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Rare case of adult pancreatic haemangioma and literature review

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Abstract

Pancreatic haemangiomas are benign vascular tumours very rare in adults. Twenty-two cases are described in the literature. The symptoms are non-specific, and therefore rarely clinically suspected, and the vast majority are incidental findings in imaging tests such as ultrasound, CT, angiography or MRI. They appear on CT as a cystic lesion with contrast enhancement in the arterial phase. We present the case of a 36-year-old male patient with no history of disease, referred with lumbar pain and suspected renal calculus after tomography showing hypervascular enhancement in the pancreatic body and infiltrative lesion (possible neuroendocrine neoplasia) on MRI and biliopancreatic echoendoscopy. He was submitted to laparotomy with subtotal pancreatectomy and splenectomy and satisfactory evolution.

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Pancreatic haemangiomas are a rare cause of pancreatic lesions in adults. Diagnosis is challenging as they are seldom suspected and difficult to differentiate on imaging. Historically, pancreatic haemangiomas have been managed surgically despite their benign nature, largely due to diagnostic uncertainty. We present the case of a 69-year-old woman who, through combination of radiological, biochemical and endoscopic investigations, was diagnosed with pancreatic haemangioma and managed conservatively, avoiding the morbidity and mortality associated with surgical resection of a benign lesion.
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Adult pancreatic hemangioma is an especially rare benign tumor that is hard to diagnose through imaging examinations, meanwhile its histopathological and immunohistochemical studies have rarely been reported. At this time, only 29 cases in 27 publications have been reported. We report a new case as well as review these literatures. A 71-year-old woman came to our hospital with slight pain on left upper abdomen for three days. But the results obtained from the physical or laboratory examination were all negative so that her symptoms could not be explained clearly. The imaging examinations including ultrasonography and computed tomography both showed a mixed mass in the neck of the pancreas. The preoperative diagnosis of cystadenoma or adenocarcinoma was made, with high suspicious of malignancy. The patient underwent a central pancreatectomy with pancreatojejunostomy, but the pathologic diagnosis was pancreatic hemangioma. The immunohistochemical examination found the positive benign vascular markers (CD31, CD34) and negative lymphocyte markers (D2-40). Moreover, Ki-67 negativity also revealed its benign nature. After 36 months of follow-up, the patient has no complaints for abdominal pain. In conclusion, adult pancreatic hemangioma is extremely rare without any specific clinical manifestations. It is usually diagnosed postoperatively by histological examination and immunohistochemical studies. Imaging examinations, including computed tomography and magnetic resonance imaging, can't give definite conclusion. Endoscopic-ultrasound guided fine needle aspiration is conditionally worth doing, which can give some hints and exclude the malignancy of the lesion. If malignancy can be safely ruled out, the surgical decision must be made according to risk-benefit analysist. Maybe close observation and regular follow-up are more beneficial options.
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To review the features of patients with benign and malignant cystadenomas of the pancreas, focusing on preoperative diagnostic accuracy and long-term outcome, especially for nonoperated serous cystadenomas and resected cystadenocarcinomas. Serous cystadenomas (SCAs) are benign tumors. Mucinous cystic neoplasms should be resected because of the risk of malignant progression. A correct preoperative diagnosis of tumor type is based on morphologic criteria. Despite the high quality of recent imaging procedures, the diagnosis frequently remains uncertain. Invasive investigations such as endosonography and diagnostic aspiration of cystic fluid may be helpful, but their assessment is limited to small series. The management of typical SCA may require resection or observation. Survival after pancreatic resection seems better for cystadenocarcinomas (MCACs) than for ductal adenocarcinomas of the pancreas. Three hundred ninety-eight cases of cystadenomas of the pancreas were collected between 1984 and 1996 in 73 institutions of the French Surgical Association. Clinical presentation, radiologic evaluation, and surgical procedures were analyzed for 144 operated SCAs, 150 mucinous cystadenomas (MCAs), and 78 MCACs. The outcome of 372 operated patients and 26 nonoperated patients with SCA was analyzed. Cystadenomas represented 76% of all primary pancreatic cystic tumors (398/522). An asymptomatic tumor was discovered in 32% of patients with SCA, 26% of those with MCA, and 13% of those with MCAC. The tumor was located in the head or uncinate process of the pancreas in 38% of those with SCA, 27% of those with MCA, and 49% of those with MCAC. A communication between the cyst and pancreatic duct was discovered in 0.6% of those with SCA, 6% of those with MCA, and 10% of those with MCAC. The main investigations were ultrasonography and computed tomography (94% for SCA, MCA, and MCAC), endosonography (34%, 28%, and 22% for SCA, MCA, and MCAC respectively), endoscopic retrograde cholangiopancreatography (16%, 14%, 22%), and cyst fluid analysis (22%, 31%, 35%). An accurate preoperative diagnosis of tumor type was proposed for 20% of those with SCA (144 cases), 30% of those with MCA, and 29% of those with MCAC. An atypical unilocular macrocyst was observed in 10% of SCA cases. The most common misdiagnosis for mucinous cystic tumors was pseudocyst (9% of MCAs, 15% of MCACs). Intraoperative frozen sections (126 cases) allowed a diagnosis according to definitive histologic examination in 50% of those with SCA and MCA and 62% of those with MCAC. For management, 93% of patients underwent surgery. Nonoperated patients (7%) had exclusively typical SCA. A complete cyst excision was performed in 94% of benign cystadenomas, with an operative mortality rate of 2% for SCA and 1.4% for MCA. Resection was possible in 74% of cases of MCAC. Mean follow-up of 26 patients with nonresected SCAs was 38 months, and no patients required surgery. For resected MCACs, the actuarial 5-year survival rate was 63%. Spiral computed tomography is the examination of choice for a correct prediction of tumor type. Endosonography may be useful to detect the morphologic criteria of small tumors. Diagnostic aspiration of the cyst allows differentiation of the macrocystic form of SCA (10% of cases) and the unilocular type of mucinous cystic neoplasm from a pseudocyst. Surgical resection should be performed for symptomatic SCAs, all mucinous cystic neoplasms, and cystic tumors that are not clearly defined. Conservative management is wholly justified for a well-documented SCA with no symptoms. An extensive resection is warranted for MCAC because the 5-year survival rate may exceed 60%.
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Abstract Pancreatic hemangiomas are benign tumors extremely rare in adults, rarer than in children. Although were reported a small number of pancreatic hemangiomas, mostly were proved to be hyper vascular tumors. We present the case of a sixty-four years old male who had acute upper abdominal pain, and who was admitted, with the suspicion of acute pancreatitis. Imagistic examinations detected a tissular nodule in the body of the pancreas, with well- defined margins, without capsule, with heterogeneous structure, and with maximum size of 1.9 cm. Also, it was discovered a similar lesion, regarding the imagistic appearance, in the fourth segment of the liver, and a hemangioma in the sixth segment of the liver. There were no lymphadenopathies in ultrasound and computed tomography exams. Although was suspected a pancreatic adenocarcinoma with liver metastasis, the patient was planned for surgery, knowing that this type of neoplasia is extremely aggressive with a poor prognosis if is not surgically removed. Histopathological exam indicate that the pancreatic mass was a hemangioma.
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Pancreatic hemangiomas are a rare type of cystic tumor, with very few cases reported in the literature. Herein, we present the case of a 28-year-old woman who was admitted to our hospital for abdominal pain. A physical examination failed to reveal any abnormalities that could explain her symptoms. A contrast-enhanced computed tomography showed a multilocular cyst with moderately enhanced septa and fluid-fluid levels in the body and tail of the pancreas. A serous cystadenoma or pseudocyst of the pancreas was initially suspected, and the patient underwent a subtotal pancreatectomy and splenectomy. The pathologic diagnosis was a pancreatic hemangioma. This is the second case of pancreatic hemangioma with fluid-fluid levels reported in the literature. Upon imaging, the presentation of this tumor can resemble serous or mucinous cystadenomas, pseudocysts of the pancreas, and side-branch type intraductal papillary mucinous neoplasms. This report reviews the clinical symptoms, radiologic features, pathologic characteristics, differential diagnoses, and treatment of this rare lesion type.
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Objective: To review the features of patients with benign and malignant cystadenomas of the pancreas, focusing on preoperative diagnostic accuracy and long-term outcome, especially for nonoperated serous cystadenomas and resected cystadenocarcinomas. Summary Background Data: Serous cystadenomas (SCAs) are benign tumors. Mucinous cystic neoplasms should be resected because of the risk of malignant progression. A correct preoperative diagnosis of tumor type is based on morphologic criteria. Despite the high quality of recent imaging procedures, the diagnosis frequently remains uncertain. Invasive investigations such as endosonography and diagnostic aspiration of cystic fluid may be helpful, but their assessment is limited to small series. The management of typical SCA may require resection or observation. Survival after pancreatic resection seems better for cystadenocarcinomas (MCACs) than for ductal adenocarcinomas of the pancreas. Methods: Three hundred ninety-eight cases of cystadenomas of the pancreas were collected between 1984 and 1996 in 73 institutions of the French Surgical Association. Clinical presentation, radiologic evaluation, and surgical procedures were analyzed for 144 operated SCAs, 150 mucinous cystadenomas (MCAs), and 78 MCACs. The outcome of 372 operated patients and 26 nonoperated patients with SCA was analyzed. Results: Cystadenomas represented 76% of all primary pancreatic cystic tumors (398/522). An asymptomatic tumor was discovered in 32% of patients with SCA, 26% of those with MCA, and 13% of those with MCAC. The tumor was located in the head or uncinate process of the pancreas in 38% of those with SCA, 27% of those with MCA, and 49% of those with MCAC. A communication between the cyst and pancreatic duct was discovered in 0.6% of those with SCA, 6% of those with MCA, and 10% of those with MCAC. The main investigations were ultrasonography and computed tomography (94% for SCA, MCA, and MCAC), endosonography (34%, 28%, and 22% for SCA, MCA, and MCAC respectively), endoscopic retrograde cholangiopancreatography (16%, 14%, 22%), and cyst fluid analysis (22%, 31%, 35%). An accurate preoperative diagnosis of tumor type was proposed for 20% of those with SCA (144 cases), 30% of those with MCA, and 29% of those with MCAC. An atypical unilocular macrocyst was observed in 10% of SCA cases. The most common misdiagnosis for mucinous cystic tumors was pseudocyst (9% of MCAs, 15% of MCACs). Intraoperative frozen sections (126 cases) allowed a diagnosis according to definitive histologic examination in 50% of those with SCA and MCA and 62% of those with MCAC. For management, 93% of patients underwent surgery. Nonoperated patients (7%) had exclusively typical SCA. A complete cyst excision was performed in 94% of benign cystadenomas, with an operative mortality rate of 2% for SCA and 1.4% for MCA. Resection was possible in 74% of cases of MCAC. Mean follow-up of 26 patients with nonresected SCAs was 38 months, and no patients required surgery. For resected MCACs, the actuarial 5-year survival rate was 63%. Conclusions: Spiral computed tomography is the examination of choice for a correct prediction of tumor type. Endosonography may be useful to detect the morphologic criteria of small tumors. Diagnostic aspiration of the cyst allows differentiation of the macrocystic form of SCA (10% of cases) and the unilocular type of mucinous cystic neoplasm from a pseudocyst. Surgical resection should be performed for symptomatic SCAs, all mucinous cystic neoplasms, and cystic tumors that are not clearly defined. Conservative management is wholly justified for a well-documented SCA with no symptoms. An extensive resection is warranted for MCAC because the 5-year survival rate may exceed 60%.
Article
Diagnostic errors in the evaluation of cystic neoplasms of the pancreas (PCNs) are quite common. Few data are available regarding the impact of these errors on clinical management. The aim of this study was to determine the accuracy of a pancreatic multidisciplinary conference in diagnosing PCNs, to assess the potential risk of misdiagnosis, and to evaluate the clinical impact of these errors. A retrospective consecutive series of patients undergoing surgery for PCNs at Karolinska University Hospital between 2004 and 2012 was analyzed. During the study period, a total of 141 patients had undergone pancreatic resection for PCN. The overall accuracy of the preoperative diagnosis was 60.9 %. The rate of concordance between preoperative diagnosis and histology was similar for asymptomatic and symptomatic lesions (62.8 vs. 59.1 %; p = NS). The rate of correct diagnosis increased over time (54.5 % in 2004-2006, 61.7 % in 2007-2012, 63.5 % in 2010-2012). Univariate analysis identified the location of the lesion (diffuse pancreatic involvement) and a mucinous nature of the lesion as factors conducive to a correct diagnosis. Reevaluation of the original indication for surgery in light of the exact diagnosis showed that a surgical procedure should not have been performed in 12 patients (8.5 %). This study confirms that diagnostic errors are fairly common in the preoperative assessment of PCNs, but the errors are clinically relevant in <10 % of patients.
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Adult pancreatic hemangiomas are rare. We report a new case and review the literature. Pancreatic hemangiomas do not produce specific symptoms, so diagnosis is not easy. Computed tomography is often misleading, since pancreatic hemangiomas behave differently from liver hemangiomas, which are more frequently seen. Instead of showing arterial peripheral nodular enhancement followed by centripetal filling they take up the contrast material more slowly and the filling is usually inhomogeneous, which may give the impression of a cystic tumor. The reason for this may be that the microscopic structure of the pancreatic hemangioma is different from that of the liver. It partly consists of thin-walled sinusoids which do not communicate with the circulation of the patient. Since no malignant cases have been published to date, surgical resection might be avoided if the diagnosis can be firmly established.
Article
Background: After pancreaticoduodenectomy, severe pancreatic fistula may require salvage relaparotomy in patients with largely disrupted pancreaticojejunal anastomosis. Completion pancreatectomy remains the gold standard but yields high mortality and severe long-term repercussions. The authors report the results of a pancreas-preserving strategy used in this life-threatening condition. Methods: Two hundred fifty-four pancreaticoduodenectomies with pancreaticojejunal anastomosis were performed between 2005 and 2011; 21 patients underwent salvage relaparotomy for grade C pancreatic fistula. Largely dehiscent pancreaticojejunal anastomoses were dismantled in 16 patients. Four patients underwent completion pancreatectomy, whereas in 12 patients detailed here, the remaining pancreas was preserved and drained by wirsungostomy with exteriorization or closure of the jejunal stump. Repeat pancreaticojejunal anastomosis was later planned to preserve pancreatic function. Results: One patient died of recurrent hemorrhage on day 1 after wirsungostomy (8.3%). All but 1 survivor developed postoperative complications, and 3 needed reoperation before hospital discharge. The median hospital stay was 62 days (range, 29 to 156 days). After a median delay of 130 days (range, 91 to 240 days) from salvage relaparotomy, repeat pancreaticojejunostomy was attempted in 10 patients and was successful in 9 (1 completion pancreatectomy was performed). One patient died postoperatively (10%). Long-term endocrine function was unaltered in 66% of patients who benefited from this conservative strategy. Conclusions: This pancreas-preserving strategy yielded a whole mortality rate of 17% for largely disrupted pancreaticojejunal anastomosis requiring salvage relaparotomy. It compares favorably with systematic completion pancreatectomy and achieved preservation of remnant pancreatic function in 75% of patients.
Article
A large pancreatic cavernous hemangioma was found in a 30-year-old man with abdominal distention. Plain and contrast-enhanced computed tomography (CT), magnetic resonance imaging (MRI), ultrasonography (US), and angiography were performed prior to operation. Contrast-enhanced CT and angiography showed a large poorly enhanced hypovascular tumor at the head of the pancreas. But MRI and US disclosed findings compatible with a cavernous hemangioma.
Article
Pancreatic hemangioma is an extremely rare tumor, with only a very few cases reported in the literature. We present the case of a 36-year-old man admitted to the hospital with jaundice and abdominal pain. Contrast-enhanced CT showed a hypervascular mass with large vessels in the pancreatic head. The mass appeared moderately hypervascular on gadolinium-enhanced MRI, and, on mangafodipir-enhanced MRI, no contrast agent uptake into the mass could be detected. Despite the size of the lesion, no dilatation of the pancreatic and the common bile duct were seen. Pancreatic hemangioma should be included in the list of differential diagnoses in case of a hypervascular pancreatic lesion without signs of mass effect.
Article
This paper analyses the early postoperative complications after 285 pancreaticoduodenectomies performed during the past 15 years in the Surgical University Clinic, Mannheim. There were 235 partial (Whipple) and 52 total pancreatectomies performed for pancreatic and periampullary tumors (181 patients) and complicated chronic pancreatitis (104 patients). A total of 92 complications requiring relaparotomy in 42 patients ended fatally in nine patients. The overall operative and hospital mortality rate was 3.1%. The most frequent and most dangerous were complications at or around the pancreaticojejunal anastomosis, which occurred 25 times with five deaths. Postoperative hemorrhage was seen in 16 patients; endoscopic treatment in four patients and operation in 12 patients was successful in stopping the bleeding in all but one patient. Eight biliary fistulae either ceased spontaneously (3 patients) or after operative reintervention (5 patients) without any mortality. Control of these complications depends on four lines of approach: (1) before operation: optimal preparation of the jaundiced patient including endoscopic transpapillary decompression of the common duct; (2) during operation: a meticulous and standardized technique is mandatory; (3) after operation: continuous observation in the surgical intensive care unit is essential for the timely detection of possible complications; and (4) early reintervention can salvage the great majority of these patients with deleterious complications.
Article
A 62 year old woman lacking any alteration in her general condition and without digestive disorders was found fortuitously to have a large mass in the epigastric region. Complementary investigations localized the lesion to the peritoneal cavity, either in the gastrocolic omentum or the mesentery, close to the pancreas. Imaging showed a multicystic heterogeneous structure. A large hemangioma of pancreatic origin was removed at operation.
Article
Anastomotic leak at the pancreaticojejunostomy remains a common and dreaded complication after pancreaticoduodenectomy. Our aim was to determine the incidence, presentation, methods of management, and preoperative and postoperative factors that influence the clinical outcome. We reviewed our collective experience with 375 consecutive patients undergoing pancreaticoduodenectomy from 1980 to 1992 for a variety of pathologic indications. Clinical, biochemical, intraoperative, and postoperative factors were reviewed in an attempt to determine prognostic factors. Sixty-six patients (18%) developed a pancreatic anastomotic leak as determined by increased amylase in drainage fluid (44%), radiographic documentation (41%), operative re-exploration (9%), or percutaneous drainage of a peripancreatic, amylase-containing fluid collection (6%). Most leaks (73%) were clinically insignificant and were managed by simple maintenance of intraoperatively placed drains. Active intervention was required in 18 patients (27%) and included percutaneous drainage in 8, completion pancreatectomy in 7, and reoperative drainage with or without anastomotic repair in 3. Although octreotide was used therapeutically in 13 patients (20%), a significant, objective response was noted in only 1 patient. Five (8%) of the 66 patients died, all related directly to the pancreatic leak. The overall operative mortality was lower, 15 (4%) of 375 patients. Of the clinical, biochemical, intraoperative, and postoperative factors reviewed to determine prognostic factors, only postoperative intra-abdominal hemorrhage predisposed the patient to mortality as a result of the pancreatic anastomotic leak. We conclude that most anastomotic leaks at the pancreaticojejunostomy after pancreaticoduodenectomy can be managed conservatively. Use of octreotide to aid in closure of the pancreatic leak was disappointing, whereas patients with postoperative intra-abdominal hemorrhage or those requiring completion pancreatectomy to manage the anastomotic leak have increased mortality.
To research the clinical features of pancreatic cavernous hemangioma. Retrospectively analyzed 3 pancreatic cavernous hemangioma cases treated in our hospital during the last 20 years, and reviewed correlative literature. The incidence of pancreatic cavernous hemangioma was very low. It occurred more often in woman. As the early symptoms were not obvious and the imaging study were not specific, the misdiagnosis rate was high. Usually, the diagnosis was confirmed by the pathological result. Pancreatic cavernous hemangioma is a rare benign tumor of the pancreas. It's difficult to diagnosis before surgery. Surgery is the best treatment with satisfactory results, and its prognosis is fairly good.
Haemangioma cavernosum pancreatic
  • Ranström