-
Cancers Apr 2024Pseudomyxoma peritonei (PMP) is a rare, progressive, slowly growing, inadequately understood neoplasm with a 5-year progression-free survival rate of as low as 48%. It... (Review)
Review
BACKGROUND
Pseudomyxoma peritonei (PMP) is a rare, progressive, slowly growing, inadequately understood neoplasm with a 5-year progression-free survival rate of as low as 48%. It is characterized by varying degrees of malignancy and the production of mucinous and gelatinous structures. Typically, the development of pseudomyxoma peritonei is associated with the rupture of appendiceal mucinous tumors and other gastrointestinal or ovarian mucinous tumors. The goal of our literature review was to identify various aspects that characterize the ovarian causes of pseudomyxoma peritonei.
MATERIALS AND METHODS
The authors performed an extensive literature search between 1 February 2024 and 2 March 2024 on the following databases: Pubmed, Scopus, Oxford Journals, and Reaxys, and the findings were summarized into seven main clinical and paraclinical situations.
RESULTS
According to our research, the main instances in which pseudomyxoma peritonei can be triggered by an ovarian cause are the following: (1) mucinous cystadenoma; (2) mucinous ovarian cancer; (3) colon cancer with ovarian metastasis; (4) malignant transformation of an ovarian primary mature cystic teratoma; (5) appendiceal mucocele with peritoneal dissemination mimicking an ovarian tumor with peritoneal carcinomatosis; (6) mucinous borderline tumor developing inside an ovarian teratoma; and (7) the association between a mucinous bilateral ovarian cancer and a colonic tumor.
CONCLUSIONS
In our study, we aimed to provide a comprehensive overview of the ovarian causes of pseudomyxoma peritonei, including its epidemiology, imagery characteristics, symptoms, current treatment, and promising future therapies, in the hopes of finding feasible solutions, as a lack of understanding of this mucus-secreting malignant disease increases the risk of delayed diagnosis or uncontrolled deterioration.
PubMed: 38672528
DOI: 10.3390/cancers16081446 -
BMC Surgery Apr 2024Nonoperative management of uncomplicated appendicitis is currently being promoted as treatment option, albeit 0.7-2.5% of appendectomies performed due to suspected acute...
BACKGROUND
Nonoperative management of uncomplicated appendicitis is currently being promoted as treatment option, albeit 0.7-2.5% of appendectomies performed due to suspected acute appendicitis show histologically malignant findings. The purpose of this study was to investigate the incidence of neoplasm and malignancy of the appendix in patients presenting with suspected acute appendicitis in real world setting.
METHODS
This is a retrospective single-centre investigation of 457 patients undergoing appendectomy between the years 2017-2020. The patients' demographics, symptoms and diagnosis, intraoperative findings, and histopathological results were analysed.
RESULTS
In 3.7% (n = 17) histological analysis revealed neoplasms or malignancies. Median age was 48 years (20-90 years), without sex predominance. Leukocytes (11.3 ± 3.7 G/l) and C-reactive protein (54.2 ± 69.0 mg/l) were elevated. Histological analysis revealed low-grade mucinous appendiceal neoplasia (n = 3), sessile serrated adenoma of the appendix (n = 3), neuroendocrine tumours (n = 7), appendiceal adenocarcinoma of intestinal type (n = 3), and goblet cell carcinoma (n = 1). Additional treatment varied between no treatment or follow-up due to early tumour stage (n = 4), follow-up care (n = 3), additional surgical treatment (n = 8), or best supportive care (n = 2).
CONCLUSIONS
Preoperative diagnosis of appendiceal tumours is difficult. Nonoperative management of patients with acute, uncomplicated appendicitis potentially prevents the correct diagnosis of malignant appendiceal pathologies. Therefore, close follow-up or surgical removal of the appendix is mandatory.
Topics: Humans; Appendiceal Neoplasms; Appendicitis; Middle Aged; Retrospective Studies; Male; Female; Adult; Aged; Appendectomy; Incidence; Aged, 80 and over; Young Adult; Acute Disease
PubMed: 38658891
DOI: 10.1186/s12893-024-02412-4 -
Cureus Mar 2024Appendicitis is one of the most common diagnoses that general surgeons encounter in practice. An exceedingly rare cause of this disease is neoplasm. We report the case...
Appendicitis is one of the most common diagnoses that general surgeons encounter in practice. An exceedingly rare cause of this disease is neoplasm. We report the case of a 24-year-old female who presented with non-specific right lower quadrant abdominal pain and equivocal findings of appendicitis and pelvic congestion syndrome on CT imaging. After an extensive work-up, the patient underwent a diagnostic laparoscopy with an appendectomy. The appendix appeared grossly normal; however, on a pathologic review of the specimen, a low-grade appendiceal mucinous neoplasm (LAMN) was found. This case is unique in that it demonstrates exclusive management of LAMN laparoscopically. It reinforces the need to approach non-specific abdominal pain from a multidisciplinary perspective and to utilize laparoscopy as a diagnostic/therapeutic modality when other, less invasive, modalities fail to diagnose a patient's pain.
PubMed: 38623096
DOI: 10.7759/cureus.56286 -
Annals of Surgical Oncology Jul 2024
Topics: Humans; Appendiceal Neoplasms; Adenocarcinoma, Mucinous; Survival Rate; Prognosis; Appendectomy
PubMed: 38622457
DOI: 10.1245/s10434-024-15289-7 -
Journal of Community Hospital Internal... 2023We present here a 66-year-old Caucasian male whose persistent abdominal pain thought to be due to appendicitis and associated acute splanchnic thrombosis. He was...
We present here a 66-year-old Caucasian male whose persistent abdominal pain thought to be due to appendicitis and associated acute splanchnic thrombosis. He was initially managed with antibiotics and anticoagulation. But further work up revealed a low-grade appendiceal mucinous neoplasm causing the splanchnic vein thrombosis. Additionally, diagnosis and management of this rare tumor and appropriate work up for splanchnic thrombosis will be briefly reviewed here.
PubMed: 38596560
DOI: 10.55729/2000-9666.1253 -
Annals of Surgical Oncology Jul 2024Mucinous appendiceal adenocarcinomas (MAA) and non-mucinous appendiceal adenocarcinomas (NMAA) demonstrate differences in rates and patterns of recurrence, which may...
BACKGROUND
Mucinous appendiceal adenocarcinomas (MAA) and non-mucinous appendiceal adenocarcinomas (NMAA) demonstrate differences in rates and patterns of recurrence, which may inform the appropriate extent of surgical resection (i.e., appendectomy versus colectomy). The impact of extent of resection on disease-specific survival (DSS) for each histologic subtype was assessed.
PATIENTS AND METHODS
Patients with resected, non-metastatic MAA and NMAA were identified in the Surveillance, Epidemiology, and End Results database (2000-2020). Multivariable models were created to examine predictors of colectomy for each histologic subtype. DSS was calculated using Kaplan-Meier estimates and examined using Cox proportional hazards modeling.
RESULTS
Among 4674 patients (MAA: n = 1990, 42.6%; NMAA: n = 2684, 57.4%), the majority (67.8%) underwent colectomy. Among colectomy patients, the rate of nodal positivity increased with higher T-stage (MAA: T1: 4.6%, T2: 4.0%, T3: 17.1%, T4: 21.6%, p < 0.001; NMAA: T1: 6.8%, T2: 11.4%, T3: 25.6%, T4: 43.8%, p < 0.001) and higher tumor grade (MAA: well differentiated: 7.7%, moderately differentiated: 19.2%, and poorly differentiated: 31.3%; NMAA: well differentiated: 9.0%, moderately differentiated: 20.5%, and 44.4%; p < 0.001). Nodal positivity was more frequently observed in NMAA (27.6% versus 16.4%, p < 0.001). Utilization of colectomy was associated with improved DSS for NMAA patients with T2 (log rank p = 0.095) and T3 (log rank p = 0.018) tumors as well as moderately differentiated histology (log rank p = 0.006). Utilization of colectomy was not associated with improved DSS for MAA patients, which was confirmed in a multivariable model for T-stage, grade, and use of adjuvant chemotherapy [hazard ratio (HR) 1.00, 95% confidence interval (CI) 0.81-1.22].
CONCLUSIONS
Colectomy was associated with improved DSS for patients with NMAA but not MAA. Colectomy for MAA may not be required.
Topics: Humans; Appendiceal Neoplasms; Female; SEER Program; Male; Adenocarcinoma, Mucinous; Middle Aged; Colectomy; Aged; Survival Rate; Appendectomy; Adenocarcinoma; Follow-Up Studies; Prognosis; Neoplasm Recurrence, Local; Neoplasm Staging; Adult
PubMed: 38594579
DOI: 10.1245/s10434-024-15233-9 -
World Journal of Gastrointestinal... Mar 2024Appendiceal mucinous neoplasms (AMNs), although not classified as rare, are relatively uncommon tumors most often discovered incidentally during colorectal surgery....
BACKGROUND
Appendiceal mucinous neoplasms (AMNs), although not classified as rare, are relatively uncommon tumors most often discovered incidentally during colorectal surgery. Accurate identification of AMNs is difficult due to non-specific symptoms, overlapping tumor markers with other conditions, and the potential for misdiagnosis. This underscores the urgent need for precision in diagnosis to prevent severe complications.
CASE SUMMARY
This case report describes the unexpected discovery and treatment of a low-grade AMN (LAMN) in a 74-year-old man undergoing laparoscopic hemicolectomy for transverse colon adenocarcinoma (AC). Preoperatively, non-specific gastrointestinal symptoms and elevated tumor markers masked the presence of AMN. The tumor, presumed to be an AMN peritoneal cyst intraoperatively, was confirmed as LAMN through histopathological examination. The neoplasm exhibited mucin accumulation and a distinct immunohistochemical profile: Positive for Homeobox protein , Cytokeratin 20, special AT-rich sequence-binding protein 2, and Mucin 2 but negative for cytokeratin 7 and Paired box gene 8. This profile aids in distinguishing appendiceal and ovarian mucinous tumors. Postoperative recovery was uncomplicated, and the patient initiated adjuvant chemotherapy for the colon AC.
CONCLUSION
This case highlights the diagnostic complexity of AMNs, emphasizing the need for vigilant identification to avert potential complications, such as pseudomyxoma peritonei.
PubMed: 38577069
DOI: 10.4240/wjgs.v16.i3.944 -
Pleura and Peritoneum Mar 2024Due to the scarcity of low-grade appendiceal mucinous neoplasm (LAMN), there is an absence of systematized guidelines concerning its management, especially after...
OBJECTIVES
Due to the scarcity of low-grade appendiceal mucinous neoplasm (LAMN), there is an absence of systematized guidelines concerning its management, especially after incidental finding on an appendiceal specimen. In this study, we evaluate the active surveillance (AS) strategy adopted for a series of patients diagnosed with LAMN on resection specimens who were considered to have a low risk of pseudomyxoma progression.
METHODS
Thirty patients were included between April 2014 and July 2021, with a female majority and a median follow-up period of 3.1 years. The inclusion criteria were as follows: LAMN diagnosis on appendiceal specimens, confirmed in an expert center, limited extra-appendiceal mucin resected and localized around the appendix, normal biology (CEA, CA199, CA125) and normal abdominopelvic MRI. AS included physical exam (trocar scar), biology and MRI, 6 months postoperatively, then yearly for 10 years.
RESULTS
As an initial surgery, 77 % had an appendectomy as their initial intervention, 17 % had a cecectomy, and 6 % had a right colectomy. After follow-up, 87 % of patients showed no sign of disease progression by MRI, while 13 % progressed to PMP. MRI performed in the first postoperative year predicted the disease prognosis in 97 % of patients.
CONCLUSIONS
The AS strategy, based on MRI, is a valid option after incidental LAMN diagnosis.
PubMed: 38558872
DOI: 10.1515/pp-2023-0032 -
JPGN Reports Feb 2024This case report describes a 17-year-old patient with a low-grade appendiceal mucinous neoplasm. The patient presented with non-bloody diarrhea, abdominal pain, and...
This case report describes a 17-year-old patient with a low-grade appendiceal mucinous neoplasm. The patient presented with non-bloody diarrhea, abdominal pain, and weight loss. A colonoscopy revealed a cecal polypoid mass that required laparoscopic surgery. The residual appendix was dilated with myxoglobulosis and histopathology confirmed the diagnosis of a low-grade appendiceal mucinous neoplasm staged pT3Nx. The potential risk of pseudomyxoma peritonei is a serious complication of these tumors. Surveillance plans include computed tomography abdomen and pelvis, and tumor markers every 6 months for the next 2 years. This case highlights the importance of considering appendiceal malignancy in patients with abdominal pain and weight loss, despite the rarity of the disease. It also emphasizes the need for careful monitoring due to the possible complications associated with these tumors. Treatment and prognosis for appendiceal neoplasms depend on the histopathologic characteristics, tumor-nodes-metastasis stage, tumor grade, and presence of peritoneal disease.
PubMed: 38545266
DOI: 10.1002/jpr3.12017 -
An Amyand Hernia With Concurrent Appendicitis Secondary to a Neuroendocrine Neoplasm: A Case Report.Cureus Feb 2024Amyand's hernia (AH) describes the rare instance of a vermiform appendix within an inguinal hernia. Primary appendiceal neoplasms are also rare with the majority of...
Amyand's hernia (AH) describes the rare instance of a vermiform appendix within an inguinal hernia. Primary appendiceal neoplasms are also rare with the majority of cases being found incidentally during routine histopathology. This case reports the management of a 15-year-old male, who presented to the emergency department with acute appendicitis located within an indirect right inguinal hernia, which was ultimately secondary to a neuroendocrine tumor (NET) with serosal involvement. Intraoperative findings included macroscopic appendicitis with no evidence of perforation. Histopathology returned as a neuroendocrine tumor (pT4) with involved proximal margin and curative treatment was undertaken with a caecectomy which returned no residual malignancy. Key considerations include management options of peritoneal spread within the inguinal canal and recommended management NET in the context of an AH. It is important to understand the varied presentations of common surgical diagnosis such as appendicitis and underlying malignancy should always be considered a differential.
PubMed: 38544647
DOI: 10.7759/cureus.54894