Pancreatology 15 (2015) 197e199
Contents lists available at ScienceDirect
Pancreatology journal homepage: www.elsevier.com/locate/pan
Case report
Solid variant type of serous cystadenocarcinoma of the pancreas: A case report and review of the literature Osamu Kainuma a, *, Hiroshi Yamamoto a, Akihiro Cho a, Hidehito Arimitsu a, Hiroo Yanagibashi a, Nobuhiro Takiguchi a, Yoshihiro Nabeya a, Hidetada Kawana b a b
Department of Gastroenterological Surgery, Chiba Cancer Center, Japan Department of Surgical Pathology, Chiba Cancer Center, Japan
a r t i c l e i n f o
a b s t r a c t
Article history: Available online 12 February 2015
Serous cystic neoplasms (SCN) of the pancreas are typically honeycombed microcystic masses, which are believed to be benign entity. This report describes a case of a 69-year-old man with a rare solid type of serous cystadenocarcinoma of the pancreas with liver metastases. A 6-cm well enhanced pancreatic tumor and multiple liver nodules were depicted with contrast medium on computed tomography scan. Distal pancreatectomy was performed at first operation. The cut surface of the tumor was solid and glossy appearance. Second operation of liver resection for all metastatic nodules was performed 27 months after the initial operation. The tumor cells in both the pancreas and the liver had cytoplasmic periodic acid-Schiff positive granules, which were completely digested by diastase. Eleven cases of serous cystadenocarcinoma of the pancreas have been reported in the literature. To our knowledge, this is the first case of a solid type serous cystadenocarcinoma. Copyright © 2015, IAP and EPC. Published by Elsevier India, a division of Reed Elsevier India Pvt. Ltd. All rights reserved.
Keywords: Pancreas Serous cystadenocarcinoma Solid type Liver metastases PAS stain pNET
Background In 1978, Compagno and Oertel proposed the concept of serous cystic neoplasm (SCN) of the pancreas [1]. SCNs are typically honeycombed microcystic masses composed of uniform, cuboidal, glycogen-rich epithelial cells. In addition to the most common microcystic type, macrocystic, and solid SCN have also been described. Although these SCNs of the pancreas are regarded as benign, some can behave in a malignant fashion and display invasion or metastasis. The present report describes a rare case of solid type of serous cystadenocarcinoma with multiple liver metastases. The case description is followed by a review of the published literature.
Case report A 69-year-old man presented to our hospital with a history of upper abdominal discomfort. Contrast-enhanced computed
* Corresponding author. Department of Gastroenterological Surgery, Chiba Cancer Center, 666-2 Nitona, Chuo-ku, Chiba 260-8717, Japan. Tel.: þ81 43 264 5431; fax: þ81 43 265 9515. E-mail address: [email protected] (O. Kainuma).
tomography (CT) scan showed a 6-cm solid mass in the body of the pancreas, peripheral of which was well enhanced in the early phase. The central portion was not enhanced until the late phase (Fig. 1a). Simultaneously, multiple liver nodules, which were wellenhanced in the early phase, were observed at the surface of the liver (Fig. 1b). On laboratory examination, anemia, jaundice, and hyperglycemia were not observed. Serum level of tumor makers (carcinoembryonic antigen and carbohydrate antigen 19-9) and pancreatic endocrine hormones (insulin, glucagon, and gastrin) were within the normal range. Serum chromogranin A was not measured, because it was uncovered by insurance in Japan. Abdominal ultrasonography (US) and endoscopic ultrasonography (EUS) revealed a low echoic solid mass in the body of the pancreas (Fig. 1c). T2-weighted magnetic resonance imaging (MRI) demonstrated a high intensity mass. The tumor signal intensity was definitely lower than that of the gallbladder (Fig. 1d). Magnetic resonance cholangiopancreatography (MRCP) showed no dilatation of the main pancreatic duct. Distal pancreatectomy was performed with a clinical diagnosis of pancreatic neuroendocrine tumor with multiple liver metastases. Histological confirmation of the liver masses was not performed at the time of that operation. On gross examination, the pancreatic tumor was well circumscribed. The cut surface showed a solid, glossy, and reddish tumor with a central fibrous scar in a
http://dx.doi.org/10.1016/j.pan.2015.02.003 1424-3903/Copyright © 2015, IAP and EPC. Published by Elsevier India, a division of Reed Elsevier India Pvt. Ltd. All rights reserved.
198
O. Kainuma et al. / Pancreatology 15 (2015) 197e199
Fig. 1. Abdominal computed tomography scan shows a well-enhanced solid tumor with a central scar in the pancreas (a). Multiple liver metastases are well-enhanced also (b). EUS shows a large low echoic tumor. Honeycomb structures are not seen. (c) T2-weighted MR image shows a hyperintense mass, of which the signal intense is lower than that of the gallbladder (d).
stellate pattern. A honeycomb structure of small cysts was not detected macroscopically (Fig. 2). Microscopic findings revealed multiple microcysts separated by hypocellular, dense collagen fibers. The inner surface of the cysts was lined by a single layer of cuboidal epithelium with a clear cytoplasm. The nuclei were round to oval and were centrally located with inconspicuous nucleoli. No mitoses or cellular atypia were noted (Fig. 3a). Ki-67 labeling index was 1e2%. Immunohistochemical staining was positive for the cytokeratins, AE1 and AE3, and negative for CD56, chromogranin A, synaptophysin, renal cell carcinoma marker, and CD10. The epithelial cells of the tumor had cytoplasmic periodic acid-Schiff (PAS)-positive granules, which were completely digested by diastase (Fig. 3b,c). Vascular and perivascular invasions, and nodal involvement were not observed. Follow-up examinations performed every 3 months after surgery via CT or MRI showed no evidence of tumor growth in the liver and other sites recurrence. Enucleation of all remnant tumors in the liver and repair of wound hernia were performed 27 months after the initial operation. Macroscopically, the liver tumors were all solid and hard. The tumor cells resembled the primary tumor cells with findings of microcysts lined by a single layer of cuboidal cells (Fig. 3d). The final diagnosis was a solid type of serous
Fig. 2. The cut surface of the tumor shows solid and glossy appearance macroscopically. A fibrous scar is seen at the central area.
Fig. 3. Microscopically, the tumor of the pancreas shows numerous cysts lined by a single layer of cuboidal epithelium with a clear cytoplasm (Hematoxylin and eosin stain, 100) (a), The cytoplasm is strongly stained by Periodic Acid-Schiff (PAS) stain (400) (b), and digested by diastase (400) (c). Histological findings of the liver tumors are similar to those of the pancreas tumor, which supports a diagnosis of metastasis (100) (d).
adenocarcinoma of the pancreas. This patient is well-being without recurrence 3 months after the second operation. Discussion SCN of the pancreas had been classified as a benign entity with no malignant potential. The first malignant case of SCN was reported in 1984 on the basis of the presence of synchronous tumors in the liver [2]. However, this case was not histologically confirmed. George et al. are generally recognized as the first investigators to describe serous cystadenocarcinoma of the pancreas in 1989 [3]. Their report was an autopsy case of a pancreatic tumor invading into the spleen and metastasis to the stomach and the liver. Subsequently, other reports described similar cases with invasion into other organs or with synchronous or metachronous metastases. Twenty-nine cases were reviewed as serous cystadenocarcinoma of the pancreas in the literature. However, according to the current WHO classification [4], only a case with distant metastases is defined as serous cystadenocarcinoma; thus, 11 cases satisfied this definition. We reviewed these 11 cases [5e13] along with our case in order to better delineate serous cystadenocarcinoma (Table 1). Of these 12 cases, the mean patient age was 63 years (range, 56e74 years), and 70% were female. Most patients with malignant SCNs were symptomatic. The most common symptoms were abdominal pain, followed by weight loss and palpable mass. The mean tumor size was 12 cm, which was greater than the 4.1e5.1 cm of serous cystadenoma [12,14]. In gross appearance, typical microcystic type was seen in seven, macrocystic type in one, and solid type in only our case. Synchronous and metachronous metastases were seen in five and seven cases, respectively. The most common site of metastasis was the liver. SCNs are morphologically classified into four types: microcystic, macrocystic, mixed, and solid. The solid type was so rare that the incidence was only 3.0% of SCNs [14,15]. Compared with the microcystic type composed of numerous tiny cysts (usually
Contents lists available at ScienceDirect
Pancreatology journal homepage: www.elsevier.com/locate/pan
Case report
Solid variant type of serous cystadenocarcinoma of the pancreas: A case report and review of the literature Osamu Kainuma a, *, Hiroshi Yamamoto a, Akihiro Cho a, Hidehito Arimitsu a, Hiroo Yanagibashi a, Nobuhiro Takiguchi a, Yoshihiro Nabeya a, Hidetada Kawana b a b
Department of Gastroenterological Surgery, Chiba Cancer Center, Japan Department of Surgical Pathology, Chiba Cancer Center, Japan
a r t i c l e i n f o
a b s t r a c t
Article history: Available online 12 February 2015
Serous cystic neoplasms (SCN) of the pancreas are typically honeycombed microcystic masses, which are believed to be benign entity. This report describes a case of a 69-year-old man with a rare solid type of serous cystadenocarcinoma of the pancreas with liver metastases. A 6-cm well enhanced pancreatic tumor and multiple liver nodules were depicted with contrast medium on computed tomography scan. Distal pancreatectomy was performed at first operation. The cut surface of the tumor was solid and glossy appearance. Second operation of liver resection for all metastatic nodules was performed 27 months after the initial operation. The tumor cells in both the pancreas and the liver had cytoplasmic periodic acid-Schiff positive granules, which were completely digested by diastase. Eleven cases of serous cystadenocarcinoma of the pancreas have been reported in the literature. To our knowledge, this is the first case of a solid type serous cystadenocarcinoma. Copyright © 2015, IAP and EPC. Published by Elsevier India, a division of Reed Elsevier India Pvt. Ltd. All rights reserved.
Keywords: Pancreas Serous cystadenocarcinoma Solid type Liver metastases PAS stain pNET
Background In 1978, Compagno and Oertel proposed the concept of serous cystic neoplasm (SCN) of the pancreas [1]. SCNs are typically honeycombed microcystic masses composed of uniform, cuboidal, glycogen-rich epithelial cells. In addition to the most common microcystic type, macrocystic, and solid SCN have also been described. Although these SCNs of the pancreas are regarded as benign, some can behave in a malignant fashion and display invasion or metastasis. The present report describes a rare case of solid type of serous cystadenocarcinoma with multiple liver metastases. The case description is followed by a review of the published literature.
Case report A 69-year-old man presented to our hospital with a history of upper abdominal discomfort. Contrast-enhanced computed
* Corresponding author. Department of Gastroenterological Surgery, Chiba Cancer Center, 666-2 Nitona, Chuo-ku, Chiba 260-8717, Japan. Tel.: þ81 43 264 5431; fax: þ81 43 265 9515. E-mail address: [email protected] (O. Kainuma).
tomography (CT) scan showed a 6-cm solid mass in the body of the pancreas, peripheral of which was well enhanced in the early phase. The central portion was not enhanced until the late phase (Fig. 1a). Simultaneously, multiple liver nodules, which were wellenhanced in the early phase, were observed at the surface of the liver (Fig. 1b). On laboratory examination, anemia, jaundice, and hyperglycemia were not observed. Serum level of tumor makers (carcinoembryonic antigen and carbohydrate antigen 19-9) and pancreatic endocrine hormones (insulin, glucagon, and gastrin) were within the normal range. Serum chromogranin A was not measured, because it was uncovered by insurance in Japan. Abdominal ultrasonography (US) and endoscopic ultrasonography (EUS) revealed a low echoic solid mass in the body of the pancreas (Fig. 1c). T2-weighted magnetic resonance imaging (MRI) demonstrated a high intensity mass. The tumor signal intensity was definitely lower than that of the gallbladder (Fig. 1d). Magnetic resonance cholangiopancreatography (MRCP) showed no dilatation of the main pancreatic duct. Distal pancreatectomy was performed with a clinical diagnosis of pancreatic neuroendocrine tumor with multiple liver metastases. Histological confirmation of the liver masses was not performed at the time of that operation. On gross examination, the pancreatic tumor was well circumscribed. The cut surface showed a solid, glossy, and reddish tumor with a central fibrous scar in a
http://dx.doi.org/10.1016/j.pan.2015.02.003 1424-3903/Copyright © 2015, IAP and EPC. Published by Elsevier India, a division of Reed Elsevier India Pvt. Ltd. All rights reserved.
198
O. Kainuma et al. / Pancreatology 15 (2015) 197e199
Fig. 1. Abdominal computed tomography scan shows a well-enhanced solid tumor with a central scar in the pancreas (a). Multiple liver metastases are well-enhanced also (b). EUS shows a large low echoic tumor. Honeycomb structures are not seen. (c) T2-weighted MR image shows a hyperintense mass, of which the signal intense is lower than that of the gallbladder (d).
stellate pattern. A honeycomb structure of small cysts was not detected macroscopically (Fig. 2). Microscopic findings revealed multiple microcysts separated by hypocellular, dense collagen fibers. The inner surface of the cysts was lined by a single layer of cuboidal epithelium with a clear cytoplasm. The nuclei were round to oval and were centrally located with inconspicuous nucleoli. No mitoses or cellular atypia were noted (Fig. 3a). Ki-67 labeling index was 1e2%. Immunohistochemical staining was positive for the cytokeratins, AE1 and AE3, and negative for CD56, chromogranin A, synaptophysin, renal cell carcinoma marker, and CD10. The epithelial cells of the tumor had cytoplasmic periodic acid-Schiff (PAS)-positive granules, which were completely digested by diastase (Fig. 3b,c). Vascular and perivascular invasions, and nodal involvement were not observed. Follow-up examinations performed every 3 months after surgery via CT or MRI showed no evidence of tumor growth in the liver and other sites recurrence. Enucleation of all remnant tumors in the liver and repair of wound hernia were performed 27 months after the initial operation. Macroscopically, the liver tumors were all solid and hard. The tumor cells resembled the primary tumor cells with findings of microcysts lined by a single layer of cuboidal cells (Fig. 3d). The final diagnosis was a solid type of serous
Fig. 2. The cut surface of the tumor shows solid and glossy appearance macroscopically. A fibrous scar is seen at the central area.
Fig. 3. Microscopically, the tumor of the pancreas shows numerous cysts lined by a single layer of cuboidal epithelium with a clear cytoplasm (Hematoxylin and eosin stain, 100) (a), The cytoplasm is strongly stained by Periodic Acid-Schiff (PAS) stain (400) (b), and digested by diastase (400) (c). Histological findings of the liver tumors are similar to those of the pancreas tumor, which supports a diagnosis of metastasis (100) (d).
adenocarcinoma of the pancreas. This patient is well-being without recurrence 3 months after the second operation. Discussion SCN of the pancreas had been classified as a benign entity with no malignant potential. The first malignant case of SCN was reported in 1984 on the basis of the presence of synchronous tumors in the liver [2]. However, this case was not histologically confirmed. George et al. are generally recognized as the first investigators to describe serous cystadenocarcinoma of the pancreas in 1989 [3]. Their report was an autopsy case of a pancreatic tumor invading into the spleen and metastasis to the stomach and the liver. Subsequently, other reports described similar cases with invasion into other organs or with synchronous or metachronous metastases. Twenty-nine cases were reviewed as serous cystadenocarcinoma of the pancreas in the literature. However, according to the current WHO classification [4], only a case with distant metastases is defined as serous cystadenocarcinoma; thus, 11 cases satisfied this definition. We reviewed these 11 cases [5e13] along with our case in order to better delineate serous cystadenocarcinoma (Table 1). Of these 12 cases, the mean patient age was 63 years (range, 56e74 years), and 70% were female. Most patients with malignant SCNs were symptomatic. The most common symptoms were abdominal pain, followed by weight loss and palpable mass. The mean tumor size was 12 cm, which was greater than the 4.1e5.1 cm of serous cystadenoma [12,14]. In gross appearance, typical microcystic type was seen in seven, macrocystic type in one, and solid type in only our case. Synchronous and metachronous metastases were seen in five and seven cases, respectively. The most common site of metastasis was the liver. SCNs are morphologically classified into four types: microcystic, macrocystic, mixed, and solid. The solid type was so rare that the incidence was only 3.0% of SCNs [14,15]. Compared with the microcystic type composed of numerous tiny cysts (usually
