Small intestinal hemolymphangioma with bleeding: a case report

Small intestinal hemolymphangioma is a very rare benign tumor. There was only one report of a hemolymphangioma of the pancreas invading to the duodenum until March 2011. Here we describe the first case of small intestinal hemolymphangioma with bleeding in a 57-year-old woman. She presented with persistent gastrointestinal bleeding and endoscopy revealed a small intestinal tumor. Partial resection of the small intestine was thus performed and the final pathological diagnosis was hemolymphangioma. We also highlight the difficultly in making an accurate preoperative diagnosis in spite of modern imaging techniques. To arrive at a definitive diagnosis and exclude malignancy, partial resection of the small intestine was considered to be the required treatment.     

Hematemesis as the initial complication of pancreatic adenocarcinoma directly invading the duodenum： A case report

Pancreatic carcinoma is a debilitating disease and carries a poor prognosis. It is a rare cause of upper gastrointestinal bleeding, even though pancreas, stomach, duodenum and jejunum are adjacent organs. The incidence of pancreatic adenocarcinoma directly invading the gastrointestinal tract leading to gastrointestinal hemorrhage is very low, and most of them present with melena and hematochezia. Here, we describe one unique case manifesting characteristically severe and unremitting hematemesis as an initial presentation of pancreatic adenocarcinoma. This tumor directly invaded the duodenal mucosa as a bleeding protruding tumor mass. Our MEDLINE search has confirmed that this is the first reported case with an initial manifestation of hematemesis from pancreatic adenocarcinoma in Asians. Pancreatic adenocarcinoma directly invading duodenum complicated by hemorrhage can be a rare cause of hematemesis, and clinicians should be reminded of it while they are making differential diagnosis.     

Hemolymphangioma: A rare differential diagnosis of cystic-solid or cystic tumors of the pancreas

We report a case of pancreatic hemolymphangioma. Hemolymphangioma is a malformation of both lymphatic vessels and blood vessels. The incidence of this disease in the pancreas is extremely rare. To the best of our knowledge, only seven cases have been reported worldwide (PubMed). A 39-year-old woman with a oneday history of abdominal pain was admitted to our hospital. There was no obvious precipitating factor. The preoperative examination, including ultrasonography and computed tomography, showed a cystic-solid tumor in the pancreas, and it was considered to be a mucinous cystadenoma or cystadenocarcinoma. Pancreaticbody-tail resection combined with splenectomy was performed. After the operation, the tumor was pathologically demonstrated to be a pancreatic hemolymphangioma. Although pancreatic hemolymphangioma is rare, we believe that it should be considered in the differential diagnosis of cystic-solid tumors of the pancreas, particularly when there is no sufficient evidence for diagnosing cystadenoma, cystadenocarcinoma or some other relatively common disease of the pancreas.     

Successful resection, using pancreas-sparing duodenectomy, of extrahepatically growing hepatocellular carcinoma associated with direct duodenal invasion

A 50‐year‐old man with extrahepatically growing hepatocellular carcinoma (HCC) associated with direct duodenal invasion underwent a right posterior segmentectomy associated with pancreas‐sparing duodenectomy. Neither periduodenal lymph‐node metastasis nor pancreatic invasion was detected, thus we separated the supra‐ampullary duodenum from the pancreatic head and performed pancreas‐sparing supra‐ampullary duodenectomy. The resected specimen was observed to be a centrally necrotic tumor that had infiltrated the duodenal wall, resulting in exposure of the lumen. Pathology examination revealed that the tumor consisted of poorly differentiated HCC, which had extensively infiltrated the mucosa of the duodenum. Gastrointestinal tract involvement in patients with HCC is rare, and pancreas‐sparing duodenectomy is a safe and effective treatment for patients with HCC associated with direct duodenal invasion.     

An extramural gastrointestinal stromal tumor of the duodenum mimicking a pancreatic head tumor

We report the case of a 53‐year‐old woman with a gastrointestinal stromal tumor (GIST) of the duodenum that showed only extramural growth, mimicking a pancreatic tumor. Preoperatively, computed tomography (CT) and angiography revealed a hypervascular mass, 3.0 cm in diameter, in the pancreatic head. Hypotonic duodenography showed compression of the second and third portions of the duodenum by the pancreatic lesion. Endoscopic examination showed no specific mucosal abnormalities in the duodenal lumen. The pancreatic head tumor was diagnosed preoperatively as a nonfunctioning islet cell tumor of the pancreas, and the patient underwent pylorus‐preserving pancreaticoduodenectomy. A hard mass was palpated intraoperatively in the pancreatic head region, and neither peritoneal dissemination nor metastasis was detected. Histologically, the tumor was composed of spindle‐shaped cells with a fascicular growth pattern, and only a few mitotic features were seen. Immunohistochemically, most of the tumor cells were positive for c‐kit oncoprotein and CD34, but negative for alpha‐smooth muscle actin and S‐100 protein. Therefore, this neoplasm was finally diagnosed as a duodenal GIST of the uncommitted type. This is a rare case of a duodenal GIST with exclusively extramural growth mimicking a pancreatic head tumor.     

Multiple carcinoids in the duodenum, pancreas and stomach accompanied with type A gastritis： A case report

We report a case of multiple duodenal, pancreatic, and gastric carcinoids. A 67-year old woman was admitted to our hospital for treatment of a duodenal carcinoid. Laboratory tests revealed that the patient was associated with macrocytic anemia and hypergastrinemia, and type A gastritis was shown by gastrofiberscopy. During surgery, another tumor was incidentally found in the head of the pancreas. The tumors in the duodenum and pancreas were completely excised by pancreatoduodenectomy and immunohistologically diagnosed as gastrin-and serotonin-producing carcinoids, respectively. Pathological examination revealed that in addition to the grossly found carcinoids, there were subclinical carcinoids, one of which was an endocrine cell micronest, located in the stomach and duodenum. The tumors in the duodenum, pancreas, and stomach showed different characteristics from one another morphologically and immunochemically. Although no definitive evidence has been obtained, some sort of genetic anomaly may have been involved in this case, and hypergastrinemia due to duodenal gastrinoma may induce multiple gastric carcinoids.     

Duodenal ulcer and pancreatitis associated with pancreatic arteriovenous malformation

Arteriovenous malformation (AVM) of the pancreas is a rare condition that may cause severe gastrointestinal bleeding. We describe a 54-year-old man with a 7-year history of recurrent duodenal ulcer due to AVM in the pancreatic head. We recommended pancreatoduodenectomy because of recurrent haemorrhage from the duodenal ulcer, but the patient refused surgery on several occasions. He was admitted to our hospital complaining of severe upper abdominal pain radiating to the back and was diagnosed with acute pancreatitis. He agreed at that stage to the surgical treatment. The resected specimen contained a highly vascular malformation in the pancreatic head and ulceration in the adjacent descending duodenum. Histopathological examination revealed numerous vascular structures with dilated and tortuous vessels in the pancreatic head, confirming the presence of AVM. Moreover, oedema, inflammatory cell infiltration, haemorrhage and necrosis were evident, confirming the presence of acute pancreatitis.     

Adenocarcinoma of the minor duodenal papilla treated with pancreas-sparing segmental duodenectomy: case report and review of the literature

Carcinoma of the minor duodenal papilla is extremely rare. We present the case of a 69-year-old man diagnosed with a tumor of the second portion of the duodenum by upper gastrointestinal endoscopy, which revealed a 1.5-cm elevated tumor with slight ulceration at the minor duodenal papilla. Biopsy revealed adenocarcinoma, and a computed tomography scan showed an enhanced tumor in the duodenum, with no abnormality in the pancreatic head. A pancreas-sparing segmental duodenectomy was performed, and the duodenum reconstructed with an end-to-end anastomosis. Microscopically, the tumor was a well-differentiated adenocarcinoma, with no infiltration at the cut end of the accessory pancreatic duct. The postoperative course was uneventful and the patient discharged on postoperative day 11. We reviewed previously reported cases of carcinoma of the minor duodenal papilla. Early and exact preoperative diagnosis of duodenal neoplasms makes it possible to select a less invasive treatment, which also maintains curability.     

A case of malignant tumor of the ascending part of duodenum with osteoclast-like giant cells

Extraskeletal neoplasms with osteoclast-like giant cells are very rare. These tumors are most frequently reported in the breast and pancreas, and but rarely in other sites. We report a case of duodenal malignant tumor with osteoclast-like giant cells. The patient was a 76-year-old man who presented with vomiting. Computed tomography, magnetic resonance imaging, and gastrointestinal endoscopy revealed a giant tumor in the ascending part of duodenum. Biopsy specimens showed an undifferentiated malignant tumor with benign multinucleated giant cells. Immunohistochemical staining indicated that the tumor cells were reactive with vimentin, but not with epithelial markers or the other mesenchymal markers, and the multinucleated giant cells were reactive with CD68. Thus, we diagnosed a malignant tumor of the ascending part of duodenum with osteoclast-like giant cells. To the best of our knowledge, this is the first case of duodenal malignant tumor with osteoclast-like giant cells in Japan.     

A gastrointestinal stromal tumor of the duodenum masquerading as a pancreatic head tumor

Gastrointestinal stromal tumor （GIST） represents the most common kind of mesenchymal tumor that arises from the alimentary tract. GIST is currently defined as a gastrointestinal tract mesenchymal tumor showing CD117 （c-kit protein） positivity at immunohistochemistry. Throughout the whole length of the gastrointestinal tract, GIST arises most commonly from the stomach followed by the small intestine, the colorectum, and the esophagus. Only 3%-5% of GISTs occur in the duodenum, and especially, if GIST arises from the C loop of the duodenum, it can be difficult to differentiate from the pancreas head mass because of its anatomical proximity. Here, we report a case of duodenal GIST, which was assessed as a pancreatic head tumor preoperatively.     

Multiple Hemolymphangioma of the Visceral Organs: A Case Report and Review of the Literature

Hemolymphangioma is a rare disease with malformation of both lymphatic and vascular vessels. Few cases of hemolymphangioma occurring in the rectum, small intestine, pancreas, esophagus, and other organs have been reported. Nevertheless, multiple hemolymphangioma of the visceral organs are extremely rare. We report a 25-year-old female with a significantly enlarged spleen full of multiple-rounded lesions. Curiously, the splenic flexure and even retroperitoneum had many lesions. The patient recovered well after splenectomy and the pathologic diagnosis of spleen was hemolymphangioma with abnormal lymphatic and blood vessels with polycystic spaces.Usually, it is hard to cure this disease. We should take much more consideration into the diagnosis, treatment, and even pathogenesis, even though it is a benign lesion.     

Retroperitoneal cavernous hemangioma resected by a pylorus preserving pancreaticoduodenectomy

A retroperitoneal hemangioma is a rare disease. We report on the diagnosis and treatment of a retroperitoneal hemangioma which had uncommonly invaded into both the pancreas and duodenum, thus requiring a pylorus preserving pancreaticoduodenectomy (PpPD). A 36-year-old man presented to our hospital with abdominal pain. An enhanced computed tomography scan without contrast enhancement revealed a 12 cm × 9 cm mass between the pancreas head and right kidney. Given the high rate of malignancy associated with retroperitoneal tumors, surgical resection was performed. Intraoperatively, the tumor was inseparable from both the duodenum and pancreas and PpPD was performed due to the invasive behavior. Although malignancy was suspected, pathological diagnosis identified the tumor as a retroperitoneal cavernous hemangioma for which surgical resection was the proper diagnostic and therapeutic procedure. Reteoperitoneal cavernous hemangioma is unique in that it is typically separated from the surrounding organs. However, clinicians need to be aware of the possibility of a case, such as this, which has invaded into the surrounding organs despite its benign etiology. From this case, we recommend that combined resection of inseparable organs should be performed if the mass has invaded into other tissues due to the hazardous nature of local recurrence. In summary, this report is the first to describe a case of retroperitoneal hemangioma that had uniquely invaded into surrounding organs and was treated with PpPD.     

Hemosuccus pancreaticus due to intraductal papillary-mucinous carcinoma of the pancreas

A 72-year-old female was referred to our hospital for evaluation of a hyperechoic mass in the pancreatic head with ultrasound sonography. She had no symptom expect slight anemia (Hb 11.3 g/dl). On endoscopy, blood was expelled from the orifice of the major duodenal papilla, but excretion of mucus was not detected. Endoscopic retrograde pancreatography revealed an irregular defect in the main pancreatic duct at the head of the pancreas. Computed tomography revealed a 2-cm mass with a low density lesion in the pancreas head. On suspicion of malignant tumor of the pancreas, pylorus-preserving pancreaticoduodenectomy was performed. Histological diagnosis was intraductal papillary-mucinous carcinoma without mucin hypersecretion. It grew within the inferior branch of the main pancreatic duct, and the top of the tumor stood out into the main pancreatic duct. As the causes of hemosuccus pancreaticus, pancreatic benign diseases, for example, chronic pancreatitis, pseudocyst, arterial aneurysm and pseudoaneurysm, are known, but pancreatic tumors are rare. In particular, this may be the first report of hemosuccus pancreaticus induced by intraductal papillary-mucinous carcinoma of the pancreas without mucin hypersecretion.     

Laparoscopic partial resection for hemangioma in the third portion of the duodenum

Benign duodenal tumors are rare and less common than malignant tumors. Furthermore, vascular lesions of the duodenum, including hemangiomas, are rare causes of gastrointestinal bleeding. This report describes a case with bleeding hemangiomas in the third portion of the duodenum and jejunum and their successful treatment using a laparoscopic approach. There is no report of totally laparoscopic resection for tumor in the third portion of duodenum. After performing a laparoscopic Kocher maneuver, the location of the duodenal hemangioma was confirmed by endoscopic and laparoscopic observation. The lesion was excised using ultrasonic coagulating shears and the defect in the duodenal wall was sutured laparoscopically. The hemangioma of the jejunum was treated extracorporeally through a 3.0 cm umbilical incision. The operating time was 241 min and blood loss was negligible. The postoperative course was uneventful. For benign duodenal tumors in the third portion, if endoscopic resection is not adapted, this less invasive technique may be a standard treatment.     

Unusual upper gastrointestinal bleeding due to late metastasis from renal cell carcinoma: a case report

A case of recurrent massive upper gastrointestinal bleeding originating from metastatic renal cell carcinoma is reported. A 63-year-old woman underwent right nephrectomy 9 years previously and experienced no recurrence during follow-up. A gradually enlarging ulcerative tumor over the bulb of the duodenum and four subsequent episodes of massive bleeding from this tumor occurred between June 2001 and March 2002. The patient underwent surgery in April 2002 for intractable bleeding from the tumor. Renal cell carcinoma metastasis to the duodenum was confirmed from the surgical specimen. Upper gastrointestinal bleeding due to malignancy is very rare and the duodenum is the least frequently involved site. Furthermore, a solitary late renal cell carcinoma metastasis 9 years after a nephrectomy is extremely uncommon. This case suggests that life-long follow-up of renal cell carcinoma patients is necessary, owing to unpredictable behavior and the possibility of long disease-free intervals. In nephrectomized patients suffering from gastrointestinal bleeding, complete evaluation, especially endoscopic examination, is indicated. The possibility of late recurrent renal cell carcinoma metastasis to the gastrointestinal tract should be kept in mind, although it is rare. If the patient is fit for surgery, metastatectomy is the first choice of treatment.     

Islet Cell Tumor Presenting as Massive Upper Gastrointestinal Bleeding in Pregnancy

A 27-yr-old woman presented in the 6th month of pregnancy with massive gastrointestinal bleeding due to erosion of a pancreatic islet cell tumor into the duodenum. Most islet cell tumors present with neuroendocrine manifestations; massive bleeding is a very rare presenting sign. This is the first reported case of a pregnant woman with massive bleeding from islet cell tumor erosion who underwent successful resection and delivery of a healthy term child 3 months later.     

Hemobilia due to biliary intraepithelial neoplasia associated with Zollinger–Ellison syndrome

A 58-year-old man was transferred to us from his local hospital because of failure to control his gastrointestinal bleeding by endoscopic hemostasis. Abdominal imaging suggested a hypervascular tumor of the pancreatic head (36?mm diameter), and laboratory testing showed an elevated serum gastrin level (17,800?pg/mL). Gastroduodenal endoscopy revealed multiple duodenal ulcers and active bleeding from the ampulla of Vater. The selective arterial secretagogue injection test suggested a gastrinoma in the pancreatic head, but no gastrinoma in the pancreatic tail. The patient was diagnosed with solitary pancreatic head gastrinoma complicated by hemosuccus pancreaticus, and pancreaticoduodenectomy was performed. Intraoperatively, the diagnosis was changed to primary peripancreatic lymph node gastrinoma without pancreatic involvement. The gastrointestinal bleeding stopped postoperatively and serum gastrin levels returned to normal. Histological examination of the surgical specimens revealed a small submucosal gastrinoma in the duodenum (7?mm diameter). The final diagnosis was microgastrinoma of the duodenum with peripancreatic lymph node metastasis. The cause of bleeding from the ampulla of Vater was initially obscure, but eventually a hemorrhagic erosion with moderate atypia was found in the common bile duct, indicating biliary intraepithelial neoplasia (BilIN). This is the first report of hemobilia due to BilIN with gastrinoma.     

Heterotopic pancreatitis with obstruction of the major duodenal papill--a rare trigger of obstructive orthotopic pancreatitis.

BACKGROUND: Heterotopic pancreas appears in 0.5 to 13% of autopsies. The most frequent locations are stomach, duodenum or upper jejunum. Pancreatitis in heterotopic pancreas is rarely described, and clinical symptoms caused by this heterotopic inflammation are uncommon. METHOD: We report a case of heterotopic pancreatitis localized in the major duodenal papilla causing biliary obstruction and mimicking a pancreatic head tumor. Clinically and radiologically, malignancy was suspected. Preoperative biopsies showed only fibrosis. A pylorus preserving resection of the pancreatic head was performed followed by an uneventful postoperative course. RESULT: Macroscopically, in the periampullary region on the pancreatic side a thickened duodenal wall with multiple lobules and cysts was found, compressing the common bile duct. Microscopic examination showed heterotopic pancreas with inflammatory lesions surrounding the ampulla. In the orthotopic pancreas a diffuse chronic pancreatitis with marked inflammation, fibrosis and atrophy of exocrine tissue was found. CONCLUSION: In our case it was impossible to differentiate between chronic pancreatitis and pancreas carcinoma preoperatively. Radiological findings and endoscopic biopsies were not sufficient to distinguish heterotopic pancreatitis from other tumors of the pancreatic head. Clear diagnosis could only be made by complete histological examinations after pancreatic head resection, being the treatment of choice for pancreatic head tumors of unclear dignity. The differential diagnosis of heterotopic pancreatitis as trigger of unclear enlargement of the pancreatic head is very seldom.