Portal annular pancreas (PAP) is a rare variant in which the uncinate process of the pancreas extends to the dorsal surface of the pancreas body and surrounds the portal vein or superior mesenteric vein. Upon pancreaticoduodenectomy (PD), when the pancreas is cut at the neck, two cut surfaces are created. Thus, the cut surface of the pancreas becomes larger than usual and the dorsal cut surface is behind the portal vein, therefore pancreatic fistula after PD has been reported frequently. We planned subtotal stomach-preserving PD in a 45-year-old woman with underlying insulinoma of the pancreas head. When the pancreas head was dissected, the uncinate process was extended and fused to the dorsal surface of the pancreas body. Additional resection of the pancreas body 1 cm distal to the pancreas tail to the left side of the original resection line was performed. The new cut surface became one and pancreaticojejunostomy was performed as usual. No postoperative complications such as pancreatic fistula occurred. Additional resection of the pancreas body may be a standardized procedure in patients with PAP in cases of pancreas cut surface reconstruction.
Portal annular pancreas; Pancreaticoduodenectomy; Pancreas fistula
Pancreatic cancer remains one of the most difficult neoplasms for early diagnosis and treatment. Recent advances of imaging including 3D volume data setting in multidetector-row CT (MDCT) and MRI are urging us to focus on the imaging of normal and pathological conditions of pancreatic parenchyme and peripancreatic structures, which are frequently involved by pancreatic cancers and are affecting the prognosis of patients with pancreatic cancers. In this Feature Section, five main topics of pancreatic imaging are addressed: pancreatic arterial territories, imaging of the intra- and peripancreatic venous anatomy and its clinical significance, imaging of the peripancreatic lymphatic network and its clinical significance for staging of pancreatic cancer, perfusion characteristics of pancreatic cancer to differentiate chronic mass-forming pancreatitis, and development of intraductal papillary mucinus neoplasms of the pancreas (IPMNs) to adenocarcinoma and pancreatic invasion. Recognition and understanding of the imaging anatomy of the pancreas might lead to precise staging of pancreatic cancer and to new approaches of less-invasive treatment. Follow-up of patients with IPMNs of the pancreas on imaging seems, at this time, to be the most valuable strategy in the high-risk group selection.
Pancreatic arterial territories; Pancreatic venous anatomy; Peripancreatic; Lymphatic network; Perfusion characteristics of pancreatic cancer; Intraductal papillary mucinus neoplasms of the pancreas
Aneurysms and pseudoaneurysms of the superior mesenteric artery are potentially lethal and should be treated as urgently as possible. In a 52-year-old man with occasional epigastric pain, we accidentally discovered a superior mesenteric artery aneurysm that was ruptured with spontaneous tamponade in the uncinate process and in the head of the pancreas. The ruptured aneurysm had a heterogeneous appearance due to its thrombotic and hemorrhagic content, and it simulated a voluminous mass in the head and uncinate process of the pancreas, associated with mild dilatation of the main pancreatic duct. Recent advances in multidetector computed tomography and magnetic resonance imaging have enabled radiologists to develop a correct diagnosis of mesenteric aneurysms and pseudoaneurysms of the visceral branches of the abdominal aorta, and to differentiate this diagnosis from that of pancreatic or peripancreatic masses; angiography is currently used to confirm a diagnosis and to develop therapeutic treatments.
Superior mesenteric artery; Magnetic resonance imaging; Computed tomography; Ruptured aneurysm
A 45 year old man presented with abdominal pain, loss of appetite, and significant weight loss over a period of about four weeks. Imaging of the abdomen showed a mass in the region of the head of the pancreas. In view of the size of the mass and the clinical picture, a Whipple’s procedure was performed. Histological evaluation of the pancreatic tumour showed an adenosquamous carcinoma (predominantly composed of squamous carcinoma), which was extensively infiltrative with perineural invasion and involvement of peripancreatic lymph nodes. Areas of pancreatic intraepithelial neoplasia grade III and merging of the squamous and adenocarcinoma components were evident. Unusual histological features that characterised this case included a pronounced acantholytic pattern within the squamous carcinoma component, and the presence of both osteoclastic and pleomorphic giant cells. Giant cells have not been documented previously in association with an adenosquamous carcinoma. Although an acantholytic pattern has been noted in squamous carcinomas in other sites, this is the first report of such a pattern in an adenosquamous carcinoma of the pancreas.
pancreas; adenosquamous carcinoma; giant cells; osteoclasts
Haemangiomas are common benign tumours that are generally detected within the skin, mucosal surfaces and soft tissues. However, intranodal haemangiomas are extremely rare and are among the benign primary vascular abnormalities of the lymph nodes that include lymphangioma, haemangioendothelioma, angiomyomatous hamartoma and haemangiomas. In this case report, we present the imaging and pathological findings of an intranodal haemangioma in the pancreatic head simulating a pancreatic neuroendocrine tumour. To the best of our knowledge, this is the first report of an intranodal haemangioma in this location.
Solid-pseudopapillary tumor (SPT) is a rare neoplasm of the pancreas that usually occurs in young females. It is generally considered a low-grade malignant tumor that can remain asymptomatic for several years. The occurrence of infiltrating varieties of SPT is around 10%-15%. Between 1986 and 2006, 282 cystic tumors of the pancreas were observed. Among them a SPT was diagnosed in 8 patients (2.8%) with only one infiltrating variety. This was diagnosed in a 49-year-old female 13 years after the sonographic evidence of a small pancreatic cystic lesion interpreted as a pseudocyst. The tumor invaded a long segment of the portal-mesenteric vein confluence, and was removed with a total pancreatectomy, resection of the portal vein and reconstruction with the internal jugular vein. Histological examination confirmed the R-0 resection of the primary SPT, although a vascular invasion was demonstrated. The postoperative course was uneventful, but 32 mo after surgery the patient experienced diffuse liver metastases. Chemotherapy with different drugs was started. The patient is alive and symptom-free, with stable disease, 75 mo after surgery. Twenty-five patients with invasion of the portal vein and/or of mesenteric vessels were retrieved from the literature, 16 recent patients with tumor relapse after potentially curative resection were also retrieved. The best treatment remains a radical resection whenever possible, even in locally advanced or metastatic disease. The role of chemotherapy, and/or radiotherapy, is still to be defined.
Solid-pseudopapillary tumor; Pancreatectomy; Vascular resection; Liver metastases; Follow-up
Early pancreatic cancer is small and limited to the pancreas. In contrast, small pancreatic cancer may include peripancreatic vasculature or metastasis involvement. This study evaluates images of early pancreatic cancer on multidetector CT (MDCT) using contrast-enhanced multiphasic imaging, and post-processed pancreatic duct images. CT findings and pathological features were analysed in eight patients with early pancreatic cancer. Pathological evaluation included location, size and histological grading of the tumour. MDCT evaluation covered the maximum diameter of the main pancreatic duct (MPD), stenosis or obstruction of the MPD, loss of normal lobar texture and associated pancreatitis. Attenuation differences between normal pancreatic parenchyma and the tumour (AD–PT) were also measured. Focal stenosis or obstruction of the MPD with dilatation of the distal MPD was demonstrated in all patients. Associated pancreatitis occurred in six patients with tumours measuring 12 mm or greater. Loss of normal lobar texture was recognised in four cases with the tumour measuring 14 mm or greater. Statistically, low-attenuated lesions and high-attenuated lesions differed with respect to the tumour size (p<0.01), and a positive relationship was demonstrated between the tumour size and AD–PT (r = 0.84). In seven cases, AD–PT is higher during the arterial phase than the pancreatic phase. Early pancreatic cancer appears as low attenuation on early phase, and as high- to iso-attenuation during the pancreatic and delayed phases in respect to the tumour size. Focal stenosis or obstruction of the MPD with dilatation of the distal MPD observed on curved reformation imaging seems important in the diagnosis of early pancreatic cancer.
Locally advanced colorectal tumors represent about 5–22% of all colorectal cancers at the time of presentation. Specifically in the case of right colon cancer, the percentage of adjacent structure involvement ranges between 11% and 28%. Organs that are most frequently invaded by right colonic tumors are the duodenum and the pancreatic head. We report the case of a 36-year old man with locally advanced right colonic cancer, invading the head of the pancreas and the superior mesenteric vein, who was successfully treated in our department with right hemicolectomy, pancreatoduodenectomy and short resection of the superior mesenteric vein with an end-to-end anastomosis, and remains alive and well, free of disease, nine years after the operation.
Locally advanced colonic cancer; Right hemicolectomy; Pancreatoduodenectomy
Erosive hemorrhage due to pseudoaneurysm is one of the most life-threatening complications after pancreatectomy. Here, we report an extremely rare case of rupture of a pseudoaneurysm of the common hepatic artery (CHA) stump that developed after distal pancreatectomy with en block celiac axis resection (DP-CAR), and was successfully treated through covered stent placement. The patient is a 66-year-old woman who underwent DP-CAR after adjuvant chemoradiotherapy for locally advanced pancreatic body cancer. She developed an intra-abdominal abscess around the remnant pancreas head 31 d after the surgery, and computed tomography (CT) showed an occluded portal vein due to the spreading inflammation around the abscess. Her general condition improved after CT-guided drainage of the abscess. However, 19 d later, she presented with melena, and CT showed a pseudoaneurysm arising from the CHA stump. Because the CHA had been resected during the DP-CAR, this artery could not be used as the access route for endovascular treatment, and instead, we placed a covered stent via the inferior pancreaticoduodenal artery originating from the superior mesenteric artery. After stent placement, cessation of bleeding and anterograde hepatic artery flow were confirmed, and the patient recovered well without any further complications. CT angiography at the 6-mo follow-up indicated the patency of the covered stent with sustained hepatic artery flow. To our knowledge, this is the first reported case of endovascular repair of a pseudoaneurysm that developed after DP-CAR.
Distal pancreatectomy; En block celiac axis resection; Pseudoaneurysm; Endovascular treatment; Covered stent
A case of pancreatic carcinoma with both acinar and endocrine features is presented. The patient was a 52-year-old female presenting with jaundice of 3 weeks' duration. The tumor was a 6 x 6 cm-sized round solid mass in the head of pancreas, invading the superior mesenteric vein. Histologically, it was composed of monotonous ovoid cells with eosinophilic granular cytoplasm in solid nests and sheets with occasional acinar and glandular differentiation. Immunohistochemical study revealed coexpression of acinar and endocrine markers; amylase, chromogranin, neuron-specific enolase, glucagon, somatostatin, and gastrin in tumor cells. This is the first documented case of mixed acinar-endocrine carcinoma of the pancreas in Korea, and its amphicrine nature reflects a close histogenetic relationship between pancreatic exocrine and endocrine cells.
Non-Hodgkin lymphoma is a rare cause of biliary obstruction. To the best of our knowledge, non-Hodgkin lymphoma in the peripancreatic region causing obstructive jaundice with simultaneous portal vein (PV) invasion has not yet been reported. We present a 50-year-old patient with obstructive jaundice whose extrahepatic portal vein was obstructed by the invasion of a peripancreatic non-Hodgkin lymphoma. The patient denied any other symptoms such as recurrent fever, night sweat and loss of body weight. Computed tomography (CT) revealed a 10 cm mass in the retroperitoneal space behind the head of the pancreas causing obstruction of the distal bile duct and the PV. A pylorus-preserving pancreaticoduodenectomy combined with a PV resection was performed. The PV was reconstructed using an autologous right internal jugular vein graft. The resected specimen showed endoluminal invasion of both the bile duct and the PV. Histological examination showed the mass consisting of diffuse sheets of large malignant lymphoid cells. These cells were positive for CD20 and CD79a, partially positive for CD10, and negative for CD3, CD4, CD5, CD8 and CD30. The pathologic diagnosis was diffuse large B-cell type non-Hodgkin lymphoma and the patient was transferred to the Department of Hematology and Oncology for chemotherapy. He received four cycles of combined chemotherapy including cyclophosphamide, doxorubicin, vincristine and prednisone plus rituximab, and three cycles of intrathecal chemoprophylaxis including methotrexate, cytosine arabinoside and prednisone. The patient is alive with no evidence of the disease for 7 mo after operation and will receive additional courses of chemotherapy.
Non-Hodgkin lymphoma; Obstructive jaundice; Portal vein obstruction
Retrograde pancreatography has been carried out at necropsy in 120 cases and the results have been analysed in statistical detail. With increasing age, changes in pancreatic anatomy occur which must not be taken to indicate pathology. These changes are: (1) low or ptotic position of the pancreas so that the papilla of Vater is below the level of L3; (2) calcification of the splenic and superior mesenteric arteries which produce calcific densities around the pancreas; (3) increasing width of main pancreatic duct along its whole length at about 8% per decade; in the elderly, widths of 1 cm can occur in the main duct in the head of the pancreas without evidence of obstruction; (4) formation of ductular ectasia which affects mainly the interlobular ductules but also intralobular ductules; (5) some ectatic ducts reach the dimensions of cysts, ie, 1-2 cm in diameter.
Other morphological changes which have been demonstrated and which may produce difficulties in radiological interpretation are: (a) narrowed ducts not due to stricture; (b) space-occupying lesions due to superior mesenteric artery, splenic artery, aorta, vertebral osteophytes, sympathetic ganglion, and lymph nodes; (c) metastases in the pancreas—these must be distinguished from primary pancreatic carcinoma.
The implications of these findings for endoscopy and isotope pancreatic scanning will be mentioned.
The purpose of this study is to provide better understanding as to how the "double" vascular arcades, in contrast to other intestinal marginal vessels, develop along the right margin of the pancreatic head.
Materials and Methods
In human fetuses between 8-30 weeks, we described the topographical anatomy of the vessels, bile duct, duodenum as well as the ventral and dorsal primordia of the pancreatic head with an aid of pancreatic polypeptide immunohisto-chemistry.
The contents of the hepatoduodenal ligament crossed the superior side of the pylorus. Moreover, the right hepatic artery originating from the superior mesenteric artery ran along the superior aspect of the pancreatic head. An arterial arcade, corresponding to the posterior pancreaticoduodenal arteries, encircled the superior part of the pancreatic head, whereas another arcade, corresponding to the anterior pancreaticoduodenal arteries, surrounded the inferior part. The dorsal promordium of the pancreas surrounded and/or mixed the ventral primordium at 13-16 weeks. Thus, both arterial arcades were likely to attach to the dorsal primordium.
The fetal anatomy of the pancreaticoduodenal vascular arcades as well as that of the hepatoduodenal ligament were quite different from adults in topographical relations. Thus, in the stage later than 30 weeks, further rotation of the duodenum along a horizontal axis seemed to be required to move the pylorus posterosuperiorly and to reflect the superior surface of the pancreatic head posteriorly. However, to change the topographical anatomy of the superior and inferior arterial arcades into the final position, re-arrangement of the pancreatic parenchyma might be necessary in the head.
Pancreaticoduodenal arterial arcades; ventral pancreas; pancreatic polypeptide immunohistochemistry; human fetus
The volume flow rate of blood in the portal vein and the hepatic artery was measured using a duplex ultrasound system. Two sections of the hepatic artery were studied; the common hepatic artery where measurements were made just after the bifurcation of the coeliac axis to splenic and hepatic arteries and the hepatic artery itself, where measurements were made just proximal to the porta hepatis in a straight stretch of artery overlying the portal vein. Total hepatic blood flow was taken as the sum of hepatic artery and portal vein flows. A group of 10 normal healthy volunteers in the fasting state was studied. The mean (SD) volume blood flow in the vessels was measured to be: hepatic artery 3.5 (45%) ml/min/kg, common hepatic artery 6.9 (30%) ml/min+/kg, portal vein 13.5 (21%) ml/min/kg, total hepatic flow 17.0 (16%) ml/min/kg.
Inflammatory myofibroblastic tumor (IMT) of the biliary tree is extremely rare and is generally a benign condition, though malignant change is possible. Making a differential diagnosis between this lesion and other malignant conditions is very difficult on preoperative imaging studies. Hence, the final diagnosis of IMT may be made during or after operation depending on the pathologic examination. We treated a 63-year-old woman who received right hepatectomy with caudate lobectomy under the suspicion of hilar cholangiocarcinoma. Frozen biopsy during the operation showed carcinoma in situ and there were stromal cells in the bile duct's resection margins. The postoperative hospital course was uneventful except for minor bile leakage. At postoperative month 4, she developed jaundice, ascites and pleural effusion. Computed tomography images showed a mass-like lesion in the porta hepatis with portal vein thrombosis and a right chest wall mass. Excisional biopsy was done and the pathology report was malignant spindle cell tumor suggestive of an aggressive form of IMT. Her condition rapidly deteriorated regardless of the best supportive care and she expired at postoperative month 5. Further investigation is necessary to clarify the reasons for recurrence and infiltration of this disease.
Inflammatory myofibroblastic tumor; Malignant spindle cell tumor
We wished to evaluate the effect of the Pringle maneuver (occlusion of both the hepatic artery and portal vein) on the pathologic changes in the hepatic vessels, bile ducts and liver parenchyma surrounding the ablation zone in rabbit livers.
Materials and Methods
Radiofrequency (RF) ablation zones were created in the livers of 24 rabbits in vivo by using a 50-W, 480-kHz monopolar RF generator and a 15-gauge expandable electrode with four sharp prongs for 7 mins. The tips of the electrodes were placed in the liver parenchyma near the porta hepatis with the distal 1 cm of their prongs deployed. Radiofrequency ablation was performed in the groups with (n=12 rabbits) and without (n=12 rabbits) the Pringle maneuver. Three animals of each group were sacrificed immediately, three days (the acute phase), seven days (the early subacute phase) and two weeks (the late subacute phase) after RF ablation. The ablation zones were excised and serial pathologic changes in the hepatic vessels, bile ducts and liver parenchyma surrounding the ablation zone were evaluated.
With the Pringle maneuver, portal vein thrombosis was found in three cases (in the immediate [n=2] and acute phase [n=1]), bile duct dilatation adjacent to the ablation zone was found in one case (in the late subacute phase [n=1]), infarction adjacent to the ablation zone was found in three cases (in the early subacute [n=2] and late subacute [n=1] phases). None of the above changes was found in the livers ablated without the Pringle maneuver. On the microscopic findings, centrilobular congestion, sinusoidal congestion, sinusoidal platelet and neutrophilic adhesion, and hepatocyte vacuolar and ballooning changes in liver ablated with Pringle maneuver showed more significant changes than in those livers ablated without the Pringle maneuver (p < 0.05)
Radiofrequency ablation with the Pringle maneuver created more severe pathologic changes in the portal vein, bile ducts and liver parenchyma surrounding the ablation zone compared with RF ablation without the Pringle maneuver. Therefore, we suggest that RF ablation with the Pringle maneuver should be performed with great caution in order to avoid unwanted thermal injury.
Animals; Liver, interventional procedure; Radiofrequency (RF) ablation
Normal hepatic arterial anatomy occurs in approximately 50–80% of cases; for the remaining cases, multiple variations have been described. Knowledge of these anomalies is especially important in hepatobiliary and pancreatic surgery in order to avoid unnecessary complications. We describe two cases of patients undergoing pancreatoduodenectomy for abnormalities in the head of the pancreas. Preoperative contrast-enhanced cross-sectional imaging demonstrated relevant, rare hepatic arterial variants: (1) a completely replaced hepatic arterial system with a gastroduodenal artery (GDA) arising directly from the celiac axis and (2) a replaced right hepatic artery originating from the superior mesenteric artery and traveling anterior to the pancreatic uncinate process and head. These findings were confirmed during pancreatoduodenectomy. Both of these variants have been rarely described with an incidence of <1.0%. In the present paper, we describe the hepatic arterial anomalies commonly encountered and clarify the important details associated with these variants as they pertain to pancreatoduodenectomy.
A 39-y-old man, who had an episode of pancreatic bleeding due to chronic pancreatitis, received total pancreatectomy with islet autotransplantation (TP with IAT). Intraoperative ultrasound (US) examination was done to detect transplanted islets and evaluate the quality of US imaging.
Islet isolation from the resected total pancreas was performed and approximately 230,000 islet equivalents (IEQ) (the tissue volume was 600 µL and the purity was 30%) were acquired. A double lumen catheter, used for transplantation and for monitoring the portal vein pressure, was inserted into the portal vein via the superior mesenteric vein, and the tip of the catheter was positioned at the bifurcation of the anterior and posterior branch of the portal vein to selectively infuse the islets into the right lobe of the liver in order to prevent total liver embolization. Intraoperative US examination (central frequency 7.5 MHz, Nemio™ XG, Toshiba Medical System Co.) was started at the same time as the transplantation.
US examination revealed the transplanted islets as hyperechoic clusters that flowed from the tip of the catheter to the periphery of the portal vein. There were no findings of portal thrombosis or bleeding in the US image, and also no increase of the portal vein pressure during transplantation.
In conclusion, we succeeded in visualizing human islets using US, which enabled us to perform islet transplantation safely. The hyperechoic images were considered to be viable islets. Intraoperative US examination can be useful for detecting islets at transplantation in a clinical setting.
islet transplantation; ultrasound; chronic pancreatitis; arteriovenous malformation; total pancreatectomy; islet autotransplantation
Complete pancreatic rupture is a rare injury. The typical mechanism by which this occurs is overstretching of the pancreas across the vertebral column during blunt abdominal trauma. The management of this injury depends on the location and extent of the injury.
A 45-year-old Caucasian woman presented with blunt abdominal trauma after she fell onto the end of a handlebar during a bicycle accident. She arrived in the emergency room with stable vital signs and an isolated bruise just above the umbilicus. A computed tomography scan revealed a complete rupture of the pancreas, just ventral to her superior mesenteric vein, and an accompanying hematoma but no additional injuries. An emergency laparotomy was performed; the head of the pancreas was oversewn with interrupted sutures and this was followed by a two-layer pancreaticojejunostomy with the tail of the pancreas. The recovery after surgery was completely uneventful.
Isolated complete pancreatic rupture is a rare injury that can be managed with complete organ preservation. The combination of suturing the pancreatic head and two-layer pancreaticojejunostomy with the pancreatic tail is a feasible technique to manage this condition.
Blunt abdominal trauma is a rare but well-recognized cause of pancreatic transection. A delayed presentation of pancreatic fracture following sport-related blunt trauma with the coexisting diagnostic pitfalls is presented.
A 17-year-old rugby player was referred to our specialist unit after having been diagnosed with traumatic pancreatic transection, having presented 24 h after a sporting injury. Despite haemodynamic stability, at laparotomy he was found to have a diffuse mesenteric hematoma involving the large and small bowel mesentery, extending down to the sigmoid colon from the splenic flexure, and a large retroperitoneal hematoma arising from the pancreas. The pancreas was completely severed with the superior border of the distal segment remaining attached to the splenic vein that was intact. A distal pancreatectomy with spleen preservation and evacuation of the retroperitoneal hematoma was performed.
Blunt pancreatic trauma is a serious condition. Diagnosis and treatment may often be delayed, which in turn may drastically increase morbidity and mortality. Diagnostic difficulties apply to both paraclinical and radiological diagnostic methods. A high index of suspicion should be maintained in such cases, with a multi-modality diagnostic approach and prompt surgical intervention as required.
Pancreatectomy; Pancreatic transection; Mesenteric hematoma
Between January 1987 and September 1991, 68 severely traumatized patients underwent emergency
laparotomy because of blunt abdominal trauma. Intraoperatively, 54.4% of the patients had a major
injury to one organ, 23.5% had injuries to two organs, 16.2% had injuries to three organs and 5.9% to
four or more organs. Additionally, in 11.8% of these cases (n = 8) a major vascular injury (portal vein n = 5, vena cava n = 2, mesenteric root n = 1) was found. Injuries to the portal vein were always associated with complete rupture of the pancreas, requiring distal pancreatic resection in four cases and a duodenum preserving resection of the head of the pancreas in one. In two of these patients the portal vein had to be reconstructed with a Goretex prosthetic graft. Mortality was 14.7% for the whole group (n = 68) and 0% for patients with additional portal venous injuries.
Selective visceral angiography should help to determine the nature and extent of pancreatic lesions and their suitability for resection. Between 1980 and 1987 coeliac and superior mesenteric angiograms were obtained in 76 patients considered for pancreatic resection. Anomalous arterial anatomy was delineated in 25%. Among arterial abnormalities observed in 42 patients (55%), increased or decreased vascularity and displacement were of limited diagnostic value, but encasement correctly predicted cancer in 18 of 21 cases and irresectability in nine of these. When present (17%), invasion or occlusion of the portal or superior mesenteric vein was even more accurate, indicating cancer in 12 of 13 cases and irresectability in 11 of these. Hepatic metastases were only detected in 7 of 15 patients (47%). Overall, angiography confirmed the diagnosis in 54%, localised the lesion in 64% and correctly forecast irresectability in 58%. Misleading data were obtained in five patients. There were no complications.
Merkel cell carcinomas are rare neoplasm of neuroendocrine origin, usually observed in elderly people in areas with abundant sunlight, and predominantly located on the head and neck, extremities, and trunk. In many patients, a local recurrence after resection of the primary tumour and even distant metastases can be found.
We report an unusual occurrence of pancreatic metastases from a previously diagnosed Merkel cell carcinoma with the discovery of a concomitant insulinoma. An 82-year old lady suffered from recurrent attacks of hypoglycemia and presented with an abdominal mass, 2 years prior she had an excision done on her eyebrow that was reported as Merkel cell carcinoma. An extended distal pancreatectomy and splenectomy along with resection of the left flexure of the colon for her abdominal mass was carried out. Final histopathology of the mass was a poorly differentiated endocrine carcinoma in the pancreatic tail, in the peripancreatic tissue and in the surrounding soft tissue consistent with metastatic Merkel cell carcinoma in addition to an insulinoma of the pancreatic body.
This is the first documented case of a metastatic Merkel cell carcinoma and a concomitant insulinoma, suggesting either a mere coincidence or an unknown neuroendocrine tumor syndrome.
A 56-year-old male was admitted with symptoms of belching, abdominal pain and weight loss of 2 weeks duration. Examination revealed hepatosplenomegaly which was confirmed by computed tomography (CT). CT images also revealed filling defects in the portal vein and intrahepatic branches consistent with thrombosis and hepatosplenic infarcts. Alkaline phosphatase was elevated at 688 units, all other investigations, including full blood count, coagulation screen and tumour markers, were normal. Magnetic resonance cholangiopancreatography did not reveal any mass in the porta hepatis. Upper gastrointestinal endoscopy and colonoscopy were normal. Liver biopsy was normal and did not reveal any evidence of lymphoma. The raised alkaline phosphatase settled to reference range over a period of 3 weeks. Thrombophilia screen was negative. Contrast CT of the abdomen performed after 4 weeks displayed revascularisation of the previously thrombosed portal vein and intrahepatic branches. The patient has remained asymptomatic since and we note spontaneous recanalisation of the previously occluded portal vein.
Portal vein thrombosis; Spontaneous resolution; Outcome