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NET of the appendix. (a) Axial CT image with intravenous and oral contrast material shows an enhancing soft-tissue mass at the base of the cecum (arrow). There are dilated small-bowel loops due to extension of the tumor into the ileocecal valve, causing proximal obstruction. (b) Coronal 111 In octreotide image shows focal activity in the right lower quadrant (arrow), corresponding to the CT abnormality. Otherwise, there is normal biodistribution of the radiotracer without evidence of metastatic disease.
Source publication
Appendiceal neoplasms are uncommon tumors of the gastrointestinal tract that may manifest with symptoms of appendicitis, right lower quadrant pain, or palpable mass, leading to imaging or surgical intervention. The majority of appendiceal masses consist of primary epithelial neoplasms and neuroendocrine tumors (NETs). Epithelial neoplasms-mucinous...
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Appendiceal diverticulosis is a rare finding associated with appendiceal neoplasms. Both can masquerade as appendicitis in patients and are overlooked in differentials of right upper quadrant pain. A 37-year-old African American female presented with appendicitis-like symptoms to the emergency room with fever and leukocytosis. Appendectomy was perf...
Rationale:
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Introduction
Appendiceal tumors are rare neoplasms detected in about 2 % of appendicectomies. The clinical presentation is often unspecific, varying from unspecific abdominal pain or presenting as an acute appendicitis or being asymptomatic.
Case presentation
We present a case of a patient presenting as an acute appendicitis with a mucocele, and t...
Introduction
Tubulo-villous adenoma is a rare benign appendiceal neoplasm often asymptomatic with the most clinical manifestation that resembles acute appendicitis. Pre-operative diagnosis is difficult by its rarity and the absence of typical symptoms. Adequate treatment is surgical resection.
Presentation of case
A 69-year-old male was admitted t...
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Citations
... Second, it is sometimes difficult to detect the primary lesion through CT when the appendiceal neoplasm is small and adheres to the metastatic peritoneum, making it difficult to distinguish from the metastatic peritoneum [10]. Third, appendiceal neoplasms are also difficult to differentiate from acute appendicitis through CT imaging [11]. Therefore, in some patients, the diagnosis of appendiceal neoplasms cannot be identified until a laparoscopic exploration or surgery is performed. ...
... CE-CT was performed on 16 patients, with 14 at the pretreatment phase and 2 in the post-treatment phase. The median time interval between CE-CT and FDG PET/ CT was 7 (3)(4)(5)(6)(7)(8)(9)(10)(11)(12)(13)(14) days. CT images were reviewed by two physicians, including one senior physician who had more than 10 years of experience in CT diagnosis. ...
Background
In the present study, we investigated the value of ¹⁸ F-fibroblast-activation protein inhibitor (FAPI) positron emission tomography/computed tomography ( ¹⁸ F-FAPI-42 PET/CT) to preoperative evaluations of appendiceal neoplasms and management for patients.
Methods
This single-center retrospective clinical study, including 16 untreated and 6 treated patients, was performed from January 2022 to May 2023 at Southern Medical University Nanfang Hospital. Histopathologic examination and imaging follow-up served as the reference standard. ¹⁸ F-FAPI-42 PET/CT was compared to ¹⁸ F-fluorodeoxyglucose ( ¹⁸ F-FDG) PET/CT and contrast-enhanced CT (CE-CT) in terms of maximal standardized uptake value (SUVmax), diagnostic efficacy and impact on treatment decisions.
Results
The accurate detection of primary tumors and peritoneal metastases were improved from 28.6% (4/14) and 50% (8/16) for CE-CT, and 43.8% (7/16) and 85.0% (17/20) for ¹⁸ F-FDG PET/CT, to 87.5% (14/16) and 100% (20/20) for ¹⁸ F-FAPI-42 PET/CT. Compared to ¹⁸ F-FDG PET/CT, ¹⁸ F-FAPI-42 PET/CT detected more regions infiltrated by peritoneal metastases (108 vs. 43), thus produced a higher peritoneal cancer index (PCI) score (median PCI: 12 vs. 5, P < 0.01). ¹⁸ F-FAPI-42 PET/CT changed the intended treatment plans in 35.7% (5/14) of patients compared to CE-CT and 25% (4/16) of patients compared to ¹⁸ F-FDG PET/CT but did not improve the management of patients with recurrent tumors.
Conclusions
The present study revealed that ¹⁸ F-FAPI-42 PET/CT can supplement CE-CT and ¹⁸ F-FDG PET/CT to provide a more accurate detection of appendiceal neoplasms and improved treatment decision making for patients.
... Other more rare tumors are metastatic tumors, lymphoma, tumors of neural origin, and mesenchymal tumors. 4 In between 30 and 50 percent of patients, appendiceal neoplastic lesions most frequently mimic or cause acute appendicitis. 5 About 10% of appendiceal polyps are detected incidentally. ...
... In the stratified epithelium, there are dysplastic changes, with crowding, nuclear atypia, and focal mitotic figures. 4 In dependence on the number and size of the adenoma, the patients are categorized into two groups, with low and high risk. The first recommended monitoring colonoscopy for low-risk patients, defined as those with one to two tubular adenomas smaller than 1 cm, should be carried out every five to ten years. ...
Appendiceal neoplasms are quite uncommon. They are detected in fewer than 0.5 percent of appendectomies and less than 0.5 percent of all gastrointestinal neoplasms. Similar to a colonic adenoma, an appendiceal adenoma is neoplasm with precancerous nature. A rare case of appendiceal adenoma is presented here in a 65-year-old female patient, incidentally discovered at the orifice of the appendix, during the screening analysis. The patient felt well. Abdominal examination and laboratory analysis were regular. Due to the inaccessibility of the lesion by colonoscopy, surgical treatment was recommended. A laparoscopic appendectomy was performed. On pathological examination, diagnosis of tubulovillous adenoma was performed. Endoscopic screening analysis of precancerous appendiceal neoplasm is very important. The method of choice for any appendiceal neoplasm is surgical removal i.e. appendectomy, preferably with a clean caecal margin, which requires stapling of the cecum. Early detection can prevent complications and decrease the risk of consequential appendiceal or colorectal carcinoma.
... Other more rare tumors are metastatic tumors, lymphoma, tumors of neural origin, and mesenchymal tumors. 4 In between 30 and 50 percent of patients, appendiceal neoplastic lesions most frequently mimic or cause acute appendicitis. 5 About 10% of appendiceal polyps are detected incidentally. ...
... In the stratified epithelium, there are dysplastic changes, with crowding, nuclear atypia, and focal mitotic figures. 4 In dependence on the number and size of the adenoma, the patients are categorized into two groups, with low and high risk. The first recommended monitoring colonoscopy for low-risk patients, defined as those with one to two tubular adenomas smaller than 1 cm, should be carried out every five to ten years. ...
Appendiceal neoplasms are quite uncommon. They are detected in fewer than 0.5 percent of appendectomies and less than 0.5 percent of all gastrointestinal neoplasms. Similar to a colonic adenoma, an appendiceal adenoma is neoplasm with precancerous nature. A rare case of appendiceal adenoma is presented here in a 65-year-old female patient, incidentally discovered at the orifice of the appendix, during the screening analysis. The patient felt well. Abdominal examination and laboratory analysis were regular. Due to the inaccessibility of the lesion by colonoscopy, surgical treatment was recommended. A laparoscopic appendectomy was performed. On pathological examination, diagnosis of tubulovillous adenoma was performed. Endoscopic screening analysis of precancerous appendiceal neoplasm is very important. The method of choice for any appendiceal neoplasm is surgical removal i.e. appendectomy, preferably with a clean caecal margin, which requires stapling of the cecum. Early detection can prevent complications and decrease the risk of consequential appendiceal or colorectal carcinoma. Sažetak Tumori apendiksa prilično su rijetki. Javljaju se u manje od 0,5 % apendektomija i u manje od 0,5 % svih gastrointestinalnih tumora. Slično kao i adenoma kolona, adenoma apendiksa prekancerozne su prirode. Prikazali smo rijedak slučaj adenoma apendiksa, incidentalno otkrivenogna u ušću apendiksa, skrininig metodom. Bolesnica se u vrijeme analize osjećala dobro. Klinički pregled i laboratorijske analize bili su uredni. Bolesnici se preporuči kirurški tretman. Uradi se laparoskopska apendektomija. Patohistološkom analizom postavi se dijagnoza tubuloviloznog adenoma. Endoskopska skrining analiza prekanceroznih neoplazmi apendiksa i kolona od iznimne je važnosti. Za sve tumore apendiksa metoda izbora je kirurška-apendektomija s dobijanjem čistih resekciskih margina, što zahtijeva stapliranje cekuma. Rana detekcija ovih neoplazmi prevenira komplikacije i smanjuje rizik od posljedičnih koloničnih i karcinoma apendiksa.
... Non-neoplastic etiologies (simple mucus retention cysts, for example) only account for about 20% of these lesions and usually measure less than 2 cm [5]. On the contrary, neoplastic ones tend to be larger at presentation [6]: cystic appendiceal dilatation with maximal diameter ≥15 mm on computerized tomography (CT) and/or ultrasound (US) have been reported to confidently diagnose AMN (CT, 71-83% sensitive and 88-92% specific [3]; US, 83% sensitive, 92% specific [7]). ...
... Ultrasonography can show an elongated hypoechoic mass due to increased dilatation of the proximal portion of the appendix. Features highly suggestive of an AMN are internal concentric echogenic layers giving the appendix an onion skin appearance and acoustic shadowing due to dystrophic mural calcifications (though present in less than 50% of cases) [6,7]. ...
... With magnetic resonance imaging (MRI), an AMN most frequently demonstrates characteristics of a simple fluid lesion, although signal intensity varies depending on the specific protein content. Appendiceal wall calcifications and intraluminal gas are more difficult to appreciate with MRI than with CT [6]. ...
There is no clear evidence on the prevalence and clinical presentation of appendiceal mucinous neoplasm (AMN) among patients with inflammatory bowel disease (IBD), so a systematic review was performed to investigate the diagnosis, management and treatment of AMN in these patients. PubMed, Medline, Scopus and the Cochrane Library were searched for articles published up to September 2023. Twenty-three studies reporting data about 34 AMN patients were included. UC patients had a median age of 52 years and a median length of disease of 10 years; CD patients had a median age of 40.5 years and a median length of disease of 5 years. A pre-operative diagnosis was achieved in 44% of patients. Most patients were symptomatic (82.6%) and showed moderate–severe disease activity (61%). Surgical procedures were performed: laparoscopic appendectomy, ileocecal resection, right hemicolectomy and colectomy/proctocolectomy. Of the patients, 73.5% were diagnosed with low-grade mucinous neoplasm (LAMN) and nine with adenocarcinoma. Synchronous colorectal dysplasia/carcinoma was present in 23.5% of patients. IBD patients with long-standing disease should be routinely screened, not only for colorectal cancer but also for AMN, during gastro-enterologic follow-up. Laparoscopic appendectomy of unruptured LAMN as well as right hemicolectomy of non-metastatic adenocarcinoma are safe procedures in IBD patients.
... Appendiceal cancer is a rare form of malignancy, with an incidence of approximately 1 per 100,000 persons in the United States annually [1]. Despite low case numbers, these tumors demonstrate significant heterogeneity, and the different histologic types vary widely in clinical presentation, response to therapy, and prognosis [2][3][4]. Classification of any appendiceal tumor is therefore essential to optimizing treatment and patient outcomes. ...
Background
Appendiceal tumors represent a range of histologies that vary in behavior. Recommendations for treatment with appendectomy versus right hemicolectomy (RHC) for different tumor types are evolving and sometimes conflicting. This study sought to characterize variation in the United States around surgical treatment of major appendiceal tumor types over time and describe differences in outcomes based on procedure.
Methods
Patients diagnosed with appendiceal goblet cell adenocarcinoma (GCA), mucinous adenocarcinoma, neuroendocrine neoplasm (NEN), or non-mucinous adenocarcinoma from 2004–2017 were identified in the National Cancer Database. Trends in RHC over time and predictors of RHC were identified. Surgical outcomes for each histologic type and stage were compared.
Results
Of 18,216 patients, 11% had GCAs, 34% mucinous adenocarcinoma, 31% NENs, and 24% non-mucinous adenocarcinoma. Rate of RHC for NEN decreased from 68% in 2004 to 40% in 2017 (p = 0.008) but remained constant around 60–75% for other tumor types. Higher stage was associated with increased odds of RHC for all tumor types. RHC was associated with higher rate of unplanned readmission (5% vs. 3%, p < 0.001) and longer postoperative hospital stay (median 5 days vs. 3 days, p < 0.001). On risk-adjusted analysis, RHC was significantly associated with increased survival versus appendectomy for stage 2 disease of all tumor types (HRs 0.43 to 0.63) and for stage 1 non-mucinous adenocarcinoma (HR = 0.56).
Conclusions
Most patients with appendiceal tumors undergo RHC, which is associated with increased readmission, longer length of stay, and improved survival for stage 2 disease of all types. RHC should be offered selectively for appendiceal tumors.
... NET may be difficult to be radiologically visualized because of their size, and generally present as submucosal masses or nodular thickening of the wall [29]. Somatostatin receptor imaging (SRI) using either somatostatin receptor scintigraphy (SRS; e.g., indium-111) including SPECT or positron emission tomography (PET) scanning using gallium-68-labelled in combination with CT may be considered in cases when curative resection is not completely assured or when distant metastasis is suspected [24][25][26][27][28]. ...
... However, recent studies randomized clinical trials have shown that in the short term, medical management with antibiotics is a safe alternative treatment option for cases of uncomplicated acute appendicitis [1,[5][6][7]. Nevertheless, the possibility of appendiceal neoplasms incidental diagnosis must be taken into account [4,5,[8][9][10]. ...
... Tumors of the appendix are unusual entities, mainly diagnosed incidentally in the anatomopathological study of appendectomy pieces, and rarely suspected before or during a surgical procedure [5,6,8,[11][12][13][14][15]. Therefore, the study of the appendiceal sample is crucial both to diagnose the presence of neoplasms, as well as to determine their histological subtype [4,10,12,15]. ...
... Nevertheless, the percentage of appendiceal neoplasms in the group of patients who did not undergo appendectomy is unclear. Moreover, advanced stages of a possible appendiceal neoplasm could be more frequently presented due to not intervening surgically in these patients leading to increased morbidity and mortality in this specific group of patients [3,8,18,19]. Nevertheless, it is still unclear if this difference is statistically significant. ...
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.
... This rate reaches 100% if the tumor is <3 cm and without lymph node and distant metastasis. [30] The results of the current study showed similar survival rates, and no recurrence was observed. ...
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.
... 10,11 The symptoms may mimic that of acute appendicitis as was seen in the study cases of this series or may rarely present as carcinoid syndrome, namely flushing, bronchoconstriction, and diarrhea caused by release of vasoactive substances. 12 Histopathological diagnosis is usually straightforward but substantiated by ancillary tests like IHCs for synaptophysin or chromogranin A in certain cases. For categorization into G1, G2, and G3 histologic grades, mitotic count and KI-67 index plays a significant role and has a bearing on the prognosis and long-term outcome of these patients. ...
Acute appendicitis is the most common surgical emergency worldwide, with appendix being the most frequently encountered specimen by a reporting histopathologist. They may sometimes show rare and uncommon histomorphologic pictures that may create diagnostic dilemmas, few of such cases being discussed here. There are two cases of appendicular neuroendocrine tumors (NETs) that initially presented as acute appendicitis clinically, with microscopic tumor foci measuring <1 cm each. Immunohistochemistry for synaptophysin substantiated the histopathological diagnosis in one case. Neurogenic appendicopathy is another non-neoplastic entity discussed that may be overdiagnosed as appendicular neoplasms such as NET, neuromas, or neurofibromas. Granulomatous appendicitis may be another cause of recurrent appendicitis due to a variety of cases, tuberculosis being one of them and antitubercular therapy being the mainstay of treatment for these cases. Xanthogranulomatous appendicitis may simulate colonic malignancy, Crohn’s disease, malakoplakia, etc. Histopathological features are the main diagnostic modalities for these instances. Pinworm is a common helminthic infection of the gastrointestinal tract. Currently, its incidence is on the declining side due to better sanitation practices. However, it must be reported in appendectomy sections, if present, to initiate a course of antihelminthic drugs. Pseudomyxoma peritonei is an uncommon entity classically characterized by mucinous ascites resulting from ruptured appendiceal mucinous tumors, one such rare case being reported here. Low-grade appendiceal mucinous neoplasm is a distinctive entity rarely seen in appendectomy cases, belonging to groups of appendiceal mucinous neoplasms. One such instance has been depicted here.
... When peritoneal spread is present, the most common reported location of the primary tumor is the ileum or the appendix [48,49]. Appendicular NET is often difficult to identify on CT and MRI because of its small size [50]. On the other hand, ileal NET shows typical imaging findings due to invasion of the adjacent mesentery. ...
... It presents as a mesenteric soft tissue mass with frequent calcifications and spiculated margins due to desmoplastic reaction, which retracts mesenteric vessels producing chronic ischemic ileitis with mural thickening, tethering and distension of ileal loops (Fig. 16) [51]. Staging of advanced NETs benefits from the use of functional imaging techniques such as [111In]-octreotide scintigraphy, [68 Ga] somatostatin analog PET/CT and [18F] FDG PET/CT, which improve the detection of peritoneal and visceral metastases [50,52]. ...
... In this sense, consensus guidelines from the Peritoneal Surface Oncology Group (PSOGI) and other panels of experts have classified PMP into three basic groups [ The main imaging feature of PMP on CT is a high-volume loculated mucinous ascites causing mass effect on solid abdominal organs like the liver or the spleen, which develop a characteristic scalloped appearance (Fig. 17). The appendix should always be scrutinized looking for a primary AMN, that presents as a dilated appendix with hypodense material, frequent septa, thin walls and peripheral calcifications (Fig. 18) [3,5,50,62,63]. Peritoneal mucinous deposits may also present linear or punctate calcifications [50]. A more infiltrative behavior of mucinous implants is seen in cases with high-grade Fig. 14 Peritoneal carcinomatosis secondary to a recurrent gastric adenocarcinoma in a 41-year-old woman. ...
Peritoneal malignancies represent a diagnostic challenge for abdominal radiologists, oncologists, surgeons and pathologists in multidisciplinary teams, who must address their differential diagnosis, staging and treatment. In this article, we explain the pathophysiology of these processes and lay out the role of different imaging techniques in their evaluation. Then, we review the clinical and epidemiological aspects, the main radiological features and the therapeutic approaches for each primary and secondary peritoneal neoplasm, with surgical and pathological correlation. We further describe other rare peritoneal tumors of uncertain origin and a variety of entities that may mimic peritoneal malignancy. Finally, we summarize the key imaging findings of each peritoneal neoplasm to facilitate an accurate differential diagnosis that may impact patient management.
Clinical relevance statement
Imaging plays an essential role in the evaluation of peritoneal malignancies, assessing their extension, detecting unfavorable sites of involvement and facilitating an accurate differential diagnosis, helping to choose the best therapeutic approach.
Graphical abstract
