to PS. This led us to propose microRNA (miRNA silencing as a potential mechanism for downregulation. Bioinformatic analysis predicted a greater number of miRNA binding sites from the 3' UTR of downregulated genes and identified as specific miRNA binding sites that were enriched when cells were grown on PAA-this supports the hypothesis that tissue elasticity plays a role in influencing miRNA expression. Thus, Dicer was examined to determine if miRNA processing was affected by tissue elasticity. Dicer genes were downregulated on PAA and had multiple predicted miRNA binding sites in its 3' UTR that matched the miRNA binding sites found enriched on PAA. Many differentially regulated genes were found to be present on PS but downregulated on PAA were mapped onto intron sequences. This suggests expression of alternative polyadenylation sites within intron regions that provide alternative 3' UTRs and alternative miRNA binding sites. This results in tissue specific transcriptional downregulation of mRNA in humans by miRNA. We propose a mechanism, driven by the physical characteristics of the microenvironment by which downregulation of genes occur. We found that tissue elasticity-mediated cytokines (TGFβ2 and TNFα signaling

  8. Peripheral primitive neuroectodermal tumor of the urinary bladder in an Arab woman with history of squamous cell carcinoma: a case report

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    Al Meshaan Mohd Khaled

    2009-04-01

    Full Text Available Abstract Introduction Peripheral primitive neuroectodermal tumors of the urinary bladder are rare and tend to occur in an older age group than do their counterparts in bones and soft tissue. Case presentation We report a case of peripheral primitive neuroectodermal tumor of the urinary bladder in a 67-year-old woman of Arab origin. She had undergone transurethral resection followed by chemotherapy because of pulmonary metastasized muscle-invasive squamous cell carcinoma of the bladder in 2005. One year later, she first presented with a history of repeated hematuria in our institution. Performing cystoscopy any tumor could be detected. Control cystoscopy two months later showed a tumor mass of 3 cm in diameter at another location than described for the first tumor. After perforating by transurethral resection partial bladder resection had to be done. Tissue specimen after pathological analysis revealed a peripheral primitive neuroectodermal tumor with tumor cells reactive to cluster of differentiation 99, neuron-specific enolase and S100 protein and stained negative for other markers such as cytokeratins, epithelial membrane antigen, desmin, smooth muscle actin, chromogranin and leucocyte common antigen. Staging computerized tomography was especially free from any hint on organ metastasis, but the patient died due to a cardiac problem only a few months later. Conclusions To the best of our knowledge, we report the eighth case of bladder peripheral primitive neuroectodermal tumors in literature and the first concerning an Arab patient. It is also the first presentation of a peripheral primitive neuroectodermal tumor patient with a history of squamous cell carcinoma of the bladder. As in other cases, expression of single-chain-type 1 glycoprotein and neural markers was positive and the disease was at an advanced stage at the time of diagnosis.

  9. Anterior chamber depth during hemodialysis

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    Gracitelli CPB

    2013-08-01

    Full Text Available Carolina Pelegrini Barbosa Gracitelli,1 Francisco Rosa Stefanini,1 Fernando Penha,1 Miguel Ângelo Góes,2 Sérgio Antonio Draibe,2 Maria Eugênia Canziani,2 Augusto Paranhos Junior1 1Ophthalmology Department, 2Division of Nephrology, Federal University of São Paulo – UNIFESP, São Paulo, Brazil Background: Exacerbation of chronic glaucoma or acute glaucoma is occasionally observed in patients undergoing hemodialysis (HD because of anterior chamber depth changes during this therapy. Purpose: To evaluate anterior chamber depth and axial length in patients during HD sessions. Methods: A total of 67 eyes of 35 patients were prospectively enrolled. Axial length and anterior chamber depth were measured using ultrasonic biometry, and these measures were evaluated at three different times during HD sessions. Body weight and blood pressure pre- and post-HD were also measured. Results: There was no difference in the axial length between the three measurements (P = 0.241. We observed a significantly decreased anterior chamber depth (P = 0.002 during HD sessions. Conclusion: Our results support the idea that there is a change in anterior chamber depth in HD sessions. Keywords: anterior chamber, hemodialysis, axial length, acute angle-closure glaucoma

  10. Diffuse anterior retinoblastoma: current concepts

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    Yang J

    2015-07-01

    Full Text Available Jing Yang,1–3 Yalong Dang,1–3 Yu Zhu,1 Chun Zhang2,3 1Department of Ophthalmology, The First Affiliated Hospital, Zhengzhou University, Zhengzhou City, Henan Province, 2Department of Ophthalmology, Peking University Third Hospital, 3Clinical Stem Cell Research Center, Peking University Third Hospital, Beijing, People’s Republic of China Abstract: Diffuse anterior retinoblastoma is a rare variant of retinoblastoma seeding in the area of the vitreous base and anterior chamber. Patients with diffuse anterior retinoblastoma are older than those with the classical types, with the mean age being 6.1 years. The original cells of diffuse anterior retinoblastoma are supposed to be cone precursor. Patients most commonly present with pseudouveitis, pseudohypopyon, and increased intraocular pressure. The retina under fundus examination is likely to be normal, and the clinical features mimic the inflammation progress, which can often lead to misdiagnosis. The published diffuse anterior retinoblastoma cases were diagnosed after fine-needle aspiration biopsy running the potential risk of inducing metastasis. The most common treatment for diffuse anterior retinoblastoma is enucleation followed by systematic chemotherapy according to the patient’s presentation and clinical course. This review summarizes the recent advances in etiology (including tumorigenesis and cell origin, pathology, diagnosis, differential diagnosis, and new treatment. The challenges of early diagnosis and prospects are also discussed. Keywords: pathology, microenvironment, treatment, diagnosis 

  11. Two cases of peripheral primitive neuroectodermal tumor%外周原始神经外胚瘤2例

    Institute of Scientific and Technical Information of China (English)

    张桂枫; 陈静波; 陈峥; 刘振华; 崔同建

    2010-01-01

    @@ 1 临床资料 患者,男性,57岁,以"右腰部疼痛2天"入院,全身PET-CT:左下腹部小肠壁稍增厚,局部放射性异常浓聚,肝左叶外侧段、右侧肾上腺、左肺上叶高代谢占位,B超引导下行"右肾上腺穿刺术",术后病理:小圆型细胞恶性肿瘤,免疫组织化学:CD99(+++)、EMA(+)、inhibin-(++)、SY(++)、Vimentin(++),CgA、HMB45、PLAP、MyoD1、CD34、Ckpan、Myosin、LCA、S100(-),符合原始神经外胚层肿瘤(primitive neuroectodermal tumor,PNET).

  12. 儿童原始神经外胚层肿瘤%Primitive neuroectodermal tumor in children

    Institute of Scientific and Technical Information of China (English)

    邵虹; 朱铭; 李玉华

    2006-01-01

    原始神经外胚层肿瘤(primitive neuroectodermal tumors,PNETs)是指一组发生于中枢交感神经系统(中央型)和周围肌肉、骨骼组织(周围型),由原始未分化的小圆细胞构成的恶性肿瘤,它在儿童及青少年中发病率较高。自1973年Hart和Earle首先提出颅内PNETs至今,关于其命名一直存在争论,随着免疫组化技术和分子生物学研究的不断进展,不少概念和相关问题也逐渐得以明确和解决。

  13. Primitive neuroectodermal tumor of the maxillary sinus in an elderly male: A case report and literature review

    Energy Technology Data Exchange (ETDEWEB)

    Shah, Saiquat [Dept. of Dental Public Health, Bangladesh Dental College, Dhaka (Bangladesh); Huh, Kyung Hoe; Yi, Won Jin; Heo, Min Suk; Lee, Sam Sun; Choi, Soon Chul [Dept. of Oral and Maxillofacial Radiology and Dental Research Institute, School of Dentistry, Seoul National University, Seoul (Korea, Republic of)

    2014-12-15

    Primitive neuroectodermal tumor (PNET), which belongs to the Ewing's sarcoma (ES) family of tumors, is mainly seen in children and young adults. PNETs are extremely rare in the maxilla. Here, we report a case of PNET of the left maxillary sinus in an elderly male. Magnetic resonance imaging (MRI) revealed a slightly enhanced solid mass occupying the left maxillary sinus and infiltrating into the retroantral space. A partial maxillectomy was performed. Despite postoperative chemotherapy, follow-up computed tomography (CT) and MRI revealed a nodal metastasis in the submandibular space. Neck dissection was performed. However, the patient died 10 months after the second surgery because of distant metastasis to the liver. MRI and CT were particularly useful in detecting the extent of the tumor, recurrence, and metastasis. Further, a literature review of the previously reported PNET cases of the maxilla was carried out. In this paper, we also discuss the current approach for the diagnosis and management of these tumors.

  14. Targeting VEGF-VEGFR Pathway by Sunitinib in Peripheral Primitive Neuroectodermal Tumor, Paraganglioma and Epithelioid Hemangioendothelioma: Three Case Reports

    Directory of Open Access Journals (Sweden)

    Tiziana Prochilo

    2013-02-01

    Full Text Available Sunitinib malate (Sutent™; Pfizer Inc., New York, N.Y., USA is a small molecule kinase inhibitor with activity against a number of tyrosine kinase receptors, including vascular endothelial growth factor receptors, stem-cell factor receptor, and platelet-derived growth factor receptors alpha and beta. Sunitinib, registered for the treatment of renal cell carcinoma and gastrointestinal stromal tumors, has recently been approved for the treatment of patients with advanced pancreatic neuroendocrine tumors. Peripheral primitive neuroectodermal tumor (pPNET, paraganglioma (PGL and epithelioid hemangioendothelioma (EHE are rare tumors in which there is an overexpression of pro-angiogenic factors and in which a high intratumoral microvessel density is a significant poor prognostic factor. On the basis of this preclinical rationale and the lack of effective treatments in pre-treated advanced stages of these rare diseases, we report our interesting experience of pPNET, PGL and EHE treatment with sunitinib.

  15. A case report of primitive neuroectodermal tumor%多发原始神经外胚瘤1例

    Institute of Scientific and Technical Information of China (English)

    吴盛荣; 黄晓龙; 高伯元

    2003-01-01

    原始神经外胚瘤(Primitive neuroectodermal tumors,PNETs)又称蓝色瘤(Blue tumors),是一组未分化的小细胞恶性肿瘤,1993年版修订的WHO中枢神经系统肿瘤的组织学分类中将原始神经外胚叶肿瘤单独提出来,列入神经上皮源性胚胎性肿瘤内。我院收治1例罕见老年性多发性的原始神经外胚瘤病人,现报告如下。

  16. A case series of transformation of teratoma to primitive neuroectodermal tumor: evolving management of a rare malignancy

    Directory of Open Access Journals (Sweden)

    Richard F. Dunne

    2014-03-01

    Full Text Available Primitive neuroectodermal tumor (PNET is a pathologic diagnosis that encompasses several different tumor types, including central nervous system tumors and Ewing’s sarcomas. Teratoma, a common element of germ cell tumor (GCT, has the ability to transform to malignant PNET in a small number of patients. Making a definitive diagnosis of PNET is difficult given its deviation from elements of GCT and its non-specific pathologic findings. Establishing the diagnosis is crucial as PNETs respond poorly to standard platinum-based chemotherapy used for treatment of GCT. Primary treatment for PNET is surgical, though this is often not feasible in many patients due to extensive disease at diagnosis. As an alternative, chemotherapy regimens traditionally used for Ewing’s sarcoma, such as vincristine, doxorubicin and cyclophosphamide alternating with ifosfamide and etoposide, have shown limited efficacy in the neoadjuvant, adjuvant, and palliative settings. Future research should delineate the genetic underpinnings of PNET and develop therapeutic options accordingly.

  17. Primary extraskeletal peripheral primitive neuroectodermal tumor of subcutaneous tissue neck in a young adult: A rare case report

    Directory of Open Access Journals (Sweden)

    Sumeet Aggarwal

    2016-01-01

    Full Text Available Primitive neuroectodermal tumors (PNETs are poorly differentiated small round cell neoplasms which primarily affect childhood age and very rarely seen in adults. Peripheral PNET (pPNET cases are very rare compared to central PNET, and most of them originate from neural crest cells located outside the central nervous system. We report a case of large extraosseous pPNET arising from subcutaneous tissue of left side neck in a young male patient. Despite aggressive inherent nature of histology, structural complexity of neck area and poor predictors like tumor size >6 cm, extraosseous nature, this case advocate that complete resolution is possible with aggressive multimodal treatment including surgery, radiation therapy, and chemotherapy. Other major concerns in such cases of pPNET are difficulty in diagnosis due to low incidence, unpredictable site involvement, histological similarity with other round cell tumors and lack of established treatment guidelines.

  18. Peripheral primitive neuroectodermal tumour in a lumbar vertebra and the liver of a dromedary camel (Camelus dromedarius).

    Science.gov (United States)

    Weiss, R; Walz, P H

    2009-01-01

    A 9-year-old castrated male dromedary camel developed weakness and ataxia, progressing to sternal recumbency and hindlimb paralysis. Necropsy revealed multiple liver tumours and a mass in the 3rd lumbar vertebra, compressing the spinal cord. The hepatic and vertebral masses consisted of uniform sheets of primitive cells, with perivascular pseudorosettes and small numbers of neuroblastic Homer-Wright rosettes. Immunohistochemically, the tumour cells were uniformly positive for vimentin and variably positive for neuron-specific enolase and glial fibrillary acidic protein. The histopathological and immunohistochemical findings indicated a peripheral primitive neuroectodermal tumour (pPNET) exhibiting neuroblastic, glial and ependymomatous differentiation, probably reflecting the tumour's primitive multipotential neuroepithelial nature. To the authors' knowledge, this is the first reported case in the camel of a pPNET, presumably intraosseous in origin with hepatic metastasis, and morphologically similar to Ewing's sarcoma in man.

  19. A prognostic analysis of primitive neuroectodermal tumor for postoperative recurrent patients and a literature review

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    Ling-ling GAO

    2016-08-01

    Full Text Available Objective  To analyze the prognostic factors of primitive neuroectodermal tumor (PNET for postoperative recurrent patients. Methods  Thirty patients admitted from Jun. 2008 to Dec. 2014 and diagnosed as PNET were retrospectively analyzed. Postoperative recurrence happened in all of the patients, of whom 2 received surgery alone, 15 received surgery and chemotherapy without radiotherapy, 3 received surgery and radiotherapy without chemotherapy, and 10 received surgery, radiotherapy and chemotherapy. Kaplan and Meier method was employed to draw the survival curve of the postoperative recurrent PNET patients, and to evaluate the effects of sex, age, radical resection, therapeutic method and relapse-free interval (RFI on the overall survival (OS. Log-rank method was used to test the significance of differences, and Cox regression was used to analyze all the factors listed above. The literatures related to PNET published domestically and abroad in recent 20 years were reviewed. Results  The overall median survival time was 30 months. The OS rates of 1, 3 and 5 years were 80.0%, 44.1% and 20.7%. Local recurrence happened in 26 patients (86.7%, and distant recurrence in 4 patients (13.3%. The overall median RFI was 4 months, and the RFI rates of 6 months, 1 year and 2 years were 33.3%, 16.7% and 6.7%, respectively. The overall median recurrence-free survival (RFS time was 14 months, and the PRS rates of 1, 3 and 5 years were 60.7%, 16.9% and 8.4%, respectively. The univariate analysis showed that radical resection, radiotherapy and RFI≥6 months predicted significantly better outcome (P=0.033, P=0.006 and P=0.001. The multivariate analysis revealed that radiotherapy and RFI≥6 months were the independent prognosis factors (P=0.047 and P=0.012, respectively. One thousand six hundred and eight cases of recurrent PNET patients were reported abroad since A.D. 2000. The initial recurrence was most often distant only (1089 cases, followed by local

  20. Supratentorial primitive neuroectodermal tumors:repotr of 3 cases%幕上原始神经外胚层肿瘤3例报告

    Institute of Scientific and Technical Information of China (English)

    孙君昭; 田增民; 于新; 王亚明; 卢旺盛

    2006-01-01

    幕上原始神经外胚层肿瘤(SUPRATENTARIAL PRIMITIVE NEUROECTODERMAL TUMOR,spnet)是神经系统胚胎性肿瘤中的一种类型,为罕见的颅内恶性肿瘤。我科近期收治了3例SPNET患者,现报告如下。

  1. Detection of N-Glycolyl GM3 Ganglioside in Neuroectodermal Tumors by Immunohistochemistry: An Attractive Vaccine Target for Aggressive Pediatric Cancer

    Directory of Open Access Journals (Sweden)

    Alejandra M. Scursoni

    2011-01-01

    Full Text Available The N-glycolylated ganglioside NeuGc-GM3 has been described in solid tumors such as breast carcinoma, nonsmall cell lung cancer, and melanoma, but is usually not detected in normal human cells. Our aim was to evaluate the presence of NeuGc-GM3 in pediatric neuroectodermal tumors by immunohistochemistry. Twenty-seven archival cases of neuroblastoma and Ewing sarcoma family of tumors (ESFT were analyzed. Formalin-fixed, paraffin-embedded tumor samples were cut into 5 μm sections. The monoclonal antibody 14F7, a mouse IgG1 that specifically recognizes NeuGc-GM3, and a peroxidase-labeled polymer conjugated to secondary antibodies were used. Presence of NeuGc-GM3 was evident in 23 of 27 cases (85%, with an average of about 70% of positive tumors cells. Immunoreactivity was moderate to intense in most tumors, showing a diffuse cytoplasmic and membranous staining, although cases of ESFT demonstrated a fine granular cytoplasmic pattern. No significant differences were observed between neuroblastoma with and without NMYC oncogene amplification, suggesting that expression of NeuGc-GM3 is preserved in more aggressive cancers. Until now, the expression of N-glycolylated gangliosides in pediatric neuroectodermal tumors has not been investigated. The present study evidenced the expression of NeuGc-GM3 in a high proportion of neuroectodermal tumors, suggesting its potential utility as a specific target of immunotherapy.

  2. Anterior cruciate ligament - updating article.

    Science.gov (United States)

    Luzo, Marcus Vinicius Malheiros; Franciozi, Carlos Eduardo da Silveira; Rezende, Fernando Cury; Gracitelli, Guilherme Conforto; Debieux, Pedro; Cohen, Moisés

    2016-01-01

    This updating article on the anterior cruciate ligament (ACL) has the aim of addressing some of the most interesting current topics in this field. Within this stratified approach, it contains the following sections: ACL remnant; anterolateral ligament and combined intra and extra-articular reconstruction; fixation devices; and ACL femoral tunnel creation techniques.

  3. Anterior cruciate ligament - updating article

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    Marcus Vinicius Malheiros Luzo

    2016-08-01

    Full Text Available ABSTRACT This updating article on the anterior cruciate ligament (ACL has the aim of addressing some of the most interesting current topics in this field. Within this stratified approach, it contains the following sections: ACL remnant; anterolateral ligament and combined intra and extra-articular reconstruction; fixation devices; and ACL femoral tunnel creation techniques.

  4. Válvula de uretra anterior Anterior urethral valves

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    Silvio Tucci Jr.

    2003-02-01

    Full Text Available Objetivo: apresentar os aspectos clínicos, diagnósticos e terapêuticos de pacientes portadores de válvula da uretra anterior. Descrição: em dois neonatos, o diagnóstico presuntivo de patologia obstrutiva do trato urinário foi sugerido pela ultra-sonografia realizada no período pré-natal, confirmando-se o diagnóstico de válvula de uretra anterior pela avaliação pós-natal. Os pacientes foram submetidos a tratamento cirúrgico paliativo, com vesicostomia temporária e, posteriormente, definitivo, pela fulguração endoscópica das válvulas. Ambos evoluíram com função renal normal. Comentários: a válvula da uretra anterior é anomalia rara que deve ser considerada em meninos com quadro radiológico pré-natal sugestivo de obstrução infravesical, secundariamente à hipótese mais comum de válvula da uretra posterior. Ressaltamos a utilização da vesicostomia como derivação urinária temporária nestes casos, prevenindo potenciais complicações pela manipulação da uretra do recém-nascido.Objective: to discuss clinical signs, diagnostic tools and therapeutics of anterior urethral valves, an obstructive anomaly of the urinary system in males. Description: signs of urinary tract obstruction were identified on pre-natal ultrasound in two male fetuses and the diagnosis of anterior urethral valves was made through post-natal evaluation. As an initial treatment, vesicostomy was performed in both patients. Later, the valves were fulgurated using an endoscopic procedure. During the follow-up period both patients presented normal renal function. Comments: anterior urethral valves are a rare form of urethral anomaly that must be ruled out in boys with pre-natal ultrasound indicating infravesical obstruction. Vesicostomy used as an initial treatment rather than transurethral fulguration may prevent potential complications that can occur due to the small size of the neonatal urethra.

  5. Suv4-20h histone methyltransferases promote neuroectodermal differentiation by silencing the pluripotency-associated Oct-25 gene.

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    Dario Nicetto

    Full Text Available Post-translational modifications (PTMs of histones exert fundamental roles in regulating gene expression. During development, groups of PTMs are constrained by unknown mechanisms into combinatorial patterns, which facilitate transitions from uncommitted embryonic cells into differentiated somatic cell lineages. Repressive histone modifications such as H3K9me3 or H3K27me3 have been investigated in detail, but the role of H4K20me3 in development is currently unknown. Here we show that Xenopus laevis Suv4-20h1 and h2 histone methyltransferases (HMTases are essential for induction and differentiation of the neuroectoderm. Morpholino-mediated knockdown of the two HMTases leads to a selective and specific downregulation of genes controlling neural induction, thereby effectively blocking differentiation of the neuroectoderm. Global transcriptome analysis supports the notion that these effects arise from the transcriptional deregulation of specific genes rather than widespread, pleiotropic effects. Interestingly, morphant embryos fail to repress the Oct4-related Xenopus gene Oct-25. We validate Oct-25 as a direct target of xSu4-20h enzyme mediated gene repression, showing by chromatin immunoprecipitaton that it is decorated with the H4K20me3 mark downstream of the promoter in normal, but not in double-morphant, embryos. Since knockdown of Oct-25 protein significantly rescues the neural differentiation defect in xSuv4-20h double-morphant embryos, we conclude that the epistatic relationship between Suv4-20h enzymes and Oct-25 controls the transit from pluripotent to differentiation-competent neural cells. Consistent with these results in Xenopus, murine Suv4-20h1/h2 double-knockout embryonic stem (DKO ES cells exhibit increased Oct4 protein levels before and during EB formation, and reveal a compromised and biased capacity for in vitro differentiation, when compared to normal ES cells. Together, these results suggest a regulatory mechanism, conserved

  6. JMJD2A attenuation affects cell cycle and tumourigenic inflammatory gene regulation in lipopolysaccharide stimulated neuroectodermal stem cells

    Energy Technology Data Exchange (ETDEWEB)

    Das, Amitabh, E-mail: amitabhdas.kn@gmail.com [Department of Bionanotechnology, Hanyang University, Seoul 133-791 (Korea, Republic of); Chai, Jin Choul, E-mail: jincchai@gmail.com [Department of Molecular and Life Science, Hanyang University, 1271 Sa 3-dong, Ansan 426-791, Gyeonggi-do (Korea, Republic of); Jung, Kyoung Hwa, E-mail: khjung2@gmail.com [Department of Molecular and Life Science, Hanyang University, 1271 Sa 3-dong, Ansan 426-791, Gyeonggi-do (Korea, Republic of); Das, Nando Dulal, E-mail: nando.hu@gmail.com [Clinical Research Centre, Inha University School of Medicine, Incheon 400-711 (Korea, Republic of); Kang, Sung Chul, E-mail: gujiju11@gmail.com [Department of Molecular and Life Science, Hanyang University, 1271 Sa 3-dong, Ansan 426-791, Gyeonggi-do (Korea, Republic of); Lee, Young Seek, E-mail: yslee@hanyang.ac.kr [Department of Molecular and Life Science, Hanyang University, 1271 Sa 3-dong, Ansan 426-791, Gyeonggi-do (Korea, Republic of); Seo, Hyemyung, E-mail: hseo@hanyang.ac.kr [Department of Molecular and Life Science, Hanyang University, 1271 Sa 3-dong, Ansan 426-791, Gyeonggi-do (Korea, Republic of); Chai, Young Gyu, E-mail: ygchai@hanyang.ac.kr [Department of Bionanotechnology, Hanyang University, Seoul 133-791 (Korea, Republic of); Department of Molecular and Life Science, Hanyang University, 1271 Sa 3-dong, Ansan 426-791, Gyeonggi-do (Korea, Republic of)

    2014-11-01

    JMJD2A is a lysine trimethyl-specific histone demethylase that is highly expressed in a variety of tumours. The role of JMJD2A in tumour progression remains unclear. The objectives of this study were to identify JMJD2A-regulated genes and understand the function of JMJD2A in p53-null neuroectodermal stem cells (p53{sup −/−} NE-4Cs). We determined the effect of LPS as a model of inflammation in p53{sup −/−} NE-4Cs and investigated whether the epigenetic modifier JMJD2A alter the expression of tumourigenic inflammatory genes. Global gene expression was measured in JMJD2A knockdown (kd) p53{sup −/−} NE-4Cs and in LPS-stimulated JMJD2A-kd p53{sup −/−} NE-4C cells. JMJD2A attenuation significantly down-regulated genes were Cdca2, Ccnd2, Ccnd1, Crebbp, IL6rα, and Stat3 related with cell cycle, proliferation, and inflammatory-disease responses. Importantly, some tumour-suppressor genes including Dapk3, Timp2 and TFPI were significantly up-regulated but were not affected by silencing of the JMJD2B. Furthermore, we confirmed the attenuation of JMJD2A also down-regulated Cdca2, Ccnd2, Crebbp, and Rest in primary NSCs isolated from the forebrains of E15 embryos of C57/BL6J mice with effective p53 inhibitor pifithrin-α (PFT-α). Transcription factor (TF) motif analysis revealed known binding patterns for CDC5, MYC, and CREB, as well as three novel motifs in JMJD2A-regulated genes. IPA established molecular networks. The molecular network signatures and functional gene-expression profiling data from this study warrants further investigation as an effective therapeutic target, and studies to elucidate the molecular mechanism of JMJD2A-kd-dependent effects in neuroectodermal stem cells should be performed. - Highlights: • Significant up-regulation of epigenetic modifier JMJD2A mRNA upon LPS treatment. • Inhibition of JMJD2A attenuated key inflammatory and tumourigenic genes. • Establishing IPA based functional genomics in JMJD2A-attenuated p53{sup

  7. Fenestration of the anterior cerebral artery

    Energy Technology Data Exchange (ETDEWEB)

    Ito, J.; Washiyama, K.; Hong, K.C.; Ibuchi, Y.

    1981-08-01

    Three cases of angiographically demonstrated fenestration of the anterior cerebral artery are reported. Fenestration occurred at the medial half of the horizontal segment of the anterior cerebral artery in all cases. Its embryology and clinical significance are briefly discussed, and the anatomical and radiological literature on fenestration of the anterior cerebral artery is reviewed.

  8. 38 CFR 3.379 - Anterior poliomyelitis.

    Science.gov (United States)

    2010-07-01

    ... 38 Pensions, Bonuses, and Veterans' Relief 1 2010-07-01 2010-07-01 false Anterior poliomyelitis. 3... Specific Diseases § 3.379 Anterior poliomyelitis. If the first manifestations of acute anterior poliomyelitis present themselves in a veteran within 35 days of termination of active military service, it...

  9. Epidermoid cyst in Anterior, Middle

    Directory of Open Access Journals (Sweden)

    Kankane Vivek Kumar

    2016-09-01

    Full Text Available Epidermoid cysts are benign slow growing more often extra-axial tumors that insinuate between brain structures, we present the clinical, imaging, and pathological findings in 35 years old female patients with atypical epidermoid cysts which was situated anterior, middle & posterior cranial fossa. NCCT head revealed hypodense lesion over right temporal and perisylvian region with extension in prepontine cistern with mass effect & midline shift and MRI findings revealed a non-enhancing heterogeneous signal intensity cystic lesion in right frontal & temporal region extending into prepontine cistern with restricted diffusion. Patient was detoriated in night of same day of admission, emergency Fronto-temporal craniotomy with anterior peterousectomy and subtotal resection was done. The histological examination confirms the epidermoid cyst. The timing of ectodermal tissue sequestration during fetal development may account for the occurrence of atypical epidermoid cysts.

  10. Low grade astrocytoma transformating primitive neuroectodermal tumour in an adult? A case report and review of literature

    Institute of Scientific and Technical Information of China (English)

    Xiaofeng Wang; Yuqiang Sun; Zeshi Tan; Anlong Ji; Xu Sun; Xinyu Li; Ningwei Che

    2016-01-01

    Background:Supratentorial primitive neuroectodermal tumors (sPNET) are rarely occurred in adults.Only 39 cases have been reported in the past decade.The transformation from low grade to aggressive astrocytoma is well known.However,such convert from a low-grade astrocytoma (LGA) into a sPNET between two completely different tumors is rarer.Case Presentation:This report discussed a 36-year-old male,who presented with the chief complaint of partial seizures and left limbs dysfunction and Magnetic resonance imaging (MRI) of whom revealed right frontal lesions which was operated to resect accompanied by the histological diagnosis of sPNET.The patient underwent operation 2.5 years ago because of seizures and was diagnosed with right frontal astrocytoma confirmed by pathology.However,radiotherapy was not preformed on him after the primary surgery.Histology now revealed high grade PNET.Conclusion:It is exactly uncertain that reports revelant to transformation from low-grade astrocytoma to a new kind of tumor or neoplasm induced by radiotation have been published.This case report is accompanied by a review of 39 cases of adult sPNET in the past decade,especially discussing about the transformation of low grade glioma into sPNET.

  11. Fulminant Buddchiari syndrome caused by renal primitive neuroectodermal tumor with inferior vena cava thrombus extending to atrium.

    Science.gov (United States)

    Mete, Uttam K; Singh, Dig Vijay; Bhattacharya, Anish; Kakkar, Nandita

    2015-01-01

    Primitive neuroectodermal tumors (PNET) of the kidney are rare, the diagnosis usually being made at histopathology. A young female presented with a massive right renal mass with features of hepatic dysfunction. Computed tomography scan of the abdomen revealed a large tumor of right kidney with tumor thrombus extending from inferior vena cava (IVC) to right atrium with features suggesting Buddchiari syndrome (BCS). Needle biopsy of mass showed a round cell neoplasm and positive staining for neuron specific enolase and minimum inhibitory concentration-2 on immunohistochemistry. She was managed with neo-adjuvant chemotherapy, surgery and adjuvant chemotherapy. To the best of our knowledge this is the first case of renal PNET with inferior IVC tumor thrombus extending to right atrium with BCS. We suggest that renal PNET should be kept in mind as a differential diagnosis in young adults presenting with a large kidney mass extending to IVC that shows evidence of necrosis on imaging, which may be associated with BCS as in index case.

  12. Diffusion-weighted imaging and fluid attenuated inversion recovery imaging in the evaluation of primitive neuroectodermal tumors

    Energy Technology Data Exchange (ETDEWEB)

    Erdem, E.; Zimmerman, R.A.; Haselgrove, J.C.; Bilaniuk, L.T.; Hunter, J.V. [Hospital of the Univ. of Pennsylvania (HUP), Philadelphia (United States). Dept. of Radiology

    2001-11-01

    The aim of our study was to determine whether fluid-attenuated inversion recovery (FLAIR) imaging and diffusion-weighted imaging (DWI) would be helpful in characterizing primitive neuroectodermal tumors (PNET) from other pediatric brain tumors. We expected that the compact cellular nature and the relatively small extracellular space of this tumor would affect the signal intensity on both pulse sequences relative to the more sparsely cellular glial tumors that have larger extracellular spaces. Eighteen pediatric patients with PNET were examined on a 1.5 T MRI with routine imaging plus FLAIR and compared with 28 patients with non-PNET. DWI was also performed in 7 PNET and 18 non-PNET. Seventy-eight percent of PNET were isointense to gray matter on FLAIR while 82 % of non-PNET were hyperintense and only one was isointense (3 %). Diffusion was abnormally restricted in all 7 PNET examined (100 %) but was restricted in non-PNET in only 1 out of 18 (6 %) patients who had DWI. The differences in the histologic architecture between PNET and non-PNET are reflected in both FLAIR imaging and in DWI. (orig.)

  13. Primitive neuroectodermal tumor/Ewing′s sarcoma in adult uro-oncology: A case series from a developing country

    Directory of Open Access Journals (Sweden)

    Rehan Mohsin

    2011-01-01

    Full Text Available Peripheral primitive neuroectodermal tumor/Ewing′s sarcoma (PNET/EWS is primarily a tumor of soft tissues and bones. Primary localization of PNET/EWS in genitourinary organs is rare. No data on this localization of PNET/EWS are available in literature from Pakistan. We searched our adult uro-oncology records from 1994 till date and identified all cases of adult genitourinary and adrenal PNET/EWS diagnosed on histology and immunohistochemistry. Their case records were reviewed to obtain data on demographics, presentation, pathologic features, management and outcome. Six cases were found; all were young and had aggressive disease at presentation. Four had renal PNET/EWS. One case each of prostate and adrenal PNET/EWS was seen. Surgery and chemotherapy formed the mainstay of management. Three patients (50% died during treatment, two were lost to follow-up and one case with renal PNET/EWS showed good initial response to chemotherapy but was later on lost to follow-up. In conclusion, PNET/EWS should be considered in the differential diagnosis of genitourinary malignant tumors in young patients. These tumors are aggressive with poor outcome.

  14. Primary cutaneous Ewing's sarcoma/primitive neuroectodermal tumor manifesting numerous small and huge ulcerated masses: its complete remission by chemotherapy and magnetic resonance imaging findings

    Energy Technology Data Exchange (ETDEWEB)

    Bahk, Won-Jong [The Catholic University of Korea, Department of Orthopedic Surgery, Uijeongbu St. Mary' s Hospital, Uijeongbu (Korea); Chang, Eun-Deok; Lee, An-Hee [The Catholic University of Korea, Department of Pathology, Uijeongbu St. Mary' s Hospital, Uijeongbu (Korea); Bae, Jung Min; Kim, Jin Woo [The Catholic University of Korea, Department of Dermatology, Uijeongbu St. Mary' s Hospital, Uijeongbu (Korea); Chun, Kyung-Ah [The Catholic University of Korea, Department of Radiology, Uijeongbu St. Mary' s Hospital, Uijeongbu (Korea); Rho, Sang-Young [The Catholic University of Korea, Department of Hemato-oncology, Uijeongbu St. Mary' s Hospital, Uijeongbu (Korea)

    2010-06-15

    Extraskeletal Ewing's sarcoma (ES) and primitive neuroectodermal tumor (PNET) are widely regarded as clinically and histologically identical tumors which consist of small blue round cells. Extraskeletal ESs/PNETs usually occur in the deep soft tissues of the paraspinal region, chest wall, or lower extremities. However, superficially located cases, so-called cutaneous ESs/PNETs, are exceedingly rare, and the vast majority of the reported cases present as a single small mass. We present magnetic resonance imaging (MRI) findings and clinical course of a unique case of primary cutaneous ES/PNET presenting as numerous huge masses with severe ulceration on them. (orig.)

  15. Primitive neuroectodermal tumor (PNET) as somatic-type malignancy arising from an extragonadal germ-cell tumor: clinical, pathological and molecular features of a case.

    Science.gov (United States)

    Garg, Amit; Nahal, Ayoub; Turcotte, Robert; Tabah, Roger; Alcindor, Thierry

    2013-01-01

    We report a rare case of a 34-year-old man with a right axillary mass. Ten years previously, he had been diagnosed with a right scapular nonseminomatous germ-cell tumor consisting of teratoma, completely resected without any further treatment. Presently he was found to have a metastatic malignant small round cell tumor consistent with a secondary somatic malignancy arising in the background of nonseminomatous germ-cell tumor, teratoma, yolk sac tumor, and primitive neuroectodermal tumor with distinct chromosome 22 translocation. Although the patient initially responded well to chemotherapy with etoposide, cisplatin, ifosfamide and mesna, he relapsed shortly after.

  16. Neuro image in neuroectodermal disorders. Part III: angiomatous and melanotic syndromes. Neuroimagen en los transtornos neuroectodermicos. Parte III: sindromes angiomatosos y melanoticos

    Energy Technology Data Exchange (ETDEWEB)

    Marti-Bonmati, L.; Menor, F.; Poyatos, C.; Cortina, H.; Esteban, M.J.; Vilar, J. (Hospital La Fe Valencia. (Spain))

    1994-01-01

    Twenty-eight consecutive patients affected by these rare angiomatous melanotic neuroectodermal disorders are assessed. The diagnostics value and clinical correlation of neuroimaging methods, both CT and MR, are established. Patients with Sturge-Weber syndrome (15 cases), Klippel-Trenaunay syndrome (1 case), Rendu-Osler disease (3 cases), multiple hemangiomatosis (4 cases), von Hippel-Lindau syndrome (3 cases), neuro cutaneous melanosis (1 case) and hypo melanosis of Ito (1 case) are included. In vascular phacomatosis, neuroimaging methods usually contribute to the positive diagnosis. In melanotic disorders, the neuroradiological findings most often are unspecific and do not contribute to the diagnosis of the disease.

  17. Anterior chest wall examination reviewed

    Directory of Open Access Journals (Sweden)

    F. Trotta

    2011-09-01

    Full Text Available Anterior chest wall involvement is not infrequently observed within inflammatory arthropaties, particularly if one considers seronegative spondiloarthritides and SAPHO syndrome. Physical examination is unreliable and conventional X-rays analysis is an unsatisfactory tool during diagnostic work-up of this region. Scintigraphic techniques yield informations both on the activity and on the anatomical extent of the disease while computerized tomography visualize the elementary lesions, such as erosions, which characterize the process. Moreover, when available, magnetic resonance imaging couple the ability to finely visualize such lesions with the possibility to show early alterations and to characterize the “activity” of the disease, presenting itself as a powerful tool both for diagnosis and follow-up. This review briefly shows the applications of imaging techniques for the evaluation of the anterior chest wall focusing on what has been done in the SAPHO syndrome which can be considered prototypical for this regional involvement since it is the osteo-articular target mainly affected by the disease.

  18. Computed tomography and magnetic resonance imaging findings of peripheral primitive neuroectodermal tumors of the head and neck

    Energy Technology Data Exchange (ETDEWEB)

    Zhang Weidong [State Key Laboratory of Oncology in South China, Department of Radiology, Cancer Center, Sun Yat-sen University, Guangzhou, Guangdong, 510060 (China); Chen Yanfeng [State Key Laboratory of Oncology in South China, Department of Head and Neck Surgery, Cancer Center, Sun Yat-sen University, Guangzhou, Guangdong, 510060 (China); Li Chuanxing; Zhang Liang; Xu Zhibin [State Key Laboratory of Oncology in South China, Department of Radiology, Cancer Center, Sun Yat-sen University, Guangzhou, Guangdong, 510060 (China); Zhang Fujun, E-mail: drzhangfj@163.com [State Key Laboratory of Oncology in South China, Department of Radiology, Cancer Center, Sun Yat-sen University, Guangzhou, Guangdong, 510060 (China)

    2011-11-15

    Purpose: We aimed to analyze the computed tomography (CT) and magnetic resonance imaging (MRI) findings of peripheral primitive neuroectodermal tumor (pPNET) of the head and neck. Methods: Eight patients with pPNET of the head and neck confirmed by histopathological examination were analyzed retrospectively. Results: The average patient age was 8 years. The tumor location in the 8 patients was as follows: maxillofacial region (right, 2; left, 1), left maxillary sinus (1), right masticator space (1), left carotid space (1), right infratemporal fossa (1), and left parotid gland (1). All 5 patients who underwent CT demonstrated ill-defined soft masses and no calcification. Three patients with tumors in the maxillofacial region showed homogeneous small masses and a mild enhancement. The patient with left maxillary sinus tumor showed a heterogeneous mass with patchy, necrotic foci and mild heterogeneous enhancement. The patient with right masticator space tumor showed a heterogeneous mass, and marked heterogeneous enhancement. The T1-weighted images of the patients with right infratemporal fossa, left carotid space, and left parotid gland tumors were isointense. The T2-weighted images were heterogeneous and mildly hyperintense in 2 patients and hyperintense in 1 patient. Heterogeneous intermediate enhancement was demonstrated in 2 patients and mild ring enhancement in 1 patient. Conclusion: The imaging features of pPNET of the head and neck are non-specific. An ill-defined, aggressive mass and variable enhancement on CT and MR images may suggest the diagnosis of pPNET. Peripheral PNET should be included in the differential diagnosis of children and adolescents' regional tumors.

  19. Proton Radiation Therapy for Pediatric Medulloblastoma and Supratentorial Primitive Neuroectodermal Tumors: Outcomes for Very Young Children Treated With Upfront Chemotherapy

    Energy Technology Data Exchange (ETDEWEB)

    Jimenez, Rachel B., E-mail: rbjimenez@partners.org [Harvard Radiation Oncology Program, Boston, Massachusetts (United States); Sethi, Roshan [Harvard Medical School, Boston, Massachusetts (United States); Depauw, Nicolas [Department of Radiation Oncology, Massachusetts General Hospital, Boston, Massachusetts (United States); Pulsifer, Margaret B. [Department of Psychiatry, Massachusetts General Hospital, Boston, Massachusetts (United States); Adams, Judith [Department of Radiation Oncology, Massachusetts General Hospital, Boston, Massachusetts (United States); McBride, Sean M. [Harvard Radiation Oncology Program, Boston, Massachusetts (United States); Ebb, David [Department of Pediatrics, Massachusetts General Hospital, Boston, Massachusetts (United States); Fullerton, Barbara C.; Tarbell, Nancy J.; Yock, Torunn I.; MacDonald, Shannon M. [Department of Radiation Oncology, Massachusetts General Hospital, Boston, Massachusetts (United States)

    2013-09-01

    Purpose: To report the early outcomes for very young children with medulloblastoma or supratentorial primitive neuroectodermal tumor (SPNET) treated with upfront chemotherapy followed by 3-dimensional proton radiation therapy (3D-CPT). Methods and Materials: All patients aged <60 months with medulloblastoma or SPNET treated with chemotherapy before 3D-CPT from 2002 to 2010 at our institution were included. All patients underwent maximal surgical resection, chemotherapy, and adjuvant 3D-CPT with either craniospinal irradiation followed by involved-field radiation therapy or involved-field radiation therapy alone. Results: Fifteen patients (median age at diagnosis, 35 months) were treated with high-dose chemotherapy and 3D-CPT. Twelve of 15 patients had medulloblastoma; 3 of 15 patients had SPNET. Median time from surgery to initiation of radiation was 219 days. Median craniospinal irradiation dose was 21.6 Gy (relative biologic effectiveness); median boost dose was 54.0 Gy (relative biologic effectiveness). At a median of 39 months from completion of radiation, 1 of 15 was deceased after a local failure, 1 of 15 had died from a non-disease-related cause, and the remaining 13 of 15 patients were alive without evidence of disease recurrence. Ototoxicity and endocrinopathies were the most common long-term toxicities, with 2 of 15 children requiring hearing aids and 3 of 15 requiring exogenous hormones. Conclusions: Proton radiation after chemotherapy resulted in good disease outcomes for a small cohort of very young patients with medulloblastoma and SPNET. Longer follow-up and larger numbers of patients are needed to assess long-term outcomes and late toxicity.

  20. Positioning of anterior teeth in removable dentures

    Directory of Open Access Journals (Sweden)

    Strajnić Ljiljana

    2002-01-01

    Full Text Available Introduction The aim of this paper was to present methods of placement of artificial anterior teeth in edentulous individuals. The following review takes account of the majority of papers published during the last 100 years. The review has been divided into sections regarding the method used to determine the position of artificial anterior teeth. Geometric aspect Gysi (1895-1920 produced the first scientific theory about the position of artificial anterior teeth. Physiognomic theory The aim of this theory is to find the most natural position for artificial anterior teeth for each individual. Camper's "face angle" as a physiognomic criterion, has been introduced in papers of Wehrli (1961, Marxhors (1966, Tanzer (1968, Lombardi (1973. Esthetic aspect Important names in the field of dental esthetics are: Schön and Singer (1961, Arnheim (1965, Krajiček (1969, Tanzer (1968, Lombardi (1973, Goldstein (1976. They have introduced principles of visual aspects for selection of contours, dimension and position of artificial anterior teeth. Constitution aspect Flagg (1880, Williams (1913 and Hrauf (1957, 1958, have considered body constitution and individual characteristics regarding position of artificial anterior teeth. Physiological theory In 1971, Marxhors pointed to the fact that the position of artificial teeth corresponds with the function of the surrounding soft tissue and from the aspect of physiognomy as well. Phonetic aspect According to Silverman (1962 artificial anterior teeth are nearest when we pronounce the sound "S". Cephalometrical research Rayson (1970, Watson (1989, Strajnić Lj. (1999, Bassi F. (2001 have presented cephalometric radiographic analyses of natural anterior teeth compared with cephalometric radiographic analyses of artificial anterior teeth. A review of dental literature shows several factors suggesting modalities which should determine the position of artificial anterior teeth. Numerous methods have been designed for

  1. Incidental Anterior Cruciate Ligament Calcification: Case Report.

    Science.gov (United States)

    Hayashi, Hisami; Fischer, Hans

    2016-03-01

    The calcification of knee ligaments is a finding noted only in a handful of case reports. The finding of an anterior cruciate ligament calcification has been reported once in the literature. Comparable studies involving the posterior cruciate ligament, medial collateral ligament and an ossicle within the anterior cruciate ligament are likewise discussed in reports of symptomatic patients. We report a case of incidentally discovered anterior cruciate ligament calcification. We discuss the likely etiology and clinical implications of this finding.

  2. Toxic Anterior Segment Syndrome (TASS

    Directory of Open Access Journals (Sweden)

    Özlem Öner

    2011-12-01

    Full Text Available Toxic anterior segment syndrome (TASS is a sterile intraocular inflammation caused by noninfectious substances, resulting in extensive toxic damage to the intraocular tissues. Possible etiologic factors of TASS include surgical trauma, bacterial endotoxin, intraocular solutions with inappropriate pH and osmolality, preservatives, denatured ophthalmic viscosurgical devices (OVD, inadequate sterilization, cleaning and rinsing of surgical devices, intraocular lenses, polishing and sterilizing compounds which are related to intraocular lenses. The characteristic signs and symptoms such as blurred vision, corneal edema, hypopyon and nonreactive pupil usually occur 24 hours after the cataract surgery. The differential diagnosis of TASS from infectious endophthalmitis is important. The main treatment for TASS formation is prevention. TASS is a cataract surgery complication that is more commonly seen nowadays. In this article, the possible underlying causes as well as treatment and prevention methods of TASS are summarized. (Turk J Oph thal mol 2011; 41: 407-13

  3. 肾上腺原始神经外胚叶肿瘤的CT表现%CT Features of Primitive Neuroectodermal Tumor of Adrenal

    Institute of Scientific and Technical Information of China (English)

    王海涛; 水淼; 张霞萍; 于志海

    2010-01-01

    @@ 原始神经外胚叶肿瘤(primitive neuroectodermal tumor,PNET)为一种少见的可能源于中枢和交感神经系统外神经嵴的恶性小圆细胞肿瘤.根据发生部位不同分为中枢性和外周性,将起源于外周神经系统的PNET称为外周性原始神经外胚叶肿瘤(peripheral primitive neuroectodermal tumors, pPNETs).而发生于肾上腺的pPNETs影像学表现报道甚少.笔者搜集了3例经手术及病理证实的肾上腺pPNETs,分析其临床表现、实验室检查及影像学资料,进行对比分析,并复习相关文献,以增强对本病的认识.

  4. A Cell ELISA for the quantification of MUC1 mucin (CD227) expressed by cancer cells of epithelial and neuroectodermal origin.

    Science.gov (United States)

    Falahat, Rana; Wiranowska, Marzenna; Gallant, Nathan D; Toomey, Ryan; Hill, Robert; Alcantar, Norma

    2015-01-01

    Quantitative analysis of MUC1, a cell membrane associated mucin, expressed by intact cells of epithelial origin previously has been limited to flow cytometry, which requires using large quantities of cells and antibodies. Here, for the first time, we report the development of a novel Cellular-based Enzyme Linked Immunosorbent Assay (Cell ELISA) to quantify the expression of MUC1 by cell lines of epithelial and neuroectodermal origin using an antibody recognizing a specific tandem repeat found in the extracellular domain of MUC1. In contrast to flow cytometry, this method requires a much lower number of cells. We report here the results obtained from two variants of this Cell ELISA in live and fixed cells. We found that the Cell ELISA in live cells was not sensitive enough to detect a difference in MUC1 levels between the normal cells and tumor cells. However, we found that Cell ELISA in fixed cells followed by whole cell staining was a dependable method of MUC1 level detection in the normal and tumor cells showing significantly higher levels of MUC1 receptor in the tumor cells when compared to the normal controls. Therefore, we conclude that the Cell ELISA in fixed cells is an efficient method for quantifying the expression of MUC1 by epithelial and neuroectodermal cancer cell lines.

  5. Mild toxic anterior segment syndrome mimicking delayed onset toxic anterior segment syndrome after cataract surgery

    Directory of Open Access Journals (Sweden)

    Su-Na Lee

    2014-01-01

    Full Text Available Toxic anterior segment syndrome (TASS is an acute sterile postoperative anterior segment inflammation that may occur after anterior segment surgery. I report herein a case that developed mild TASS in one eye after bilateral uneventful cataract surgery, which was masked during early postoperative period under steroid eye drop and mimicking delayed onset TASS after switching to weaker steroid eye drop.

  6. Anterior urethral diverticulum: A rare presentation

    Directory of Open Access Journals (Sweden)

    Annavarupu Gopalkrishna

    2016-01-01

    Full Text Available Congenital anomalies of the urogenital tract are the most common anomalies found in the foetus, neonates and infants, but anterior urethral valves and diverticula are rare. Here, we present a case with congenital anterior urethral diverticulum associated with patent ductus arteriosus and polydactyly.

  7. Totally thrombosed giant anterior communicating artery aneurysm

    Directory of Open Access Journals (Sweden)

    V R Roopesh Kumar

    2015-01-01

    Full Text Available Giant anterior communicating artery aneurysmsarerare. Apatient presented with visual dysfunction, gait ataxia and urinary incontinence. MRI showed a giant suprasellar mass.At surgery, the lesion was identified as being an aneurysm arising from the anterior communicating artery.The difficulty in preoperative diagnosis and relevant literature are reviewed.

  8. Anterior segment complications of retinal photocoagulation.

    Science.gov (United States)

    Kanski, J J

    1975-03-01

    Seven patients had anterior segment complications following xenon arc retinal photocoagulation. Irreversible keratopathy was induced in two cases; all patients showed evidence of iris injury. The absorption of radiation by the iris was considered the main factor in producing overheating of the anterior segment.

  9. Anterior cervical hypertrichosis: a sporadic case.

    Science.gov (United States)

    Bostan, Sezen; Yaşar, Şirin; Serdar, Zehra Aşiran; Gizlenti, Sevda

    2016-03-01

    Anterior cervical hypertrichosis is a very rare form of primary localized hypertrichosis. It consists of a tuft of terminal hair on the anterior neck just above the laryngeal prominence. The etiology is still unknown. In this article, we reported a 15-year-old female patient who presented to our clinic with a complaint of hypertrichosis on the anterior aspect of the neck for the last five years. Her past medical history revealed no pathology except for vesicoureteral reflux. On the basis of clinical presentation, our patient was diagnosed with anterior cervical hypertrichosis and she was considered to be a sporadic case due to lack of other similar cases in familial history. To date, 33 patients with anterior cervical hypertrichosis have been reported. Anterior cervical hypertrichosis can be associated with other abnormalities, but it frequently presents as an isolated defect (70%). The association of vesicoureteral reflux and anterior cervical hypertrichosis which was observed in our patient might be coincidental. So far, no case of anterior cervical hypertrichosis associated with vesicoureteral reflux has been reported in the literature.

  10. Molecular detection of EWS-Ets fusion transcripts and their clinicopathologic significance in Ewing's sarcoma/peripheral primitive neuroectodermal tumor

    Institute of Scientific and Technical Information of China (English)

    WANG Hua; ZHENG Jie; WANG Yu-ping; YANG Yu; YOU Jiang-feng

    2005-01-01

    Background Ewing's sarcoma/peripheral primitive neuroectodermal tumor (ES/pPNET) is often difficult to distinguish from other small round cell tumors. The EWS-Ets gene fusions that result from chromosomal translocations in this tumor provide potential molecular diagnostic markers. To apply these molecular markers to commonly available archival materials, we evaluated the feasibility of detecting EWS-Ets including EWS-Fli1 and EWS-ERG fusion transcripts in paraffin-embedded tissues and its diagnostic value for detecting ES/pPNET.Methods Thirteen paraffin-embedded samples of ES/pPNETs were retrieved from archives. Thirteen cases of other tumors with small round cell features (including rhabdomyosarcoma, neuroblastoma, lymphoma, small cell carcinoma, and desmoplastic small round cell tumor) were used as negative controls. Β-actin and β2-microglobulin were used as internal controls. A nested reverse transcriptase-polymerase chain reaction (RT-PCR)-based assay was performed to detect the EWS-Fli1 and EWS-ERG fusion transcripts.Results β-actin and β2-microglobulin were detected in 10/13 and 13/13 ES/pPNETs, respectively. EWS-Fli1 fusion transcripts were detected in 11 of 13 (85%) ES/pPNETs. Three chimeric transcripts, all EWS-Fli1, were detected in ES/pPNET samples. Among 11 EWS-Fli1-positive cases, 7 cases had a typeⅠfusion transcript involving fusion of EWS exon 7 with Fli1 exon 6, 2 cases had a typeⅡfusion transcript involving EWS exon 7 with Fli1 exon 5, and 2 cases expressed fusion transcripts involving EWS exon 7 and Fli1 exon 8. Type Ⅰ EWS-Fli1 fusion predominated over other types. Fusion types could not be distinguished in the remaining 2 cases. Thirteen negative controls did not show detectable chimeric messages. There was a significant relationship between EWS-Fli1 fusion transcripts and CD99 expression. Conclusions Molecular detection of EWS-Fli1 fusion transcripts in formalin-fixed paraffin-embedded material by nested RT-PCR is feasible and is

  11. Diagnosis and treatment of spinal primitive neuroectodermal tumor%椎管内原始神经外胚层肿瘤的诊断与治疗

    Institute of Scientific and Technical Information of China (English)

    顾文韬; 车晓明; 徐启武; 顾士欣; 张明广; 孙兵; 寿佳俊; 诸华光

    2012-01-01

    Objective To summarize the clinical characteristics and treatment of spinal primitive neuroectodermal tumor.Methods Clinical data of 8 patients with spinal cord primitive neuroectodermal tumor were analyzed retrospectively. Results Microsurgery was performed in all of the 8 cases.Total removal was performed in 5 patients,subtotal in 1,most part in 1and partial in 1.During a follow - up of 1month to 24 months,Six patients died of disease,and 2 survived,mean follow up of survivor time is 12months. Conclusions Spinal primitive neuroectodermal tumor is rare,malignant,and poor prognoses tumor.The diagnosis of PNET is by means of routine pathology,immune histology and cytogenetic analysis.Although the best treatment is unavailable due to the limited data,surgery combined with radiotherapy or chemotherapy was recommended.%目的 总结椎管内原始神经外胚层肿瘤的临床特点和治疗方法.方法 回顾性分析2005年1月至2010年12月上海华山医院治疗8例椎管内原始神经外胚层肿瘤的临床资料,对临床特点、诊断和治疗进行分析、总结.结果 8例患者均行显微外科治疗,其中全切除5例,次全切除1例,大部切除1例,部分切除1例.随访1-24个月,其中6例死亡,2例存活,平均生存时间12个月.结论 椎管内原始神经外胚层肿瘤发病率低,恶性度高,预后极差,常规病理辅以免疫组化和分子遗传学方法可明确诊断.手术切除肿瘤结合放化疗的综合治疗是目前的主要治疗原则.

  12. Mini-open anterior lumbar interbody fusion.

    Science.gov (United States)

    Gandhoke, Gurpreet S; Ricks, Christian; Tempel, Zachary; Zuckerbraun, Brian; Hamilton, D Kojo; Okonkwo, David O; Kanter, Adam S

    2016-07-01

    In deformity surgery, anterior lumbar interbody fusion provides excellent biomechanical support, creates a broad surface area for arthrodesis, and induces lordosis in the lower lumbar spine. Preoperative MRI, plain radiographs, and, when available, CT scan should be carefully assessed for sacral slope as it relates to pubic symphysis, position of the great vessels (especially at L4/5), disc space height, or contraindication to an anterior approach. This video demonstrates the steps in an anterior surgical procedure with minimal open exposure. The video can be found here: https://youtu.be/r3bC4_vu1hQ .

  13. How I do it: Anterior pull-through tympanoplasty for anterior eardrum perforations.

    Science.gov (United States)

    Harris, Jeffrey P; Wong, Yu-Tung; Yang, Tzong-Hann; Miller, Mia

    2016-01-01

    Conclusions This technique is offered as a convenient and reliable method for cases with anterior TM perforation and inadequate anterior remnant. Objectives Chronic otitis media surgery is one of the most common procedures in otology. Anterior tympanic membrane (TM) perforation with inadequate anterior remnant is associated with higher rates of graft failure. It was the goal of this series to evaluate the anatomical and functional outcomes of a modified underlay myringoplasty technique-the anterior pull-through method. Materials and methods In a retrospective clinical study, 13 patients with anterior TM perforations with inadequate anterior remnants underwent tympanoplasty with anterior pull-through technique. The anterior tip of the temporalis fascia was pulled through and secured in a short incision lateral to the anterior part of the annulus. Data on graft take rate, pre-operative, and post-operative hearing status were analyzed. Results A graft success rate of 84.6% (11 out of 13) was achieved, without lateralization, blunting, atelectasia, or epithelial pearls. The air-bone gap was 21.5 ± 6.8 dB before intervention and 11.75 ± 5.7 dB after surgery (p = 0.003).

  14. Quadriceps muscle contraction protects the anterior cruciate ligament during anterior tibial translation.

    Science.gov (United States)

    Aune, A K; Cawley, P W; Ekeland, A

    1997-01-01

    The proposed skiing injury mechanism that suggests a quadriceps muscle contraction can contribute to anterior cruciate ligament rupture was biomechanically investigated. The effect of quadriceps muscle force on a knee specimen loaded to anterior cruciate ligament failure during anterior tibial translation was studied in a human cadaveric model. In both knees from six donors, average age 41 years (range, 31 to 65), the joint capsule and ligaments, except the anterior cruciate ligament, were cut. The quadriceps tendon, patella, patellar tendon, and menisci were left intact. One knee from each pair was randomly selected to undergo destructive testing of the anterior cruciate ligament by anterior tibial translation at a displacement rate of 30 mm/sec with a simultaneously applied 889 N quadriceps muscle force. The knee flexion during testing was 30 degrees. As a control, the contralateral knee was loaded correspondingly, but only 5 N of quadriceps muscle force was applied. The ultimate load for the knee to anterior cruciate ligament failure when tested with 889 N quadriceps muscle force was 22% +/- 18% higher than that of knees tested with 5 N of force. The linear stiffness increased by 43% +/- 30%. These results did not support the speculation that a quadriceps muscle contraction contributes to anterior cruciate ligament failure. In this model, the quadriceps muscle force protected the anterior cruciate ligament from injury during anterior tibial translation.

  15. Guideline on anterior cruciate ligament injury

    NARCIS (Netherlands)

    D.E. Meuffels (Duncan); M.T. Poldervaart (Michelle T.); R.L. Diercks (Ron L.); A.W.F.M. Fievez (Alex W.F.M.); T.W. Patt (Thomas W.); C.P. van der Hart (Cor P.); E.R. Hammacher (Eric); F. van der Meer (Fred); E.A. Goedhart (Edwin A.); A.F. Lenssen (Anton F); S.B. Muller-Ploeger (Sabrina B); M.A. Pols (Margreet); D.B.F. Saris (Daniel)

    2012-01-01

    textabstractThe Dutch Orthopaedic Association has a long tradition of development of practical clinical guidelines. Here we present the recommendations from the multidisciplinary clinical guideline working group for anterior cruciate ligament injury. The following 8 clinical questions were formulate

  16. Care of children with anterior uveitis.

    Science.gov (United States)

    Kanski, J J

    1981-09-01

    The clinical features of 290 children with anterior uveitis are presented. The vast majority suffered from chronic uveitis. Specific uveitis entities in children include the syndrome of 'chronic iridocyclitis' in girls, heterochromic cyclitis, and pars planitis. Systemic associations include sarcoidosis, the Vogt-Harada-Koyanagi syndrome, and the seronegative arthritides (juvenile chronic arthritis, juvenile ankylosing spondylitis, psoriatic arthritis, and rarely Reiter's and Beçet's syndromes). Children with a pauciarticular onset of juvenile chronic arthritis, especially when combined with positive findings for antinuclear antibody, are at particular risk of developing chronic anterior uveitis. Most cases of chronic anterior uveitis can be controlled with topical corticosteroids. Those that are resistant to both topical and systemic corticosteroids may have to be treated with chlorambucil. The operation of lensectomy is a great advance in the management of complicated cataract. Secondary glaucoma is the most devastating complication of chronic anterior uveitis in children and responds poorly to therapy.

  17. Head positioning for anterior circulation aneurysms microsurgery

    Directory of Open Access Journals (Sweden)

    Feres Chaddad-Neto

    2014-11-01

    Full Text Available Objective To study the ideal patient's head positioning for the anterior circulation aneurysms microsurgery. Method We divided the study in two parts. Firstly, 10 fresh cadaveric heads were positioned and dissected in order to ideally expose the anterior circulation aneurysm sites. Afterwards, 110 patients were submitted to anterior circulation aneurysms microsurgery. During the surgery, the patient's head was positioned accordingly to the aneurysm location and the results from the cadaveric study. The effectiveness of the position was noted. Results We could determine mainly two patterns for head positioning for the anterior circulation aneurysms. Conclusion The best surgical exposure is related to specific head positions. The proper angle of microscopic view may minimize neurovascular injury and brain retraction.

  18. Primitive neuroectodermal tumor presenting with diffuse leptomeningeal involvement in a 55-year-old woman: a case report and brief summary of current diagnostic tests and treatment.

    Science.gov (United States)

    Kalidindi, Navya; Torres, Carlos H; Michaud, Jean; Zwicker, Jocelyn Christine

    2014-05-01

    Primitive neuroectodermal tumors (PNETs) are typically present as masses in children and adolescents, but rarely in adults. Diagnoses, management strategies, and prognostication factors are not well established in adult cases of PNETs. We describe the case of a central nervous system PNET diagnosed in a 55-year-old woman presenting with a sudden onset of symptoms consisting of increased intracranial pressure and findings of diffuse leptomeningeal enhancement and a small medullary lesion seen on MRI. Amongst the small database of PNETs diagnosed in adults, our case report stands out as one of few cases describing a primarily leptomeningeal PNET diagnosed on biopsy. We also review the literature on PNETs presenting with diffuse leptomeningeal disease and the treatment of PNETs in the adult population.

  19. Primitive Neuroectodermal Tumor Presenting with Diffuse Leptomeningeal Involvement in a 55-Year-Old Woman: A Case Report and Brief Summary of Current Diagnostic Tests and Treatment

    Directory of Open Access Journals (Sweden)

    Navya Kalidindi

    2014-07-01

    Full Text Available Primitive neuroectodermal tumors (PNETs are typically present as masses in children and adolescents, but rarely in adults. Diagnoses, management strategies, and prognostication factors are not well established in adult cases of PNETs. We describe the case of a central nervous system PNET diagnosed in a 55-year-old woman presenting with a sudden onset of symptoms consisting of increased intracranial pressure and findings of diffuse leptomeningeal enhancement and a small medullary lesion seen on MRI. Amongst the small database of PNETs diagnosed in adults, our case report stands out as one of few cases describing a primarily leptomeningeal PNET diagnosed on biopsy. We also review the literature on PNETs presenting with diffuse leptomeningeal disease and the treatment of PNETs in the adult population.

  20. Upper anterior zone restoration with composites

    OpenAIRE

    Lamas Lara, César; CD, Docente del Área de Operatoria Dental y Endodoncia de la Facultad de Odontología de la UNMSM.; Angulo de la Vega, Giselle; CD, Alumna de la Especialidad de Rehabilitación Oral de la Facultad de Odontología de la UNMSM.

    2014-01-01

    The anterior sector problems are very common in our professional practice and became vital importance to make a suitable rehabilitation in these cases; we can not do a good rehabilitation if we do not know the basic characteristics, both aesthetic and functional. Today the composites are a valid alternative for the restoration of the anterior sector, since they offer to us a conservative and aesthetic possibility, but independently of the material to use we have to based on certain rules or p...

  1. Erlotinib-related bilateral anterior uveitis

    Science.gov (United States)

    Ali, Kashif; Kumar, Indu; Usman-Saeed, Muniba; Usman Saeed, Muhammad

    2011-01-01

    The authors report the case of a 68-year-old woman with secondary adenocarcinoma of the lungs from an unknown primary. Erlotinib was started which produced symptoms suggestive of uveitis. Erlotinib was stopped and restarted a month later at a lower dose, which resulted in severe bilateral anterior uveitis. The uveitis settled after stopping erlotinib and treatment with topical steroids and cycloplegics. To the best of the authors’ knowledge, this is the first case of erlotinib-related anterior uveitis. PMID:22694887

  2. Anterior Eye Imaging with Optical Coherence Tomography

    Science.gov (United States)

    Huang, David; Li, Yan; Tang, Maolong

    The development of corneal and anterior segment optical coherence tomography (OCT) technology has advanced rapidly in recently years. The scan geometry and imaging wavelength are both important choices to make in designing anterior segment OCT systems. Rectangular scan geometry offers the least image distortion and is now used in most anterior OCT systems. The wavelength of OCT light source affects resolution and penetration. An optimal choice of the OCT imaging wavelength (840, 1,050, or 1,310 nm) depends on the application of interest. Newer generation Fourier-domain OCT technology can provide scan speed 100-1000 times faster than the time-domain technology. Various commercial anterior OCT systems are available on the market. A wide spectrum of diagnostic and surgical applications using anterior segment OCT had been investigated, including mapping of corneal and epithelial thicknesses, keratoconus screening, measuring corneal refractive power, corneal surgery planning and evaluation in LASIK, intracorneal ring implantation, assessment of angle closure glaucoma, anterior chamber biometry and intraocular lens implants, intraocular lens power calculation, and eye bank donor cornea screening.

  3. A Primary Primitive Neuroectodermal Tumor Arising from Left Subclavian Vein and Extending along Left Brachiocephalic Vein and Superior Vena Cava into Right Atrium.

    Science.gov (United States)

    Wang, Jian; Wang, Weici; Li, Yiqing; Jin, Bi; Yu, Miao; Liu, Wenqi; Yao, Shaohua; Liao, Yonggui; Ouyang, Chenxi

    2015-01-01

    Primitive neuroectodermal tumor (PNET) is an extremely rare malignancy thought to be derived from fetal neuroectodermal precursor cells. It usually occurs in central and peripheral nervous system or soft tissue and bone, while intravenous or intracavitary PNET is considered as an extremely rare tumor. We reported a case of a 44-year-old woman who presented with the left unilateral facial and neck swelling. Magnetic resonance imaging revealed a tape-shaped solid mass within left subclavian vein, left brachiocephalic vein, superior vena cava, and right atrium; the proximal end proportion occupied almost the entire right atrium with a pedicle flip protruded into the right ventricle. Ultrasonography revealed an irregular hypoechnoic mass arising from the left subclavian vein, which extended along the left brachiocephalic vein and superior vena cava into the right atrium and up to the right ventricle. Positron emission tomography-computed tomography revealed several hypermetabolic thyroid nodules with no evidence of intravenous hyperactive lesion. The patient underwent tumor resection under cardiopulmonary bypass. At 15 days postoperatively, total thyroidectomy and resection of the left subclavian vein were simultaneously performed. The patient received chemotherapy and radiotherapy later. Histologically, the neoplasm displayed small, round, blue cells with hyperchromatic nuclei and scant cytoplasm. The neoplastic cells showed a strong immunopositivity for CD99, synaptophysin, CD56, CD57, and friend leukemia integration 1, thus confirming a diagnosis of the PNET. Histopathological examination of the thyroid showed papillary carcinoma. Thus, this PNET had no definitive organ or tissue of origin, which primarily originated from the left subclavian vein with tumor extension along the superior vena cava to the right ventricle.

  4. The clinical features of rare thymic primitive neuroectodermal tumor%罕见胸腺原始神经外胚层肿瘤的临床特点

    Institute of Scientific and Technical Information of China (English)

    杜亚奇; 刘东屏; 孙明军; 赵梅芬

    2016-01-01

    Objective To explore primitive neuroectodermal tumor (PNET) clinical characteristics and method of diagnosis and treatment. Methods The clinical data of a case of thymic primitive neural ectodermal tumor was retrospectively analyzed. Results By ultrasound guided puncture biopsy, immunohistochemistry was performed, and histological evidence was obtained, for the diagnosis of thymic primitive neuroectodermal tumors (stage IV). Conclusion This disease is highly malignant tumor. It is prone to distant metastasis and the prognosis is poor. The clinical and pathological doctors know little about this disease and could be easily misdiagnosed.%目的:探讨原始神经外胚层肿瘤(PNET)的临床特点和诊疗方法。方法回顾性分析中国医科大学附属第一医院收治的1例胸腺原始神经外胚层肿瘤的临床资料,分析其临床、病理及治疗的特点。结果经超声引导下穿刺取病理,行免疫组化,获得组织学证据,诊断为胸腺原始神经外胚层肿瘤(IV期)。结论 PNET属于高度恶性肿瘤,易发生远处转移,且预后差,因发病罕见,临床及病理医师对其认识不足,易造成误诊或漏诊。

  5. [Surgical anatomy of the anterior mediastinum].

    Science.gov (United States)

    Biondi, Alberto; Rausei, Stefano; Cananzi, Ferdinando C M; Zoccali, Marco; D'Ugo, Stefano; Persiani, Roberto

    2007-01-01

    The mediastinum is located from the thoracic inlet to the diaphragm between the left and right pleural cavities and contains vital structures of the circulatory, respiratory, digestive, and nervous system. Over the years, since there are no fascial or anatomic planes, anatomists and radiologists have suggested various schemes for subdividing the mediastinum and several anatomical and radiological classifications of the mediastinum are reported in the literature. The most popular of these scheme divides medistinum, for purposes of description, into two parts: an upper portion, above the upper level of the pericardium, which is named the superior mediastinum; and a lower portion, below the upper level of the pericardium. For clinical purposes, the mediastinum may be subdivided into three major areas, i.e. anterior, middle, and posterior compartments. The anterior mediastinum is defined as the region posterior to the sternum and anterior to the heart and brachiocephalic vessels. It extends from the thoracic inlet to the diaphragm and contains the thymus gland, fat, and lymph nodes. This article will review surgical anatomy of the anterior mediastinum and will focus on the surgical approch to anterior mediastinum and thymic diseases.

  6. Extradural and supratentorial primitive neuroectodermal tumor in one adult%成人幕上硬膜外原始神经外胚层肿瘤一例报告

    Institute of Scientific and Technical Information of China (English)

    刘新社; 杨志林

    2010-01-01

    @@ 原始神经外胚层肿瘤(primitive neuroectodermal tumor,PNET)主要发生于儿童中枢神经系统轴内中线部位,幕下为主,成人、幕上、轴外少见.广东省司法警察医院神经外科2009年10月收治1例幕上硬膜外PNET成人患者.现报道如下.

  7. Melanotic neuroectodermal tumor of infancy (progonoma): a case report emphasizing the computed tomography findings and literature review; Tumor neuroectodermico melanocitico da infancia (progonoma): relato de caso enfatizando os aspectos tomograficos e revisao da literatura

    Energy Technology Data Exchange (ETDEWEB)

    Araujo Junior, Cyrillo Rodrigues de; Carvalho, Tarcisio Nunes; Fraguas Filho, Sergio Roberto; Costa, Marlos Augusto Bitencourt; Borba, Ana Olivia Cardoso; Figueiredo, Sizenildo da Silva; Machado, Marcio Martins; Teixeira, Kim-Ir-Sen Santos [Goias Univ., Goiania, GO (Brazil). Hospital das Clinicas. Servico de Diagnostico por Imagem]. E-mail: radiologia@brturbo.com

    2004-12-01

    The melanotic neuroectodermal tumor of infancy, also known as progonoma, is a rare benign disease of neural crest origin that occurs within the first year of life and affects mainly the maxilla. The authors report a case of a 10-month-old child presenting with this uncommon tumor in the maxilla, emphasizing the diagnostic findings on computed tomography, and present a literature review. (author)

  8. THYMOLIPOMA: A RARE, LARGE ANTERIOR MEDIASTINAL MASS

    Directory of Open Access Journals (Sweden)

    Premananth

    2015-07-01

    Full Text Available Thymolipoma is a rare benign tumor of anterior mediastinum, described by Lange in 1916. 1 Less than 200 cases have been reported worldwide. 2 It accounts for 2% to 9% of thymic tumours. 3 We report a case of thymolipoma in a 37 year s old male patient, who pre sented with cough, dys p nea, chest pain for 2 months. CT THORAX revealed a large anterior mediastinal mass extending in to right hemithorax arising from thymus gland, with multiple areas of fat density, no significant mediastinal adenopathy, complete collap se of right middle and lower lobe suggestive of thymolipoma. CT guided biopsy suggestive of thymic neoplasm. The tumour was removed enbloc through surgery. Histopathological examination of large mass lesion confirmed thymolipoma. We report this case to emp hasize the importance of considering thymolipoma as a differential diagnosis of anterior mediastinal mass, although rare.

  9. Esthetic crown lengthening for maxillary anterior teeth.

    Science.gov (United States)

    Sonick, M

    1997-08-01

    In the maxillary anterior region, the gingival labial margin position is an important parameter in the achievement of an ideal smile. The relationship between the periodontium and the restoration is critical if gingival health and esthetics are to be achieved. Periodontal therapy is a necessary and useful adjunct when any anterior restoration is undertaken. Anterior surgical crown lengthening may be undertaken to avoid restorative margin impingement on the biologic width. Crown lengthening is also used to alter the gingival labial profiles. This article discusses the esthetic parameters of ideal gingival labial positions and presents a classification of crown-lengthening procedures and the procedure for a two-stage crown-lengthening technique. The two-stage crown-lengthening technique is surgically precise because healing is predictable.

  10. Nonnecrotizing anterior scleritis mimicking orbital inflammatory disease

    Directory of Open Access Journals (Sweden)

    Lynch MC

    2013-08-01

    Full Text Available Michelle Chen Lynch,1 Andrew B Mick21Optometry Clinic, Ocala West Veterans Affairs Specialty Clinic, Ocala, FL, USA; 2Eye Clinic, San Francisco VA Medical Center, San Francisco, CA, USABackground: Anterior scleritis is an uncommon form of ocular inflammation, often associated with coexisting autoimmune disease. With early recognition and aggressive systemic therapy, prognosis for resolution is good. The diagnosis of underlying autoimmune disease involves a multidisciplinary approach.Case report: A 42-year-old African American female presented to the Eye Clinic at the San Francisco Veteran Affairs Medical Center, with a tremendously painful left eye, worse on eye movement, with marked injection of conjunctiva. There was mild swelling of the upper eyelid. Visual acuity was unaffected, but there was a mild red cap desaturation. The posterior segment was unremarkable. The initial differential diagnoses included anterior scleritis and orbital inflammatory disease. Oral steroid treatment was initiated with rapid resolution over a few days. Orbital imaging was unremarkable, and extensive laboratory work-up was positive only for antinuclear antibodies. The patient was diagnosed with idiopathic diffuse, nonnecrotizing anterior scleritis and has been followed for over 5 years without recurrence. The rheumatology clinic monitors the patient closely, as suspicion remains for potential arthralgias including human leukocyte antigen-B27-associated arthritis, lupus-associated arthritis, seronegative rheumatoid arthritis, recurrent juvenile idiopathic arthritis, and scleroderma, based on her constitutional symptoms and clinical presentation, along with a positive anti-nuclear antibody lab result.Conclusion: Untreated anterior scleritis can progress to formation of cataracts, glaucoma, uveitis, corneal melting, and posterior segment disease with significant risk of vision loss. Patients with anterior scleritis must be aggressively treated with systemic anti

  11. Anterior ischemic optic neuropathy following dengue fever.

    Science.gov (United States)

    Ramakrishnan, Reshma; Shrivastava, Saurabh; Deshpande, Shrikant; Patkar, Priyanka

    2016-01-01

    Dengue fever is caused by a flavivirus. This infection is endemic in the tropics and warm temperate regions of the world. Ocular manifestations of dengue fever include subconjunctival, vitreous, and retinal haemorrhages; posterior uveitis; optic neuritis; and maculopathies, haemorrhage, and oedema. However anterior ischemic optic neuropathy is a rare presentation. Optic nerve ischemia most frequently occurs at the optic nerve head, where structural crowding of nerve fibers and reduction of the vascular supply may combine to impair perfusion to a critical degree and produce optic disc oedema. Here we present a case of anterior ischemic optic neurapathy associated with dengue fever.

  12. Study of aqueous humour in anterior uveitis

    Directory of Open Access Journals (Sweden)

    Kalsy Jairaj

    1990-01-01

    Full Text Available Aetiological diagnosis of anterior uveitis was made clinically and substantiated with relevant investigations. Aqueous humour obtained under aseptic conditions, was analyzed for the cells study, culture and protein profile, using polyacrylamide gel electrophoresis. The results were analysed with the help of known clinical facts. Culture and smears were invariably negative, while the lymphocytes were present in varying numbers, polymorphs and macrophages afforded a useful clue for confirmatory diagnosis. The electrophoretic pattern of the proteins was related to the duration of the disease and was same in a group while it was distinctive among different groups of anterior uveitis.

  13. ANTERIOR OSTEOPHYTE IDENTIFICATION IN CERVICAL VERTEBRAE

    Directory of Open Access Journals (Sweden)

    A. T. Chougale

    2011-06-01

    Full Text Available Radiologist always examines X-ray to determine abnormal changes in cervical, lumbar & thoracic vertebrae. Osteophyte (bony growth may appear at the corners of vertebrae so that vertebral shape becomes abnormal. This paper presents the idea from Image processing techniques such as customised Hough transform which will be used for segmentation which should be independent of rotation, scale, noise & shape. This segmented image will be then used for computing size invariant, convex hull based features to differentiate normal cervical vertebrae from cervical vertebrae containing anterior osteophyte. This approach effectively finds anterior osteophytes in cervical vertebrae.

  14. Dual (type IV left anterior descending artery

    Directory of Open Access Journals (Sweden)

    Ozdil Baskan

    2013-11-01

    Full Text Available Congenital coronary artery anomalies are uncommon. Dual left anterior descending coronary artery (LAD is defined as the presence of two LADs within the anterior interventricular sulcus (AIVS, and is classified into four types. Type IV is a rarely reported subtype and differs from the others, with a long LAD originating from the right coronary artery (RCA. Dual LAD is a benign coronary artery anomaly, but should be recognised especially before interventional procedures. With the increasing use of multidedector computed tomography (MDCT, it is essential for radiologists to be aware of this entity and the cross-sectional findings.

  15. 原始神经外胚叶肿瘤的影像诊断%The imaging diagnose of primitive neuroectodermal tumors

    Institute of Scientific and Technical Information of China (English)

    吴刚

    2012-01-01

    目的 分析原始神经外胚叶肿瘤(PNET)的CT、MRI表现,以提高对该病的认识.方法 回顾分析29例经病理证实的PNET的临床表现和CT、MRI征象.结果 原发于上肢PNET 3例,上颌窦1例,肺1例,纵隔2例,腹膜后2例,颈部1例,下肢2例,额叶6例,颞叶3例,脑室内3例,CPA区1例,椎管内2例,盆腔内2例.病灶体积较大,呈浸润生长,推压邻近结构,并可凸入椎管.肿瘤密度/信号不均,CT为软组织密度,轻至中度强化;MR T1WI肿块与肌肉等信号,T2 WI呈不均匀高信号,增强扫描呈花环状或蜂窝状强化.3例有钙化,2例邻近骨质破坏,呈溶骨性破坏伴骨膜反应.肿瘤DWI上呈高信号,ADC值低于其他颅内原发性肿瘤.结论 PNET的常规MRI表现和DWI特点有助于其术前定性诊断和鉴别诊断.%Objective To describe the conventional MR findings and the diagnostic value of MRI and CT in primitive neuroectodermal tumors so as to improve the diagnosis of this entity. Methods The conventional MRI and CT features were retrospectively analyzed in cases of primitive neuroectodermal tumors proved by operation and pathology, and diffusion weighted imaging feature of cases were retrospectively studied. Results Primary sites were upper limbs (n=3), maxillary sinus (n= 1), lung( n=l), mediastinum (n= 2), retroperitoneum (n= 2), neck(n= 1), legs(n= 2), frontal lobe(n= 6), temporal lobe(n= 3), intraventricular(n= 3), CPA district(n= 1), spinal canal(n = 2), and pevic cavi-ty(n= 2). The tumors usually had big size with aggressive behavior, tended to displace adjacent soft tissue structures. Some case had calcification in tumor. Local or bony invasion in bone marrow involvement with periosteal new bone formation were seen in a few patients. The tumors were tended to be round in shape with clear margin. The tumors were appeared slight hypointensity or isointensity on T1 weighted images and slight hyperintensity or isointensity on T2 weighted images, and surrounded by slight

  16. Anterior Chamber Live Loa loa: Case Report.

    Science.gov (United States)

    Kagmeni, G; Cheuteu, R; Bilong, Y; Wiedemann, P

    2016-01-01

    We reported a case of unusual intraocular Loa loa in a 27-year-old patient who presented with painful red eye. Biomicroscopy revealed a living and active adult worm in the anterior chamber of the right eye. After surgical extraction under local anesthesia, parasitological identification confirmed L. loa filariasis.

  17. Causes of anterior cruciate ligament injuries

    Directory of Open Access Journals (Sweden)

    Ristić Vladimir

    2010-01-01

    Full Text Available In order to prevent anterior cruciate ligament injuries it is necessary to define risk factors and to analyze the most frequent causes of injuries - that being the aim of this study. The study sample consisted of 451 surgically treated patients, including 400 sportsmen (65% of them being active and 35% recreational sportsmen, 29% female and 71% male; of whom 90% were younger than 35. Sports injuries, as the most frequent cause of anterior cruciate ligament injuries, were recorded in 88% of patients (non-contact ones in 78% and contact ones in 22%, injuries occurring in everyday activities in 11% and in traffic in 1%. Among sportsmen, reconstruction of the anterior cruciate ligament was most frequently performed in football players (48%, then in handball players (22%, basketball players (13%, volleyball players (8%, martial arts fighters (4%. However, the injury incidence was the highest among the active basketball players (1 injured among 91 active players. Type of footwear, warming up before the activity, genetic predisposition and everyday therapy did not have a significant influence on getting injured. Anterior cruciate ligament injuries happened three times more often during matches, in the middle and at the end of a match and training session (79%, at landing after the jump or when changing direction of movement (75% without a contact with other competitors, on dry surfaces (79%, among not so well prepared sportsmen.

  18. ANTERIOR COLUMN FRACTURES OF THE ACETABULUM

    NARCIS (Netherlands)

    HEEG, M; OTTER, N; KLASEN, HJ

    1992-01-01

    We retrospectively reviewed 20 patients at three to 19 years after displaced anterior fracture-dislocations of the hip. Eighteen of them were treated by traction, after ensuring that the femoral head was adequately reduced beneath the undisrupted part of the weight-bearing dome. Two required operati

  19. Balanitis xerotica obliterans involving anterior urethra.

    Science.gov (United States)

    Herschorn, S; Colapinto, V

    1979-12-01

    Balanitis xerotica obliterans (BXO) is known to affect the urethral meatus, glans, and prepuce. We describe a case of biopsy-proved BXO that involves not only the usual areas but the anterior urethra as well. Of added interest is the subsequent development of squamous cell carcinoma in the fossa navicularis. The literature is reviewed.

  20. Treatment for acute anterior cruciate ligament tear

    DEFF Research Database (Denmark)

    Frobell, Richard B; Roos, Harald P; Roos, Ewa M;

    2013-01-01

    To compare, in young active adults with an acute anterior cruciate ligament (ACL) tear, the mid-term (five year) patient reported and radiographic outcomes between those treated with rehabilitation plus early ACL reconstruction and those treated with rehabilitation and optional delayed ACL...

  1. Guideline on anterior cruciate ligament injury

    NARCIS (Netherlands)

    Meuffels, Duncan E; Poldervaart, Michelle T; Diercks, Ronald; Fievez, Alex W F M; Patt, Thomas W; Hart, Cor P van der; Hammacher, Eric R; Meer, Fred van der; Goedhart, Edwin A; Lenssen, Anton F; Muller-Ploeger, Sabrina B; Pols, Margreet A; Saris, Daniel B F

    2012-01-01

    The Dutch Orthopaedic Association has a long tradition of development of practical clinical guidelines. Here we present the recommendations from the multidisciplinary clinical guideline working group for anterior cruciate ligament injury. The following 8 clinical questions were formulated by a steer

  2. Novel Insights into Anterior Cruciate Ligament Injury

    NARCIS (Netherlands)

    D.E. Meuffels (Duncan)

    2011-01-01

    textabstractAnterior cruciate ligament (ACL) injury is one of the most common sports injuries of the knee. ACL reconstruction has become, standard orthopaedic practice worldwide with an estimated 175,000 reconstructions per year in the United States.6 The ACL remains the most frequently studied liga

  3. Treatment for acute anterior cruciate ligament tear

    DEFF Research Database (Denmark)

    Frobell, Richard B; Roos, Harald P; Roos, Ewa M;

    2015-01-01

    STUDY QUESTION: In young active adults with an acute anterior cruciate ligament (ACL) rupture, do patient reported or radiographic outcomes after five years differ between those treated with rehabilitation plus early ACL reconstruction and those treated with rehabilitation and optional delayed ACL...

  4. Diagnosis and treatment of supratentorial primitive neuroectodermal tumors: report of 5 cases%幕上原始神经外胚瘤五例

    Institute of Scientific and Technical Information of China (English)

    张彤旭; 李聪慧; 胡昱红; 门换丽; 杨利军; 张伟; 苏现辉; 宋贺; 聂建刚

    2011-01-01

    目的 探讨幕上原始神经外胚瘤(supratentorial primitive neuroectodermal tumor,SPNET)的临床表现、影像和病理特征.方法 对5例经病理证实的SPNET的资料,分析其临床表现、影像学组织病理学特征、治疗经过及预后.结果 5例中3例为儿童,2例为成人,主要临床表现为颅内高压症状.CT和MRI检查示肿瘤为幕上肿块,呈类圆形或分叶状,边界清楚,2例有钙化灶,3例无瘤周水肿.病理结果 提示肿瘤组织主要由小圆形细胞组成,部分为梭形细胞,细胞密集成巢,由纤维结缔组织分隔成小叶状,核染色质颗粒状,核仁不明显,多数可见核分裂象,形成Homer Wright菊形团.结论 SPNET的影像学和组织病理学表现均有一定特征,影像学及临床表现相结合有助于术前与其他肿瘤相鉴别,提高诊断准确性.%Objective To discuss the clinical, imaging findings, and histopathologic features of supratentorial primitive neuroectodermal tumor ( SPNET ) supply data for treatment and diagnosis successfully. Methods The clinical materials of 5 SPNETs which included manifestations, imaging findings , histopathologic features diagnosis, treatment and prognosis were summarized and analyzed retrospectively. All the patients underwent microsurgery. Results Three cases happene.d in children and two in adults . The most manifestations were increased intracranial pressure .The tumors showed calcification in 2cases and minimal peritumoral edema in 3 cases. The lesions were round-like or lobulated in shape and well demarked. The histopathologic features showed fibrous bands surrounding nests of small round cell or spindle cell. Homer Wright rosettes could be found. There were granular nuclear chromatin and obscure nucleoli in the tumor cells. Conclusion There are some characteristics on imaging and pathology of SPNET. It is helpful to differentiate SPENT from other tumors before operation by combining the imagines with clinical manifestation and

  5. Perawatan Ortodontik Gigi Anterior Berjejal dengan Tulang Alveolar yang Tipis

    Directory of Open Access Journals (Sweden)

    Miesje K. Purwanegara

    2015-09-01

    Full Text Available Anterior teeth movement in orthodontic treatment is limited to labiolingual direction by very thin alveolar bone. An uncontrolled anterior tooth movement to labiolingual direction can cause alveolar bone perforation at its root segment. This case report is to remind us that alveolar bone thickness limits orthodontc tooth movement. A case of crowded anterior teeth with thin alveolar bone in malocclusion I is reported. This case is treated using adgewise orthodontic appliance. Protraction of anterior teeth is anticipated due to thin alveolar bone on the anterior surface. The conclusion is although the alveolar bone surrounding the crowded anterior teeth is thin, by controlling the movement the teeth reposition is allowed.

  6. Current approach in diagnosis and management of anterior uveitis

    Directory of Open Access Journals (Sweden)

    Agrawal Rupesh

    2010-01-01

    Full Text Available Uveitis is composed of a diverse group of disease entities, which in total has been estimated to cause approximately 10% of blindness. Uveitis is broadly classified into anterior, intermediate, posterior and panuveitis based on the anatomical involvement of the eye. Anterior uveitis is, however, the commonest form of uveitis with varying incidences reported in worldwide literature. Anterior uveitis can be very benign to present with but often can lead to severe morbidity if not treated appropriately. The present article will assist ophthalmologists in accurately diagnosing anterior uveitis, improving the quality of care rendered to patients with anterior uveitis, minimizing the adverse effects of anterior uveitis, developing a decision-making strategy for management of patients at risk of permanent visual loss from anterior uveitis, informing and educating patients and other healthcare practitioners about the visual complications, risk factors, and treatment options associated with anterior uveitis.

  7. Surgical Management of Intramyocardial Left Anterior Descending Artery.

    Science.gov (United States)

    De Salvatore, Sergio; Segreto, Antonio; Chiusaroli, Alessandro; Congiu, Stefano; Bizzarri, Federico

    2015-11-01

    An intramyocardial left anterior descending artery can be found in up to 30% of patients undergoing coronary artery bypass graft procedures. We review the various techniques available to identify an intramyocardial left anterior descending artery.

  8. 原始神经外胚层瘤16例临床分析%Clinical analysis of 16 patients with primitive neuroectodermal tumors

    Institute of Scientific and Technical Information of China (English)

    魏瑞; 张阳德; 何剪太; 伍海军

    2007-01-01

    目的:分析原始神经外胚层瘤(primitive neuroectodermal tumors,PNET)的病理学特征、诊断及治疗.方法:收集16例PNET患者的临床资料,肿瘤位于颅内4例,纵隔4例,眶内2例,胸髓2例,下肢2例,颈部1例,腹腔1例.6例单纯手术切除,10例行术后放、化疗.结果:16例中14例死亡,中位生存期为22个月,6例单纯手术切除组及10例术后放、化疗组的中位生存期分别为13个月、28个月,2组相比差异有统计学意义(P=0.0194).结论:PNET是一种高度恶性的神经上皮瘤,侵袭性强,手术加放疗加化疗是PNET治疗的最佳选择.

  9. Detection of EWS-FLI1 fusion transcripts in paraffin embedded tissues of peripheral primitive neuroectodermal tumors by nested reverse transcription polymerase chain reaction

    Institute of Scientific and Technical Information of China (English)

    Qixing Gong; Qinhe Fan; Zhihong Zhang; Weiming Zhang

    2005-01-01

    Objective: To assess the feasibility and significance of detecting EWS-FLIlfusion transcripts in paraffin embedded tissues of peripheral primitive neuroectodermal tumors (PNETs) by nested reverse transcription polymerase chain reaction (RT-PCR).Methods: Twelve formalin-fixed and paraffin-embedded (FFPE) samples of PNET were retrieved from archive and consultation materials,together with eight cases of controlled tumor. EWS-FLI1 fusion transcripts were detected by nested RT-PCR. Home-keeping gene β-actin was used to detect the quality of mRNA. Results: β-actin mRNA was detected in 9 of the 12 tumor cases. EWS-FLI1 fusion transcripts were detected in 6 cases, among which 4 had a "type 1" fusion transcript and 2 had a "type 2" fusion transcript. None of the controlled tumor was detected the fusion gene. Conclusion: RT-PCR is a feasible method for the detection of EWS-FLI1 fusion transcripts in FFPE tissues in PNET and the result is meaningful in differential diagnosis and prognostic evaluation.

  10. Diagnosis and Treatment of 27 Cases of Primitive Neuroectodermal Tumor%27例原始神经外胚层瘤诊治分析

    Institute of Scientific and Technical Information of China (English)

    陈煜; 陈伟高; 余智华

    2011-01-01

    目的 分析原始神经外胚层瘤(primitive neuroectodermal tumor,PNET)的临床特点、诊断、治疗方法和预后.方法 回顾性分析27例PNET患者的临床资料,其中19例行综合治疗,8例行非综合治疗.结果 27例患者均获随访,随访时间为8~65个月,总的生存期为6~63个月,中位生存期21个月.其中1年生存期为77.8%,2年生存期为48.1%,3年生存期25.9%.综合治疗中位生存期为29个月,非综合治疗中位生存期为14个月,2种治疗方法比较差异有统计学意义(P<0.05).结论 PNET是一种高度恶性肿瘤,好发于青少年,病理检查是可靠的诊断方法,预后较差,综合治疗可有效地提高患者生存率.

  11. Primary primitive neuroectodermal tumor of the cervix confirmed with molecular analysis in a 23-year-old woman: A case report.

    Science.gov (United States)

    Masoura, Sophia; Kourtis, Anargyros; Kalogiannidis, Ioannis; Kotoula, Vassiliki; Anagnostou, Eleftherios; Angelidou, Stamatia; Agorastos, Theodoros

    2012-04-15

    Primitive Neuroectodermal Tumor (PNET) of the genital tract is very rare, especially in the cervix. A case report of a young woman with a diagnosis of PNET originating from the uterine cervix is presented here. A 23-year-old woman presented with abnormal uterine bleeding and sharp lower abdominal pain of two months duration. CT and MRI of the abdomen and thorax revealed the presence of a large mass in the uterine cervix, enlarged pelvic lymph nodes and broncho-pulmonary infiltrations with regional nodes. Pathological examination of the tumor revealed a malignant neoplasm composed predominantly of small cells, immunohistochemically positive for CD99, vimentin and c-Kit. Molecular testing demonstrated the expression of EWS/FLI1 fusion transcripts corresponding to the t(11;22)(q24;q12) translocation, which confirmed the diagnosis of PNET of the uterine cervix. Despite surgical excision and administration of the first cycle of adjuvant chemotherapy, the patient died from multiple-organ failure and cardiac arrest. PNET arising from the genital tract, especially in the uterine cervix, is very rare and presents a diagnostic challenge. A timely confirmation with molecular analysis is essential for the diagnosis of such a tumor at an unusual site as in the present case.

  12. Combined irradiation and chemotherapy using ifosfamide, cisplatin, and etoposide for children with medulloblastoma/posterior fossa primitive neuroectodermal tumor. Results of a pilot study

    Energy Technology Data Exchange (ETDEWEB)

    Sawamura, Yutaka; Ikeda, Jun; Ishii, Nobuaki; Kato, Tsutomu; Tada, Mitsuhiro; Abe, Hiroshi; Shirato, Hiroki [Hokkaido Univ., Sapporo (Japan). School of Medicine

    1996-09-01

    Ten children with newly diagnosed medulloblastoma/primitive neuroectodermal tumor of the posterior fossa were treated with total surgical resection, radiation therapy, and ICE chemotherapy regimen with ifosfamide (900 mg/m{sup 2}, days 1-5), cisplatin (20 mg/m{sup 2}, days 1-5), and etoposide (60 mg/m{sup 2}, days 1-5) every 4 weeks for eight cycles. Four children under 2 years old were at first treated with eight cycles of ICE chemotherapy, and then irradiated. The ICE regimen was well tolerated by all children, with no irreversible adverse effects. However, dose reductions during the eight cycles were inevitable mainly due to myelosuppression. Complete remissions were achieved in eight of 10 patients at 1 month after completion of the treatment. One child showed recurrence 21 months after complete remission. The disease-free survival rate was 70% with a mean observation period of 24 months after surgery. The ICE regimen is a useful treatment modality for children with medulloblastoma. Further study is warranted to clarify long-term outcome in a number of patients. (author)

  13. The role of H1 linker histone subtypes in preserving the fidelity of elaboration of mesendodermal and neuroectodermal lineages during embryonic development.

    Directory of Open Access Journals (Sweden)

    Giang D Nguyen

    Full Text Available H1 linker histone proteins are essential for the structural and functional integrity of chromatin and for the fidelity of additional epigenetic modifications. Deletion of H1c, H1d and H1e in mice leads to embryonic lethality by mid-gestation with a broad spectrum of developmental alterations. To elucidate the cellular and molecular mechanisms underlying H1 linker histone developmental functions, we analyzed embryonic stem cells (ESCs depleted of H1c, H1d and H1e subtypes (H1-KO ESCs by utilizing established ESC differentiation paradigms. Our study revealed that although H1-KO ESCs continued to express core pluripotency genes and the embryonic stem cell markers, alkaline phosphatase and SSEA1, they exhibited enhanced cell death during embryoid body formation and during specification of mesendoderm and neuroectoderm. In addition, we demonstrated deregulation in the developmental programs of cardiomyocyte, hepatic and pancreatic lineage elaboration. Moreover, ectopic neurogenesis and cardiomyogenesis occurred during endoderm-derived pancreatic but not hepatic differentiation. Furthermore, neural differentiation paradigms revealed selective impairments in the specification and maturation of glutamatergic and dopaminergic neurons with accelerated maturation of glial lineages. These impairments were associated with deregulation in the expression profiles of pro-neural genes in dorsal and ventral forebrain-derived neural stem cell species. Taken together, these experimental observations suggest that H1 linker histone proteins are critical for the specification, maturation and fidelity of organ-specific cellular lineages derived from the three cardinal germ layers.

  14. Type 1 (11; 22)(q24: q12) translocation is common in Ewing's sarcoma/peripheral neuroectodermal tumour in south Indian patients

    Indian Academy of Sciences (India)

    T Parija; S Shirley; S Uma; K R Rajalekshmy; S Ayyappan; T Rajkumar

    2005-06-01

    The Ewing’s sarcoma family can present diagnostic difficulties. In the past the basis of diagnosis has been a exclusion. Identification of a specific translocation especially t(11; 22) (EWS-FLI 1 fusion gene), which is seen in nearly 85% of Ewing’s sarcoma cases can help in precise diagnosis. We have carried out a study on twenty patient samples diagnosed to have Ewing’s sarcoma/peripheral neuroectodermal tumour (PNET)/small round cell malignant tumour. The study involved RT-PCR analysis for the fusion transcript, followed by sequencing to identify the specific type of fusion. Ninety percent (18/20) of the samples tested were found to be t(11; 22) translocations involving EWS-FLI 1 genes. Sixty-one percent (11/18) were found to be type 1 fusion and seven were type 2 (39%). This is the first study in India with quantitative information about the types of EWS-FLI 1 translocations present in Ewing’s family of tumours in south Indian patients.

  15. Primitive neuroectodermal tumour of kidney with thrombosis of the inferior vena cava and good responsive to surgical and medical treatment: description of a case and revision of literature.

    Science.gov (United States)

    Giliberto, Giovanni L; Di Franco, Carmelo A; Rovereto, Bruno

    2017-03-15

    Primitive neuroectodermal tumour (PNET) of kidney is a rare cancer typical of young adults with few cases described in the literature. We report a case of renal PNET in a 31-year-old man who presented to our department with a computed tomographic (CT)-scan revealing a large renal mass of 20 cm, massive thrombosis of the inferior vena cava (IVC). The patient underwent radical nephrectomy with contextual retroperitoneal lymphadenectomy and resection of IVC needing Dacron prosthesis substitution. Definitive histopathological examination showed PNET of kidney infiltrating ipsilateral adrenal gland, massive cava thrombosis with infiltration of vena cava wall and one lymph nodal metastasis. Postoperative PET-scan showed metastatic lesions in bilateral adrenal glands and pancreas. The patient received chemotherapy, and currently, he is in follow-up after 26 months from first diagnosis without any sign of recurrence of disease. Kidney PNET usually is associated with poor prognosis, so, it needs an early identification and differentiation from other similar small cells tumours in order to obtain a good response to the treatments.

  16. The role of H1 linker histone subtypes in preserving the fidelity of elaboration of mesendodermal and neuroectodermal lineages during embryonic development.

    Science.gov (United States)

    Nguyen, Giang D; Gokhan, Solen; Molero, Aldrin E; Yang, Seung-Min; Kim, Byung-Ju; Skoultchi, Arthur I; Mehler, Mark F

    2014-01-01

    H1 linker histone proteins are essential for the structural and functional integrity of chromatin and for the fidelity of additional epigenetic modifications. Deletion of H1c, H1d and H1e in mice leads to embryonic lethality by mid-gestation with a broad spectrum of developmental alterations. To elucidate the cellular and molecular mechanisms underlying H1 linker histone developmental functions, we analyzed embryonic stem cells (ESCs) depleted of H1c, H1d and H1e subtypes (H1-KO ESCs) by utilizing established ESC differentiation paradigms. Our study revealed that although H1-KO ESCs continued to express core pluripotency genes and the embryonic stem cell markers, alkaline phosphatase and SSEA1, they exhibited enhanced cell death during embryoid body formation and during specification of mesendoderm and neuroectoderm. In addition, we demonstrated deregulation in the developmental programs of cardiomyocyte, hepatic and pancreatic lineage elaboration. Moreover, ectopic neurogenesis and cardiomyogenesis occurred during endoderm-derived pancreatic but not hepatic differentiation. Furthermore, neural differentiation paradigms revealed selective impairments in the specification and maturation of glutamatergic and dopaminergic neurons with accelerated maturation of glial lineages. These impairments were associated with deregulation in the expression profiles of pro-neural genes in dorsal and ventral forebrain-derived neural stem cell species. Taken together, these experimental observations suggest that H1 linker histone proteins are critical for the specification, maturation and fidelity of organ-specific cellular lineages derived from the three cardinal germ layers.

  17. Surgical treatment of anterior cruciate ligament injury in adults.

    Science.gov (United States)

    Alazzawi, Sulaiman; Sukeik, Mohamed; Ibrahim, Mazin; Haddad, Fares S

    2016-04-01

    Anterior cruciate ligament injury is among the most common soft tissue injuries of the knee joint and reconstruction of the anterior cruciate ligament is the gold standard treatment for young active symptomatic patients. This review summarizes the surgical treatment of anterior cruciate ligament injury.

  18. Anterior diffuse scleritis diagnosed as conjunctivitis

    Directory of Open Access Journals (Sweden)

    K. P. Mashige

    2012-12-01

    Full Text Available This article presents a case of anterior diffuse scleritis that initially was diagnosed as conjunctivitis. Anterior diffuse scleritis (ADS is a potentially vision-threatening inflammation of the sclera whose etiology may include autoimmune and systemic conditions such as rheumatoid arthritis and tuberculosis. The signs and symptoms of ADS include pain, tearing, tenderness, redness, painful sensitivity to light and decreased visual acuity. Ocular and physical examinations including blood tests to rule out underlying causes are important. Medications such as corticosteroids, non-steroidal anti-inflam-matory drugs and possibly immune-suppressants are used in the management of ADS. If care is not taken, ADS can be mis-diagnosed as conjunctivitis because the redness is similar in both conditions. Such mis-diagnosis can be sight-threatening and therefore it is essential that primary eye care practitioners are cautious in all diagnoses of red eye conditions. (S Afr Optom 2012 71(1 51-54

  19. Cataract Surgery in Anterior Megalophthalmos: A Review

    Science.gov (United States)

    GALVIS, Virgilio; TELLO, Alejandro; M. RANGEL, Carlos

    2015-01-01

    Anterior megalophthalmos is characterized by megalocornea associated with a very broad anterior chamber and ciliary ring elongation. It is also called X-linked megalocornea. It is accompanied by early development of cataracts, zonular anomalies, and, rarely, vitreoretinal disorders. Subluxation of a cataract can occur in cataract surgery because of zonular weakness. In addition, in most patients, standard intraocular lens (IOL) decentration is a risk because of the enlarged sulcus and capsular bag. These unique circumstances make cataract surgery challenging. To date, several approaches have been developed. Implantation of a retropupillary iris-claw aphakic intraocular lens may be a good option because it is easier than suturing the IOL and can have better and more stable anatomic and visual outcomes, compared to other techniques. PMID:27350950

  20. [Esthetic restorations of primary anterior teeth].

    Science.gov (United States)

    Elqadir, A Jamil; Shapira, J; Ziskind, K; Ram, D

    2013-04-01

    Esthetic treatment of primary teeth is one of the greatest challenges to pediatric dentists. A variety of restorative options using full coverage are available for anterior primary teeth. In the last half century the emphasis on treatment of severely decayed primary teeth shifted from extraction to restoration. In the past, restorations consisted of placement of stainless steel crowns on severely decayed teeth. However, they are esthetically unacceptable today. Over the last decade parents expect a higher esthetic standard for their children's primary teeth. Thus, the restoration should provide esthetic appearance and durability in addition to restoring function. The purpose of this review is to describe the types of full coverage options for anterior primary teeth currently available.

  1. Displaced fracture through the anterior atlantal synchondrosis

    Energy Technology Data Exchange (ETDEWEB)

    Thakar, Chrishan; Allibone, James [Royal National Orthopaedic Hospital NHS Trust, Department of Spinal Deformity, Stanmore, Middlesex (United Kingdom); Harish, Srinivasan [Royal National Orthopaedic Hospital NHS Trust, Department of Radiology, Stanmore, Middlesex (United Kingdom); Saifuddin, Asif [Royal National Orthopaedic Hospital NHS Trust, Department of Radiology, Stanmore, Middlesex (United Kingdom); University College, The Institute of Orthopaedics and Musculoskeletal Sciences, London (United Kingdom)

    2005-09-01

    In the acute setting, accurate radiological interpretation of paediatric cervical spine trauma can be difficult due to a combination of normal variants and presence of multiple synchondroses. We present a rare case of a fracture through the anterior atlantal synchondrosis in a paediatric spine. A five-year-old boy, who fell backwards onto the top of his head while swinging across on a monkey bar frame, presented with neck pain, cervical muscle spasm and decreased right lateral rotation and extension of his neck. Computed tomography showed a displaced diastatic fracture through right anterior atlantal synchondrosis. There are only 12 cases of paediatric C1 fractures reported in the world literature. The importance of considering this diagnosis in the appropriate clinical setting, and the normal variants in the paediatric atlas that can cause diagnostic dilemma to the interpreting radiologist, are discussed in this case report. (orig.)

  2. Anterior uveitis in juvenile rheumatoid arthritis.

    Science.gov (United States)

    Kanski, J J

    1977-10-01

    The ocular and systemic characteristics of 160 patients with anterior uveitis and seronegative juvenile rheumatoid arthritis are reviewed. Chronic uveitis occurred in 131 patients, 76% of whom were girls. Both eyes were involved in 70% of the cases. Band keratopathy occurred in 41% of the eyes, cataract in 42%, and secondary glaucoma in 19%. Only 11 patients had uveitis before the onset of arthritis. Notable correlations included a pauciarticular onset of arthritis in 95% of the patients, and positive tests for antinuclear antibody in 82%. Of 29 patients with acute anterior uveitis, 27 were boys. The inflammation responded well to therapy, and serious complications did not occur. At follow-up 21 patients had typical ankylosing spondylitis, and five had sacroiliitis. The incidence of positive results of tests for HLA-B27 antigen was 94%.

  3. Anterior Tibial Artery Pseudoaneurysm: Case Report

    Directory of Open Access Journals (Sweden)

    Funda Tor

    2012-06-01

    Full Text Available The aneurysmsatic changes of the infrapopliteal arteries are rarely seen. They are pseudoaneurysms rather than true aneursyms. The most important cause of them is trauma. There is not a standart treatment for infrapopliteal aneursyms. In this study, we have evaluated a case operated for anterior tibial artery pseudoaneurysm developed after penetrant trauma and diagnosed two weeks later. [Cukurova Med J 2012; 37(3.000: 172-175

  4. THERAPY OF FIBRINOUS PLASTIC ANTERIOR UVEITIS

    Directory of Open Access Journals (Sweden)

    A. L. Onishchenko

    2015-01-01

    Full Text Available Aim. To analyze the efficacy of modified pupillary massage technique using cycloplegic agent Appamide Plus in the treatment of anterior uveitis. Patients and methods. 45 patients (25 men and 20 women aged 21‑69 with endogenous uveitis (51 eyes were enrolled in the study. Etiology of uveitis was identified in 57.7 % of cases: herpes simplex virus (22.2 %; systemic disorders — rheumatoid arthritis, Reiter syndrome, Bechterew’s disease, psoriasis (17.7 %; local infection — purulent periodontitis or sinusitis (11 %. Patients were divided into two groups. Group I received basic therapy in combination with traditional pupillary massage. Group II received basic therapy in combination with modified pupillary massage using Appamide Plus (muscarinic receptor antagonist and alpha adrenergic agonist and plasma exchange with cell mass ozonation. Results. Inflammation of the uveal tract was prevented in all patients. In group I (traditional pupil massage, posterior synechiae persisted in 26 % of cases. In group II (pupil massage using Appamide Plus, anterior chamber humor was transparent, posterior synechiae were broken, normal pupillary response was re-established. Additionally, inflammatory exudate in the anterior chamber and endothelial precipitates resolved by 3.3 days earlier than in group I (р < 0.05. Visual outcomes were also better in Appamide Plus group. T cell-mediated immunity study revealed initial CD3+ cell and T cell subpopulation deficiency. Relative and absolute references of T cells and their subpopulations tended to decrease even when uveitis signs reduced. Conclusions. T cell-mediated immunity depression is probably due to pharmacotherapy. Appamide Plus eye drops are highly effective for therapeutic mydriasis and pupillary massage in the course of anterior uveitis treatment to prevent synechiae formation in enlarged or small pupil.

  5. Anterior sacral meningocele presenting as constipation

    Directory of Open Access Journals (Sweden)

    Anup Mohta

    2011-01-01

    Full Text Available Anterior sacral meningocele (ASM is a rare form of spinal dysraphism in children. Usually asymptomatic, it can present as constipation, urinary problems or rarely neurological symptoms. High index of suspicion with careful clinical examination is necessary to make early diagnosis. Magnetic resonance imaging is the investigation of choice. We describe a successfully managed young child with ASM associated with rib and vertebral defects.

  6. 颅内原始神经外胚层肿瘤的MRI表现和病理分析%MRI Features and Pathological Analysis of Intracranial Primitive Neuroectodermal Tumors

    Institute of Scientific and Technical Information of China (English)

    黄海歆; 张勇; 崔恒

    2013-01-01

    Objective To evaluate MRI features of intracranial primitive neuroectodermal tumors (PNET). Methods The MRI features of 7 cases with intracranial PNET which were diagnosed by pathology were retrospectively analyzed and evaluated. Results The tumor masses were most commonly found in frontal, parietal, temporal and occipital lobe aside lateral ventricluars, and grew invasively. The tumors were large, with clear margin and slight edema. The tumors showed inhomogenous low T1, and equal or high T2 signal intensity, and the edge of tumors always had cystic degeneration, necrosis and hemorrhage. Tumors of 7 cases showed homogenous enhancement in enhanced scanning. Conclusion MRI manifestations of intracranial primitive neuroectodermal tumors have certain features; MRI is helpful to the diagnosis and treatment of this disease.%目的 探讨成人颅内原始神经外胚层肿瘤(primitive neuroectodermal tumors,PNET)的MRI特征,以期提高对该病的认识.方法 回顾性分析了7例经手术和病理证实的颅内PNET的MRI表现,并与手术、病理相对照.结果 肿瘤多位于侧脑室旁额颞顶叶,浸润性生长,MRI上肿瘤较大,边界较清楚,瘤周水肿轻微;平扫呈不均匀较低T1、等或高T2信号,内部常伴有囊变和坏死区,多位于病灶边缘,可有出血;增强扫描7例肿瘤实质部分有明显的不均一强化.结论 颅内PNET的MRI表现有一定特点;MRI有助于该病的诊断和指导治疗.

  7. MRI appearances of the anterior fibulocalcaneus muscle: a rare anterior compartment muscle

    Energy Technology Data Exchange (ETDEWEB)

    Upadhyay, Bhavin [Basildon and Thurrock University Hospitals NHS Foundation Trust, Imaging Department, Essex (United Kingdom); Amiras, Dimitri [Imperial College Health Care NHS Trust, Imaging Department, London (United Kingdom)

    2015-05-01

    MRI of a 62-year-old female presenting with ankle pain demonstrated an accessory muscle within the anterior compartment of the lower leg. The muscle originated from the fibula and anterior crural septum. The tendon passed anterior to the lateral malleolus and inserted at the critical angle of Gissane on the calcaneus. This muscle was initially described in the anatomic literature by Lambert and Atsas in 2010. To our knowledge, this is the first time the MRI appearances of this muscle has been described in the radiological literature. Awareness of the fibulocalcaneal muscle is important as it may represent a cause of ankle pain. In addition, the tendon could potentially be harvested for use in reconstructive procedures. (orig.)

  8. Non-cerebellar primitive neuroectodermal tumors (PNET): summary of the Milan consensus and state of the art workshop on marrow ablative chemotherapy with hematopoietic cell rescue for malignant brain tumors of childhood and adolescents.

    Science.gov (United States)

    Fangusaro, Jason; Massimino, Maura; Rutkowski, Stefan; Gururangan, Sridharan

    2010-04-01

    CNS non-cerebellar primitive neuroectodermal tumors (PNET) represent <5% of all childhood brain tumors. Despite similar therapies, these patients have had significantly worse outcomes compared to patients with medulloblastoma. Although these tumors have historically been considered analogous to medulloblastoma, there is growing evidence that they are biologically distinct. Since these tumors are relatively rare, there are few large series in the literature. Here we present the international experience treating these patients with a variety of therapies, including marrow ablative chemotherapy followed by autologous hematopoietic cell rescue.

  9. Renal primitive neuroectodermal tumor as a second malignancy after chemotherapy and radiation for Non-Hodgkin's Lymphoma--treatment-related or just poor old bad luck?: A case report.

    Science.gov (United States)

    de Menezes, Jean-Louis; Patil, Hitendra M; Kannan, R; Pradhan, Sultan A

    2015-01-01

    Peripheral primitive neuroectodermal tumor (PNET) is a rare histology to be found in primary tumors of the kidney. There are less than a hundred cases reported in the English literature. Most of these have been diagnosed after surgery for a renal neoplasm diagnosed on imaging. PNET has rarely been reported as a second malignancy, and has never been reported as a second malignancy after non-Hodgkin's lymphoma (NHL). Herein, we present our case of a 38-year-old female who developed a second malignancy in the kidney after the treatment for NHL.

  10. Manifestation and literature review of primitive neuroectodermal tumor imaging%原始神经外胚层肿瘤影像表现与文献回顾

    Institute of Scientific and Technical Information of China (English)

    段世军; 秦越; 王玮; 崔光彬; 李挚; 杜滂

    2012-01-01

    Objective To analyze the imaging and pathological manifestation of primitive neuroectodermal tumors( PNETs )in order to improve the understanding of this disease. Methods A retrospective analysis was made in the clinical symptoms and CT and MRI signs of 8 patients with PNETs who had complete imaging data and were pathologically confirmed. Results PNETs images showed similar or slightly longer T1 signal, inhomogeneous long T2 signal and high signal changes of STIR sequences. All the 8 cases were not calcified, in which 5 showed inhomogeneous enhancements after the routine enhancement scanning. The specific pathological manifestation of PNETs was Homer - Wright rosettes. Immunohistochemical results showed that the CD99 was expressed in all tumors and epithelial membrane antigen( EMA ), neurofilament protein( NF ), synaptophysin( Syn ). Vimentin and S - 100 were also expressed in different degrees in those tumors. Conclusion The clinical symptoms of PNETs have certain characteristics which can be diagnosed according to imageology manifestations and clinical data. CT and MRI examinations play significant roles in finding the location, size, internal structure, the extent of the tumor and its correlation with the adjacent tissues, as well as in the formulation of treatment plan, evaluation of the therapeutic efficacy and the control check.%目的 分析原始神经外胚层肿瘤(primitive neuroectodermal tumors,PNETs)的影像学及病理学表现,以提高对本病的认识.方法 回顾性分析有完整影像学资料及经病理证实的8例PNETs的临床症状和CT、MRI征象.结果 原始神经外胚层肿瘤呈等或稍长T1、不均匀长T2信号,STIR序列呈高信号改变.8例均没有钙化,其中5例行增强扫描,呈不均匀强化.病理形态上Home-Wright菊形团为其特异性表现.免疫组织化学结果,肿瘤均表达CD99,并不同程度地表达上皮细胞膜抗原EMA、神经微丝蛋白NF、突触素Syn、Vimentin、S-100.结论 PNETs临

  11. 肾脏外周型原始神经外胚层肿瘤影像诊断%Imaging diagnosis of peripheral primitive neuroectodermal tumor in kidney

    Institute of Scientific and Technical Information of China (English)

    刘欣; 周小兵; 刘波; 荣凡令

    2016-01-01

    Objective To investigate the imaging manifestations and diagnostic characteristics of peripheral primitive neuroec-todermal tumor ( pPNET) in kidney.Methods The clinical, ultrasonographic, CT and pathological features in 4 cases with pP-NET in kidney proved by operative and pathological results were reviewed retrospectively.Results The ultrasonograghic appear-ance of the tumors showed a hybrid echo-mass with irregular echo free zone inside, and most of the tumors were detected blood flow signal without high resistance index ( RI);the CT appearance of the tumors showed large cyst-solid mixed soft tissue masses with capsules, and the inside of the tumors showed some separation structures and a small amount of blood and calcifications.In CT dynamic enhancement, the solid component of the tumors showed slight enhancement in arterial phase and still slight enhance-ment in delay phases, the liquid area always showed no enhancement, but the separation structures were enhanced slightly.In some cases,there were emboli in renal veins or inferior cava veins.Conclusion The pPNET formed in kidney has no characteris-tic manifestations in ultrasonography, however, it has some on CT scan.CT can detect the internal structures and the extent of the tumor, identify whether there is some distant metastasizes, and provide reference value to the establishment of surgical operation and the assessment of response to treatment.%目的:探讨肾脏外周型原始神经外胚层肿瘤( peripheral primitive neuroectodermal tumor, pPNET)的影像学表现及诊断特征。方法回顾分析4例经术后病理证实的发生于肾脏的pPNET的临床表现、超声征象、CT征象及其病理表现。结果肿瘤声像图均表现为混合回声团块,伴有不规则无回声区,大部分瘤内可探及血流信号,阻力指数( RI)不高;CT图像肿瘤均表现为体积较大的、有包膜的囊实性软组织肿块,内部可见分隔,可有少量出血及钙化,

  12. Toxic anterior-segment syndrome (TASS

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    Cetinkaya S

    2014-10-01

    Full Text Available Servet Cetinkaya,1 Zeynep Dadaci,2 Hüsamettin Aksoy,3 Nursen Oncel Acir,2 Halil Ibrahim Yener,4 Ekrem Kadioglu5 1Ophthalmology Clinics, Turkish Red Crescent Hospital, Konya, 2Department of Ophthalmology, Faculty of Medicine, Mevlana University, Konya, 3Ophthalmology Clinics, Karaman State Hospital, Karaman, 4Konya Eye Center Hospital, Konya, 5Ophthalmology Clinics, Beyhekim State Hospital, Konya, Turkey Purpose: To evaluate the clinical findings and courses of five patients who developed toxic anterior-segment syndrome (TASS after cataract surgery and investigate the cause.Materials and methods: In May 2010, on the same day, ten patients were operated on by the same surgeon. Five of these patients developed TASS postoperatively.Results: Patients had blurred-vision complaints on the first day after the operation, but no pain. They had different degrees of diffuse corneal edema, anterior-chamber reaction, fibrin, hypopyon, iris atrophies, and dilated pupils. Their vision decreased significantly, and their intraocular pressures increased. Both anti-inflammatory and antiglaucomatous therapies were commenced. Corneal edema and inflammation resolved in three cases; however, penetrating keratoplasty was needed for two cases and additional trabeculectomy was needed for one case. Although full investigations were undertaken at all steps, we could not find the causative agent.Conclusion: TASS is a preventable complication of anterior-segment surgery. Recognition of TASS, differentiating it from endophthalmitis, and starting treatment immediately is important. Controlling all steps in surgery, cleaning and sterilization of the instruments, and training nurses and other operation teams will help us in the prevention of TASS. Keywords: cataract, phacoemulsification, TASS, corneal edema, inflammation

  13. Infections after reconstructions of anterior cruciate ligament

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    Ristić Vladimir

    2014-01-01

    Full Text Available Introduction. Infections after anterior cruciate ligament reconstructions are rare, but, on the other hand, they are difficult to be treated. The aim of this study was to analyze causes of infections, risk factors, diagnostics, and possibilities of their prevention. Material and Methods. Seventeen deep infections (1.2% were found in 1425 patients who had undergone anterior cruciate ligament reconstructions. Fifteen patients were males and two were females. Out of 475 professional athletes nine (1.9% had this postoperative complication. Eleven patients with septic arthritis were allergic to penicillin. Three of them had immunosuppressive diseases. Results. Staphylococcus aureus was isolated in eleven cases (65%, other Staphylococcus and Streptococcus groups were found in four and three patients, respectively; while one patient had infection although the punctate was negative. Out of 965 patients with the patellar tendon grafts, ten (1.03% had this complication, while the incidence was 1.52% (7/460 in those with the hamstring grafts. Fifteen infections were acute with obvious symptoms within 14 days after surgery. Severe pain, limited range of motion, swelling of the knee joint and fever were the most common symptoms, while rubor and pus developed rarely. The infection was three times more frequent in the patients who had undergone surgery lasting more than 1.5 hour. Discussion and Conclusion. The following population groups are at risk of developing septic arthritis after anterior cruciate ligament reconstructions: professional athletes, those who are allergic to penicillin, and those with immunosuppressive diseases. Staphyllococus aureus is the most common cause of infection. The patients with the hamstring autografts have a higher risk than those with the patellar tendon grafts. Preventive measures that should be performed include aseptic conditions in operative rooms, irrigation of the graft before its placement into the bone tunnels

  14. Tumor neuroectodérmico primitivo da bexiga urinária: uma rara neoplasia Primitive neuroectodermal tumor of the urinary bladder: a rare neoplasm

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    Daniel Cury Ogata

    2010-02-01

    Full Text Available Os autores relatam o caso de paciente do sexo feminino, 52 anos, com queixas de hematúria macroscópica e dor pélvica. Foi realizado exame ultrassonográfico, que mostrou massa expansiva em assoalho vesical. A cistoscopia confirmou a presença dessa lesão, sendo realizada biópsia. O exame histológico revelou neoplasia de pequenas células. A análise imuno-histoquímica foi necessária para elucidação diagnóstica. O referido exame demonstrou positividade para os marcadores EWS-FLI1, CD99 e S-100. O diagnóstico foi de tumor neuroectodérmico primitivo. A paciente foi tratada com quimioterapia adjuvante. Depois de sete meses de seguimento, a paciente encontra-se livre de doença.The authors report the case of a 52 year-old woman that presented macroscopic hematuria and pelvic pain. Ultrasound examination was performed, which showed an expansive mass in the bladder floor. A cystoscopy confirmed the presence of this lesion and a biopsy was carried out. Histological analysis showed a small cell neoplasm. The immunohistochemical analysis was required to establish diagnosis. This analysis revealed positivity for EWS-FLI1, CD99 and S-100. The conclusive diagnosis was primitive neuroectodermal tumor. The patient was treated with chemotherapy. After a seven month follow-up period, the patient is disease-free.

  15. Cyclin D1 is a useful marker for soft tissue Ewing's sarcoma/peripheral Primitive Neuroectodermal Tumor in children and adolescents: A comparative immunohistochemical study with rhabdomyosarcoma.

    Science.gov (United States)

    Magro, Gaetano; Brancato, Franca; Musumeci, Giuseppe; Alaggio, Rita; Parenti, Rosalba; Salvatorelli, Lucia

    2015-01-01

    Cyclin D1 amplification and/or overexpression contribute to the loss of the regulatory circuits that govern G1-S transition phase of the cell cycle, playing pivotal roles in different human malignant tumors, including breast, colon, prostate cancer, lymphoma, melanoma and neuroblastoma. In vitro studies have shown that cyclin D1 is overexpressed in Ewing's sarcoma (EWS)/peripheral Primitive Neuroectodermal Tumor (pPNET), but not in rhabdomyosarcoma cell lines. Only a few immunohistochemical studies are available on cyclin D1 expression in EWS/pPNET, which confirmed its expression only in a limited number of cases. The aim of the present study was a comparative immunohistochemical analysis of the expression and distribution of cyclin D1 in a large series of pediatric/adolescent soft tissue EWS/pPNETs and rhabdomyosarcomas (both embryonal and alveolar subtypes) to assess its potential usefulness in their differential diagnosis. Notably cyclin D1 was strongly and diffusely expressed in all cases (20/20) of EWS/pPNET, while it was lacked in all cases (15/15) of rhabdomyosarcomas. Immunohistochemical overexpression of cyclin D1 in EWS/pPNET is a novel finding which could be exploitable as a diagnostic immunomarker for this tumor. Although highly sensitive, cyclin D1 is not specific for EWS/pPNET, and thus it should not be evaluated alone but in the context of a wide immunohistochemical panel. Accordingly, we first emphasize that when pathologists are dealing with a small round blue cell tumor of soft tissues in pediatric/adolescent patients, a strong and diffuse nuclear expression of cyclin D1 is of complementary diagnostic value to CD99 and FLI-1 in confirming diagnosis of EWS/pPNET and in ruling out rhabdomyosarcoma.

  16. 原始神经外胚层肿瘤的研究进展%Research progress of primitive neuroectodermal tumor

    Institute of Scientific and Technical Information of China (English)

    李文一; 周俊林(审校)

    2014-01-01

    原始神经外胚层肿瘤(PNET)是罕见的高度恶性小圆细胞肿瘤,可分为中枢型(cPNET)与外周型(pPNET)。两者除发病部位不同以外,在好发年龄、转移途径、免疫组化染色、染色体易位等方面也不同。因此需对两者进行鉴别,尤其是病灶邻近脑膜或位于椎管内时更有必要。对cPNET及pPNET的肿瘤遗传学、临床表现、病理诊断、影像表现、治疗和预后,以及cPNET与pPNET,cPENT与髓母细胞瘤,pPNET 与尤文氏肉瘤之间的关系等进行综述。%Primitive neuroectodermal tumor is a rare and highly malignant small round cell tumor. It can be divided into central PNET (cPNET) and peripheral PNET (pPNET). In addition to the lesions’ location, however,they are different in predilection age, route of metastasis, immunohistochemical staining, and chromosome translocation. So it is very necessary to identify each other, especially, when the lesions are close to the meninges or located in the vertebral canal. The aim of this review was to investigate the tumor genetics, clinical manifestation, pathologic diagnosis, imaging findings, treatment, and prognosis of the two kinds of tumors, also investigate the relationships between cPNET and pPNET, cPENT and medulloblastoma, pPNET and Ewing’s sarcoma.

  17. Diagnosis and treatment of primitive neuroectodermal tumor%原始神经外胚层瘤的诊断和治疗

    Institute of Scientific and Technical Information of China (English)

    姜龙; 龙浩

    2011-01-01

    原始神经外胚层瘤是一类好发于儿童及青少年的高度恶性肿瘤,发病率呈上升趋势,其早期诊断率较低,治疗效果欠佳。随着逆转录PCR、荧光原位杂交技术等分子生物学技术应用于病理诊断领域,磁共振、正电子发射型计算机断层显像(PET) -CT等应用于影像学诊断领域;靶向治疗、多学科综合治疗理念在治疗中的贯彻,原始神经外胚层瘤的发病机制、诊断技术与治疗手段取得了突破性的进展。%Primitive neuroectodermal tumor (PNET) is a kind of malignant tumor, which most often affects children and adolescents. The incidence of PNET has risen recently. PNET has low early diagnosis rate and poor treatment effect. With the application of RT-PCR and FISH in pathological diagnosis, and MRI and PET-CT in imageological diagnosis, as well as the implementation of target treatment and multidisciplinary integrated treatment concept in the treatment, the pathogenesis, diagnosis technology and treatment means of of PNET have made remarkable progress.

  18. Risk factors for astrocytic glioma and primitive neuroectodermal tumor of the brain in young children: a report from the Children's Cancer Group.

    Science.gov (United States)

    Bunin, G R; Buckley, J D; Boesel, C P; Rorke, L B; Meadows, A T

    1994-01-01

    We conducted a matched case-control study to investigate risk factors for the two most common types of brain tumors in children, astrocytic glioma and primitive neuroectodermal tumor (PNET). Since the study focused on gestational exposures, we restricted it to young children because these exposures would be expected to act early in life. Parents of 155 astrocytic glioma cases, 166 PNET cases, and controls identified by random digit dialing completed telephone interviews. Few associations occurred with the hypothesized risk factors, which were gestational exposure to alcohol, hair coloring products, farms, and substances containing N-nitroso compounds (passive smoking, makeup, incense, new cars, pacifiers, baby bottles, beer). Of the products studied that contain N-nitroso compounds, only beer was associated with a significantly increased risk of either tumor type [odds ratio (OR) for PNET = 4.0; 95% confidence interval (CI), 1.1-22.1; P = 0.04]. Elevated ORs for PNET were observed for farm residence of the mother during the pregnancy (OR = 3.7; 95% CI, 0.8-23.9; P = 0.06) and of the child for at least a year (OR = 5.0; 95% CI, 1.1-46.8; P = 0.04). Significant associations with astrocytoma were observed for mother's use of kerosene (OR = 8.9; 95% CI, 1.1-71.1; P = 0.04) and birth by Caesarean section (OR = 1.8; 95% CI, 1.1-3.2; P = 0.03). History of miscarriage was associated with a lower risk of PNET (OR = 0.5; 95% CI, 0.3-0.9; P = 0.02).(ABSTRACT TRUNCATED AT 250 WORDS)

  19. Dynamic association with donor cell filopodia and lipid-modification are essential features of Wnt8a during patterning of the zebrafish neuroectoderm.

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    Marta Luz

    Full Text Available BACKGROUND: Wnt proteins are conserved signaling molecules that regulate pattern formation during animal development. Many Wnt proteins are post-translationally modified by addition of lipid adducts. Wnt8a provides a crucial signal for patterning the anteroposterior axis of the developing neural plate in vertebrates. However, it is not clear how this protein propagates from its source, the blastoderm margin, to the target cells in the prospective neural plate, and how lipid-modifications might influence Wnt8a propagation and activity. RESULTS: We have dynamically imaged biologically active, fluorescently tagged Wnt8a in living zebrafish embryos. We find that Wnt8a localizes to membrane-associated, punctate structures in live tissue. In Wnt8a expressing cells, these puncta are found on filopodial cellular processes, from where the protein can be released. In addition, Wnt8a is found colocalized with Frizzled receptor-containing clusters on signal receiving cells. Combining in vitro and in vivo assays, we compare the roles of conserved Wnt8a residues in cell and non-cell-autonomous signaling activity and secretion. Non-signaling Wnt8 variants show these residues can regulate Wnt8a distribution in producing cell membranes and filopodia as well as in the receiving tissue. CONCLUSIONS: Together, our results show that Wnt8a forms dynamic clusters found on filopodial donor cell and on signal receiving cell membranes. Moreover, they demonstrate a differential requirement of conserved residues in Wnt8a protein for distribution in producing cells and receiving tissue and signaling activity during neuroectoderm patterning.

  20. The Anterior Cingulate Cortex and Pain Processing

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    Perry Neil Fuchs

    2014-05-01

    Full Text Available The neural network that contributes to the suffering which accompanies persistent pain states involves a number of brain regions. Of primary interest is the contribution of the cingulate cortex in processing the affective component of pain. The purpose of this review is to summarize recent data obtained using novel behavioral paradigms in animals based on measuring escape and/or avoidance of a noxious stimulus. These paradigms have successfully been used to study the nature of the neuroanatomical and neurochemical contributions of the anterior cingulate cortex to higher order pain processing in rodents.

  1. Biological fixation in anterior cruciate ligament surgery

    Directory of Open Access Journals (Sweden)

    Chih-Hwa Chen

    2014-04-01

    Full Text Available Successful anterior cruciate ligament (ACL reconstruction with tendon graft requires extensive tendon-to-bone healing in the bone tunnels and progressive graft ligamentization for biological, structural, and functional recovery of the ACL. Improvement in graft-to-bone healing is crucial for facilitating early, aggressive rehabilitation after surgery to ensure an early return to pre-injury activity levels. The use of various biomaterials for enhancing the healing of tendon grafts in bone tunnels has been developed. With the biological enhancement of tendon-to-bone healing, biological fixation of the tendon graft in the tunnel can be achieved in ACL reconstruction.

  2. Tunnel widening in anterior cruciate ligament reconstruction

    DEFF Research Database (Denmark)

    Clatworthy, M G; Annear, P; Bulow, J U

    1999-01-01

    .1% in the patella tendon group (P = film measurements. Tunnel widening did not correlate with the clinical findings, knee scores, KT-1000 or isokinetic muscle strength. Tunnel widening is marked in the hamstring group. Tunnel widening does not correlate with instability......We report a prospective series evaluating the incidence and degree of tunnel widening in a well-matched series of patients receiving a hamstring or patella tendon graft for anterior cruciate ligament (ACL) deficiency. We correlated tunnel widening with clinical factors, knee scores, KT-1000...

  3. [LAPAROSCOPIC ANTERIOR UTERINE LIGAMENTOPEXY--OUR EXPERIENCE].

    Science.gov (United States)

    Blagovest, Bechev; Magunska, Nadya; Kovachev, Emil; Ivanov, Stefan

    2015-01-01

    A great number operative techniques for correction of retroverted uterus are reported in the last years. The aim of these different methods is correction of the retroversion of the uterus, which is connected with pelvic congestion and symptomatic relief. We present a clinical case of 26 years old patient with one Caesarean section. The lady complains of chronic pelvic pain, dispareunia and dismenorrhea. The US exam shows an uterus in strong retroversion position. We restored the anatomic position of the uterus using laparoscopic anterior ligementopexy.

  4. Anterior segment spectral domain optical coherence tomography imaging of patients with anterior scleritis.

    Science.gov (United States)

    Levison, Ashleigh L; Lowder, Careen Y; Baynes, Kimberly M; Kaiser, Peter K; Srivastava, Sunil K

    2016-08-01

    The purpose of the study was to describe the findings seen on anterior segment spectral domain optical coherence tomography (SD-OCT) in patients with anterior scleritis and determine the feasibility of using SD-OCT to image and grade the degree of scleral inflammation and monitor response to treatment. All patients underwent slit lamp examination by a uveitis specialist, and the degree of scleral inflammation was recorded. Spectral domain OCT imaging was then performed of the conjunctiva and scleral tissue using a standardized acquisition protocol. The scans were graded and compared to clinical findings. Twenty-eight patients with anterior scleritis and ten patients without ocular disease were included in the study. Seventeen of the scleritis patients were followed longitudinally. Common findings on SD-OCT in patients with active scleritis included changes in hyporeflectivity within the sclera, nodules, and visible vessels within the sclera. There was significant variation in findings on SD-OCT within each clinical grade of active scleritis. These changes on SD-OCT improved with treatment and clinical improvement. SD-OCT imaging provided various objective measures that could be used in the future to grade inflammatory activity in patients with anterior scleritis. Longitudinal imaging of patients with active scleritis demonstrated that SD-OCT may have great utility in monitoring response to treatment.

  5. Late spontaneous resolution of a double anterior chamber post deep anterior lamellar keratoplasty

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    Andrea Passani

    2017-01-01

    Full Text Available A 31-year-old healthy male underwent deep anterior lamellar keratoplasty with big-bubble technique for treatment of keratoconus in his right eye. One week after surgery, he presented with detachment of the endothelium-Descemet complex with formation of a double anterior chamber, despite the apparent absence of an intraoperative Descemet membrane rupture. A subsequent intervention with the intent to relocate the corneal graft button was not effective, because the detachment appeared again one day later. The authors hypothesized that, at the time of the stromal dissection with big bubble technique, a small amount of air penetrated into the anterior chamber, creating a false pathway through the trabecular meshwork. The aqueous humor then penetrated the graft flowing through the false pathway, causing the endothelium-Descemet detachment. The persistence of that pathway, even after the intervention of graft repositioning, caused the failure of the latter procedure and persistence of the double chamber. We decided to wait and observe. The double anterior chamber spontaneously resolved in approximately three months.

  6. Accuracy of Lachman and Anterior Drawer Tests for Anterior Cruciate Ligament Injuries

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    Hadi Makhmalbaf

    2013-12-01

    Full Text Available   Background: The knee joint is prone to injury because of its complexity and weight-bearing function. Anterior cruciate ligament (ACL ruptures happen in young and physically active population and can result in instability, meniscal tears, and articular cartilage damage. The aim of this study is to evaluate the accuracy of Lachman and anterior drawer test in ACL injury in compare with arthroscopy.   Methods: In a descriptive, analytical study from 2009 to 2013, 653 patients who were suspected to ACL rapture were entered the study. Statistical analysis was performed by the usage of SPSS 19.0. Multiple comparison procedure was performed for comparing data between clinical examination and arthroscopic findings and their relation with age and sex. Results: Mean age of patients was 28.3±7.58 years (range from 16 to 68 years. From 428 patients, 41.2% (175 patients were between 26 and 35, 38.8% (165 ones between 15 and 25 and 20% (85 patients over 36 years. 414 patients were male (97.2% and 12 were female (2.8%. Sensitivity of anterior drawer test was 94.4% and sensitivity of Lachman test was 93.5%. Conclusion: The diagnosis and decision to reconstruct ACL injury can be reliably made regard to the anterior drawer and Lachman tests result. The tests did not have privilege to each other. These test accuracy increased considerably under anesthesia especially in women.

  7. Accuracy of Lachman and Anterior Drawer Tests for Anterior Cruciate Ligament Injuries

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    Hadi Makhmalbaf

    2013-12-01

    Full Text Available Background: The knee joint is prone to injury because of its complexity and weight-bearing function. Anterior cruciate ligament (ACL ruptures happen in young and physically active population and can result in instability, meniscal tears, and articular cartilage damage. The aim of this study is to evaluate the accuracy of Lachman and anterior drawer test in ACL injury in compare with arthroscopy.   Methods: In a descriptive, analytical study from 2009 to 2013, 653 patients who were suspected to ACL rapture were entered the study. Statistical analysis was performed by the usage of SPSS 19.0. Multiple comparison procedure was performed for comparing data between clinical examination and arthroscopic findings and their relation with age and sex. Results: Mean age of patients was 28.3±7.58 years (range from 16 to 68 years. From 428 patients, 41.2% (175 patients were between 26 and 35, 38.8% (165 ones between 15 and 25 and 20% (85 patients over 36 years. 414 patients were male (97.2% and 12 were female (2.8%. Sensitivity of anterior drawer test was 94.4% and sensitivity of Lachman test was 93.5%. Conclusion: The diagnosis and decision to reconstruct ACL injury can be reliably made regard to the anterior drawer and Lachman tests result. The tests did not have privilege to each other. These test accuracy increased considerably under anesthesia especially in women.

  8. The normal anterior cruciate ligament as a model for tensioning strategies in anterior cruciate ligament grafts

    NARCIS (Netherlands)

    Arnold, MP; Verdonschot, N; van Kampen, A

    2005-01-01

    Background: There is some confusion about the relationship between the tension placed on the graft and the joint position used in the fixation of anterior cruciate ligament grafts. This is because of deficiency in accurate basic science about this important interaction in the normal and reconstructe

  9. The normal anterior cruciate ligament as a model for tensioning strategies in anterior cruciate ligament grafts.

    NARCIS (Netherlands)

    Arnold, M.P.; Verdonschot, N.J.J.; Kampen, A. van

    2005-01-01

    BACKGROUND: There is some confusion about the relationship between the tension placed on the graft and the joint position used in the fixation of anterior cruciate ligament grafts. This is because of deficiency in accurate basic science about this important interaction in the normal and reconstructe

  10. Anterior cutaneous nerve entrapment syndrome: management challenges

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    Chrona E

    2017-01-01

    Full Text Available Eleni Chrona,1,2 Georgia Kostopanagiotou,1 Dimitrios Damigos,3 Chrysanthi Batistaki1 1Second Department of Anesthesiology, School of Medicine, National and Kapodistrian University of Athens, “Attikon” Hospital, Athens, 2Department of Anesthesiology, General Hospital of “Ag. Panteleimon,” Piraeus, 3Department of Medical Psychology, Medical School of Ioannina, University of Ioannina, Ioannina, Greece Abstract: Anterior cutaneous nerve entrapment syndrome (ACNES is a commonly underdiagnosed and undertreated chronic state of pain. This syndrome is characterized by the entrapment of the cutaneous branches of the lower thoracoabdominal intercostal nerves at the lateral border of the rectus abdominis muscle, which causes severe, often refractory, chronic pain. This narrative review aims to identify the possible therapeutic strategies for the management of the syndrome. Seventeen studies about ACNES therapy were reviewed; of them, 15 were case–control studies, case series, or case reports, and two were randomized controlled trials. The presently available management strategies for ACNES include trigger point injections (diagnostic and therapeutic, ultrasound-guided blocks, chemical neurolysis, and surgical ­neurectomy, in combination with systemic medication, as well as some emerging techniques, such as radiofrequency ablation and neuromodulation. An increased awareness of the syndrome and the use of specific diagnostic criteria for its recognition are required to facilitate an early and successful management. This review compiles the proposed ­management strategies for ACNES. Keywords: anterior cutaneous nerve entrapment syndrome, intercostal, neuralgia, management

  11. Adjacent Segment Pathology after Anterior Cervical Fusion.

    Science.gov (United States)

    Chung, Jae Yoon; Park, Jong-Beom; Seo, Hyoung-Yeon; Kim, Sung Kyu

    2016-06-01

    Anterior cervical fusion has become a standard of care for numerous pathologic conditions of the cervical spine. However, subsequent development of clinically significant disc disease at levels adjacent to fused discs is a serious long-term complication of this procedure. As more patients live longer after surgery, it is foreseeable that adjacent segment pathology (ASP) will develop in increasing numbers of patients. Also, ASP has been studied more intensively with the recent popularity of motion preservation technologies like total disc arthroplasty. The true nature and scope of ASP remains poorly understood. The etiology of ASP is most likely multifactorial. Various factors including altered biomechanical stresses, surgical disruption of soft tissue and the natural history of cervical disc disease contribute to the development of ASP. General factors associated with disc degeneration including gender, age, smoking and sports may play a role in the development of ASP. Postoperative sagittal alignment and type of surgery are also considered potential causes of ASP. Therefore, a spine surgeon must be particularly careful to avoid unnecessary disruption of the musculoligamentous structures, reduced risk of direct injury to the disc during dissection and maintain a safe margin between the plate edge and adjacent vertebrae during anterior cervical fusion.

  12. MRI of anterior cruciate ligament autografts

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    Ogi, Shigeyuki; Ariizumi, Mitsuko; Yamagishi, Tsuneo [The Aoyama Tokyo Metropolitan office' s Hospital (Japan); Agata, Toshihiko; Tada, Shinpei; Fukuda, Kunihiko

    2000-09-01

    The purpose of this study was to assess the usefulness of MRI in the evaluation of autografts after anterior cruciate ligament reconstruction. The subjects were 110 patients with anterior cruciate ligament reconstruction using patellar tendon autografts who underwent clinical examination, MRI, and arthroscopy of the knee. T1- and T2-weighted MR images were obtained in sagittal plane. Clinical findings were categorized into three groups: normal, borderline, and abnormal. The MRI appearances of the autografts were categorized into three types: straight continuous band (type I), interrupted band (type II) and generalized increased intensity band (type III). The clinical findings and MRI findings were compared with arthroscopic findings. Ninety-six percent of the type I showed no autograft tear on arthroscopy. In comparison with the clinical findings, MRI was found to be well correlated with arthroscopic findings. In conclusion, if the clinical findings are normal, patients are to be followed-up without MRI and arthroscopy. However, if clinical findings are either borderline or abnormal, MRI should be performed prior to arthroscopy. (author)

  13. Penghentian Karies Gigi Sulung Anterior (Laporan Penelitian

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    Titi Pratiwi Indra Yoga

    2015-10-01

    Full Text Available Salah satu cara menanggulangi karies pada gigi sulung anterior adalah dengan mengasah gigi menjadi bentuk self cleansing atau tapered dan kemudian mengolesnya dengan larutan SnF2 10%. Cara ini relatif mudah mengerjakannya serta murah biayanya. Keburukannya hanya pada masalah estetis, yaitu bentuk gigi menjadi lebih kecil, serta adanya staining kecoklatan karena pengendapan Sn. Penelitian ini dilakukan untuk mengetahui apakah cara ini dapat menghentikan karies pada gigi sulung anterior, serta sampai berapa lama pengaruh SnF2 10% dapat menghentikan karies. Sampel diambil dari murid TK yang berusia 2,5 sampai 5 tahun, gigi sulung anteriornya terkena karies email pada bagian proksimal satu atau dua sisi. Mengingat usia sampel yang masih muda, maka sampai akhir penelitian hanya didapat 20 orang anak yang memenuhi kriteria, dan hasil penelitian dihitung secara statistik dengan X2 – test. Hasil penelitian ternyata prosedur perawatan ini berhasil secara bermakna setelah 3-6 bulan (X2 = 0.056, df = 1, dan p < 0.05.

  14. A Rare Nasal Bone Fracture: Anterior Nasal Spine Fracture

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    Egemen Kucuk

    2014-04-01

    Full Text Available Anterior nasal spine fractures are a quite rare type of nasal bone fractures. Associated cervical spine injuries are more dangerous than the nasal bone fracture. A case of the anterior nasal spine fracture, in a 18-year-old male was presented. Fracture of the anterior nasal spine, should be considered in the differential diagnosis of the midface injuries and also accompanying cervical spine injury should not be ignored.

  15. Winging of scapula due to serratus anterior tear

    Institute of Scientific and Technical Information of China (English)

    Varun Kumar Singh; Gauresh Shantaram Vargaonkar

    2014-01-01

    Winging of scapula occurs most commonly due to injury to long thoracic nerve supplying serratus anterior muscle.Traumatic injury to serratus anterior muscle itself is very rare.We reported a case of traumatic winging of scapula due to tear of serratus anterior muscle in a 19-year-old male.Winging was present in neutral position and in extension of right shoulder joint but not on "push on wall" test.Patient was managed conservatively and achieved satisfactory result.

  16. Transanal stent in anterior resection does not prevent anastomotic leakage

    DEFF Research Database (Denmark)

    Bülow, Steffen; Bulut, O; Christensen, Ib Jarle;

    2006-01-01

    OBJECTIVE: A defunctioning transanal stent may theoretically reduce the leakage rate after anterior rectal resection. We present a randomized open study with the aim of comparing the leakage rate after anterior resection with a loop ileostomy, a transanal stent, both or neither. PATIENTS....... On this basis it was decided to discontinue the study prematurely for ethical reasons. CONCLUSION: Decompression of the anastomosis with a transanal stent does not reduce the risk of anastomotic leakage after anterior resection....

  17. Double anterior chamber in a patient with glaucoma and microspherophakia

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    Hamid Khakshoor

    2011-01-01

    Full Text Available We report the case of a 16-year-old woman with microspherophakia and secondary open angle glaucoma. The patient presented with a membrane dividing the anterior chamber into two segments without edema or Descemet′s membrane detachment. Slit lamp biomicroscopy, Pentacam, and specular microscopy images were obtained. Double anterior chamber is primarily found in patients with anterior chamber anomalies when there is no history of surgery or trauma.

  18. Early Diagnosis of Keratoconus with Orbscan- Ⅱ Anterior System

    Institute of Scientific and Technical Information of China (English)

    李新宇; 刘磊; 邱良秀

    2002-01-01

    Summary: Orbscan- Ⅱ anterior system was used for early diagnosis of keratoconus. 48 Eyes of 24patients with suspicious keratoconus were examined by Orbscan-Ⅱ anterior system from Dec.1999 to Dec. 2000 and followed up. The values of Diff and anterior chamber depth (ACD) wererecorded. Results indicated that values of Diff and ACD were increased in 4 eyes of 2 patients withkeratoconus trend during follow-up. Taking advantage of Orbscan- Ⅱ anterior system to observethe values of Diff and ACD can early diagnose the sub-clinical keratoconus. The values of Diff andACD can sensitively report the progression of keratoconus.

  19. Morphogenesis of the anterior segment in the zebrafish eye

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    Link Brian A

    2005-06-01

    Full Text Available Abstract Background The ocular anterior segment is critical for focusing incoming light onto the neural retina and for regulating intraocular pressure. It is comprised of the cornea, lens, iris, ciliary body, and highly specialized tissue at the iridocorneal angle. During development, cells from diverse embryonic lineages interact to form the anterior segment. Abnormal migration, proliferation, differentiation, or survival of these cells contribute to diseases of the anterior segment such as corneal dystrophy, lens cataract, and glaucoma. Zebrafish represent a powerful model organism for investigating the genetics and cell biology of development and disease. To lay the foundation for genetic studies of anterior segment development, we have described the morphogenesis of this structure in zebrafish. Results As in other vertebrates, the zebrafish anterior segment derives from diverse origins including surface ectoderm, periocular mesenchyme, and neuroepithelium. Similarly, the relative timing of tissue differentiation in the anterior segment is also conserved with other vertebrates. However, several morphogenic features of the zebrafish anterior segment differ with those of higher vertebrates. These include lens delamination as opposed to invagination, lack of iris muscles and ciliary folds, and altered organization in the iridocorneal angle. In addition, substantial dorsal-ventral differences exist within the zebrafish anterior segment. Conclusion Cumulatively, our anatomical findings provide a reference point to utilize zebrafish for genetic studies into the mechanisms of development and maintenance of the anterior segment.

  20. Winging of scapula due to serratus anterior tear

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    Varun Singh Kumar

    2014-10-01

    Full Text Available 【Abstract】Winging of scapula occurs most commonly due to injury to long thoracic nerve supplying serratus anterior muscle. Traumatic injury to serratus anterior muscle itself is very rare. We reported a case of traumatic winging of scapula due to tear of serratus anterior muscle in a 19-year-old male. Winging was present in neutral position and in extension of right shoulder joint but not on "push on wall" test. Patient was managed conservatively and achieved satisfactory result. Key words: Serratus anterior tear; Scapula; Wounds and injuries

  1. 子宫原始神经外胚层瘤2例并文献复习%2 Cases of Primitive Neuroectodermal Tumors of the Uterine and Literature Review

    Institute of Scientific and Technical Information of China (English)

    刘超霞; 张克强; 桂玲; 冉晓敏; 周萍; 肖凌志

    2014-01-01

    目的:探讨子宫原始神经外胚层瘤的临床表现、病理特点、治疗方法及预后。方法对2例子宫原始神经外胚叶肿瘤患者的临床资料进行回顾性分析并复习相关文献进行总结。结果2例患者的年龄分别为17岁和44岁,均经病理诊断确诊,Homer-Wright 菊形团为其特异性病理形态学表现,CD99表达均呈阳性。1例患者为IV 期,接受了肿瘤细胞减灭术,首次治疗结束后4个月复发,再次术后仍未获得满意生存,仅存活20个月;另1例仅行肿瘤切除术,术后给予化疗4疗程,随访至今未提示复发及转移。结论子宫原始神经外胚叶肿瘤主要依赖于病理及免疫组化结果明确诊断;手术为其主要治疗手段,术后可辅以放化疗,其预后与分期有关。%Objective To investigate the clinical and pathological characteristics, treatment, and prognosis of Peripheral Primitive Neuroectodermal Tumors of the uterus. Methods 2 cases of primary Primitive Neuroectodermal Tumor of uterus were included in Hunan Provincial Tumor Hospital. A retrospective analysis was carried out to analyze the clinical characteristics, clinicopathological features, treatment and prognosis of these patients. Results 2 patients aged 17 and 44 years old respectively. The final diagnosis was established by the pathological diagnosis. Homer-Wright daisy-group was a specific pathologic feature of pPNET. Positive expression of the CD99 was observed in all cases.One case who was FIGO stages IVB received cytoreduc-tive surgery and chemotherapy, But recurred after only 4 months. and died 20 months . another patient who received tumorec-tomy was survived in 5 months of follow-up. Conclusion Peripheral primitive neuroectodermal tumor (PNET) of Uterus was a very rare disease. CD99 and Homer-Wright daisy-group were both specific markers for PNET. The therapy included surgery, chemotherapy and radiotherapy. The prognosis of the primitive

  2. Síndrome tóxica do segmento anterior Toxic anterior segment syndrome

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    Luiz Filipe de Albuquerque Alves

    2013-02-01

    Full Text Available A síndrome tóxica do segmento anterior (STSA é uma severa reação inflamatória aguda causada por agente não infeccioso que entra no segmento anterior, resultando em lesão celular tóxica com necrose e apoptose mediado por resposta imunológica. Neste relato de caso de STSA são enfatizadas as causas mais comuns para o aparecimento da síndrome, apontam para os cuidados que devem ser tomados no processo de esterilização do material cirúrgico além de revisar a melhor conduta diante desses casos. Em conclusão notou-se que o foco principal deve ser a prevenção, pois o tratamento busca apenas suprimir a resposta inflamatória secundária. O tratamento nos casos de STSA consiste em intensa instilação de esteróides tópicos com seguimento rigoroso e controle de complicações tardias como o glaucoma.Toxic anterior segment syndrome is acute inflammatory reaction caused by a noninfectious substance that enters the anterior segment, resulting in extracellular damage with necrosis and apoptosis during an immune response. We have the report of a case of toxic anterior segment syndrome (TASS, in which the authors seek to emphasize the most common causes of the appearance of these syndrome. They point out the care that must be taken in the process of sterilization of surgical material, in addition to reviewing the best conduct when faced with these cases. In conclusion, it was noted that the main focus should be on prevention, as treatment only seeks to suppress the secondary inflammatory response. Treatment in cases of toxic anterior segment syndrome (TASS consists of intense instillation of topical steroids with strict follow-up and control of late complications such as glaucoma.

  3. RETAINED STONE PIECE IN ANTERIOR CHAMBER

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    ZvornicaninJasmin, Nadarevic-VodencarevicAmra

    2015-04-01

    Full Text Available ABSTRACT We read with interest the article by Surekha et al. regarding the retained stone piece in anterior chamber. Similar to the results of previous studies, the authors found that delayed intraocular foreign body (IOFB management can result in good visual outcome without an apparent increased risk of endophthalmitis or other deleterious side effects. However, the authors failed to explain the exact reason for the diminution of vision in patients left eye. It is unclear what the uncorrected visual acuity was and what kind of correction was used, more precisely type and amount of cylinder, given the presence of the corneal opacity. Since the size of the IOFB is approximately 4x4x1mm, significant irido-corneal angle changes resulting in intraocular pressure raise and optic nerve head damage can be expected. Traumatic glaucoma following open globe injury can occur in 2.7 to 19% of cases, with several risk factors associated with glaucoma development (advanced age, poor visual acuity at presentation,perforating rather than penetrating ocular injury,lens injury, presence of vitreous hemorrhage and presence of an IOFB. Earlier reportsof latetraumaticoptic neuropathy onset, even after several years, indicate that this possibility cannot be completely ruled out too. Therefore, repeated intraocular pressure measurements, gonioscopy, pupillary reaction assessment, together with through posterior segment examination including visual field and optical coherence tomography examinations can be useful in determining the possible optic nerve damage as one of the possible reasons for visual acuity reduction. The authors did not suggest any operative treatment at this time. However, it should bear in mind that the inert anterior chamber IOFB could be a risk factor for non-infectious endophthalmitis development even after many years. Also, long term retained anterior chamber foreign body leads to permanent endothelial cell loss and can even result in a corneal

  4. Neurologic complication after anterior sciatic nerve block.

    Science.gov (United States)

    Shah, Shruti; Hadzic, Admir; Vloka, Jerry D; Cafferty, Maureen S; Moucha, Calin S; Santos, Alan C

    2005-05-01

    The lack of reported complications related to lower extremity peripheral nerve blocks (PNBs) may be related to the relatively infrequent application of these techniques and to the fact that most such events go unpublished. Our current understanding of the factors that lead to neurologic complications after PNBs is limited. This is partly the result of our inability to conduct meaningful retrospective studies because of a lack of standard and objective monitoring and documentation procedures for PNBs. We report a case of permanent injury to the sciatic nerve after sciatic nerve block through the anterior approach and discuss mechanisms that may have led to the injury. Intraneural injection and nerve injury can occur in the absence of pain on injection and it may be heralded by high injection pressure (resistance).

  5. Anterior uveitis and diabetes mellitus: immunological study.

    Science.gov (United States)

    Castagna, I; Famà, F; Salmeri, G

    1995-01-01

    The association between anterior uveitis (AU) and diabetes mellitus (DM) has always been known. The purpose of this study was to estimate the incidence of this association and to consider a possible role of the cell-mediated immune system. During the years 1989-1992, 196 diabetics (66 patients affected by type I DM and 130 by type II DM) were studied. The study of the lymphocytic subsets and the measurement of the circulating immunocomplexes and autoantibodies (ANA, AMA, ADNA, ASMA, APCA) were carried out, and the results were compared with those of a control group. The results underline the correlation between AU and type I DM. Many immunological reactions could play a crucial role: the high levels of CD8 subsets found could be an expression of the unstable lymphocytic equilibrium.

  6. Tumores neuroectodérmicos primitivos periféricos de localización en el área orocervical: presentación de dos casos clínicos Peripheral primitive neuroectodermal tumors located in orocervical area: presentation of two clinical cases

    Directory of Open Access Journals (Sweden)

    C. Sánchez Acedo

    2010-03-01

    Full Text Available Introducción: Los tumores neuroectodérmicos primitivos (PNET, de primitive neuroectodermal tumors son una familia de neoplasias malignas de células pequeñas y redondas, que derivan de la cresta neural. Se distinguen tres tipos: PNET del sistema nervioso central, PNET del sistema nervioso autónomo y PNET periféricos. Los más frecuentes dentro del grupo de PNET periféricos son el neuroepitelioma periférico y el sarcoma de Ewing, que se consideran la misma neoplasia pero con diferente grado de diferenciación. Casos clínicos: Presentamos dos casos de PNET periféricos, uno de aparición en la región cervical y otro originado en el cóndilo mandibular. Discusión: Los PNET son neoplasias muy raras y altamente agresivas. En todos ellos aparecen células redondas pequeñas poco diferenciadas y una traslocación cromosómica característica del gen EWS. En general se considera que tienen un pronóstico desfavorable. Además, la baja frecuencia de estos tumores, así como la escasez de casos publicados hacen difícil valorar el tratamiento más adecuado.Introduction: Peripheral primitive neuroectodermal tumors (PNET are a family of smallround cell tumors of presumed neuroectodermal origin. This broad family can be subdivided into three major groups: PNET from the central nervous system, PNET from the autonomic nervous system or peripheral PNET. Ewing´s sarcoma and peripheral neuroepitelioma, the two most frequently encountered members of the peripheral PNET family, are considered to represent a spectrum according to the extent of neuroectodermal differentiation, ranging from the least differentiated (Ewing´s sarcoma to the most differentiated (peripheral neuroepithelioma. Case report: We present a patient with a peripheral neuroectodermal tumor located in the neck and another one with a peripheral neuroectodermal tumor of the mandibular condyle. Discussion: Peripheral neuroectodermal tumors are a very rare and aggressive tumors. They

  7. Endoscopic anterior decompression in cervical disc disease

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    Yad Ram Yadav

    2014-01-01

    Full Text Available Background: Although microscopic anterior cervical discectomy with or without fusion are common surgical procedures for treatment of cervical herniated discs, loss of disc height, pseudarthrosis, and adjacent disc degeneration are some of the problems associated with it. This study is aimed to evaluate results of endoscopic microforaminotomy in cervical disc diseases. Materials and Methods: A prospective study of 50 patients of mono segmental soft or hard disc causing myeloradiculopathy was undertaken. A visual analogue scale (VAS for neck and arm pain and functional outcomes using the Nurick grading system were assessed. There were 28, 12, 8, and 2 patients at C5-6, C6-7, C4-5, and C3-4 levels disc diseases, respectively. Patients with two or more level disc, instabilities, disc extending more than half vertebral body height, and previous operation at the same segment were excluded. Results: Age ranged from 21 to 67 years. Average postoperative reduction in disc height, operating time, and blood loss was 1.1 mm, 110 minutes, and 30 ml, respectively. Average pre-operative VAS score for arm pain and Nurick grading was 7.6 and 2.7, which improved to 1.9 and 0.82, respectively. All patients improved; 1, 2, 3 grade improvement was seen in 10, 27, and 10 patients, respectively. There was no significant complication or any mortality. Conclusion: Although longer follow up of large number of patients is required, endoscopic microforaminotomy is a safe and an effective alternative to microscopic anterior discectomy with or without fusion.

  8. Clinical Outcomes Following Revision Anterior Shoulder Stabilization

    Science.gov (United States)

    Frank, Rachel M.; Mellano, Chris; Shin, Jason J.; Feldheim, Terrence F.; Mascarenhas, Randhir; Yanke, Adam Blair; Cole, Brian J.; Nicholson, Gregory P.; Romeo, Anthony A.; Verma, Nikhil N.

    2015-01-01

    Objectives: The purpose of this study was to determine the clinical outcomes following revision anterior shoulder stabilization performed either via all-arthroscopic soft tissue repair or via Latarjet coracoid transfer. Methods: A retrospective review of prospectively collected data on 91 shoulders undergoing revision anterior shoulder stabilization was performed. All patients underwent prior soft tissue stabilization; those with prior open bone grafting procedures were excluded. For patients with 25% glenoid bone loss, Latarjet was performed (n=28). Patients were queried regarding recurrent instability (subluxation or dislocation). Clinical outcomes were evaluated using validated patient reported outcome questionnaires including the American Shoulder and Elbow Surgeons (ASES) score, Simple Shoulder Test (SST), visual analog scale (VAS) for pain, and Western Ontario Shoulder Instability Index (WOSI). Results: A total of 63 shoulders in 62 patients (46 males, 16 females) with an average age of 23.2 ± 6.9 years were included in the revision arthroscopy group. At an average follow-up of 46.9 ± 16.8 months (range, 15 to 78), the mean WOSI score was 80.1 (range, 15.0 to 100), and there were significant improvements (pLatarjet group. Thirteen (46%) had more than one previous stabilization attempt. ), the average WOSI score was 71.9, and there were significant improvements (pLatarjet. Conclusion: Both arthroscopic revision stabilization and Latarjet coracoid transfer result in satisfactory outcomes in patients who have failed previous arthroscopic capsulolabral repair. Recurrent instability rates were higher in the all-arthroscopic group (19% versus 7%). Longer-term studies are required to determine whether similar results are maintained over time, and to provide guidance on focused clinical indications for this challenging patient population.

  9. Etiological aspects of anterior open bite

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    Stojanović Ljiljana

    2007-01-01

    Full Text Available Introduction. Open bite is a multifactorial phenomenon and no single factor can account for open-bite. Etiology plays an important role in diagnosis. Heredity, unfavorable growth patterns, incorrect jaw postoure, are the characteristics of skeletal open bite. Digit sucking. Depending on where the thumb is placed, a number of different types of dental problems can develop. Malocclusions of the late mixed or permanent dentitions, caused by thumb sucking are not self corrected and orthodontic treatment is necessary for their correction. Lymphatic tissue. In order to produce oral respiration, the mandible is postured inferiorly with the tongue protruded and resting against the oral floor. This postural alteration induces dental and skeletal modifications similar to those caused by thumb sucking. This may cause excessive eruption of the posterior teeth, leading to an increase in the vertical dimension of the face and result in development of anterior open bite. Tongue thrust. Tongue habits cause an anterior open bite or they develop secondarily to thumb sucking. In skeletal open bite the tongue habit acts as a secondary factor which helps to maintain or exacerbate the condition. Many orthodontists have had a discouraging experience of completing dental treatment, with what appeared to be good results, only to discover that the case had relapsed because the patient had a tongue thrust swallowing pattern. Conclusion. Dentoalveolar or habitual open bite is caused by habits, which influence the growth and development of dentoalveolar processes and contribute to occlusal disharmonies. Prior to eruption of adult dentition, open bite related to oral habits is usually not a concern as when the habits stop, because the erupting dentition tends to improve spontaneously. Treatment is usually not necessary until permanent teeth erupt (~6 years old. .

  10. Combined operative technique with anterior surgical approach and video-assisted thoracoscopic surgical lobectomy for anterior superior sulcus tumours.

    Science.gov (United States)

    Yokoyama, Yuhei; Chen, Fengshi; Aoyama, Akihiro; Sato, Toshihiko; Date, Hiroshi

    2014-11-01

    Video-assisted thoracoscopic surgery (VATS) has been widely used, but surgical resections of superior sulcus tumours remain challenging because of their anatomical location. For such cases, less-invasive procedures, such as the anterior transcervical-thoracic and transmanubrial approaches, have been widely performed because of their excellent visualization of the subclavian vessels. Recently, a combined operative technique with an anterior surgical approach and VATS for anterior superior sulcus tumours has been introduced. Herein, we report three cases of anterior superior sulcus tumours successfully resected by surgical approaches combined with a VATS-based lobectomy. In all cases, operability was confirmed by VATS, and upper lobectomies with hilar and mediastinal lymph node dissections were performed. Subsequently, dissections of the anterior inlet of the tumours were performed using the transmanubrial approach in two patients and the anterior trans-cervical-thoracic approach in one patient. Both approaches provided excellent access to the anterior inlet of the tumour and exposure of the subclavian vessels, resulting in radical resection of the tumour with concomitant resection of the surrounding anatomical structures, including the chest wall and vessels. In conclusion, VATS lobectomy combined with the anterior surgical approach might be an excellent procedure for the resection of anterior superior sulcus tumours.

  11. Treatment of Children With Central Nervous System Primitive Neuroectodermal Tumors/Pinealoblastomas in the Prospective Multicentric Trial HIT 2000 Using Hyperfractionated Radiation Therapy Followed by Maintenance Chemotherapy

    Energy Technology Data Exchange (ETDEWEB)

    Gerber, Nicolas U., E-mail: nicolas.gerber@kispi.uzh.ch [Department of Pediatric Oncology, University Children' s Hospital, Zurich (Switzerland); Hoff, Katja von; Resch, Anika [Department of Pediatric Hematology and Oncology, University Medical Center Hamburg-Eppendorf, Hamburg (Germany); Ottensmeier, Holger [Department of Pediatric Oncology, University of Wuerzburg, Wuerzburg (Germany); Kwiecien, Robert; Faldum, Andreas [Institute of Biostatistics and Clinical Research, University of Muenster (Germany); Matuschek, Christiane [Department of Radiation Oncology, Medical Faculty, Heinrich Heine University of Duesseldorf, Duesseldorf (Germany); Hornung, Dagmar [Department of Radiotherapy and Radio-Oncology, University Medical Center Hamburg-Eppendorf, Hamburg (Germany); Bremer, Michael [Institute for Radiation Therapy and Special Oncology, Hannover Medical School, Hannover (Germany); Benesch, Martin [Division of Pediatric Hematology and Oncology, Department of Pediatrics and Adolescent Medicine, Medical University of Graz, Graz (Austria); Pietsch, Torsten [Department of Neuropathology, University of Bonn, Bonn (Germany); Warmuth-Metz, Monika [Department of Neuroradiology, University of Wuerzburg, Wuerzburg (Germany); Kuehl, Joachim [Department of Pediatric Oncology, University of Wuerzburg, Wuerzburg (Germany); Rutkowski, Stefan [Department of Pediatric Hematology and Oncology, University Medical Center Hamburg-Eppendorf, Hamburg (Germany); Kortmann, Rolf D. [Department of Radiation Oncology, University of Leipzig, Leipzig (Germany)

    2014-07-15

    Purpose: The prognosis for children with central nervous system primitive neuroectodermal tumor (CNS-PNET) or pinealoblastoma is still unsatisfactory. Here we report the results of patients between 4 and 21 years of age with nonmetastatic CNS-PNET or pinealoblastoma diagnosed from January 2001 to December 2005 and treated in the prospective GPOH-trial P-HIT 2000-AB4. Methods and Materials: After surgery, children received hyperfractionated radiation therapy (36 Gy to the craniospinal axis, 68 Gy to the tumor region, and 72 Gy to any residual tumor, fractionated at 2 × 1 Gy per day 5 days per week) accompanied by weekly intravenous administration of vincristine and followed by 8 cycles of maintenance chemotherapy (lomustine, cisplatin, and vincristine). Results: Twenty-six patients (15 with CNS-PNET; 11 with pinealoblastoma) were included. Median age at diagnosis was 11.5 years old (range, 4.0-20.7 years). Gross total tumor resection was achieved in 6 and partial resection in 16 patients (indistinct, 4 patients). Median follow-up of the 15 surviving patients was 7.0 years (range, 5.2-10.0 years). The combined response rate to postoperative therapy was 17 of 20 (85%). Eleven of 26 patients (42%; 7 of 15 with CNS-PNET; 4 of 11 with pinealoblastoma) showed tumor progression or relapse at a median time of 1.3 years (range, 0.5-1.9 years). Five-year progression-free and overall survival rates (±standard error [SE]) were each 58% (±10%) for the entire cohort: CNS-PNET was 53% (±13); pinealoblastoma was 64% (±15%; P=.524 and P=.627, respectively). Conclusions: Postoperative hyperfractionated radiation therapy with local dose escalation followed by maintenance chemotherapy was feasible without major acute toxicity. Survival rates are comparable to those of a few other recent studies but superior to those of most other series, including the previous trial, HIT 1991.

  12. Tireoide ectópica no mediastino anterior Ectopic thyroid in the anterior mediastinum

    Directory of Open Access Journals (Sweden)

    Maria José Araújo da Cunha Guimarães

    2009-04-01

    Full Text Available A ectopia de tireoide é rara, e a sua localização no mediastino anterior é excepcional, estando descritos apenas 5 casos nos últimos 30 anos. Os autores apresentam 2 casos clínicos, além de uma revisão da literatura abordando a etiologia, a embriologia e manifestações clínicas de ectopia de tireoide.Ectopic thyroid is a rare condition, and its location in the anterior mediastinum is even rarer, there having been only 5 reported cases in the past 30 years. Here, we describe 2 clinical cases and present a review of the literature regarding the etiology, embryology and clinical manifestations of ectopic thyroid.

  13. Experimental Study on the Prevention of Anterior Segment Ischemia by Preservation of Anterior Ciliary Vessels

    Institute of Scientific and Technical Information of China (English)

    Yanna Li; Guanghuan Mai; Zhijian Wang; Xinping Yu; Huanyun Yu; Yan Guo; Xiaoming Lin; Daming Deng; Ying Kang

    2003-01-01

    Purpose: To observe the effect of preserving anterior ciliary vessels (ACVs) on anteriorsegments of rabbit eyes undergoing tenotomy of extraocular muscles.Methods: Thirty-two adult New Zealand white rabbits were divided into four groups.Same procedures were done in both eyes in each group except that left eyes underwentpreservation of ACVs. In the first group medial and lateral recti, in the second group,superior and inferior recti, in the third group, medial, lateral and superior or inferior rectiand in the fourth group, all four recti, underwent tenotomy. Slit-lamp examination,intraocular pressure (IOP) measurement, total protein and lactic acid quantification inaqueous humor were done in all eyes pre- and post-operatively. By four weeks afteroperation, the eyes were enucleated for histological examination and electron microscopy.All data were analyzed using SPSS version 10.Results: In the left eyes of both group 1 and group 2, no inflammatory response wasobserved. In the left eyes of group 3 and 4, we observed mild inflammatory response withslit-lamp examination, which disappeared in one wk. However, we did not findsignificant changes in IOP, total protein and lactic acid of aqueous humor, histology andelectron microscopic examination in these groups. In the right eyes in group 2, 3 and 4,we observed moderate to severe inflammatory changes, a few even developed anteriorsegment ischemia, appeared as decreased IOP, increased total protein and lactic acid inaqueous humor, along with pathological and electron-microscopic changes.Conclusion: Simultaneous tenotomy of three or four recti or two vertical recti on one eyemay decrease anterior segment blood flow even lead to ischemia. ACVs preservation mayprotect the blood circulation in anterior segment. Our study suggests that ACVspreservation in strabismus surgeries especially those involving multi-recti tenotomies mayprevent potential anterior segment ischemia.

  14. Transphyseal anterior cruciate ligament reconstruction in a skeletally immature knee using anterior tibialis allograft.

    Science.gov (United States)

    Cho, Yool; Jang, Soo-Jin; Son, Jung-Hwan

    2011-05-18

    Anterior cruciate ligament (ACL) injury in the skeletally immature individual is being recognized with increasing frequency. Nonoperative treatment of ACL injuries in skeletally immature patients have not been favorable. Surgical treatment options for complete ACL tears include primary ligament repair, extraarticular tenodesis, transphyseal reconstruction, partial transphyseal reconstruction, and physeal-sparing reconstruction. The advantage of transphyseal reconstruction is placement of the graft tissue in an isometric position, which provides better results, according to the literature. The potential disadvantage is angular or limb-length discrepancy caused by physeal violation. Controversy exists in allograft selection about whether bone or soft tissue passes into physes. The use of standard tunnels provides reliable results, but carries the risk of iatrogenic growth disturbance from physeal injury.This article presents 4 cases of transphyseal ACL reconstruction using anterior tibialis allograft in skeletally immature patients that had satisfactory functional outcomes with no growth disturbances. This is the first report of transphyseal ACL reconstruction using anterior tibialis allograft in skeletally immature patients in the English-speaking literature. All patients underwent transphyseal ACL reconstruction using anterior tibialis tendon allograft. None of the patients had angular deformities. No early physeal arrest was measured between the preoperative and postoperative radiographs. At last follow-up, the results of the Lachman test were normal for 3 patients and nearly normal for 1 patient. All patients demonstrated full range of knee motion (comparing the reconstructed knee to the contralateral knee). The results of the pivot-shift test were normal for 3 patients and nearly normal for 1 patient. No patients reported giving way.

  15. Temporal and spatial requirements for Nodal-induced anterior mesendoderm and mesoderm in anterior neurulation.

    Science.gov (United States)

    Gonsar, Ngawang; Coughlin, Alicia; Clay-Wright, Jessica A; Borg, Bethanie R; Kindt, Lexy M; Liang, Jennifer O

    2016-01-01

    Zebrafish with defective Nodal signaling have a phenotype analogous to the fatal human birth defect anencephaly, which is caused by an open anterior neural tube. Previous work in our laboratory found that anterior open neural tube phenotypes in Nodal signaling mutants were caused by lack of mesendodermal/mesodermal tissues. Defects in these mutants are already apparent at neural plate stage, before the neuroepithelium starts to fold into a tube. Consistent with this, we found that the requirement for Nodal signaling maps to mid-late blastula stages. This timing correlates with the timing of prechordal plate mesendoderm and anterior mesoderm induction, suggesting these tissues act to promote neurulation. To further identify tissues important for neurulation, we took advantage of the variable phenotypes in Nodal signaling-deficient sqt mutant and Lefty1-overexpressing embryos. Statistical analysis indicated a strong, positive correlation between a closed neural tube and presence of several mesendoderm/mesoderm-derived tissues (hatching glands, cephalic paraxial mesoderm, notochord, and head muscles). However, the neural tube was closed in a subset of embryos that lacked any one of these tissues. This suggests that several types of Nodal-induced mesendodermal/mesodermal precursors are competent to promote neurulation.

  16. Review for the generalist: evaluation of anterior knee pain

    Directory of Open Access Journals (Sweden)

    Houghton Kristin M

    2007-05-01

    Full Text Available Abstract Anterior knee pain is common in children and adolescents. Evaluation and management is challenging and requires a thorough history and physical exam, and understanding of the pediatric skeleton. This article will review common causes of chronic anterior knee pain in the pediatric population with a focus on patellofemoral pain.

  17. [Key points in anterior esthetic restorations with all ceramic].

    Science.gov (United States)

    Luo, Xiaoping; Qian, Dongdong; Yuan, Yu; Meng, Xiangfeng

    2013-04-01

    This paper introduced the key points in fabricating anterior esthetic restorations with all ceramic materials, including pre-operative smile design, standard tooth preparation, provisional restoration fabrication, all ceramic materials selection, all ceramic restoration bonding, ceramic crack and fracture prevention. And then, the authors summarized and reviewed the clinical common problems in anterior esthetic restorations.

  18. Modified Anchor Shaped Post Core Design for Primary Anterior Teeth

    OpenAIRE

    R. Rajesh; Kusai Baroudi; K. Bala Kasi Reddy; Praveen, B. H.; V. Sumanth Kumar; Amit, S

    2014-01-01

    Restoring severely damaged primary anterior teeth is challenging to pedodontist. Many materials are tried as a post core but each one of them has its own drawbacks. This a case report describing a technique to restore severely damaged primary anterior teeth with a modified anchor shaped post. This technique is not only simple and inexpensive but also produces better retention.

  19. Modified Anchor Shaped Post Core Design for Primary Anterior Teeth

    Directory of Open Access Journals (Sweden)

    R. Rajesh

    2014-01-01

    Full Text Available Restoring severely damaged primary anterior teeth is challenging to pedodontist. Many materials are tried as a post core but each one of them has its own drawbacks. This a case report describing a technique to restore severely damaged primary anterior teeth with a modified anchor shaped post. This technique is not only simple and inexpensive but also produces better retention.

  20. Modified anchor shaped post core design for primary anterior teeth.

    Science.gov (United States)

    Rajesh, R; Baroudi, Kusai; Reddy, K Bala Kasi; Praveen, B H; Kumar, V Sumanth; Amit, S

    2014-01-01

    Restoring severely damaged primary anterior teeth is challenging to pedodontist. Many materials are tried as a post core but each one of them has its own drawbacks. This a case report describing a technique to restore severely damaged primary anterior teeth with a modified anchor shaped post. This technique is not only simple and inexpensive but also produces better retention.

  1. [Bilateral anterior uveiopapillitis, suspicious of Lyme disease--case report].

    Science.gov (United States)

    Nicula, Cristina; Nicula, D; Rusu, Ioana; Popescu, Raluca

    2013-01-01

    We present the case of a patient which associated bilateral anterior uveitis manifestations with those of bilateral anterior inflammatory optic neuropathy. We followed the evolution of the case under treatment and we discussed the differential diagnosis and the association of the two ocular pathologies.

  2. Anterior urethral recurrence of superficial bladder cancer: its clinical significance.

    Directory of Open Access Journals (Sweden)

    Saika T

    2003-12-01

    Full Text Available The aim of this study was to reveal the clinical features of anterior urethral recurrence in patients with superficial bladder cancer, and to determine the appropriate treatment. Three hundred and three patients with superficial bladder cancer, who were newly diagnosed and initially treated conservatively in our hospital between 1965 and 1990, were followed for at least 5 years and their clinical outcomes were analyzed. Clinical factors, including anterior urethral recurrence, were evaluated statistically regarding tumor progression. Eight patients (2.6% had anterior urethral recurrence following superficial bladder cancer. Twenty-four patients (7.9% had tumor progression and 149 (49.2% had tumor recurrence. In a multivariate analysis using a logistic model, anterior urethral recurrence was the most important factor, followed by histological grade. Four of 5 patients who were treated for anterior urethral recurrent tumors by transurethral resection showed progression and died of the cancer within one year. Two of the remaining three patients who underwent radical cysto-urethrectomy at the time of anterior urethral recurrence survived. Anterior urethral recurrence following superficial bladder cancer is a predictor for rapid subsequent malignant progression. Once there is anterior urethral recurrence, radical intensive therapy, including radical cysto-urethrectomy, should be carried out immediately.

  3. Anterior commissure absence without callosal agenesis: a new brain malformation.

    Science.gov (United States)

    Mitchell, T N; Stevens, J M; Free, S L; Sander, J W; Shorvon, S D; Sisodiya, S M

    2002-04-23

    The authors report a novel human brain malformation characterized by the absence of the anterior commissure without callosal agenesis, but associated with gross unilateral panhemispheric malformation incorporating subependymal heterotopia, subcortical heterotopia, and gyral abnormalities including temporal malformation and polymicrogyria. In contrast, a normal anterior commissure was found in 125 control subjects and in 113 other subjects with a range of brain malformations.

  4. Agenesis of internal carotid artery associated with congenital anterior hypopituitarism

    Energy Technology Data Exchange (ETDEWEB)

    Moon, W.-J. [Department of Diagnostic Radiology, Samsung Medical Center, Seoul (Korea); Institute of Neuroradiology, University of Frankfurt (Germany); Porto, L.; Lanfermann, H.; Zanella, F.E. [Institute of Neuroradiology, University of Frankfurt (Germany); Weis, R. [Department of Pediatric Neurology, University of Frankfurt (Germany)

    2002-02-01

    We report a rare case of unilateral agenesis of the internal carotid artery in association with congenital anterior hypopituitarism. The collateral circulation is supplied by a transsellar intercavernous anastomotic vessel connecting the internal carotid arteries. These abnormalities are well depicted on MRI and MRA. The agenesis of the internal carotid artery may explain the pathogenesis of some of congenital anterior hypopituitarism. (orig.)

  5. Reconstruction of the Anterior Cruciate Ligament : Alternative Strategies

    NARCIS (Netherlands)

    van Eijk, F.

    2009-01-01

    This thesis describes the long-term results of reconstruction of the anterior cruciate ligament with an allograft. Due to the poor results found, further studies were performed to investigate alternative strategies for reconstruction of the anterior cruciate ligament in the field of tissue engineeri

  6. Bilateral agenesis of the anterior cruciate ligament: MRI evaluation

    Energy Technology Data Exchange (ETDEWEB)

    Bedoya, Maria A.; Jaramillo, Diego [The Children' s Hospital of Philadelphia, Radiology Department, Philadelphia, PA (United States); McGraw, Michael H. [Hospitalof theUniversityof Pennsylvania, Divisionof Orthopaedics, Philadelphia, PA (United States); Wells, Lawrence [The Children' s Hospital of Philadelphia, Division of Orthopaedics, Philadelphia, PA (United States)

    2014-09-15

    Bilateral agenesis of the anterior cruciate ligament (ACL) is extremely rare. We describe a 13-year-old girl who presented with bilateral knee pain without history of trauma; she has two family members with knee instability. Magnetic resonance imaging showed bilateral absence of the ACL, and medial posterior horn meniscal tears. Bilateral arthroscopic partial meniscectomy and anterior cruciate ligament reconstruction was performed. (orig.)

  7. Anterior endoderm and head induction in early vertebrate embryos.

    Science.gov (United States)

    de Souza, F S; Niehrs, C

    2000-05-01

    Early work on the formation of the vertebrate body axis indicated the existence of separate head- and trunk-inducing regions in Spemann's organizer of the amphibian gastrula. In mammals some head-organizing activity may be located in anterior visceral (extraembryonic) endoderm (AVE). By analogy, the equivalent structure in the Xenopus laevis gastrula, the anterior endoderm, has been proposed to be the amphibian head organizer. Here we review recent data that challenge this notion and indicate that the involvement of AVE in head induction seems to be an exclusively mammalian characteristic. In X. laevis and chick, it is the prechordal endomesoderm that is the dominant source of head-inducing signals during early gastrulation. Furthermore, head induction in mammals needs a combination of signals from anterior primitive endoderm, prechordal plate, and anterior ectoderm. Thus, despite the homology of vertebrate anterior primitive endoderm, a role in head induction seems not to be conserved.

  8. Variant attachments of the anterior horn of the medial meniscus.

    Science.gov (United States)

    Jakubowicz, Marian; Ratajczak, Wojciech; Pytel, Andrzej

    2003-01-01

    The purpose of this study was to analyse the occurrence of variants of anomalous insertions of the anterior horn of the medial meniscus in human knee joints. The study was carried out on 78 human lower limbs of both sexes (42 males and 36 females). Out of 78 knee joints, 10 knee joints (12.82%) presented atypical attachments of the anterior horn of the medial meniscus. In 9 cases we found that the anterior horn of the medial meniscus was attached to the transverse ligament of the knee and in 1 case it was attached to the coronary ligament. In the remaining cases the anterior horn of the medial meniscus was attached to the anterior intercondylar area of the tibia.

  9. Method for measuring anterior chamber volume by image analysis

    Science.gov (United States)

    Zhai, Gaoshou; Zhang, Junhong; Wang, Ruichang; Wang, Bingsong; Wang, Ningli

    2007-12-01

    Anterior chamber volume (ACV) is very important for an oculist to make rational pathological diagnosis as to patients who have some optic diseases such as glaucoma and etc., yet it is always difficult to be measured accurately. In this paper, a method is devised to measure anterior chamber volumes based on JPEG-formatted image files that have been transformed from medical images using the anterior-chamber optical coherence tomographer (AC-OCT) and corresponding image-processing software. The corresponding algorithms for image analysis and ACV calculation are implemented in VC++ and a series of anterior chamber images of typical patients are analyzed, while anterior chamber volumes are calculated and are verified that they are in accord with clinical observation. It shows that the measurement method is effective and feasible and it has potential to improve accuracy of ACV calculation. Meanwhile, some measures should be taken to simplify the handcraft preprocess working as to images.

  10. 外周性原始神经外胚层肿瘤的诊断及治疗%Diagnosis and treatment of peripheral primitive neuroectodermal tumors

    Institute of Scientific and Technical Information of China (English)

    闫坤; 葛燕燕; 李向阳; 严士光; 丁友宏

    2016-01-01

    目的:探讨外周性原始神经外胚层肿瘤(peripheral primitive neuroectodermal tumors,pPNETs)的诊断及治疗。方法:回顾性分析我院收治的5例经病理证实的 pPNETs 患者的诊治过程,并复习文献。结果:本组病例均为软组织,1例位于颈部,1例位于肩部,1例位于腋窝,2例位于胸壁。CT 平扫显示肿物多呈不均匀的等、低密度,所有肿瘤增强后均可见不均匀强化。3例患者行 MRI,显示 T1WI 呈与肌肉类似的混杂信号, T2WI 表现为不均匀的高信号。2例患者行 B 超检查,显示肿物局部低密度影,内可见血流信号,未压迫周围血管。镜下可见肿瘤细胞形成典型的 Homer -Wright 菊形团。免疫组化显示肿瘤均表达 CD99和 VIM,NSE和 Syn 多数阳性,CK 阴性,并且不表达 LCA,部分低表达 S100。结论:pPNETs 是一种少见的小圆细胞恶性肿瘤。CT 及 MRI 检查可评估肿瘤可切除性。B 超可了解肿物的血供及与周围血管的位置关系。pPNETs 的确诊依靠病理及免疫组化,尤其是 Homer -Wright 菊形团及神经内分泌标记物对肿瘤的确诊有重要意义。手术是直接有效的治疗手段,术前新辅助化疗可以减少 pPNETs 局部淋巴结转移。%Objective:To explore the diagnosis and treatment of peripheral primitive neuroectodermal tumors (pPNETs).Methods:Retrospectively analyze the patients'diagnosis and treatment with pPNETs.Results:All cases were soft tissue.1 case located in the neck,1 case in the shoulder,1 case in axillary,2 cases in the chest wall.CT scan showed density was uneven,and all tumors were visible uneven enhancement.3 cases did MRI which showed a mixed signal liked muscle's in T1WI and an uneven high signal in T2WI.2 cases did ultrasound B which showed low density shadow with blood flow signals in part of the mass and not oppression axillary vein.Microscopically,the tumor celles formed typical chrysanthemum form

  11. 原始神经外胚层肿瘤的CT和MRI影像表现%CT and MRI manifestations of primitive neuroectodermal tumors

    Institute of Scientific and Technical Information of China (English)

    刘书涵; 付国丽; 颜岩

    2015-01-01

    Objective To investigate the CT and MRI manifestations of primitive neuroectodermal tumors (PNET) in or‐der to improve understanding of PNET .Methods A retrospective analysis of imaging data of 45 cases of PNET confirmed pathologically ,which included 29 males ,19 females ,with a mean age of 36 .4 years old ,was conducted in this work .Re‐sults PNET can be divided into central (cPNET ) and peripheral (pPNET ) types according to the occurrence at different parts .9 cases of cPNET exhibited the larger supratentorial tumor ,cystic degeneration and necrosis ,none or mild edema a‐round the tumor with clear boundary ,and relative uniform density /signal .CT showed that the density was equal or slightly higher than that of brain gray matter ,MRI T1 WI showed equal or slightly lower signal ,and T2 WI showed a slightly higher or mixed signal ,which may be associated with CSF spread .As for the 36 cases of pPNET ,the clinical manifestations were the obvious soft tissue mass ,with unclear boundary ,the density /signal was not uniform ,a little hemorrhage was visible ,and the calcification was rare .CT showed that the density was equal or slightly lower than that of the surrounding muscle and the MRI signal was complex ,without significant specificity .Tumor involved in the skeletal system can appeare as a large sheet of osteolytic bone destruction ,without periosteal reaction .Conclusion Although the imaging diagnostics of PNET is not taken as a gold standard ,it can determine the extent of lesions ,which is of importance for the preoperative staging ,postoperative evaluation ,and differential diagnosis .%目的:探讨原始神经外胚层肿瘤(primitive neuroectodermal tumor ,PNET)的CT和MRI特征,以提高对该病的影像学认识。方法回顾性分析经病理证实45例PNET 的影像资料,其中男性29例,女性19例,平均年龄36.4岁。结果原始神经外胚层肿瘤按发生部位不同可分为中枢性(cPNET

  12. Diagnosis and treatment of spinal primitive neuroectodermal tumor%脊柱原始神经外胚叶肿瘤的诊断与治疗

    Institute of Scientific and Technical Information of China (English)

    崔益亮; 姜亮; 马庆军; 刘忠军; 刘晓光; 韦峰; 党耕町

    2011-01-01

    目的 总结脊柱原始神经外胚叶肿瘤(primitive neuroectodermal tumor,PNET)的诊断和治疗经验.方法 回顾性分析1999年至2009年收治的PNET患者共13例,男8例,女5例.年龄4~43岁,平均(26.9±11.1)岁.肿瘤累及颈椎6例,腰椎5例,胸椎1例,骶椎1例.9例为手术后依据病理学诊断而确诊,其中6例术前曾CT引导下穿刺诊断;4例未手术患者为CT引导下穿刺诊断.13例患者中10例有椎体骨质破坏或神经功能损害,3例局部疼痛或不适.9例患者选择手术治疗,4例未手术者接受放、化疗.结果 11例患者获得随访,平均随访21.8个月,其中7例手术治疗的患者4例死亡,平均生存时间11.3个月,3例存活,平均已生存36个月;4例未手术患者,3例死亡,平均生存时间7个月,存活1例,已生存5个月.结论 PNET的诊断主要靠检查,重点是免疫组化;CT引导下穿刺活检是术前诊断的可靠手段.脊柱PNET的恶性度高,无论手术与否均有较高死亡率,但手术切除病灶可减轻症状,改善患者生活质量.%Objective To sum up the clinical experience in diagnosis and treatment of spinal primitive neuroectodermal tumor (PNET). Methods Thirteen patients with spinal PNET were included in the study from 1999 to 2009. There were 8 males and 5 females with the mean age of 26.9 years. The lesions involved the cervical spine in 6 cases, the thoracic segment in 1 case, the lumbar segment in 5 cases and the sacrum in 1 case. The diagnosis of PNETs was made in nine patients by postoperative pathological examination. Among them, 6 patients received a preoperative CT-guided percutaneous biopsy. The other four patients were diagnosed only by CT-guided percutaneous biopsy. Osteolytic bony lesions and obvious neurological deficit were found in ten patients, while the other 3 had complained of local pain only. Nine patients had received operation followed by chemotherapy and radiotherapy. The other 4 underwent only chemotherapy and radiotherapy

  13. Anterior commissure versus corpus callosum: A quantitative comparison across mammals.

    Science.gov (United States)

    Ashwell, Ken W S

    2016-04-01

    Mammals rely on two major pathways to transfer information between the two hemispheres of the brain: the anterior commissure and the corpus callosum. Metatheria and monotremes rely exclusively on the anterior commissure for interhemispheric transfer between the isocortices and olfactory allocortices of each side, whereas Eutheria use a combination of the anterior commissure and an additional pathway exclusive to Eutheria, the corpus callosum. Midline cross-sectional area of the anterior commissure and corpus callosum were measured in a range of mammals from all three infraclasses and plotted against brain volume to determine how midline anterior commissure area and its size relative to the corpus callosum vary with brain size and taxon. In Metatheria, the square root of anterior commissure area rises in almost direct proportion with the cube root of brain volume (i.e. the ratio of the two is relatively constant), whereas among Eutheria the ratio of the square root of anterior commissure area to the cube root of brain volume declines slightly with increasing brain size. The total of isocortical and olfactory allocortical commissure area rises more rapidly with increasing brain volume among Eutheria than among Metatheria. This means that the midline isocortical and olfactory allocortical commissural area of metatherians with large brains (about 70 ml) is only about 50% of that among eutherians with similarly sized brains. On the other hand, isocortical and olfactory allocortical commissural area is similar in Metatheria and Eutheria at brain volumes around 1 ml. Among the Eutheria, some groups make less use of the anterior commissure pathway than do others: soricomorphs, rodents and cetaceans have smaller anterior commissures for their brain size than do afrosoricids, erinaceomorphs and proboscideans. The findings suggest that use of the anterior commissural route for isocortical commissural connections may have placed limitations on interhemispheric transfer of

  14. The relationship between the morphological features of A1 segment of anterior cerebral artery and anterior communicating artery aneurysms

    Institute of Scientific and Technical Information of China (English)

    冯文峰

    2013-01-01

    Objective To improve the predictability of surgical clipping and guide the steam shaping of microcatheters in endovascular embolization by analyzing the association of morphological features of A1 segment of anterior cerebral artery(ACA) with formation and classification of anterior

  15. Anterior tension band plating for anterior tibial stress fractures in high-performance female athletes - A report of 4 cases

    NARCIS (Netherlands)

    O. Borens; M.K. Sen; R.C. Huang; J. Richmond; P. Kloen; J.B. Jupiter; D.L. Helfet

    2006-01-01

    Stress fracture of the anterior tibial cortex is an extremely challenging fracture to treat, especially in the high-performance female athlete who requires rapid return to competition. Previous reports have not addressed treating these fractures in the world-class athlete with anterior plating. We h

  16. 宫颈原始神经外胚叶肿瘤临床分析并文献复习%Primitive neuroectodermal tumors of the cervix: report of three cases and review of the literature

    Institute of Scientific and Technical Information of China (English)

    张凯; 雷呈志; 李青; 张洵

    2012-01-01

    Objective:To investigate the clinical and pathological characteristics,treatment, and prognosis of Peripheral Primitive Neuroectodermal Tumors of the cervix. Methods:3 cases of primary Primitive Neuroectodermal Tumor of cervix were included in Cancer Institute & Hospital from 2009 to 2011. We carried out a retrospective analysis on clinical character, clini-copathological features, treatment and prognosis in those patients and a review of relative clinical data. Results;3 patients aged 22~39 years old. The final diagnosis was established by the pathological diagnosis. Positive expression of the CD99 was observed in all cases. 3 patients received multimodality therapy including surgery, chemotherapy, and radiotherapy, one patient survival of 13 months. The other two patients are free of disease until now after surgery. Conclusions : Peripheral primitive neuroectodermal tumor( PNET) of cervix is a very rare disease. Most occur in young women. Fewer had distant metastasis at diagnosis. CD99 is a specific marker for PNET. Multimodality therapy including surgery,chemotherapy,radiotherapy and other treatment may prolong survival.%目的:探讨宫颈原始神经外胚叶肿瘤的临床表现、病理特点、治疗方法及预后.方法:回顾性分析中国医学科学院肿瘤医院2009年至2011年收治的3例宫颈原始神经外胚叶肿瘤患者病例资料并复习文献.结果:3例患者年龄22~ 39岁,均经病理诊断确诊,其中CD99均为强阳性表达.3例患者就诊时肿瘤均无远处转移.均接受了手术治疗和化疗,1例接受了盆腔局部放疗.3例患者中1例术后存活13个月,2例术后无瘤存活至今.结论:宫颈原始神经外胚叶肿瘤临床罕见,多发生于年轻妇女,病理及免疫组化有助于明确诊断,初诊时较少有远处转移,采用手术为主、结合化疗和放疗的综合治疗有望提高预后.

  17. Catarata polar anterior piramidal deslocada para a câmara anterior causando edema de córnea: relato de caso Corneal edema caused by a pyramidal anterior polar cataract dislocated to the anterior chamber: case report

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    Ramon Coral Ghanem

    2004-08-01

    Full Text Available Cataratas polares anteriores piramidais são opacidades cônicas que se projetam para a câmara anterior a partir da cápsula anterior do cristalino. Na grande maioria dos pacientes a opacidade permanece aderida e estável durante toda a vida. O objetivo deste trabalho é documentar uma manifestação incomum desse tipo de catarata: a deiscência espontânea das pirâmides para a câmara anterior causando descompensação endotelial e edema corneal bilateral. Relatamos o caso de uma paciente feminina, de 66 anos, branca, que apresentava edema corneal localizado inferiormente no olho direito associado à lesão nodular branco-esclerótica compatível com a pirâmide anterior da catarata polar. O olho esquerdo apresentava edema corneal difuso intenso e presença de uma catarata polar anterior com a região piramidal deslocada para a câmara anterior. Sabe-se que a pirâmide anterior pode permanecer inabsorvida na câmara anterior por longo período, pois é composta de tecido colágeno denso. Isto causa perda endotelial progressiva e edema corneal e deve ser considerada indicação de remoção cirúrgica da catarata polar anterior e de seu fragmento. Ressalta-se, também, a importância do bom senso no julgamento das cataratas polares anteriores, considerando-se tamanho da opacidade, simetria das opacidades e componente cortical associado, na tentativa de se evitar ambliopia.Pyramidal anterior polar cataracts are conical opacities that project into the anterior chamber from the anterior capsule of the lens. In the vast majority of patients the opacity remains bound and stable throughout life. We report an unusual complication of this type of cataract: spontaneous dehiscence of the pyramids to the anterior chamber causing bilateral endothelial damage and corneal edema. 66-year-old white woman presented with inferior corneal edema in the right eye and diffuse corneal edema in the left eye. A white nodular lesion was observed in the inferior angle

  18. Restoring primary anterior teeth: updated for 2014.

    Science.gov (United States)

    Waggoner, William F

    2015-01-01

    The purpose of this paper was to review the current literature associated with the techniques and materials for the restoration of primary anterior teeth and make clinical recommendations based upon the available literature. A variety of esthetic restorative materials are available to utilize for restoring primary incisors. Awareness of the specific strengths, weakness, and properties of each material can enhance the clinician's ability to make the best choice of selection for each individual situation. Intracoronal restorations of primary teeth may utilize resin composites, glass ionomer cements, resin-modified ionomers, or polyacid-modified resins. Full coronal restoration of primary incisors may be indicated for a number of reasons. Crowns available for restoration of primary incisors include those that are directly bonded onto the tooth, which generally are a resin material, and crowns that are luted onto the tooth and are either some type of stainless steel or zirconia crown. There is insufficient controlled, clinical data to suggest that one type of restoration is superior to another. Operator preferences, esthetic demands by parents, the child's behavior, the amount of tooth structure remaining, and moisture and hemorrhage control are all variables that affect the decision and ultimate outcome of whatever restorative solution is chosen.

  19. Anterior cutaneous nerve entrapment syndrome: management challenges

    Science.gov (United States)

    Chrona, Eleni; Kostopanagiotou, Georgia; Damigos, Dimitrios; Batistaki, Chrysanthi

    2017-01-01

    Anterior cutaneous nerve entrapment syndrome (ACNES) is a commonly underdiagnosed and undertreated chronic state of pain. This syndrome is characterized by the entrapment of the cutaneous branches of the lower thoracoabdominal intercostal nerves at the lateral border of the rectus abdominis muscle, which causes severe, often refractory, chronic pain. This narrative review aims to identify the possible therapeutic strategies for the management of the syndrome. Seventeen studies about ACNES therapy were reviewed; of them, 15 were case–control studies, case series, or case reports, and two were randomized controlled trials. The presently available management strategies for ACNES include trigger point injections (diagnostic and therapeutic), ultrasound-guided blocks, chemical neurolysis, and surgical neurectomy, in combination with systemic medication, as well as some emerging techniques, such as radiofrequency ablation and neuromodulation. An increased awareness of the syndrome and the use of specific diagnostic criteria for its recognition are required to facilitate an early and successful management. This review compiles the proposed management strategies for ACNES. PMID:28144159

  20. [Rehabilitation after arthroscopic anterior cruciate ligament reconstruction].

    Science.gov (United States)

    Smékal, D; Kalina, R; Urban, J

    2006-12-01

    Rehabilitation is an important part of therapy in patients who have had arthroscopic anterior cruciate ligament reconstruction. A well-designed rehabilitation program avoids potential graft damage and speeds up patients' return to their full function level. The course of rehabilitation depends on the type of surgery, mode of fixation and possible co-existing injury to the knee's soft tissues. The rehabilitation program presented here is based on the present-day knowledge of neurophysiological and biomechanical principles and is divided into five phases. In the pre-operative phase (I), the main objective is to prepare patients for surgery in terms of maximum muscle strength and range of motion. It also includes providing full information on the procedure. In the early post-operative phase (II) we are concerned with pain alleviation and reduction of knee edema. After suture removal we begin with soft techniques for the patella and post-operative physical therapy to reduce scarring. In the next post-operative phase (III) patients are able to walk with their full weight on the extremity operated on, and we continue doing exercises that improve flexor/extensor co-contraction. In this phase we also begin with exercises improving the patient's proprioceptive and sensorimotor functions. In the late post-operative phase (IV) we go on with exercises promoting proprioception of both lower extremities with the aim of increasing muscle control of the knee joints. In the convalescent phase (V) patients gradually return to their sports activities.

  1. Anterior Interhemispheric Approach for Olfactory Groove Meningioma

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    Imam Hidayat

    2016-09-01

    Full Text Available Objective: To evaluate the surgical technique with bifrontal interhemispheric approach for total removal of tumor in olfactory groove meningioma (OGM. Methods: This study described a case of a 38-year-old woman with bilateral blindness, anosmia, and behaviour changes. Imaging studies show a tumor mass in midfrontal base. Surgery using a bifrontal interhemispheric approach was performed and total removal was achieved and postoperative computed tomography (CT scan was performed to confirm the result. Histopathological findings established a diagnosis of meningioma. Results: A coronal skin incision behind the hairline was utilized. The scalp was elevated, taking care to reserve the vascularized pericranium medial to the linea temporalis of each side, and preserving the 2 supraorbital nerves. Eight burr holes were used, with the two initial holes made on each side of the orbitotemporal region, and the other four holes at the midline. A bifrontal craniotomy was performed. The tumor was first detached from its attachment with bipolar cautery and debulked. During this step, the main tumor feeder arteries from the anterior and posterior ethmoidal artery were interrupted, and the tumor devascularized. Total tumor removal through surgical intervention was achieved and confirmed by head CT-scan postoperatively. Conclusions: This case report supports the suitability of the bifrontal interhemispheric approach for OGM resection with additional radiation therapy.

  2. Measurement of anterior chamber volume with rotating scheimpflug camera and anterior segment optical coherence tomography

    Institute of Scientific and Technical Information of China (English)

    FU Jing; LI Shu-ning; WANG Xiao-zhen; WU Ge-wei; MU Da-peng; WANG Jian; WANG Ning-li

    2010-01-01

    Background Measurement of anterior segment parameters plays an important role in diagnosis and treatment of glaucoma. The objective of this study was to evaluate the repeatability and reproducibility of anterior chamber volume (ACV) measurements with rotating scheimpflug camera (RSC) and to examine agreement with anterior segment optical coherence tomography (AS-OCT). Methods Thirty nine healthy normal subjects were recruited from the Eye Center of Tongren Hospital. ACV was measured using RSC and AS-OCT in a randomly selected eye for each subject. For RSC measurements, both automatic and manual ACV measurements and 2 independent operators' ACV measurements were obtained. All subjects were invited for 3 visits within a week to evaluate repeatability and reproducibility of ACV measurement by RSC. Agreement was evaluated between RSC and AS-OCT. Results Good repeatability and reproducibility were found for both automatic and manual ACV measurements obtained by RSC. For intrasession repeatability, coefficient of variation (CVw) and intraclass correlation coefficient (ICC) values for automatic were 3.52% and 0.98; the values for manual were 3.44% and 0.97, respectively. For intersession reproducibility, the respective CVw and ICC values were 3.96% and 0.96. Good agreement was also found in 2 operators for both automatic and manual ACV measurements; nevertheless, poor agreement was found between RSC and AS-OCT (95% confidence interval (CI) for agreement of automatic RSC measurement versus AS-OCT were -96.3 to 72.8 μl and 95% CI for agreement of manual RSC measurement versus AS-OCT were between -41.7 to 10.1 μl). Conclusions Both RSC automatic and manual ACV measurements showed good repeatability and reproducibility, and showed comparable agreement between 2 independent operators, but poor agreement was found between RSC and AS-OCT.

  3. Orbicularis oris musculomucosal flap for anterior palatal fistula

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    Tiwari V

    2006-01-01

    Full Text Available Anterior palatal fistulae or residual anterior clefts are a frequent problem following palatoplasty. Various techniques have been used to repair such fistulae, each having its own advantages and disadvantages. We have successfully used orbicularis oris musculomucosal flap to close anterior fistula and residual clefts in 25 patients. This study shows the superiority of this flap over other techniques because of its reliable blood supply, easy elevation and transfer to fistula site and finally because it is a single-stage procedure.

  4. Anterior cruciate ligament remnant and its values for preservation

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    Takeshi Muneta

    2017-01-01

    Full Text Available Controversy surrounds the remnant-preserving anterior cruciate ligament surgery. Advantages of remnant preservation have been reported in regard to better healing and knee function, although no consensus has been reached. This review article discussed the value and meaning of anterior cruciate ligament remnant preservation in several sections such as effects on healing, remnant classification, biomechanical evaluation, relation to proprioception, animal studies, and clinical studies. We hope that this review will facilitate further discussion and investigation for better treatment of anterior cruciate ligament injuries. So far, the current reviews have not provided sufficient scientific evidence to support the value of preserving the remnant.

  5. Management of anterior dental crossbite with removable appliances

    Directory of Open Access Journals (Sweden)

    Ayca Tuba Ulusoy

    2013-01-01

    Full Text Available This case report describes the treatment of an 8-year-old girl with anterior dental crossbite using a series of removable appliances to bring the teeth into a normal position. Clinical presentation and intervention: A removable acrylic appliance with a bite plate incorporating a screw was used to correct the anterior dental crossbite and align the incisors. The subsequent eruption of the maxillary left lateral incisor on the palatinal side was treated with a second acrylic plate incorporating a labiolingual spring. After an 8-month period, the anterior crossbite involving multiple incisors was corrected.

  6. Symptomatic Type IV Dual Left Anterior Descending Coronary Artery

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    Kyriacos Papadopoulos MD

    2016-12-01

    Full Text Available Dual left anterior descending coronary artery is a rare congenital anomaly with 4 subtypes. Double left anterior descending coronary artery originating from the left main stem and the right coronary artery (type IV dual left anterior descending artery has been reported to occur in 0.01% to 0.7% of patients undergoing cardiac catheterization. We report a case of a 49-year-old woman who was found to have this anomaly during coronary angiography. The patient had been complaining of chest pain that mimics angina pectoris and exercise tolerance test was positive for myocardial ischemia.

  7. Multi drug resistant tuberculosis presenting as anterior mediastinal mass

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    Parmarth Chandane

    2016-01-01

    Full Text Available Enlargement of the mediastinal lymphatic glands is a common presentation of intrathoracic tuberculosis (TB in children. However, usually, the mediastinal TB nodes enlarge to 2.8 ± 1.0 cm. In this report, we describe a case of anterior mediastinal lymphnode TB seen as huge mass (7 cm on computed tomography (CT thorax without respiratory or food pipe compromise despite anterior mediastinum being an enclosed space. CT guided biopsy of the mass cultured Mycobacterium TB complex which was resistant to isoniazide, rifampicin, streptomycin ofloxacin, moxifloxacin, and pyrazinamide. Hence, we report primary multi drug resistant TB presenting as anterior mediastinal mass as a rare case report.

  8. Anterior Cruciate Ligament Injuries in Wakeboarding

    Science.gov (United States)

    Starr, Harlan M.; Sanders, Brett

    2012-01-01

    Background: Wakeboarding is an increasingly popular sport that involves aggressive stunts with high risk for lower extremity injury, including anterior cruciate ligament (ACL) rupture. Little has been reported on prevalence or mechanism of ACL injury while wakeboarding. Hypothesis: The prevalence of ACL injury in wakeboarding approaches that of other high-risk sports. Analyzing the mechanism of ACL injury may aid in future efforts of prevention. Study Design: Descriptive epidemiology study. Methods: In sum, 1580 surveys were sent internationally to professional and amateur wakeboarders. The survey questioned the participants on their history of an ACL tear while wakeboarding and asked them to describe the mechanism of injury and treatment. Results: A total of 123 surveys were returned. Of this group, 52 (42.3%) acknowledged having had an ACL tear while wakeboarding. The majority described feeling a pop or buckle after attempting to land a high jump. Only 5 participants (13.5%) described a rotational mechanism created by catching the board edge in the water. Thirty-seven participants (71.15%) said that the injury ruined their ability to wakeboard before reconstruction, and 41 (78.85%) had the injury repaired surgically. Conclusion: The prevalence of ACL tears in this data set, 42.3%, is the highest reported in the literature for wakeboarding and one of the highest for any sport. The main mechanism of injury appears to involve axial compression while one lands in a provocative position; it is not related to a rotational force created by fixed bindings. The injury should be surgically repaired to effectively continue the sport. Further study is needed to determine if wakeboarding represents a high-risk sport for ACL injury. Clinical Significance: Wakeboarding may be a high-risk sport for ACL injury. Noncontact axial compression appears to be the main mechanism of injury. PMID:23016104

  9. 儿童外周型原始神经外胚层肿瘤的CT、MRI表现%Imaging Findings of Pediatric Peripheral Primitive Neuroectodermal Tumor

    Institute of Scientific and Technical Information of China (English)

    唐雯娟; 吴伟军; 张军; 韩燕乔

    2012-01-01

    Objective To analyze CT and MRI findings of pediatric peripheral primitive neuroectodemal tumor(pP-NET) for improving the level of diagnosis. Methods To report clinic, CT, MRI findings and pathologic results of 14 pediatric pPNET. Results Among 14 tumor in 14 children, 3 were located in cervical part, 4 in thoracic part, 3 aside spine, 2 in pelvic cavity. As for density and signal, 3 were heterogeneous or ascoma, 11 were parenchymatous. CT attenuations were lower than muscles and without calcification. There were osseous scleroses or eroded edges in most of the tumors. Periosteal proliferation was found in some cases. The tumor signals were equal or low on T1WI, and heterogeneous or high on T2WI and STIR. After injection of contrast agents, parenchyma enhanced heterogeneous while cystic and necrot-ic tissue did not. Conclusion There are no typical imaging findings on pediatric pPNET. However, it has some imaging characteristics. CT and MRI are helpful in differential diagnosis and making operation protocol.%目的 探讨儿童外周型原始神经外胚层肿瘤(peripheral primitive neuroectodermal tumor,pPNET)的CT、MRI表现,提高对该病的诊断水平.方法 回顾性分析14例经手术病理证实的儿童pPNET的临床、CT、MRI及病理组织学表现,并复习相关文献.结果 14例患儿中,肿瘤分别位于颈部(3例),胸部(4例),脊柱旁(3例),盆腔(2例)及四肢(2例);3例肿瘤均呈不均质囊实性密度或信号,11例为实性密度或信号.CT平扫密度低于肌肉组织,未见明显钙化,周围骨性组织多可见硬化或虫蚀样破坏,部分可见骨膜反应.MRT1WI肿块呈等低信号,T2WI、短恢复时间反转恢复法(STIR)呈不均质混杂高信号,增强扫描明显不均匀强化,中心囊变、坏死区未见明显强化.结论 儿童pPNET缺乏典型的特异性影像学表现,但具有一定的影像学特点.CT和MRI有助于鉴别诊断、制定手术计划.

  10. Crowned odontoid process and osteoarthrosis of the anterior atlantoaxial joint

    Energy Technology Data Exchange (ETDEWEB)

    Skaane, P.; Klott, K.J.

    1981-01-01

    Crowned odontoid process and osteoarthrosis of the anterior atlantoaxial joint. The so-called peridentale aureole ( crowned odontoid ), a horseshoelike calcification around the odontoid process, can occasionally be shown on transbuccal views of the occipito-atlantoaxial region, but is commonly only seen on a.p. tomography in patients with osteoarthrosis of the anterior atlantoxial joint. Tomographic examinations reveal that these irregular horseshoe-like calcifications around the odontoid peg represent mainly the osteophyte formation on the superior border of the anterior arch of the atlas. These calcifications are often surrounding a smaller calcification on the tip of the odontoid peg sometimes with a bucket-handle appearance corresponding to the ostephyte formation on the odontoid process. The peridentale aureole or crowned odontoid process is easily overlooked unless tomography is performed, and misinterpretations are possible if the radiologist is not familiar with this appearance of osteoarthrosis and some other joint diseases of the anterior atlantoaxial joint.

  11. Anterior chest wall involvement in patients with pustulosis palmoplantaris.

    Science.gov (United States)

    Jurik, A G

    1990-01-01

    With the aim of determining the frequency and radiographic features of anterior chest wall involvement in patients with pustulosis palmoplantaris, a questionnaire was sent to 107 patients. Ninety-three patients returned the questionnaire, five of whom were excluded from further analysis due to the appearance of psoriatic lesions. Twenty-five (28%) of the remaining 88 patients reported pain and/or swelling of joints or bones in the anterior chest wall. All were examined radiographically, using tomography, and a group of 20 patients without anterior chest wall complaints were examined similarly. Sixteen of the patients with, but none of the patients without, complaints were found to have arthro-osteitis of the anterior chest wall, consisting of diffuse sclerosis of the manubrium sterni in one patient, localized sclerosis in seven patients, and sequelae of arthritis of the sternoclavicular, upper sternocostal and/or manubriosternal joint in eight patients.

  12. QUADROS CONCEITUAIS DO CETICISMO ANTERIOR A SEXTO EMPÍRICO

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    Rodrigo Pinto de Brito

    2013-08-01

    Full Text Available Artigo em que demonstramos, através de tabelas que servem como ferramentas depesquisa, os principais ganhos conceituais do ceticismo anterior a Sexto Empírico, em suasdiferentes fases.

  13. Esthetic Challenges in Rehabilitating the Anterior Maxilla: A Case Report.

    Science.gov (United States)

    Miranda, M E; Olivieri, K A; Rigolin, F J F; de Vasconcellos, A A

    2016-01-01

    The rehabilitation of an unesthetic smile in the anterior maxilla is always a clinical challenge, especially when an improper shape and size, old restorations, and unesthetic shading are present. In addition, an irregular gingival zenith contour in the anterior maxilla can affect the smile's harmony. Thus, detailed treatment planning is needed to define a functional and esthetic prosthetic rehabilitation. This study describes a clinical case in which a 55-year-old woman was rehabilitated using Digital Smile Design planning and full ceramic crowns (metal free) in the anterior zone of the maxilla and mandible. To normalize the gingival zenith, a dynamic compression technique was performed using provisional restorations to condition the gingival tissues and harmonize the proportional length of the anterior upper teeth.

  14. Restoration of primary anterior teeth: review of the literature.

    Science.gov (United States)

    Lee, Jacob K

    2002-01-01

    This paper reviews the published data on restorations of primary anterior teeth. The discussion includes Class III restorations, Class V restorations, various forms of full coronal restorations, atraumatic restorative technique (ART) and recommendations for future research.

  15. Cytomegalovirus as a cause of anterior uveitis in immunocompetent patients

    NARCIS (Netherlands)

    van Boxtel, Lonneke A. A.; van der Lelij, Allegonda; van der Meer, Johannes; Los, Leonoor I.

    2007-01-01

    Purpose: To describe 7 cases of unilateral, chronic and/or recurrent anterior uveitis caused by cytomegalovirus (CMV) in immunocompetent patients; to identify specific ophthalmologic characteristics; and to evaluate the clinical effect of valganciclovir treatment. Design: Retrospective observational

  16. Sandwich-like reconstruction of anterior skull base defects

    Institute of Scientific and Technical Information of China (English)

    WANG Zhengmin; WANG Dehui

    2002-01-01

    @@ RESULTS From October, 1984 to October, 1998, 116 patients underwent transcranial or transcranial-facial approach for the resection of malignant or benign aggressive tumor, and sandwich-like repairs were performed for the anterior skull base defect.

  17. CARTILAGE-LIKE PHENOMENON IN THE ANTERIOR CRUCIATE LIGAMENT

    Institute of Scientific and Technical Information of China (English)

    2001-01-01

    Objective. To detect histological characteristic of anterior cruciate ligament (ACL) and medial collateral ligament (MCL). Methods. In each of 20 skeletally mature male mongrels and 4 men, the ACL and MCL were examined by standard hematoxylin-eosin procedure and toluidine blue staining for histologic observation. Results. The fibroblasts in medial collateral are elongated to spindle shape and aligned in a row between the bundles of collagenous fibers. Toluidine blue staining is negative. The anterior cruciate ligament demonstrated more heterogenous cell types and arrangement. It had three major cell forms:spindle, round and ovoid type, which were shorter but greater than the cells in medial collateral ligament. Toluidine blue staining was positive in anterior cruciate ligament. Most cells in anterior cruciate ligament were enclosed within lacunae. Conclusion. This study suggests that the ACL has different histological characteristics from MCL, and is more cartilage-like in nature.

  18. CARTILAGE-LIKE PHENOMENON IN THE ANTERIOR CRUCIATE LIGAMENT

    Institute of Scientific and Technical Information of China (English)

    蒋青; 林共周; 典绵域; 崔国庆; 滕华建

    2001-01-01

    Objective. To detect histological characteristic of anterior cruciate ligament (ACL) and medial collateral ligament (MCL). Methods. In each of 20 skeletally mature male mongrels and 4 men, the ACL and MCL were examined by standard hematoxylin-eosin procedure and toluidine blue staining for histologic observation. Results. The fibroblasts in medial collateral are elongated to spindle shape and aligned in a row between the bundles of collagenous fibers. Toluidine blue staining is negative. The anterior cruciate ligament demonstrated more heterogenous cell types and arrangement. It had three major cell forms: spindle, round and ovoid type,which were shorter but greater than the cells in medial collateral ligament. Toluidine blue staining was positive in anterior cruciate ligament. Most cells in anterior cruciate ligament were enclosed within lacunae. Conclusion. This study suggests that the ACL has different histological characteristics from MCL, and is more cartilage-like in nature.

  19. Anterior sacral meningocoele presenting as a peri-anal abscess.

    Science.gov (United States)

    Buxton, N; Bassi, S; Firth, J

    2002-06-01

    Anterior sacral meningoceole is a rare occurrence and presentation as a perianal abscess has not been previously reported. The case is presented and the condition discussed. The potential risks of failing to establish the diagnosis, prior to surgery, are outlined.

  20. Elevator Muscle Anterior Resection: A New Technique for Blepharoptosis.

    Science.gov (United States)

    Zigiotti, Gian Luigi; Delia, Gabriele; Grenga, Pierluigi; Pichi, Francesco; Rechichi, Miguel; Jaroudi, Mahmoud O; d'Alcontres, Francesco Stagno; Lupo, Flavia; Meduri, Alessandro

    2016-01-01

    Blepharoptosis is a condition of inadequate upper eyelid position, with a downward displacement of the upper eyelid margin resulting in obstruction of the superior visual field. Levator resection is an effective technique that is routinely used to correct aponeurotic ptosis. The anterior levator resection is the procedure of choice in moderate blepharoptosis when there is moderate to good levator muscle function, furthermore, with an anterior approach, a greater resection can be achieved than by a conjunctival approach. The authors describe a modification in the Putterman technique with a resection done over a plicated elevator, plication that was suggested by Mustardè. The technique has been named as elevator muscle anterior resection. The elevator muscle anterior resection inspires from the Fasanella-Servat operation by the use of a clamp, making the operation simple and predictable.

  1. The role of the anterior commissure in callosal agenesis.

    Science.gov (United States)

    Barr, Melodie S; Corballis, Michael C

    2002-10-01

    Two individuals with callosal agenesis (J.P. and M.M.) and 10 neurologically normal participants were tested on tasks requiring interhemispheric visual integration. M.M., whose anterior commissure was within normal limits, was much worse at matching colors and letters between visual fields than within visual fields, whereas J.P., whose anterior commissure was greatly enlarged, showed no evidence of interhemispheric disconnection. This suggests that in some cases of callosal agenesis, probably a minority, an enlarged anterior commissure may compensate for the lack of the corpus callosum. Neither acallosal participant showed interhemispheric disconnection on tasks requiring integration of location and orientation, however, suggesting that the anterior commissure plays no role in such tasks. These tasks may depend on subcortical commissures, such as the intertectal commissure.

  2. Involuntary masturbation and hemiballismus after bilateral anterior cerebral artery infarction.

    Science.gov (United States)

    Bejot, Yannick; Caillier, Marie; Osseby, Guy-Victor; Didi, Roy; Ben Salem, Douraied; Moreau, Thibault; Giroud, Maurice

    2008-02-01

    Ischemia of the areas supplied by the anterior cerebral artery is relatively uncommon. In addition, combined hemiballismus and masturbation have rarely been reported in patients with cerebrovascular disease. We describe herein a 62-year-old right-handed man simultaneously exhibiting right side hemiballismus and involuntary masturbation with the left hand after bilateral infarction of the anterior cerebral artery territory. Right side hemiballismus was related to the disruption of afferent fibers from the left frontal lobe to the left subthalamic nucleus. Involuntary masturbation using the left hand was exclusively linked to a callosal type of alien hand syndrome secondary to infarction of the right side of the anterior corpus callosum. After 2 weeks, these abnormal behaviours were completely extinguished. This report stresses the wide diversity of clinical manifestations observed after infarction of the anterior cerebral artery territory.

  3. Acute exertional anterior compartment syndrome in an adolescent female.

    Science.gov (United States)

    Fehlandt, A; Micheli, L

    1995-01-01

    Acute compartment syndromes usually occur as a complication of major trauma. While the chronic exertional anterior tibial compartment syndrome is well described in the sports medicine literature, reports of acute tibial compartment syndromes due to physical exertion, or repetitive microtrauma, are rare. The case of an adolescent female who developed an acute anterior compartment syndrome from running in a soccer game is described in this report. Failure to recognize the onset of an acute exertional compartment syndrome may lead to treatment delay and serious complications. Whereas the chronic exertional anterior compartment syndrome is characterized by pain that diminishes with the cessation of exercise, the onset of the acute exertional anterior compartment syndrome is heralded by pain that continues, or increases, after exercise has stopped. Compartment pressure measurement confirms the clinical diagnosis and helps guide treatment. True compartment syndromes require urgent fasciotomy.

  4. Case study: limitations of panoramic radiography in the anterior mandible.

    LENUS (Irish Health Repository)

    Walker, Cameron

    2009-12-01

    Dental Panoramic Tomography (DPT) is a widely used and valuable examination in dentistry. One area prone to artefacts and therefore misinterpretation is the anterior region of the mandible. This case study discusses a periapical radiolucency related to lower anterior teeth that is discovered to be a radiographic artefact. Possible causes of the artefact include a pronounced depression in the mental region of the mandible or superimposition of intervertebral spaces. Additional limitations of the DPT image include superimposition of radio-opaque structures, reduced image detail compared to intra-oral views and uneven magnification. These problems often make the DPT inappropriate for imaging the anterior mandible. Clinical Relevance: Panoramic radiography is often unsuitable for radiographic examination of the anterior mandible.

  5. Increased CD40 ligand in patients with acute anterior uveitis

    DEFF Research Database (Denmark)

    Øgard, Carsten; Sørensen, Torben Lykke; Krogh, Erik

    2005-01-01

    The inflammatory response in acute anterior uveitis (AU) is believed to be primarily mediated by autoreactive T-cells. We wanted to evaluate whether the T-cell activation marker CD40 ligand is involved in the AU immunopathogenesis.......The inflammatory response in acute anterior uveitis (AU) is believed to be primarily mediated by autoreactive T-cells. We wanted to evaluate whether the T-cell activation marker CD40 ligand is involved in the AU immunopathogenesis....

  6. An overlay partial denture to restore worn mandibular anterior teeth.

    Science.gov (United States)

    Samant, Asha; DeSciscio, Peter

    2014-01-01

    Restoring worn anterior mandibular teeth is a challenge, especially when teeth are small, esthetics are a concern, the long-term prognosis is questionable, and/or patient finances are an issue. This article describes an alternate treatment for a patient with a collapsed bite, missing posterior mandibular teeth, an ill-fitting complete maxillary denture with poor esthetics, and irregular, worn mandibular anterior teeth.

  7. Anterior Cruciate Ligament OsteoArthritis Score (ACLOAS)

    DEFF Research Database (Denmark)

    Roemer, Frank W; Frobell, Richard; Lohmander, Stefan

    2014-01-01

    OBJECTIVE: To develop a whole joint scoring system, the Anterior Cruciate Ligament OsteoArthritis Score (ACLOAS), for magnetic resonance imaging (MRI)-based assessment of acute anterior cruciate ligament (ACL) injury and follow-up of structural sequelae, and to assess its reliability. DESIGN...... and longitudinal changes including osteoarthritis (OA) features. Joint features assessed were acute osteochondral injury, traumatic and degenerative bone marrow lesions (BMLs), meniscus morphology and extrusion, osteophytes, collateral and cruciate ligaments including ACL graft, Hoffa-synovitis and effusion...

  8. [Chondroblastoma in the anterior cruciate ligament origo: a case report].

    Science.gov (United States)

    Aydin, Hafız; Turhan, Ahmet Uğur; Karataş, Metin; Onay, Atilgan; Yildiz, Kadriye

    2012-01-01

    Chondroblastoma is a rarely seen cartilage originated tumor. It is mostly localized in the epiphysis of long bones. In this article, we present an 18-year-old male case in whom the tumor was located in the right distal femoral lateral condyle and destroyed anterior cruciate ligament origo. The tumor was curetted and the cavity was filled with cement. Anterior cruciate ligament resection was mandatory for this treatment. The patient had no complaint in the postoperative period.

  9. Resorption of labial bone in maxillary anterior implant

    OpenAIRE

    Cho, Young-Bum; Moon, Seung-Jin; Chung, Chae-Heon; Kim, Hee-Jung

    2011-01-01

    PURPOSE The purpose of this study was to evaluate the amount of resorption and thickness of labial bone in anterior maxillary implant using cone beam computed tomography with Hitachi CB Mercuray (Hitachi, Medico, Tokyo, Japan). MATERIALS AND METHODS Twenty-one patients with 26 implants were followed-up and checked with CBCT. 21 OSSEOTITE NT® (3i/implant Innovations, Florida, USA) and 5 OSSEOTITE® implants (3i/implant Innovations, Florida, USA) were placed at anterior region and they were posi...

  10. Neovascular glaucoma treatment with extraction of anterior chamber fibrovascular tissue.

    Science.gov (United States)

    Nadal, Jeroni; Carreras, Elisa; Kudsieh, Bachar; Canut, Maribel

    2013-08-01

    The use of antibody to vascular endothelial growth factor to treat neovascular glaucoma yields good anatomic results in most cases. However, this type of glaucoma can cause angle closure with decompensation of intraocular pressure secondary to fibrovascular tissue contraction in the anterior chamber. Our surgical technique treats the cause by removing the anterior chamber fibrous complex after administration of antibody to vascular endothelial growth factor, thus restoring the chamber angle.

  11. Altered anterior visual system development following early monocular enucleation

    Directory of Open Access Journals (Sweden)

    Krista R. Kelly

    2014-01-01

    Conclusions: The novel finding of an asymmetry in morphology of the anterior visual system following long-term survival from early monocular enucleation indicates altered postnatal visual development. Possible mechanisms behind this altered development include recruitment of deafferented cells by crossing nasal fibres and/or geniculate cell retention via feedback from primary visual cortex. These data highlight the importance of balanced binocular input during postnatal maturation for typical anterior visual system morphology.

  12. Relevance of anterior mandibular body ostectomy in mandibular prognathism

    Science.gov (United States)

    Bansal, Pankaj; Singh, Virender; Anand, S. C.; Bansal, Sumidha

    2013-01-01

    Purpose: We tried to find out the relevance of anterior mandibular body ostectomy in deformities of the mandible specially prognathism, which is primarily limited to anterior part only. Patients and Methods: Ten patients with skeletal deformity along with malocclusion, which was limited to anterior body of mandible were selected. Selected patients had proper molar interdigitation (even if class 3) and in general had anterior crossbite (except one). All patients had crossed their growth spurts and had no hormonal influence on facial deformity. Specific protocol, including cephelometric analysis cephalometry for orthognathic surgery, prediction tracing and model surgeries were devised. Pre and post-surgical orthodontics and body ostectomy were performed in all patients along with 18-month post-op follow-up. Results: There was significant reduction in prognathism and horizontal dysplasia in all ten patients. Anterior crossbite as well as axis of incisiors over mandibular plane was corrected in all patients due to decrease in length of mandibular body. All patients showed decreased facial height and better lip competence with intact posterior occlusion and no (negligible or transient) sensory loss. Conclusions: Our study could confirm that people whose deformity is limited to the anterior part of mandible with reasonable occlusion posteriorly can get satisfactory cosmetic and functional results through body ostectomy alone rather than going for surgical procedure in the ramal area, which is liable to cause sensory and occlusal disturbances. PMID:24163554

  13. A Technique of Improved Medial Meniscus Visualization by Anterior Cruciate Ligament Graft Placement in Chronic Anterior Cruciate Deficient Knees.

    Science.gov (United States)

    Vertullo, Christopher J; Wijenayake, Lahann; Grayson, Jane E

    2016-04-01

    It is customary to perform medial meniscus repair before anterior cruciate ligament (ACL) graft placement when undertaken as a combined procedure. However, in chronic ACL-deficient knees, intraoperative anterior tibiofemoral translation can cause the medial meniscus repair to be more technically challenging. Intraoperative anterior tibiofemoral translation can both reduce the visualization of the medial meniscus and make its reduction unstable. An operative sequence alteration of ACL graft placement and tensioning before medial meniscal repair improves medial meniscus visualization in chronically ACL-deficient knees by using the ACL graft's ability to prevent anterior tibiofemoral translation. The technique sequence is as follows: (a) the medial meniscus is reduced, (b) ACL reconstruction is undertaken using a hamstring graft without final tibia fixation,

  14. 骨外周性原始神经外胚层瘤的影像学诊断(附3例报告)%Imaging diagnosis of peripheral primitive neuroectodermal tumor of the bone:a report of 3 cases

    Institute of Scientific and Technical Information of China (English)

    刘衡; 操玉萍; 李晓东; 王永涛; 李邦国; 骆科进

    2011-01-01

    @@ 原始神经外胚层瘤(primitive neuroectodermal tumor, PNET)是一种衍生于神经嵴的恶性小圆细胞肿瘤,根据发生部位的不同分为中枢性和外周性.外周性原始神经外胚层瘤(peripheral primitive neuroectodermal tumors,pPNET)最常见于胸肺部,其次是盆腔、后腹膜、脊柱旁,发生于骨的外周性原始神经外胚层瘤少见[1].现搜集本院经病理证实的患者3例,结合国内外文献分析其影像学表现,旨在提高对其的认识和诊断水平.

  15. Humeral avulsion of the anterior shoulder stabilizing structures after anterior shoulder dislocation: demonstration by MRI and MR arthrography

    Energy Technology Data Exchange (ETDEWEB)

    Tirman, P.F.J. [San Francisco Magnetic Resonance Center, San Francisco, CA (United States); Steinbach, L.S. [Department of Radiology, University of California, San Francisco, CA (United States); Feller, J.F. [Department of Veterans Affairs, David Grant USAF Medical Center, Travis Air Force Base, CA (United States); Stauffer, A.E. [Radiologic Imaging Associates, Mission Viejo, CA (United States)

    1996-11-01

    Objective. To demonstrate the MRI findings of an anterior shoulder capsular avulsion from the humerus, with or without subscapularis rupture, after anterior dislocation or severe abduction external rotation injury. Design and patients. We retrospectively reviewed the MRI and MR arthrographic examinations of seven patients who were identified at surgery with avulsion of the anterior shoulder stabilizers from the humerus. MRI was correlated with clinical history and surgical results. Results. MRI findings included: inhomogeneity or frank disruption of the anterior capsule at the humeral insertion (all), fluid intensity anterior to the shoulder (six patients), tear of the subscapularis tendon (six patients), dislocation of the biceps tendon (four patients), and a Hill-Sachs deformity (four patients). MR arthrography additionally found extravasation of contrast through the capsular defect (two patients). Conclusions. Our findings suggest that MRI is helpful for diagnosing humeral avulsion of the anterior glenohumeral capsule, especially when a tear of the subscapularis tendon insertion is present. MR arthrography may be of benefit for diagnosing capsular avulsion without associated subscapularis tendon abnormality. (orig.). With 4 figs.

  16. IGF-2/IGF-1R signaling has distinct effects on Sox1, Irx3, and Six3 expressions during ES cell derived-neuroectoderm development in vitro.

    Science.gov (United States)

    Takata, Nozomu; Sakakura, Eriko; Sasai, Yoshiki

    2016-05-01

    Insulin-like growth factors (IGFs) are involved in growth and tissue development, including diseases such as type-2 diabetes and cancers. However, their roles in lineage specification, especially in early mammalian neural development, are poorly understood. Here, we analyzed the protein expression of IGF-2 in early mouse embryo, and it was preferentially detected in anterior mesodermal tissue, adjacent to the neural plate. We utilized a self-organizing neural tissue culture system and analyzed the direct effect of IGF-2 on the general neural marker Sox1. Interestingly, using recombinant IGF-2 and a chemical inhibitor of its receptor (IGF-1R), we found that the IGF-2/IGF-1R pathway positively regulated Sox1 expression in embryonic stem (ES) cell-derived neural tissue. Furthermore, to visualize the expression patterns of other neural markers, we used reporter ES cell lines and we found that the IGF-2/IGF-1R signaling upregulated the expression of the posterior neural marker Irx3. In contrast, the anterior neural marker Six3 was downregulated by IGF-2/IGF-1R signaling. Together, our results demonstrate that IGF-2/IGF-1R signaling has different effects on neural marker expression, which may influence the early regional identity of ES cell-derived neural tissues.

  17. Silicone Oil Tamponade Combined with Lensectomy Preserving Anterior Lens Capsule

    Institute of Scientific and Technical Information of China (English)

    LuL; YangJ

    1999-01-01

    Purpose:To investigate the therapeutic effect and indicatio n of silicone oil tamponade combined with lensectomy preserving anterior lens capsule.Methods:Silicone oil tamponade combined with lensectomy preserving anterior lens capsule was performed in 33 cases (33 eyes)of high myopia ,proliferative vitroretinopathy(PVR)D grade and giant retinal tear(GRT).10 cases were onlyeye and 11 cases had harder nucleus of lens.The surgical methods included:1.smashing lens nucleus and lensectomy preserving complete anterior capsule;2,vitrectomy and membrane peeling;3,usage of liquid perfluorocarbin or retinotomy or drainage;4.silicone oil tamponade;5.postoperative Nd;YAG laser for anterior capsulectomy. Results:Follow-up time was 6 months or more in 29 cases.Total retinal reattachment was achieved in 22 cases,macular retinal reattachment in 5 cases.The visual acuity was 20/800 to 20/200 in 13 cases,20/100 to 20/50 in 12 cases.Visual acuity was significantly improved in GRT group(P<0.05).Complications included unexpected anterior capsule break intraoperatively,anterior capsule opacity,silicone oil emulsification and liquid perfluorocarbin remainig postoperatively.Conclusions:Silicone oil tamponade combined with lensectomy preserving anterior capsule was safe and can reduce the operative complications.The indications included:1.GRT complicated with high myopia;2,advanced PVR cases complicated with high myopia in which silicone oil must be used;3.severe lens opacity cases in which silicone oil must be used;4.complex retinal detachment of only ye.

  18. MRI anatomy of anteriorly displaced anus: what obstructs defecation?

    Energy Technology Data Exchange (ETDEWEB)

    AbouZeid, Amr Abdelhamid [Ain-Shams University, Department of Pediatric Surgery, Cairo (Egypt); Mohammad, Shaimaa Abdelsattar; Khairy, Khaled Talaat [Ain-Shams University, Department of Radiodiagnosis, Cairo (Egypt)

    2014-07-15

    Anteriorly displaced anus is an anomaly that is debated with regard to its nomenclature, diagnosis and management. To describe MRI anatomy of the anal canal in children with anteriorly displaced anus and its impact on the process of defecation. We prospectively examined ten children (7 girls, 3 boys; age range 7 months to 8 years, mean 3 years) with anteriorly displaced anus between August 2009 and April 2012. Noncontrast MRI examinations were performed on a 1.5-T magnet. T1- and T2-weighted turbo spin-echo images were acquired in axial, sagittal and coronal planes of the pelvis. The anorectal angle and the relative hiatal distance were measured in mid-sagittal images, and compared with those of a control group using the Mann-Whitney test. In children with anteriorly displaced anus, no anatomical abnormality was depicted at the level of the proximal anal canal. However, the distal anal canal was displaced anteriorly, running out its external muscle cuff, which remained un-displaced at the usual site of the anus. This changes the orientation of the central axis of the anal canal by passing across instead of along the fibers of the longitudinal muscle coat. Children with anteriorly displaced anus had a more obtuse anorectal angle (mean 112.1 ), which was significantly greater than that of the control group (mean 86.2 ). MRI is a valuable tool in studying the anatomy of the anal canal in children with anteriorly displaced anus. The abnormal orientation of the longitudinal muscle across the anal canal can explain the obstructed defecation in these children. Based on this study, it might be of interest to use MRI in studying equivocal cases and children with unexplained constipation. (orig.)

  19. CT and MR diagnosis for abdominal peripheral primitive neuroectodermal tumor%腹部外周性原始神经外胚层肿瘤的CT及MR诊断

    Institute of Scientific and Technical Information of China (English)

    彭晓容; 马春浓; 陈恩炎; 黄海涛

    2016-01-01

    目的:研究分析CT与磁共振(MR)在诊断腹部外周性原始神经外胚层肿瘤中的应用价值。方法60例腹部外周性原始神经外胚层肿瘤患者,在研究分析患者临床资料的基础上,患者均给予CT与MR扫描检查,观察检查结果。结果诊断结果显示,有32例患者的神经外胚层肿瘤出现于腹膜腔,16例患者肿瘤位于腹膜后间隙,另12例患者肿瘤分别位于左肾、右肾上腺与胰腺(各4例);神经外胚层肿瘤位于胰腺部位的患者出现恶心、呕吐与黄疸等症状;在患者接受CT平扫时,影像学资料表现出不均匀的软组织肿块,且呈现出浸润性生长,与周围组织之间的界限并不是非常清晰,肿块直径3~15 cm,且平扫显示等密度或低密度;而在进行MR平扫时,发现T1WI出现等或低较为混杂的信号,肿瘤表现出的信号与肌肉相等或略低,而T2WI及抑脂像表现出不均匀的高信号或混杂信号,检查出的大部分肿瘤是不均匀的高信号,少数有出血征兆;在CT与MR增强扫描时,肿瘤表现出不均匀强化,且还会出现不同程度坏死的现象,并且还可观察到肿瘤出现隔样改变及网格状强化,在肿瘤靠近周围骨质时,就会发现相邻骨质被破坏,有的甚至可观察到骨膜反应。结论在诊断腹部外周性原始神经外胚层肿瘤时,利用CT与MR可充分显示肿瘤内部结构和相邻组织之间的关系,同时还能够观察到肿瘤转移情况,在临床中具有非常高的应用价值。%ObjectiveTo research and analyze application value of CT and magnetic resonance (MR) in diagnosis of abdominal peripheral primitive neuroectodermal tumor.MethodsA total of 60 patients with abdominal peripheral primitive neuroectodermal tumor received CT and MR scan examination in addition to clinical data analysis, and their examination outcomes were observed.ResultsDiagnosis outcome showed 32 cases with neuroectodermal tumor in peritoneal cavity, 16 cases in

  20. [Microsurgical anatomy importance of A1-anterior communicating artery complex].

    Science.gov (United States)

    Monroy-Sosa, Alejandro; Pérez-Cruz, Julio César; Reyes-Soto, Gervith; Delgado-Hernández, Carlos; Macías-Duvignau, Mario Alberto; Delgado-Reyes, Luis

    2013-01-01

    Antecedentes: la arteria cerebral anterior se origina de la bifurcación de la arteria carótida interna lateral al quiasma óptico, posteriormente se une con su homóloga contralateral mediante la arteria comunicante anterior. El complejo precomunicante(A1)-arteria comunicante anterior es el lugar más frecuente de variantes anatómicas y el sitio con mayor cantidad de aneurismas (30 a 37%). Objetivo: conocer la anatomía microquirúrgica, las variantes anatómicas y la importancia del complejo segmento precomunicante-arteria comunicante anterior en cirugía neurológica de la patología vascular, principalmente aneurismas, en población mexicana. Material y métodos: estudio prospectivo y descriptivo efectuado en el Departamento de Anatomía de la Facultad de Medicina (UNAM) en 30 encéfalos inyectados. Se estudió la anatomía microquirúrgica (longitud y calibre) del complejo segmento precomunicante-arteria comunicante anterior de la arteria cerebral anterior y sus variantes. Resultados: se encontraron 60 segmentos precomunicantes. La longitud promedio del lado izquierdo fue de 11.35 mm y del derecho de 11.84 mm. El calibre medio en el lado izquierdo fue de 1.67 mm y en el derecho de 1.64 mm. El número promedio de perforantes en el lado izquierdo fue de 7.9 y en el derecho de 7.5. La arteria comunicante anterior se encontró en 29 encéfalos sobre el quiasma óptico, su trayecto dependió de la longitud del segmento A1. La longitud media del segmento fue de 2.84 mm, el calibre fue de 1.41 mm y el número promedio de perforantes de 3.27. En 18 encéfalos (60%) se encontraron variantes del complejo A1-arteria comunicante anterior y dos aneurismas tipo blíster. Conclusión: es necesario entender la anatomía microquirúrgica del complejo segmento precomunicante-arteria comunicante anterior y conocer las variantes para tener una visión en tercera dimensión durante la cirugía de aneurismas.

  1. Integrated imaging appearances of peripheral primitive neuroectodermal tumors and pathology correlation%外周性原始神经外胚层瘤的影像表现

    Institute of Scientific and Technical Information of China (English)

    陈一平; 钟丽琴; 朱建英

    2012-01-01

    Objective: To explore the integrated imaging appearances of peripheral primitive neuroectodermal tumors (pPNETs). Methods: CT or MRI were performed in 12 patients of pPNETs (1 patient with DSA), which were confirmed by pathology. Results:5 cases were located in the soft tissue of chest wall, 1 case in the inferior lobe of right lung. 2 cases in the humerus. 1 case in the femur, 1 case in the tibia, and 2 cases in the vertebra. CT scan for soft tissue pPNETs of chest wall showed a large soft tissue mass with necrosis that boundary was not clear, without calcification, and contrast-enhanced CT showed marked inhomogeneous enhancement. CT scan for pPNETs of inferior lobe of right lung showed lob-ulated mass with hemorrhage that boundary was clear, and contrast-enhanced CT showed marked enhancement. PPNETs of the long bone showed bone destruction with a huge soft tissue mass, without calcification, the mass showed iso-intensi-ty or hypo-intensity on MR Ti WI, heterogeneous high signal on T2WI, contrast-enhanced showed marked enhancement. PPNETs of the spine showed vertebral body or accessory bone destruction with soft tissue mass, not involving the inter-vertebral disc. DSA of the sacrum pPNETs revealed the tumor from the internal iliac artery and a lot of newborn blood vessels. Conclusion:CT and MRI can show the pPNETs well with its internal structure, extent of disease, whether invading the adjacent organs and tissueor or metastasis. The final diagnosis still depends on pathological and immunohisto-chemical examination. DSA can determine the supplying artery of the tumor and reveal the rich tumor blood vessels, which can serve as a complementary examination.%目的:探讨外周性原始神经外胚层肿瘤的影像学表现.方法:回顾性分析经病理证实的12例外周性神经外胚层瘤的影像学资料.9例行CT扫描,4例行MR扫描,其中1例行DSA检查.结果:胸壁软组织5例,右肺下叶1例,肱骨2例,股骨1例,胫骨1例,脊椎2

  2. Spontaneous Scleral Perforation of an Anterior Chamber Intraocular Lens

    Directory of Open Access Journals (Sweden)

    Oriel Spierer

    2016-05-01

    Full Text Available A routine eye examination of a 69-year-old man revealed a scleral perforation of one of the haptics of the anterior chamber intraocular lens (AC IOL which had been implanted many years ago. The patient was asymptomatic with good visual acuity. His history was negative for any trauma, eye rubbing, topical corticosteroid use, or autoimmune disease. The horizontal and vertical white-to-white diameters of the cornea in the right eye were 11.5 and 10.5 mm, respectively. Anterior segment optical coherence tomography showed the anterior chamber length to be 12.28 mm horizontally and 10.63 mm vertically. The patient underwent an IOL exchange, and the length of the explanted AC IOL was measured to be 12 mm. We speculate that the AC IOL, which was vertically aligned, was oversized. This case demonstrates the need for proper sizing and positioning of an AC IOL. In complex cases where AC IOL may be used, measuring the horizontal and vertical lengths of the anterior chamber by anterior segment optical coherence tomography prior to surgery may be useful.

  3. Spontaneous Scleral Perforation of an Anterior Chamber Intraocular Lens

    Science.gov (United States)

    Spierer, Oriel; O'Brien, Terrence P.

    2016-01-01

    A routine eye examination of a 69-year-old man revealed a scleral perforation of one of the haptics of the anterior chamber intraocular lens (AC IOL) which had been implanted many years ago. The patient was asymptomatic with good visual acuity. His history was negative for any trauma, eye rubbing, topical corticosteroid use, or autoimmune disease. The horizontal and vertical white-to-white diameters of the cornea in the right eye were 11.5 and 10.5 mm, respectively. Anterior segment optical coherence tomography showed the anterior chamber length to be 12.28 mm horizontally and 10.63 mm vertically. The patient underwent an IOL exchange, and the length of the explanted AC IOL was measured to be 12 mm. We speculate that the AC IOL, which was vertically aligned, was oversized. This case demonstrates the need for proper sizing and positioning of an AC IOL. In complex cases where AC IOL may be used, measuring the horizontal and vertical lengths of the anterior chamber by anterior segment optical coherence tomography prior to surgery may be useful. PMID:27462251

  4. Spontaneous Scleral Perforation of an Anterior Chamber Intraocular Lens.

    Science.gov (United States)

    Spierer, Oriel; O'Brien, Terrence P

    2016-01-01

    A routine eye examination of a 69-year-old man revealed a scleral perforation of one of the haptics of the anterior chamber intraocular lens (AC IOL) which had been implanted many years ago. The patient was asymptomatic with good visual acuity. His history was negative for any trauma, eye rubbing, topical corticosteroid use, or autoimmune disease. The horizontal and vertical white-to-white diameters of the cornea in the right eye were 11.5 and 10.5 mm, respectively. Anterior segment optical coherence tomography showed the anterior chamber length to be 12.28 mm horizontally and 10.63 mm vertically. The patient underwent an IOL exchange, and the length of the explanted AC IOL was measured to be 12 mm. We speculate that the AC IOL, which was vertically aligned, was oversized. This case demonstrates the need for proper sizing and positioning of an AC IOL. In complex cases where AC IOL may be used, measuring the horizontal and vertical lengths of the anterior chamber by anterior segment optical coherence tomography prior to surgery may be useful.

  5. Myringoplasty for anterior and subtotal perforations using KTP-532 laser.

    Science.gov (United States)

    Gerlinger, Imre; Ráth, Gábor; Szanyi, István; Pytel, József

    2006-09-01

    A retrospective study was performed on patients who underwent myringoplasty for either anterior or subtotal perforations over an 8-year period (from 1994 till 2004). We used the KTP-laser assisted anterior anchoring technique combining with anterior "pull-back" method. Patients' ages ranged from 6-62 years (median 36.5). The mean follow-up period was 2.8 years (minimum 6 months). The audiological results were analysed with the "Pytel software", which was developed in our department. As for the procedure, the drum remnant was freed from the malleus handle with the use of the laser and elevated out of it's sulcus anterior-superiorly. Large fascia graft was fashioned with a split of 4-5 mm in the middle of one edge. The graft was placed using the underlay technique medial to the handle of the malleus. A pull-back tunnel was created at the border of the anterior quadrants to further facilitate the survival of the graft. In this series the graft taking rate was 100%. Reperforation due to an undersized fascia was observed in one case. Post-operative audiological results indicated no bone conduction threshold elevation in any frequencies. Using the laser, cochlear trauma can be prevented, double fixation of the drum prevents lateralisation and blunting. Wide canalplasty makes both the approach and the follow-up very easy. Thorough soft tissue and bone work is advantageous from the fascia taking rate point of view.

  6. Evaluation of Anterior Segment's Structures in Tilted Disc Syndrome

    Science.gov (United States)

    Ozsoy, Ercan; Demirel, Ersin Ersan; Cumurcu, Tongabay

    2016-01-01

    Purpose. To evaluate anterior segment's structures by Pentacam in patients with tilted disc syndrome (TDS). Methods. Group 1 included forty-six eyes of forty-six patients who have the TDS. Group 2 including forty-six eyes of forty-six cases was the control group which was equal to the study group in age, gender, and refraction. A complete ophthalmic examination was performed in both groups. All cases were evaluated by Pentacam. The axial length (AL) of eyes was measured by ultrasound. Quantitative data obtained from these measurements were compared between two groups. Results. There was no statistically significant difference for age, gender, axial length, and spherical equivalent measurements between two groups (p = 0.625, p = 0.830, p = 0.234, and p = 0.850). There was a statistically significant difference for central corneal thickness (CCT), corneal volume (CV), anterior chamber angle (ACA), and pupil size measurements between two groups (p = 0.001, p = 0.0001, p = 0.003, and p = 0.001). Also, there was no statistically significant difference for anterior chamber depth (ACD), anterior chamber volume (ACV), and lens thickness (LT) measurements between two groups (p = 0.130, p = 0.910, and p = 0.057). Conclusion. We determined that CCT was thinner, CV was less, and ACA was narrower in patients with TDS. There are some changes in the anterior segment of the eyes with tilted disc. PMID:27648303

  7. Psychological Aspects of Recovery Following Anterior Cruciate Ligament Reconstruction.

    Science.gov (United States)

    Christino, Melissa A; Fantry, Amanda J; Vopat, Bryan G

    2015-08-01

    Recovery following anterior cruciate ligament reconstruction is an arduous process that requires a significant mental and physical commitment to rehabilitation. Orthopaedic research in recent years has focused on optimizing anterior cruciate ligament surgical techniques; however, despite stable anterior cruciate ligament reconstructions, many athletes still never achieve their preinjury ability or even return to sport. Psychological factors associated with patient perceptions and functional outcomes following anterior cruciate ligament reconstruction are important to acknowledge and understand. Issues related to emotional disturbance, motivation, self-esteem, locus of control, and self-efficacy can have profound effects on patients' compliance, athletic identity, and readiness to return to sport. The psychological aspects of recovery play a critical role in functional outcomes, and a better understanding of these concepts is essential to optimize the treatment of patients undergoing anterior cruciate ligament reconstruction, particularly those who plan to return to sport. Identifying at-risk patients, encouraging a multidisciplinary approach to patient care, and providing early referral to a sports psychologist may improve patient outcomes and increase return-to-play rates among athletes.

  8. Anterior chamber lenses. Part II: A laboratory study.

    Science.gov (United States)

    Apple, D J; Hansen, S O; Richards, S C; Ellis, G W; Kavka-Van Norman, D; Tetz, M R; Pfeffer, B R; Park, R B; Crandall, A S; Olson, R J

    1987-03-01

    An analysis of 606 surgically removed anterior chamber intraocular lens (IOL) specimens revealed that 351 or 58% of these were small-diameter, round loop, closed-loop styles. Because of the extremely high percentage of IOLs with this design received in our laboratory and the correlation of clinical histories with our histopathologic findings, we have concluded that such IOLs do not provide the safety and efficacy achieved by other anterior chamber lens designs. The finely polished, one-piece, all-PMMA styles fared well in our study. Although these one-piece styles comprise well over 50% of the American market share of anterior chamber IOLs, they comprise only 14% of all anterior chamber IOLs accessioned in our laboratory, compared to 58% for closed-loop designs. We believe that implantation of anterior chamber lenses with small-diameter, round, closed loops is no longer warranted. Patients in whom these IOLs have already been implanted should be carefully followed. It is our opinion that the FDA should recall or closely monitor all IOLs of this design and that implantation of closed-loop lenses should be discontinued in the United States. Furthermore, we believe that an IOL deemed to be not medically sound or worthy of implantation in the United States should not be marketed or donated outside of this country.

  9. Features extraction in anterior and posterior cruciate ligaments analysis.

    Science.gov (United States)

    Zarychta, P

    2015-12-01

    The main aim of this research is finding the feature vectors of the anterior and posterior cruciate ligaments (ACL and PCL). These feature vectors have to clearly define the ligaments structure and make it easier to diagnose them. Extraction of feature vectors is obtained by analysis of both anterior and posterior cruciate ligaments. This procedure is performed after the extraction process of both ligaments. In the first stage in order to reduce the area of analysis a region of interest including cruciate ligaments (CL) is outlined in order to reduce the area of analysis. In this case, the fuzzy C-means algorithm with median modification helping to reduce blurred edges has been implemented. After finding the region of interest (ROI), the fuzzy connectedness procedure is performed. This procedure permits to extract the anterior and posterior cruciate ligament structures. In the last stage, on the basis of the extracted anterior and posterior cruciate ligament structures, 3-dimensional models of the anterior and posterior cruciate ligament are built and the feature vectors created. This methodology has been implemented in MATLAB and tested on clinical T1-weighted magnetic resonance imaging (MRI) slices of the knee joint. The 3D display is based on the Visualization Toolkit (VTK).

  10. Identification of a Colonial Chordate Histocompatibility Gene

    Science.gov (United States)

    Voskoboynik, Ayelet; Newman, Aaron M.; Corey, Daniel M.; Sahoo, Debashis; Pushkarev, Dmitry; Neff, Norma F.; Passarelli, Benedetto; Koh, Winston; Ishizuka, Katherine J.; Palmeri, Karla J.; Dimov, Ivan K.; Keasar, Chen; Fan, H. Christina; Mantalas, Gary L.; Sinha, Rahul; Penland, Lolita; Quake, Stephen R.; Weissman, Irving L.

    2013-01-01

    Histocompatibility is the basis by which multicellular organisms of the same species distinguish self from non-self. Relatively little is known about the mechanisms underlying histocompatibility reactions in lower organisms. Botryllus schlosseri is a colonial urochordate, a sister group of vertebrates, that exhibits a genetically determined natural transplantation reaction, whereby self-recognition between colonies leads to formation of parabionts with a common vasculature, whereas rejection occurs between incompatible colonies. Using genetically defined lines, whole-transcriptome sequencing, and genomics, we identified a single gene that encodes self/non-self and determines “graft” outcomes in this organism. This gene is significantly upregulated in colonies poised to undergo fusion or rejection, is highly expressed in the vasculature, and is functionally linked to histocompatibility outcomes. These findings establish a platform for advancing the science of allorecognition. PMID:23888037

  11. Tendinopatia do compartimento anterior do tornozelo Tendinopathy of the anterior compartment of the ankle

    Directory of Open Access Journals (Sweden)

    Antonio Egydio de Carvalho Junior

    2010-01-01

    Full Text Available OBJETIVO: Análise retrospectiva da etiopatogenia, diagnóstico e opções de tratamento nos casos de tendinopatias do compartimento anterior do tornozelo (TCAT. MÉTODO: No período de setembro de 1998 a fevereiro de 2009, 13 pacientes foram operados por tendinopatia do compartimento anterior do tornozelo. A casuística constou de 10 pacientes do sexo masculino e três do feminino. O lado direito foi acometido em 12 pés e um do esquerdo. A média de idade foi de 35 anos (15-67. A etiologia foi traumática em oito pacientes e em cinco, degenerativa (atraumática. O tempo médio do diagnóstico ao tratamento foi de 19 meses (1-60 e o seguimento foi de 34 meses (4-127. O diagnóstico foi feito através da história e exame clínico. A ressonância magnética foi realizada em nove pacientes para estadiamento e planejamento. O tratamento cirúrgico foi personalizado para cada caso (sinovectomia, ressecção de ventre muscular, solidarização com o tendão adjacente e enxerto livre de tendão semitendíneo. Para a avaliação dos resultados foram utilizadas as escalas: 1 graduação subjetiva de satisfação, 2 AOFAS e 3 Maryland. RESULTADO: Em relação à escala de graduação subjetiva de satisfação, 12 pacientes satisfeitos e um paciente insatisfeito. A média da escala AOFAS foi de 80 pontos, a média da escala Maryland foi de 86 pontos. CONCLUSÃO: O tratamento cirúrgico é eficaz para recuperação funcional. As técnicas cirúrgicas devem ser personalizadas. A opção do enxerto livre de tendão semitendíneo é eficiente nas falhas maiores que cinco centímetros.OBJECTIVE: To carry out a retrospective analysis of the etiopathogeny, diagnosis and therapeutic options in cases of tendinopathies of the anterior compartment of the ankle. METHOD: 13 patients underwent surgery between September 1998 and February 2009; ten men and three women. The right side was involved in twelve patients and the left in one. The averaging age was 35 years of

  12. Primary primitive neuroectodermal tumor of cervix: a clinicopathologic study of 3 cases%原发性宫颈原始神经外胚层肿瘤3例临床病理分析

    Institute of Scientific and Technical Information of China (English)

    李青; 李凌; 张洵

    2012-01-01

    Objective To investigate the clinicopathological features, diagnosis, differential diagnosis and prognosis of primitive neuroectodermal tumor of cervix. Methods Three cases of primary primitive neuroectodermal tumor of cervix were observed by morphologic and immunohistochemical techniques and relative clinical data were analyzed. Results All 3 cases underwent surgery, within which 1 case received both preoperative and postoperative chemotherapy and radiation; 1 case underwent preoperative radiation and postoperative chemotherapy; 1 case gave up chemotherapy and radiation. Clinically, all patients presented with symptoms of vaginal bleeding and or pelvic pain with cervical masses. Histologically, all tumors showed unvarying small round cells, rare cytoplasm, indistinct cell borders, hyperchromatic blue round to oval nuclei, inconspicuous nucleoli and presenting pseudorosettes in one case. Immunohistochemically, the tumor cells were positive for CD99, vimentin, CD56, NSE and Syn in all 3 cases. Now all cases were still alive with a follow-up interval ranging from 15-10 months. Conclusion Primitive neuroectodermal tumor occurs rarely as a primary cervical neoplasm with a poor prognosis, which can be diagnosed on the base of pathologic detection, immunohistochemistry, cytogeneties and electron microscopy. Combined therapy including surgery, chemotherapy and radiation is recommended for the disease.%目的 探讨原发性宫颈原始神经外胚层肿瘤(PNET)的临床病理特点、诊断与鉴别诊断及预后.方法 回顾性分析3例宫颈PNET所有临床资料,对肿瘤组织进行HE切片、免疫组化染色,并进行随访.结果 临床表现为阴道出血、下腹疼痛及宫颈肿物.镜下肿瘤主要由胞质少、分化幼稚的小圆细胞构成,细胞界限不清,细胞核圆形、深染,核仁不明显;其中1例可见Homer-Wright菊形团及神经胶质分化.免疫组化主要表现为CD99、vimentin、CD56、NSE和Syn(+).3

  13. CT and MRI Features of Peripheral Primitive Neuroectodermal Tumors%外周性原始神经外胚层肿瘤的CT及MRI影像学特征

    Institute of Scientific and Technical Information of China (English)

    许倩; 徐凯; 李绍东; 孟闫凯; 杨春; 刘颖

    2011-01-01

    Objective To analyze the CT and MRI features of peripheral primitive neuroectodermal tumors (pPNETs) , and to improve its diagnostic accuracy. Materials and Methods Retrospectively analysis of CT and MRI features of 11 cases with peripheral primitive neuroectodermal tumors confirmed pathologically and immunohistochemically was performed. Correlation with clinical data and pathology were obtained. Spiral CT{9/11) and MRI(2/11) scanning were performed pre-operatively. Results 11 lesions were located in nasal cavity (n = 1) ,chest wall (n = 4) ,retroperitoneum (n = 2) .pelvic cavity ( n = 2 ) , spine (n = 2 ). Nine lesions arising from soft (issue mainly demonstrated large and infiltrative mass with or without bony destruction. Most of them showed inhomogeneous density on CT plain scans and moderate heterogeneous enhancement after injection of contrast agents. Tiny feeding artery within some tumors could be found in the arterial phase. In the venous phase, the lesion persistently enhanced and the density was more homogeneous. Bony destruction of vertebrae surrounded by soft tissue mass having no clear interface with neighboring normal tissue were features of spine pPNET on MRI, which appeared heterogeneous enhancement Conclusion pPNETs have clinical characteristics and imaging features. The key to correct diagnosis is to inosculate imaging and clinical data.%目的 分析外周性原始神经外胚层肿瘤(peripheral primitive neuroectodermal tumor,pPNET)的CT和MRI影像学表现,以提高对其认识和诊断的准确性.资料与方法 回顾性分析经病理证实的11例pPNET患者的CT及MRI影像学表现.11例pPNET中9例行CT检查,2例行脊柱MRI检查.结果 11例病灶中1例位于鼻腔,4例位于胸壁,2例位于腹膜后,2例位于盆腔,2例位于脊柱.9例软组织pPNET表现为较大且浸润性生长的软组织肿块,伴或不伴邻近骨质的溶骨性破坏.肿块内密度多不均匀,增强后呈中等程度以上不均匀强化.双期扫

  14. 外周原始神经外胚层肿瘤的影像诊断(附31例病例复习)%Imaging Findings of Peripheral Primitive Neuroectodermal Tumors ( Report of Thirty-One Cases)

    Institute of Scientific and Technical Information of China (English)

    董天明; 娄昕; 侯俊; 安宁豫

    2011-01-01

    Objective To analyze the imaging findings of peripheral primitive neuroectodermal tumors ( pPNET). Materials and Methods The clinical and imaging data of 31 cases (13 cases located in abdominal and pelvis cavity, 12 cases in bone and muscle,6 cases in thoracic region) with pathologically proved pPNETs were analyzed retrospectively. The imaging data included X-ray (4 cases) , CT ( 21 cases) and MRI ( 19 cases). Results The tumors mostly appeared ill -defined soft tissue mass (the maximum diameter of 3 - 19cm, average 8. 8cm) with complicated internal components, including necrosis( 18/30) , septation (13/30) , cystic change ( 12/30) , hemorrhage (4/30) , and calcification (4/21 ). On CT, pPNET were presented as isodensity or sligt low density relative to muscle. On MR images, the kind of tumor often shows intermediate signal intensity on T1 -weighted images and hyperintense signal on T2 -weighted and STIR sequences. The tumors showed variable enhancement types on post ontrast images. Slight to moderate and inhomogeneous delay enhancement was the most common pattern. There was relationship between inhomogeneous degree of enhancement and tumor size. Local invasion (15/30) and distant metastases(8/30) were often detected when the primary tumor was found. Conclusion Peripheral primitive neuroectodermal tumors (pPNETs) tend to be large and aggressive. Although the imaging and clinical appearances is nonspecific, these tumors should be considered in the differential diagnosis when a large mass with aggressive features and heterogenous density or signal.%目的 探讨外周性原始神经外胚层肿瘤(peripheral primitive neuroectodermal tumors,pPNET)的影像学表现.资料与方法 回顾性分析31例(腹盆腔13例,骨及软组织12例及胸部6例)经病理证实的pPNET患者的临床及影像学特点.其中普通X线检查4例,CT检查21例,MRI检查19例.结果 影像学结果表明pPNET多表现为较为实体软组织肿块(最大径长约3~19 cm,平均8

  15. 肺原发原始神经外胚叶肿瘤的临床病理观察%Clinical and pathological observation on primary pulmonary primitive neuroectodermal tumor

    Institute of Scientific and Technical Information of China (English)

    彭李博; 魏雪; 时姗姗; 周晓军; 吴波

    2015-01-01

    Objective Primitive neuroectodermal tumor (PNET) is a rare malignant small round cell tumor .This paper aimed to study the clinical and pathological features of primary pulmonary primitive neuroectodermal tumor . Methods We collected 2 cases of primary pulmonary PNET to review the clinical and pathological features .Immunohistochemical staining was used to detect immune mark-ers, and fluorescence in situ hybridization (FISH) was applied to detect EWS translocation. Results 2 patients were aged 33 years and 17 years.Microscopically, the tumor cell was composed of single small round cells in diffusion or in distribution of sheets or beams , with scant cytoplasm , oval or spindle-shaped nucleus , high mitotic count .Irregular tumor necrosis scattered in the tumor along with visi-ble rosette structure.Immunohistochemical study showed that the tumor cells were positive for CD 99, FLI-1 and Syn, while CKpan, EMA, Desmin, CgA, TTF1, CD34 were negative.EWS/FLI1 translocations were detected positive in both the cases .2 patients died 7 months and 32 months after operation , respectively . Conclusion Primary pulmonary PNET is rare , so the selection of appropriate im-mune markers (CD99, FLI-1, Syn) and FISH for the detection of EWS translocation helps to improve the accuracy of diagnosis .%目的:原始神经外胚叶肿瘤( primitive neuroectodermal tumour , PNET)是一种少见的恶性小圆细胞肿瘤,文中探讨肺原发原始神经外胚叶肿瘤的临床病理特征。方法收集2例原发于肺的PNET患者资料,分析其临床病理特点,采用免疫组化检测相关免疫标志物,并通过荧光原位杂交( fluorescent in situ hybridization , FISH)检测EWS基因易位情况。结果2例患者年龄分别为33岁和17岁,显微镜下肿瘤细胞为形态单一的小圆细胞,弥漫或片状、梁状分布,细胞质少,核呈椭圆形或短梭形,核分裂象易见,肿瘤内散在不规则坏死灶,可见菊形团结构

  16. Misdiagnosis induced intraocular lens dislocation in anterior megalophthalmos

    Institute of Scientific and Technical Information of China (English)

    WANG Qi-wei; XU Wen; ZHU Ya-nan; LI Jin-yu; ZHANG Li; YAO Ke

    2012-01-01

    Anterior megalophthalmos (AM) is an uncommon developmental anomaly of the anterior segment of the eye with a constellation of findings that includes enlarged cornea,deep anterior chamber,posterior positioning of the iris and lens,iris stroma atrophy,hypoplasia of iris dilator,pupil displacement,large capsular bag,lens subluxation,prematurely cataract and the tendency to retinal detachment.AM,especially when symptoms are mild,is not an easy disease to diagnose.We present 3 AM cases that were misdiagnosed as congenital cataract with weak zonule and megalocornea.Intraocular lenses (IOLs) dislocated after standard cataract surgeries and subsequent surgery (replacing the dislocated IOLs with iris-claw intraocular lenses) achieved satisfactory outcome.Although rare,AM should be included in the differential diagnosis of enlarged cornea and we recommend implanting Artisan lens in AM patients.

  17. Unilateral persistent fetal vasculature coexisting with anterior segment dysgenesis.

    Science.gov (United States)

    Khokhar, Sudarshan; Gupta, Shikha; Arora, Tarun; Gogia, Varun; Dada, Tanuj

    2013-08-01

    Persistent fetal vasculature (PFV) is a common congenital developmental anomaly of the eye which results from failure of the embryological primary vitreous and hyaloid vasculature to regress by the time of birth (Int Ophthalmol Clin 48: 53-62, 2008). Typically, it is divided into anterior, posterior or combined types and is characterized by the presence of a vascular stalk located between the optic disc and the posterior lens capsule (Int Ophthalmol Clin 48: 53-62, 2008). Although it has been reported to manifest itself differently, in our case it presented in a microphthalmic eye as anterior segment dysgenesis with broad-based mid-peripheral synechiae, posterior embryotoxon, iridoschisis, ectropion uveae, hypotony and subluxated cataractous lens with a taut anterior hyaloid face which are rare associations with PFV.

  18. Anterior Segment Imaging in Ocular Surface Squamous Neoplasia

    Directory of Open Access Journals (Sweden)

    Sally S. Ong

    2016-01-01

    Full Text Available Recent advances in anterior segment imaging have transformed the way ocular surface squamous neoplasia (OSSN is diagnosed and monitored. Ultrasound biomicroscopy (UBM has been reported to be useful primarily in the assessment of intraocular invasion and metastasis. In vivo confocal microscopy (IVCM shows enlarged and irregular nuclei with hyperreflective cells in OSSN lesions and this has been found to correlate with histopathology findings. Anterior segment optical coherence tomography (AS-OCT demonstrates thickened hyperreflective epithelium with an abrupt transition between abnormal and normal epithelium in OSSN lesions and this has also been shown to mimic histopathology findings. Although there are limitations to each of these imaging modalities, they can be useful adjunctive tools in the diagnosis of OSSN and could greatly assist the clinician in the management of OSSN patients. Nevertheless, anterior segment imaging has not replaced histopathology’s role as the gold standard in confirming diagnosis.

  19. Complex aesthetic treatment on anterior maxillary teeth with malposition

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    Febriastuti Febriastuti

    2008-12-01

    Full Text Available Background: Complex aesthetic treatment on anterior teeth involves more than one caries tooth with malformed shape and malposition. Purpose: The purpose of this paper is to find the alternative treatment for anterior maxillary teeth with malposition. Case: In this case, a 25 year-old man with a peg shaped teeth and caries on several teeth and malposition can be treated with complex aesthetic treatment. Case management: Endodontic pulpectomy treatment on anterior maxillary teeth and post construction with splint porcelain fused to metal crowns on 11, 12, and 21, 22 to correct the shape and position into normal position. Conclusion: Malformed and malpositioned teeth with caries can be treated with complex aesthetic treatment.

  20. Compartment syndrome with mononeuropathies after anterior cruciate ligament reconstruction.

    Science.gov (United States)

    Kindle, Brett J; Murthy, Naveen; Stolp, Kathryn

    2015-05-01

    Compartment syndrome rarely follows anterior cruciate ligament reconstruction. However, when it does, it may result in mononeuropathies that are amenable to neurolysis. The authors of this study present an 18-yr-old woman who sustained a right anterior cruciate ligament tear and underwent uneventful anterior cruciate ligament reconstruction using femoral and popliteal nerve blocks. Postoperatively, she developed compartment syndrome requiring emergent fasciotomies. At 11 wks after fasciotomy, results of electrophysiologic tests showed evidence of severe fibular and tibial neuropathies. Magnetic resonance images showed extensive tricompartmental myonecrosis. Fibular and tibial neurolysis as well as decompression were performed, followed by intensive outpatient rehabilitation. At the 6-mo follow-up, she reported resolution of pain as well as significant improvement in sensation, strength, and function. Early recognition and intervention are crucial to prevent serious neurologic damage. Excessive tourniquet pressure and anesthetic nerve blocks may have been responsible.

  1. Dysphagia Secondary to Anterior Osteophytes of the Cervical Spine.

    Science.gov (United States)

    Egerter, Alexander C; Kim, Eric S; Lee, Darrin J; Liu, Jonathan J; Cadena, Gilbert; Panchal, Ripul R; Kim, Kee D

    2015-10-01

    Study Design Retrospective case series. Objective Diffuse idiopathic skeletal hyperostosis (DISH) or Forestier disease involves hyperostosis of the spinal column. Hyperostosis involving the anterior margin of the cervical vertebrae can cause dysphonia, dyspnea, and/or dysphagia. However, the natural history pertaining to the risk factors remain unknown. We present the surgical management of two cases of dysphagia secondary to cervical hyperostosis and discuss the etiology and management of DISH based on the literature review. Methods This is a retrospective review of two patients with DISH and anterior cervical osteophytes. We reviewed the preoperative and postoperative images and clinical history. Results Two patients underwent anterior cervical osteophytectomies due to severe dysphagia. At more than a year follow-up, both patients noted improvement in swallowing as well as their associated pain. Conclusion The surgical removal of cervical osteophytes can be highly successful in treating dysphagia if refractory to prolonged conservative therapy.

  2. Perforation forces of the intact porcine anterior lens capsule.

    Science.gov (United States)

    Ullrich, Franziska; Lussi, Jonas; Felekis, Dimitrios; Michels, Stephan; Petruska, Andrew J; Nelson, Bradley J

    2016-09-01

    During the first step of cataract surgery, the lens capsule is perforated and a circular hole is created with a sharp instrument, a procedure called capsulorhexis. To develop automated systems that can assist ophthalmologists during capsulorhexis, the forces required must be quantified. This study investigates perforation forces of the central anterior lens capsule in porcine eyes, which are used as a conservative model for the human eye. A micro-mechanical characterisation method is presented that measures capsular bag perforation forces with a high precision positioning and high-resolution force sensing system. The force during perforation of the anterior lens capsule was measured with various sized needles and indentation speeds and is found to be 15-35mN. A bio-mechanical model is identified that describes an exponential correlation between indentation force and depth, indicating strain hardening behaviour of the porcine anterior lens capsule.

  3. The significance of biometric parameters in determining anterior teeth width

    Directory of Open Access Journals (Sweden)

    Strajnić Ljiljana

    2013-01-01

    Full Text Available Background/Aim. An important element of prosthetic treatment of edentulous patients is selecting the size of anterior artificial teeth that will restore the natural harmony of one’s dentolabial structure as well as the whole face. The main objective of this study was to determine the correlation between the inner canthal distance (ICD and interalar width (IAW on one side and the width of both central incisors (CIW, the width of central and lateral incisors (CLIW, the width of anterior teeth (ATW, the width between the canine cusps (CCW, which may be useful in clinical practice. Methods. A total of 89 subjects comprising 23 male and 66 female were studied. Their age ranged from 19 to 34 years with the mean of 25 years. Only the subjects with the preserved natural dentition were included in the sample. All facial and intraoral tooth measurements were made with a Boley Gauge (Buffalo Dental Manufacturing Co., Brooklyn NY, USA having a resolution of 0.1mm. Results. A moderate correlation was established between the interalar width and combined width of anterior teeth and canine cusp width (r = 0.439, r = 0.374. A low correlation was established between the inner canthal distance and the width of anterior teeth and canine cusp width (r = 0.335, r = 0.303. The differences between the two genders were highly significant for all the parameters (p < 0.01. The measured facial distances and width of anterior teeth were higher in men than in women. Conclusion. The results of this study suggest that the examined interalar width and inner canthal distance cannot be considered reliable guidelines in the selection of artificial upper anterior teeth. However, they may be used as a useful additional factor combined with other methods for objective tooth selection. The final decision should be made while working on dentures fitting models with the patient’s consent.

  4. Refraction and eye anterior segment parameters in schizophrenic patients

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    Tongabay Cumurcu

    2015-06-01

    Full Text Available ABSTRACT Purpose: To evaluate the difference in terms of refractive errors and anterior segment parameters between schizophrenic patients and healthy volunteers. Methods: This study compared 70 patients (48 men who were diagnosed with schizophrenia with a control group of 60 (35 men who were similar in terms of age, gender, education, and socioeconomic level. Anterior segment examination was performed using a Scheimflug system. Axial length and lens thickness (LT were measured using optic biometry. The following tests were administered to the psychiatric patient group: Brief Psychiatric Rating Scale (BPRS, Scale for the Assessment of Negative Symptoms (SANS, and Scale for the Assessment of Positive Symptoms (SAPS. Results: Mild myopia was detected in both the schizophrenic and control groups, with no statistically significant difference (p>0.005. Corneal volume (CV, anterior chamber volume (ACV, anterior chamber depth (ACD, and central corneal thickness (CCT values were lower in the schizophrenic group, and there was a statistically significant between-group difference (p=0.026, p=0.014, p=0.048, and p=0.005, respectively. LT was greater in schizophrenics, and the difference was found to be statistically significant (p=0.006. A statistically significant negative correlation was found between SAPS and cylinder values (p=0.008. The axial eye length, cylinder value, pupil diameter, mean keratometric value, and anterior chamber angle revealed no statistically significant difference between the groups (p>0.05. Conclusion: No statistically significant difference was detected in terms of refraction disorders between schizophrenics and the healthy control group, while some differences in anterior chamber parameters were present. These results demonstrate that schizophrenics may exhibit clinical and structural differences in the eye.

  5. Sandwich-like Reconstruction of Anterior Skull Base Defects

    Institute of Scientific and Technical Information of China (English)

    Wang Zheng-min; Wang De-hui

    2001-01-01

    Objective: To evaluate the safety and efficacy of new modality of anterior skull base repair,namely sandwich-like reconstruction of anterior skull base defects. Methods: A retrospective analysis of patients who underwent transcranial or transcranial-facial resections of malignant or benign aggressive tumors involving the anterior skull base was conducted in our department. We used the sandwich-like reconstruction, using pedicled pericranial flap, frontal muscle flap and free abdominal adipose tissue between them, to separate of cranial cavity and aerodigest tract and keep the frontal lobes in place following resections of anterior skull base tumors. Results: From October, 1984 to October, 1998, 116 patients underwent transcranial or transcranialfacial approach for the resection of malignant or aggressive benign tumor, and sandwich-like repairs were performed for the anterior skull base defect. 54 (46.6 % ) patients had previous operation, with a maximum of 5 surgeries. The average age of patients was 35.9 years old, ranging form 6 to 73 years old. Forty-eight (41.4%)patients had malignant neoplasmas, and sixty-eight (58.6%) patients had benign aggressive tumors. In our series, with the maximal follow-ups for as long as 14 years, NO one had early failure of the one-stage reconstruction. CSF fluid leakage was not encountered, nor was ascending bacterial meningitis observed. No immediate or delayed prolapse of dura or frontal lobes was observed. Conclusion: We conclude that the sandwich-like reconstruction, using pericranial flap, frontal muscle flap and free abdominal adipose between them, is an extremely safe and effective procedure for the repair of skull base defect, even when tumor extensively involves anterior skull base.

  6. Sandwich-like Reconstruction of Anterior Skull Base Defects

    Institute of Scientific and Technical Information of China (English)

    WangZheng-min,MD; WangDe-hui,MD

    2001-01-01

    Objective:To evaluate the safety and efficacy of new modality of anterior skull base repair,namely sandwich-like reconstruction of anterior skull base defects. Methods : A retrospective analysis of patients who underwent wanscranial or wanscranial-facial resections of malignant or benign aggressive tumors involving the anterior skull base was conducted in our department. We used the sandwich-like reconstruction, using pedicled pericranial flap, frontal muscle flap and free abdominal adipose tissue between them, to separate of cranial cavity and aerodigest tract and keep the frontal lobes in place following resections of anterior skull base tumors. Results: From October, 1984 to October, 1998, 116 patients underwent tmnscranial or tmnscranial-facial approach for the resection of malignant or aggressive benign tumor, and sandwich-like repairs were performed for the anterior skull base defect.54 (46.6%) patients had previous operation, with a maximum of 5 surgeries. The average age of patients was 35.9 years old, ranging form 6 to 73 years old. Forty-eight (41.4%) patients had malignant neoplasmas, and sixty-eight (58.6%) patients had benign aggressive tumors. In our series, with the maximal follow-ups for as long as 14 years, NO one had early failure of the one-stage reconstruction. CSF fluid leakage was not encountered, nor was ascending bacterial meningitis observed. No immediate or delayed prolapse of dura or frontal lobes was observed. Conclusion: We conclude that the sandwich-like reconstruction, using pericranial flap, frontal muscle flap and free abdominal adipose between them, is an extremely safe and effective procedure for the repair of skull base defect, even when tumor extensively involves anterior skull base.

  7. Role of Optical Coherence Tomography in Assessing Anterior Chamber Angles

    Science.gov (United States)

    Kochupurakal, Reema Thomas; Jha, Kirti Nath; Rajalakshmi, A.R.; Nagarajan, Swathi; Ezhumalai, G.

    2016-01-01

    Introduction Gonioscopy is the gold standard in assessing anterior chamber angles. However, interobserver variations are common and there is a need for reliable objective method of assessment. Aim To compare the anterior chamber angle by gonioscopy and Spectral Domain Optical Coherence Tomography (SD-OCT) in individuals with shallow anterior chamber. Materials and Methods This comparative observational study was conducted in a rural tertiary multi-speciality teaching hospital. A total of 101 eyes of 54 patients with shallow anterior chamber on slit lamp evaluation were included. Anterior chamber angle was graded by gonioscopy using the shaffer grading system. Angles were also assessed by SD-OCT with Trabecular Iris Angle (TIA) and Angle Opening Distance (AOD). Chi-square test, sensitivity, specificity, positive and negative predictive value to find correlation between OCT parameters and gonioscopy grading. Results Females represented 72.7%. The mean age was 53.93 ±8.24 years and mean anterior chamber depth was 2.47 ± 0.152 mm. Shaffer grade ≤ 2 were identified in 95(94%) superior, 42(41.5%) inferior, 65(64.3%) nasal and 57(56.4%) temporal quadrants. Cut-off values of TIA ≤ 22° and AOD ≤ 290 μm were taken as narrow angles on SD-OCT. TIA of ≤ 22° were found in 88(92.6%) nasal and 87(87%) temporal angles. AOD of ≤ 290 μm was found in 73(76.8%) nasal and 83(83%) temporal quadrants. Sensitivity in detecting narrow angles was 90.7% and 82.2% for TIA and AOD, while specificity was 11.7% and 23.4%, respectively. Conclusion Individuals were found to have narrow angles more with SD-OCT. Sensitivity was high and specificity was low in detecting narrow angles compared to gonioscopy, making it an unreliable tool for screening. PMID:27190851

  8. Component rotation and anterior knee pain after total knee arthroplasty.

    Science.gov (United States)

    Barrack, R L; Schrader, T; Bertot, A J; Wolfe, M W; Myers, L

    2001-11-01

    All patients undergoing cruciate-retaining primary total knee arthroplasty for degenerative osteoarthritis at one center were studied prospectively. Clinical and radiographic followup was obtained at a minimum 5 years in 102 knees in 73 patients. Patients were asked specifically about the presence of the pain in the anterior aspect of the knee in the vicinity of the patella and rated the severity of the pain on a visual analog scale. Significant anterior knee pain rating at least 3 of 10 on the visual analog scale was present in 16 knees (13 patients). Eleven patients with 14 symptomatic knees agreed to undergo computed tomography scanning to accurately determine the rotation of the tibial and femoral components. The epicondylar axis and tibial tubercle were used as references using a previously validated technique. A control group of 11 asymptomatic patients (14 knees), matched for age, gender, and length of followup also underwent computed tomography scanning. All patients in both groups had normal axial alignment. There was a highly significant difference in tibial component rotation between the two groups with the patients with anterior knee pain averaging 6.2 degrees internal rotation compared with 0.4 degrees external rotation in the control group. There also was a significant difference in combined component rotation with the patients with anterior knee pain averaging 4.7 degrees internal rotation compared with 2.6 degrees external rotation in the control group. There was no significant difference in the degree of radiographic patellar tilt or patellar subluxation between the two groups. Patients with combined component internal rotation were more than five times as likely to experience anterior knee pain after total knee arthroplasty compared with those with combined component external rotation. Component malrotation is a significant factor in the development of anterior knee pain after total knee arthroplasty.

  9. Anterior mediastinal synovial sarcoma: A case report and literature review

    Directory of Open Access Journals (Sweden)

    Wen-xiang YUE

    2015-01-01

    Full Text Available Objective To study the clinical manifestations, pathologic features, diagnosis, treatment and prognosis of primary synovial sarcoma in the anterior mediastinum. Methods A case of primary synovial sarcoma in the anterior mediastinum was reported. Clinical features, imaging manifestations, pathology features and therapeutic effect were analysed and the relevant literature was reviewed. Results A 48-year-male patient was admitted with complaint of right chest pain for 4 days. Chest computerized tomography revealed a large mass located at the right anterior mediastinum, and it was primarily diagnosed as invasive thymoma. Pathological examination by CT-guided percutaneous needle biopsy manifested that, under microscope, the tumor cells were short and spindle in shape forming a nest structure, suggested it was a thymoma. The patient then underwent resection of thymoma with removal of fat and connective tissue in the anterior mediastinum. During the operation the size of the tumor was 15cm×15cm×10cm, being located at the anterior mediastinum, and it tended to bleed. The diagnosis of primary monophasic synovial sarcoma in the mediastinum was confirmed by postoperative/pathology examination. Immunohistochemistry staining showed that the tumor cells were positive for the markers Bcl-2 and EMA, but negative for the markers CK (pan and S100. The patient suffered from local recurrence with metastases to lung 4 months after surgery. The patient received 2 chemotherapeutic courses with ifosfamide, epirubicin and cisplatin. He died 6 months after surgery. Conclusion Primary synovial sarcoma in the anterior mediastinum is an extremely rare and highly malignant tumor with poor prognosis. The diagnosis depends on the pathological features, immunohistochemistry and RT-PCR. Radical resection combined with comprehensive treatment may improve the survival rate. DOI: 10.11855/j.issn.0577-7402.2014.12.12

  10. The diagnosis and treatment analysis for primitive neuroectodermal tumor of the adrenal gland:2 cases report and literature review%肾上腺原始神经外胚层肿瘤二例诊治分析并文献复习

    Institute of Scientific and Technical Information of China (English)

    裴再军; 付海英

    2013-01-01

    Objective To explore the diagnosis and treatment for primitive neuroectodermal tumor of the adrenal gland.Methods The clinical data of 2 patients with primitive neuroectodermal tumor of the adrenal gland were retrospectively analyzed.Literatures about diagnosis and treatment of primitive neuroectodermal tumor arising from the adrenal gland were also reviewed.Results B type ultrasonography and computer tomography scans showed a cystic and solid soft tissue masses about 3 cm (patient 1) and 15 cm (patient 2) in maximum diameter with rough border.Surgeries were performed successfully in 2 patients.The diagnosis of primitive neuroectodermal tumor arising from the adrenal gland was confirmed by histopathology and immunohistochemistry.During follow up,evidences of tumor recurrence were not found in the first patient.However,the second patient had diffuse retroperitoneal lymph node metastasis 3 months after surgery.Then,the patient died 6 months after surgery.Conclusion The primitive neuroectodermal tumor of the adrenal gland is extremely rare with highly malignant and poor prognosis.Its diagnosis is based on histopathology and immunohistochemistry.Multimodal therapeutic regimens including surgery,combination with chemotherapy and radiotherapy were recommended.%目的 探讨国内外肾上腺原始神经外胚层肿瘤(PNET)的诊断和治疗.方法 回顾性分析桐乡市中医医院诊治的2例肾上腺PNET患者的临床资料,并结合相关国内外文献探讨其诊断和治疗方法.结果 B超、CT检查示两例患者肾上腺区边界不清的囊实性软组织肿块,最大直径分别约3 cm和15 cm.2例患者均行手术治疗.术后病理及免疫组化确诊为肾上腺PNET.患者1尚无肿瘤复发证据.患者2随访3个月后发现腹膜后广泛淋巴结转移.6个月后死亡.结论 肾上腺PNET临床罕见,恶性程度高且预后不良,确诊主要依靠病理和免疫组化,治疗提倡多手段综合治疗.

  11. Anterior hippocampus: the anatomy of perception, imagination and episodic memory

    Science.gov (United States)

    Zeidman, Peter; Maguire, Eleanor A.

    2017-01-01

    The brain creates a model of the world around us. We can use this representation to perceive and comprehend what we see at any given moment, but also to vividly re-experience scenes from our past and imagine future (or even fanciful) scenarios. Recent work has shown that these cognitive functions — perception, imagination and recall of scenes and events — all engage the anterior hippocampus. Here we capitalise on new findings from functional neuroimaging to propose a model that links high-level cognitive functions to specific structures within the anterior hippocampus. PMID:26865022

  12. Anterior mediastinal paraganglioma: A case for preoperative embolization

    Directory of Open Access Journals (Sweden)

    Shakir Murtaza

    2012-07-01

    Full Text Available Abstract Background Paraganglioma is a rare but highly vascular tumor of the anterior mediastinum. Surgical resection is a challenge owing to the close proximity to vital structures including the heart, trachea and great vessels. Preoperative embolization has been reported once to facilitate surgical treatment. Case presentation We report a case of anterior mediastinal paraganglioma that was embolized preoperatively, and was resected without the need for cardiopulmonary bypass and without major bleeding complications. Conclusion We make a case to further the role of preoperative embolization in the treatment of mediastinal paragangliomas.

  13. Brain stimulation for intractable epilepsy: Anterior thalamus and responsive stimulation

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    Vibhor Krishna

    2014-01-01

    Full Text Available Despite medications, resective surgery, and vagal nerve stimulation, some patients with epilepsy continue to have seizures. In these patients, other approaches are urgently needed. The biological basis of stimulation of anterior thalamic nucleus and epileptogenic focus is presented. Results from two large randomized controlled trials Stimulation of Anterior Nucleus of Thalamus for Epilepsy (SANTE and Neuropace pivotal trial are discussed. Neuromodulation provides effective treatment for a select group of refractory epilepsy patients. Future investigations into the mechanism underlying ′response′ to brain stimulation are desired.

  14. Correction of anterior open bite in a case of achondroplasia

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    Karpagam S

    2005-01-01

    Full Text Available Treatment planning for patients with skeletal deformities is often considered challenging. This article reports a female patient with achondroplasia who presented with severe maxillary retrognathism and vertical excess along with anterior open bite. The clinical and cephalometric findings of the patient are detailed here. The treatment plan consisted of modified anterior maxillary osteotomy for simultaneous vertical and sagittal augmentation along with orthodontic intervention. The course of surgical-orthodontic treatment and the results are presented. This treatment is to be followed by correction of vertical maxillary excess after completion of growth. This paper concludes that the dentoalveolar component of a skeletal deformity can be handled independent of the craniofacial management.

  15. Traumatic Anterior Dislocation of Hip in a Child- Case Report

    Directory of Open Access Journals (Sweden)

    S Ahmad

    2015-03-01

    Full Text Available Traumatic hip dislocation in children is relatively rare accounting for about 5% of all hip dislocations. Most of the hip dislocations seen in children are of the posterior type but the much rarer anterior and anterior-inferior (obturator types have also been described. We present the case of an eight years old girl with an obturator type of hip dislocation following trivial trauma. She was treated with closed reduction and immobilisation in skin traction for three weeks. She was followed up closely for one year and did not develop any complications during that period.

  16. Anterior internal lenticonus accompanied by congenital nuclear cataract

    Institute of Scientific and Technical Information of China (English)

    LIU Zhe; SUN Chuan-bin; YAO Ke

    2011-01-01

    Internal lenticonus is a very rare morphologic abnormality of crystalline lens which has been reported in only several cases in the literature.We herein reported the clinical characteristics and surgical findings of the anterior internal lenticonus accompanied by congenital nuclear cataract.Cataract extraction accompanied with intraocular lens implantation was uneventfully performed,and a good visual outcome was achieved in this case.Viral infection during embryonal and fetal period might account for the formation of the anterior internal lenticonus and congenital nuclear cataract in our case.

  17. Usurering af osteosyntesemateriale gennem øsofagus efter anterior cervikalkirurgi

    DEFF Research Database (Denmark)

    Wiis, Julie Therese; Nittby, Henrietta Carolina; Lauritsen, Anne Oberg

    2014-01-01

    The rare, potentially life-threatening complication to anterior cervical surgery, oesophageal perforation, occurs after surgical trauma or due to erosion by migrating hardware. Symptoms are hoarseness, dysphagia, neck/throat pain, subcutaneous emphysema and fever. Imaging and endoscopic diagnosis...... can give false negative results. We present a case of a 74-year-old male, who was readmitted with sepsis and abscess in the operation area three weeks after anterior cervical surgery. Veillonella parvula was found in the abscess material and computed tomography confirmed the diagnosis of oesophageal...

  18. Hypermethylation of TIG1 promoter in intracranial primitive neuroectodermal tumors%颅内原始神经外胚叶肿瘤中TIG1启动子的高甲基化

    Institute of Scientific and Technical Information of China (English)

    常青; 吴浩强

    2006-01-01

    目的 检测颅内原始神经外胚叶肿瘤(primitive neuroectodermal tumor, PNET)中TIG1(tazarotene induced gene-1)基因启动子的甲基化状态.方法 使用甲基化特异PCR(MSP)法检测25例原发髓母细胞瘤(medulloblastoma,MB)、9例原发幕上原始神经外胚叶肿瘤(supratentorial primitive neuroectodermal tumor,SPNET) 和7株PNET细胞系中TIG1的甲基化水平.用RT-PCR方法检测PNET细胞中TIG1的转录水平.并用去甲基化试剂处理PNET细胞,观察基因转录水平与甲基化状态间的关系.结果 原发MB及SPNET中TIG1启动子的异常甲基化率分别为40%(10/25)和44%(4/9).该基因在PNET中的甲基化异常是肿瘤特异性的.PNET细胞系中均有TIG1高甲基化,并伴有转录水平的表达减少或丢失(7/7).TIG1的表达水平与启动子甲基化状态呈明显负相关.并且,这种相关性得到去甲基化试剂处理实验的支持.经去甲基化试剂5-aza-2′-deoxycytidine 处理的全部细胞系中TIG1的表达都得到了恢复.结论 大部分PNET细胞系中都有TIG1表达的减少和缺失,且该基因缺失与启动子高甲基化密切相关.同时,TIG1的高甲基化率在原发PNET中也能检测到,提示TIG1可能在颅内PNET的肿瘤发生中起重要作用.

  19. Peripheral Primitive Neuroectodermal Tumors of Submaxillary Gland:Report of 1 Case and Review of Literature%颌下腺外周原始神经外胚层肿瘤1例并文献复习

    Institute of Scientific and Technical Information of China (English)

    傅建军

    2012-01-01

    Objective To investigate the clinicopathological features and the diagnosis and treatment of peripheral primitive neuroectodermal tumor (pPNET) of submandibular gland. Methods A case of submaxillary gland pPNET was analyzed by using light microscopy and im-munohistochemical staining. The relevant literature was reviewed. Results The tumor was located in the left medial submandibular gland. Tumor cells showed patchy diffuse distribution, and were identified as single small round cells with less cytoplasm and round nuclei. Immunohisto-chemistry displayed positive expression of CD99, Syn and Vim. Conclusion The pPNET,a rare malignant peripheral nerve tumor of primitive origin with high invasiveness and poor prognosis, u-sually occurs in deep soft tissues and is extremely rare in submandibular gland. Immunohisto-chemical staining is helpful in the diagnosis of pPNET and surgical excision combined with chemotherapy or radiotherapy is the initial treatment for pPNET.%目的 探讨颌下腺外周原始神经外胚层肿瘤(peripheral primitive neuroectodermal tumor,pPNET) 的临床、病理组织学特征及诊治方法.方法 通过光镜及免疫组织化学染色分析1 例颌下腺原发的pPNET,同时复习相关文献.结果 肿瘤位于左侧颌下腺内侧,肿瘤细胞呈片状弥漫分布,为单一的小圆形细胞,胞质少,核圆.免疫组织化学示CD99、Syn 和Vim均为阳性(+).结论 pPNET 是一种少见的起源于原始神经外胚层的恶性肿瘤,好发于深部软组织,极罕见于颌下腺,具有高度侵袭性,预后差;免疫组织化学有助于pPNET的诊断;目前治疗主要是手术切除加化、放疗.

  20. Proprioception in anterior cruciate ligament deficient knees and its relevance in anterior cruciate ligament reconstruction

    Directory of Open Access Journals (Sweden)

    Mandeep S Dhillon

    2011-01-01

    Full Text Available Injury to the anterior cruciate ligament (ACL not only causes mechanical instability but also leads to a functional deficit in the form of diminished proprioception of the knee joint. "Functional" recovery is often incomplete even after "anatomic" arthroscopic ACL reconstruction, as some patients with a clinically satisfactory repair and good ligament tension continue to complain of a feeling of instability and giving way, although the knee does not sublux on clinical testing. Factors that may play a role could be proprioceptive elements, as the intact ACL has been shown to have significant receptors. Significant data have come to light demonstrating proprioceptive differences between normal and injured knees, and often between injured and reconstructed knees. ACL remnants have been shown to have proprioceptive fibers that could enhance functional recovery if they adhere to or grow into the reconstructed ligament. Conventionally the torn remnants are shaved off from the knee before graft insertion; modern surgical techniques, with remnant sparing methods have shown better outcomes and functional recovery, and this could be an avenue for future research and development. This article analyzes and reviews our understanding of the sensory element of ACL deficiency, with specific reference to proprioception as an important component of functional knee stability. The types of mechanoreceptors, their distribution and presence in ACL remnants is reviewed, and suggestions are made to minimize soft tissue shaving during ACL reconstruction to ensure a better functional outcome in the reconstructed knee.

  1. Anterior cruciate ligament: 3-D fiber anatomy, fluorescence arthroscopy & healing

    NARCIS (Netherlands)

    Nguyen, D.T.

    2015-01-01

    One of the current emphases in optimizing anterior cruciate ligament (ACL) reconstructions is closer mimicking the anatomy of the ACL. The aim of Chapter 2 and Chapter 3 is to develop and validate a methodology to quantify the 3-D collagen fiber orientation of ligaments, accurately and at a high res

  2. Anterior Colporrhaphy Technique and Approach Choices: Turkey Evaluation

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    Serdar Aydın

    2016-09-01

    Full Text Available Aim: To evaluate the diversity in techniques and approaches for anterior colporrhaphy among operators in Turkey. Methods: A survey evaluating the preoperative examination, technique of anterior colporrhaphy, operation choice and postoperative care was presented to surgeons. We contacted via directly, mail or telephone. We used 28 item questionnaire. Results: Majority (87.9% was composed of young gynecologists. Urologists composed of the 9.5% of the study population. The rate of paravaginal defect evaluation was 75.9% and mostly by inspection the presence of vaginal rugae. The use of transperineal 3D pelvic floor ultrasonography was low (5.7%. The evaluation of levator ani muscle defect was 46.6%. The usage of the transperineal 3D ultrasonography for levator ani muscle defect was 19 percent of operators. There were diversity in use of hydrodissection, fascial plication, excision of vaginal mucosa and suture choice. Usage of mesh for anterior colporrhaphy was limited (17.8% and mostly in recurrent cases (12.2%. Paravaginal defect repair rate was 31.9%. The urinary catheter was generally removed one or two day after operation. Vaginal pack usually removed 24 hours after. Conclusion: Several techniques and approaches for anterior vaginal wall repair among operators in Turkey. The variety of techniques suggested that there is no consensus on best surgical technique.

  3. Complications of the anterior approach to the cervical spine

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    Marcelo Lemos Vieira da Cunha

    2014-09-01

    Full Text Available OBJECTIVE: To evaluate the complications of anterior approach to the cervical spine in patients who underwent cervical arthrodesis with instrumentation. METHODS: Prospective and descriptive study was conducted from January 2009 to April 2010. All patients who underwent arthrodesis of the cervical spine by anterior approach were included, regardless the diagnosis. Access was made by the anterior approach on the right side. We evaluated the number of operated levels (1, 2 or 3 levels and, the type of procedure performed: discectomy and placement of cage and plate (D+C+P, discectomy with placement of a cage (D+C or corpectomy with placement of cage and plate (C+C+P. All complications related to surgical approach were reported. RESULTS: We studied 34 patients, 70% male. The average age was 50 years and mean follow-up was 8 months. Eighteen percent of patients had complications, distributed as follows: dysphasia (33% and dysphonic (67%. Among patients who developed complications, most underwent to D+C+P (83% and no complications were found in patients where no cervical plate was used. Regarding levels, both complications were identified in patients operated to one or two levels. However, in patients operated on three levels, only dysphonia was identified. CONCLUSION: The most frequent complication was dysphonia. Patients who presented more complications were those undergoing discectomy and fusion with cage and anterior cervical plate. All cases of dysphonia were in this group. The number of accessible levels does not seem to have affected the incidence of complications.

  4. ATP economy of force maintenance in human tibialis anterior muscle

    DEFF Research Database (Denmark)

    Nakagawa, Yoshinao; Ratkevicius, Aivaras; Mizuno, Masao

    2005-01-01

    PURPOSE: The aim of this study was investigate ATP economy of force maintenance in the human tibialis anterior muscle during 60 s of anaerobic voluntary contraction at 50% of maximum voluntary contraction (MVC). METHODS: ATP turnover rate was evaluated using P magnetic resonance spectroscopy (P...

  5. A case of anterior ischemic optic neuropathy associated with uveitis

    Directory of Open Access Journals (Sweden)

    Sugahara M

    2013-05-01

    Full Text Available Michitaka Sugahara, Takayuki Fujimoto, Kyoko Shidara, Kenji Inoue, Masato Wakakura Inouye Eye Hospital, Tokyo, Japan Introduction: Here, we describe a patient who presented with anterior ischemic optic neuropathy (AION and subsequently developed uveitis. Case: A 69-year-old man was referred to our hospital and initially presented with best-corrected visual acuities (BCVA of 20/40 (right eye and 20/1000 (left eye and relative afferent pupillary defect. Slit-lamp examination revealed no signs of ocular inflammation in either eye. Fundus examination revealed left-eye swelling and a pale superior optic disc, and Goldmann perimetry revealed left-eye inferior hemianopia. The patient was diagnosed with nonarteritic AION in the left eye. One week later, the patient returned to the hospital because of vision loss. The BCVA of the left eye was so poor that the patient could only count fingers. Slit-lamp examination revealed 1+ cells in the anterior chamber and the anterior vitreous in both eyes. Funduscopic examination revealed vasculitis and exudates in both eyes. The patient was diagnosed with bilateral panuveitis, and treatment with topical betamethasone was started. No other physical findings resulting from other autoimmune or infectious diseases were found. No additional treatments were administered, and optic disc edema in the left eye improved, and the retinal exudates disappeared in 3 months. The patient's BCVA improved after cataract surgery was performed. Conclusion: Panuveitis most likely manifests after the development of AION. Keywords: anterior ischemic optic neuropathy, uveitis

  6. Left Anterior Descending Artery-Pulmonary Artery Fistula

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    Turan Ege

    2011-12-01

    Full Text Available Despite the fact that coronary arteriovenous fistulas constitute approximately half (48% of coronary artery anomalies, they are rarely seen anomalies. In this report,we aim to present a coronary arteriovenous fistula case detected during a coronary angiography between left anterior descending artery and pulmonary artery.

  7. Anterior orbital varix presenting as a lacrimal sac mucocele.

    Science.gov (United States)

    Nasr, A M; Huaman, A M

    1998-05-01

    A 35-year-old woman was referred to the Oculoplastics Clinic because of a left nasal swelling and intermittent tearing of 1 year's duration. The clinical examination and echographic and radiologic findings were consistent with an anterior venous anomaly. Complete surgical excision was performed and histopathology confirmed the diagnosis of a varix.

  8. Anterior Cingulate Cortex in Schema Assimilation and Expression

    Science.gov (United States)

    Wang, Szu-Han; Tse, Dorothy; Morris, Richard G. M.

    2012-01-01

    In humans and in animals, mental schemas can store information within an associative framework that enables rapid and efficient assimilation of new information. Using a hippocampal-dependent paired-associate task, we now report that the anterior cingulate cortex is part of a neocortical network of schema storage with NMDA receptor-mediated…

  9. Computed tomography of the human developing anterior skull base

    NARCIS (Netherlands)

    J. van Loosen (J.); A.I.J. Klooswijk (A. I J); D. van Velzen (D.); C.D.A. Verwoerd (Carel)

    1990-01-01

    markdownabstractAbstract The ossification of the anterior skull base, especially the lamina cribrosa, has been studied by computed tomography and histopathology. Sixteen human fetuses, (referred to our laboratory for pathological examination after spontaneous abortion between 18 and 32 weeks of ge

  10. Predictive factors for anterior chamber fibrin formation after vitreoretinal surgery

    Directory of Open Access Journals (Sweden)

    Leonardo Provetti Cunha

    2014-04-01

    Full Text Available Purpose: The aim of this study was to investigate possible predictive factors related to anterior chamber fibrin formation after vitreoretinal surgery in a large series of patients. Methods: The data of 185 eyes of 185 patients submitted to vitreoretinal surgery was reviewed. The following variables were evaluated: the postoperatively presence of fibrin, age, diabetes mellitus, the vitrectomy system gauge (20, 23 or 25 gauge, the type of vitreous substitute, the influence of prior surgical procedures and the combination with cataract extraction. To evaluate predictive factors for anterior chamber fibrin formation, univariate analysis was performed. A multivariate stepwise logistic regression model was adjusted to investigate factors associated with fibrin formation (p<0.05. Results: Fibrinoid anterior chamber reaction was found in 12 (6.4% patients. For multivariate logistic regression analysis, balanced salt solution (BSS, the chance of fibrin occurrence was 5 times greater (odds ratio 4.83, CI 95% 1.302 - 17.892; p=0.019, while combination with phacoemulsification increased the chance of fibrin formation by 20 times (odds ratio 20, CI 95% 2.480 - 161.347; p=0.005. No significant difference was found regarding other variables. Conclusion: Anterior chamber fibrin formation is an unwanted complication after vitreoretinal surgery. Factors such as combined performance of phacoemulsification and the use of balanced salt solution as a vitreous substitute may predispose the occurrence of this complication.

  11. Rare times rare: The hyponatremia, rhabdomyolysis, anterior compartment syndrome sequence.

    Science.gov (United States)

    Dubin, Ina; Gelber, Moshe; Schattner, Ami

    2016-05-01

    Primary polydipsia occurs in up to 25% of patients with chronic psychiatric disorders (especially schizophrenia), related to the disease, its treatment or both. Urine output fails to match intake >10 L/day and water intoxication may develop. Rhabdomyolysis is a rare complication of hyponatremia, and an acute anterior compartment syndrome of the leg, an emergency, may be very rarely associated.

  12. Anterior cingulate cortex involvement in subclinical social anxiety.

    Science.gov (United States)

    Duval, Elizabeth R; Hale, Lisa R; Liberzon, Israel; Lepping, Rebecca; N Powell, Joshua; Filion, Diane L; Savage, Cary R

    2013-12-30

    We demonstrated differential activation in the anterior cingulate cortex (ACC) between subjects with high and low social anxiety in response to angry versus neutral faces. Activation in the ACC distinguished between facial expressions in the low, but not the high, anxious group. The ACC's role in threat processing is discussed.

  13. Clinical outcomes of surgical management of anterior bilateral mandibular fractures

    NARCIS (Netherlands)

    Boffano, P.; Gallesio, C.; Roccia, F.; van den Bergh, B.; Forouzanfar, T.

    2013-01-01

    Purpose: The aims of this study were to assess the clinical outcomes of patients with anterior bifocal mandibular fractures and to discuss the management of this peculiar type of trauma. Methods: From the systematic computer-assisted database that has continuously recorded patients hospitalized with

  14. Editorial Commentary: Anterior Cruciate Ligament Reconstruction: Auto or Allo?

    Science.gov (United States)

    Verma, Nikhil N

    2016-01-01

    Considerable controversy exists regarding appropriate graft choice for patients undergoing anterior cruciate ligament reconstruction. Allografts pretreated with high-dose irradiation should be avoided. Otherwise, multiple factors should be considered to individualize patient decision making, including patient age and activity level, graft type, and fixation type.

  15. Innovation in prediction planning for anterior open bite correction.

    Science.gov (United States)

    Almuzian, Mohammed; Almukhtar, Anas; O'Neil, Michael; Benington, Philip; Al Anezi, Thamer; Ayoub, Ashraf

    2015-05-01

    This study applies recent advances in 3D virtual imaging for application in the prediction planning of dentofacial deformities. Stereo-photogrammetry has been used to create virtual and physical models, which are creatively combined in planning the surgical correction of anterior open bite. The application of these novel methods is demonstrated through the surgical correction of a case.

  16. Value, search, persistence and model updating in anterior cingulate cortex

    NARCIS (Netherlands)

    Kolling, N.; Wittmann, M.K.; Behrens, T.E.J.; Boorman, E.D.; Mars, R.B.; Rushworth, M.F.S.

    2016-01-01

    Dorsal anterior cingulate cortex (dACC) carries a wealth of value-related information necessary for regulating behavioral flexibility and persistence. It signals error and reward events informing decisions about switching or staying with current behavior. During decision-making, it encodes the avera

  17. Absence of sensory function in the reconstructed anterior cruciate ligament

    DEFF Research Database (Denmark)

    Krogsgaard, Michael R; Fischer-Rasmussen, Torsten; Dyhre-Poulsen, Poul

    2011-01-01

    Cruciate ligaments provide sensory information that cause excitatory as well as inhibitory effects to the activity of the muscles around the knee. The aim of the study was to determine whether these muscular reflexes are reestablished after anterior cruciate ligament (ACL) re-construction. Wire e...

  18. Orthodontic and orthopaedic treatment for anterior open bite in children

    NARCIS (Netherlands)

    Lentini-Oliveira, D.; Carvalho, F. R.; Qingsong, Y.; Junjie, L.; Saconato, H.; Machado, M. A. C.; Prado, L. B. F.; Prado, G. F.

    2007-01-01

    Background Anterior open bite occurs when there is a lack of vertical overlap of the upper and lower incisors. The aetiology is multifactorial including: oral habits, unfavourable growth patterns, enlarged lymphatic tissue with mouth breathing. Several treatments have been proposed to correct this m

  19. Esthetic rehabilitation of crowded and protruded anterior dentition

    Directory of Open Access Journals (Sweden)

    Cecilia G. J. lunardhi

    2009-03-01

    Full Text Available Background: Recent trends put estheticrehabilitationas a demandingtreatmentin order to correct malpositionedanteriordentition. This is enhanced by the patient’s background, especially careers that require prime appearance for the public. Purpose: To describe that even though there are many treatment alternatives and procedures, esthetic rehabilitation on crowded and protruded anterior dentition using endodontic treatment, cast posts and all ceramic crowns, can improve patient’s appearance. Case: This article presents a case report on esthetic rehabilitation of crowded and protruded anterior dentition. Treatment was done due to patient’s refusal in receiving orthodontic treatment. The patient requested esthetic rehabilitation as an expectation for faster and instant esthetic result. Case management: Endodontic treatment was done to the involved dentition prior to the final restoration. Cast posts and all ceramic crowns were used as final restoration to correct the crowded and protruded anterior teeth. Conclusion: Esthetic rehabilitation can be done successfully on crowded and protruded anterior dentition. Instant result could be achieved by this treatment. This is supported by the fact that dentists should be aware of not only choosing the right treatment and materials but also patient’s expectations and conditions.

  20. Breast Reinnervation: DIEP Neurotization Using the Third Anterior Intercostal Nerve

    Directory of Open Access Journals (Sweden)

    Aldona J. Spiegel, MD

    2013-11-01

    Conclusion: DIEP flap neurotization using the third anterior intercostal nerve is an effective technique to provide a significant increase in sensory recovery for breast reconstruction patients, while adding minimal surgical time. Additionally, the use of a nerve conduit produces increased sensory recovery when compared direct coaptation.

  1. Holistic approach to understanding anterior knee pain. Clinical implications.

    Science.gov (United States)

    Sanchis-Alfonso, Vicente

    2014-10-01

    Anterior knee pain is one of the most frequent reasons for consultation within knee conditions. The aetiology is not well known, which explains the sometimes unpredictable results of its treatment. Normally, when we see a patient in the office with anterior knee pain, we only study and focus on the knee. If we do this, we are making a big mistake. We must not forget to evaluate the pelvis and proximal femur, as well as the psychological factors that modulate the course of the illness. Both the pelvifemoral dysfunction as well as the psychological factors (anxiety, depression, catastrophization and kinesiophobia) must be included in our therapeutic targets of the multidisciplinary treatment of anterior knee pain. We must not only focus on the knee, we must remember to "look up" to fully understand what is happening and be able to solve this difficult problem. The aetiology of anterior knee pain is multifactorial. Therefore, diagnosis and treatment of patellofemoral disorders must be individualized. Our findings stress the importance of tailoring physiotherapy, surgery and psycho-educational interventions to each patient.

  2. Nocardia Septic Arthritis Complicating an Anterior Cruciate Ligament Repair.

    Science.gov (United States)

    Yong, Elaine X L; Cheong, Elaine Y L; Boutlis, Craig S; Chen, Darren B; Liu, Eunice Y-T; McKew, Genevieve L

    2015-08-01

    Nocardia infection following anterior cruciate ligament (ACL) allograft reconstruction is a rare occurrence. We report a case of Nocardia infection of an allograft ACL reconstruction and septic arthritis of the knee joint due to an organism most similar to the novel Nocardia species Nocardia aobensis.

  3. Anastomotic leakage after anterior resection for rectal cancer: risk factors

    DEFF Research Database (Denmark)

    Bertelsen, C A; Andreasen, A H; Jørgensen, Torben;

    2010-01-01

    OBJECTIVE: The study aimed to identify risk factors for clinical anastomotic leakage (AL) after anterior resection for rectal cancer in a consecutive national cohort. METHOD: All patients with an initial first diagnosis of colorectal adenocarcinoma were prospectively registered in a national...

  4. Primary treatment of the anterior vocal commissure squamous carcinoma

    NARCIS (Netherlands)

    Bradley, Patrick J.; Rinaldo, Alessandra; Suarez, Carlos; Shaha, Ashok R.; Leemans, C. Rene; Langendijk, Johannes A.; Patel, Snehal G.; Ferlito, Alfio

    2006-01-01

    Squamous cell carcinoma may involve the anterior commissure (AC) area of the laryngeal glottis, and can be grouped morphologically into four groups; (1) tumor confined to the AC, (2) tumor involving one cord and the AC, (3) tumor involving the AC and a portion of both vocal cords, and (4) tumor invo

  5. Genetic studies in congenital anterior midline cervical cleft

    DEFF Research Database (Denmark)

    Jakobsen, L P; Pfeiffer, P; Andersen, M

    2012-01-01

    Congenital anterior midline cervical cleft (CAMCC) is a rare anomaly, with less than 100 cases reported. The cause of CAMCC is unknown, but genetic factors must be considered as part of the etiology. Three cases of CAMCC are presented. This is the first genetic study of isolated CAMCC. Conventional...

  6. The effect of anterior cruciate ligament injury on bone curvature

    DEFF Research Database (Denmark)

    Hunter, D J; Lohmander, Stefan; Makovey, J;

    2014-01-01

    OBJECTIVE: Investigate the 5-year longitudinal changes in bone curvature after acute anterior cruciate ligament (ACL) injury, and identify predictors of such changes. METHODS: In the KANON-trial (ISRCTN 84752559), 111/121 young active adults with an acute ACL tear to a previously un-injured knee...

  7. Removal of a giant intrathoracic cyst from the anterior mediastinum

    NARCIS (Netherlands)

    Bouma, Wobbe; Klinkenberg, Theo J.; Van De Wauwer, Caroline; Timens, Wim; Mariani, Massimo A.

    2014-01-01

    A 45-year-old caucasian man with progressive dyspnea appeared to have a giant intrathoracic cyst in the anterior mediastinum encasing the heart and compressing both lungs. He underwent succesful removal of the cyst through a median sternotomy. Recovery was uneventful. Gross examination revealed a th

  8. Esthetic Rehabilitation of Anterior Teeth with Laminates Composite Veneers

    OpenAIRE

    Dino Re; Gabriele Augusti; Massimo Amato; Giancarlo Riva; Davide Augusti

    2014-01-01

    No- or minimal-preparation veneers associated with enamel preservation offer predictable results in esthetic dentistry; indirect additive anterior composite restorations represent a quick, minimally invasive, inexpensive, and repairable option for a smile enhancement treatment plan. Current laboratory techniques associated with a strict clinical protocol satisfy patients’ restorative and esthetic needs. The case report presented describes minimal invasive treatment of four upper incisors with...

  9. Anastomoselaekage efter lav anterior resektion for cancer recti

    DEFF Research Database (Denmark)

    Bülow, S; Moesgaard, F A; Billesbølle, P;

    1997-01-01

    the routine use of a peroperative leakage test and selective use of prophylactic ostomy in cases of unsatisfactory anastomosis. Furthermore, it is recommended that low anterior resection for rectal cancer is limited to few surgeons in each department in order to ensure a uniform quality and hopefully also...

  10. MRI Features of Atypical Primitive Neuroectodermal Tumors in the Brain%颅脑不典型原始神经外胚层肿瘤MRI影像表现(附3例报道)

    Institute of Scientific and Technical Information of China (English)

    韩立志; 黄文才; 王鹰; 曾晓华

    2015-01-01

    目的 探讨颅脑不典型原始神经外胚层肿瘤(primitive neuroectodermal tumors,PNET)的磁共振(magnetic resonance imaging,MRI)影像特征.方法 回顾分析3例少见部位PNET的平扫及增强MRI检查资料.结果 3例不典型PNET分别位于左侧丘脑、脑干、右侧顶骨等罕见部位,3例肿瘤存在液化坏死且其均位于肿瘤边缘;肿瘤强化程度与囊变程度无关,与病理结果一致.结论 发生于颅脑罕见部位的不典型PNET,易与其它肿瘤混淆,误诊率高,正确认识其MRI特征性表现,对术前诊断本病及提高诊断率有重要参考价值.

  11. FUNCTIONAL OUTCOME OF ARTHROSCOPIC RECONSTRUCTION OF ANTERIOR CRUCIATE LIGAMENT TEARS

    Directory of Open Access Journals (Sweden)

    Ashok Kumar

    2016-02-01

    Full Text Available BACKGROUND Anterior Cruciate Ligament (ACL tear is the most common serious ligamentous injury to the knee joint. Anterior Cruciate Ligament (ACL injury is quite common among young active population, athletes and contact sports. The exact incidence of anterior cruciate ligament tears is not known as the cases are being under reported. The ACL is the primary stabilizer against anterior translation of the tibia on the femur and is important in counteracting rotation and valgus stress. MATERIALS AND METHODS Between November 2012 to October 2014, 34 consecutive patients who underwent arthroscopic assisted ACL reconstructions in the Department of Orthopedics and Traumatology, King George Hospital, Visakhapatnam were the material in our study. Age groups between 18 to 45 years considered. We utilised both BPTB and Quadrupled hamstring graft depending on the patient’s age, outcome testing in all cases was performed at the latest follow-up (at least 6 months. Post-operative physiotherapy rehabilitation protocol followed for 06 months. RESULTS Standard protocol of Lysholm and IKDC knee scoring system were used for evaluation of the results of the surgery during followup. Patients were evaluated periodically at preop, 3 months, 6 months, 12 months, 18 months and 24 months. CONCLUSION Patients with isolated ACL injury had better outcome compared to patients who underwent associated meniscectomy. Most common mechanism of injury was activity of sports in 20 patients. Postoperatively at 3 months, anterior drawer’s was 1+ in 6, 29 (85.2% patients had normal range of motion; 29 (85.29% patients had 5/5 quadriceps power (MRC grading 94% of them had 5/5 power at latest followup. No significant difference between outcomes of BPTB and Hamstrings graft. Functional outcome of our study were similar to the previously published studies.

  12. Bilateral Traumatic Anterior Dislocation of Shoulder – a rare entity

    Directory of Open Access Journals (Sweden)

    Yashavantha Kumar C

    2013-01-01

    Full Text Available Introduction: Bilateral shoulder dislocation are most commonly posterior type. These are most commonly due to seizure disorder and electrocution. Anterior shoulder dislocations occurring bilaterally without any predisposing factors are very rare. These types of injuries are due to trauma with a unique mechanism of injury. To best of our knowledge there are only few cases of similar kind are reported in literature. We hereby report a interesting case of posttraumatic, bilateral anterior dislocation of shoulder without associated fracture in a 45 old women without any predisposing pathoanatomy.Case Report: A 45-year-old women presented to casualty with sudden onset of pain and restriction of movement in both shoulders fallowing trauma. Immediately post trauma she had severe pain and restriction of both shoulders. On examination arms were abducted and externally rotated. Bilateral shoulder movements were painful and restricted . There was loss of round contour of shoulder with increased vertical diameter of axilla anteriorly. Radiological examination revealed bilateral anterior dislocation of the shoulders without any associated fractures. Closed reduction done by Milch technique after intraraticular lignocaine injection. MRI of bilateral shoulder showed no pathological lesion. Both shoulders were immobilized with a shoulder immobilizer for three weeks.Conclusion: Most of the bilateral shoulder dislocations are posterior type seen in seizure disorders. Bilateral traumatic anterior shoulder dislocations are rare and are seen as a result of unique mechanism of injury. In our case patient had a fall on her elbows causing forced extension. If diagnosed and treated promptly completely normal function of the shoulders can be restored.

  13. Anterior and posterior centers jointly regulate Bombyx embryo body segmentation.

    Science.gov (United States)

    Nakao, Hajime

    2012-11-15

    Insect embryo segmentation is largely divided into long and short germ types. In the long germ type, each segment primordium is represented on a large embryonic rudiment of the blastoderm, and segmental patterning occurs nearly simultaneously in the syncytium. In the short germ type, however, only anterior segments are represented in the small embryonic rudiment, usually located on the egg posterior, and the rest of the segments are added sequentially from the posterior growth zone in a cellular context. The long germ type is thought to have evolved from the short germ type. It is proposed that this transition, which appears to have occurred multiple times over the course of evolution, was realized through the acquisition of a localized anterior instruction center. Here, I examined the early segmentation process in the silkmoth Bombyx mori, a lepidopteran insect, in which the mechanisms of anterior-posterior (AP) axis formation have not been well analyzed. In this insect, both the long germ and short germ features have been reported. The mRNAs for two key genes involved in insect AP axis formation, orthodenticle (Bm-otd) and caudal (Bm-cad), are localized maternally in the germ anlage, where they act as anterior and posterior instruction centers, respectively. RNAi studies indicate that, while Bm-cad affects the formation of all the even skipped (Bm-eve) stripes, there is also anterior Bm-eve stripe formation activity that involves Bm-otd. Thus, there is redundancy in Bm-eve stripe formation activity that must be coordinated. Some genetic interactions, identified either experimentally or hypothetically, are also introduced, which might enable robust AP formation in this organism.

  14. The management of over closured anterior teeth due to attrition

    Directory of Open Access Journals (Sweden)

    Eha Djulaeha

    2009-12-01

    Full Text Available Background: Tooth is the hardest tissue in human body, that can be injured because of attrition process. For old people, denture attrition process is caused by psysiological process relating with the mastication function which also supported by some bad habits such an bruxism, premature contact, and consuming habit of abrasive food. Attrition or abrasion can also be happened with patien’t dentition who does not have teeth subtutition for long time due the lost of their maxillary as well as mandibulary. The pasient will loose their vertical dimension of occlusion, injure, and the lower jaw becomes over closed which is called over closure. Purpose: This article reported the management of over closured anterior teeth due to attrition. Case: a seventy six year old woman patient came to Prosthodontic Clinic in Faculty of Dentistry, Airlangga University, to rehabilitate her upper and lower severe attrited anterior teeth and her posterior teeth. The patient has experienced of wearing acrylic removable mandibular partial denture ten years ago. Unfortunaly, the denture was uncomfortable, and she did not wear it anymore since five years ago. Case management: The severe attrition of anterior teeth with the lost of occlusal vertical dimension can be treated by improving the occlusal vertical dimension gradually. The treatment is then followed by the increasing of the height of the anterior teeth by lengthening the crown teeth of upper jaw with 12 units of span bridge and the acrylic removable partial denture of lower jaw. Conclusion: The severe attrition of anterior teeth with the lost of occlusal vertical dimension can be treated by improving the occlusal vertical dimension gradually, using long span bridge and acrylic removable partial denture.

  15. Diagnóstico e tratamento do lenticone anterior

    Directory of Open Access Journals (Sweden)

    Ana Luiza Biancardi

    2011-08-01

    Full Text Available Relato de caso de um paciente de 18 anos com sindrome de Alport apresentando perda visual progressiva. A biomicroscopia revelou lenticone anterior bilateral. O paciente realizou tomografia de córnea e segmento anterior com o sistema Scheimpflug (Pentacam e aberrometria e topografia corneana (i-Trace. O paciente foi submetido à facoemulsificação com implante de lente intraocular peça única hidrofóbica (Acrysof® SN60AT. As imagens de Scheimpflug documentaram o lenticone anterior. A aberrometria total mostrou acentuado astigmatismo miópico com acentuada aberração esférica negativa, havendo grande impacto das aberrações de alta ordem (HOA na conversão da letra E de Snellen. O mapa de integração da aberrometria do olho todo com a topografia corneana mostrou maior semelhança das aberrrações totais com as aberrações intraoculares do que com as aberrações da superfície anterior da córnea. Após a cirurgia, o paciente apresentou acuidade visual corrigida igual a 20/20 em ambos os olhos, as imagens de Scheimpflug revelaram lentes intraoculares tópicas e os mapas diferenciais revelaram resolução da miopia e redução das aberrações da alta ordem (total e interna. Os exames de imagem foram úteis para demonstrar o impacto do lenticone anterior na qualidade visual e a resolução das aberrações ópticas após a cirurgia.

  16. Morphological character of cervical spine for anterior transpedicular screw fixation

    Directory of Open Access Journals (Sweden)

    Rong-Ping Zhou

    2013-01-01

    Full Text Available Background: Anterior cervical interbody grafts/cages combined with a plate were frequently used in multilevel discectomies/corpectomies. In order to avoid additional posterior stabilization in patients who undergo anterior reconstructive surgery, an anterior cervical transpedicular screw fixation, which offers higher stability is desirable. We investigated in this study the anatomical (morphologic characters for cervical anterior transpedicular screw fixation. Materials and Methods: Left pedicle parameters were measured on computed tomography (CT images based on 36 cervical spine CT scans from healthy subjects. The parameters included outer pedicle width (Distance from lateral to medial pedicle surface in the coronal plane, outer pedicle height (OPH (Distance from upper to lower pedicle surface in the sagittal plane, maximal pedicle axis length (MPAL, distance transverse insertion point (DIP, distance of the insertion point to the upper end plate (DIUP, pedicle sagittal transverse angle (PSTA and pedicle transverse angle (PTA at C3 to C7. Results: The values of outer pedicle width and MPAL in males were larger than in females from C3 to C7. The OPH in males was larger than in females at C3 to C6, but there was no difference at C7. The DIP and PTA were significantly greater in males than in females at C3, but there was no difference in the angle at C4-7. The PSTA was not statistically different between genders at C3, 4, 7, but this value in males was larger than females at C5, 6. The DIUP was significantly greater in males at C3, 4, 6, 7 but was non significant at C5. Conclusions: The placement of cervical anterior transpedicular screws should be individualized for each patient and based on a detailed preoperative planning.

  17. Simple self-reduction method for anterior shoulder dislocation

    Institute of Scientific and Technical Information of China (English)

    Reiner Wirbel; Martin Ruppert; Elmar Schwarz; Bernhard Zapp

    2014-01-01

    Objective:To demonstrate and evaluate a modified simple method about self-reduction of anterior shoulder dislocation for significance in the emergency room. Methods:TheBoss-Holzach-Matter method for self-reduction of anterior shoulder disloaction is described.Patients with an anterior shoulder dislocation were retrospectively analysed concerning age, gender, type of anterior shoulder dislocation, occurrence of associated fractures, time between injury and reduction, reduction time, and method of reduction with its respective success rate. Results:Eighty-six patients(52 men,34 women, mean age49 years) were treated fromJanuary 2010 toJune2014.The reduction time ranged between20 seconds and6 min(mean1.5 min). Subcoracoid type of shoulder dislocation was seen in72 cases(84%), subglenoid type in14 cases(16%).Associated factures were seen in20 cases, proportionally more often in subgleboid dislocations,12 at the greater tuberosity,6 at the inferior rim of the glenoid fossa and2 at both localizations.TheBoss-Holzach-Matter method was used in35 cases with a success rate of 71.5%; dieKocher method and traction/countertraction method with premedication were used in 14 cases and17 cases with success rates of64% and70%, respectively.All other cases and the failed primary attempts required hyponotic medication.All patients older than70(n=16) were not able to perform the self reducing procedure. Conclusion:The presentedBoss-Holzach-Matter method for reduction of anterior shoulder dislocation is a simple method without the need of anaesthesia, but cooperation from patients is crucial.The successful rate is comparable with other established methods.

  18. Fibrolipoma of the Anterior Abdominal Wall: an Atypical Presentation; Fibrolipoma de la pared abdominal anterior de presentacion atipica

    Energy Technology Data Exchange (ETDEWEB)

    Lorente, R.M.; Diaz, J. M.; Valle, Y. del; Gallego, I. [Hospital Universitario Santa Cristina. Madrid (Spain)

    2004-07-01

    Fibrolipoma is a type of lipoma that contains fibrous tissue. We present the case of an unusually large anterior abdominal wall fibrolipoma presenting rarely seem radiological characteristics which hinder its radiological diagnosis. We present the findings in ultrasound scan CT, surgery and pathological anatomy, as well as the lesion's differential diagnosis. (Author) 12 refs.

  19. Miniplacas permitem tratamento eficiente e eficaz da mordida aberta anterior Miniplates allow efficient and effective treatment of anterior open bites

    Directory of Open Access Journals (Sweden)

    Jorge Faber

    2008-10-01

    Full Text Available INTRODUÇÃO: o tratamento das deformidades e más oclusões que incluem mordidas abertas anteriores foi uma das primeiras aplicações de miniplacas como forma de ancoragem ortodôntica. A implementação desse sistema de tratamento reduz o número de pacientes indicados para a cirurgia ortognática e simplifica muitos problemas. Nessa abordagem, os dentes posteriores são intruídos e a mandíbula sofre um giro no sentido anti-horário, diminuindo a altura facial inferior e projetando os pogônios de tecidos duro e mole. OBJETIVO: o presente artigo apresenta os fundamentos da mecânica ortodôntica para correção da mordida aberta anterior e os ilustra com uma série de casos clínicos.INTRODUCTION: The treatment of facial deformities and malocclusions, such as anterior open bite, was one of the first applications of miniplates for orthodontic anchorage. The use of this treatment system reduces the number of patients referred to orthognathic surgery and simplifies many problems. This approach applies intrusive forces to posterior teeth, and the mandible undergoes counterclockwise rotation, which decreases lower facial height and advances the projection of hard and soft tissue pogonions. OBJECTIVE: This study describes the principles of orthodontic mechanics in the correction of anterior open bite and illustrates these principles with a series of cases.

  20. Effect of primary iris and ciliary body cyst on anterior chamber angle in patients with shallow anterior chamber

    Institute of Scientific and Technical Information of China (English)

    Bing-hong WANG; Yu-feng YAO

    2012-01-01

    Objective:To evaluate the prevalence of primary iris and/or ciliary body cysts in eyes with shallow anterior chamber and their effect on the narrowing of the anterior chamber angle.Methods:Among the general physical check-up population,subjects with shallow anterior chambers,as judged by van Herick technique,were recruited for further investigation.Ultrasound biomicroscope (UBM) was used to detect and measure the cysts located in the iris and/or ciliary body,the anterior chamber depth (ACD),the angle opening distance at 500 μm (AOD500),and the trabecular-iris angle (TIA).A-scan ultrasonography was used to measure the ocular biometry,including lens thickness,axial length,lens/axial length factor (LAF),and relative lens position (RLP).The effect of the cyst on narrowing the corresponding anterior chamber angle and the entire angle was evaluated by the UBM images,ocular biometry,and gonioscopic grading.The eye with unilateral cyst was compared with the eye without the cyst for further analysis.Results:Among the 727 subjects with shallow anterior chamber,primary iris and ciliary body cysts were detected in 250 (34.4%) patients; among them 96 (38.4%) patients showed unilateral single cyst,21 (8.4%) patients had unilateral double cysts,and 42 (16.8%) patients manifested unilateral multiple and multi-quadrants cysts.Plateau iris configuration was found in 140 of 361 (38.8%) eyes with cysts.The mean size of total cysts was (0.6547±0.2319) mm.In evaluation of the effect of the cyst size and location on narrowing the corresponding angle to their position,the proportion of the cysts causing corresponding angle narrowing or closure among the cysts larger than 0.8 mm (113/121,93.4%) was found to be significantly higher than that of the cysts smaller than 0.8 mm (373/801,46.6%),and a significant higher proportion was also found in the cysts located at iridociliary sulcus (354/437,81.0%) than in that at the pars plicata (131/484,27.1%).In evaluating the effect