Clinical Review & Education
JAMA Surgery Clinical Challenge
Incidentally Discovered Intraperitoneal Masses Mersadies Martin, MD, MS; Hiromichi Ito, MD
A
B
Figure 1. Computed tomographic images. During the trauma workup after a motor vehicle crash, 2 large peritoneal tumors were identified in her upper abdomen. A, An exophilic mass in segment 3 of the liver invading the distal stomach. B, A mass in the lessor sac attaching to the body of pancreas. The arrowheads indicate 2 isolated tumors close to the liver, pancreas, and stomach.
A 20-year-old otherwise healthy woman presented to the emergency department after a motor vehicle crash in which computed tomographic images of the abdomen were obtained as part of the trauma evaluation. Small free fluid within the pelvis and 2 large mesenteric masses were discovered adjacent to the liver, pancreas, and stomach (Figure 1). An endoscopic ultrasound-guided fine-needle aspiration of the abdominal mass was obtained and the diagnosis was made. The patient underwent exploratory laparotomy and en-bloc resection of the intraperitoneal tumors.
jamasurgery.com
WHAT IS YOUR DIAGNOSIS?
A. Non-Hodgkin lymphoma B. Gastrointestinal stromal tumor C. Fibrolamellar hepatocellular carcinoma D. Neuroendocrine tumor of the pancreas
(Reprinted) JAMA Surgery Published online May 6, 2015
Copyright 2015 American Medical Association. All rights reserved.
Downloaded From: http://archsurg.jamanetwork.com/ by a New York University User on 05/11/2015
E1
Clinical Review & Education JAMA Surgery Clinical Challenge
Diagnosis C. Fibrolamellar hepatocellular carcinoma (FL-HCC)
Discussion The patient had 2 isolated tumors without signs of diffuse carcinomatosis that were successfully removed by partial hepatectomy with distal gastrectomy and distal pancreatosplenectomy (Figure 2). The microscopic examination confirmed moderately differentiated FL-HCC invading the visceral peritoneum and omentum. The mass in the lessor sac was the isolated lymph node metastasis without histological evidence of invasion to the pancreas. All resection margin findings were negative for exposure of cancer. Fibrolamellar hepatocellular carcinoma is a rare entity of liver primary malignancy. In the United States, the incidence of FL-HCC is 0.02 per 100 000 per year and it is approximately 100 times less common than conventional non-FL HCC. 1,2 Unlike non-FL HCC, FL-HCC often affects younger patients without underlying chronic liver disease. In addition, FL-HCC constitutes one of the major liver primary tumors among pediatric and young adult patients. 3 Patients with FL-HCC typically present with nonspecific abdominal pain/discomfort or may not present with any symptoms and the tumor(s) may be discovered incidentally during workup of an unrelated medical condition, as with our patient. The histological diagnosis can be made preoperatively by fine-needle aspiration or core needle biopsy. Although the current data regarding the natural history and inherited biology of FL-HCC are limited, aggressive surgical resection has been recommended even for patients with advanced disease given its favorable long-term outcome. Mavros et al3 reviewed 19 previously reported case series including 206 patients with FL-HCC and demonstrated good long-term outcomes of patients who underwent surgical resection, with 18.5 years of median overall survival. Notably, in a series of 41 patients with FL-HCC who underwent
Figure 2. Intraoperative photograph of exploratory laparotomy revealing 2 isolated tumors: the 15-cm primary tumor located at segment 3 of the liver invading into the distal stomach and the other 12-cm mass in the lessor sac adhered to the superior border of the pancreas.
resection from the University of Pittsburgh, 37 patients (90%) had stage IVA or IVB disease and their 5-year overall survival rates were still 66%, and 50%, respectively.4 On the other hand, however, the recurrence of FL-HCC after complete resection is not uncommon. In another series, the recurrence-free survival was reported as only 18% at 5 years and the sites of recurrence included the liver, lymph nodes, peritoneum, and lung.2 In fact, most long-term survivors underwent repeat resection(s) of recurrent disease in these series.2,4 There is no consensus for the role of adjuvant chemotherapy and the optimal regimens for FL-HCC owing to the paucity of this disease. Our patient competed gemcitabine-based adjuvant chemotherapy and the recurrence was discovered at 6 months from the initial resection. She underwent repeat hepatectomy and is currently disease free.
ARTICLE INFORMATION
Conflict of Interest Disclosures: None reported.
Author Affiliations: Department of Surgery, Michigan State University, College of Human Medicine, East Lansing.
REFERENCES
Corresponding Author: Hiromichi Ito, MD, Department of Surgery, Michigan State University, College of Human Medicine, 1200 E Michigan Ave, Ste 655, Lansing, MI 48912 (hiromichi.ito @hc.msu.edu). Section Editor: Pamela A. Lipsett, MD, MHPE.
1. El-Serag HB, Davila JA. Is fibrolamellar carcinoma different from hepatocellular carcinoma? a US population-based study. Hepatology. 2004;39(3): 798-803.
3. Mavros MN, Mayo SC, Hyder O, Pawlik TM. A systematic review: treatment and prognosis of patients with fibrolamellar hepatocellular carcinoma. J Am Coll Surg. 2012;215(6):820-830. 4. Pinna AD, Iwatsuki S, Lee RG, et al. Treatment of fibrolamellar hepatoma with subtotal hepatectomy or transplantation. Hepatology. 1997;26(4):877-883.
2. Stipa F, Yoon SS, Liau KH, et al. Outcome of patients with fibrolamellar hepatocellular carcinoma. Cancer. 2006;106(6):1331-1338.
Published Online: May 6, 2015. doi:10.1001/jamasurg.2015.0260.
E2
JAMA Surgery Published online May 6, 2015 (Reprinted)
Copyright 2015 American Medical Association. All rights reserved.
Downloaded From: http://archsurg.jamanetwork.com/ by a New York University User on 05/11/2015
jamasurgery.com
JAMA Surgery Clinical Challenge
Incidentally Discovered Intraperitoneal Masses Mersadies Martin, MD, MS; Hiromichi Ito, MD
A
B
Figure 1. Computed tomographic images. During the trauma workup after a motor vehicle crash, 2 large peritoneal tumors were identified in her upper abdomen. A, An exophilic mass in segment 3 of the liver invading the distal stomach. B, A mass in the lessor sac attaching to the body of pancreas. The arrowheads indicate 2 isolated tumors close to the liver, pancreas, and stomach.
A 20-year-old otherwise healthy woman presented to the emergency department after a motor vehicle crash in which computed tomographic images of the abdomen were obtained as part of the trauma evaluation. Small free fluid within the pelvis and 2 large mesenteric masses were discovered adjacent to the liver, pancreas, and stomach (Figure 1). An endoscopic ultrasound-guided fine-needle aspiration of the abdominal mass was obtained and the diagnosis was made. The patient underwent exploratory laparotomy and en-bloc resection of the intraperitoneal tumors.
jamasurgery.com
WHAT IS YOUR DIAGNOSIS?
A. Non-Hodgkin lymphoma B. Gastrointestinal stromal tumor C. Fibrolamellar hepatocellular carcinoma D. Neuroendocrine tumor of the pancreas
(Reprinted) JAMA Surgery Published online May 6, 2015
Copyright 2015 American Medical Association. All rights reserved.
Downloaded From: http://archsurg.jamanetwork.com/ by a New York University User on 05/11/2015
E1
Clinical Review & Education JAMA Surgery Clinical Challenge
Diagnosis C. Fibrolamellar hepatocellular carcinoma (FL-HCC)
Discussion The patient had 2 isolated tumors without signs of diffuse carcinomatosis that were successfully removed by partial hepatectomy with distal gastrectomy and distal pancreatosplenectomy (Figure 2). The microscopic examination confirmed moderately differentiated FL-HCC invading the visceral peritoneum and omentum. The mass in the lessor sac was the isolated lymph node metastasis without histological evidence of invasion to the pancreas. All resection margin findings were negative for exposure of cancer. Fibrolamellar hepatocellular carcinoma is a rare entity of liver primary malignancy. In the United States, the incidence of FL-HCC is 0.02 per 100 000 per year and it is approximately 100 times less common than conventional non-FL HCC. 1,2 Unlike non-FL HCC, FL-HCC often affects younger patients without underlying chronic liver disease. In addition, FL-HCC constitutes one of the major liver primary tumors among pediatric and young adult patients. 3 Patients with FL-HCC typically present with nonspecific abdominal pain/discomfort or may not present with any symptoms and the tumor(s) may be discovered incidentally during workup of an unrelated medical condition, as with our patient. The histological diagnosis can be made preoperatively by fine-needle aspiration or core needle biopsy. Although the current data regarding the natural history and inherited biology of FL-HCC are limited, aggressive surgical resection has been recommended even for patients with advanced disease given its favorable long-term outcome. Mavros et al3 reviewed 19 previously reported case series including 206 patients with FL-HCC and demonstrated good long-term outcomes of patients who underwent surgical resection, with 18.5 years of median overall survival. Notably, in a series of 41 patients with FL-HCC who underwent
Figure 2. Intraoperative photograph of exploratory laparotomy revealing 2 isolated tumors: the 15-cm primary tumor located at segment 3 of the liver invading into the distal stomach and the other 12-cm mass in the lessor sac adhered to the superior border of the pancreas.
resection from the University of Pittsburgh, 37 patients (90%) had stage IVA or IVB disease and their 5-year overall survival rates were still 66%, and 50%, respectively.4 On the other hand, however, the recurrence of FL-HCC after complete resection is not uncommon. In another series, the recurrence-free survival was reported as only 18% at 5 years and the sites of recurrence included the liver, lymph nodes, peritoneum, and lung.2 In fact, most long-term survivors underwent repeat resection(s) of recurrent disease in these series.2,4 There is no consensus for the role of adjuvant chemotherapy and the optimal regimens for FL-HCC owing to the paucity of this disease. Our patient competed gemcitabine-based adjuvant chemotherapy and the recurrence was discovered at 6 months from the initial resection. She underwent repeat hepatectomy and is currently disease free.
ARTICLE INFORMATION
Conflict of Interest Disclosures: None reported.
Author Affiliations: Department of Surgery, Michigan State University, College of Human Medicine, East Lansing.
REFERENCES
Corresponding Author: Hiromichi Ito, MD, Department of Surgery, Michigan State University, College of Human Medicine, 1200 E Michigan Ave, Ste 655, Lansing, MI 48912 (hiromichi.ito @hc.msu.edu). Section Editor: Pamela A. Lipsett, MD, MHPE.
1. El-Serag HB, Davila JA. Is fibrolamellar carcinoma different from hepatocellular carcinoma? a US population-based study. Hepatology. 2004;39(3): 798-803.
3. Mavros MN, Mayo SC, Hyder O, Pawlik TM. A systematic review: treatment and prognosis of patients with fibrolamellar hepatocellular carcinoma. J Am Coll Surg. 2012;215(6):820-830. 4. Pinna AD, Iwatsuki S, Lee RG, et al. Treatment of fibrolamellar hepatoma with subtotal hepatectomy or transplantation. Hepatology. 1997;26(4):877-883.
2. Stipa F, Yoon SS, Liau KH, et al. Outcome of patients with fibrolamellar hepatocellular carcinoma. Cancer. 2006;106(6):1331-1338.
Published Online: May 6, 2015. doi:10.1001/jamasurg.2015.0260.
E2
JAMA Surgery Published online May 6, 2015 (Reprinted)
Copyright 2015 American Medical Association. All rights reserved.
Downloaded From: http://archsurg.jamanetwork.com/ by a New York University User on 05/11/2015
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