Mucinous cystic neoplasms of the liver with biliary prolapse.

OBJECTIVES: To describe the prevalence, clinical and radiological findings of biliary prolapse in pathologically proven mucinous cystic neoplasm of the liver (MCN-L). METHODS: Thirty-four patients, all female with median age 50 years (range, 14-82), with histologically confirmed MCN-L were enrolled. Median tumor size was 9 cm (range, 2-21 cm). Fifty-seven examinations (17 ultrasound, 25 CT, and 15 MR) among 34 MCN-Ls were reviewed. Two radiologists retrospectively assessed images for tumor location, size and other morphological features of the tumor, presence of biliary prolapse and upstream bile duct dilatation. Ultrasound, CT, and MR were assessed separately. Clinical features were evaluated. Clinical and radiological characteristics of MCN-L with and without biliary prolapse were compared. RESULTS: 15% (5/34) of MCN-Ls showed biliary prolapse confirmed at pathology. None of MCN-Ls were associated with invasive carcinoma. Patients with biliary prolapse were significantly younger than those without (median 27 years [22-56] vs. median 51 years [14-82], p = 0.03). MCN-Ls with biliary prolapse were significantly smaller than those without (median 6.4 cm [2.2-7.5] vs. median 9.6 cm [3.1-21], p = 0.01). The upstream bile duct was dilated more frequently in MCN-Ls with biliary prolapse (100% vs. 38%, p = 0.02). Jaundice was significantly more common in MCN-Ls with biliary prolapse (80 vs 3%, p = 0.0005). Other clinical or radiological features were not significantly different between two groups. CONCLUSIONS: Biliary prolapse was found in 15% of MCN-Ls. MCN-Ls with biliary prolapse were significantly smaller and were more commonly associated with upstream bile duct dilation and jaundice than those without biliary prolapse.

[A Case of Early Appendiceal Adenocarcinoma Coexisting with High-Grade Appendiceal Mucinous Neoplasm].

We report a case of a female in her fifties with early appendiceal adenocarcinoma coexisting with high-grade appendiceal mucinous neoplasm(HAMN)with a review of the literature. The patient presented to our hospital because of an enlarged appendix noted by contrast-enhanced CT performed for hematuria. Contrast-enhanced CT showed that the appendix had swollen to 10 mm and mucus had accumulated inside, which had no evidence of obvious malignancy. She was followed up on CT once a year. Four years after her first visit, she underwent laparoscopic appendectomy for a definitive diagnosis. There were no adhesions or inflammation in her abdominal cavity, and the appendix root was dissected with an automatic anastomosis device. Her resected specimen macroscopically showed mild wall thickening, but no obvious neoplastic lesion. Pathological examination revealed that in many areas centered on the tip of the appendix, highly columnar atypical epithelium with enhanced mucus production was densely proliferated in the form of glandular tubular and papillary. The nuclei of the proliferating epithelium were large and the fission image was conspicuous, but they remained in the mucosa. Pathological examination diagnosed as HAMN according to the WHO classification. The atypical epithelium in a small area at the tip was particularly strong in nuclear atypia, and showed a strong positive diffusely in p53, which was an image of well-differentiated tubular adenocarcinoma. The pathological diagnosis was V, Type 0-Ⅱb, 2 mm, tub1 in HAMN, pTis, Ly0, V0, Pn0, pPM0, pDM0, pRM0, R0. Six months have passed since the operation, but no recurrence has been observed.

Biological and prognostic value of ETV5 in high-grade serous ovarian cancer.

BACKGROUND: ETS transcription factors are known to act as either positive or negative regulators of the expression of genes involved in various biological processes. It was reported that ETS variant transcription factor 5 (ETV5), a key member of the ETS family, mainly plays a role as an potential oncogene in various malignant tumors. However, the role and mechanism of ETV5 in high-grade serous ovarian cancer (HGSOC) have not been elucidated. METHODS: Quantitative real-time polymerase chain reaction (qRT-PCR) assay was used to detect ETV5 messenger ribonucleic acid (mRNA) expression in 87 HGSOC tissues and 35 normal fallopian tube tissues. Western blotting and qRT-PCR were used to detect the protein and mRNA expression of ETV5 in six ovarian cancer (OC) and human embryonic cell lines. Knockdown or overexpression of ETV5 in HGSOC cell lines, Cell Counting Kit-8, colony formation, and transwell assays were used to detect HGSOC cell proliferation, invasion, and migration capabilities. The chi-square test was used to analyze the clinicopathological characteristics of HGSOC patients. Survival analysis was performed using the Kaplan-Meier method, and the log-rank test was used to analyze the correlation between ETV5 expression and HGSOC patient prognosis. Univariate and multivariate analyses using the Cox regression model were conducted to determine the independent significance of relevant clinical covariates. RESULTS: Bioinformatic analysis demonstrated that ETV5 expression was significantly upregulated in OC (p < 0.05). qRT-PCR showed that ETV5 was significantly overexpressed in HGSOC tissues than in fallopian tube tissues (p < 0.05). qRT-PCR and western blotting assays demonstrated that ETV5 was relatively highly expressed in OC cell lines. ETV5 overexpression was positively associated with poor survival in HGSOC patients, therefore making it a high-risk factor for HGSOC progression. Furthermore, ETV5 promoted the proliferation, migration, and invasion capabilities of HGSOC cells. CONCLUSION: ETV5 has a carcinogenic effect in HGSOC and can be used as a clinically effective biomarker to determine the prognosis of HGSOC patients.

Identification of endometrial cancers and atypical hyperplasia: Development and validation of a simplified system for ultrasound scoring of endometrial pattern.

OBJECTIVES: To derive and validate a practical scoring system for identification of endometrial cancer (EC) or atypical hyperplasia (AH) using transvaginal ultrasonography (TVS) and gel infusion sonography (GIS) in women with postmenopausal bleeding (PMB). STUDY DESIGN: Endometrial pattern was correlated with endometrial pathology in consecutive women with PMB in both a derivation study (N = 164) and a validation study (N = 711). Logistic regression was used to derive and validate two scoring systems (A and B) for prediction of EC/AH: scoring system A was Doppler score + interrupted endo-myometrial junction (IEJ) (2 points); and scoring system B was Doppler score + IEJ (1 point) + Irregular Endometrial Outline (IESO) by GIS (1 point); the Doppler score was based on the presence of more than one single or double vessel (1 point) + multiple vessels (1 point) + large vessels (1 point). OUTCOME MEASURES: Diagnostic performance and calibration curves for identification of EC/AH. RESULTS: Both scoring systems had good observer agreement. VALIDATION DATA: Scoring was most effective with endometrial thickness (ET) ≥ 8 mm. Both scoring systems were well calibrated and performed satisfactorily in women with ET ≥ 8 mm. The sensitivity and specificity of a score of ≥ 2 points in system A were 92% and 84%; the respective values were 89% and 88% in system B. CONCLUSIONS: Scoring was highly efficient in identifying EC/AH. Four risk groups of EC/AH may guide the management of women with PMB: very low (ET < 4 mm), low (ET 4-7.9 mm), intermediate (ET ≥ 8 mm and score < 2 points) and high risk (ET ≥ 8 mm and score ≥ 2 points).

The Sign of Leser-Trelát and Malignant Acanthosis Nigricans Associated With Fallopian Tube Carcinoma.

BACKGROUND: The sign of Leser-Trelát is controversial and rarely reported in gynecologic malignancies. It is characterized by rapid development of new or enlarging seborrheic keratoses. CASE: A 78-year-old woman presented with unintentional weight loss and new-onset erythematous patches and plaques with thickened, rugated skin and stuck-on brown waxy papules on the chest and back. Her symptoms were concerning for a paraneoplastic eruption; workup revealed an elevated CA 125 level and an ovarian mass on abdominal computed tomography scan. Exploratory laparotomy revealed a stage IIIC serous fallopian tube carcinoma and a synchronous low-grade endometrioid adenocarcinoma of the endometrium. CONCLUSION: The sign of Leser-Trelát can be associated with fallopian tube carcinoma. When recognized, paraneoplastic dermatoses can prompt clinicians to initiate a workup for occult malignancy.

Mucinous Cystic Neoplasm of the Liver or Intraductal Papillary Mucinous Neoplasm of the Bile Duct? A Case Report and a Review of Literature.

Mucinous cystic neoplasm of the liver (MCN-L) and intraductal papillary mucinous neoplasm of the bile duct (IPMN-B) are diagnoses that were classified by the World Health Organization in 2010 as mucin-producing bile duct tumors of the hepatobiliary system. The preoperative differential diagnosis between these two entities is difficult; the presence of a communication with the bile duct is usually considered as a typical sign of IPMN-B. However, the presence of an ovarian-like stroma (OLS) has been established to define the diagnosis of MCN-L. We present the case of a 33-year-old woman with a rapid progression of a cystic tumor of the liver. In 2 years, the lesion increased from 27 to 64 mm and a dilation of the left hepatic duct appeared. Percutaneous transhepatic drainage with a biopsy was performed. No malignant cells were found on biopsy. Because of the rapid progression of the cystic tumor and unclear malignant potential, left hemihepatectomy was performed. Even though tumor masses were present in the biliary duct, on the basis of the presence of OLS, histology finally confirmed MCN-L with intermediate-grade intraepithelial dysplasia to high-grade intraepithelial dysplasia. The patient is currently under oncologic follow-up with no signs of recurrence of the disease. We present a rare case where MCN-L caused a dilation of the left hepatic duct, a sign that is usually a characteristic of IPMN-B.

Is there a survival advantage in diagnosing endometrial cancer in asymptomatic postmenopausal patients? An Israeli Gynecology Oncology Group study.

BACKGROUND: Incidental ultrasonographic findings in asymptomatic postmenopausal women, such as thickened endometrium or polyps, often lead to invasive procedures and to the occasional diagnosis of endometrial cancer. Data supporting a survival advantage of endometrial cancer diagnosed prior to the onset of postmenopausal bleeding are lacking. OBJECTIVE: To compare the survival of asymptomatic and bleeding postmenopausal patients diagnosed with endometrial cancer. STUDY DESIGN: This was an Israeli Gynecology Oncology Group retrospective multicenter study of 1607 postmenopausal patients with endometrial cancer: 233 asymptomatic patients and 1374 presenting with postmenopausal bleeding. Clinical, pathological, and survival measures were compared. RESULTS: There was no significant difference between the asymptomatic and the postmenopausal bleeding groups in the proportion of patients in stage II-IV (23.5% vs 23.8%; P = .9) or in high-grade histology (41.0% vs 38.4%; P = .12). Among patients with stage-I tumors, asymptomatic patients had a greater proportion than postmenopausal bleeding patients of stage IA (82.1% vs 66.2%; P < .01) and a smaller proportion received adjuvant postoperative radiotherapy (30.5% vs 40.6%; P = .02). There was no difference between asymptomatic and postmenopausal bleeding patients in the 5-year recurrence-free survival (79.1% vs 79.4%; P = .85), disease-specific survival (83.2% vs 82.2%; P = .57), or overall survival (79.7% vs 76.8%; P = .37). CONCLUSION: Endometrial cancer diagnosed in asymptomatic postmenopausal women is not associated with higher survival rates. Operative hysteroscopy/curettage procedures in asymptomatic patients with ultrasonographically diagnosed endometrial polyps or thick endometrium are rarely indicated. It is reasonable to reserve these procedures for patients whose ultrasonographic findings demonstrate significant change over time.

Synchronous intraductal papillary mucinous neoplasm and a pancreatic neuroendocrine tumor: more than a coincidence?

BACKGROUND: Although the association between intraductal papillary mucinous neoplasm of the pancreas (IPMN) and pancreatic neuroendocrine tumor (PNET) has been increasingly reported, whether this association is real or coincidence remains unclear. We report a case of synchronous IPMN and a PNET which were diagnosed preoperatively and discuss the tumorigenesis, clinicopathological features and management of these rare tumors based on the published literature. CASE REPORT: A 56-year-old male was incidentally diagnosed with a 14 mm branch duct IPMN and a 3.6 mm non-functional PNET during an evaluation due to persistent upper abdominal pain via endoscopic ultrasound. Close follow-up of the patient was decided as the IPMN had no worrisome features. A review of twenty-two previously reported cases of synchronous IPMN and PNET indicated that: a) only seven cases were diagnosed preoperatively; b) abdominal pain was the main presenting symptom; c) IPMN was the dominant tumor and presented with low grade dysplasia; d) the PNET was small and non-functional and had an indolent behavior; and e) only one case underwent radiologic follow-up. DISCUSSION: IPMN are associated with other pancreatic and extrapancreatic malignancies. Thus, the entire pancreatic parenchyma should be examined closely during the evaluation of an IPMN in order to exclude other pancreatic lesions, for example, a PNET.

Subtypes of stage IV ovarian cancer; response to treatment and patterns of disease recurrence.

BACKGROUND: To compare three different patterns of stage IV epithelial ovarian cancer; pleural effusion, parenchymal metastases and extra-abdominal lymph node metastases with treatment response and pattern of disease recurrence, and correlate treatment modality with outcome. METHODS: Retrospective analysis of FIGO stage IV epithelial ovarian cancer diagnosed between 2008 and 2012 in three gynaecologic oncology centres in New Zealand. RESULTS: 124 patients were analysed, 58 had pleural effusions, 38 parenchymal metastases, and 28 extra-abdominal lymph nodes. There was no significant difference in overall survival between these three groups. The most common site of first or any recurrence in all three groups was the abdomen with only a small number of recurrences arising in extra-abdominal sites. When looking at treatment modality, 13% had primary debulking surgery, 47% had neoadjuvant chemotherapy with interval debulking surgery, and 40% never had surgery. Overall survival was highest in patients with no residual abdominal disease after surgery. CONCLUSION: The site of extra-abdominal disease did not alter prognosis or pattern of disease recurrence in stage IV epithelial ovarian cancer, with most recurrences in the abdomen suggesting controlling abdominal disease with surgery may be important in all stage IV disease.

Hemosuccus pancreaticus caused by a mucinous cystic neoplasm of the pancreas.

Hemosuccus pancreaticus is a gastrointestinal hemorrhage through the main pancreatic duct. Here, we report a rare case of hemosuccus pancreaticus due to a mucinous cystic neoplasm of the pancreas. A 62-year-old woman who had been followed for a branch duct intraductal papillary mucinous neoplasm visited our emergency room due to severe abdominal pain and bloody discharge. Computed tomography revealed that the pancreatic cyst increased the tension of the wall and a high-density area indicative of bleeding into the cyst was observed. Endoscopy showed opening of and hemorrhaging from the papilla of Vater. The patient was diagnosed with hemosuccus pancreaticus caused by hemorrhaging into the cyst from the branch duct intraductal papillary mucinous neoplasm. Based on this diagnosis, elective distal pancreatectomy was performed. The histopathological diagnosis was a mucinous cystic neoplasm with intermediate-grade dysplasia based upon the pathological findings that fibrous ovarian-type stroma existed abundantly and the stroma cells were positive for progesterone receptor and inhibin. Hemosuccus pancreaticus caused by a mucinous cystic neoplasm is extremely rare and there has been only one case reported to date. In conclusion, it should be recognized that pancreatic cystic neoplasms including mucinous cystic neoplasms may cause hemosuccus pancreaticus.

Brain metastases in patients with EOC: Clinico-pathological and prognostic factors. A multicentric retrospective analysis from the MITO group (MITO 19).

BACKGROUND: Brain metastases (BM) from epithelial ovarian cancer (EOC) are considered a rare and unfavourable event. There is no consensus regarding the best management of these patients. METHODS: A multicenter retrospective analysis of patients with BM from EOC treated between 1997 and 2014 in 18 institutions of the MITO (Multicenter Italian Trials in Ovarian cancer) group was conducted. Univariate and multivariate analysis were performed. RESULTS: A total of 174 women were identified as having BM from EOC. The median time interval between primary diagnosis of EOC and occurrence of BM was 26months (range 2-129months). The median overall survival from primary EOC diagnosis was 48months (95% CI 39.5-56.4months) and from diagnosis of BM was 12months (95% CI 9.6-14.3months). The majority of enrolled women (81.7%) were classified as sensitive to platinum-based chemotherapy. Four variables were significantly associated with poor overall survival in multivariate analysis: multiple BM [HR: 1.86 (95% CI: 1.22-2.84)], presence of extracranial disease [HR: 1.77 (95% CI: 1.11-2.83)] age [HR: 1.74 (95% CI: 1.17-2.59)], and monotherapy [HR: 2.57 (95% CI: 1.64-3.86)]. On the contrary, residual tumor at primary surgery, FIGO stage at primary diagnosis and platinum sensitivity were found to have no significant impact on survival from diagnosis of brain lesions. CONCLUSIONS: Our results suggest that BM is a rare and late manifestation of EOC, with a 12-month life-span expectation. Multiple approach is a positive independent prognostic factor and should be proposed to carefully selected patients.

Ileocecal Intussusception Caused by an Appendiceal Neoplasm.

Mucinous appendiceal neoplasm occurs in less than 1% of appendicectomies. Majority of what is known in the literature is about pseudomyxoma peritonei, which exists as its disseminated form. Pictorial imagery of its pre-disseminated form is rarely observed. We present in a case report form a case of low-grade mucinous neoplasm of the appendix resulting in focal intussusception including images captured from this unique case that will become a learning case for readers of the journal.