Correlation of World Health Organization 2010 Classification for Gastroenteropancreatic Neuroendocrine Neoplasms with the Prognosis of Ovarian Neuroendocrine Neoplasms: Kansai Clinical Oncology Group-Protocol Review Committee/Intergroup Study.

BACKGROUND: In 2014, the World Health Organization (WHO) released a classification system introducing neuroendocrine neoplasms (NENs) of the female reproductive tract, excluding the ovaries. This study aimed to evaluate whether retrospective adaption of the gastroenteropancreatic (GEP)-NEN classification is feasible for ovarian NENs (O-NENs) and correlates with prognosis. METHODS: Sixty-eight patients diagnosed with carcinoid, small cell carcinoma (pulmonary type), paraganglioma, non-small/large cell neuroendocrine carcinoma (NEC), mixed NEC, or undifferentiated carcinomas at 20 institutions in Japan were included in this retrospective cross-sectional study. We identified O-NENs through central pathological review using a common slide set, followed by reclassification according to WHO 2010 guidelines for GEP-NENs. A proportional hazards model was used to assess the association of prognostic factors (age, stage, performance status, histology, and residual disease) with overall survival (OS) and progression-free survival (PFS). RESULTS: Of the 68 enrolled patients, 48 were eligible for analysis. All carcinoids (n = 32) were reclassified as NET G1/G2, whereas 14 of 16 carcinomas were reclassified as NEC/mixed adeno-NEC (MANEC) (Fisher's exact test; p < 0.01). The OS/PFS was 49.0/42.5 months and 6.5/3.9 months for NET G1/G2 and NEC/MANEC, respectively. Histology revealed that NEC/MANEC was associated with increased risk of death (HR = 48.0; 95% CI, 3.93-586; p < 0.01) and disease progression (HR = 51.6; 95% CI, 5.54-480; p < 0.01). CONCLUSION: Retrospective adaption of GEP-NEN classification to O-NENs is feasible and correlates well with the prognosis of O-NENs. This classification could be introduced for ovarian tumors.

Pathological processes and pretreatment cytologic diagnosis of neuroendocrine carcinoma of the endometrium: addendum report of the Kansai Clinical Oncology Group/Intergroup study in Japan.

A previous retrospective study of a neuroendocrine carcinoma of the endometrium including 42 cases employed a central pathologic review to ensure the reliability of the findings. However, the pathological processes were not described in detail. In this study, we further analyzed these processes and the results of pretreatment endometrial cytology of neuroendocrine carcinoma. Of the 65 patients from 18 institutions registered in the study, 42 (64.6%) were diagnosed with neuroendocrine carcinoma of the endometrium based on the central pathological review. Thirteen of the 23 excluded cases conflicted from their original diagnoses: 5 (38.5%) were diagnosed with endometrioid adenocarcinoma, 5 (38.5%) with undifferentiated carcinoma, and 3 (23.1%) with carcinosarcoma. Immunohistochemical staining led to a change in diagnosis for 8 (61.5%) of the 13 cases. Pretreatment endometrial cytology was examined in 38 (90.5%) cases; 34 (89.5%) of these 38 cases were found, or suspected, to be positive. To ensure the selection of appropriate therapy and keeping patients correctly informed, it is important to distinguish neuroendocrine carcinoma from other similar histologic types. Endometrial cytology may help in the early detection of this disease.

Etiology and impact on outcomes of polycystic kidney disease in abdominal aortic aneurysm.

PURPOSE: We investigated the etiology and impact on outcomes of polycystic kidney disease in patients with abdominal aortic aneurysm. METHODS: Eight-hundred patients who underwent open (n = 603) or endovascular aortic repair (n = 197) were divided into three groups: no cyst (n = 204), non-polycystic kidney (n = 503), and polycystic kidney (≥ 5 cysts in the bilateral kidneys, n = 93). The characteristics and outcomes were compared among the groups. RESULTS: In the polycystic kidney group, the age was increased and the proportions of patients with male sex, hypertension, and estimated glomerular filtration rate < 30 mL/min/1.73 m2 were greater. The overall hospital mortality rates were similar. The incidence of acute kidney injury after elective open aortic repair was increased in the polycystic kidney group (12%, 17%, and 29%, P = 0.020). In the polycystic kidney group, 80 patients did not have renal enlargement or a family history of renal disease, while 13 (corresponding to 1.6% [13/800] of the overall patients), had renal enlargement, suggesting the possibility of hereditary polycystic kidney disease. CONCLUSIONS: In our cohort, 1.6% of the patients with abdominal aortic aneurysm who underwent surgery were at risk of hereditary polycystic kidney disease. Polycystic kidney disease was associated with acute kidney injury after open aortic repair.

Perioperative factors associated with aneurysm sac size changes after endovascular aneurysm repair.

PURPOSE: To identify the perioperative factors associated with aneurysm size changes after endovascular aortic aneurysm repair (EVAR). METHODS: Between August, 2008 and December, 2014, 187 patients underwent EVAR treatment in our institution. The subjects of this study were 135 of these patients without peripheral artery disease, who were followed up with computed tomography (CT) for 3 years. Significant aneurysm size change was defined as sac size change of more than 5 mm from the baseline. RESULTS: Sac enlargement was identified in 25 patients (18.5%) and sac shrinkage was identified in 59 (43.7%) patients. The factors associated with sac enlargement were postoperative pulse wave velocity (OR: odds ratio 3.80, p = 0.047), prevalence of a type 2 endoleak 1 week after surgery (OR 4.26, p = 0.022), inner diameter (OR 1.10, p = 0.005), and distance from the lower renal artery to the terminal aorta (OR 1.05, p = 0.017). The factors associated with sac shrinkage were prevalence of a type 2 endoleak (OR 0.09, p < 0.001) and preoperative pulse wave velocity (OR 0.32, p = 0.022). The factors independently associated with type 2 endoleak were the use of an Excluder device (OR 3.99, p = 0.002) and the length of the aneurysm (OR 1.02, p = 0.027). CONCLUSION: Inner diameter, treatment length, perioperative pulse wave velocity, and type 2 endoleak were associated with sac size changes after EVAR.

Ruptured external iliac artery pseudoaneurysm following surgery for ovarian cancer: A case report and literature review.

Ruptured pseudoaneurysm following pelvic surgery is a rare and fatal complication. Because of its rarity, existing evidence is limited to a small case series. A 60-year-old woman underwent staging laparotomy, including pelvic and para-aortic lymphadenectomy, for ovarian cancer. On the 11th day, the patient developed a sudden lumbar pain and loss of consciousness, which resulted in a state of shock. She was diagnosed as having a pelvic abscess and ruptured external iliac artery pseudoaneurysm. We performed ligation of the external iliac artery to restrain hemorrhage and femoro-femoral artery bypass to prevent infection, which saved the patient's life. Our study had two findings. First, this is the first reported case of ruptured external iliac artery pseudoaneurysm following surgery for ovarian cancer. Second, treatment of ruptured pseudoaneurysm requires rapid hemostasis, prevention of infection, and revascularization.

Combined large-cell neuroendocrine carcinoma and endometrioid adenocarcinoma of the endometrium: A case report and survey of related literature.

Primary large-cell neuroendocrine carcinoma of the endometrium is extremely rare and has a poor prognosis. This report describes a case of combined large-cell neuroendocrine carcinoma and endometrioid adenocarcinoma of the endometrium diagnosed as stage IIIA. The patient underwent surgery and chemotherapy and has been well with no evidence of disease for 20 months. The optimal treatment for this rare tumor has not been established. Considering its rarity and variability, it is difficult to establish an evidence-based therapeutic regimen.

Intravenous leiomyomatosis treated with radical hysterectomy and adjuvant aromatase inhibitor therapy.

Intravenous leiomyomatosis (IVL), a rare disease that is histologically benign but clinically aggressive, is characterized by the intraluminal growth of benign leiomyoma in the intrauterine and systemic veins. Preoperative diagnosis of IVL is difficult, because the symptoms of early stage IVL are similar to those of uterine leiomyoma. The efficacy of adjuvant hormone therapy after surgical resection of IVL remains unclear. Herein is described a case of IVL that was diagnosed preoperatively, in which successful total resection of the tumor was achieved by radical hysterectomy. The patient, a 50-year-old premenopausal Japanese woman, also underwent aromatase inhibitor treatment and was free of disease at 36 months after surgery. Contrast-enhanced computed tomography is suggested as the best assessment for identifying and diagnosing IVL. Radical hysterectomy can be considered a successful therapy for total resection. Aromatase inhibitor treatment may be effective, especially when the patient has not yet entered menopause.

[Total Aortic Arch Replacement by Minimally Invasive Approach in a Patient with Permanent Tracheostomy;Report of a Case].

Standard full median sternotomy for total aortic arch replacement in patients with tracheostomy has higher risks for mediastinitis and graft infection. To avoid surgical site infection, it is necessary to keep a sufficient distance between the tracheostomy and the site of surgical skin incision. We herein report a case of a 74-year-old man with permanent tracheostomy after total laryngectomy, who underwent total aortic arch replacement for an aneurysm. Antero-lateral thoracotomy in the 2nd intercostal space with lower partial sternotomy( ALPS approach) provided an enough distance between the tracheostomy and the surgical field. It also provided a good view for surgical procedure and enabled the standard setup of cardiopulmonary bypass with ascending aortic cannulation, venous drainage from the right atrium and the left ventricular venting through the upper right pulmonary vein. The operation was completed in 345 minutes and the patient was discharged on the 11th postoperative day without any complications.

[Valve replacement after omentum flap for medicastinitis;report of a case].

A 71-year-old woman presented with heart failure due to aortic and mitral valve regurgitation. She had developed midiastinitis and graft infection, 15 months before, following replacement of the ascending aorta for acute aortic dissection. Omentum flap operation had been performed and the infection had been controlled. This time, she underwent re-thoracotomy, and replacement of ascending aorta, aortic valve replacement and mitral valve plasty were performed. The omenal tissue was exfoliated without any damage to the heart or the great vessels by using an ultrasonic scalpel. As the omental tissue was viable, it was placed back in the mediastinal space.

Recurrent immature teratoma of the ovary with long-term disease-free interval.

Grade 3 immature teratoma of the ovary is rare and has a poor prognosis due to early recurrence. This report describes a case of grade 3 immature teratoma of the ovary that recurred 16 years after the last treatment. The patient underwent abdominal simple total hysterectomy, bilateral salpingo-oophorectomy, pelvic-para-aortic lymphadenectomy, and adjuvant chemotherapy under the diagnosis of grade 3 immature teratoma of the ovary when she was 15 years old, and complete remission was achieved. Sixteen years after the initial treatment, the tumor relapsed in her liver and pleura. She was treated by 10 courses of tri-weekly paclitaxel and carboplatin, and was alive with stable disease for 12 months after the disease relapse. After 1 year, the tumor progressed, and she died 16 months after the relapse. This is the first report of grade 3 immature teratoma of the ovary that relapsed more than 5 years after the prior treatment.

Retrospective analysis of outcomes of secondary debulking surgery for recurrent epithelial ovarian cancer with favorable prognostic factors.

AIM: Ovarian cancer is the second most common gynecological malignancy, yet it has the highest case-fatality ratio of all gynecologic malignancies. Surgery followed by combination platinum-taxane chemotherapy is the standard approach to the management of primary epithelial ovarian cancer. However, standard treatment of patients with recurrent ovarian cancer remains poorly defined. Secondary cytoreductive surgery (SDS) at the time of relapse has been proposed as a means of improving the prognosis of recurrent ovarian cancer patients with a treatment-free interval of at least 6 months. METHODS: In the present study, we retrospectively collected 16 patients with recurrent epithelial ovarian cancer who might benefit most from SDS and evaluated the impact of SDS on the outcomes for this highly select patient group. RESULTS: We found that SDS led to excellent outcomes, with a 73.1% 8-year overall survival rate after initial treatment, a 67.9% 5-year overall survival rate after prior SDS, and a 31.3% 5-year progression-free survival rate after prior SDS. Although the findings were not significant, these results suggest that repeated SDS might improve outcomes for this patient group. CONCLUSION: The present study may provide a platform for discussion of the impact of aggressive or repeated SDS on the survival of patients with recurrent epithelial ovarian cancer and favorable prognostic factors. Further multi-institutional studies with larger number of patients are mandatory to confirm the present findings.

Revision Surgery for Venous Graft Stenosis of SMA Bypass.

A 67-year-old male with postprandial abdominal pain for 4 months obtained medical attention for severe pain. He was diagnosed with small intestinal necrosis, secondary to chronic mesenteric ischemia by CT scan. We performed the surgery including a partial resection of the small intestine and left external iliac artery to the superior mesenteric artery bypass using saphenous vein graft. His symptoms improved after surgery. However, 5 months later, abdominal pain appeared after eating. A CT scan identified graft stenosis, leading to a revascularization. A synthetic vessel was used to perform the re-bypass surgery. Postoperatively, the patient's abdominal pain improved.

Extragonadal recurrence of benign mature teratoma in the pouch of Douglas.

Laparoscopic cystectomy for mature teratomas is associated with a high incidence of intraperitoneal spillage and tumor spread; however, extragonadal recurrence of this benign tumor is rare. We hereby present an additional case of extragonadal mature teratoma that recurred in the pouch of Douglas after ovarian cystectomy. A 43-year-old Japanese woman presented with atypical genital bleeding. A 7 cm mature teratoma was detected using transvaginal ultrasonography and magnetic resonance imaging. At 26 years old, she underwent bilateral cystectomy for bilateral mature teratoma of the ovary. During laparoscopic surgery, a cystic tumor appeared in the pouch of Douglas and was firmly adhered to the surrounding tissues. Both ovaries were normal. The resected tumor was diagnosed as extragonadal, benign, mature teratoma. To avoid the extragonadal recurrence of mature teratoma, removal of tumor contents from intraperitoneal spillage by lavage should be performed at the end of surgery.

Effect of eicosapentaenoic acid agent on aggravated hypertriglyceridemia during pregnancy.

Aggravated hypertriglyceridemia with a serum triglyceride of more than 1000 mg/dL is a risk of acute pancreatitis during pregnancy. However, there have been few reports on the administration of an eicosapentaenoic acid (EPA) agent for aggravated hypertriglyceridemia during pregnancy. A 29-year-old multiparous Japanese woman was transferred to our hospital at 29 + 0 weeks of gestation due to hypertriglyceridemia of 898 mg/dL. Because diet control was not enough, we decided to use an EPA agent, resulting in a reduction in triglyceride levels to 550 mg/dL. A male infant, weighing 2667 g, was born at 37 + 2 weeks transabdominally, and was complicated with respiratory distress syndrome. The final diagnosis was type III hyperlipoproteinemia with the apolipoprotein E3/2 phenotype and a broad ß-migrating lipoprotein on polyacrylamide gel electrophoresis of serum lipoproteins. In conclusion, an EPA agent may be a possible therapeutic approach for aggravated hypertriglyceridemia during pregnancy, although it may increase a risk of respiratory distress syndrome.

[Brain protection during operation for aortic arch aneurysms].

Aortic arch replacement has been safely performed by moderate hypothermic circulatory arrest, and antegrade selective cerebral perfusion. To prevent permanent neurological deficit, it is important to precisely evaluate brain, neck vessels and atherosclerotic thoracic aorta by computed tomography(CT), magnetic resonance imaging (MRI)and intraoperative epiaortic echography, which can lead the safest cannulation site, careful manipulation of cerebral perfusion catheters. It is also important to make good exposure of surgical site, especially in distal anastomosis, and irrigate and flush atheromatous debris. Since 1991 to 2013, our consecutive 410 cases of total and hemi arch replacement of aorta, including 57 emergency cases, result in 22 cases( 5.4%) of mortality( 3.1% of elective, 19.3% of emergency) and 10 cases( 2.4%) of stroke( 2.3% of elective, 3.5% of emergency). Mortality and morbidities are more likely be occurred in emergency cases. It is possible to keep good operative results with reliable brain protection of selective antegrade cerebral perfusion which gives enough time for secure open distal anastomosis to every surgeon, but atheroemborism from shaggy aorta remains as a problem yet to be solved.

Successful Crossover Bypass Using a Lateral Femoral Circumflex Artery as an Outflow Vessel for Indirect Revascularization in Critical Limb Ischemia: A Case Report.

A 78-year-old man presented with severe stage 3 (Fontaine IV, Rutherford 5, W1 I3 FI0) right limb ischemia. Although his artery was completely occluded from below the right external iliac to the popliteal artery, collateral circulation from the right lateral femoral circumflex artery was well developed and supplied the lower extremity arteries. We selected an uncommon crossover bypass strategy with the left common femoral artery to the right lateral femoral circumflex artery to improve lower extremity perfusion via indirect revascularization. Bypass using the lateral femoral circumflex artery as an outflow is an option for patients with major lower extremity artery occlusions.

Small cell carcinoma of the endometrium: a report of two cases.

Primary small cell carcinoma of the endometrium is rare and has an extremely poor prognosis. This report describes two cases of small cell carcinoma of the endometrium diagnosed as stage III. Case 1 was diagnosed as stage IIIc. She underwent surgery and chemotherapy. For a locally recurrent tumor, she received radiotherapy. She has been well with no evidence of disease for 4 years. Case 2 was diagnosed as stage IIIa. She underwent surgery. The tumor recurred soon after the surgery, and she died 33 days after the surgery. In the literature, the median survival reported for patients with stage III and IV is only 5 months. Case 1 is the 4th case showing long-term survival with advanced-stage disease. The optimal treatment for this rare tumor has not been established. Considering its rarity and variability, it is difficult to establish an evidence-based therapeutic regimen.

Uterine torsion in non-gravid women: A case report and review of cases reported in the last 20 years.

Uterine torsion is defined as a rotation of >45° around the long axis of the uterus. Uterine torsion is an uncommon event but is even rarer in non-gravid women, with only 25 cases reported in the last 20 years. Here, we report a case of uterine torsion associated with multiple pedunculated subserosal uterine leiomyomas in an 83-year-old woman. She presented at the hospital with lower abdominal pain, and a computed tomography scan revealed multiple uterine leiomyomas with calcifications. Subsequent magnetic resonance imaging raised suspicion for torsion of pedunculated subserosal uterine leiomyomas. Emergency laparotomy was performed, and the patient was diagnosed with uterine torsion with multiple pedunculated subserosal uterine leiomyomas. Total abdominal hysterectomy and bilateral salpingo-oophorectomy were performed. The patient's postoperative course was uneventful. Although difficult to diagnose due to its rarity, uterine torsion can be life-threatening and may cause infertility. Therefore, early diagnosis with imaging and surgical intervention are crucial to avoid serious complications.

Prosthesis selection for aortic valve replacement in patients on hemodialysis.

OBJECTIVE: The purpose of this study was to evaluate the outcomes of prosthesis selection in hemodialysis patients undergoing valve replacement for aortic valve stenosis. METHODS: From July 2008 to December 2016, 76 patients on hemodialysis underwent aortic valve replacement for aortic valve stenosis. Of these patients, 30 patients were treated by a mechanical valve and 46 patients were treated by a bioprosthesis. Early outcomes and long-term outcomes were compared. RESULTS: The mean age of the patients treated by a mechanical valve was younger than the patients treated by a bioprosthesis (p < 0.001). There were no significant differences in in-hospital mortality (p = 0.52). For the long-term outcomes, complications associated with bleeding were higher in patients who received a mechanical valve (p = 0.032). However, no significant difference was observed in mortality (p = 0.65) and major adverse cardiovascular cerebrovascular event (MACCE: p = 0.59). The actuarial survival rate with a mechanical valve was 56.7% (95% CI 36.4-72.8%) at 3 years and 48.6% (95% CI 28.9-65.8%) at 5 years. The actuarial survival rate with a bioprosthesis was 61.2% (95% CI 44.0-74.5%) at 3 years and 39.5% (95% CI 20.9-57.8%) at 5 years. No patients from both groups needed redo surgery for valvular deterioration. Further, there was no significant difference in long-term mortality (p = 0.91) and MACCE (p = 0.63) in a propensity score-matched patient comparison. CONCLUSIONS: Although bleeding complications were higher in patients who received a mechanical valve, there were no significant differences in early- and long-term mortality, and MACCE between patients treated by a mechanical valve and a bioprosthesis.

Risk factors for spinal cord injury in patients undergoing frozen elephant trunk technique for acute aortic dissection.

BACKGROUND: The purpose of this study was to evaluate the risk factors for spinal cord injury (SCI) in patients with acute aortic dissection undergoing surgery with frozen elephant trunk technique (FET). METHODS: From December 2014 to February 2018, 17 patients with acute aortic dissection underwent surgical treatment of the aortic arch with FET. SCI occurred in 3 patients. Risk factors for SCI were evaluated. RESULTS: Mean age of the patients was 56 years and 88.2% were male. The ratio of true lumen to total aortic diameter at the level of carina (before: 0.48 vs. after: 0.75, P < 0.001), aortic valve (before: 0.47 vs. after: 0.67, P = 0.001), and celiac artery (before: 0.48 vs. after: 0.68, P = 0.003) increased after surgery. There were no significant differences in perioperative minimum hemoglobin level and postoperative mean arterial pressure between patients with and without SCI. However, patients with SCI had higher creatinine level before surgery (SCI: 1.32 mg/dL vs. no SCI: 0.81 mg/dL, P = 0.023). Although there was no difference in number of patent intercostal arteries before surgery, those originating from the true lumen were fewer in patients with SCI (SCI: 2.7 vs. no SCI: 8.6, P = 0.021). Furthermore, with entry closure, significant decrease in patency was observed in intercostal arteries originating from the false lumen (before: 3.1 vs. after: 1.0, P < 0.001). CONCLUSION: FET was useful in entry closure. However, FET in patients with higher creatinine level and those who may have significant spinal cord perfusion from the false lumen could be a risk factor for postoperative SCI.