Pradeep Chopra

Large duodenal gastrointestinal stromal tumor presenting with acute bleeding managed by a whipple resection. A review of surgical options and the prognostic indicators of outcome.

CONTEXT Duodenal gastrointestinal stromal tumors (GISTs) are uncommon and constitute a relatively small subset of GISTs which presents a unique dilemma having various surgical options. A case of a large ulcerating duodenal GIST arising from the second and third parts of the duodenum and involving the pancreas which was managed by a Whipple resection is presented. The literature is also reviewed to present the current status on surgical options, outcome, prognostic indicators and the role of imatinib mesylate in its management. CASE REPORT A 58-year-old patient presented with acute gastrointestinal bleeding which was diagnosed to be due to a duodenal GIST following CT scan and endoscopic biopsy. The mass which measured about 10x9 cm originated from the 2nd part and extended into the 3rd part of the duodenum. He underwent a Whipple resection, and histopathology confirmed a duodenal GIST having a greater than 10 mitotic count per fifty high power field and areas of necrosis. Postoperatively, he received imatinib mesylate 400 mg bid; however, 4 months later, he presented with multiple disseminated peritoneal metastases and succumbed to the disease 2 months later. CONCLUSION GISTs of the duodenum which are small in size and do not involve the papilla of Vater are better resolved using a limited resection of the duodenum since the outcome in terms of operative risk or disease recurrence is not influenced in these cases. However, large tumors with more extensive involvement would require a pancreaticoduodenectomy to achieve adequate tumor clearance. Even though duodenal GISTs have a relatively better prognosis as compared to GISTs at other sites, their aggressiveness ranges from small indolent tumors to aggressive sarcomas. Following tumor resection, a recurrence rate of about 40% has been reported. A more favorable prognosis in duodenal GISTs is attributed to a lower prevalence of P53 loss, the duodenal location of the tumor, a smaller size of the lesion and a low mitotic count. Imatinib mesylate is reported to play a role in neoadjuvant therapy as well as in the management of metastatic and recurrent disease, although some of these tumors may fail to respond.

Pancreatic Metastasis from Colon Carcinoma Nine Years after a Hemicolectomy Managed by Distal Pancreatectomy. A Review of the Literature Regarding the Role and Outcome of Pancreatic Resection for Colorectal Metastasis

CONTEXT Pancreatic metastasis from colorectal malignancy is rare and accounts for less than 2% of all pancreatic metastases. A case of colonic metastasis to the pancreas is reported and the literature is reviewed to assess the role and outcome of pancreatic resection for metastatic tumors from colorectal malignancy. CASE REPORT A 58-year-old female underwent an emergency left hemicolectomy for an obstructing descending colon growth. The lesion was reported to be adenocarcinoma, Dukes C, with involvement of the serosa and 3 lymph nodes. A postoperative staging CT scan showed no other metastases and she received 6 cycles of FOLFOX chemotherapy (folinic acid, 5-flurouracil and oxaliplatin). Nine years after the colectomy during a routine follow-up, there was a sudden rise in her CEA levels. A CT scan revealed a 6.8x4.8 cm mixed consistency lesion in the tail of the pancreas which, on fine needle aspiration cytology, was confirmed to be adenocarcinoma. She underwent a distal pancreatectomy, and histopathology of the resected specimen confirmed a metastatic tumor from colon cancer. She then received 5 cycles of adjuvant chemotherapy. She was symptom free for nine months and subsequently succumbed to recurrent disease. CONCLUSION Pancreatic metastasis from colorectal malignancy is rare. These patients could be asymptomatic in 17% of cases. The time-interval between the diagnosis of colorectal cancer and the detection of pancreatic metastasis varies widely but is approximately 24 months. The median survival time for post-pancreatic resection is 16 months. Pancreatic resection appears to offer good palliation until recurrence of the disease occurs and the possibility of long term cure is rare.

Choledochal Cyst in Adults: Etiopathogenesis, Presentation, Management, and Outcome-Case Series and Review.

Background. Choledochal cyst, a rare congenital cystic dilatation of biliary tree, is uncommon in adults. Their presentations differ from children and surgical management has evolved. Methods. A retrospective review of the records of all the patients above 15 years, who underwent therapeutic intervention in our hospital, was carried out. Results. Ten cases of choledochal cyst were found; 8 female, with mean age 31 years. These included 8 cases of Todani type I and one case each of type II and type III. The predominant symptoms were abdominal pain and jaundice. Abdominal mass and past history of cholangitis and pancreatitis were seen in 2 patients. Investigations included ultrasound in 8 patients, CT in 7, ERCP in 3, and MRCP in 5. Surgical intervention included complete excision of the cyst with hepaticojejunostomy and cholecystectomy (type I), excision of the diverticulum (type II), and ERCP sphincterotomy (type III). Malignancy was not seen in any patients. The long-term postoperative complications included cholangitis in two patients. Conclusion. Choledochal cyst is rare in adults. The typical triad of abdominal pain, jaundice, and mass is uncommon in adults. The surgical strategy aims for single stage complete excision of the cyst with hepaticojejunostomy.

Portal vein thrombosis postlaparoscopic splenectomy presenting with infarction of gut: review of risk factors, investigations, postoperative surveillance, and management.

Portal vein thrombosis after laparoscopic splenectomy is a known complication even though it is underappreciated. Presenting symptoms are usually mild and nonspecific. Progression to intestinal infarction and portal hypertension are potentially life threatening complications. The short hospital stay associated with laparoscopic approach could delay early diagnosis, unless routine imaging studies is planned after discharge. We present a patient who after laparoscopic splenectomy for idiopathic thrombocytopenic purpura developed portal vein thrombosis leading to infarction of small intestine 9 days after the surgery. She made uneventful recovery after resection and anticoagulation. Literature is reviewed to assess the risk factors and discuss the present status regarding investigations, surveillance in postoperative period, management of established case, and role of prophylactic anticoagulation.

Laparoscopic Splenectomy Using LigaSure.

LigaSure as a vessel sealing system appears to allow safe performance of laparoscopic splenectomy.

Axillary lymph node metastasis from thyroid malignancy: unusual presentation with ominous implications.

Lymph node metastases to cervical group of nodes occur frequently in well-differentiated thyroid carcinoma. Metastasis to axillary lymph nodes however, is exceptional, leading to diagnostic and management dilemma. While presenting a case of thyroid malignancy with axillary lymph node metastasis, the literature is reviewed for similar cases to analyze their clinico-pathological features, investigations, management and outcome. Fifteen cases have been reported so far. Majority of these patients were female (male: female ratio 6:9) and their mean age was 54.5 years. Axillary lymph node metastases occurred concurrently or as recurrent disease after initial treatment of primary disease in about equal number of these patients. The predominant histological type was papillary carcinoma and 83.3% of these were poorly differentiated. Visceral metastases to lungs and bones were often seen. Despite treating these patients with thyroidectomy, lymph node clearance and adjuvant therapy, 40% of them died; some within one year and among the 60% who were alive, 30% still had active disease. Conclusion: Axillary lymph node metastasis from thyroid cancer, even though rare should be considered in the differential diagnosis in patients presenting with axillary mass and thyroid malignancy. Based on the limited number of cases reported in the

Acute massive splenic infarction with complete liquefaction of the spleen in sickle cell disease

To the Editor: The link between splenic sequestration and massive infarction has been noted by several authors.1,2 Large splenic infarcts have been reported in sickle cell trait patients with normal sized spleens, an event precipitated by hypoxia during high altitude flights in an unpressurised aircraft or during mountain climbing. Massive splenic infarction has also been reported in patients with other sickle cell variants, where splenomegaly is common mainly in Hb sickle cell disease (SCD), in Sβ+ thalassaemia and Hb SE disease.3 Many of these patients start with splenic sequestration syndrome, followed by clinical deterioration leading to splenic infarction. In SCD, infarction is reportedly induced by stressful conditions including severe generalized painful crisis, septicemia, the postoperative period, strenuous exercise and in the postpartum state. It has also been reported following high altitude travel, including flying in modern pressurized aircraft. Hence SCD patients with splenomegaly are advised against air travel when possible, and if deemed unavoidable, they are advised to have a pre-travel simple or exchange transfusion in addition to adequate hydration. In a study of 134 SCD patients who underwent splenectomy in Saudi Arabia, only two patients (1.49%) had massive splenic infarction.3 Repeated attacks of vaso-occlusion, which are usually small and often asymptomatic leading to splenic infarction, often occur in children and result in autosplenectomy. However a significant number of SCD patients in Oman (60%) have preserved splenic function with some splenomegaly, well into adulthood, making them liable for splenic complications including sequestration and infarction.4,5 The preserved spleen is due in part at least to the high prevalence of both alpha-thalassaemia (48%), and betathalassaemia (2.6%) in the country.5 Similar results in the temporal sequence of splenic dysfunction in SCD have been reported by others in the region.6 While splenic infarction is a well recognized complication of SCD, massive splenic infarction with complete liquefaction of the spleen is rarely reported.7 We report our experience with three patients with SCD who had massive splenic infarction among a large number of patients with SCD who had splenectomy (Table 1, 2). All our patients with splenic sequestration had elevated HbF at a mean of 7.1% in general and 22% among the three patients with massive infarction. Noticeably, patients with massive splenic infarction had a significantly elevated HbF (P=.0001, unpaired t test). Although elevated HbF is usually compatible with a milder course for SCD, and in association with thalassaemia, helps to preserve the spleen into adulthood, high Hb F in these patients did not seem to protect them from getting splenic infarction or sequestration.8 On the contrary, this study suggest that a significantly elevated HbF may indeed be a predictor of high risk for massive splenic infarction when the spleen is preserved into adulthood, and under conditions that may lead to tissue hypoxia like stress, infections, hypoxia, strenuous exercise and high altitude. Sickling of erythrocytes occurs in efferent channels of the spleen, which sets a chain of reactions that progressively involves more efferent channels culminating in a large splenic infarction. Studies of blood flow through the spleen done during periods of acute splenic sequestration, verify that blood flow is greatly retarded and sluggish.7 Therefore, in the presence of the above precipitating factors, patients with SCD who develop acute splenic sequestration are at risk of Table 1. laboratory test results in sickle cell disease patients, including three patients with massive splenic infarction.