Clinical Challenges and Images in GI
2008; Elsevier BV; Volume: 134; Issue: 7 Linguagem: Inglês
10.1053/j.gastro.2008.05.006
ISSN1528-0012
AutoresJin-Dong Kim, U I. Chang, Jin Mo Yang,
Tópico(s)Vascular Malformations and Hemangiomas
ResumoImage 3Question: A 33-year-old, nonalcoholic woman visited our clinic with shortness of breath and abdominal distension for 2 months. On physical examination, jaundice, tender hepatomegaly, and peripheral edema were observed. The laboratory examinations showed aspartate aminotransferase level of 71 IU/L, alanine aminotransferase level of 67 IU/L, total bilirubin level of 3.9 mg/dL, and serum albumin level of 3.0 g/dL. Hemoglobin, platelet count, serum creatinine, prothrombin time, and alkaline phosphatase level were all normal. Viral markers for hepatitis B and C were negative. Chest radiograph was normal, but simple abdomen radiograph showed marked hepatomegaly. Contrast-enhanced dynamic abdominal computed tomography (CT) scan demonstrated the following findings (A, B, C). What is the diagnosis?BView Large Image Figure ViewerDownload Hi-res image Download (PPT)CView Large Image Figure ViewerDownload Hi-res image Download (PPT)Look on page 2197 for the answer and see the Gastroenterology website (www.gastrojournal.org) for more information on submitting your favorite image to Clinical Challenges and Images in GI.Answer to the Clinical Challenges and Images in GI Question: Image 3 (page 1830): Diffuse Hepatic Hemangiomatosis Involving the Entire LiverNumerous delayed enhancing nodules and masses in both hepatic lobes with capsular retraction of hepatic surface were shown on the dynamic abdominal CT scan (Figure C). Serum α-fetoprotein level was 6.58 ng/ml. Ultrasound-guided biopsy of the hepatic tumor revealed variably dilated nonanastomotic vascular spaces, which were lined by flat endothelial cells and supported by fibrous tissue (Figure D; hematoxylin and eosin stain; original magnification, 200×). Immunohistochemical staining for CD34 (Figure E; original magnification, 200×) was positive on the endothelial cells. After histologic confirmation of the diagnosis of hepatic hemangiomatosis, the patient died of liver failure on the hospital day 12 while a donor for liver transplantation was being prepared.DView Large Image Figure ViewerDownload Hi-res image Download (PPT)EView Large Image Figure ViewerDownload Hi-res image Download (PPT)Cavenous hepatic hemangioma is the most common hepatic tumor, but hepatic hemangiomatosis, which is defined as extensive hemangioma throughout the liver, has been rarely reported in adults, especially in patients without extrahepatic lesions. Most cases of hemangiomatosis were usually reported in infants with abdominal masses or congestive heart failure.1Bernard G. Mion F. Henry L. et al.Hepatic involvement in hereditary hemorrhagic telangiectasia: clinical, radiological, and hemodynamic studies of 11 cases.Gastroenterology. 1993; 105: 482-487PubMed Google Scholar The etiologic association with some medications such as estrogen and metoclopramide has been reported,2Conter R.L. Longmire W.P. Recurrent hepatic hemangiomas Possible association with estrogen therapy.Ann Surg. 1988; 207: 115-119Crossref PubMed Scopus (96) Google Scholar, 3Feurle G.E. Arteriovenous shunting and cholestasis in hepatic hemangiomatosis associated with metoclopramide.Gastroenterology. 1990; 99: 258-262PubMed Google Scholar but the etiology and clinical course of hepatic hemangiomatosis are not completely understood because of its rareness. Surgical resection is suggested when the boundaries of the tumor are well-delineated in 1 lobe; ligation of hepatic artery is recommended when the tumor involves both lobes.4Kawarada Y. Mizumoto R. Surgical treatment of giant hemangioma of the liver.Am J Surg. 1984; 148: 287-291Abstract Full Text PDF PubMed Scopus (40) Google Scholar If these methods are not employed, then liver transplantation should be considered as the treatment modality for patients with congestive heart failure or progressive hepatic dysfunction.5Frangides C. Kounis N.G. Papadaki P.J. et al.Diffuse hepatic haemangiomatosis in the elderly.Br J Clin Pract. 1995; 49: 215-216PubMed Google Scholar Image 3Question: A 33-year-old, nonalcoholic woman visited our clinic with shortness of breath and abdominal distension for 2 months. On physical examination, jaundice, tender hepatomegaly, and peripheral edema were observed. The laboratory examinations showed aspartate aminotransferase level of 71 IU/L, alanine aminotransferase level of 67 IU/L, total bilirubin level of 3.9 mg/dL, and serum albumin level of 3.0 g/dL. Hemoglobin, platelet count, serum creatinine, prothrombin time, and alkaline phosphatase level were all normal. Viral markers for hepatitis B and C were negative. Chest radiograph was normal, but simple abdomen radiograph showed marked hepatomegaly. Contrast-enhanced dynamic abdominal computed tomography (CT) scan demonstrated the following findings (A, B, C). What is the diagnosis?CView Large Image Figure ViewerDownload Hi-res image Download (PPT)Look on page 2197 for the answer and see the Gastroenterology website (www.gastrojournal.org) for more information on submitting your favorite image to Clinical Challenges and Images in GI. Question: A 33-year-old, nonalcoholic woman visited our clinic with shortness of breath and abdominal distension for 2 months. On physical examination, jaundice, tender hepatomegaly, and peripheral edema were observed. The laboratory examinations showed aspartate aminotransferase level of 71 IU/L, alanine aminotransferase level of 67 IU/L, total bilirubin level of 3.9 mg/dL, and serum albumin level of 3.0 g/dL. Hemoglobin, platelet count, serum creatinine, prothrombin time, and alkaline phosphatase level were all normal. Viral markers for hepatitis B and C were negative. Chest radiograph was normal, but simple abdomen radiograph showed marked hepatomegaly. Contrast-enhanced dynamic abdominal computed tomography (CT) scan demonstrated the following findings (A, B, C). What is the diagnosis? Look on page 2197 for the answer and see the Gastroenterology website (www.gastrojournal.org) for more information on submitting your favorite image to Clinical Challenges and Images in GI. Answer to the Clinical Challenges and Images in GI Question: Image 3 (page 1830): Diffuse Hepatic Hemangiomatosis Involving the Entire LiverNumerous delayed enhancing nodules and masses in both hepatic lobes with capsular retraction of hepatic surface were shown on the dynamic abdominal CT scan (Figure C). Serum α-fetoprotein level was 6.58 ng/ml. Ultrasound-guided biopsy of the hepatic tumor revealed variably dilated nonanastomotic vascular spaces, which were lined by flat endothelial cells and supported by fibrous tissue (Figure D; hematoxylin and eosin stain; original magnification, 200×). Immunohistochemical staining for CD34 (Figure E; original magnification, 200×) was positive on the endothelial cells. After histologic confirmation of the diagnosis of hepatic hemangiomatosis, the patient died of liver failure on the hospital day 12 while a donor for liver transplantation was being prepared.EView Large Image Figure ViewerDownload Hi-res image Download (PPT)Cavenous hepatic hemangioma is the most common hepatic tumor, but hepatic hemangiomatosis, which is defined as extensive hemangioma throughout the liver, has been rarely reported in adults, especially in patients without extrahepatic lesions. Most cases of hemangiomatosis were usually reported in infants with abdominal masses or congestive heart failure.1Bernard G. Mion F. Henry L. et al.Hepatic involvement in hereditary hemorrhagic telangiectasia: clinical, radiological, and hemodynamic studies of 11 cases.Gastroenterology. 1993; 105: 482-487PubMed Google Scholar The etiologic association with some medications such as estrogen and metoclopramide has been reported,2Conter R.L. Longmire W.P. Recurrent hepatic hemangiomas Possible association with estrogen therapy.Ann Surg. 1988; 207: 115-119Crossref PubMed Scopus (96) Google Scholar, 3Feurle G.E. Arteriovenous shunting and cholestasis in hepatic hemangiomatosis associated with metoclopramide.Gastroenterology. 1990; 99: 258-262PubMed Google Scholar but the etiology and clinical course of hepatic hemangiomatosis are not completely understood because of its rareness. Surgical resection is suggested when the boundaries of the tumor are well-delineated in 1 lobe; ligation of hepatic artery is recommended when the tumor involves both lobes.4Kawarada Y. Mizumoto R. Surgical treatment of giant hemangioma of the liver.Am J Surg. 1984; 148: 287-291Abstract Full Text PDF PubMed Scopus (40) Google Scholar If these methods are not employed, then liver transplantation should be considered as the treatment modality for patients with congestive heart failure or progressive hepatic dysfunction.5Frangides C. Kounis N.G. Papadaki P.J. et al.Diffuse hepatic haemangiomatosis in the elderly.Br J Clin Pract. 1995; 49: 215-216PubMed Google Scholar Numerous delayed enhancing nodules and masses in both hepatic lobes with capsular retraction of hepatic surface were shown on the dynamic abdominal CT scan (Figure C). Serum α-fetoprotein level was 6.58 ng/ml. Ultrasound-guided biopsy of the hepatic tumor revealed variably dilated nonanastomotic vascular spaces, which were lined by flat endothelial cells and supported by fibrous tissue (Figure D; hematoxylin and eosin stain; original magnification, 200×). Immunohistochemical staining for CD34 (Figure E; original magnification, 200×) was positive on the endothelial cells. After histologic confirmation of the diagnosis of hepatic hemangiomatosis, the patient died of liver failure on the hospital day 12 while a donor for liver transplantation was being prepared. Cavenous hepatic hemangioma is the most common hepatic tumor, but hepatic hemangiomatosis, which is defined as extensive hemangioma throughout the liver, has been rarely reported in adults, especially in patients without extrahepatic lesions. Most cases of hemangiomatosis were usually reported in infants with abdominal masses or congestive heart failure.1Bernard G. Mion F. Henry L. et al.Hepatic involvement in hereditary hemorrhagic telangiectasia: clinical, radiological, and hemodynamic studies of 11 cases.Gastroenterology. 1993; 105: 482-487PubMed Google Scholar The etiologic association with some medications such as estrogen and metoclopramide has been reported,2Conter R.L. Longmire W.P. Recurrent hepatic hemangiomas Possible association with estrogen therapy.Ann Surg. 1988; 207: 115-119Crossref PubMed Scopus (96) Google Scholar, 3Feurle G.E. Arteriovenous shunting and cholestasis in hepatic hemangiomatosis associated with metoclopramide.Gastroenterology. 1990; 99: 258-262PubMed Google Scholar but the etiology and clinical course of hepatic hemangiomatosis are not completely understood because of its rareness. Surgical resection is suggested when the boundaries of the tumor are well-delineated in 1 lobe; ligation of hepatic artery is recommended when the tumor involves both lobes.4Kawarada Y. Mizumoto R. Surgical treatment of giant hemangioma of the liver.Am J Surg. 1984; 148: 287-291Abstract Full Text PDF PubMed Scopus (40) Google Scholar If these methods are not employed, then liver transplantation should be considered as the treatment modality for patients with congestive heart failure or progressive hepatic dysfunction.5Frangides C. Kounis N.G. Papadaki P.J. et al.Diffuse hepatic haemangiomatosis in the elderly.Br J Clin Pract. 1995; 49: 215-216PubMed Google Scholar
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