scholarly journals Systemic chemotherapy in appendiceal adenocarcinomas with peritoneal metastases. is it worth it?

2019 ◽  
Vol 2 (1) ◽  
pp. 3-9
Author(s):  
Francisco J Morera Ocón ◽  
Bruno Camps Vilata
Keyword(s):  
Mucinous Adenocarcinoma ◽  
Systemic Chemotherapy ◽  
Hepatic Metastases ◽  
Progression Free Survival ◽  
Standard Of Care ◽  
Peritoneal Metastases ◽  
Low Grade ◽  
High Grade ◽  
Grade Group ◽  
Histologic Subtype

Introduction: The role of systemic chemotherapy (SC) in the management of metastatic appendiceal adenocarcinomas is not defined.Methods: We perform an observational study of patients treated of peritoneal metastases from appendiceal neoplasm between June 2004 and December 2017. They were referred to our hospital for CRS HIPEC. Systemic Chemotherapy regimens were decided by the referring oncologist. We analyze PCI, CC, and use of SC. Overall survival and progression free survival were compared attending to histologic subtype (high- and low-grade adenocarcinomas) and the use of SC.Results: 60 patients were included, 26 were male, median age 63 years (26-81). Median follow-up of 38 months [2-155]. The predominant histologic subtype was mucinous adenocarcinoma (58/60), 1 was colonic type, and 1 adenocarcinoid with signet ring cells. Four patients had extraperitoneal metastases, 2 hepatic metastases (1 with high-grade mucinous adenocarcinoma and 1 with colonic adenocarcinoma), and 2 in the low-grade group developed parenchymal lung disease. Three patients died in the postoperative period. Systemic chemotherapy was administered in 26 patients/57. No difference were observed in the OS and PFS regardless the use of SC in the high- and in the lo-grade groups. Conclusions: The use of SC in low-grade mucinous adenocarcinoma subtype is not supported by our results, and there is no literature date supporting it neither. Palliative SC in high-grade patients deserves of clinical trials for been accepted as standard of care, when benefits are not clearly established by evidence and toxicities are not negligible.

scholarly journals SYSTEMIC CHEMOTHERAPY IN APPENDICEAL ADENOCARCINOMAS WITH PERITONEAL METASTASES. IS IT WORTH IT?

2019 ◽  
Vol 2 (1) ◽  
pp. 3-9 ◽  
Author(s):  
Francisco J Morera Ocón ◽  
Bruno Camps Vilata
Keyword(s):  
Mucinous Adenocarcinoma ◽  
Systemic Chemotherapy ◽  
Hepatic Metastases ◽  
Progression Free Survival ◽  
Standard Of Care ◽  
Peritoneal Metastases ◽  
Low Grade ◽  
High Grade ◽  
Grade Group ◽  
Histologic Subtype

Introduction: The role of systemic chemotherapy (SC) in the management of metastatic appendiceal adenocarcinomas is not defined.Methods: We perform an observational study of patients treated of peritoneal metastases from appendiceal neoplasm between June 2004 and December 2017. They were referred to our hospital for CRS HIPEC. Systemic Chemotherapy regimens were decided by the referring oncologist. We analyze PCI, CC, and use of SC. Overall survival and progression free survival were compared attending to histologic subtype (high- and low-grade adenocarcinomas) and the use of SC.Results: 60 patients were included, 26 were male, median age 63 years (26-81). Median follow-up of 38 months [2-155]. The predominant histologic subtype was mucinous adenocarcinoma (58/60), 1 was colonic type, and 1 adenocarcinoid with signet ring cells. Four patients had extraperitoneal metastases, 2 hepatic metastases (1 with high-grade mucinous adenocarcinoma and 1 with colonic adenocarcinoma), and 2 in the low-grade group developed parenchymal lung disease. Three patients died in the postoperative period. Systemic chemotherapy was administered in 26 patients/57. No difference were observed in the OS and PFS regardless the use of SC in the high- and in the lo-grade groups. Conclusions: The use of SC in low-grade mucinous adenocarcinoma subtype is not supported by our results, and there is no literature date supporting it neither. Palliative SC in high-grade patients deserves of clinical trials for been accepted as standard of care, when benefits are not clearly established by evidence and toxicities are not negligible.

Outcomes of a phase II study of intraperitoneal paclitaxel plus systemic capecitabine and oxaliplatin (XELOX) for gastric cancer with peritoneal metastases.

2021 ◽  
Vol 39 (3_suppl) ◽  
pp. 165-165
Author(s):  
Daryl Chia ◽  
Raghav Sundar ◽  
Guo Wei Kim ◽  
Jiajun Ang ◽  
Jeffrey Lum ◽  
...  
Keyword(s):  
Gastric Cancer ◽  
Systemic Chemotherapy ◽  
Performance Status ◽  
Progression Free Survival ◽  
Standard Of Care ◽  
Peritoneal Metastases ◽  
Conversion Surgery ◽  
Current Standard ◽  
Peritoneal Disease ◽  
Phase 2 Trial

165 Background: The addition of intraperitoneal (IP) paclitaxel (PTX) to systemic chemotherapy comprising taxane/fluoropyrimidine doublet has shown promising results for patients with gastric cancer (GC) and peritoneal metastases (PM). However, this has not been studied in combination with platinum/fluoropyrimidine doublet which is the current standard-of-care for metastatic GC. We conducted a prospective phase 2 trial of IP PTX with capecitabine and oxaliplatin (XELOX) in patients with GCPM. Methods: The trial enrolled 44 patients with GCPM who received treatment comprising IP PTX (40mg/m2 on day 1,8), PO capecitabine (1000mg/m2 twice daily from day 1-14) and IV oxaliplatin (100mg/m2 on day 1) in 21-day cycles. Patients with synchronous GCPM were eligible for conversion surgery comprising radical gastrectomy if they had good response after chemotherapy, negative cytology on 2 consecutive peritoneal fluid assessments, no extraperitoneal metastasis and no peritoneal disease during surgery. The primary endpoint was overall survival and secondary endpoints were progression-free survival and safety. Outcomes from the trial were also compared with a retrospective cohort of 39 patients with GCPM who received identical systemic chemotherapy (SC) comprising platinum/fluoropyrimidine agents alone. Results: The median OS for the IP and SC groups was 14.6 and 10.6 months (HR 0.44; 95% CI, 0.26-0.74; P=0.002). The 1-year OS was 67.8% in the IP group and 32.3% in the SC group (Logrank p<0.001). The median PFS for the IP and SC group was 9.5 and 4.4 months respectively (HR 0.39; 95% CI, 0.25-0.66; P<0.001). Patients in the SC group were younger (IP vs. SC, 61 vs. 56 years, p=0.021) and had better baseline performance status (ECOG 0, IP vs. SC, 47.7% vs. 76.9%, p=0.007) compared to the IP cohort. In the IP group, conversion surgery was performed in 36.1% (13/36) of patients, with a median OS of 24.2 (95%CI 13.1 – 35.3) months and 1-year OS of 84.6%. Wound-related complications requiring the port to be explanted or re-sited occurred in 9% (4/44) of patients. Conclusions: IP PTX with XELOX is a promising treatment option for GCPM patients. For patients with good response, conversion surgery was feasible with favourable outcomes. Clinical trial information: NCT01739894. [Table: see text]

scholarly journals Current FDA-Approved Therapies for High-Grade Malignant Gliomas

Biomedicines ◽  
2021 ◽  
Vol 9 (3) ◽  
pp. 324
Author(s):  
Jacob P. Fisher ◽  
David C. Adamson
Keyword(s):  
Controlled Trial ◽  
Malignant Gliomas ◽  
Progression Free Survival ◽  
Standard Of Care ◽  
High Grade ◽  
Tumor Treatment ◽  
Free Survival ◽  
Randomized Controlled ◽  
Significant Survival ◽  
One Year

The standard of care (SOC) for high-grade gliomas (HGG) is maximally safe surgical resection, followed by concurrent radiation therapy (RT) and temozolomide (TMZ) for 6 weeks, then adjuvant TMZ for 6 months. Before this SOC was established, glioblastoma (GBM) patients typically lived for less than one year after diagnosis, and no adjuvant chemotherapy had demonstrated significant survival benefits compared with radiation alone. In 2005, the Stupp et al. randomized controlled trial (RCT) on newly diagnosed GBM patients concluded that RT plus TMZ compared to RT alone significantly improved overall survival (OS) (14.6 vs. 12.1 months) and progression-free survival (PFS) at 6 months (PFS6) (53.9% vs. 36.4%). Outside of TMZ, there are four drugs and one device FDA-approved for the treatment of HGGs: lomustine, intravenous carmustine, carmustine wafer implants, bevacizumab (BVZ), and tumor treatment fields (TTFields). These treatments are now mainly used to treat recurrent HGGs and symptoms. TTFields is the only treatment that has been shown to improve OS (20.5 vs. 15.6 months) and PFS6 (56% vs. 37%) in comparison to the current SOC. TTFields is the newest addition to this list of FDA-approved treatments, but has not been universally accepted yet as part of SOC.

scholarly journals Impact of Pathological Stratification on the Clinical Outcomes of Advanced Well-Differentiated/Dedifferentiated Liposarcoma Treated with Trabectedin

Cancers ◽  
2021 ◽  
Vol 13 (6) ◽  
pp. 1453
Author(s):  
Chiara Fabbroni ◽  
Giovanni Fucà ◽  
Francesca Ligorio ◽  
Elena Fumagalli ◽  
Marta Barisella ◽  
...  
Keyword(s):  
Clinical Outcomes ◽  
Proportional Hazards ◽  
Case Series ◽  
Progression Free Survival ◽  
Cox Proportional Hazards ◽  
Low Grade ◽  
High Grade ◽  
Retrospective Case ◽  
Well Differentiated ◽  
The Impact

Background. We previously showed that grading can prognosticate the outcome of retroperitoneal liposarcoma (LPS). In the present study, we aimed to explore the impact of pathological stratification using grading on the clinical outcomes of patients with advanced well-differentiated LPS (WDLPS) and dedifferentiated LPS (DDLPS) treated with trabectedin. Patients: We included patients with advanced WDLPS and DDLPS treated with trabectedin at the Fondazione IRCCS Istituto Nazionale dei Tumori between April 2003 and November 2019. Tumors were categorized in WDLPS, low-grade DDLPS, and high-grade DDLPS according to the 2020 WHO classification. Patients were divided in two cohorts: Low-grade (WDLPS/low-grade DDLPS) and high-grade (high-grade DDLPS). Results: A total of 49 patients were included: 17 (35%) in the low-grade cohort and 32 (65%) in the high-grade cohort. Response rate was 47% in the low-grade cohort versus 9.4% in the high-grade cohort (logistic regression p = 0.006). Median progression-free survival (PFS) was 13.7 months in the low-grade cohort and 3.2 months in the high-grade cohort. Grading was confirmed as an independent predictor of PFS in the Cox proportional-hazards regression multivariable model (adjusted hazard ratio low-grade vs. high-grade: 0.45, 95% confidence interval: 0.22–0.94; adjusted p = 0.035). Conclusions: In this retrospective case series, sensitivity to trabectedin was higher in WDLPS/low-grade DDLPS than in high-grade DDLPS. If confirmed in larger series, grading could represent an effective tool to personalize the treatment with trabectedin in patients with advanced LPS.

PATH-41. POLYMORPHOUS LOW-GRADE NEUROEPITHELIAL TUMOR OF THE YOUNG WITH FGFR3-TACC3 FUSION MIMICKING HIGH-GRADE GLIOMA: CASE REPORT AND SERIES OF HIGH-GRADE CORRELATES

2021 ◽  
Vol 23 (Supplement_6) ◽  
pp. vi124-vi124
Author(s):  
Danielle Golub ◽  
Peter C Pan ◽  
Benjamin Liechty ◽  
Cheyanne Slocum ◽  
Tejus Bale ◽  
...  
Keyword(s):  
Progression Free Survival ◽  
High Grade Glioma ◽  
Proliferation Index ◽  
Molecular Profile ◽  
Low Grade ◽  
High Grade ◽  
Molecular Features ◽  
Tert Promoter ◽  
Tert Promoter Mutations ◽  
Promoter Mutations

Abstract BACKGROUND Polymorphous low-grade neuroepithelial tumor of the young (PLNTY) is a recently-described entity that can occasionally histologically and molecularly mimic high-grade glioma. The histologic and molecular features that predict aggressive behavior in FGFR3-TACC3 altered tumors are unclear. CASES We present a rare case of an indolent neuroepithelial neoplasm in a 59-year-old female with imaging initially suggestive of high-grade glioma and analyze common molecular features between this case and a series of high-grade gliomas. After total resection, pathology of the case patient revealed predominantly low-grade cytomorphology, abundant microcalcifications, unusual neovascularization, and a low proliferation index. The lesion was diffusely CD34 immunoreactive and harbored both an FGFR3-TACC3 fusion and a TERT promoter mutation. Based on the overall histologic and molecular profile, a diagnosis of PLNTY was favored. The patient was thereafter observed without adjuvant therapy with no evidence of progression at 15-month follow-up. In contrast, a series of eight adult patients with glioblastomas harboring FGFR3-TACC3 fusions and correspondingly aggressive clinical courses are also presented. Common molecular findings included IDH-wildtype status, absence of 1p19q codeletion, and CDKN2A loss. TERT promoter mutations and lack of MGMT promoter methylation were also frequently observed. These patients demonstrated a median 15-month overall survival and a 6-month progression-free survival. CONCLUSIONS PLNTY is a rare low-grade entity that can display characteristics of high-grade glioma, particularly in adults. The potential for a unique entity mimicking PLNTY which may act as a precursor lesion for a more malignant phenotype should be considered in cases with FGFR3-TACC3 fusions and other high-grade features.

scholarly journals Everolimus and Bevacizumab in the Management of Recurrent, Progressive Intracranial NF2 Mutated Meningioma

2019 ◽  
Vol 12 (1) ◽  
pp. 126-130 ◽  
Author(s):  
Vinay Mathew Thomas ◽  
Poorva Bindal ◽  
James J. Vredenburgh
Keyword(s):  
Survival Time ◽  
Progression Free Survival ◽  
Genetic Alteration ◽  
Cns Tumors ◽  
Good Effect ◽  
Genomic Sequencing ◽  
Low Grade ◽  
High Grade ◽  
Free Survival

Meningiomas are primary CNS tumors that arise from the arachnoid layer of the meninges. Genomic sequencing has revealed that NF2 mutations are the most common genetic alteration seen in meningiomas. Meningiomas although usually low grade, can sometimes progress to high grade. A patient who had several recurrences of meningiomas since childhood presented with recurrent headaches. Imaging showed that he had another recurrence of a meningioma. He underwent surgery for resection of the meningioma and histopathology showed NF2 mutation. He was started on everolimus and bevacizumab with good effect. Studies have shown that NF-2 mutated meningiomas have a good response to everolimus and bevacizumab with increased progression-free survival time and progression-free survival time at 6 months.

scholarly journals Concomitant intraperitoneal and systemic chemotherapy for extensive peritoneal metastases of colorectal origin: protocol of the multicentre, open-label, phase I, dose-escalation INTERACT trial

BMJ Open ◽  
2019 ◽  
Vol 9 (12) ◽  
pp. e034508 ◽  
Author(s):  
Nadine Leonie de Boer ◽  
Alexandra R M Brandt-Kerkhof ◽  
Eva V E Madsen ◽  
Marjolein Diepeveen ◽  
Esther van Meerten ◽  
...  
Keyword(s):  
Colorectal Cancer ◽  
Phase I ◽  
Dose Escalation ◽  
Intraperitoneal Chemotherapy ◽  
Systemic Chemotherapy ◽  
Standard Of Care ◽  
Peritoneal Metastases ◽  
Access Port ◽  
Abdominal Disease ◽  
Colorectal Origin

IntroductionCytoreductive surgery and hyperthermic intraperitoneal chemotherapy (CRS-HIPEC) has become standard of care for patients with peritoneal metastases of colorectal origin with a low/moderate abdominal disease load. In case of a peritoneal cancer index (PCI) score >20, CRS-HIPEC is not considered to be beneficial. Patients with a PCI >20 are currently offered palliative systemic chemotherapy. Previous studies have shown that systemic chemotherapy is less effective against peritoneal metastases than it is against haematogenous spread of colorectal cancer. It is suggested that patients with peritoneal metastases may benefit from the addition of intraperitoneal chemotherapy to systemic chemotherapy. Aim of this study is to establish the maximum tolerated dose of intraperitoneal irinotecan, added to standard of care systemic therapy for colorectal cancer. Secondary endpoints are to determine the safety and feasibility of this treatment and to establish the pharmacokinetic profile of intraperitoneally administered irinotecan.Methods and analysisThis phase I, ‘3+3’ dose-escalation, study is performed in two Dutch tertiary referral centres. The study population consists of adult patients with extensive peritoneal metastases of colorectal origin who have a good performance status and no extra-abdominal metastases. According to standard work-up for CRS-HIPEC, patients will undergo a diagnostic laparoscopy to score the PCI. In case of a PCI >20, a peritoneal access port will be placed in the abdomen of the patient. Through this port we will administer intraperitoneal irinotecan, in combination with standard systemic treatment consisting of 5-fluorouracil/leucovorin with oxaliplatin and the targeted agent bevacizumab. Therapy consists of a maximum of 12 cycles 2-weekly.Ethics and disseminationThis study protocol is approved by a research medical ethics committee (Rotterdam, Netherlands) and the Dutch Competent Authority (CCMO, The Hague, Netherlands). The results of this trial will be submitted for publication in a peer-reviewed scientific journal.Trail registration numberNL6988 and NL2018-000479-33; Pre-results.

Early Evidence of Anti-Lymphoma Activity of the Cyclin Dependent Kinase Inhibitor Dinaciclib (SCH 727965) In Heavily Pre-Treated Low Grade Lymphoma and Diffuse Large Cell Lymphoma Patients

Blood ◽  
2010 ◽  
Vol 116 (21) ◽  
pp. 3966-3966 ◽  
Author(s):  
Robert A Baiocchi ◽  
Joseph M. Flynn ◽  
Jeffrey A. Jones ◽  
Kristie A. Blum ◽  
Craig C. Hofmeister ◽  
...  
Keyword(s):  
Cell Lymphoma ◽  
Median Number ◽  
Low Grade ◽  
High Grade ◽  
Cytokine Release ◽  
Cytokine Release Syndrome ◽  
Employment Equity ◽  
Grade Group ◽  
Tumor Mass ◽  
Equity Ownership

Abstract Abstract 3966 Background: Dinaciclib (SCH 727965) is a potent and selective inhibitor of the cyclin dependent kinases CDKs 1, 2, 5, and 9, with in vitro activity against lymphoma. Methods: A phase 1 trial of dinaciclib administered by 2-hour i.v. infusion on days 1, 8 and 15 of a 28-day cycle was undertaken in solid tumor patients with 12 mg/m2 established as the recommended phase 2 dose (RPTD). The RPTD was explored in expansion cohorts of up to 10 patients each with low grade lymphomas (primarily follicular lymphoma (FL)), and intermediate/high grade lymphomas (diffuse large B-cell lymphoma (DLBCL)). Sixteen patients have been treated with dinaciclib, including 7 with FL, 1 with mantle cell lymphoma (MCL) and 1 with marginal zone lymphoma (MZL) in the low grade group and 7 with DLBCL in the intermediate/high grade group. Patient median age was 66 (range 43–82) and the median number of prior therapies was 2.5 (range 1–8). All patients received prior rituximab and 4 patients had previously received flavopiridol. All patients were treated with dinaciclib at the 12 mg/m2 dose. Dose de-escalation to 10 mg/m2 was required in 2 patients during treatment. The median number of cycles administered was 3 (range 1–9) with 3 patients continuing treatment for 3 cycles, 4 for 6 cycles, and 1 for 9 cycles. Of the 16 lymphoma patients treated, 2 remain on treatment. Response: Responses were assessed using the revised Cheson criteria for lymphoma (J Clin Oncol 25: 579-86, 2007). One partial response in a patient with DLBCL was observed with an 85% decrease in tumor mass. This patient, previously treated with R-CHOP from May-August 2008 and R-ICE from December 2008-March 2009, was able to continue on to autologous stem cell transplantation for potential curative salvage. Activity not meeting criteria for partial response was seen in 2 patients with FL (decreased tumor mass of 27% and 39%, respectively) and one patient with MCL (8% decrease in tumor mass). Toxicity: Treatment-related adverse events occurring in ≥ 25% of pts included anemia, cytokine release syndrome, nausea, vomiting, diarrhea, fatigue, leucopenia, lymphopenia, neutropenia, and thrombocytopenia. The most common CTCAE v3.0 treatment-related grade 3 and 4 toxicities, occurring in 3 or more patients, were leukopenia, lymphopenia, and neutropenia. Cytokine release syndrome was observed in 4 patients, was manageable with dexamethasone and did not prevent continued treatment on protocol. Conclusion: Dinaciclib demonstrates clinical responses in heavily pre-treated lymphoma patients supporting further study in lymphoma and other hematologic malignancies. Updated information with longer follow-up will be presented. Disclosures: Off Label Use: SCH 727965 is an investigational drug. Small:Merck & Co.: Employment, Equity Ownership. Statkevich:Merck & Co.: Employment, Equity Ownership. Bannerji:Merck & Co: Employment, Equity Ownership.

Is there a role for neoadjuvant chemotherapy in the management of non-carcinoid epithelial neoplasms of the appendix?

2007 ◽  
Vol 25 (18_suppl) ◽  
pp. 15115-15115
Author(s):  
M. H. Katz ◽  
P. F. Mansfield ◽  
C. Eng ◽  
R. A. Wolff ◽  
P. Diaz ◽  
...  
Keyword(s):  
Neoadjuvant Chemotherapy ◽  
Standard Of Care ◽  
Low Grade ◽  
High Grade ◽  
Appendiceal Neoplasms ◽  
Tertiary Center ◽  
Standardized Treatment ◽  
Crs And Hipec ◽  
Epithelial Neoplasms

15115 Background: Cytoreductive surgery and hyperthermic intraperitoneal chemotherapy (CRS and HIPEC) are considered the standard of care for patients with pseudomyxoma peritonei (PMP) and carcinomatosis of appendiceal origin. The role of neoadjuvant chemotherapy (CTX) in the management of these patients is unknown. Methods: Retrospective analysis of all patients evaluated for the treatment of appendiceal epithelial neoplasms at a tertiary center between 1992 and August 2006. All diagnoses were confirmed pathologically and classified by a single group of pathologists. Patients with carcinoid tumors or metastases to the appendix were excluded. Tumor histology, stage, peritoneal-based disease, and the potential for complete cytoreduction dictated treatment. Results: 250 consecutive patients were evaluated, 140 of whom had low grade disease. 114 underwent CRS and HIPEC. Median follow-up was 24 mos from referral. Average time to referral was 13 mos after diagnosis (range 0–243); 85% had undergone prior surgical therapy (median 1.3 operations, range 1–4); 22% had previous CTX consisting of many different regimens. 5- and 10-year survival for patients with low grade tumors treated with CRS and HIPEC alone (n=80) were 84% and 68%, respectively. 21 patients with low grade tumors had CTX prior to CRS and HIPEC. There was no effect on overall survival (p = 0.61). 5-and 10-year survival of 39 patients with low grade histology who did not receive CRS and HIPEC was 55% and 30%, respectively (p = 0.009). 83 patients with intermediate and high-grade disease who received CTX but not CRS and HIPEC had a 5- year survival of 27%. 5-year survival for patients with intermediate or high grade disease who underwent CRS and HIPEC (n=13) was 67%. Conclusions: Patients with peritoneal-based disease from non-carcinoid epithelial neoplasms of the appendix who undergo CRS and HIPEC have a more favorable survival. Currently there is no survival advantage to the use of CTX before CRS and HIPEC for low grade appendiceal neoplasms. The role of neoadjuvant CTX and biologic agents for patients with high grade neoplasms needs to be determined. Early referral to a peritoneal malignancy center will help standardized treatment for these patients. No significant financial relationships to disclose.

Retrospective study of non-mucinous appendiceal adenocarcinomas: Role of systemic chemotherapy and cytoreductive surgery.

2016 ◽  
Vol 34 (4_suppl) ◽  
pp. 263-263
Author(s):  
Marc Isamu Uemura ◽  
Wei Qiao ◽  
Keith F. Fournier ◽  
Jeffrey Morris ◽  
Paul F. Mansfield ◽  
...  
Keyword(s):  
Cytoreductive Surgery ◽  
Systemic Chemotherapy ◽  
Stage Iv ◽  
Stage Ii ◽  
Stage Iii ◽  
Rank Test ◽  
Low Grade ◽  
Histologic Subtype ◽  
Well Differentiated ◽  
Quantitative Response

263 Background: The majority of studies evaluating appendiceal epithelial neoplasms have focused on those with mucinous histology. Few studies have reported on non-mucinous appendiceal adenocarcinomas. We performed the largest single-center study to investigate this histologic subtype, in order to describe the natural history and impact of both cytoreductive surgery (CRS) and systemic chemotherapy. Methods: We retrospectively reviewed 172 pts with non-mucinous appendiceal adenocarcinoma evaluated at the UT-MD Anderson Cancer Center between 1990 and 2015. Patient demographics, tumor characteristics, therapy received, and outcomes were recorded. Response assessment was semi-quantitative (response vs. no response) according to the treating physician. Overall survival (OS) and time to progression (TTP) were calculated using Kaplan Meier product-limit method and survival rates compared using the log rank test. Results: Median age at diagnosis was 52.9 yrs (M:F 1:1). Most pts presented with advanced stage: stage I (1.7%), stage II (32.5%), stage III (14.5%), and stage IV (51.2%). No patient had well-differentiated histology. 56% had moderate and 44% poor histology. Median OS by stage was 90.9m [95% CI: 70.8 to 172.9] for stage II, 52.1m [95% CI: 28.9 to NA] for stage III and 28.3m [95% CI: 22.9 to 31.9] for stage IV, (p < 0.0001). In pts with metastatic disease (n = 128) CRS was attempted in 20 (15.6%) and was complete (CCS 0/1) in 12. The median OS for pts achieving complete CRS was 48.6m. Systemic chemotherapy was administered to 92% (118/128) of metastatic pts. The median TTP was 9.4m [95% CI: 8.0 to 11.5] and semi-quantitative response rate was 54%. The majority of pts received either oxaliplatin-based, 57%, or irinotecan-based, 23%, first-line chemotherapy regimens. No statistical difference in TTP (p = 0.9) or OS (p = 0.07) between different chemotherapies was seen. Conclusions: In contrast to mucinous appendiceal neoplasms, non-mucinous appendiceal adenocarcinomas rarely present with low-grade (well-differentiated) histology. Treatment approaches appear more akin to colorectal cancer with most metastatic pts undergoing systemic chemotherapy and a minority undergoing CRS.

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