Sinonasal Neuroendocrine Carcinoma: 15 Years of Experience at a Single Institution

Author(s):  
Charles A. Keilin ◽  
Kyle K. VanKoevering ◽  
Jonathan B. McHugh ◽  
Erin L. McKean

Abstract Objectives Sinonasal neuroendocrine carcinomas (SNECs) are among the rarest paranasal sinus cancers. Consensus guidelines for therapy are difficult to develop due to limited data regarding the natural history and successful treatment of these tumors. This study presents 15 years of experience treating SNEC at a single institution and a review of the literature. Design Retrospective review. Setting Academic medical center in the United States. Participants Patients diagnosed with primary SNEC. Main Outcome Measures Overall survival. Results Thirteen patients were identified and included. Overall estimated survival was 74.6% at 5 years. Ten of 13 (76.9%) patients were diagnosed with high-grade neuroendocrine carcinoma and three (23.1%) with intermediate or low grade. All three patients with low- or intermediate-grade cancer survived more than 10 years from their initial diagnosis (median survival: 11.6 years) and are currently alive. The four patients who died had high-grade carcinoma, and estimated overall 5-year survival for all patients with high-grade carcinomas was 65.6%. Five patients, all with high-grade carcinoma, of seven who completed primary chemoradiation therapy (CRT) required salvage resection, and 60% are alive without disease. Conclusion This cohort has a higher overall rate of survival than many recent case series and reviews. There is consensus that multimodal therapy is preferred over monotherapy, but approaches to treatment vary widely. Our approach of surgical resection as primary therapy for low-grade tumors and primary CRT for high-grade SNEC has been successful, and could indicate hope for improved survival among these patients.

Medicina ◽  
2021 ◽  
Vol 57 (12) ◽  
pp. 1338
Author(s):  
Tiberiu-Augustin Georgescu ◽  
Roxana Elena Bohiltea ◽  
Octavian Munteanu ◽  
Florentina Furtunescu ◽  
Antonia-Carmen Lisievici ◽  
...  

Neuroendocrine neoplasms (NENs) are particularly rare in all sites of the gynecological tract and include a variety of neoplasms with variable prognosis, dependent on histologic subtype and site of origin. Following the expert consensus proposal of the International Agency for Research on Cancer (IARC), the approach in the latest World Health Organization (WHO) Classification System of the Female Genital Tumours is to use the same terminology for NENs at all body sites. The main concept of this novel classification framework is to align it to all other body sites and make a clear distinction between well-differentiated neuroendocrine tumors (NETs) and poorly differentiated neuroendocrine carcinomas (NECs). The previous WHO Classification System of the Female Genital Tumours featured more or less the same principle, but used the terms ‘low-grade neuroendocrine tumor’ and ‘high-grade neuroendocrine carcinoma’. Regardless of the terminology used, each of these two main categories include two distinct morphological subtypes: NETs are represented by typical and atypical carcinoid and NEC are represented by small cell neuroendocrine carcinoma (SCNEC) and large cell neuroendocrine carcinoma (LCNEC). High-grade NECs, especially small cell neuroendocrine carcinoma tends to be more frequent in the uterine cervix, followed by the endometrium, while low-grade NETs usually occur in the ovary. NENs of the vulva, vagina and fallopian tube are exceptionally rare, with scattered case reports in the scientific literature.


2013 ◽  
Vol 23 (9) ◽  
pp. 1620-1628 ◽  
Author(s):  
Joyce N. Barlin ◽  
Robert A. Soslow ◽  
Megan Lutz ◽  
Qin C. Zhou ◽  
Caryn M. St. Clair ◽  
...  

ObjectiveWe propose a new staging system for stage I endometrial cancer and compare its performance to the 1988 and 2009 International Federation of Gynecology and Obstetrics (FIGO) systems.MethodsWe analyzed patients with 1988 FIGO stage I endometrial cancer from January 1993 to August 2011. Low-grade carcinoma consisted of endometrioid grade 1 to grade 2 lesions. High-grade carcinoma consisted of endometrioid grade 3 or nonendometrioid carcinomas (serous, clear cell, and carcinosarcoma). The proposed system is as follows:IA. Low-grade carcinoma with less than half myometrial invasionIA1: Negative nodesIA2: No nodes removedIB. High-grade carcinoma with no myometrial invasionIB1: Negative nodesIB2: No nodes removedIC. Low-grade carcinoma with half or greater myometrial invasionIC1: Negative nodesIC2: No nodes removedID. High-grade carcinoma with any myometrial invasionID1: Negative nodesID2: No nodes removedResultsData from 1843 patients were analyzed. When patients were restaged with our proposed system, the 5-year overall survival significantly differed (P < 0.001): IA1, 96.7%; IA2, 92.2%; IB1, 92.2%; IB2, 76.4%; IC1, 83.9%; IC2, 78.6%; ID1, 81.1%; and ID2, 68.8%. The bootstrap-corrected concordance probability estimate for the proposed system was 0.627 (95% confidence interval, 0.590–0.664) and was superior to the concordance probability estimate of 0.530 (95% confidence interval, 0.516–0.544) for the 2009 FIGO system.ConclusionsBy incorporating histological subtype, grade, myometrial invasion, and whether lymph nodes were removed, our proposed system for stage I endometrial cancer has a superior predictive ability over the 2009 FIGO staging system and provides a novel binary grading system (low-grade including endometrioid grade 1–2 lesions; high-grade carcinoma consisting of endometrioid grade 3 carcinomas and nonendometrioid carcinomas).


2011 ◽  
Vol 135 (6) ◽  
pp. 753-758 ◽  
Author(s):  
Shuting Bai ◽  
Dezhi Wang ◽  
Michael J. Klein ◽  
Gene P. Siegal

Abstract Context.—Alterations in molecular elements derived from the CXC chemokine receptor 4 (CXCR4)/stromal-derived factor 1 (SDF-1) cytokine system have been found to strongly correlate with neoplastic progression leading to metastasis in a number of tumors, including osteosarcoma. Excluding hematologic malignancies, chondrosarcoma of bone is the most common primary malignant tumor of bone in adults in the United States. Like osteosarcoma, chondrosarcoma preferentially metastasizes to lung, bone, and very rarely to regional lymph nodes. However, the role of the signal pathway(s) driving neoplastic progression in chondrosarcoma has not yet been clearly elucidated. Objective.—To test whether CXCR4 was detectable in chondrosarcoma and whether CXCR4 expression levels correlated with chondrosarcoma grade. Design.—Twenty-two chondrosarcoma samples banked at our institution between 2001 and 2006 were retrieved for study. By using invasive ductal carcinoma of the breast and osteosarcoma as the positive controls, immunohistochemistry was performed on paraffin-embedded tissue sections and the intensity of the tumor cells was analyzed by morphometric techniques. Results.—All chondrosarcoma cases (22 of 22) were immunoreactive for CXCR4. However, the staining intensity of the CXCR4 between the low- and high-grade groups was significantly different. There was a higher staining intensity in high-grade chondrosarcoma cells (P &lt; .001). Conclusion.—CXCR4 is expressed in chondrosarcomas. CXCR4 expression levels were higher in high-grade chondrosarcoma cells than in low-grade specimens. A larger number of cases will be required to confirm these results and expand the observation, but preliminary data would argue for CXCR4 immunohistochemistry as a potential marker for biologic aggressiveness in chondrosarcoma of bone.


Author(s):  
Ian M. Thompson

Overview: Prostate cancer is a ubiquitous disease, affecting as many as two-thirds of men in their 60s. Through widespread prostate-specific antigen (PSA) testing, increasing rates of prostate biopsy, and increased sampling of the prostate, a larger fraction of low-grade, low-volume tumors have been detected, consistent with tumors often found at autopsy. These tumors have historically been treated in a manner similar to that used for higher-grade tumors but, more recently, it has become evident that with a plan of active surveillance that reserves treatment for only those patients whose tumors show evidence of progression, very high disease-specific survival can be achieved. Unfortunately, the frequency of recommendation of an active surveillance strategy in the United States is low. An alternative strategy to improve prostate cancer detection is through selected biopsy of those men who are at greater risk of harboring high-grade, potentially lethal cancer. This strategy is currently possible through the use of risk assessment tools such as the Prostate Cancer Prevention Trial Risk Calculator ( www.prostate.cancer.risk.calculator.com ) as well as others. These tools can predict with considerable accuracy a man's risk of low-grade and high-grade cancer, allowing informed decision making for the patient with a goal of detection of high-risk disease. Ultimately, other biomarkers including PCA3, TMPRSS2:ERG, and [-2]proPSA will likely aid in discriminating these two types of cancer before biopsy.


2019 ◽  
Vol 3 (3) ◽  
Author(s):  
Alexandra Thomas ◽  
Anthony Rhoads ◽  
Elizabeth Pinkerton ◽  
Mary C Schroeder ◽  
Kristin M Conway ◽  
...  

Abstract Background Although recent findings suggest that de novo stage IV breast cancer is increasing in premenopausal women in the United States, contemporary incidence and survival data are lacking for stage I–III cancer. Methods Women aged 20–29 (n = 3826), 30–39 (n = 34 585), and 40–49 (n = 126 552) years who were diagnosed with stage I–III breast cancer from 2000 to 2015 were identified from the Surveillance, Epidemiology, and End Results 18 registries database. Age-adjusted, average annual percentage changes in incidence and 5- and 10-year Kaplan-Meier survival curves were estimated by race and ethnicity, stage, and hormone receptor (HR) status and grade (low to well and moderately differentiated; high to poorly and undifferentiated) for each age decade. Results The average annual percentage change in incidence was positive for each age decade and was highest among women aged 20–29 years. Increased incidence was driven largely by HR+ cancer, particularly HR+ low-grade cancer in women aged 20–29 and 40–49 years. By 2015, incidence of HR+ low- and high-grade cancer each independently exceeded incidence of HR− cancer in each age decade. Survival for HR+ low- and high-grade cancer decreased with decreasing age; survival for HR− cancer was similar across age decades. Among all women aged 20–29 years, 10-year survival for HR+ high-grade cancer was lower than that for HR+ low-grade or HR− cancer. Among women aged 20–29 years with stage I cancer, 10-year survival was lowest for HR+ high-grade cancer. Conclusions HR+ breast cancer is increasing in incidence among premenopausal women, and HR+ high-grade cancer was associated with reduced survival among women aged 20–29 years. Our findings can help guide further evaluation of preventive, diagnostic, and therapeutic strategies for breast cancer among premenopausal women.


Author(s):  
Martee L. Hensley

Overview: Uterine sarcomas are rare cancers, they comprise only 5% of all uterine malignancies. There are about 2,000 cases of uterine sarcoma diagnosed annually in the United States. Uterine sarcomas may be categorized as either favorable-risk, low-grade malignancies with a relatively good prognosis or as poor-risk, high-grade cancers that carry a high risk for tumor recurrence and disease progression. Expert histologic review is critical for appropriate diagnosis and management. Uterine sarcoma histologies considered to carry a more favorable prognosis include low-grade endometrial stromal sarcomas and adenosarcomas. The high-grade sarcomas include high-grade leiomyosarcomas, high-grade undifferentiated endometrial sarcomas, and adenosarcomas with sarcomatous overgrowth. The favorable histology, low-grade uterine sarcomas may be cured with surgical resection of uterus-limited disease. These tumors are often hormone-sensitive, and treatment with hormonal therapies may be efficacious for patients with advanced, unresectable disease. High-grade uterine leiomyosarcomas and undifferentiated endometrial sarcomas carry a high risk for recurrence, even after complete resection of uterus-limited disease. No adjuvant intervention has been shown to improve survival outcomes. Advanced, metastatic disease is generally treated with systemic cytotoxic therapies, which may result in objective response but is not curative. Selected patients with isolated metastatic disease and a long disease-free interval may benefit from metastatectomy.


2002 ◽  
Vol 41 (3) ◽  
pp. 250-259 ◽  
Author(s):  
R H W Simpson ◽  
J S Reis-Filho ◽  
E M Pereira ◽  
A C Ribeiro ◽  
A Abdulkadir

2016 ◽  
Vol 61 (1) ◽  
pp. 71-76 ◽  
Author(s):  
Rosario Granados ◽  
Joanny A. Duarte ◽  
Teresa Corrales ◽  
Encarnación Camarmo ◽  
Paloma Bajo

The Paris System (TPS) for reporting urinary cytology attempts to unify the terminology in this field. Objectives: To analyze the impact of adopting TPS by measuring nomenclature agreement and cytohistological correlation. Materials and Methods: Voided urine liquid-based cytology samples corresponding to 149 biopsy-proven cases (76 high-grade carcinomas, 40 low-grade carcinomas, and 33 benign lesions), were reclassified by the same pathologist using TPS. Diagnostic agreement and sensitivity for both nomenclature systems was measured. Results: When using TPS, the rate of atypical samples increased 8 times (from 3 to 24.2%) in benign cases, 10 times (from 2.5 to 25%) in low-grade carcinomas, and 2.4 times (from 6.6 to 15.8%) in high-grade carcinomas. The false-positive rate (abnormal cytology in negative or low-grade carcinoma cases) increased from 11 to 34.2%. Sensitivity was higher (63 vs. 49%) with TPS at the expense of a lower specificity (73 vs. 91%). The agreement between both nomenclatures was moderate for negative and high-grade carcinoma cases (k = 0.42 and 0.56, respectively) and weak for low-grade tumors (k = 0.35). Conclusions: Adopting TPS for reporting urine cytology results in a considerable increase in atypical diagnoses, improving sensitivity but lowering specificity. Appropriate management recommendations for patients with an atypical cytological diagnosis are required.


2009 ◽  
Vol 27 (15_suppl) ◽  
pp. e16528-e16528 ◽  
Author(s):  
M. Schlumbrecht ◽  
D. Urbauer ◽  
D. Gershenson ◽  
R. Broaddus

e16528 Background: Obesity is an epidemic public health problem in the United States. In gynecologic oncology, obesity is an established risk factor for endometrial cancer. However, its role in the pathogenesis and survival in ovarian cancer is debated. Recent studies have attempted to elucidate a possible relationship, but variations in design and heterogeneity in patient characteristics make it difficult to draw definitive conclusions. The purpose of our study was to determine if body mass index at the time of treatment initiation for high grade serous ovarian carcinoma has an effect on patient outcome. Methods: Nine-hundred four patients treated for ovarian cancer at M.D. Anderson Cancer Center were identified between 2002 and 2007. Patients were excluded for low grade or non-serous histology, neoadjuvant treatment, or if presenting with recurrent disease. Clinicopathologic data were extracted by retrospective chart review. Patients were stratified by body mass index (BMI) as normal (BMI<25), overweight (BMI 25-<30), or obese (BMI>30). All were treated with primary cytoreduction and standard platinum/taxane chemotherapy. Chemotherapy was dosed using adjusted body weights. Outcomes included time to recurrence, overall survival, success of surgical debulking, and chemotherapeutic toxicities. Statistical analysis was performed using Fisher's exact test, Wilcoxon test, and Kaplan-Meier estimates. Results: A total of 127 patients were included for analysis. Patients were followed for a mean of 37 months (range 3–86 months). Twenty-one patients were obese (16.5%), and 35 were overweight (27.5%). Diabetes was more prevalent in the obese cohort (p = 0.0038). There was a trend towards greater likelihood of suboptimal debulking in obese patients, but this did not reach statistical significance (p = 0.06). BMI had no effect on recurrence-free survival (HR 0.69 [CI 0.39–1.23], p = 0.21) or overall survival (HR 0.95 [CI 0.68–2.43], p = 0.91). There was no difference in chemotherapy side effects or chemoresistance across BMI strata. Conclusions: Body mass index has no effect on survival in women with high grade serous ovarian cancer. Effectively managing comorbidities and ensuring adequate chemotherapy dosing in the obese patient is crucial for optimizing outcome. No significant financial relationships to disclose.


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