Ventricular efficiency in pregnant women with congenital heart disease.

BACKGROUNDS: Pregnant women with congenital heart disease (CHD) are at risk of cardiovascular events during pregnancy as well as postpartum. The aim of our study is to address the feasibility of echocardiography-derived ventricular-arterial coupling during pregnancy and postpartum among women with CHD. METHODS: In 31 pregnant women with CHD, we performed serial echocardiography at the first and third trimesters, early and late postpartum. The indices of contractility (single-beat determined end-systolic elastance, Eesab) and afterload (effective arterial elastance, Ea) were approximated on the basis of the systemic blood pressure and systemic ventricular volume. The ratio of stroke work and pressure-volume area (SW/PVA) representing ventricular efficiency was also calculated. RESULTS: Age at the delivery was 28 (24-31) years. ZAHARA score was 0.75 (0.75-1.50). Gestational age and birth weight of newborns were 38 (37-39) weeks and 2.73 (2.42-2.92) kg, respectively. Heart rate, systemic ventricular end-diastolic volume and stroke volume significantly increased from the first trimester to the third trimester and reversed postpartum to the values of the first trimester. Eesab and Ea significantly decreased from the first trimester to the third trimester (Eesab; 4.90 [2.86-7.14] vs 3.41 [2.53-4.61] mm Hg/ml, p = 0.0001, Ea; 2.83 [1.74-3.30] vs 2.18 [1.67-2.68] mm Hg/ml, p = 0.0012), and reversed early postpartum parallelly. Ejection fraction and SW/PVA remained unchanged throughout pregnancy and postpartum. CONCLUSIONS: Echocardiography-derived ventricular-arterial coupling is feasible to understand ventricular function in pregnant women with CHD.

A Possible Case of Hepatitis due to Hypereosinophilic Syndrome.

A 63-year-old Japanese man whose white blood cell count and total-bilirubin and aminotransferase levels were elevated was referred to our hospital. Computed tomography did not reveal any abnormalities, and there was no evidence of gastritis or colitis on esophagogastroduodenoscopy. Although the patient had no history of drug use or allergies, a high concentration of eosinophils (80%) was noted. A liver biopsy revealed hepatitis with eosinophilic infiltration. The patient's alanine aminotransferase and eosinophil levels improved with the administration of steroids. A second biopsy, performed 6 months later, showed the improvement of the eosinophilic infiltration. The patient was diagnosed with eosinophilic hepatitis due to the presence of hypereosinophilic syndrome without the dysfunction of other organs.

Ultrasonography screening for hepatocellular carcinoma in Japanese patients with diabetes mellitus.

BACKGROUND: Effective surveillance for hepatocellular carcinoma (HCC) in diabetes mellitus (DM) has not been established. We elucidated the risk factors for HCC in DM patients. METHODS: From 2000 to 2014, 80 patients diagnosed with HCC for the first time who had concomittant DM but no other etiology of liver disease were enrolled as the DM-HCC group. From 2005 October to 2014, after introduction of the abdominal ultrasonography (US) report database, 2083 DM patients with no viral hepatitis, no known autoimmune hepatic diseases, and/or no evidence of alcohol abuse (>60 g/day) were enrolled as the DM-US group. Findings from the first US screening were evaluated. "Elderly" patients were defined as those aged >65 years. Clinical features of DM-HCC patients were evaluated and compared with those of DM-US patients. RESULTS: In the DM-HCC group (54 men, 26 women), the mean (± SD age was 74.1 ± 8.5 years, and mean HbA1c and fibrosis-4 (FIB-4) index were 7.3 ± 1.3% and 4.50 ± 3.42, respectively. Mean tumor diameter was 5.7 ± 3.5 cm, there were 63/13/2/2 patients classified as Child-Pugh A/B/C/unknown, and 56/24 were single/multiple lesions. In the DM-US group, HCC was detected in three patients (0.14%; 0.3% of those classified as elderly). The mean age and FIB-4 index of these three patients (one man, two women) were 75.6 years (range 67-92 years) and 4.84 (range 2.87-6.98), respectively. Mean tumor diamter was 7.6 cm and there were one and two single and multiple lesions, respectively. In elderly DM-US patients with a high FIB-4 index (≥4), the rate of HCC detection was 5.0%. CONCLUSIONS: Being elderly and having a high FIB-4 index are characteristic features of DM-HCC. Similar characteristics were noted for patients with HCC in the DM-US group. HCC surveillance with US is recommended for DM patients, especially those who are elderly (≥65 years) and have a high FIB-4 index.

Safety and efficacy of thromboprophylaxis using enoxaparin sodium after cesarean section: A multi-center study in Japan.

OBJECTIVE: Pulmonary embolism (PE) is the leading cause of maternal death in developed countries, and the prevention of venous thromboembolism (VTE) is a pivotal part of current obstetric care. This study evaluated the safety and efficacy of enoxaparin sodium for thromboprophylaxis after cesarean section (C/S), and analyzed the risk factors associated with VTE. MATERIALS AND METHODS: One hundred and forty-three women deemed to be at high risk of postoperative deep vein thrombosis (DVT) were enrolled between January 2011 and May 2012 in seven institutions in Japan. Subcutaneous administration of enoxaparin 4000 units/d was initiated 24-36 hours after C/S for 5 days. Adverse events, based on the Common Terminology Criteria for Adverse Events, Version 4, were recorded. The diagnoses of PE and DVT were made on clinical signs. Venous ultrasonography in the lower extremities was performed in 102 patients. The association between VTE and various risk factors was evaluated using univariate analysis. RESULTS: There were 10 (7.0%) Grade 1 adverse events: elevated aspartate aminotransferase or alanine aminotransferase levels in eight patients, chest pain in one patient, and subcutaneous hematoma in one patient. No patients showed clinical signs of PE and/or DVT. Among 102 patients who underwent venous ultrasonography, thrombus was detected in unilateral soleus veins in four (3.9%) patients. A body mass index (BMI) ≥ 25 kg/m(2) before pregnancy was associated with asymptomatic DVT. CONCLUSION: The current study demonstrates the safety and efficacy of enoxaparin for thromboprophylaxis after C/S. Further studies are required to determine the best method of preventing asymptomatic DVT.

Tumor Markers AFP, AFP-L3, and DCP in Hepatocellular Carcinoma Refractory to Transcatheter Arterial Chemoembolization.

BACKGROUND/AIM: We examined tumor marker levels to assess in more detail transcatheter arterial chemoembolization (TACE)-refractory hepatocellular carcinoma (HCC). MATERIALS AND METHODS: We enrolled patients treated from 2000 to 2011 for HCC beyond the Milan criteria who had good hepatic reserve function (Child-Pugh A) and no portal vein thrombosis or metastases (n = 154). The modified criteria for being TACE-refractory according to the Liver Cancer Study Group of Japan (m-LCSGJ), from which the tumor marker item was excluded, and the Assessment for Retreatment with TACE (ART) score were used for determining whether the HCC was TACE refractory. α-Fetoprotein ≥ 100 ng/ml, fucosylated α-fetoprotein ≥ 10%, and des-gamma-carboxy prothrombin ≥ 100 mAU/ml were used to define whether tumor markers were positive. We added up the number of positive tumor markers as a prognostic score to assess in more detail the evaluation of TACE-refractory HCC. RESULTS: In order to divide the patients into a refractory and nonrefractory group, the m-LCSGJ criteria [mean survival time (MST) 27.1 vs. 49.9 months; p < 0.001] were superior to the ART score (MST 22.0 vs. 35.1 months; p = 0.051). In the refractory group according to the m-LCSGJ criteria, the patients with a low score of positive tumor markers (<2) after 2 sessions of TACE (n = 36) showed a better prognosis than the others (n = 72) (MST 37.7 vs. 23.2 months; p = 0.014). CONCLUSION: Patients being nonrefractory according to the m-LCSGJ criteria had a better response, and using the number of tumor markers (≥ 2) is an easy method for predicting the response to TACE and for a more detailed evaluation of TACE-refractory HCC.

Muscle atrophy as pre-sarcopenia in Japanese patients with chronic liver disease: computed tomography is useful for evaluation.

BACKGROUND/AIM: The definition of muscle atrophy (pre-sarcopenia) and its diagnostic criteria have not been well reported. To elucidate the frequency of pre-sarcopenia in chronic liver disease (CLD), we examined clinical features of Japanese CLD patients using abdominal computed tomography (CT) findings. METHODS: We enrolled 988 CLD (736 with naïve hepatocellular carcinoma) and 372 normal control subjects (NCs). The psoas muscle area index [PI, psoas muscle area at the mid-L3 level in CT (cm(2))/height (m)(2)] was calculated using personal computer software. The cut-off level for pre-sarcopenia was defined as less than two standard deviations (SDs) below the mean PI value in the NCs under 55 years old [males, 45.6 ± 5.7 years (n = 61), 4.24 cm(2)/m(2); females, 47.0 ± 6.1 years (n = 49), 2.50 cm(2)/m(2)]. Elderly was defined as 65 years or older. Clinical features were retrospectively evaluated. RESULTS: In the CLD group (HCV:HBV:HBV and HCV:alcohol:non-HBV and HCV = 652:88:7:82:159), pre-sarcopenia was observed in 15.3% of patients with chronic hepatitis (CH), 24.4% of those with liver cirrhosis (LC) Child-Pugh A, 37.7% of those with LC Child-Pugh B, and 37.1% of those with LC Child-Pugh C. A comparison between NC and CH by age (<55, 55-64, 65-74, ≥75 years) showed that the frequency of pre-sarcopenia was higher in CH regardless of age (1.8 vs. 3.6%, 3.2 vs. 15.9%, 4.9 vs. 13.4%, 14.3 vs. 20.2%, respectively). PI values showed correlations with BMI (r = 0.361), age (r = -0.167), albumin (r = 0.115), and branched-chain amino acids (r = 0.199) (P < 0.01). CONCLUSION: Retrospective evaluate for pre-sarcopenia was easy to perform with CT findings. Nutrition and exercise instruction should be considered for early stage and even non-elderly CLD as well as LC.

Prognosis and therapy for ruptured hepatocellular carcinoma: problems with staging and treatment strategy.

BACKGROUND: There are no clear criteria established for treating a ruptured hepatocellular carcinoma (HCC). To elucidate the clinical features of affected patients, we examined prognosis and therapy choices. MATERIALS/METHODS: We enrolled 67 patients treated for a ruptured HCC (HCV 44, HBV 5, HBV+HCV 1, alcohol 2, others 15; naïve HCC 34, recurrent 33) from 2000 to 2013, and investigated their clinical background and prognosis. RESULTS: Median survival time (MST) for all cases was 4 months. For patients who survived for more than 1 year after rupture, the percentages of Child-Pugh C and positive for portal vein tumor thrombosis (PVTT)/extrahepatic metastasis were less than for those who died within 1 year. Child-Pugh classification (A:B:C=14:15:5 vs. 4:9:20, P<0.001) was better, while the percentage of patients with multiple tumors was lower [19/34 (55.9%) vs. 29/33 (87.9%), respectively; P<0.001] in the naïve group. The 1- and 3-year survival rates were better in the naïve as compared to the recurrent group (60.6% and 33.3% vs. 12.6% and 0%, respectively; P<0.01). MST according to modified TNM stage (UICC 7th) calculated after exclusion of T4 factor of rupture, stage I was better than others (22.7 vs. (II) 2.2, (III) 1.2, and (IV) 0.7 months) (P=0.010). CONCLUSION: In patients with a ruptured HCC, especially those with a single tumor, and without decompensated liver cirrhosis and PVTT/extrahepatic metastasis, better prognosis can be expected with curative treatment. The present naïve group included more of such cases than the recurrent group, indicating the effectiveness of curative therapy.

Perinatal management of preterm premature ruptured membranes affects neonatal prognosis.

AIM: To determine the factors affecting neonatal prognosis in preterm premature rupture of membranes (PPROM). METHOD: We conducted a case-control study involving 92 women between the years 2000 and 2010 diagnosed with PPROM between 25 and 31 weeks' gestation, who received antenatal steroids, and delivered between 26 and 31 weeks' gestation; a retrospective cohort study was conducted based on the results. We used data from four tertiary centers and compared the frequencies of neonatal neurologic deficits and neonatal deaths. RESULTS: There was a difference between the two groups; specifically, the ND group (n=18) consisted of patients whose infants had neurologic deficits and/or neonatal deaths and the neurologically normal (NN) group (n=74) included NN neonates amongst the patients who had expectant management (94% vs. 73%, respectively). Multivariable analysis revealed that expectant management was independently associated with an increased risk for neonatal neurologic deficits and neonatal deaths (odds ratio, 16.14). All neonates with poor prognosis in the expectant-management group delivered within 14 days after PPROM. CONCLUSIONS: Expectant management within 14 days after PPROM is associated with poor neonatal outcomes. Decisions regarding an expectant strategy should be made carefully. An immediate, planned delivery after steroid administration should be considered to improve neonatal prognosis in patients who have PPROM after 26 weeks' gestation.

Real-time tissue elastography: non-invasive evaluation of liver fibrosis in chronic liver disease due to HCV.

BACKGROUND/AIMS: We evaluated the diagnostic efficacy for advanced fibrosis of real-time tissue elastography (RTE), is a newly introduced non-invasive method, in hepatitis C virus (HCV) patients. METHODOLOGY: Sixty-six chronic liver diseases with HCV (CLD-HCV) were evaluated by RTE, FIB-4, and APRI, as well as biopsy or hepatectomy findings. Seventeen healthy volunteers, and 1 with elevation of transaminase and 5 with liver tumors within their normal liver were enrolled for normal controls. Severe fibrosis was defined as METAVIR score 3 or 4. The tissue elasticity value was expressed by LF-index created by the program incorporated in the ultrasound device. The percentage of fibrosis area in each specimen (%FA) was determined with a personal computer. A receiver operating characteristic curve (ROC) was calculated for each non-invasive method of estimation of fibrosis. RESULTS: The values for the area under the ROC for LF-index, APRI, and FIB-4 were 0.88, 0.81, and 0.84, respectively. Accuracy, sensitivity, and specificity for prediction of LF-index (>2.8) for severe fibrosis were 86.5%, 78.6%, and 93.6%, respectively, which were better than those of FIB-4 and APRI. LF-index showed a good relationship to %FA (r=0.276, P=0.020). CONCLUSION: RTE is an effective method for predicting severe fibrosis in CLD-HCV.

Hepatitis B virus reactivation induced by infliximab administration in a patient with Crohn's disease.

A 47-year-old man diagnosed with Crohn's disease was treated with infliximab. He tested negative for hepatitis B surface antigen (HBsAg) and hepatitis B surface antibody (anti-HBs) but positive for anti-HB core antibody (anti-HBc). He tested positive for hepatitis B virus (HBV-) DNA 3 months after treatment and was administered entecavir. HBV-DNA test showed negative results 1 month later. ALT was persistently within the normal range, and HBV-DNA was persistently negative thereafter despite the continuation of infliximab every 8 weeks. In our hospital, 14 patients with inflammatory bowel disease, who tested negative for HBsAg, were treated with infliximab; 2 of them tested positive for anti-HBs and/or anti-HBc, and HBV reactivation was observed in 1 patient (the present patient). The present case and these findings highlight that careful follow-up is needed in patients with inflammatory bowel disease treated with infliximab who test positive for anti-HBc and/or anti-HBs.

Huge pancreatic acinar cell carcinoma with high levels of AFP and fucosylated AFP (AFP-L3).

A 25-year-old Japanese woman with a large abdominal tumor (10 cm in diameter) and high level of serum alpha-fetoprotein (AFP, 1,222.4 ng/mL) was introduced and admitted to our hospital in January 2011. Pre-operative imaging modalities could not identify the tumor origin. There was no chronic hepatic viral disease, however, the elevated level of fucosylated AFP (87.8%) and connection with the left hepatic lobe led us to diagnose the tumor as a hepatocellular carcinoma. Surgical resection was performed in February. Histological findings revealed a pancreatic acinar cell carcinoma (ACC). Herein, we provide details of this difficulty to diagnose a case of ACC.

Hepatocellular carcinoma in a case of Wilson's disease treated with radiofrequency ablation therapy.

A 37-year-old Japanese man was diagnosed with liver cirrhosis due to Wilson's disease in 2001 and treated with D-penicillamine. Thereafter, he was admitted to our hospital for further examination of a space occupying lesion in the liver. The patient was diagnosed with hepatocellular carcinoma (HCC) (7th segment, 2.5 cm in diameter) in May 2010 and treated with radiofrequency ablation therapy. Biopsy findings from a non-cancerous area revealed a fatty liver, though cirrhotic nodules were not found. Long-term treatment for Wilson's disease may improve hepatic fibrosis, and careful screening for HCC by abdominal imaging is needed in such cases.

Severe hypoglycemia associated with insulin-like growth factor II-producing liver metastasis from gastric carcinoma treated with overnight total parenteral nutrition via a central vein catheter reserve port.

Hypoglycemia caused by insulin-like growth factor II is difficult to control. A 77-year-old woman was diagnosed with gastric cancer and multiple liver metastases in September 2006 and underwent chemotherapy; however, at that time there were no symptoms of hypoglycemia. From January 2007 onwards, hypoglycemic comas and symptoms of hypoglycemia began to appear frequently. Her serum level of insulin was normal; thus, we suspected the presence of big insulin-like growth factor II was causing the hypoglycemia. This was proven by Western immunoblotting and we diagnosed non-islet cell tumor hypoglycemia associated with gastric cancer. Overnight nutrition provided via a central venous catheter port to prevent hypoglycemia allowed the patient to become ambulant and to remain free of hypoglycemic coma at follow-up until her death 7 months later.

Malignant pheochromocytoma with liver metastasis treated by transcatheter arterial chemo-embolization (TACE).

Multiple liver metastatic lesions were shown by computed tomography and scintigraphy with 123I- metaiodobenzylguanidine (MIBG) in a 76-year-old woman 2 years after resection of a pheochromocytoma of the right adrenal gland. Transcatheter arterial chemo-embolization (TACE) was performed for the liver metastasis, with blood pressure strictly controlled by administration of doxazosin and phentolamine for the prevention of hypertensive crisis after TACE. There were no severe adverse events associated with the treatment, and the number and size of the lesions were decreased. TACE with strict blood pressure control was an effective and safe therapy for multiple liver metastatic lesions related to malignant pheochromocytoma.

Amino acid imbalance in patients with chronic liver diseases.

AIM: The aim of this study is to clarify the amino acid imbalance in patients with chronic hepatitis (CH) as well as those with liver cirrhosis (LC). METHODS: We assayed total branched-chain amino acids (BCAA), tyrosine (Tyr) levels and their ratio (BTR) in sera of 101 patients with CH (37 in fibrosis stage F1, 23 in F2, 21 in F3) and 20 with LC (F4) who were diagnosed by liver biopsy. Their levels in relation to the staging of liver fibrosis were analyzed. RESULTS: The percentage of patients whose BTR was less than the normal range was 32.1% in CH and 75.0% in LC. The levels of BTR and BCAA were significantly lower (P < 0.001, P < 0.05, respectively) and that of Tyr was significantly higher (P < 0.001) in patients with LC than those in CH. The levels of BTR decreased according to the staging. The levels of Tyr increased according the staging, whereas the levels of BCAA deceased prominently in F4 (487 +/- 103 in F1, 483 +/- 122 in F2, 487 +/- 111 in F3 and 423 +/- 94 in F4). CONCLUSION: A considerable number of patients not only with LC but also with CH showed lower levels of BTR. It has been clarified that amino acid imbalance of Tyr was found in the early stage of liver disease, whereas decrease of BCAA was found mainly in F4 stage.

Clinical translation in the treatment of hepatocellular carcinoma following the introduction of contrast-enhanced ultrasonography with Sonazoid.

Some hepatocellular carcinoma (HCC) nodules are detectable with dynamic computed tomography, but not by conventional B-mode ultrasonography (US). Contrast-enhanced US (CEUS) with Sonazoid, a new injectable contrast agent, has been used in Japan since January 2007. The primary advantage of this agent is the ability to maintain observations continuously in the Kupffer phase. We assessed the clinical role of CEUS with Sonazoid for radiofrequency ablation (RFA). From January 2005 to December 2008, 1142 patients were treated with surgical resection, RFA, percutaneous ethanol injection or transcatheter arterial chemoembolization, following the exclusion of those patients treated with chemotherapy or supportive care. The patients included in the study were divided into the pre-CEUS (n=451, 2005 and 2006) and post-CEUS (n=691, 2007 and 2008) groups. Clinical background (e.g., etiology, Child-Pugh classification, tumor node metastasis stage, percentage of patients matched with Milan criteria and selected therapies) was compared between the two groups. In addition, naïve cases were compared between the groups. There were 130 naïve HCC cases in the pre-CEUS group and 171 in the post-CEUS group. Although there were no significant differences for clinical background, the percentage of RFA cases increased from 21 (n=95) to 32% (n=219) and from 32 (n=41) to 52% (n=89) for total and naïve subjects, respectively, after CEUS was introduced (P<0.01). In naïve cases treated with RFA, tumor numbers in the post-CEUS group were larger than those of the pre-CEUS group (1.15±0.48 vs. 1.40±0.67; P<0.01). CEUS with Sonazoid, therefore, makes it possible to perform RFA in a considerable number of HCC cases that would otherwise be invisible by conventional B-mode US.

Evaluation of contrast-enhanced ultrasonography using perfluorobutane (Sonazoid(®)) in patients with small hepatocellular carcinoma: comparison with dynamic computed tomography.

This study aimed to elucidate the efficacy of contrast-enhanced ultrasonography (CEUS) with perfluorobutane (Sonazoid(®)) in the diagnosis of hepatocellular carcinomas (HCCs), particularly small HCCs, by comparing the results with dynamic computed tomography (Dy-CT). Seventy-nine nodules in 69 patients with chronic liver disease, suspected as HCCs were studied. The nodules were selected based on the results of B-mode ultrasonography and/or Dy-CT conducted between January and August 2007. The nodules were divided into two groups: the S-group with tumors ≤2 cm (49 nodules), and the L-group with tumors >2 cm (30 nodules). Typical HCCs were defined, and the nodules were enhanced and shown as defects in the arterial and late phase of Dy-CT, respectively. Target lesions were scanned using CEUS, and the results were compared with those of Dy-CT. The L-group nodules diagnosed as HCCs using Dy-CT were also diagnosed as HCCs using CEUS. In the S-group, the diagnostic sensitivity of CEUS was 94.7% and the specificity was 81.8%. We diagnosed two liver tumors that were detected by CEUS but not by Dy-CT; biopsies revealed one tumor to be a well-differentiated HCC and the other to be an atypical adenomatous hyperplasia. The sensitivity and specificity of CEUS against HCC were high even in the small-size HCCs. Thus, Sonazoid is useful in the screening for small HCCs.

Diagnostic value of sonazoid for hepatic metastasis: comparison with FDG PET/CT.

BACKGROUND/AIMS: This study was conducted to evaluate the diagnostic efficacy of contrast enhanced ultrasonography (CEUS) with perflubutane (Sonazoid) for hepatic metastasis and compared it with that of 18-fluoro-2-deoxyglucose (FDG) positron emission tomography (PET) computed tomography (CT). METHODOLOGY: From January 2007 to July 2009, 109 Japanese patients with gastrointestinal tract cancer were enrolled, of whom 4 had esophageal cancer, 28 gastric cancer, 1 duodenal cancer, and 76 colorectal cancer. After a bolus injection with Sonazoid (0.5 ml/body), the liver was scanned in both arterial and Kupffer phases. The results of PET/CT and other abdominal imaging examinations were not shown to the CEUS operators. We compared diagnostic efficacy between CEUS and PET/CT. RESULTS: Average values for age, body mass index, and maximum diameter of the hepatic metastasis were 68.7 +/- 11.0 years, 21.2 +/- 4.2, and 29.2 +/- 20.5mm, respectively. Hepatic metastasis were suspected in 31 patients based on PET/CT findings and 32 by CEUS. Finally, hepatic metastasis was diagnosed in 30 patients. The sensitivity, specificity, and accuracy rates for CEUS and PET/CT were similar (100% vs. 100%, 97.5% vs. 98.7%, and 95.5% vs. 93.6%, respectively). CONCLUSION: CEUS had a diagnostic value similar to that of PET/CT for hepatic metastasis.

Primitive neuroectodermal tumor of the pancreas.

The primitive neuroectodermal tumor (PNET) of the pancreas, a member of Ewing's sarcoma family of tumors, is extremely rare. We treated a 37-year-old Japanese man who had a solitary pancreatic tumor 40 mm in diameter and multiple hepatic tumors with surgical resection. The PNET was positive for CD99 on immunohistochemical staining. Fluorescence in situ hybridization (FISH) was also performed, which revealed a Ewing sarcoma breakpoint region 1 (EWSR1) 22q12 rearrangement. According to the Japan-Ewing protocol, chemotherapy with Ifomide (ifosfamide), etoposide, vincristine, and cyclophosphamide was given after surgery. To the best of our knowledge, to date 13 PNET cases have been reported with a mean age for all patients of 19.3 years old. Surgical resection was performed in most cases and some patients received postoperative chemotherapy. The clinicopathologic characteristics and management of this extremely rare disease are also discussed.