Available online at www.sciencedirect.com
ScienceDirect EJSO 40 (2014) 1572e1577
www.ejso.com
Solid pseudopapillary tumor of the pancreas with liver metastasis: Clinical features and management Wei-Bin Wang a, Tai-Ping Zhang a, Meng-Qing Sun, Zheng Peng, Ge Chen, Yu-Pei Zhao* Department of General Surgery, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing, People’s Republic of China Accepted 22 May 2014 Available online 12 June 2014
Abstract Objective: To summarize the diagnosis and management of metastatic pancreatic solid pseudopapillary tumors (SPTs). Methods: We retrospectively analyzed the clinical data of patients with metastatic pancreatic SPTs who were admitted to Peking Union Medical College Hospital from November 2001 to September 2013. Results: A total of 187 patients with pancreatic SPTs were admitted. Four patients had liver metastasis, and all four were female patients aged 20e72 years old. Each patient with metastases underwent surgical resection with good postoperative recovery. The mean follow-up period was 30 months (range, 1e64 months). None of the patients had obvious recurrence or distant metastasis. Conclusions: Pancreatic SPT with liver metastasis is very rare, and surgical resection is an effective treatment option. The principle of surgical treatment is to resect the primary and metastatic lesions as completely as possible. The affected patients require long-term postoperative follow-up. Ó 2014 Elsevier Ltd. All rights reserved. Keywords: Pancreas; Pancreatic solid pseudopapillary tumor; Liver metastasis
Introduction Pancreatic solid pseudopapillary tumors (SPTs) are rare pancreatic tumors with low-grade malignancy and were first reported by Frantz in 1959.1 The pathogenesis and tissue origin of pancreatic SPT remain unclear. This tumor accounts for 1%e2% of all pancreatic tumors and 13% of surgically resected pancreatic cystic tumors.2e4 Pancreatic SPT is an epithelial neoplasm consisting of discohesive polygonal cells that surround delicate blood vessels and form a solid mass as well as pseudopapillary structures formed by morphologically consistent cells; hemorrhage and cystic degeneration are also present. It usually occurs in young female patients with a median age of 20e30 years
old, and the male:female ratio is 1:10. SPT can be located in any part of the pancreas; extrapancreatic SPT is extremely rare. Most patients have no specific clinical symptoms or signs, and tumor markers are usually within the normal ranges.3,5 Pancreatic SPT can also be easily misdiagnosed. Metastasis of SPT is very rare. The most common metastatic site is the liver, and when metastasis occurs, it often results in death.6,7 We reviewed the medical records of patients with SPTs with liver metastasis who were admitted to Peking Union Medical College Hospital from November 2001 to September 2013, and we summarize herein the clinical manifestations and management of this condition. Materials and methods
* Corresponding author. Department of General Surgery, Peking Union Medical College Hospital, Chinese Academy of Medical Science and Peking Union Medical College, No. 1 Shuai Fu Yuan, Dongcheng District, Beijing 100730, People’s Republic of China. Tel.: þ86 10 69152600; fax: þ86 10 69155818. E-mail address: [email protected] (Y.-P. Zhao). a W.-B. Wang and T. Zhang contributed equally to this work. 0748-7983/$ - see front matter Ó 2014 Elsevier Ltd. All rights reserved. http://dx.doi.org/10.1016/j.ejso.2014.05.012
General data From November 2001 to September 2013, a total of 187 patients with SPTs were admitted to Peking Union Medical College Hospital (male:female ratio, 28:159 [1.0:5.7]), and
W.-B. Wang et al. / EJSO 40 (2014) 1572e1577
the median patient age was 30 years old (range, 12e72 years). Four patients (2.1%) had liver metastasis. All four patients with metastasis were female, aged 20e72 years old. The time at which liver metastasis was discovered ranged from the first clinical visit to 13 years postoperation. All four patients underwent surgery; all of them experienced good postoperative recovery and were followed up for 1e64 months (mean, 30 months). All of the patients are currently alive and tumor-free. The respective Ethics Committees approved the collection of these materials. Written informed consent was obtained from these patients. Results Case 1 A 50-year-old woman had been diagnosed with a malignant islet-cell tumor in the head of the pancreas 13 years previously. At that time, she underwent duodenumpreserving pancreatic head resection, Roux-en-Y pancreaticojejunostomy, common bile duct exploration, and T-tube drainage at a local hospital. Her postoperative recovery was good. Her elder sister had breast cancer. Six years prior to the present admission, the patient developed upper abdominal pain and an elevated blood amylase level. Abdominal computed tomography (CT) suggested a mass in the pancreatic area. She was diagnosed with an inflammatory pseudotumor at a local hospital. Abdominal CT showed a 3.5- 5.0-cm irregular mass in the head of the pancreas with a poorly defined margin relative to the surrounding tissue and CT values of 36.2 and 60.0 HU before and after enhancement, respectively. The mass lacked uniform density. The abdominal CT findings suggested tumor recurrence. The patient underwent surgical resection of the pancreatic uncinate process and duodenum, as well as cholecystectomy, cholangiojejunostomy, and gastrojejunostomy. A 5- 6- 5-cm mass was found in the pancreatic head. Consultation with a pathologist after the first operation in the local hospital indicated that the vast majority of the pancreatic tumor tissue was necrotic; only a few tumor cells were present at the edges. The lesion was consistent with an SPT. The pathological examination findings after the second operation were also consistent with a pancreatic SPT that was invading the submucosa and muscular wall of the duodenum. Lymph node metastasis was observed around the small intestine (1/4). The immunohistochemistry results were as follows: CgA(), vimentin(þ), PP focal(þ), AACT(þ) and AAT(þ). Six years after the second operation, the patient was found to have multiple lesions in the liver. Abdominal CT revealed three irregularly shaped lesions with clear borders in the liver. The lesion in the left lobe had the largest crosssection, measuring 8.3 6.6 cm and demonstrating uneven density and heterogeneous enhancement. The other lesion
1573
in the right lobe had the largest section, measuring 7.9 6.5 cm and demonstrating uneven density and mild enhancement of the solid component. The cystic component had no enhancement. The smaller lesion in the right lobe was 4.1 2.2 cm with mild to moderate enhancement. There were multiple lymph nodes in the hepatic hilum, retroperitoneum, and mesenteric root. The imaging findings indicated the presence of liver lesions and possibly metastasis. Positron-emission tomography revealed that the liver was enlarged and contained three low-density lesions in the left and right lobes (2.5 3.0 2.0 cm, 5.5 7.1 6.0 cm, and 8.1 5.3 6.5 cm, respectively). The radioactivity uptake was abnormal and unevenly increased, with standardized uptake values of 3.2, 2.0, and 2.8, respectively, in the three lesions. The central parts of the lesions had reduced uptake. The positron-emission tomography findings indicated the presence of multiple lesions with increased metabolism in the liver and possible metastasis. The patient underwent surgical resection of all three liver masses. The pathological findings were as follows: pancreatic SPT in the liver, vimentin(þ), AE1/ AE3(), CgA(), and Syn(). There was no recurrence for 23 months after the operation. Case 2 Six years ago, a 72-year-old woman was diagnosed with a pancreatic mass. Abdominal ultrasound revealed a 3.78 2.80-cm hypoechoic lesion in the pancreatic tail, with clear borders and no blood flow. There was no expansion of the main pancreatic duct. The B-mode ultrasound findings indicated a mass in the pancreatic tail, and cancer could not be excluded. Abdominal CT revealed a 4.1 2.5-cm solid mass in the pancreatic body consistent with an SPT, with a fairly clear border and CT values of 36 and 62 HU before and after enhancement, respectively. Magnetic resonance imaging revealed a 3- 3- 2-cm cystic mass in the pancreatic body. The patient had no fever or jaundice. She had experienced intermittent diarrhea for the past 2 years, characterized by watery stools once or twice daily. She also had a 4-year history of diabetes. Her mother had died of breast cancer. The patient’s diagnosis on admission was considered to be either a pancreatic cystadenoma or pancreatic cancer. She underwent resection of the pancreatic body and tail with splenectomy. A 3.5- 3.0- 3.0-cm tumor with an uneven surface and soft texture was located in the pancreatic body. There were signs of hemorrhage in the tumor. Pathological examination confirmed a pancreatic SPT. Six years after the operation, the patient was found to have a mass in the liver and lesser omentum. Abdominal ultrasound revealed multiple hypoechoic lesions in the pancreatic head. The largest was 4.5 3.9 cm with an unclear border. Pancreatic magnetic resonance imaging showed multiple masses and nodules in the hepatogastric gap, consistent with enlarged lymph nodes. A low-density
1574
W.-B. Wang et al. / EJSO 40 (2014) 1572e1577
lesion was present in segment III, and a mass was present in segment VIII of the liver. An abdominal CT in May 2013 revealed small nodules in the left lobe of the liver. Metastatic lesions could not be excluded. The lymph nodes in the lesser curvature of the stomach were enlarged and fused, enveloping the left gastric artery. An abdominal CT in September 2013 revealed a mass of soft tissue density between the left lobe of the liver and lesser curvature of the stomach, which was slightly larger than previously shown. Nodules were present in the lateral segment of the left hepatic lobe and in the left adrenal gland, which were also slightly larger than previously shown, and were considered to be probable metastatic lesions. During surgery, we found a 1.5-cm, dark red nodule located in the lateral part of the left hepatic lobe, on the edge of the liver near the hepatic round ligament. The nodule was cystic, solid, and soft in texture. There was a 0.5-cm mass in the anterior wall of the stomach near the greater curvature. The nodule was gray-red and soft. A 10- 8- 8-cm mass with multiple fusions was found within and invading through the lesser omentum; it was dark red and gray and had a mixed cystic and solid composition. There were carrion-like tumors and blood clots in the omental foramen just near the first hepatic hilum, invading the stomach serosa and surrounding the left gastric artery. The patient underwent resection of the masses located in the left hepatic lobe, hepatogastric gap, and stomach wall. Pathological examination revealed liver metastasis of a pancreatic SPT. No recurrence was present 1 month after the operation. Case 3 A 20-year-old woman had undergone resection of a pancreatic mass 10 years before admission and was found to have an abdominal mass 4 years before admission. Ten years before the present admission, the patient presented at Peking Children’s Hospital because of intermittent abdominal pain. Abdominal ultrasound revealed a pancreatic mass of approximately 10 cm. She underwent resection of the pancreatic body and tail with splenectomy. The pathological findings after the operation were consistent with a pancreatic SPT. Four years before the present admission, she developed persistent epigastric pain with a fever. Ultrasound revealed an abdominal mass, and liver biopsy revealed a low-grade malignant tumor. The patient underwent treatment with a focused ultrasound knife. Ultrasound at Peking Union Medical College Hospital showed multiple anechoic lesions in the liver. The largest was located in the right lobe, measuring 7.4 8.8 cm, and had a clear border, irregular shape, and separations; part of the lesion demonstrated poor sound transmission with no blood flow signals. A moderately echoic to hyperechoic lesion with a clear border was present in the right anterior lobe. A 15.5- 13.9 8.3-cm lesion with a clear border, separations, and slight
blood flow was also located in the upper abdomen. Overall, ultrasound showed multiple cystic-solid masses in the liver; a moderately echoic to hyperechoic lesion in the right hepatic lobe, for which hemangioma could not be excluded; and a cystic-solid mass in the upper abdomen. CT revealed an abdominal mass (15 12 cm) and multiple cystic-solid masses in the liver. Therefore, her diagnosis on admission was an intra-abdominal mass with multiple liver metastases. The patient underwent retroperitoneal tumor resection with combined hepatic segmental resection. The tumor occupied the entire left upper abdomen and measured 15 12 cm. The root of the tumor originated from the small intestinal mesentery of the duodenojejunum junction. The tumor protruded from the retroperitoneum into the abdominal cavity along the root of the mesentery. There were multiple liver metastatic lesions of varying sizes, the largest being 5 cm. The pathological and immunohistochemical findings were as follows: retroperitoneal pancreatic SPT with left-lobe liver metastasis, a-AT(þ), AFP(þ), vimentin(þ), AE1/AE3(), CgA(), Syn(þ), and calretinin(partiallyþ). No recurrence was present 64 months after surgery. Case 4 A 22-year-old woman had experienced epigastric bloating after meals for 6 months and was found to have a mass in the right upper quadrant of the abdomen 1.5 months before admission. She found local swelling on her right upper abdominal quadrant while bathing. Abdominal CT at a local hospital showed a pancreatic mass and multiple liver masses. She underwent needle biopsy; the pathological findings suggested SPT, and rapid intraoperative pathology was recommended. Thus, her diagnosis on admission was a possible pancreatic SPT with liver metastasis. Abdominal ultrasound showed multiple moderately to strongly hyperechoic lesions, the largest being 7.0 5.7 cm with fairly clear borders, uneven echogenicity, and no blood flow. A heteroechoic lesion with a clear border and measuring 13.8 11.7 9.6 cm was present in the pancreatic head. There seemed to be a capsule around the mass. The lesion was mainly moderately echoic to hypoechoic, with several patches of anechoic areas, some of which had mesh-like hypoechoic separations. The main duct was 0.45 cm in width. There were multiple moderately echoic to hyperechoic lesions in the liver, and a hemangioma could not be excluded. A cystic-solid mass was located in the pancreatic head, and the presence of an SPT was considered. The main pancreatic duct was dilated. The CT results are shown in Figs. 1 and 2. A soft tissue mass was present in the area of the pancreatic head (largest cross-section, 10.7 9.7 cm). The lesion was of uneven density, similar to the liver lesions. The lesion had dot calcifications and was heterogeneously enhanced. The pancreatic duct was dilated. The duodenum was compressed and
W.-B. Wang et al. / EJSO 40 (2014) 1572e1577
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Figure 1. Multiple soft tissue masses in liver and in pancreatic head. A. Multiple variously sized lesions in the liver with uneven and slightly low density, smooth edges, and uneven enhancement, the largest section measured 7.0 5.7 cm; B. Pancreatic head huge mass, largest cross section was 10.7 9.7 cm, this lesion was uneven density.
displaced forward; the right kidney was displaced backward, the portal vein upward, and the superior mesenteric vein leftward. The superior and inferior pancreaticoduodenal arteries supplied blood to the lesion. An SPT was considered possible. There were multiple variously sized lesions in the liver with uneven and slightly low density, smooth edges, and uneven enhancement. The hepatic vein was compressed. There were multiple lymph nodes in the retroperitoneal area, some of which were enlarged. The patient underwent pancreatoduodenectomy and resection of the multiple liver lesions. We found a 15 10- 8-cm mass with an unclear border located in the uncinate process of the pancreas, and it was closely associated with the superior mesenteric vein. The pancreatic duct was 6 mm in diameter. Seven lesions in the liver were resected (Fig. 3). Pathological examination revealed a pancreatic SPT. The common bile duct and small intestine were
Figure 2. Specimen of pancreatoduodenectomy, including distal stomach, pancreatic head with that huge mass (largest cross section 10.7 9.7 cm), duodenum and proximal jejunum.
not involved. The edges of the pancreas, common bile duct, stomach, and small intestine showed no abnormalities. No lymph node metastasis was observed. The liver was determined to contain a metastatic pancreatic SPT. The immunohistochemistry results were as follows: AACT(þ), AAT(þ), AEI/AE3(þ), CD10(þ), CD56(NK1)(þ), CgA(), Syn(), vimentin(þ), B-catenin(þ), Ki67 index 5 cm, local infiltration at the first operation, metastasis, and other factors.21,22 However, disagreement persists.6 With regard to age, two patients in the present report were
ScienceDirect EJSO 40 (2014) 1572e1577
www.ejso.com
Solid pseudopapillary tumor of the pancreas with liver metastasis: Clinical features and management Wei-Bin Wang a, Tai-Ping Zhang a, Meng-Qing Sun, Zheng Peng, Ge Chen, Yu-Pei Zhao* Department of General Surgery, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing, People’s Republic of China Accepted 22 May 2014 Available online 12 June 2014
Abstract Objective: To summarize the diagnosis and management of metastatic pancreatic solid pseudopapillary tumors (SPTs). Methods: We retrospectively analyzed the clinical data of patients with metastatic pancreatic SPTs who were admitted to Peking Union Medical College Hospital from November 2001 to September 2013. Results: A total of 187 patients with pancreatic SPTs were admitted. Four patients had liver metastasis, and all four were female patients aged 20e72 years old. Each patient with metastases underwent surgical resection with good postoperative recovery. The mean follow-up period was 30 months (range, 1e64 months). None of the patients had obvious recurrence or distant metastasis. Conclusions: Pancreatic SPT with liver metastasis is very rare, and surgical resection is an effective treatment option. The principle of surgical treatment is to resect the primary and metastatic lesions as completely as possible. The affected patients require long-term postoperative follow-up. Ó 2014 Elsevier Ltd. All rights reserved. Keywords: Pancreas; Pancreatic solid pseudopapillary tumor; Liver metastasis
Introduction Pancreatic solid pseudopapillary tumors (SPTs) are rare pancreatic tumors with low-grade malignancy and were first reported by Frantz in 1959.1 The pathogenesis and tissue origin of pancreatic SPT remain unclear. This tumor accounts for 1%e2% of all pancreatic tumors and 13% of surgically resected pancreatic cystic tumors.2e4 Pancreatic SPT is an epithelial neoplasm consisting of discohesive polygonal cells that surround delicate blood vessels and form a solid mass as well as pseudopapillary structures formed by morphologically consistent cells; hemorrhage and cystic degeneration are also present. It usually occurs in young female patients with a median age of 20e30 years
old, and the male:female ratio is 1:10. SPT can be located in any part of the pancreas; extrapancreatic SPT is extremely rare. Most patients have no specific clinical symptoms or signs, and tumor markers are usually within the normal ranges.3,5 Pancreatic SPT can also be easily misdiagnosed. Metastasis of SPT is very rare. The most common metastatic site is the liver, and when metastasis occurs, it often results in death.6,7 We reviewed the medical records of patients with SPTs with liver metastasis who were admitted to Peking Union Medical College Hospital from November 2001 to September 2013, and we summarize herein the clinical manifestations and management of this condition. Materials and methods
* Corresponding author. Department of General Surgery, Peking Union Medical College Hospital, Chinese Academy of Medical Science and Peking Union Medical College, No. 1 Shuai Fu Yuan, Dongcheng District, Beijing 100730, People’s Republic of China. Tel.: þ86 10 69152600; fax: þ86 10 69155818. E-mail address: [email protected] (Y.-P. Zhao). a W.-B. Wang and T. Zhang contributed equally to this work. 0748-7983/$ - see front matter Ó 2014 Elsevier Ltd. All rights reserved. http://dx.doi.org/10.1016/j.ejso.2014.05.012
General data From November 2001 to September 2013, a total of 187 patients with SPTs were admitted to Peking Union Medical College Hospital (male:female ratio, 28:159 [1.0:5.7]), and
W.-B. Wang et al. / EJSO 40 (2014) 1572e1577
the median patient age was 30 years old (range, 12e72 years). Four patients (2.1%) had liver metastasis. All four patients with metastasis were female, aged 20e72 years old. The time at which liver metastasis was discovered ranged from the first clinical visit to 13 years postoperation. All four patients underwent surgery; all of them experienced good postoperative recovery and were followed up for 1e64 months (mean, 30 months). All of the patients are currently alive and tumor-free. The respective Ethics Committees approved the collection of these materials. Written informed consent was obtained from these patients. Results Case 1 A 50-year-old woman had been diagnosed with a malignant islet-cell tumor in the head of the pancreas 13 years previously. At that time, she underwent duodenumpreserving pancreatic head resection, Roux-en-Y pancreaticojejunostomy, common bile duct exploration, and T-tube drainage at a local hospital. Her postoperative recovery was good. Her elder sister had breast cancer. Six years prior to the present admission, the patient developed upper abdominal pain and an elevated blood amylase level. Abdominal computed tomography (CT) suggested a mass in the pancreatic area. She was diagnosed with an inflammatory pseudotumor at a local hospital. Abdominal CT showed a 3.5- 5.0-cm irregular mass in the head of the pancreas with a poorly defined margin relative to the surrounding tissue and CT values of 36.2 and 60.0 HU before and after enhancement, respectively. The mass lacked uniform density. The abdominal CT findings suggested tumor recurrence. The patient underwent surgical resection of the pancreatic uncinate process and duodenum, as well as cholecystectomy, cholangiojejunostomy, and gastrojejunostomy. A 5- 6- 5-cm mass was found in the pancreatic head. Consultation with a pathologist after the first operation in the local hospital indicated that the vast majority of the pancreatic tumor tissue was necrotic; only a few tumor cells were present at the edges. The lesion was consistent with an SPT. The pathological examination findings after the second operation were also consistent with a pancreatic SPT that was invading the submucosa and muscular wall of the duodenum. Lymph node metastasis was observed around the small intestine (1/4). The immunohistochemistry results were as follows: CgA(), vimentin(þ), PP focal(þ), AACT(þ) and AAT(þ). Six years after the second operation, the patient was found to have multiple lesions in the liver. Abdominal CT revealed three irregularly shaped lesions with clear borders in the liver. The lesion in the left lobe had the largest crosssection, measuring 8.3 6.6 cm and demonstrating uneven density and heterogeneous enhancement. The other lesion
1573
in the right lobe had the largest section, measuring 7.9 6.5 cm and demonstrating uneven density and mild enhancement of the solid component. The cystic component had no enhancement. The smaller lesion in the right lobe was 4.1 2.2 cm with mild to moderate enhancement. There were multiple lymph nodes in the hepatic hilum, retroperitoneum, and mesenteric root. The imaging findings indicated the presence of liver lesions and possibly metastasis. Positron-emission tomography revealed that the liver was enlarged and contained three low-density lesions in the left and right lobes (2.5 3.0 2.0 cm, 5.5 7.1 6.0 cm, and 8.1 5.3 6.5 cm, respectively). The radioactivity uptake was abnormal and unevenly increased, with standardized uptake values of 3.2, 2.0, and 2.8, respectively, in the three lesions. The central parts of the lesions had reduced uptake. The positron-emission tomography findings indicated the presence of multiple lesions with increased metabolism in the liver and possible metastasis. The patient underwent surgical resection of all three liver masses. The pathological findings were as follows: pancreatic SPT in the liver, vimentin(þ), AE1/ AE3(), CgA(), and Syn(). There was no recurrence for 23 months after the operation. Case 2 Six years ago, a 72-year-old woman was diagnosed with a pancreatic mass. Abdominal ultrasound revealed a 3.78 2.80-cm hypoechoic lesion in the pancreatic tail, with clear borders and no blood flow. There was no expansion of the main pancreatic duct. The B-mode ultrasound findings indicated a mass in the pancreatic tail, and cancer could not be excluded. Abdominal CT revealed a 4.1 2.5-cm solid mass in the pancreatic body consistent with an SPT, with a fairly clear border and CT values of 36 and 62 HU before and after enhancement, respectively. Magnetic resonance imaging revealed a 3- 3- 2-cm cystic mass in the pancreatic body. The patient had no fever or jaundice. She had experienced intermittent diarrhea for the past 2 years, characterized by watery stools once or twice daily. She also had a 4-year history of diabetes. Her mother had died of breast cancer. The patient’s diagnosis on admission was considered to be either a pancreatic cystadenoma or pancreatic cancer. She underwent resection of the pancreatic body and tail with splenectomy. A 3.5- 3.0- 3.0-cm tumor with an uneven surface and soft texture was located in the pancreatic body. There were signs of hemorrhage in the tumor. Pathological examination confirmed a pancreatic SPT. Six years after the operation, the patient was found to have a mass in the liver and lesser omentum. Abdominal ultrasound revealed multiple hypoechoic lesions in the pancreatic head. The largest was 4.5 3.9 cm with an unclear border. Pancreatic magnetic resonance imaging showed multiple masses and nodules in the hepatogastric gap, consistent with enlarged lymph nodes. A low-density
1574
W.-B. Wang et al. / EJSO 40 (2014) 1572e1577
lesion was present in segment III, and a mass was present in segment VIII of the liver. An abdominal CT in May 2013 revealed small nodules in the left lobe of the liver. Metastatic lesions could not be excluded. The lymph nodes in the lesser curvature of the stomach were enlarged and fused, enveloping the left gastric artery. An abdominal CT in September 2013 revealed a mass of soft tissue density between the left lobe of the liver and lesser curvature of the stomach, which was slightly larger than previously shown. Nodules were present in the lateral segment of the left hepatic lobe and in the left adrenal gland, which were also slightly larger than previously shown, and were considered to be probable metastatic lesions. During surgery, we found a 1.5-cm, dark red nodule located in the lateral part of the left hepatic lobe, on the edge of the liver near the hepatic round ligament. The nodule was cystic, solid, and soft in texture. There was a 0.5-cm mass in the anterior wall of the stomach near the greater curvature. The nodule was gray-red and soft. A 10- 8- 8-cm mass with multiple fusions was found within and invading through the lesser omentum; it was dark red and gray and had a mixed cystic and solid composition. There were carrion-like tumors and blood clots in the omental foramen just near the first hepatic hilum, invading the stomach serosa and surrounding the left gastric artery. The patient underwent resection of the masses located in the left hepatic lobe, hepatogastric gap, and stomach wall. Pathological examination revealed liver metastasis of a pancreatic SPT. No recurrence was present 1 month after the operation. Case 3 A 20-year-old woman had undergone resection of a pancreatic mass 10 years before admission and was found to have an abdominal mass 4 years before admission. Ten years before the present admission, the patient presented at Peking Children’s Hospital because of intermittent abdominal pain. Abdominal ultrasound revealed a pancreatic mass of approximately 10 cm. She underwent resection of the pancreatic body and tail with splenectomy. The pathological findings after the operation were consistent with a pancreatic SPT. Four years before the present admission, she developed persistent epigastric pain with a fever. Ultrasound revealed an abdominal mass, and liver biopsy revealed a low-grade malignant tumor. The patient underwent treatment with a focused ultrasound knife. Ultrasound at Peking Union Medical College Hospital showed multiple anechoic lesions in the liver. The largest was located in the right lobe, measuring 7.4 8.8 cm, and had a clear border, irregular shape, and separations; part of the lesion demonstrated poor sound transmission with no blood flow signals. A moderately echoic to hyperechoic lesion with a clear border was present in the right anterior lobe. A 15.5- 13.9 8.3-cm lesion with a clear border, separations, and slight
blood flow was also located in the upper abdomen. Overall, ultrasound showed multiple cystic-solid masses in the liver; a moderately echoic to hyperechoic lesion in the right hepatic lobe, for which hemangioma could not be excluded; and a cystic-solid mass in the upper abdomen. CT revealed an abdominal mass (15 12 cm) and multiple cystic-solid masses in the liver. Therefore, her diagnosis on admission was an intra-abdominal mass with multiple liver metastases. The patient underwent retroperitoneal tumor resection with combined hepatic segmental resection. The tumor occupied the entire left upper abdomen and measured 15 12 cm. The root of the tumor originated from the small intestinal mesentery of the duodenojejunum junction. The tumor protruded from the retroperitoneum into the abdominal cavity along the root of the mesentery. There were multiple liver metastatic lesions of varying sizes, the largest being 5 cm. The pathological and immunohistochemical findings were as follows: retroperitoneal pancreatic SPT with left-lobe liver metastasis, a-AT(þ), AFP(þ), vimentin(þ), AE1/AE3(), CgA(), Syn(þ), and calretinin(partiallyþ). No recurrence was present 64 months after surgery. Case 4 A 22-year-old woman had experienced epigastric bloating after meals for 6 months and was found to have a mass in the right upper quadrant of the abdomen 1.5 months before admission. She found local swelling on her right upper abdominal quadrant while bathing. Abdominal CT at a local hospital showed a pancreatic mass and multiple liver masses. She underwent needle biopsy; the pathological findings suggested SPT, and rapid intraoperative pathology was recommended. Thus, her diagnosis on admission was a possible pancreatic SPT with liver metastasis. Abdominal ultrasound showed multiple moderately to strongly hyperechoic lesions, the largest being 7.0 5.7 cm with fairly clear borders, uneven echogenicity, and no blood flow. A heteroechoic lesion with a clear border and measuring 13.8 11.7 9.6 cm was present in the pancreatic head. There seemed to be a capsule around the mass. The lesion was mainly moderately echoic to hypoechoic, with several patches of anechoic areas, some of which had mesh-like hypoechoic separations. The main duct was 0.45 cm in width. There were multiple moderately echoic to hyperechoic lesions in the liver, and a hemangioma could not be excluded. A cystic-solid mass was located in the pancreatic head, and the presence of an SPT was considered. The main pancreatic duct was dilated. The CT results are shown in Figs. 1 and 2. A soft tissue mass was present in the area of the pancreatic head (largest cross-section, 10.7 9.7 cm). The lesion was of uneven density, similar to the liver lesions. The lesion had dot calcifications and was heterogeneously enhanced. The pancreatic duct was dilated. The duodenum was compressed and
W.-B. Wang et al. / EJSO 40 (2014) 1572e1577
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Figure 1. Multiple soft tissue masses in liver and in pancreatic head. A. Multiple variously sized lesions in the liver with uneven and slightly low density, smooth edges, and uneven enhancement, the largest section measured 7.0 5.7 cm; B. Pancreatic head huge mass, largest cross section was 10.7 9.7 cm, this lesion was uneven density.
displaced forward; the right kidney was displaced backward, the portal vein upward, and the superior mesenteric vein leftward. The superior and inferior pancreaticoduodenal arteries supplied blood to the lesion. An SPT was considered possible. There were multiple variously sized lesions in the liver with uneven and slightly low density, smooth edges, and uneven enhancement. The hepatic vein was compressed. There were multiple lymph nodes in the retroperitoneal area, some of which were enlarged. The patient underwent pancreatoduodenectomy and resection of the multiple liver lesions. We found a 15 10- 8-cm mass with an unclear border located in the uncinate process of the pancreas, and it was closely associated with the superior mesenteric vein. The pancreatic duct was 6 mm in diameter. Seven lesions in the liver were resected (Fig. 3). Pathological examination revealed a pancreatic SPT. The common bile duct and small intestine were
Figure 2. Specimen of pancreatoduodenectomy, including distal stomach, pancreatic head with that huge mass (largest cross section 10.7 9.7 cm), duodenum and proximal jejunum.
not involved. The edges of the pancreas, common bile duct, stomach, and small intestine showed no abnormalities. No lymph node metastasis was observed. The liver was determined to contain a metastatic pancreatic SPT. The immunohistochemistry results were as follows: AACT(þ), AAT(þ), AEI/AE3(þ), CD10(þ), CD56(NK1)(þ), CgA(), Syn(), vimentin(þ), B-catenin(þ), Ki67 index 5 cm, local infiltration at the first operation, metastasis, and other factors.21,22 However, disagreement persists.6 With regard to age, two patients in the present report were
