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The Oncologist Dec 2023Appendiceal cancer is a rare, orphan disease with no therapies currently approved by the FDA for its treatment. Given the limited data regarding drug efficacy, these...
Appendiceal cancer is a rare, orphan disease with no therapies currently approved by the FDA for its treatment. Given the limited data regarding drug efficacy, these tumors have historically been treated with chemotherapy designed for colon cancer. However, an overwhelming body of molecular data has demonstrated that appendiceal adenocarcinoma is a distinct entity with key molecular differences from colon cancer, notably rare APC mutation. Recognizing that APC loss-of-function is thought to contribute to taxane resistance and that taxanes are effective in the treatment of other gastrointestinal tumors, including gastric, esophageal, and small bowel adenocarcinoma, we completed a single-center retrospective study to assess efficacy. In a cohort of 13 patients with metastatic appendiceal adenocarcinoma, treated with taxane chemotherapy the median overall survival was 8.8 months. Of 10 evaluable patients, we observed 3 responses, 4 patients with stable disease, and 3 with progression (30% response rate, 70% disease control rate). The results of this study showing activity of taxane-based chemotherapy in appendiceal adenocarcinoma support further clinical investigation of taxane therapy in this orphan disease.
Topics: Humans; Retrospective Studies; Rare Diseases; Taxoids; Appendiceal Neoplasms; Adenocarcinoma; Colonic Neoplasms; Antineoplastic Combined Chemotherapy Protocols
PubMed: 37738316
DOI: 10.1093/oncolo/oyad263 -
BMC Surgery Sep 2023Non-operative management has been suggested as a therapy for uncomplicated appendicitis. Notwithstanding, the risk of missing an appendiceal tumor must be considered,...
BACKGROUND
Non-operative management has been suggested as a therapy for uncomplicated appendicitis. Notwithstanding, the risk of missing an appendiceal tumor must be considered, being the surgical piece crucial to rule out neoplasms. Therefore, we aim to determine the incidence of appendiceal neoplasms in patients with acute appendicitis, tumor types and the importance of the anatomopathological study of the surgical piece.
STUDY DESIGN
Retrospective study in which we described patients who underwent emergent appendectomy with histopathological findings of appendiceal neoplasms from January 2012 to September 2018. Descriptive analysis included demographic variables, diagnostic methods, and surgical techniques.
RESULTS
2993 patients diagnosed with acute appendicitis who underwent an emergency appendectomy. 64 neoplasms of the appendix were found with an incidence of 2,14%. 67.2% were women, the mean age was 46,4 years (± 19.5). The most frequent appendiceal neoplasms were neuroendocrine tumors (42,2%), followed by appendiceal mucinous neoplasms (35,9%), sessile serrated adenomas (18,8%), and adenocarcinomas (3,1%). In 89,1% of the cases, acute appendicitis was determined by imaging, and 14% of cases were suspected intraoperatively. Appendectomy was performed in 78,1% without additional procedures.
CONCLUSIONS
Appendiceal tumors are rare and must be ruled out in patients with suspected acute appendicitis. The incidence of incidental neoplasms is higher in this study than in the previously reported series. This information must be included in decision-making when considering treatment options for acute appendicitis.
Topics: Humans; Female; Male; Appendiceal Neoplasms; Appendectomy; Incidence; Appendicitis; Retrospective Studies; Adenocarcinoma
PubMed: 37735406
DOI: 10.1186/s12893-023-02183-4 -
Gastroenterology Nursing : the Official...
Review
Topics: Humans; Neoplasms; Neoplasms, Cystic, Mucinous, and Serous
PubMed: 37725797
DOI: 10.1097/SGA.0000000000000778 -
Scandinavian Journal of Surgery : SJS :... Dec 2023Appendectomy has historically been the standard treatment of acute appendicitis, but lately, conservative treatment of uncomplicated acute appendicitis with antibiotics...
BACKGROUND AND AIMS
Appendectomy has historically been the standard treatment of acute appendicitis, but lately, conservative treatment of uncomplicated acute appendicitis with antibiotics has successfully been used in selected patients. Complicated acute appendicitis is often treated conservatively initially, but may benefit from interval appendectomy due to the higher risk of appendiceal malignancy and recurrence. Recommendations for follow-up after conservatively treated appendicitis vary. Furthermore, the risk of underlying malignancy and the necessity of routine interval appendectomy are unclear. This study aims to evaluate follow-up status, recurrence, and underlying appendiceal malignancy in conservatively treated uncomplicated and complicated acute appendicitis.
METHODS
This study included patients with conservatively treated acute appendicitis at Skåne University Hospital, Sweden during 2012-2019. Information on patient demographics at index admission and data on follow-up, recurrence, number of appendectomies after initial conservative treatment, and underlying malignancy were retrieved from medical charts.
RESULTS
The study cohort included 391 patients, 152 with uncomplicated and 239 with complicated acute appendicitis. Median time of study follow-up was 52 months. The recurrence risk was 23 (15.1%) after uncomplicated and 58 (24.3%) after complicated acute appendicitis ( = 0.030). During follow-up, 55 (23%) patients with complicated acute appendicitis underwent appendectomy. Appendiceal malignancies were found in 12 (5%) patients with previous complicated acute appendicitis versus no appendiceal malignancies after uncomplicated acute appendicitis ( = 0.002).
CONCLUSION
The risk of appendiceal malignancy and recurrent appendicitis was significantly higher in patients with complicated acute appendicitis compared with uncomplicated acute appendicitis.
Topics: Humans; Appendicitis; Appendiceal Neoplasms; Anti-Bacterial Agents; Appendectomy; Hospitalization; Acute Disease
PubMed: 37705259
DOI: 10.1177/14574969231190293 -
Ulusal Travma Ve Acil Cerrahi Dergisi =... Sep 2023Appendix neuroendocrine tumors (NETs) are the most common tumors of the appendix and are most often diagnosed incidentally. The aim of this study was to retrospectively...
BACKGROUND
Appendix neuroendocrine tumors (NETs) are the most common tumors of the appendix and are most often diagnosed incidentally. The aim of this study was to retrospectively evaluate appendix NETs diagnosed incidentally in our clinic.
METHODS
Of 8304 patients who underwent appendectomy with the diagnosis of acute appendicitis in Ankara Training and Re-search Hospital, General Surgery Clinic between January 2009 and January 2022, 33 had histopathology results evaluated as appendix NET, and a retrospective analysis was made of these cases. The patients were evaluated in terms of age, gender, tumor infiltration, tumor location, tumor size, surgical margin, tumor World Health Organization grade, surgery performed, lymph node metastasis, Ki67 index, number of mitosis, follow-up time, and survival.
RESULTS
The rate of appendix NET was found to be 0.4%. The 33 cases comprised 15 (45.5%) males and 18 (54.5%) females with a mean age of 35.48 years (range: 16-84 years). Positive surgical margin was determined in 1 (3.03%) case, in which right hemicolectomy was performed. All other cases were followed up after appendectomy. The median follow-up was 89 (7-145) months. No recurrence was observed in any case. Mortality developed during follow-up in one case due to non-tumoral causes.
CONCLUSION
Appendix NETs are generally asymptomatic and appear incidentally after appendectomy due to acute appendicitis. Appendix NETs diagnosed incidentally are generally below 2 cm and have a good prognosis.
Topics: Female; Male; Humans; Adult; Neuroendocrine Tumors; Appendiceal Neoplasms; Retrospective Studies; Appendix; Appendicitis
PubMed: 37681719
DOI: 10.14744/tjtes.2023.78038 -
Cureus Aug 2023Low-grade appendiceal mucinous neoplasm (LAMN) is a relatively rare, non-invasive appendiceal tumor. We experienced a case of LAMN that led to surgery after 12 years of...
Low-grade appendiceal mucinous neoplasm (LAMN) is a relatively rare, non-invasive appendiceal tumor. We experienced a case of LAMN that led to surgery after 12 years of no treatment. Until now, LAMN has been reported to progress more slowly than other tumors, but there have been no reports of long-term follow-up of appendiceal tumors without treatment. Although the tumor had grown over the course of 12 years, there was no mixing or migration of other histological types, and it did not lead to pseudomyxoma peritonei. As this course is considered to be relatively rare, we report it along with a literature review.
PubMed: 37674965
DOI: 10.7759/cureus.43024 -
Scandinavian Journal of Surgery : SJS :... Dec 2023
Randomized Controlled Trial
Topics: Humans; Appendicitis; Appendiceal Neoplasms; Follow-Up Studies; Abscess; Appendectomy; Acute Disease
PubMed: 37655682
DOI: 10.1177/14574969231192128 -
ESMO Open Oct 2023Appendix adenocarcinomas (AAs) are rare tumours that often present late, with a propensity for peritoneal metastases (PMs). This study aimed to evaluate outcomes of AA...
BACKGROUND
Appendix adenocarcinomas (AAs) are rare tumours that often present late, with a propensity for peritoneal metastases (PMs). This study aimed to evaluate outcomes of AA patients undergoing cytoreductive surgery (CRS) with curative intent and determine the role of systemic chemotherapy.
MATERIALS AND METHODS
Data were collected from a prospective database and classified according to World Health Organization (WHO) 2019 classification. Tumour clearance from CRS was described using a completeness of cytoreduction (CC) score ranging from 0 [no residual disease (RD)] to 3 (>2.5 cm RD). Patients with CC0-2 CRS received hyperthermic intraperitoneal chemotherapy (HIPEC). Systemic chemotherapy was categorised as 'prior' (>6 months before), 'neoadjuvant' (<6 months before), 'adjuvant' (<6 months after CC0-1 CRS) or 'palliative' (after CC2-3 CRS). Analyses used Kaplan-Meier and Cox regression methods.
RESULTS
Between January 2005 and August 2021, 216 AA patients were identified for inclusion. Median age was 59 years (21-81 years). CRS/HIPEC was carried out in 182 (84%) patients, of whom 164/182 (76%) had mitomycin C HIPEC. CC0-1 was achieved in 172 (80%) patients. Systemic chemotherapy was given to 97 (45%) patients from the whole cohort and to 37/46 (80%) patients with positive nodes. Median overall survival (OS) was 122 months (95% confidence interval 61-182 months). After multivariate analysis, patients with acellular and lower-grade PM had similar OS to those with localised (M0) disease (P = 0.59 and P = 0.19). For patients with positive nodes, systemic chemotherapy was associated with reduced risk of death compared to no chemotherapy (P < 0.0019).
CONCLUSION
This study identifies AA patients with positive lymph nodes derive the most benefit from systemic chemotherapy. We confirm the prognostic importance of stage and peritoneal grade, with excellent outcomes in patients with acellular mucin and lower-grade PM.
Topics: Humans; Middle Aged; Appendix; Peritoneal Neoplasms; Appendiceal Neoplasms; Prognosis; Adenocarcinoma
PubMed: 37625193
DOI: 10.1016/j.esmoop.2023.101619 -
Annals of Surgery Open : Perspectives... Sep 2022To determine causes of treatment failure of low-grade appendiceal mucinous neoplasms (LAMN). (Review)
Review
OBJECTIVE
To determine causes of treatment failure of low-grade appendiceal mucinous neoplasms (LAMN).
BACKGROUND
For 3 decades, LAMN have been treated by cytoreductive surgery (CRS) and perioperative intraperitoneal chemotherapy. This combined treatment has resulted in a large change in the survival of these patients.
METHODS
A retrospective review of a prospectively maintained database was performed. A restricted cohort of patients with only LAMN histology and complete CRS were included in the statistical analysis.
RESULTS
Four hundred and fifty patients were available with a median follow-up of 15.3 years (range 10-35 years). The median age was 49.7 and there were 196 males (43.6%). The mean survival was 24.5 years. Extent of parietal peritonectomy, resection of uterus, ovaries and apex of vagina had no impact on survival. Variables that indicated an increased extent of disease on visceral peritoneal surfaces had a significant impact on survival. Early postoperative intraperitoneal chemotherapy with 5-fluorouracil did not augment hyperthermic intraperitoneal chemotherapy (HIPEC). Patients who required reoperation for recurrence or patients with class 4 adverse events had a reduced prognosis.
CONCLUSIONS
The mean survival of LAMN treated by complete CRS and perioperative chemotherapy was 24.5 years. Extent of disease quantitated on visceral peritoneal surfaces by the extent of visceral resections was the variable associated with treatment failure. Peritonectomy plus HIPEC was able to control disease on parietal peritoneal surfaces. Not only a larger extent of disease but also its location on visceral peritoneal surfaces controlled survival.
PubMed: 37601148
DOI: 10.1097/AS9.0000000000000193 -
Gynecologic Oncology Reports Oct 2023Several types of mucinous lesions of the fallopian tube have been reported, including metaplastic and neoplastic lesions, most of which exhibit gastric phenotypes. Here,...
Several types of mucinous lesions of the fallopian tube have been reported, including metaplastic and neoplastic lesions, most of which exhibit gastric phenotypes. Here, we report a unique case of a mucinous tumor arising in the right fallopian tube of a 36-year-old female who presented with refractory abdominal pain for approximately one year. Abdominal CT and MRI found a cystic lesion leading to the diagnosis of hematosalpinx, thus right salpingo-oophorectomy and appendectomy were performed. Macroscopic findings included cystic dilatation of the distal portion of the right fallopian tube, filled with gelatinous mucin. Histologically, mucinous columnar cells proliferated in papillary configurations in the cystic region without invasion, resembling low-grade appendiceal mucinous neoplasms. Immunohistochemical analysis revealed that the neoplastic cells expressed CDX-2 and SATB2, but not WT-1, PAX8, ER, PgR, or claudin 18. Sanger sequencing of the mucinous lesion identified a KRAS exon 2 mutation (p.G12A), confirming similar pathologic and genetic characteristics to ovarian mucinous borderline tumors. This rare low grade intestinal-type mucinous tumor indicates the fallopian tube epithelium can give rise to tumors resembling low-grade appendiceal mucinous neoplasms and cause pseudomyxoma peritonei without appendiceal lesions.
PubMed: 37583589
DOI: 10.1016/j.gore.2023.101252