Plexiform neurofibromatosis of the liver and mesentery in a child
Plexiform neurofibromatosis of the liver was recognized by needle biopsy of the liver in an 11‐yr‐old boy who had a 2‐yr history of diarrhea, intermittent abdominal pain, failure to gain weight and progressive abdominal distention. Imaging studies demonstrated a large retroperitoneal mass; a laparot...
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| Veröffentlicht in: | Hepatology (Baltimore, Md.) Md.), 1990-09, Vol.12 (3), p.559-564 |
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| container_title | Hepatology (Baltimore, Md.) |
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| creator | Partin, Jacqueline S. Lane, Bernard P. Partin, John C. Edelstein, Lawrence R. Priebe, Cedric J. |
| description | Plexiform neurofibromatosis of the liver was recognized by needle biopsy of the liver in an 11‐yr‐old boy who had a 2‐yr history of diarrhea, intermittent abdominal pain, failure to gain weight and progressive abdominal distention. Imaging studies demonstrated a large retroperitoneal mass; a laparotomy was performed. At surgery, the mesentery was greatly thickened by neurofibromas, and plexiform neurofibroma extended through the hilum of the liver. Light and electron microscopy demonstrated that in addition to the direct involvement by tumor, neural hyperplasia existed throughout the liver. The most distal ramifications of the portal spaces were filled with Schwann cells, bundles of unmyelinated nerves and perineurium‐surrounded nerves containing myelinated and unmyelinated fibers. The ultrastructural findings were consistent with stimulation of proliferation of all the portal neural elements and tumoral tissue. The nontumoral response was more than simple hyperplasia because it appeared to result in fibrotic changes in the most involved areas and active breaching of the limiting plate with destruction of hepatocytes and collagen deposition throughout the liver. (HEPATOLOGY 1990;12:559–564). |
| doi_str_mv | 10.1002/hep.1840120318 |
| format | Article |
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Imaging studies demonstrated a large retroperitoneal mass; a laparotomy was performed. At surgery, the mesentery was greatly thickened by neurofibromas, and plexiform neurofibroma extended through the hilum of the liver. Light and electron microscopy demonstrated that in addition to the direct involvement by tumor, neural hyperplasia existed throughout the liver. The most distal ramifications of the portal spaces were filled with Schwann cells, bundles of unmyelinated nerves and perineurium‐surrounded nerves containing myelinated and unmyelinated fibers. The ultrastructural findings were consistent with stimulation of proliferation of all the portal neural elements and tumoral tissue. The nontumoral response was more than simple hyperplasia because it appeared to result in fibrotic changes in the most involved areas and active breaching of the limiting plate with destruction of hepatocytes and collagen deposition throughout the liver. (HEPATOLOGY 1990;12:559–564).</description><identifier>ISSN: 0270-9139</identifier><identifier>EISSN: 1527-3350</identifier><identifier>DOI: 10.1002/hep.1840120318</identifier><identifier>PMID: 2119333</identifier><identifier>CODEN: HPTLD9</identifier><language>eng</language><publisher>Philadelphia, PA: W.B. Saunders</publisher><subject>Biological and medical sciences ; Biopsy, Needle ; Child ; Humans ; Hyperplasia - pathology ; Liver - innervation ; Liver - ultrastructure ; Liver Neoplasms - diagnosis ; Liver Neoplasms - pathology ; Lymphatic Metastasis ; Male ; Medical sciences ; Mesentery - pathology ; Microscopy, Electron ; Neurofibromatosis 1 - diagnosis ; Neurofibromatosis 1 - pathology ; Neurology ; Peritoneal Neoplasms - diagnosis ; Peritoneal Neoplasms - pathology ; Tumors of the nervous system. Phacomatoses</subject><ispartof>Hepatology (Baltimore, Md.), 1990-09, Vol.12 (3), p.559-564</ispartof><rights>Copyright © 1990 American Association for the Study of Liver Diseases</rights><rights>1991 INIST-CNRS</rights><lds50>peer_reviewed</lds50><oa>free_for_read</oa><woscitedreferencessubscribed>false</woscitedreferencessubscribed><citedby>FETCH-LOGICAL-c4768-50a9722ce5a6d38793b58514d1f535b84882f2606d5e8c5716ca0a1f5294fc883</citedby><cites>FETCH-LOGICAL-c4768-50a9722ce5a6d38793b58514d1f535b84882f2606d5e8c5716ca0a1f5294fc883</cites></display><links><openurl>$$Topenurl_article</openurl><openurlfulltext>$$Topenurlfull_article</openurlfulltext><thumbnail>$$Tsyndetics_thumb_exl</thumbnail><linktopdf>$$Uhttps://onlinelibrary.wiley.com/doi/pdf/10.1002%2Fhep.1840120318$$EPDF$$P50$$Gwiley$$H</linktopdf><linktohtml>$$Uhttps://onlinelibrary.wiley.com/doi/full/10.1002%2Fhep.1840120318$$EHTML$$P50$$Gwiley$$H</linktohtml><link.rule.ids>314,776,780,1411,27901,27902,45550,45551</link.rule.ids><backlink>$$Uhttp://pascal-francis.inist.fr/vibad/index.php?action=getRecordDetail&idt=19593493$$DView record in Pascal Francis$$Hfree_for_read</backlink><backlink>$$Uhttps://www.ncbi.nlm.nih.gov/pubmed/2119333$$D View this record in MEDLINE/PubMed$$Hfree_for_read</backlink></links><search><creatorcontrib>Partin, Jacqueline S.</creatorcontrib><creatorcontrib>Lane, Bernard P.</creatorcontrib><creatorcontrib>Partin, John C.</creatorcontrib><creatorcontrib>Edelstein, Lawrence R.</creatorcontrib><creatorcontrib>Priebe, Cedric J.</creatorcontrib><title>Plexiform neurofibromatosis of the liver and mesentery in a child</title><title>Hepatology (Baltimore, Md.)</title><addtitle>Hepatology</addtitle><description>Plexiform neurofibromatosis of the liver was recognized by needle biopsy of the liver in an 11‐yr‐old boy who had a 2‐yr history of diarrhea, intermittent abdominal pain, failure to gain weight and progressive abdominal distention. Imaging studies demonstrated a large retroperitoneal mass; a laparotomy was performed. At surgery, the mesentery was greatly thickened by neurofibromas, and plexiform neurofibroma extended through the hilum of the liver. Light and electron microscopy demonstrated that in addition to the direct involvement by tumor, neural hyperplasia existed throughout the liver. The most distal ramifications of the portal spaces were filled with Schwann cells, bundles of unmyelinated nerves and perineurium‐surrounded nerves containing myelinated and unmyelinated fibers. The ultrastructural findings were consistent with stimulation of proliferation of all the portal neural elements and tumoral tissue. The nontumoral response was more than simple hyperplasia because it appeared to result in fibrotic changes in the most involved areas and active breaching of the limiting plate with destruction of hepatocytes and collagen deposition throughout the liver. (HEPATOLOGY 1990;12:559–564).</description><subject>Biological and medical sciences</subject><subject>Biopsy, Needle</subject><subject>Child</subject><subject>Humans</subject><subject>Hyperplasia - pathology</subject><subject>Liver - innervation</subject><subject>Liver - ultrastructure</subject><subject>Liver Neoplasms - diagnosis</subject><subject>Liver Neoplasms - pathology</subject><subject>Lymphatic Metastasis</subject><subject>Male</subject><subject>Medical sciences</subject><subject>Mesentery - pathology</subject><subject>Microscopy, Electron</subject><subject>Neurofibromatosis 1 - diagnosis</subject><subject>Neurofibromatosis 1 - pathology</subject><subject>Neurology</subject><subject>Peritoneal Neoplasms - diagnosis</subject><subject>Peritoneal Neoplasms - pathology</subject><subject>Tumors of the nervous system. Phacomatoses</subject><issn>0270-9139</issn><issn>1527-3350</issn><fulltext>true</fulltext><rsrctype>article</rsrctype><creationdate>1990</creationdate><recordtype>article</recordtype><sourceid>EIF</sourceid><recordid>eNqFkD1PwzAURS0EKqWwsiF5gS3FH3FijxUqFKkSHWCOHOdZNXKSYqdA_z2pGlE2Jg_3vPv8DkLXlEwpIex-DZsplSmhjHAqT9CYCpYnnAtyisaE5SRRlKtzdBHjOyFEpUyO0IhRqjjnYzRbefh2tg01bmAbWuvK0Na6a6OLuLW4WwP27hMC1k2Fa4jQdBB22DVYY7N2vrpEZ1b7CFfDO0Fvj_PXh0WyfHl6fpgtE5PmmUwE0SpnzIDQWcVlrngppKBpRa3gopSplMyyjGSVAGlETjOjie5DplJrpOQTdHfo3YT2YwuxK2oXDXivG2i3sZD9dTmlWQ9OD6AJbYwBbLEJrtZhV1BS7J0VvbPi6KwfuBmat2UN1S8-SOrz2yHX0Whvg26Mi8dWJRRP1Z5TB-7Ledj9s7VYzFd__vADKEuDVA</recordid><startdate>199009</startdate><enddate>199009</enddate><creator>Partin, Jacqueline S.</creator><creator>Lane, Bernard P.</creator><creator>Partin, John C.</creator><creator>Edelstein, Lawrence R.</creator><creator>Priebe, Cedric J.</creator><general>W.B. Saunders</general><general>Wiley</general><scope>IQODW</scope><scope>CGR</scope><scope>CUY</scope><scope>CVF</scope><scope>ECM</scope><scope>EIF</scope><scope>NPM</scope><scope>AAYXX</scope><scope>CITATION</scope><scope>7X8</scope></search><sort><creationdate>199009</creationdate><title>Plexiform neurofibromatosis of the liver and mesentery in a child</title><author>Partin, Jacqueline S. ; Lane, Bernard P. ; Partin, John C. ; Edelstein, Lawrence R. ; Priebe, Cedric J.</author></sort><facets><frbrtype>5</frbrtype><frbrgroupid>cdi_FETCH-LOGICAL-c4768-50a9722ce5a6d38793b58514d1f535b84882f2606d5e8c5716ca0a1f5294fc883</frbrgroupid><rsrctype>articles</rsrctype><prefilter>articles</prefilter><language>eng</language><creationdate>1990</creationdate><topic>Biological and medical sciences</topic><topic>Biopsy, Needle</topic><topic>Child</topic><topic>Humans</topic><topic>Hyperplasia - pathology</topic><topic>Liver - innervation</topic><topic>Liver - ultrastructure</topic><topic>Liver Neoplasms - diagnosis</topic><topic>Liver Neoplasms - pathology</topic><topic>Lymphatic Metastasis</topic><topic>Male</topic><topic>Medical sciences</topic><topic>Mesentery - pathology</topic><topic>Microscopy, Electron</topic><topic>Neurofibromatosis 1 - diagnosis</topic><topic>Neurofibromatosis 1 - pathology</topic><topic>Neurology</topic><topic>Peritoneal Neoplasms - diagnosis</topic><topic>Peritoneal Neoplasms - pathology</topic><topic>Tumors of the nervous system. Phacomatoses</topic><toplevel>peer_reviewed</toplevel><toplevel>online_resources</toplevel><creatorcontrib>Partin, Jacqueline S.</creatorcontrib><creatorcontrib>Lane, Bernard P.</creatorcontrib><creatorcontrib>Partin, John C.</creatorcontrib><creatorcontrib>Edelstein, Lawrence R.</creatorcontrib><creatorcontrib>Priebe, Cedric J.</creatorcontrib><collection>Pascal-Francis</collection><collection>Medline</collection><collection>MEDLINE</collection><collection>MEDLINE (Ovid)</collection><collection>MEDLINE</collection><collection>MEDLINE</collection><collection>PubMed</collection><collection>CrossRef</collection><collection>MEDLINE - Academic</collection><jtitle>Hepatology (Baltimore, Md.)</jtitle></facets><delivery><delcategory>Remote Search Resource</delcategory><fulltext>fulltext</fulltext></delivery><addata><au>Partin, Jacqueline S.</au><au>Lane, Bernard P.</au><au>Partin, John C.</au><au>Edelstein, Lawrence R.</au><au>Priebe, Cedric J.</au><format>journal</format><genre>article</genre><ristype>JOUR</ristype><atitle>Plexiform neurofibromatosis of the liver and mesentery in a child</atitle><jtitle>Hepatology (Baltimore, Md.)</jtitle><addtitle>Hepatology</addtitle><date>1990-09</date><risdate>1990</risdate><volume>12</volume><issue>3</issue><spage>559</spage><epage>564</epage><pages>559-564</pages><issn>0270-9139</issn><eissn>1527-3350</eissn><coden>HPTLD9</coden><abstract>Plexiform neurofibromatosis of the liver was recognized by needle biopsy of the liver in an 11‐yr‐old boy who had a 2‐yr history of diarrhea, intermittent abdominal pain, failure to gain weight and progressive abdominal distention. Imaging studies demonstrated a large retroperitoneal mass; a laparotomy was performed. At surgery, the mesentery was greatly thickened by neurofibromas, and plexiform neurofibroma extended through the hilum of the liver. Light and electron microscopy demonstrated that in addition to the direct involvement by tumor, neural hyperplasia existed throughout the liver. The most distal ramifications of the portal spaces were filled with Schwann cells, bundles of unmyelinated nerves and perineurium‐surrounded nerves containing myelinated and unmyelinated fibers. The ultrastructural findings were consistent with stimulation of proliferation of all the portal neural elements and tumoral tissue. The nontumoral response was more than simple hyperplasia because it appeared to result in fibrotic changes in the most involved areas and active breaching of the limiting plate with destruction of hepatocytes and collagen deposition throughout the liver. (HEPATOLOGY 1990;12:559–564).</abstract><cop>Philadelphia, PA</cop><pub>W.B. Saunders</pub><pmid>2119333</pmid><doi>10.1002/hep.1840120318</doi><tpages>6</tpages><oa>free_for_read</oa></addata></record> |
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| subjects | Biological and medical sciences Biopsy, Needle Child Humans Hyperplasia - pathology Liver - innervation Liver - ultrastructure Liver Neoplasms - diagnosis Liver Neoplasms - pathology Lymphatic Metastasis Male Medical sciences Mesentery - pathology Microscopy, Electron Neurofibromatosis 1 - diagnosis Neurofibromatosis 1 - pathology Neurology Peritoneal Neoplasms - diagnosis Peritoneal Neoplasms - pathology Tumors of the nervous system. Phacomatoses |
| title | Plexiform neurofibromatosis of the liver and mesentery in a child |
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