Apocrine gland adenocarcinoma in a persian cat remission with homeopathic treatment: case report

Apocrine gland carcinomas are rare malignant neoplasms that occur in cats. Available treatment is surgical, would lead to total ablation of the external acoustic meatus and usually recurrent [1,2].Methodology:In December 2020, a homeopathic consultation of the feline, male, Persian, 13 years old, a history of the disease was reported, which started in 2015, adding up to six recurrences of tumor processes in the eyelids and recent formation in the left ear canal.The result of the histopathological examination confirmed apocrine adenocarcinoma.Prior to homeopathic treatment, tumors recurred despite surgical interventions and prophylactic treatment with trichloracetic acid.However, remaining formations were noted in the eyelids and ear canal. It has a rounded blackened shape in the upper right eyelid measuring 0.4 cm and the lower 0.2 cm, round. In the left ear canal, around 1.6 cm, in addition to 4 points scattered in the ear folds. Homeopathic treatment was started for two months with Arsenicum album30 cH, twice a day; Carcinosinum 200 cH, once a day, and complex containing Avena sativa4 cH, Echinacea angustifolia4 cH, Conium maculatum6 cH, Thuja officinalis6 cH and Silicea terra6 cH, four times a day. Every two months, the clinical picturewas reassessed, potenciesand frequencies readjusted.In a few months,there was complete remission of the tumor, recovery of welfare, and improvement in mood and appetite. The free and informed consent term was signed. There was no recurrence of tumors until May2022. Conclusion:This study proved to be effective, documented with photos and exams. Approaching a rare case may provide a new therapeutic possibility. The credibility of quality homeopathic case reports has been increasing due to methodological requirements using tools developed in recent studies.

Intraductal Adenocarcinoma of the Prostate With Cribriform or Papillary Ductal Morphology: Rare Biopsy Cases Lacking Associated Invasive High-grade Carcinoma.

Prostatic duct adenocarcinoma, characterized by pseudostratified columnar epithelium, has historically been considered invasive carcinoma, although it may commonly have an intraductal component. Usual (acinar) intraductal carcinoma of the prostate (IDC-P) is a noninvasive high-risk lesion typically associated with high-grade, high-stage prostate cancer. Whereas there have been rare biopsy studies of pure acinar IDC-P or IDC-P associated with only low-grade carcinoma, there have been no analogous series of IDC-P with cribriform or papillary ductal morphology on biopsy unassociated with invasive high-grade carcinoma. We identified 14 patients with biopsies showing IDC-P with ductal morphology, defined as prostatic duct adenocarcinoma confined to glands/ducts with immunohistochemically proven retention of basal cells. Our series includes 12 patients with pure IDC-P and 2 patients with concurrent low-volume Grade Group 1 invasive cancer in unassociated cores. Three patients underwent radical prostatectomy: 2/3 had high-grade cancer in their resection specimen (Grade Group 3, Grade Group 5), including 1 with advanced stage and nodal metastases; 1/3 had Grade Group 1 organ-confined carcinoma and spatially distinct IDC-P with ductal morphology. Five men had only follow-up biopsies: 2/5 had cancer (Grade Group 2, Grade Group 4); 1/5 had IDC-P (on 2 repeat biopsies); and 2/5 had benign transurethral resection of the prostate. In all 5 cases with invasive cancer, the invasive portion was comprised purely of acinar morphology; no invasive ductal component was identified. Five patients did not have follow-up biopsies and were treated with radiation therapy±androgen deprivation. One patient had no follow-up information. In an analogous situation to acinar IDC-P, we propose that rarely there is a precursor form of ductal adenocarcinoma that can exist without concurrent invasive high-grade carcinoma and propose the term "IDC-P with ductal morphology," consistent with the terminology for acinar prostate adenocarcinoma. Until more evidence is accumulated, we recommend reporting and treating patients with IDC-P with ductal morphology in a manner analogous to those with acinar IDC-P. As with pure IDC-P with acinar morphology, we would also recommend not grading pure IDC-P with ductal morphology. Finally, we propose a new addition to the diagnostic criteria of IDC-P to include intraductal lesions with ductal morphology consisting of papillary fronds or cribriform lesions lined by cytologically atypical pseudostratified epithelium.

Comprehensive subgroup analyses of survival outcomes between clear cell renal cell adenocarcinoma and papillary renal cell adenocarcinoma.

To comprehensively compare the survival outcomes of clear cell renal cell carcinoma (ccRCC) and papillary renal cell carcinoma (pRCC), the study cohort included ccRCC and pRCC patients in 2004-2017 from the Surveillance, Epidemiology, and End Results (SEER) database, which comprises 18 registries. Primary outcomes including overall mortality (OM) and cancer-specific mortality (CSM) were evaluated. Subgroup analyses were conducted for different ages, race, and disease stages. A total of 112,270 cases were eligible for the current analysis, including 92,209 cases of ccRCC and 20,061 cases of pRCC. Univariate analyses suggested that pRCC has a more favorable outcome than ccRCC in terms of CSM (HR: 0.72, 95% CI: 0.68-0.75, p < 0.001) and OM (HR: 0.90, 95% CI: 0.88-0.93, p < 0.001). Multivariate-adjusted HRs suggested that pRCC has worse survival outcomes than ccRCC (adjusted HR: 1.08 for CSM and 1.05 for OM, both p < 0.05). Subgroup analyses showed that pRCC had a significantly poorer prognosis than ccRCC among patients ≤45 years old (HRCSM : 1.59, 95% CI: 1.31-1.93, p < 0.001; HROM : 1.63, 95% CI: 1.40-1.90, p < 0.001). Among patients with distant metastasis, those with pRCC had a higher risk of CSM and OM than those with ccRCC (HRCSM : 1.28, 95% CI: 1.19-1.39, p < 0.001; HROM : 1.30, 95% CI: 1.21-1.40, p < 0.001). Propensity score analyses for patients ≤45 years old and those with metastasis showed similar results. The lack of information on pRCC subtypes in the SEER database was a limitation. In conclusion, pRCC has poorer survival outcomes than ccRCC among patients younger than 45 years old and patients with distant metastasis.

Solid pseudopapillary neoplasm of pancreas: Two case reports.

RATIONALE: About 8384 cases of solid pseudopapillary neoplasms (SPN) of pancreas have been published in English literature, from 1933 to 2018. This is a low-grade tumor that usually occurs in children but is rare in adults and, in exceptional cases, can show extrapancreatic localization. In this paper we present 2 unusual cases of SPNs, 1 with retroperitoneal location (case 1) and 1 that was firstly diagnosed as a G1 neuroendocrine tumor (NET) and showed hepatic metastases after 13 years (case 2). PATIENT CONCERNS: No symptoms in first case. The tumor was incidentally diagnosed, during ultrasound examination. In the second case, the metastasis was observed during regular follow-up. DIAGNOSES: The diagnosis was established based on the histological features and immunohistochemical profile that showed positivity for vimentin, nuclear ß-catenin, cyclin D1, CD10, and SRY-related high-mobility group box 11 and negativity for maspin. INTERVENTIONS: Surgical excision, in both cases. OUTCOMES: No recurrences in first case, at 5 months after diagnosis. Hepatic metastases in the second case, at 13 years after diagnosis, with portal invasion after another 15 months. LESSONS: Without a complex immunoprofile, SPN can be misdiagnosed as NET. SPN can be a low-grade tumor but long-time follow-up is mandatory to detect delayed metastases. A correct diagnosis is necessary for a proper therapeutic management.

Clinico-pathological outcomes after total parietal peritonectomy, cytoreductive surgery and hyperthermic intraperitoneal chemotherapy in advanced serous papillary peritoneal carcinoma submitted to neoadjuvant systemic chemotherapy- largest single institute experience.

INTRODUCTION: Serous papillary peritoneal carcinoma (SPPC) is a rare clinical entity. Based on the understanding of the pattern of spread, its multifocality, polyclonality and the high frequency of diffuse, widespread peritoneal metastasis, a robust rationale for cytoreductive surgery (CRS) and hyperthermic intraperitoneal chemotherapy (HIPEC) for SPPC exists. Herein we report the clinical outcomes of SPPC patients treated with neoadjuvant systemic chemotherapy (NACT) followed by CRS including total parietal peritonectomy and HIPEC. METHODS: Clinico-pathological data of 22 patients of serous papillary peritoneal carcinoma (SPPC) was retrospectively analyzed from a prospectively maintained database from June 2000 to July 2017. Patients were treated with CRS, total parietal peritonectomy and HIPEC with cisplatin (42 mg/L of perfusate) and doxorubicin (15 mg/L of perfusate) after NACT. Survival curves were calculated from the date of surgery. RESULTS: 22 patients underwent CRS, total parietal peritonectomy and HIPEC. The median age was 62 years (Range 47-72). On histological evaluation, 18/30 (60%) parietal peritonectomy specimens showed microscopic disease, when no disease was evident macroscopically at surgical exploration. Grade III-IV surgical complications were recorded in 4/22 (18%) patients. There was no postoperative mortality. At a median follow up of 12 months, the five-year overall survival (OS) was 64.9%. The median OS was not reached. Median progression-free survival was 32.9 months and progression-free survival at 5 years was 33.2%. CONCLUSION: CRS with total peritonectomy + HIPEC after NACT, presents as a promising treatment modality for SPPC, and could be associated with good survival results in patients with SPPC.

Micropapillary Cervical Adenocarcinoma: A Clinicopathologic Study of 44 Cases.

Micropapillary adenocarcinoma has been reported as an aggressive variant of adenocarcinoma in several organs, where it is associated with poor clinical outcome. This study reports the clinicopathologic features and outcomes of cervical adenocarcinomas with a micropapillary component (micropapillary cervical adenocarcinomas); this represents the largest reported study of these neoplasms. The study comprised 44 cervical adenocarcinomas of usual (human papillomavirus-related)-type (84%), mucinous, not otherwise specified (4.5%), gastric-type (4.5%), endometrioid (4.5%), and adenosquamous carcinoma (2%). The micropapillary component comprised >50% of the neoplasm in 34 cases (77%) (group 1), and 10% to 50% in 10 cases (23%) (group 2). Lymph node metastasis was present in 41 of 44 (93%) cases and typically the nodal tumor retained a prominent micropapillary morphology. Follow-up ranged from 7 to 123 months (mean, 65.9 mo). Seventeen of 44 (38.6%) patients had no evidence of disease on follow-up, 6/44 (13.6%) were alive with disease, and 21/44 (47.7%) died of disease. There were no survival differences between group 1 and group 2. On univariate analysis, lymph node metastasis (P=0.0015), lymphovascular space invasion (P=0.002), parametrial involvement (P=0.03), and depth of stromal invasion (P=0.045) were related to tumor recurrence. On multivariate analysis, lymph node metastasis (P=0.001), and extent of lymphovascular space invasion (P=0.027) were significant independent predictors of tumor recurrence. Our study shows that a micropapillary component in cervical adenocarcinoma may be associated with aggressive behavior and that a micropapillary architecture may occur within a variety of types of cervical adenocarcinoma.

Risk of Recurrence in Differentiated Thyroid Cancer: A Population-Based Comparison of the 7th and 8th Editions of the American Joint Committee on Cancer Staging Systems.

BACKGROUND: Differentiated thyroid cancer (DTC) survival is excellent, making recurrence a more clinically relevant prognosticator. We hypothesized that the new American Joint Committee on Cancer (AJCC) 8th edition improves on the utility of the 7th edition in predicting the risk of recurrence in DTC. METHODS: A population-based retrospective review compared the risk of recurrence in patients with DTC according to the AJCC 7th and 8th editions using the Surveillance, Epidemiology, and End Results-based Kentucky Cancer Registry from 2004 to 2012. RESULTS: A total of 3248 patients with DTC were considered disease-free after treatment. Twenty percent of patients were downstaged from the 7th edition to the 8th edition. Most patients had stage I disease (80% in the 7th edition and 94% in the 8th edition). A total of 110 (3%) patients recurred after a median of 27 months. The risk of recurrence was significantly associated with stage for both editions (p < 0.001). In the 7th edition, there was poor differentiation between lower stages and better differentiation between higher stages (stage II hazard ratio [HR] 0.91, 95% confidence interval [CI] 0.39-2.11; stage III HR 3.72, 95% CI 2.29-6.07; stage IV HR 11.66, 95% CI 7.10-19.15; all compared with stage I). The 8th edition better differentiated lower stages (stage II HR 4.06, 95% CI 2.38-6.93; stage III HR 13.07, 95% CI 5.30-32.22; stage IV 11.88, 95% CI 3.76-37.59; all compared with stage I). CONCLUSIONS: The AJCC 8th edition better differentiates the risk of DTC recurrence for early stages of disease compared with the 7th edition. However, limitations remain, emphasizing the importance of adjunctive strategies to estimate the risk of recurrence.

Intracholecystic Papillary Neoplasms Are Distinct From Papillary Gallbladder Cancers: A Clinicopathologic and Exome-sequencing Study.

Although intracholecystic papillary neoplasms (ICPNs) have been increasingly recognized, their features remain unclear because of the lack of standardized definition. This study aimed to elucidate clinicopathologic and genetic features of ICPNs using stringent diagnostic criteria. On the basis of the recently proposed criteria, gallbladder neoplasms showing delicate papillary growth were diagnosed as ICPNs, while polypoid papillary adenocarcinomas arranged in a complex architecture were categorized as papillary gallbladder cancers (GBCs). Clinicopathologic features were compared among ICPNs (n=7), papillary GBCs (n=24), and nonpapillary GBCs (n=44). Whole-exome and validation Sanger sequencing was also conducted. Gross mucin hypersecretion was detected in 3/7 ICPNs (43%), 1/24 papillary GBCs (4%), and 1/44 nonpapillary GBCs (2%) (P<0.001). All patients with ICPN lacked lymphovascular invasion and nodal metastasis, while these features were occasionally observed in patients with papillary or nonpapillary GBC (13% to 59%). ICPNs were less advanced than papillary and nonpapillary GBCs (P<0.001) with all cases of ICPNs being recurrence-free. Whole-exome and Sanger sequencing identified somatic mutations in STK11 (a causative gene of Peutz-Jegher syndrome; n=3), CTNNB1 (n=2), and APC (a gene of familial adenomatous polyposis; n=1) in ICPNs, while those alterations were exceptional in papillary and nonpapillary GBCs. ICPNs more commonly showed cytoplasmic and/or nuclear expressions of ß-catenin than papillary and nonpapillary GBCs. In conclusion, the histology-based classification of gallbladder papillary neoplasms is useful for identifying ICPNs that share clinicopathologic features with the pancreatic counterpart. ICPNs meeting the criteria were genetically distinct from papillary and nonpapillary GBCs, with STK11, CTNNB1, and APC being identified as major driver genes for ICPNs.

Comparison of the Survival Outcomes of Pancreatic Cancer and Intraductal Papillary Mucinous Neoplasms.

OBJECTIVES: The aims of the study were to compare survival outcomes between patients with pancreatic ductal adenocarcinoma (PDAC) and invasive intraductal papillary mucinous neoplasms (IPMN) and to determine candidates for adjuvant chemotherapy. METHODS: A total of 579 consecutive patients, including 375 PDAC and 204 IPMN patients, were reviewed. Stage-matched comparisons of survival data were conducted using the Cox proportional hazards model and propensity analysis. To evaluate prognostic factors, univariate and multivariate Cox regression analyses were performed. RESULTS: The overall survival for invasive IPMN was significantly longer than that for PDAC (hazard ratio, 2.34; P = 0.0001). When the analysis was limited to stage I patients, the 5-year overall survival rate of invasive IPMN patients was significantly better than that of PDAC patients (100% vs 74.1%, P = 0.0092); however, no difference was observed between stage II patients with invasive IPMN and PDAC (hazard ratio, 1.49; P = 0.09). The Cox proportional hazards model and propensity analysis demonstrated no difference in stage-matched survival. Multivariate analysis revealed that only T (≥3) was an independent prognostic factor for invasive IPMN. CONCLUSIONS: Stage-matched analysis did not show a significant survival difference between invasive IPMN and PDAC patients, and T3 or higher was an independent prognostic factor for invasive IPMN.

A protocol for a Canadian prospective observational study of decision-making on active surveillance or surgery for low-risk papillary thyroid cancer.

INTRODUCTION: Low-risk papillary thyroid cancer (PTC) is increasingly being diagnosed throughout the world; yet the mortality risk is low compared with other malignancies. Traditional management includes thyroid surgery, sometimes followed by radioactive iodine and thyroid hormone treatment. Active surveillance (AS) has been proposed as a means to reduce overtreatment of PTC. AS involves close disease follow-up, with the intention to intervene if the disease progresses, or on patient request. METHODS AND ANALYSIS: This is a multiphase prospective observational study. In the first phase of this study, consenting eligible adults with low-risk PTC, that is, <2 cm in maximal diameter, confined to the thyroid and not immediately adjacent to critical structures in the neck, are provided verbal and written information about PTC disease prognosis following surgery or AS. Questionnaires are administered at baseline and after the disease management decision on AS or surgery is finalised. Patients may choose either option (surgery or AS), and the primary outcome is the frequency with which either disease management option is chosen. Secondary outcomes include: rationale for the decision, role of the patient in decision-making and decision satisfaction. In the second phase of the study, consenting eligible adult patients who completed the first study phase may enrol in respective AS or surgery group follow-up studies. The following outcomes are examined 1 year after enrolment in the follow-up phase: decision regret about disease management choice (primary outcome), psychological distress, disease-specific quality of life, fear of disease progression, body image satisfaction, disease progression, crossover to surgery in the AS group, new chronic thyroid hormone use and healthcare resource utilisation. ETHICS AND DISSEMINATION: The University Health Network Research Ethics Board approved this study (ID 15-8942). The results will be published in an open access journal. TRIAL REGISTRATION NUMBER: NCT03271892; Pre-results.

Aggressive Digital Papillary Adenocarcinoma: Population-Based Analysis of Incidence, Demographics, Treatment, and Outcomes.

BACKGROUND: As a rare cutaneous malignancy, epidemiologic and outcomes data for aggressive digital papillary adenocarcinoma (ADPA) are limited and no treatment guidelines exist. OBJECTIVE: To provide a population-based study of ADPA incidence and outcomes with a subgroup comparison of patients with localized versus regional disease. METHODS: Data from 18 registries within the National Cancer Institute's Surveillance, Epidemiology, and End Results (SEER) program were examined for patients with ADPA (1995-2013) to provide demographic- and cancer-related information, and to calculate race- and age-specific rate ratios, incidence, and mortality. Patients were stratified by the stage for further comparison. RESULTS: Ninety-four cases of ADPA were identified. Overall, ADPA incidence was 0.08 per 1,000,000 person-years, 4 times higher in males than in females (0.13 vs 0.03, p < .001), and most common in Caucasians. Regional disease spread occurred in 22.3% of patients and disease-specific mortality in 2.1% of patients. Patients with regional versus localized disease at diagnosis did not differ significantly in sex, age, race, primary site, tumor size, or mortality. CONCLUSION: Aggressive digital papillary adenocarcinoma is a rare malignancy with increasing incidence. Regional disease spread is not infrequent, but mortality is rare. Identification of patients best suited for additional diagnostic procedures or more extensive surgical resection remains challenging.

[A case of intracholecystic papillary neoplasm of the gallbladder (ICPN) examined using contrast-enhanced endoscopic ultrasonography].

A 78-year-old woman was found to have gallbladder wall thickening on ultrasonography during a routine health check-up and was referred to our clinic. On contrast-enhanced endoscopic ultrasonography, a papillary lesion measuring 14mm was detected in the fundus, which showed a heterogeneous enhancement at the early phase. She underwent cholecystectomy and gallbladder bed resection. Histological examination revealed that the tumor consisted of mucinous atypical cells, regularly arranged in a high-papillary architecture with delicate fibrovascular cores, which led to the diagnosis of intracholecystic papillary neoplasm of the gallbladder.

Prognostic significance and adjuvant chemotherapy survival benefits of a solid or micropapillary pattern in patients with resected stage IB lung adenocarcinoma.

OBJECTIVE: To evaluate the prognostic significance and beneficiaries of adjuvant chemotherapy (ACT) in various histological patterns of stage IB lung adenocarcinoma according to the 8th tumor-node-metastasis (TNM) classification. METHODS: A total of 1131 patients with pathological stage IB lung adenocarcinoma according to the 8th TNM classification who underwent lobectomy or segmentectomy were enrolled in this study. Based on the proportion of solid/micropapillary components, the patients were classified into 3 groups: solid/micropapillary-negative (SMPN) (n = 719; median survival, 49.7 months; interquartile range [IQR]. 35.1-67.0 months), solid/micropapillary-minor (SMPM; >5% but not predominant) (n = 272; median survival, 38.8 months; IQR, 26.6-51.5 months) and solid/micropapillary-predominant (SMPP; >5% and the most dominant) (n = 140; median survival, 39.6 months; IQR, 26.8-52.5 months). The predictors of disease-specific survival and recurrence-free survival were investigated. To reduce selection bias, propensity score-matching analysis was implemented before survival data were compared. RESULTS: Our data show significant differences in survival rates based on the proportion of solid/micropapillary patterns. The SMPM group had significantly higher cumulative incidences of lung cancer-specific death (P = .000) and recurrence (P = .000) compared with the SMPN group, so did the SMPP group when compared with SMPM patients (P = .000 for both). Multivariate analysis showed that the SMPM and SMPP patterns were poor prognostic factors for disease-specific survival (hazard ratio [HR], 1.86; 95% confidence interval [CI], 1.12-3.09 and HR, 4.56; 95% CI, 2.69-7.71, respectively) and recurrence-free survival (HR, 1.64; 95% CI, 1.20-2.24 and HR, 2.43; 95% CI, 1.64-3.60, respectively), as were older age, male sex, smoking history, larger tumor size, necrosis, and abnormal pulmonary function. Survival analysis stratified by histological pattern showed that patients with the SMPP pattern who received ACT had obviously lower cumulative incidences of lung cancer-specific death (HR, 0.46; 95% CI, 0.22-0.93; P = .031) and recurrence (HR, 0.48; 95% CI, 0.26-0.88; P = .017). CONCLUSIONS: Solid/micropapillary patterns were associated with poor prognosis, even if they were not predominant. ACT contributed to survival benefits in the SMPP subgroup of patients with stage IB lung adenocarcinoma.

Surveillance and Outcomes of Nonresected Presumed Branch-Duct Intraductal Papillary Mucinous Neoplasms: A Meta-analysis.

OBJECTIVES: Guidelines regarding the surveillance of intraductal papillary mucinous neoplasms (IPMNs) are controversial because of uncertain risk of malignancy, agnosticism regarding the use of endoscopic ultrasound, and their recommendation to stop surveillance after 5 years. We present a systematic review and meta-analysis of the risk of malignancy and other end points and estimate the value of endoscopic ultrasound for surveillance. METHODS: We systematically searched MEDLINE for studies with a cohort of patients with presumed branch-duct IPMN who initially were managed nonsurgically. Data regarding study characteristics, surveillance, and outcomes were extracted. Incidence rates of morphologic progression, malignancy, surgery, and death were calculated with a random effects model. RESULTS: Twenty-four studies with 3440 patients and 13,097 patient-years of follow-up were included. Rates of morphologic progression, surgery, malignancy, and death were 0.0379, 0.0250, 0.0098, and 0.0043 per patient-year, respectively. Endoscopic ultrasound was not associated with significantly different rates of these outcomes. CONCLUSIONS: The risk of malignancy calculated in this study was low and in line with recent systematic reviews. Endoscopic ultrasound does not have marginal use in surveillance. Given the limitations of a systematic review of nonrandomized studies, further studies are needed to determine the optimal surveillance of branch-duct IPMNs.

Uptodate in the assessment and management of intraductal papillary mucinous neoplasms of the pancreas.

Intraductal Papillary Mucinous Neoplasms (IPMNs) are the most common cystic tumors of the pancreas and are considered premalignant lesions. IPMNs are characterized by the papillary growth of the ductal epithelium with rich mucin production, which is responsible for cystic segmental or diffuse dilatation of the main pancreatic duct (MPD) and/or its branches. According to the different involvement of pancreatic duct system, IPMNs are divided into main duct type (MD-IPMN), branch duct type (BD-IPMN), and mixed type (MT-IPMN). IPMNs may be incidentally discovered in asymptomatic patients, particularly in those with BD-IPMNs, when imaging studies are performed for unrelated indications. The increase in their frequency may reflect the combined effects of new diagnostic techniques, the improvement of radiologic exams and progress in the recognition of the pathology. MD-IPMNs present a higher risk of malignant progression than BD-IPMNs; as a consequence, all the guidelines strictly suggest the need of surgery for MD- and MT- IPMNs with MPD > 10 mm, while the management of BD-IPMNs is still controversial and depends on several cysts and patients features. The choice between non-operative and surgical management depends on the distinction between benign and invasive IPMN forms, assessment of malignancy risk, patient's wellness and its preferences. This manuscript revises the different guidelines for the management of IPMNs that have been published in different world countries: the international (Sendai 2006 and Fukuoka 2012), the 2013 European, the 2014 Italian, and finally the 2015 American guidelines. In summary, this review will integrate the recent insights in the combination of diagnostic techniques, such as Magnetic Resonance Imaging (MRI) and endoscopic ultrasound (EUS), pathology classification, and management of IPMNs.

Active Surveillance Beyond 5 Years Is Required for Presumed Branch-Duct Intraductal Papillary Mucinous Neoplasms Undergoing Non-Operative Management.

OBJECTIVES: To evaluate the results of active surveillance beyond 5 years in patients with branch-duct intraductal papillary mucinous neoplasms (BD-IPMNs) without worrisome features (WF) and high-risk stigmata (HRS) undergoing non-operative management. METHODS: Patients with a minimum follow-up of 5 years who underwent surveillance with at least yearly magnetic resonance imaging were included. New onset of and predictors of WF/HRS during follow-up as well as long-term survival were analyzed. RESULTS: In all, 144 patients were followed for a median of 84 months. At diagnosis multifocal BD-IPMNs were found in 53% of cases and mean size of the largest cyst was 15.5 mm. Changes during follow-up were observed in 69 patients (48%). New onset of WF/HRS were observed in 26 patients (18%) but the rate of HRS was only 4%. WF and HRS developed after a median follow-up of 71 and 77.5 months from diagnosis, respectively, and without previous changes in 19/26 patients. Independent predictors of WF/HRS development were size at diagnosis>15 mm, increase in number of lesions, main pancreatic duct growth rate ≥0.2 mm/year, cyst growth rate >1 mm/year. Overall, the rate of pancreatic invasive malignancy was 2% and the 12-year disease-specific survival was 98.6%. CONCLUSIONS: Long-term nonoperative management is safe for BD-IPMNs without WF and HRS. Discontinuation of surveillance cannot be recommended since one out of six patients developed WF/HRS far beyond 5 years of surveillance and without previous relevant modifications. An intensification of follow-up should be considered after 5 years.

Disparities in receipt of care for high-grade endometrial cancer: A National Cancer Data Base analysis.

PURPOSE: To examine patterns of care and survival for Hispanic women compared to white and African American women with high-grade endometrial cancer. METHODS: We utilized the National Cancer Data Base (NCDB) to identify women diagnosed with uterine grade 3 endometrioid adenocarcinoma, carcinosarcoma, clear cell carcinoma and papillary serous carcinoma between 2003 and 2011. The effect of treatment on survival was analyzed using the Kaplan-Meier method. Factors predictive of outcome were compared using the Cox proportional hazards model. RESULTS: 43,950 women were eligible. African American and Hispanic women had higher rates of stage III and IV disease compared to white women (36.5% vs. 36% vs. 33.5%, p<0.001). African American women were less likely to undergo surgical treatment for their cancer (85.2% vs. 89.8% vs. 87.5%, p<0.001) and were more likely to receive chemotherapy (36.8% vs. 32.4% vs. 32%, p<0.001) compared to white and Hispanic women. Over the entire study period, after adjusting for age, time period of diagnosis, region of the country, urban or rural setting, treating facility type, socioeconomic status, education, insurance, comorbidity index, pathologic stage, histology, lymphadenectomy and adjuvant treatment, African American women had lower overall survival compared to white women (Hazard Ratio 1.21, 95% CI 1.16-1.26). Conversely, Hispanic women had improved overall survival compared to white women after controlling for the aforementioned factors (HR 0.87, 95% CI 0.80-0.93). CONCLUSIONS: Among women with high-grade endometrial cancer, African American women have lower all-cause survival while Hispanic women have higher all-cause survival compared to white women after controlling for treatment, sociodemographic, comorbidity and histopathologic variables.

Paraneoplastic Evans syndrome in a patient with adenocarcinoma of the lung: A case report.

We present a rare case of newly diagnosed Evans syndrome associated with lung papillary adenocarcinoma in which the patient showed prompt restoration of blood cell count and long-lasting complete remission of Evans syndrome after lung cancer resection. Detailed investigation led to a diagnosis of Evans syndrome. In the first year of the disease, left lower lung papillary adenocarcinoma was diagnosed. Pulmonary lobectomy and three courses of chemotherapy were performed. Six months after the initial visit, the primary lung cancer and the autoimmune diseases appeared to be well controlled. We hypothesized that our patient's initial presentation of hematological manifestation was a paraneoplastic phenomenon associated to her underlying malignancy. This rare case report illustrates the unique relationship between primary lung cancer and the development of paraneoplastic Evans syndrome.

Are Clinical Guidelines for the Management of Intraductal Papillary Mucinous Neoplasms Followed?: A Single-Center Analysis.

OBJECTIVES: This study aimed to determine how frequently guidelines for the management of intraductal papillary mucinous neoplasms (IPMNs) are followed and establish factors associated with failure. METHODS: Four hundred forty-five patients with radiographic diagnosis of IPMN 1 cm or greater between January 1, 2003 and January 1, 2013 were included. We defined failure of guideline adherence if the following occurred: (a) failure of acknowledgment of IPMN, (b) failure to undergo endoscopic ultrasound, (c) failure to undergo resection, or (d) failure to undergo at least 1 surveillance image within 2 years after diagnosis. RESULTS: Failure of guideline adherence was observed in 58% of patients and evident across all the respective criteria (A: 38%, B: 25%, C: 29%, D: 33%). Age older than 68 years (P < 0.01), American Society of Anesthesiologists score of 3 or higher (P < 0.0001), benign findings on imaging (P < 0.0001), and major comorbid conditions (P < 0.01) were factors associated with higher rate of failure to compliance. On multivariate logistic regression, American Society of Anesthesiologists score of 3 or higher and benign features were associated with 4.0 times (95% confidence interval, 2.02-8.06) and 2.6 times (95% confidence interval, 1.60-4.07) higher odds of failure to compliance with guidelines, respectively. CONCLUSIONS: Compliance with clinical guidelines for the management of IPMN is poor. Socioeconomic factors do not seem to create a disparity to care. However, many patients with IPMN have other medical diagnoses that take priority over IPMN surveillance and treatment.

Intraductal papillary neoplasm of the bile duct: a case report.

Intraductal papillary neoplasm of the bile duct (IPNB) is a rare variant of bile duct tumors, characterized by papillary growth within the bile duct lumen and is regarded as a biliary counterpart of intraductal papillary mucinous neoplasm (IPMN) of the pancreas. IPNBs are mainly found in patients from Far Eastern areas, where hepatolithiasis and clonorchiasis are endemic. The Western experience, however, remains limited. In this article, we report a 56-year-old man, referred to our hospital because of deranged liver function tests. Further imaging modalities showed a cystic lesion of 9 cm diameter, arising from the left hepatic duct. Inlying was a heterogeneous, lobulated mass. The patient underwent a left hemihepatectomy and adjuvant chemotherapy. Despite recent advanced technologies, diagnosis of IPNB is still challenging, especially in western countries due to its rarity. Early identification and resection of lesions, even in asymptomatic or minimally symptomatic patients, are however important prognostic factors.