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An Unusual Cause of Epigastric Pain
Electronic Clinical Challenges and Images in GI An Unusual Cause of Epigastric Pain Tan To Cheung,1 Regina Lo,2 and Ronnie Tung Ping Poon1 1Department
of Surgery and 2Department of Pathology, Queen Mary Hospital, The University of Hong Kong, Hong Kong, China
Question: A 56-year-old man presented with epigastric pain of 6 months’ duration. He was a nonsmoker and did not complain of passing tarry stool. The patient had a meningeal tumor resected 16 years ago. On physical examination, the patient was not pale and there was no jaundice. A vague, nonpulsatile, 10-cm mass was found at the epigastrium that moved with respiration. Investigations showed hemoglobin 10.2 g/dL, leukocytes 4.7 ⫻ 109/L, platelets 140 ⫻ 109/L, bilirubin 28 mol/L (reference, ⬍40), aspartate transaminase 34 U/L (reference, 15–38), alanine transaminase 40 U/L (reference, 8 –58), and ␥-glutamyltransferase 26 U/L (reference, 11– 62). He tested negative for hepatitis B virus surface antigen and hepatitis C virus antibody. Tumor markers, including alpha fetoprotein and carcinoembryonic antigen, were not elevated. Upper endoscopy did not review any abnormality. Computed tomography with intravenous contrast was arranged and revealed a large tumor at the left lobe of the liver (Figure A). Liver resection was performed and the postoperative recovery was uneventful. The cut open specimen showed a well encapsulated tumour which was hard in consistency (Figure B). Grossly, the tumor was 16 cm in size with homogeneous component. Figure C, D shows a high-power view of the microscopic sections (stain: hematoxylin and eosin). Figure E, F shows the recticulin staining and CD34 membrane staining at high-power view. What is the diagnosis? See the GASTROENTEROLOGY web site (www.gastrojournal.org) for more information on submitting your favorite image to Clinical Challenges and Images in GI. Conflicts of interest: The authors disclose no conflicts. © 2013 by the AGA Institute 0016-5085/$36.00 http://dx.doi.org/10.1053/j.gastro.2013.01.060
GASTROENTEROLOGY 2013;144:e11– e12
Electronic Clinical Challenges and Images in GI, continued Answer to the Clinical Challenges and Images in GI Question: Image 6: Hemangiopericytoma of the Liver Left hepatectomy was performed. Microscopic examination revealed rare tumor histology. The tumor comprised spindle cells of moderate cellularity and moderate pleomorphism. They were arranged in fascicles among a fibrous stroma. Gapping blood vessels were scattered among the tumor cells (stain: hematoxylin and eosin; original magnification, 100⫻; Figure C). Tumor necrosis was present (stain: hematoxylin and eosin; original magnification, 100⫻; Figure D). Reticulin stain highlighted the contour of each individual tumor cell, suggesting its mesenchymal nature (Figure E). Immunohistochemically, some of the tumor cells expressed CD34 (membranous staining; Figure F). On review, the histology of meningeal and liver tumors was similar. Hence the liver tumor was possibly a metastatic lesion. The diagnosis was hemangiopericytoma of the liver. Hemangiopericytoma is a rare mesenchymal tumor that develops from the pericyte of Zimmermann. Theoretically, it can affect every organ in the body. The more common site for its development is from the lower extremities especially over the thigh.1 It can also present as peritoneal tumors. The incident of this lesion is low and it accounts for ⬍2% of all soft tissue sarcomas.2 Most patients are asymptomatic until the tumors grow to considerable size and lead to local pressure symptoms. It has also been reported that the lesion can be associated with paraneoplastic syndrome leading to hypoglycemia. Contrast-enhanced computed tomography usually shows a heterogeneously enhanced vascular lesion with strong fibrous content inside the lesion. Because the incidence of this tumor is very rare, only histopathology can provide adequate information for diagnosis. Although hepatectomy for resectable disease provides chance for cure, long-term follow-up is required because the natural history this disease is not well-studied.1,3 References 1. Sano T, Terada T, Hayashi F, et al. Malignant hemangiopericytoma of the liver: report of a case. Jpn J Surg 1991;21:462– 465. 2. Prakash M, Mumtaz HA, Sodhi KS, et al. Hemangiopericytoma: an unusual cause of peritoneal carcinomatosis. Cancer Imaging 2009;9:32–34. 3. Lorigan JG, David CL, Evans HL, et al. The clinical and radiologic manifestations of hemangiopericytoma. AJR Am J Roentgenol 1989;153:345–349.
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of Surgery and 2Department of Pathology, Queen Mary Hospital, The University of Hong Kong, Hong Kong, China
Question: A 56-year-old man presented with epigastric pain of 6 months’ duration. He was a nonsmoker and did not complain of passing tarry stool. The patient had a meningeal tumor resected 16 years ago. On physical examination, the patient was not pale and there was no jaundice. A vague, nonpulsatile, 10-cm mass was found at the epigastrium that moved with respiration. Investigations showed hemoglobin 10.2 g/dL, leukocytes 4.7 ⫻ 109/L, platelets 140 ⫻ 109/L, bilirubin 28 mol/L (reference, ⬍40), aspartate transaminase 34 U/L (reference, 15–38), alanine transaminase 40 U/L (reference, 8 –58), and ␥-glutamyltransferase 26 U/L (reference, 11– 62). He tested negative for hepatitis B virus surface antigen and hepatitis C virus antibody. Tumor markers, including alpha fetoprotein and carcinoembryonic antigen, were not elevated. Upper endoscopy did not review any abnormality. Computed tomography with intravenous contrast was arranged and revealed a large tumor at the left lobe of the liver (Figure A). Liver resection was performed and the postoperative recovery was uneventful. The cut open specimen showed a well encapsulated tumour which was hard in consistency (Figure B). Grossly, the tumor was 16 cm in size with homogeneous component. Figure C, D shows a high-power view of the microscopic sections (stain: hematoxylin and eosin). Figure E, F shows the recticulin staining and CD34 membrane staining at high-power view. What is the diagnosis? See the GASTROENTEROLOGY web site (www.gastrojournal.org) for more information on submitting your favorite image to Clinical Challenges and Images in GI. Conflicts of interest: The authors disclose no conflicts. © 2013 by the AGA Institute 0016-5085/$36.00 http://dx.doi.org/10.1053/j.gastro.2013.01.060
GASTROENTEROLOGY 2013;144:e11– e12
Electronic Clinical Challenges and Images in GI, continued Answer to the Clinical Challenges and Images in GI Question: Image 6: Hemangiopericytoma of the Liver Left hepatectomy was performed. Microscopic examination revealed rare tumor histology. The tumor comprised spindle cells of moderate cellularity and moderate pleomorphism. They were arranged in fascicles among a fibrous stroma. Gapping blood vessels were scattered among the tumor cells (stain: hematoxylin and eosin; original magnification, 100⫻; Figure C). Tumor necrosis was present (stain: hematoxylin and eosin; original magnification, 100⫻; Figure D). Reticulin stain highlighted the contour of each individual tumor cell, suggesting its mesenchymal nature (Figure E). Immunohistochemically, some of the tumor cells expressed CD34 (membranous staining; Figure F). On review, the histology of meningeal and liver tumors was similar. Hence the liver tumor was possibly a metastatic lesion. The diagnosis was hemangiopericytoma of the liver. Hemangiopericytoma is a rare mesenchymal tumor that develops from the pericyte of Zimmermann. Theoretically, it can affect every organ in the body. The more common site for its development is from the lower extremities especially over the thigh.1 It can also present as peritoneal tumors. The incident of this lesion is low and it accounts for ⬍2% of all soft tissue sarcomas.2 Most patients are asymptomatic until the tumors grow to considerable size and lead to local pressure symptoms. It has also been reported that the lesion can be associated with paraneoplastic syndrome leading to hypoglycemia. Contrast-enhanced computed tomography usually shows a heterogeneously enhanced vascular lesion with strong fibrous content inside the lesion. Because the incidence of this tumor is very rare, only histopathology can provide adequate information for diagnosis. Although hepatectomy for resectable disease provides chance for cure, long-term follow-up is required because the natural history this disease is not well-studied.1,3 References 1. Sano T, Terada T, Hayashi F, et al. Malignant hemangiopericytoma of the liver: report of a case. Jpn J Surg 1991;21:462– 465. 2. Prakash M, Mumtaz HA, Sodhi KS, et al. Hemangiopericytoma: an unusual cause of peritoneal carcinomatosis. Cancer Imaging 2009;9:32–34. 3. Lorigan JG, David CL, Evans HL, et al. The clinical and radiologic manifestations of hemangiopericytoma. AJR Am J Roentgenol 1989;153:345–349.
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