Hepatobiliary Involvement in von Recklinghausen's Disease
Authors: GEORGE W. MEYER
Journal: Annals of Internal Medicine (1982)

Brief Reports1 November 1982Hepatobiliary Involvement in von Recklinghausen's DiseaseGEORGE W. MEYER, WILLIAM J. GRIFFITHS, M.D., Ph.D., JACK WELSH, M.D., LEONARD COHEN, M.D., LEWIS JOHNSON, M.D., MICHAEL J. WEAVER, M.D.GEORGE W. MEYERSearch for more papers by this author, WILLIAM J. GRIFFITHS, M.D., Ph.D.Search for more papers by this author, JACK WELSH, M.D.Search for more papers by this author, LEONARD COHEN, M.D.Search for more papers by this author, LEWIS JOHNSON, M.D.Search for more papers by this author, MICHAEL J. WEAVER, M.D.Search for more papers by this authorAuthor, Article, and Disclosure Informationhttps://doi.org/10.7326/0003-4819-97-5-722 SectionsAboutPDF ToolsAdd to favoritesDownload CitationsTrack CitationsPermissions ShareFacebookTwitterLinkedInRedditEmail ExcerptVon Recklinghausen's disease is a syndrome characterized by multiple cutaneous neurofibromas, cafe-aulait spots, and a broad spectrum of clinical, pathologic, and radiologic findings. Von Recklinghausen's disease is inherited as a variably expressed dominant gene, with 50% of the patients having affected relatives. As many as 25% of patients with this illness have neurofibromas or other related neurogenic tumors in the gastrointestinal tract (1). Patients with these tumors usually present with gastrointestinal hemorrhage or obstruction. Hepatobiliary complications are rare, and we know of only one reported case (2). We report three patients with von Recklinghausen's disease and prominent hepatobiliary involvement.Patient...References1. GHRIST T. Gastrointestinal involvement in neurofibromatosis. Arch Intern Med. 1963;112:111-6. CrossrefGoogle Scholar2. YOUNG S. Primary malignant neurilemmoma (Schwanoma) of the liver in a case of neurofibromatosis. J Pathol. 1975;117:151-3. CrossrefMedlineGoogle Scholar3. JOHNSONWEAVER LM. von Recklinghausen's disease and gastrointestinal carcinoids [Letter]. JAMA. 1981; 245:2496. CrossrefMedlineGoogle Scholar4. RIVERSILVERSTEINTOPE LJJ. Benign neoplasms of the small intestine. Int Abstr Surg. 1956;102:1-38. Google Scholar5. SIVAKSULLIVANFARMER MBR. Neurogenic tumors of the small intestine. Gastroenterology. 1975; 68:374-80. CrossrefMedlineGoogle Scholar6. DAVISBERK GR. Intestinal neurofibromas in von Recklinghausen's disease. Am J Gastroenterol. 1973;60:410-4. MedlineGoogle Scholar7. HOCHBERGDASILVAGALDABINIRICHARDSON FAJE. Gastrointestinal involvement in von Recklinghausen's neurofibromatosis. Neurology. 1974;24:1144-51. CrossrefMedlineGoogle Scholar8. WESTONMARRENCOHAN SMM. Neurofibroma of the rectum and colon. J Int Coll Surg. 1963; 40:285-93. MedlineGoogle Scholar9. LUKASHJOHNSON WR. Gastrointestinal neoplasms in von Recklinghausen's disease. South Med J. 1969;62:1237-9. CrossrefMedlineGoogle Scholar This content is PDF only. To continue reading please click on the PDF icon. Author, Article, and Disclosure InformationAffiliations: USAF Medical Center Wright-Patterson Air Force Base Ohio University of Oklahoma Health Science Center Oklahoma City, Oklahoma University of California San Francisco, California Brown University School of Medicine Providence, Rhode Island PreviousarticleNextarticle Advertisement FiguresReferencesRelatedDetails Metrics Cited ByThe Liver in Systemic DiseaseTumors and Tumor-Like Lesions of the Schwann Cell Lineage and the Nerve SheathHilar biliary neurofibroma without neurofibromatosis: case report with contrast-enhanced ultrasound findingsTumors and Tumor-like Lesions of the Schwann Cell Lineage and the Nerve SheathSystemic diseaseAbnormal Presentation of Neurofibromatosis: Review of the LiteraturePrimary Neurofibroma of the Common Bile Duct as an Unusual Cause of Obstructive Jaundice: A Case ReportAn unusual cause of biliary stricture in a patient with neurofibromatosis type 1Neurofibroma of the Common Bile DuctDuodenal Epithelial Neoplasms Complicating von Recklinghausen's Disease: An Immunohistochemical Analysis in Two PatientsPlexiform neurofibromatosis of the liver: Case report and review of the literatureVon recklinghausen's disease associated with duodenal somatostatinoma: Contrast of duodenal versus pancreatic somatostatinomasProtein-losing enteropathy caused by mesenteric vascular involvement of neurofibromatosisAnomalous pancreaticobiliary communication in a neurofibromatosis patientVon Recklinghausen's Disease Associated with Somatostatin-rich Duodenal Carcinoid (Somatostatinoma), Medullary Thyroid Carcinoma and Diffuse Adrenal Medullary HyperplasiaPlexiform neurofibromatosis of the liver and mesentery in a childSynchronous duodenal neuroendocrine tumours in von Recklinghausen's disease?a case report of co-existing gangliocytic paraganglioma and somatostatin-rich glandular carcinoidPrimary Bile Duct Cancer and von Recklinghausen DiseaseDIRK P. SPRENGERS, M.D., DANIEL C. KNOCKAERT, M.D., WERNER VAN STEENBERGEN, M.D., FREDDY PENNINCKX, M.D.Chronic recurrent pancreatitis secondary to a submucosal ampullary tumor in a patient with neurofibromatosisHepatic neurofibromatosis, malignant schwannoma, and angiosarcoma in von Recklinghausen's diseaseGastrointestinal involvement in neurofibromatosis: Angiographic presentationNeurofibromatosis, generalized pruritus and cholestatic liver dysfunction-report of two cases 1 November 1982Volume 97, Issue 5Page: 722-723KeywordsGastrointestinal hemorrhageGastrointestinal tractNeurofibromatosis type 1 Issue Published: 1 November 1982 PDF DownloadLoading ...