Carcinoid tumor of the duodenum and accessory papilla associated with polycythemia vera

Carcinoid tumors have been reported in a wide range of organs but most frequently involve the gastrointestinal tract; however, duodenal carcinoid tumors are rare. We report a 50-year-old male patient complaining of multiple melenas for 3 wk. The panendoscopy and endoscopic retrograde cholangiopancreaticography revealed swelling accessory papilla with an ulcer. The biopsy taken showed a carcinoid tumor. The lesion was removed by wide resection. Patient was found to have an abnormal blood cell count during the follow-up period with elevated levels of hemoglobin and hematocrit of 21.2 g/dL and 63.5%, respectively, thrombocytosis of 501 000/μL, and leukocytosis of 20 410/μL. He was diagnosed as a polycythemia vera by a hematologist after further evaluation. He received periodic phlebotomy and hydroxyurea treatment. The response was good and his hematocrit was stabilized by periodic phlebotomy in the range of 44-49% during the last 2 years. The possible origin of UGI bleeding by a duodenal carcinoid tumor, although rare, should be considered. There has been one case report of a duodenal carcinoid tumor that involved accessory papilla of the pancreas divisum and one case report of metastatic carcinoid tumor associated with polycythemia vera. It is different in our patient as compared with the latter report, which mentioned a polycythemia vera patient who was found to have a metastatic carcinoid in the 17 years follow-up period. Chemotherapy had been given before the carcinoid tumor was revealed. Our patient had no previous chemotherapy for polycythemia vera before he was found to have duodenal carcinoid tumor; this excludes the possibility of chemotherapy induced carcinoid tumor, although it had been suspected in the previous report. In our patient, the existence of both diseases may be by predisposition of each other since both diseases have an increased incidence of other neoplasm, or they may be coexistent incidentally.     

Management of parastomal ulcers

Management of surgically placed ostomies is an important aspect of any general surgical or colon and rectal surgery practice. Complications with surgically placed ostomies are common and their causes are multifactorial. Parastomal ulceration, although rare, is a particularly difficult management problem. We conducted a literature search using MD Consult, Science Direct, OVID, Medline, and Cochrane Databases to review the causes and management options of parastomal ulceration. Both the etiology and treatments are varied. Different physicians and ostomy specialists have used a large array of methods to manage parastomal ulcers; these including local wound care; steroid creams; systemic steroids; and, when conservative measures fail, surgery. Most patients with parastomal ulcers who do not have associated IBD or peristomal pyoderma gangrenosum (PPG) often respond quickly to local wound care and conservative management. Patients with PPG, IBD, or other systemic causes of their ulceration need both systemic and local care and are more likely to need long term treatment and possibly surgical revision of the ostomy. The treatment is complicated, but improved with the help of ostomy specialists.     

Management of chronic pancreatitis complicated with a bleeding pseudoaneurysm

Chronic pancreatitis is an ongoing disease characterized by persistent inflammation of pancreatic tissues.With disease progression,patients with chronic pancreatitis may develop troublesome complications in addition to exocrine and endocrine pancreatic functional loss.Among them,a pseudoaneurysm,mainly induced by digestive enzyme erosion of vessels in proximity to the pancreas,is a rare and life-threatening complication if bleeding of the pseudoaneurysm occurs.At present,no prospective randomized trials have investigated the therapeutic strategy for this rare but critical situation.The role of arterial embolization,the timing of surgical intervention and even surgical procedures are still controversial.In this review,we suggest that dynamic abdominal computed tomography and angiography should be performed first to localize the bleedersand to evaluate the associated complications such as pseudocyst formation,followed by arterial embolization to stop the bleeding and to achieve early stabilization of the patient’s condition.With advances and improvements in endoscopic devices and techniques,therapeutic endoscopy for pancreatic pseudocysts is technically feasible,safe and effective.Surgical intervention is recommended for a bleeding pseudoaneurysm in patients with chronic pancreatitis who are in an unstable condition,for those in whom arterial embolization of the bleeding pseudoaneurysm fails,and when endoscopic management of the pseudocyst is unsuccessful.If a bleeding pseudoaneurysm is located over the tail of the pancreas,resection is a preferential procedure,whereas if the lesion is situated over the head or body of the pancreas,relatively conservative surgical procedures are recommended.     

Acute ulcerative jejunal diverticulitis： Case report of an uncommon entity

Jejunal diverticulosis is a rare entity with variable clinical and anatomical presentations. Its reported incidence varies from 0.05% to 6%. Although there is no consensus on the management of asymptomatic jejunal diverticular disease, some complications are potentially life threatening and require early surgical treatment. We report a case of an 88-year-old man investigated for acute abdominal pain with a high biological inflammatory syndrome. Inflammation of multiple giant jejunal diverticulum was discovered at abdominal computed tomography （CT）. As a result of the clinical and biological signs of early peritonitis, an emergency surgical exploration was performed. The first jejunal loop showed clear signs of jejunal diverticulitis. Primary segmental jejunum resection with end-toend anastomosis was performed. Histopathology report confirmed an ulcerative jejunal diverticulitis with imminent perforation and acute local peritonitis. The patient made an excellent rapid postoperative recovery. Jejunal diverticulum is rare but may cause serious complications. It should be considered a possible etiology of acute abdomen, especially in elderly patients with unusual symptomatology. Abdominal CT is the diagnostic tool of choice. The best treatment is emergency surgical management.     

Uncomplicated spontaneous rupture of pancreatic pseudocyst into stomach: A case report

Pseudocysts of the pancreas are not rare, but spontaneous perforation and/or fistulization occurs in fewer than 3% of these pseudocysts. Perforation into the free peritoneal cavity, stomach, duodenum, colon, portal vein, pleural cavity and through the abdominal wall has been reported. Spontaneous rupture of the pancreatic pseudocyst into the surrounding hollow viscera is rare and, may be associated with life-threatening bleeding. Such cases require emergency surgical intervention. Uncomplicated rupture of pseudocyst is an even rarer occurrence. We present a case of spontaneous resolution of a pancreatic pseudocyst with gastric connection without bleeding. A 67-year-old women with a large pancreatic pseudocyst resulting from a complication of chronic pancreatitis was referred to our institution. During hospital stay, there was sudden decrease in the size of epigastric lump. Repeat computed tomography(CT) revealed that the size of the pseudocyst had decreased significantly; however, gas was observed in stomach and pseudocyst along with rent between lesser curvature of stomach and pseudocyst suggestive of spontaneous cystogastric fistula.The fistula tract occluded spontaneously and the patient recovered without any complication or need for surgical treatment. After 5 wk, follow up CT revealed complete resolution of pseudocyst. Esophagogastroduodenoscopy revealed that the orifice was completely occluded with ulcer at the site of previous fistulous opening.     

Hepatocellular carcinoma masquerading as a bleeding gastric ulcer： A case report and a review of the surgical management

Hepatocellular Carcinoma (HCC) is a common malignancyworldwide. While bleeding from the gastrointestinal tract(BGIT) has a well known association with HCC, suchcases are mainly due to gastric and esophageal varices.BGIT as a result of invasion of the gastrointestinal tractby HCC is extremely rare and is reportedly associatedwith very poor prognosis. We describe a 67-year-oldmale who presented with BGIT. Endoscopy showed thesite of bleeding to be from a gastric ulcer, but endoscopictherapy failed to control the bleeding and emergencysurgery was required. At surgery, the ulcer was found tohave arisen from direct invasion of the gastrointestinaltract by HCC of the left lobe. Control of the bleedingwas achieved by surgical resection of the HCC en-bloc with the lesser curve of the stomach. The patientremains alive 33 mo after surgery. Direct invasion of thegastrointestinal tract by HCC giving rise to BGIT is veryuncommon. Surgical resection may offer significantlybetter survival over non-surgical therapy, especially if thepatient is a good surgical candidate and has adequatefunctional liver reserves. Prognosis is not uniformly grave.     

Complete resection of isolated pancreatic metastatic melanoma: A case report and review of the literature

Isolated metastatic melanoma of the pancreas is very rare.Currently,there is very limited experience with surgical resection of pancreatic metastasis.The potential benefit of metastasectomy can improve the quality of life and survival time of patients.We present a case of a 39-year-old Chinese male with a solitary pancreatic tumor which was considered a cystic benign lesion for years.Pathology and immunohistochemistry showed that the tumor in pancreatic tail was a metastasis from a malignant melanoma of the eyeball.No other metastastic foci were found in abdomen.The tumor was completely resected with combined distal pancreatectomy and splenectomy.The patient has survived 25 mo without any signs of local recurrence or other metastatic lesions after operation,indicating that complete surgical resection of a solitary metastatic melanoma of the pancreas can prolong the survival time of patients.     

Acute lower gastrointestinal bleeding from a dieulafoy lesion proximal to the anorectal junction post-orthotopic liver transplant

INTRODUCTION A dieulafoy lesion is a submucosal ectatic arterial lesion associated with a tiny mucosal defect. It is a rare cause of profuse, but intermittent upper or lower gastrointestinal bleeding. The bleeding is often unresponsive to conservative tre…     

Resection of multiple rectal carcinoids with transanal endoscopic microsurgery:Case report

Multiple rectal carcinoids are rare.Due to the unreliability of endoscopic polypectomy in treating these submucosal lesions,a laparotomy is usually performed.We present a case report on multiple rectal carcinoids with three carcinoid foci<10 mm in diameter located in the midrectum.Preoperative examination showed the lesions to be confined to the submucosal layer with no perirectal nodal involvement.A transanal endoscopic microsurgerywas successfully performed to remove the three lesions with accurate full-thickness resection followed by secured suture closure.The postoperative pathology revealed neuroendocrine tumors G1(carcinoids)located within the submucosal layer without lymphatic or vessel infiltration.Both the deep and lateral surgical margins were completely free of tumor cells.The patient recovered quickly and uneventfully.No tumor recurrence was observed at the six-month follow-up.For the multiple small rectal carcinoids without muscularis propria or lymphatic invasion,transanal endoscopic microsurgery offers a reliable and efficient alternative approach to traditional laparotomy for select patients,with the added advantages of minimally invasive surgery.     

Rectal arterio-portal fistula: An unusual cause of persistent bleeding per rectum following a proximal spleno-renal shunt

Gastrointestinal arterio-venous malformations are a known cause of gastrointestinal bleeding. We present a rare case of persistent rectal bleeding due to a rectal arterio-portal venous fistula in the setting of portal hypertension secondary to portal vein thrombosis. The portal hypertension was initially surgically treated with splenectomy and a proximal splenorenal shunt. However, rectal bleeding persisted even after surgery, presenting us with a diagnostic dilemma. The patient was re-evaluated with a computed tomography mesenteric angiogram which revealed a rectal arterio-portal fistula. Arterio-portal fistulas are a known but rare cause of portal hypertension, and possibly the underlying cause of continued rectal bleeding in this case. This was successfully treated using angiographic localizationand super-selective embolization of the rectal arterioportal venous fistula via the right internal iliac artery. The patient subsequently went on to have a full term pregnancy. Through this case report, we hope to high-light awareness of this unusual condition, discuss the diagnostic workup and our management approach.     

Gastrointestinal hemorrhage needing blood transfusion as the first manifestation of small bowel carcinoid tumor

Carcinoid tumors arise from enterochromaffin or enterochromaffin-like cells that are present in the gastrointestinal tract, ovaries, and lungs. Over 90% of carcinoids originate in the gastrointestinal tract with the most common sites in order of frequency being the appendix, terminal ileum, rectum, and the remainder of the colon. Gastroduodenal and pancreatic carcinoids are infrequent. Carcinoid syndrome is associated with small intestine carcinoids in about 40%. Common symptoms include intermittent intestinal obstruction with crampy abdominal pain and vomiting, and weight loss. Upper gastrointestinal bleeding with melaena or hematochezia is a relatively rare early symptom of patients with small intestine carcinoid tumors. We report on a 69-year-old man, treated with acenocoumarol for previous thromboembolic complications of hereditary protein S deficiency. He was admitted to hospital because of an acute episode of hematochezia followed by melaena. Endoscopic evaluation of esophagus, stomach, duodenum and colonoscopy revealed no apparent source of bleeding. Selective angiographic evaluation of mesenterial arteries showed pathologic vasculature approximately in mid jejunum. Laparotomy revealed bleeding from a small submucosal malignant carcinoid tumor in small intestine and multiple large metastases within mesenteric tissue. Segmental resection of small intestine and exstirpation of the metastatic masses was performed. Postoperative period was uneventful. Cytotoxic chemotherapy in this adjuvant setting has not been recommended. Small intestinal carcinoid tumor has to be considered as a rare cause of gastrointestinal bleeding with melaena or hematochezia. Nevertheless, bleeding is a relatively rare early symptom of patients with small intestine carcinoid tumor.     

Resolution of Right-Sided Heart Failure Symptoms after Resection of a Primary Ovarian Carcinoid Tumor

Carcinoid tumors are rare neuroendocrine malignancies that typically originate from the gastrointestinal tract. Patients who are diagnosed with carcinoid heart disease generally have poor prognoses because of advanced metastases during staging and few therapeutic options. We present the case of a 61-year-old woman with right-sided heart failure, secondary to carcinoid heart disease caused by a primary ovarian carcinoid tumor. After undergoing surgical resection of the left ovary and fallopian tube, the patient experienced complete resolution of her heart failure symptoms. In addition to the patient''s case, we discuss the diagnosis, nature, and treatment of this rare condition.     

Jejunal carcinoid tumor mimicking leiomyosarcoma: preoperative diagnosis by endoscopic biopsy

Primary carcinoid tumor of the jejunum is rare, and is an unusual cause of massive gastrointestinal bleeding. A case of primary jejunal carcinoid tumor in a 39-year-old woman who presented with massive hematochezia is described. Both upper and lower gastrointestinal endoscopies showed no abnormalities. An abdominal computed tomographic scan, small-bowel barium contrast studies, and small-bowel endoscopy showed a subserosal mass, of 5 × 4 cm, with a cavity suggesting central necrosis, and a deep mucosal ulceration, located in the proximal jejunum. Although these clinical presentations were strongly suggestive of a leiomyosarcoma, histologic examination of biopsy samples obtained by enteroscopy confirmed the diagnosis of jejunal carcinoid tumor. The patient underwent radical jejunal resection and recovered uneventfully. In spite of the large size of the tumor, there was one solitary lymph node metastasis, but no evidence of liver metastases. This kind of jejunal carcinoid tumor, presenting with massive gastrointestinal bleeding and a subserosal bulky growth mimicking a leiomyosarcoma, has not been reported previously. Moreover, this is a rare case of a jejunal carcinoid which was diagnosed preoperatively by small bowel-endoscopic biopsy. Received: August 9, 1999 / Accepted: April 28, 2000     

Ileal carcinoid tumor complicated by retroperitoneal fibrosis and a prolactinoma

A patient with mid-gut carcinoid tumor and the unusual complication of retroperitoneal fibrosis was also found to have a prolactinoma. This case brings the number of reported mid-gut carcinoid tumors complicated by a second endocrine neoplasm to five. Three of the second tumors were parathyroid in origin, and the fourth was an insulinoma. In view of the rarity of second tumors and in the absence of documented familial occurrence, it is inappropriate to exhaustively study each person with mid-gut carcinoid tumor, or their families, for a second endocrine neoplasm; however, physicians caring for patients with mid-gut carcinoid should be aware that second tumors are possible. Retroperitoneal fibrosis is also a rare complication of carcinoid, but can be associated with renal failure that can be prevented by surgical intervention. Thus, physicians caring for patients with the carcinoid syndrome should also be aware of this complication.     

Duodenal gangliocytic paraganglioma with lymph node metastasis and an 8-year follow-up: a case report

Gangliocytic paraganglioma (GP) is a rare tumor, usually located in the second part of the duodenum. On pathological examination, GP is characterized by the association of the histological features of paragangliomas, ganglioneuromas, and carcinoid tumors. Classical clinical presentations are upper gastrointestinal bleeding and abdominal pain. Preoperative diagnosis is difficult because of the submucosal site of the tumor, with usually negative mucosal biopsies. Endoscopic ultrasound helps establish the diagnosis and allows lymph node staging, which will guide the choice of the treatment. If GPs usually follow a benign course, metastatic spread to regional lymph nodes treated by surgical resection alone has been reported. We report a case of GP with lymph node metastases treated by duodenopancreatectomy with long-term disease-free survival, suggesting that surgical resection is a reasonable approach for metastatic GPs.     

Carcinoid tumor of the thymus: a new case

A new case of carcinoid tumour of the thymus is reported. Such tumours are extremely rare and of somber prognosis when accompanied by local or regional metastases or metastases in other organs.     

Concurrent small bowel adenocarcinoma and carcinoid tumor in Crohn's disease — Case report and literature review

Adenocarcinomas of the large and small bowel, as well as intestinal carcinoid tumors have been reported at increased rates in Crohn's disease. We herein report a rare case of concurrent adenocarcinoma and carcinoid tumor of the small bowel presenting as intestinal obstruction and found incidentally at laparotomy in a 55 year old male with longstanding ileal Crohn's disease. We performed a Medline Pubmed search for cases of synchronous or composite adenocarcinoma and carcinoid tumor in the setting of Crohn's disease and identified four similar cases. Concurrent adenocarcinoma and carcinoid tumor occurred both in newly diagnosed and longstanding Crohn's disease, most commonly involved the terminal ileum and presented with symptoms mimicking Crohn's disease. Diagnosis was made incidentally at laparotomy in all cases. Lymph node involvement was variable. Clinicians should be aware of this rare entity for expeditious surgical intervention.     

Factors associated with complete local excision of small rectal carcinoid tumor

Purpose

Although small rectal carcinoid tumors can be treated using local excision, complete resection can be difficult because tumors are located in the submucosal layer. We evaluate the factors associated with pathologically complete local resection of rectal carcinoid tumors.

Methods

Data were analyzed of 161 patients with 166 rectal carcinoid tumors who underwent local excision with curative intent from January 2001 to December 2010. A pathologically complete resection (P-CR) was defined as an en bloc resection with tumor-free lateral and deep margins. The study classified treatments into three categories for analysis: conventional polypectomy (including strip biopsy, snare polypectomy, and hot biopsy), advanced endoscopic techniques (including endoscopic mucosal resection with cap and endoscopic submucosal dissection), and surgical local excision (including transanal excision and transanal endoscopic microsurgery). We evaluated the P-CR rate according to treatment method, tumor size, initial endoscopic impression and the use of endoscopic ultrasound (EUS) or transrectal ultrasound (TRUS).

Results

The mean tumor size was 5.51?±?2.43 mm (range 2–18 mm) and all lesions were confined to the submucosal layer. The P-CR rates were 30.9, 72.0, and 81.8 % for conventional polypectomy, advanced endoscopic techniques, and surgical local excision, respectively. Univariate analysis showed that P-CR was associated with treatment method, use of EUS or TRUS, and initial endoscopic impression. Multivariate analysis showed that only treatment method was associated with P-CR.

Conclusion

Pathologically complete resection of small rectal carcinoid tumors was more likely to be achieved when using advanced endoscopic techniques or surgical local excision rather than conventional polypectomy.     

Bronchial carcinoid tumors: radiologic observations in 49 cases.

A retrospective evaluation was performed of radiographs obtained in 49 cases of bronchial carcinoid at presentation and during a follow-up period of 12 years. Histologic diagnosis from the surgical specimen was available in all cases. Carcinoids appeared most frequently (77%) as round or oval opacities with sharp and often notched margins. They often induced airway compression with pulmonary atelectasis; enlarged hilar lymph nodes from metastasis were rare. Recurrence after surgical removal was not frequent; the recurrent masses had the same radiographic features as the original tumor. The diagnosis of bronchial carcinoid must be taken into consideration when a slowly growing radiopaque mass with well-defined margins is discovered on chest films. The radiologist must remember that these tumors can be resected with a fairly good prognosis even when they are large.