Correlation of the ICG test with risk factors and postoperative outcomes following hepatic resection.

PURPOSE: Liver failure is a major cause of early mortality following hepatectomy. The future-remnant liver function is an important factor when assessing the risk for postoperative liver functional impairment. Several techniques have been established for this evaluation, including the indocyanine green (ICG) test. The aim of this study was to evaluate the ICG clearance in patients scheduled for liver resection regarding perioperative and postoperative risk factors. METHODS: Thirty-one patients, scheduled for liver resection, underwent the ICG test. Peri-operative and postoperative variables were recorded and analyzed using non-parametric tests. RESULTS: Procedures extended from wedge excisions to extended hepatectomies. Plasma disappearance rate (PDR) was found positively correlated with total blood loss, transfusion and operation duration. There were 11 primary hepatic malignancies, including hepatocellular carcinomas and cholangiocarcinomas, 13 metastatic carcinomas, mainly of colorectal origin, and 7 benign lesions. The uninvolved liver parenchyma was normal in 20 (64. 5%) cases. Two patients died due to myocardial infarction and postoperative liver failure, respectively. CONCLUSION: The role of residual liver function and particularly the hepatic reserve assessment on liver surgery may be of most benefit in the routine stratification of risk, enabling surgical procedures to be performed with safety. The ICG clearance markers were found significantly correlated with perioperative risk factors in histologically "normal" liver parenchyma. In addition to computed tomographic (CT) volumetry, functional assessment of the hepatic reserve with the ICG test may persuade the preoperative planning and prevent postoperative liver failure.

"Enteroatmospheric fistulae"--gastrointestinal openings in the open abdomen: a review and recent proposal of a surgical technique.

The occurrence of an enteric fistula in the middle of an open abdomen is called an enteroatmospheric fistula, which is the most challenging and feared complication for a surgeon to deal with. It is in fact not a true fistula because it neither has a fistula tract nor is covered by a well-vascularized tissue. The mortality of enteroatmospheric fistulae was as high as 70% in past decades but is currently approximately 40% due to advanced modern intensive care and improved surgical techniques. Management of patients with an open abdomen and an enteroatmospheric fistula is very challenging. Intensive care support of organs and systems is vital in order to manage the severely septic patient and the associated multiple organ failure syndrome. Many of the principles applied to classic enterocutaneous fistulae are used as well. Control of enteric spillage, attempts to seal the fistula, and techniques of peritoneal access for excision of the involved loop are reviewed in this report. Additionally, we describe our recent proposal of a lateral surgical approach via the circumference of the open abdomen in order to avoid the hostile and granulated surface of the abdominal trauma, which is adhered to the intraperitoneal organs.

Management of retroperitoneal sarcomas: main prognostic factors for local recurrence and survival.

PURPOSE: Retroperitoneal sarcomas (RPs) are characterized by slow indolent growth and metastasize at a late point in their natural course. The purpose of this study was to review our experience in the management of RSs and identify prognostic factors for local control and survival. METHODS: Between January 1990 and December 2010, the hospital records of 75 patients with RSs were retrospectively studied. Sixty-four (85.3%) patients had undergone surgical resection in our hospital for primary RS, whereas 11 (14.7%) were referred to our department for recurrent disease. RESULTS: The patient median age was 57 years. Median tumor size was 18.5 cm. The most common histologic type was liposarcoma (44%) followed by leiomyosarcoma (17%), paraganglioma (10%), malignant fibrous histiocytoma (6.5%) and rare tumors such as 2 chondrosarcomas and 1 pecoma. Complete initial resection with negative macroscopic margins (R0) was achieved in 39 (60.9%) patients. En bloc resection of adjacent organs was required in 8 (12.5%) patients with primary RS and in 8 (72.9%) with recurrent disease. Mortality rate was 4%. For the group of patients initially treated in our hospital, 1-year recurrence rate was 34.3%. The 3- and 5-year overall survival rates were 56.2% and 53.1%, respectively. Satellite tumors were recognised in 13 (20.3%) patients treated for primary RS, from whom 11 (84%) recurred within one year. Seven patients received adjuvant chemotherapy. CONCLUSION: Radical surgical resection is the treatment of choice for patients with primary and locally recurrent RSs.

Long-term results with the modified Sugiura procedure for the management of variceal bleeding: standing the test of time in the treatment of bleeding esophageal varices.

BACKGROUND: The surgical approaches to the treatment of bleeding esophageal varices in cirrhotic patients have been reduced since the clinical development of endoscopic sclerotherapy, transjugular intrahepatic portosystemic shunt (TIPS), and liver transplantation. However, when acute sclerotherapy fails, and in cases where no further treatment is accessible, emergency surgery may be life saving. In the present study we retrospectively analyzed the results of the modified Sugiura procedure, performed as emergency and semi-elective treatment in the patient with bleeding esophageal varices. METHODS: Ninety patients with cirrhosis and portal hypertension were managed in our department for variceal esophageal bleeding between January 1985 and December 1992. The modified Sugiura procedure was performed in 46 patients on an emergency (25 patients) or semi-elective (21 patients) basis. Liver cirrhosis stage according to Child classification was A in 4 patients, B in 16 patients, and C in 26 patients. RESULTS: Acute bleeding was controlled in all patients. Postoperative mortality was 23.9% (11 of 46 patients). The mortality rate was 34.6% in Child class C patients (9 of 26 patients), and 12.5% in Child class B patients (2 of 16 patients). Twenty-four patients had long-term follow-up extending from 14 months to 22 years (mean 83.1 months). Ten of 24 patients (41.6%) did not develop rebleeding for 5-22 years (mean 10.3 years). Overall 5-year survival in these 24 patients was 62.5%. CONCLUSIONS: The modified Sugiura procedure remains an effective rescue therapy for patients with bleeding esophageal varices when alternative treatments fail or are not indicated. Moreover, it can be a life-saving procedure in patients with anatomy unsuitable for shunt surgery or for patients treated in nonspecialized centers where surgical expertise for a shunt operation is not available.

Super-selective hepatic arterial infusions as established technique ('ARETAIEION' Protocol) of [177Lu]DOTA-TATE in inoperable neuroendocrine liver metastases of gastro-entero-pancreatic (GEP) tumors.

AIM: Aim of this study was to evaluate the effectiveness of non-carrier added (n. c. a.) [177Lu]DOTA-TATE in inoperable liver metastases, positive for sst2 receptor overexpression (verified by Octreoscan and confirmed by biopsy) due to neuroendocrine gastroenteropancreatic (GEP) tumors. [177Lu]DOTA-TATE has been infused after selective catheterization of the hepatic artery, minimising in parallel the toxicity of non-target tissues. METHODS: The dose per session administered to each patient (12 cases in total) was 7400 MBq (200 mCi). Repetitions did not exceed 6-fold with treatment intervals of 5-8 weeks. Response assessment was classified according to the therapeutic benefit. Absorbed doses delivered to metastases, kidneys and red marrow were calculated according to OLINDA 1.1 program and the derived values were correlated to the Response Evaluating Criteria in Solid Tumors (RECIST). CT/MRI scans were performed as baseline before, during and after the end of treatment and monthly ultrasound images for follow-up estimation and measurements. Toxicity (World Health Organization criteria) was measured using blood and urine tests of renal, hepatic and bone marrow function. RESULTS: None of the patients resulted complete response (0.0%); partial response was assessed in 8 (66.7%), disease stabilization in 3 (25%) and progressive disease in 1(8.3%). A 14-month median survival time was estimated for all patients, so far. Eight of 12 (66.7%) showed a mean target diameter shrinkage ranging from 33% to 45%. The organ average radiation dose estimation was found as follows: a) liver tumor 20.8 mGy/MBq; b) liver 0.14 mGy/MBq; c) kidneys 0.41 mGy/MBq; d) spleen 1.4 mGy/MBq; and f) bone marrow 0.022 mGy/MBq. The average absorbed dose per session to a tumor for a spherical mass of 20 g was estimated to be 20.8 mGy/MBq, depending on the histotype of the tumor. WHO toxicity grade 2 to 3 erythro-, leuko- and thrombo-cytopenia occurred in 9 (75%) cases observed about after the third session. CONCLUSION: In unresectable metastatic liver lesions positive for somatostatin receptors repeated, trans-hepatic high doses of [177Lu]DOTA-TATE resulted in a more than promising therapeutic outcome with a partial response in 75% of the treated patients. Given the loco-regional modality character of the administration technique, no nephro-toxicity has been so far observed whereas a remarkable myelotoxicity was noticed.

Solitary adrenal metastasis from esophageal adenocarcinoma: a case report and review of the literature.

Introduction. In patients with extra-adrenal malignancy, an adrenal mass necessitates investigating the possibility of metastatic tumor. Curable adrenal metastasis are considered as a rare event. Case report. A 52-year-old male suffering from lower esophageal adenocarcinoma with a solitary left adrenal metastasis is presented herein, who underwent concomitant transhiatal esophagectomy and left adrenalectomy. The patient remains disease-free 18 months later. Discussion. Adrenal metastases mostly occur in patients with lung, kidney, breast, and gastrointestinal carcinomas. Primary esophageal adenocarcinoma gives adrenal metastatic deposits according to autopsy series with an incidence of about 3%-12%. When no other evidence of metastatic disease in cancer patients exists, several authors advocate adrenalectomy with curative intent. Isolated cases of long-term survival after resection of solitary adrenal metastasis from esophageal adenocarcinoma, like in our case, have been reported only as case reports. Conclusion. This study concludes that surgical resection may result in survival benefit in selected patients with solitary adrenal metastasis from esophageal adenocarcinoma.

Effect of collagen powder on lymphorrhea after modified radical mastectomy. A randomized controlled trial.

AIM: Postoperative lymphorrhea is a major complication of axillary lymphadenectomy. The aim of our study was to evaluate the impact of type I collagen in postoperative lymphorrhea in mastectomy patients. METHODS: Eighty patients that underwent modified radical mastectomy for breast cancer were randomized in two groups. In group A (collagen group, n = 42) collagen type I (Cellerate RX powder) was applied in the axillary cavity after lymphadenectomy while in group B (control group, n = 38) lymphadenectomy was performed in the standard fashion without the use of a sealant. Suction drains remained in place until the daily amount of lymphatic drainage fell under 30 ml. The total amount and the duration of drainage, as well as the morbidity and severity of arm pain were compared in the two groups. RESULTS: There was a non significant trend towards lower overall drainage in the collagen group. The duration of drainage and postoperative pain were similar in the two groups, as was morbidity. Subgroup analysis of patients according to the number of lymph nodes excised, revealed significantly less lymphorrhea in terms of volume and duration in patients who had more than ten lymph nodes excised. CONCLUSION: Collagen type I (Cellerate RX powder) appears to attenuate postoperative lymphorrhea in patients undergoing axillary lymphadenectomy especially when > 10 lymph nodes are removed.

Results of salvage abdominoperineal resection after failed chemoradiation therapy for epidermoid anal canal carcinoma: Retrospective analysis at a single institution.

PURPOSE: The standard therapeutic approach to epidermoid anal canal cancer consists of combined radiation therapy and chemotherapy. Although disease control has been reported to have excellent results, as many as 40% of patients will develop locoregional disease progression. The treatment of choice for patients with persistent (PD) or recurrent disease (RD) is salvage abdominoperineal resection (APR). The purpose of this study was to review our experience with salvage surgery in this group of patients. METHODS: The medical records of all patients with epidermoid anal canal cancer treated from 1997 to 2010 in our department were retrospectively reviewed. Nine patients who presented with persistent or locally recurrent anal canal cancer were subjected to salvage APR. Before surgery, all of the patients had received chemoradiation therapy (CRT). RESULTS: There were 9 patients (7 women, 2 men) with a median age of 59 years (range 40-79). Six patients underwent radical salvage APR for persistent disease and 3 patients for recurrent disease. There were no deaths attributable to operation. The median follow-up time was 31.75 months (range 3-108) after salvage surgery. Two patients died of disease progression, with a median survival time of 24 months (range 12-36). At the time of last follow-up, 6 patients were alive without evidence of recurrent disease, and one patient was lost to follow-up. The median follow-up time for survivors was 34.3 months (range 3-108). CONCLUSION: Long-term survival can be achieved in the majority of patients who undergo radical salvage APR after failed CRT for epidermoid carcinoma of the anal canal.

Colorectal cancer emergencies during pregnancy case reports.

Colorectal carcinoma emergencies during pregnancy are exceptionally rare. Three women 38, 31 and 36 years old, in the third trimester of gestation received treatment, respectively, for acute abdomen due to perforation of rectal carcinoma, ileus due to a sigmoid tumor, and deep venous thrombosis (DVT) from a cecal tumor compromising the right iliac vein. In the first two patients urgent cesarean sections were carried out with Hartmann's procedure and a loop colostomy was performed to resolve the ensuing intraabdominal sepsis and ileus, respectively. In the third patient, a cesarean section was carried out to treat the underlying DVT more aggressively, while right colectomy was postponed for three weeks. Restoration of the alimentary tract was achieved two months later in the first case, while in the second and third cases total colectomy due to familial polyposis and right colectomy were performed three weeks after the cesarean section. An overview of the clinical features, diagnostic pitfalls and therapeutic approaches to manage complications of colorectal cancer during pregnancy are discussed.

Identification of germline BRCA1 and BRCA2 genetic alterations in Greek breast cancer moderate-risk and low-risk individuals--correlation with clinicopathological data.

The current study was designed to evaluate the prevalence of BRCA1 and BRCA2 germline mutations in Greek moderate- and low-risk individuals with respect to clinicopathological phenotype and clinical outcome of breast cancer. Ninety-four consecutive individuals were prospectively recruited from two University Breast Cancer Clinics (Hippokrateion Hospitan and Laikon Hospital) between 1989 and 1999 and were categorized as moderate-risk and low-risk individuals for carrying BRCA1/2 germline mutations. To identify the underlying mutations, protein-truncation test and single-strand conformation polymorphism methods were used, followed by direct sequencing. Three novel BRCA1 missense mutations, one novel BRCA1 intronic deletion, three novel (previously reported) BRCA2 truncating mutations, and one novel BRCA2 missense mutation were identified in the moderate-risk group of individuals studied. The BRCA1/2 missense mutations as well as the single intronic variant identified were designated as unclassified genetic variants. Two BRCA1 unclassified genetic variants (missense mutations) were detected in two of the three (66.7%) male breast cancer patients analyzed, while the third one was identified in a sporadic (low-risk) breast cancer patient. Clinicopathological characteristics of breast carcinomas originating from BRCA1/2 heterozygotes were consistent with those already reported and not different from those observed in BRCA1/2 mutation (-) breast cancer patients. Furthermore, BRCA1/2 mutation carriers presented an excellent 4.5-year overall survival (100%). Our results reveal the unique characteristics of BRCA1/2 mutation status, genotype-phenotype correlations, and prognosis, in moderate- and low-risk individuals of Greek ancestry. Breast cancer due to mutations in BRCA1 and BRCA2 genes appears to be a heterogeneous syndrome in the Greek population.

Leucovorin and fluorouracil vs levamisole and fluorouracil as adjuvant chemotherapy in rectal cancer.

The aim of this study was to evaluate the effectiveness of 6-month therapy with leucovorin (LV) + 5-fluorouracil (5-FU) vs 12 months of therapy with levamisole (LVZ) + 5-FU, as adjuvant chemotherapy in patients with completely resected Dukes' stage B2 or C rectal cancer. One hundred and fifty patients with surgically resected rectal carcinoma, were enrolled in the present study; Dukes' stage B2 (n=70) or C (n=80), were randomly assigned to chemotherapy with 5-FU + LV x 6 months or 5-FU + LVZ x 12 months. Patient characteristics were equally balanced between the examined groups. Adjuvant CT consisted of LV 20 mg/m(2) intravenously (i.v.) plus 5-FU 450 mg/m(2) i.v., on days 1-5 every 4 weeks for 6 cycles or 5-FU 450 mg/m(2) i.v. every week plus LVZ 50 mg t.i.d x 3 days for 1 year. All patients received radiotherapy with a three-field technique to a total dose of 45 Gy, over 5 weeks. After a median follow-up of 7.4 years there were no significant differences between the two treatment groups with respect to the recurrence rates (P=0.821). Moreover, there was no difference in disease-free survival for patients stage Dukes' B2 (log-rank p=0.73); median for LV group 90 (8-131) months, and for LVZ group 86.5 (3-129) months. No difference was noted in disease-free survival for patients stage Dukes' C (log-rank p=0.73); median for LV group 60 (17-128) months, and for LVZ group 64 (2-123) months. There was no difference in overall survival for patients stage Dukes' B2 (log-rank p=0.75); median for LV group 90 (22-131) months, and for LVZ group 86 (10-129) months. For stage Dukes' C (log-rank p=0.73); median for LV group 67 (17-128) months, and for LVZ group 64 (5-123) months. Toxicities were as follows in the 5-FU + LVZ vs 5-FU + LV group; myelosuppression (leucopenia grade 3, 12% vs 4%, p<0.04), diarrhea (grade 0, 60% vs 76%, p<0.02), and liver toxicity (increase of transaminases >3-fold, 12 patients vs 2, p<0.03), were more frequent in LVZ group. None of the patients stopped chemotherapy because of the toxicity, and there were no toxicity-related deaths. In conclusion, adjuvant chemotherapy in RC with LV + 5-FU for 6 months is equally effective and less toxic than LVZ + 5-FU for 12 months.

Surgical adjuvant therapy of rectal carcinoma: a controlled evaluation of leucovorin, 5-fluorouracil and radiation therapy with or without interferon-alpha2b.

BACKGROUND: The aim of this study was to determine whether the efficacy of the combination of 5-fluorouracil (5-FU), leucovorin (LV) and radiation therapy (RT) could be improved by the addition of interferon-alpha2b (IFN-alpha) in patients who have had a 'curative' resection, for rectal adenocarcinoma (Dukes' B2/C; T3 N0, T4 N0, N1-3). PATIENTS AND METHODS: A total of 207 eligible patients with a performance status of 0 or 1 were randomized postoperatively between days 21 and 70 to one of the two treatment groups: group A, LV 20 mg/m2 i.v. bolus and 5-FU 425 mg/m2 i.v. days 1-5 and 29-33, LV 20 mg/m2 and 5-FU 400 mg/m2 days 57-60 and 85-88, LV 20 mg/m2 and 5-FU 380 mg/m2 days 1-5 and 29-33 with the second day 1 occurring 28 days after the completion of RT (45 Gy); group B, LV, 5-FU and RT as in group A, and IFN-alpha 5 x 10(6) IU s.c. three times during each week chemotherapy is given. RESULTS: 104 patients were randomized into group A and 103 into group B. There was no statistically significant difference in either disease-free survival or overall survival between the two groups. Toxicity was also the same, except for the flu-like syndrome associated with the IFN-alpha administration. CONCLUSIONS: There was no difference in efficacy between the two combinations. Toxicity was greater with the LV + 5-FU + IFN-alpha regimen because of the flu-like syndrome.

Unusual spread of liposarcoma.

BACKGROUND: The aim of our study was to investigate the unusual pattern of metastatic spread of liposarcoma. MATERIALS AND METHODS: We retrospectively studied thirty-two patients with liposarcoma (in seventeen located in the retroperitoneum and in fifteen in the extremities) who were managed at our institution over a ten-year period. Six patients with extremity liposarcoma developed local recurrence and or distal metastases and in three the site of the distal spread was other than the lungs. In only one patient with retroperitoneal liposarcoma we observed extra-pulmonary spread. The histologic type of all four cases with unusual spread was myxoid, with small areas of round cell differentiation in two cases. RESULTS: The survival of the patients after the initial recurrence was relatively prolonged (6, 1.5, 8 and 3 years with the last patient alive and well). CONCLUSION: Physicians should maintain a low threshold for initiating evaluation of relatively minor symptoms in patients with liposarcoma, as these symptoms may reflect the first manifestation of an extrapulmonary metastasis. Such an approach should result in earlier detection and hopefully improve survival for patients with liposarcoma.

Splenic abscess. An old disease with new interest.

BACKGROUND/AIMS: To study the demographics, signs and symptoms, causes, risk factors, imaging findings, bacteriologic profile, treatment and outcome of patients with splenic abscess. METHOD: The medical records of 17 patients with splenic abscess at two tertiary-care hospitals between 1989 and 1997 were retrospectively reviewed. The demographic data, physical and radiological findings, treatment, bacteriology reports and outcome of treatment were reviewed. RESULTS: The mean age of patients was 43 years (range 7-79 years). Fever and abdominal pain were the most prominent signs. Seven patients were immunocompromised, three had abscessed hydatic cysts, two were drug users and three suffered from splenic trauma, infarction, and endocarditis, respectively. No predisposing factor was identified in 2 patients. In all cases, CT demonstrated the splenic lesion(s). Staphylococcus species and Bacteriodes were the most common microbes, identified in the blood and abscess cultures. Thirteen patients underwent splenectomy, two medical therapy and two no therapy with respective survival rates of 92, 100 and 0%. CONCLUSION: Splenic abscess is a rare surgical entity encountered mostly in immunocompromised patients. CT scan is the gold standard for the definite diagnosis. Splenectomy is the treatment of choice, while medical therapy should be reserved for unusual pathogens provided that an effective antimicrobial agent is available.

Imbalance of demand and supply for regionalized injury services: a case study in Greece.

OBJECTIVE(S): To study regionalized acute injury services on an island with high seasonal fluctuation of the population at risk. STUDY PARTICIPANTS: Of 9432 individuals with traumatic injuries who contacted the hospital during 1996, 1204 were hospitalized. Information was recorded on several injury-related clinical and sociodemographic variables. Possible residual disabilities, 6 months after the injury, were also assessed. MAIN OUTCOME MEASURES: Injury Severity Score (ISS), clinical outcome and duration of hospitalization, odds of transfer to other institutions. RESULTS: Non-residents, whether Greek or foreign nationals are hospitalized for shorter periods. Motor vehicle accident victims are hospitalized on average for 15% longer. Injury victims admitted on a Friday are hospitalized for a longer period. Finally, ISS is a powerful positive predictor of duration of hospitalization. Male injury victims, those injured during late night or early morning and patients injured in July are more likely to be transferred to another institution. Age of the patient and ISS are powerful and independent predictors of an unfavourable outcome. CONCLUSION: The extra demand created by injured tourists is reflected in the seasonality of admissions for injuries. The district hospital of Kerkyra cannot be considered as deficient in comparison to other district hospitals. Nevertheless, the suboptimal function of the hospital, with respect to injuries, is reflected in the high proportion of injured patients transferred when the injury occurs outside the full working schedule of the hospital. Patients with burns, bone fractures or dislocations and head injuries or concussion are transferred with an overall frequency of about 15%--too high to be compatible with a well functioning secondary care institution.

Fatal wounding of the Byzantine Emperor Julian the Apostate (361-363 A.D.): approach to the contribution of ancient surgery.

Byzantine surgery flourished from the early stages of the Byzantine empire (324-1453 A.D.). The first great Byzantine physicians, among the most eminent being Oribasius from Pergamun (fourth century), not only compiled anthologies of the works of ancient Greek, Alexandrian, and Roman physicians but added their own personal practical experience and observations. The circumstances surrounding, and the treatment of, the fatal abdominal wounding of one of the most renowned emperors of Byzantium, Julian the Apostate (361-363 A.D.), is examined based on historical accounts unknown in the broader medical bibliography, namely, the histories and chronicles of Byzantine writers. From this analysis it can be concluded that the emperor's physician, Oribasius, in all probability, applied knowledge of ancient Greek and Roman surgery to save his famous patient. The techniques of "gastrorrhaphy" and pharmaceutical confrontation of the wound in that era, adopted later by the surgeons of the Medieval West, seem to constitute noteworthy roots of modern surgery.

Duodenal gangliocytic paraganglioma as a radiological moving defect.

BACKGROUND: Gangliocytic paraganglioma of the duodenum is a peculiar neuroendocrine tumor, often revealed as an incidental radiographic finding or as a result of gastrointestinal hemorrhage, due to frequent ulceration of the overlying mucosa. Most lesions are pedunculated and submucosal with distinctive histology, consisting of endocrine, ganglion and spindle-shaped Schwann cells. METHODS: A case of a duodenal gangliocytic paraganglioma is reported in a 50-year-old woman presenting with episodes of melena. Enteroclysis revealed a pedunculated tumor observed either in the second or third portion of the duodenum. Upper gastrointestinal endoscopy revealed a pedunculated ulcerated tumor which was subsequently confirmed by computed tomography. The tumor was simply excised through a duodenotomy. RESULTS: The size of the tumor was 4.5 x 3 x 1.5 cm. Immunohistochemical and ultrastructural features of the tumor classified it as a typical duodenal gangliocytic paraganglioma with positive cellular reaction for neuron-specific enolase, chromogranin, somatostatin, gastrin and S100. CONCLUSION: Forty-six months after surgery, the patient is well with no evidence of recurrence. The majority of the reported duodenal gangliocytic paragangliomas were of benign nature. However, the fact that in 4 isolated cases there was lymph node involvement indicates that thorough investigation for lymph node metastases is needed, as well as thorough postoperative follow-up.