CLINICAL CHALLENGES AND IMAGES IN GI Abdominal Pain Caused by Spontaneous Rupture of a Liver Tumor Run-De Jiang, Zong-Li Zhang, and Tao Li Department of General Surgery, Qilu Hospital, Shandong University, Jinan, P.R. China
Question: A 47-year-old man was admitted to our hospital with an 8-hour history of intermittent abdominal pain that had developed suddenly. He had a history of liver cirrhosis and chronic hepatitis B virus infection for 10 years. Physical examination revealed tenderness of the right abdomen. His liver function was abnormal: g-glutamyltransferase, 110U/L (normal range, 10-60), alkaline phosphatase, 135U/L (normal range, 45-125), glutamate dehydrogenase, 22.4U/L (normal range, 0-7.4), prealbumin, 11.7 mg/dL (normal range, 17-40). Other laboratory test results, including tumor marker of alpha-fetoprotein, carcinoembryonic antigen, and carbohydrate antigen were all within normal ranges. CT revealed a large, heterogeneous mass occupying the right lobe of the liver, with hemoperitoneum around the liver. The lesion showed a heterogeneous enhancement in the arterial phase and a 4-cm area of calcification was detected in the lesion (Figure A). Rupture of the liver tumor was suspected and operative exploration was undertaken. Approximately 300 mL of old hematocele was found around the cirrhotic liver and the tumor was palpated in the right lobe. A right hepatic lobectomy was performed and the patient recovered uneventfully. The patient refused any adjuvant chemotherapy and is still alive without recurrence 3 months after surgery. Pathologic and histologic examination was performed (Figure B). Pathologic examination revealed an 11 10 8-cm, nonencapsulated, tan–gray, heterogenous mass with areas of focal calcification, hemorrhage, and necrosis. Postoperative bone scintigraphy demonstrated no skeletal lesion (Figure C). What is the diagnosis of this tumor? Look on page 36 for the answer and see the Gastroenterology web site (www.gastrojournal.org) for more information on submitting your favorite image to Clinical Challenges and Images in GI.
Acknowledgments Address correspondence to: Tao Li, Associate professor, Department of General Surgery, Qilu Hospital, Shandong University, 107 Wen Hua Xi Road Jinan, 250012, People’s Republic of China. e-mail: [email protected]. Conflicts of interest The authors disclose no conflicts. Funding This work was supported by the Major Program of Shandong Provincial Natural Science Foundation (Grant No. ZR2014HZ002). © 2015 by the AGA Institute 0016-5085/$36.00 http://dx.doi.org/10.1053/j.gastro.2015.02.026
Gastroenterology 2015;149:35–36
CLINICAL CHALLENGES AND IMAGES IN GI Answer to: Image 3 (page 35): Primary Hepatic Osteosarcoma
Histologic examination showed diffuse proliferation of undifferentiated polygonal or spindle-shaped malignant stromal cells, which were admixed with abundant eosinophilic osteoids arranged in a lace-like pattern (Figure B). Immunohistochemistry showed negative expression of alpha-fetoprotein, hepatocyte, cytokeratin, and epithelial membrane antigen, whereas expression of vimentin and S-100 was positive. In addition, a mitotic rate about 30% staining for proliferative marker Ki-67 was detected (Figure D). The diagnosis of primary hepatic osteosarcoma (PHO) was confirmed. Osteosarcomas usually arise from skeletal lesions, and extraosseous osteosarcomas are rare, accounting for approximately 4% of all osteosarcomas. Extraosseous osteosarcomas are most being described in the limbs and limb girdles.1 Even fewer are reports of parenchymal osteosarcoma, which have been documented in thyroid, kidney, and breast.2 PHO is extremely uncommon, with only 7 cases reported in the English-language literature. Including our case, the majority of PHO occurred in males >50 years old, and one-half of the PHO had underlying liver cirrhosis. Sumiyoshi and Niho3 suggested the possible mutability or tumorigenesis of the proliferating mesenchymal tissue in the cirrhotic liver. However, the pathogenesis of PHO remains unknown. Before diagnosing PHO, a carcinosarcoma or malignant mesenchymoma with an osteosarcomatous component must be ruled out. In addition, the possibility of metastasis from osseous osteosarcomas should also be excluded. Although the histopathologic features of PHO are similar to those of osseous osteosarcomas, PHO should be regarded as a separate disease that is clinically and therapeutically distinct from osseous osteosarcoma, for its dismal prognosis and poor response to multimodality therapy.1 PHO is characterized by large size and aggressive behavior, and local recurrence and distant metastasis were common after excision. Tumor size has been reported the main prognostic factor in extraosseous osteosarcoma. Although chemotherapy was not effective for patients with advanced disease, operative resection followed by chemoradiation therapy seemed to be the most effective therapy.1
References 1. 2. 3.
Sordillo PP, Hajdu SI, Magill GB, et al. Extraosseous osteogenic sarcoma. A review of 48 patients. Cancer 1983; 51:727–734. Nawabi A, Rath S, Nissen N, et al. Primary hepatic osteosarcoma. J Gastrointest Surg 2009;13:1550–1553. Sumiyoshi A, Niho Y. Primary osteogenic sarcoma of the liver–report of an autopsy case. Acta Pathol Jpn 1971; 21:305–312.
For submission instructions, please see the Gastroenterology web site (www.gastrojournal.org).
36
Question: A 47-year-old man was admitted to our hospital with an 8-hour history of intermittent abdominal pain that had developed suddenly. He had a history of liver cirrhosis and chronic hepatitis B virus infection for 10 years. Physical examination revealed tenderness of the right abdomen. His liver function was abnormal: g-glutamyltransferase, 110U/L (normal range, 10-60), alkaline phosphatase, 135U/L (normal range, 45-125), glutamate dehydrogenase, 22.4U/L (normal range, 0-7.4), prealbumin, 11.7 mg/dL (normal range, 17-40). Other laboratory test results, including tumor marker of alpha-fetoprotein, carcinoembryonic antigen, and carbohydrate antigen were all within normal ranges. CT revealed a large, heterogeneous mass occupying the right lobe of the liver, with hemoperitoneum around the liver. The lesion showed a heterogeneous enhancement in the arterial phase and a 4-cm area of calcification was detected in the lesion (Figure A). Rupture of the liver tumor was suspected and operative exploration was undertaken. Approximately 300 mL of old hematocele was found around the cirrhotic liver and the tumor was palpated in the right lobe. A right hepatic lobectomy was performed and the patient recovered uneventfully. The patient refused any adjuvant chemotherapy and is still alive without recurrence 3 months after surgery. Pathologic and histologic examination was performed (Figure B). Pathologic examination revealed an 11 10 8-cm, nonencapsulated, tan–gray, heterogenous mass with areas of focal calcification, hemorrhage, and necrosis. Postoperative bone scintigraphy demonstrated no skeletal lesion (Figure C). What is the diagnosis of this tumor? Look on page 36 for the answer and see the Gastroenterology web site (www.gastrojournal.org) for more information on submitting your favorite image to Clinical Challenges and Images in GI.
Acknowledgments Address correspondence to: Tao Li, Associate professor, Department of General Surgery, Qilu Hospital, Shandong University, 107 Wen Hua Xi Road Jinan, 250012, People’s Republic of China. e-mail: [email protected]. Conflicts of interest The authors disclose no conflicts. Funding This work was supported by the Major Program of Shandong Provincial Natural Science Foundation (Grant No. ZR2014HZ002). © 2015 by the AGA Institute 0016-5085/$36.00 http://dx.doi.org/10.1053/j.gastro.2015.02.026
Gastroenterology 2015;149:35–36
CLINICAL CHALLENGES AND IMAGES IN GI Answer to: Image 3 (page 35): Primary Hepatic Osteosarcoma
Histologic examination showed diffuse proliferation of undifferentiated polygonal or spindle-shaped malignant stromal cells, which were admixed with abundant eosinophilic osteoids arranged in a lace-like pattern (Figure B). Immunohistochemistry showed negative expression of alpha-fetoprotein, hepatocyte, cytokeratin, and epithelial membrane antigen, whereas expression of vimentin and S-100 was positive. In addition, a mitotic rate about 30% staining for proliferative marker Ki-67 was detected (Figure D). The diagnosis of primary hepatic osteosarcoma (PHO) was confirmed. Osteosarcomas usually arise from skeletal lesions, and extraosseous osteosarcomas are rare, accounting for approximately 4% of all osteosarcomas. Extraosseous osteosarcomas are most being described in the limbs and limb girdles.1 Even fewer are reports of parenchymal osteosarcoma, which have been documented in thyroid, kidney, and breast.2 PHO is extremely uncommon, with only 7 cases reported in the English-language literature. Including our case, the majority of PHO occurred in males >50 years old, and one-half of the PHO had underlying liver cirrhosis. Sumiyoshi and Niho3 suggested the possible mutability or tumorigenesis of the proliferating mesenchymal tissue in the cirrhotic liver. However, the pathogenesis of PHO remains unknown. Before diagnosing PHO, a carcinosarcoma or malignant mesenchymoma with an osteosarcomatous component must be ruled out. In addition, the possibility of metastasis from osseous osteosarcomas should also be excluded. Although the histopathologic features of PHO are similar to those of osseous osteosarcomas, PHO should be regarded as a separate disease that is clinically and therapeutically distinct from osseous osteosarcoma, for its dismal prognosis and poor response to multimodality therapy.1 PHO is characterized by large size and aggressive behavior, and local recurrence and distant metastasis were common after excision. Tumor size has been reported the main prognostic factor in extraosseous osteosarcoma. Although chemotherapy was not effective for patients with advanced disease, operative resection followed by chemoradiation therapy seemed to be the most effective therapy.1
References 1. 2. 3.
Sordillo PP, Hajdu SI, Magill GB, et al. Extraosseous osteogenic sarcoma. A review of 48 patients. Cancer 1983; 51:727–734. Nawabi A, Rath S, Nissen N, et al. Primary hepatic osteosarcoma. J Gastrointest Surg 2009;13:1550–1553. Sumiyoshi A, Niho Y. Primary osteogenic sarcoma of the liver–report of an autopsy case. Acta Pathol Jpn 1971; 21:305–312.
For submission instructions, please see the Gastroenterology web site (www.gastrojournal.org).
36
