Appendix Cancers
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Vermiform appendix and the Gastrointestinal SystemCancer of the Appendix (also known as appendiceal cancer) occurs when cells in the appendix become abnormal and multiply without control. There are a range of different types of tumours and cancers which can start in the appendix. About two thirds of appendix tumours are carcinoid tumours which develop in hormone-producing cells that are present in many parts of the body, including the appendix. Mucinous cystadenocarcinoma account for about a fifth of appendix tumours, these produces mucin (a jelly-like substance). Pseudomyxoma Peritonei (PMP) is a more common name given to the "jelly belly" syndrome caused by mucinous adenoma and mucinous adenocarcinoma. Less common tumours of the appendix include signet-ring cell adenocarcinoma and paraganglioma. Treatment varies according to the type and stage of tumour.

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Latest Research Publications

This list of publications is regularly updated (Source: PubMed).

Matkowskyj KA, Rao MS, Raparia K
Transcription factor E3 protein-positive perivascular epithelioid cell tumor of the appendix presenting as acute appendicitis: a case report and review of the literature.
Arch Pathol Lab Med. 2013; 137(3):434-7 [PubMed]
Perivascular epithelioid cell tumors (PEComas) are a group of mesenchymal tumors that coexpress melanocytic and smooth muscle markers; their exact origin remains unknown. This group includes renal angiomyolipoma, clear cell sugar tumor, and lymphangioleiomyomatosis, although the term perivascular epithelioid cell tumors is currently used for lesions that exhibit a similar morphologic and immunohistochemical profile throughout the human body. Recently, a distinct subset of PEComas has been shown to harbor transcription factor E3 gene (TFE3) fusions. We report, for the first time, a unique case of TFE3-positive PEComa presenting as acute appendicitis in a 24-year-old woman. Microscopically, the tumor was composed of benign-appearing epithelioid cells with clear and eosinophilic cytoplasm, and arranged in nested and alveolar patterns. Immunohistochemical studies showed diffuse strong positivity for neuron-specific enolase, TFE3, and progesterone receptor and focal strong positivity for human melanoma black-45 (HMB-45) and melanocyte differentiation antigen (Melan-A) in the tumor cells. Although rare, PEComa should be included in the differential diagnosis of mesenchymal tumors of the appendix.


Volante M, Daniele L, Asioli S, et al.
Tumor staging but not grading is associated with adverse clinical outcome in neuroendocrine tumors of the appendix: a retrospective clinical pathologic analysis of 138 cases.
Am J Surg Pathol. 2013; 37(4):606-12 [PubMed]
Appendiceal neuroendocrine neoplasms (NENs) are rare and usually incidentally discovered. Most cases are clinically indolent, although the rare aggressive ones are poorly predictable. The aim of this study was to test the applicability and prognostic significance of the new World Health Organization (WHO) classification and to test the several pathologic features and TNM staging systems (American Joint Committee on Cancer and European Neuroendocrine Tumor Society) in these tumors. A multi-institutional retrospective series of 138 appendiceal NENs was selected on the basis of the availability of both pathologic material and clinical information, including follow-up data. All cases were reviewed to record pathologic features and to apply year 2000 and 2010 WHO classifications, as well as European Neuroendocrine Tumor Society and American Joint Committee on Cancer TNM stages. Clinical and pathologic characteristics were compared with disease outcome by contingency, univariate, and multivariate survival analyses. Although up to one third of cases presented several malignancy-associated pathologic features, only 4 patients died of the disease. Adverse outcome was significantly associated with extramural extension (including mesoappendix), well-differentiated carcinoma diagnosis (2000 WHO classification), pT3-4 stage, older age, and presence of positive resection margins, but not with tumor size, mitotic or proliferative indexes, and, consequently, 2010 WHO grading. In the appendix, at variance with midgut/hindgut NENs, the 2000 WHO classification performs better than the grading-based 2010 WHO scheme and, together with tumor stage, is the most relevant parameter associated with clinical aggressiveness.


Nishikawa G, Sekine S, Ogawa R, et al.
Frequent GNAS mutations in low-grade appendiceal mucinous neoplasms.
Br J Cancer. 2013; 108(4):951-8 [PubMed] Article available free on PMC after 05/03/2014
BACKGROUND: The molecular basis for the development of appendiceal mucinous tumours, which can be a cause of pseudomyxoma peritonei, remains largely unknown.
METHODS: Thirty-five appendiceal mucinous neoplasms were analysed for GNAS and KRAS mutations. A functional analysis of mutant GNAS was performed using a colorectal cancer cell line.
RESULTS: A mutational analysis identified activating GNAS mutations in 16 of 32 low-grade appendiceal mucinous neoplasms (LAMNs) but in none of three mucinous adenocarcinomas (MACs). KRAS mutations were found in 30 LAMNs and in all MACs. We additionally analysed a total of 186 extra-appendiceal mucinous tumours and found that GNAS mutations were highly prevalent in intraductal papillary mucinous tumours of the pancreas (88%) but were rare or absent in mucinous tumours of the colorectum, ovary, lung and breast (0-9%). The prevalence of KRAS mutations was quite variable among the tumours. The introduction of the mutant GNAS into a colorectal cancer cell line markedly induced MUC2 and MUC5AC expression, but did not promote cell growth either in vitro or in vivo.
CONCLUSION: Activating GNAS mutations are a frequent and characteristic genetic abnormality of LAMN. Mutant GNAS might play a direct role in the prominent mucin production that is a hallmark of LAMN.


Zaharie F, Tomuş C, Mocan L, et al.
Intestinal obstruction secondary to appendiceal mucocele.
Chirurgia (Bucur). 2012 Nov-Dec; 107(6):802-4 [PubMed]
Few cases of intestinal obstruction complicating an appendiceal mucocele have been recorded. We report the case of a young woman who presented to the emergency room with diffusely abdominal pain, nausea, vomiting and disruption of bowel movements. Her abdomen was mildly distended and tympanic. A flat film of the abdomen revealed dilated small bowel loops with air-fluid levels suggestive of small bowel obstruction. She also had leukocytosis. An emergency operation was performed under the diagnosis of intestinal obstruction. The intraoperatory findings showed a tumoral appendiceal mass permeated into the ileum in two distinct points, causing an enteral stenosis. We performed an appendectomy "en bloc" with two enteral loop resections of the permeated ileum followed by two T-T enteral anastomoses. The pathologic examination revealed appendiceal mucinous cystadenoma. Postoperative course was favorable, the patient being discharged on the seventh postoperative day. Postoperative checks performed at 3, 6, 12 and 24 months (including colonoscopy) have not showed pathological changes.


Irfan M, Hogan AM, Gately R, et al.
Management of the acute appendix mass: a survey of surgical practice.
Ir Med J. 2012; 105(9):303-5 [PubMed]
Management of the appendix mass is controversial with no consensus in the literature. Traditionally, the approach has been conservative followed by interval appendicectomy. A survey was distributed to 117 surgeons (100 consultants and 17 final year specialist registrars) to determine how the appendix mass is currently treated in Ireland. In total, 70 surgeons responded. 51 (73%) adopt a conservative approach initially. 48 (68%) favoured interval appendicectomy at six weeks after a period of successful conservative management. 34 (49%) gave risk of recurrence as the reason for performing interval appendicectomy and 16 (22%) would perform interval appendicectomy in order to obtain histological analysis to outrule caecal or appendiceal neoplasm. 44 (63%) opted for a laparoscopic rather than an open approach for interval appendicectomy. No consensus exists in Ireland for management of the appendix mass presenting acutely. The present series demonstrates a trend towards conservative approach initially followed by interval appendicectomy.


Baykal C, Türkmen IC, Hizli F, et al.
Primary mucinous borderline tumor of the vermiform appendix mimicking ovarian carcinoma; case report.
Eur J Gynaecol Oncol. 2012; 33(5):528-9 [PubMed]
INTRODUCTION: Primary adenocarcinoma of the vermiform appendix is a very rare entity. Borderline mucinous tumor of the appendix is a much more rare disease.
CASE: We present a 71-year-old woman with primary mucinous borderline tumor of the vermiform appendix. She was misdiagnosed as an ovarian carcinoma patient and operated on by a gynecologic oncology team. Her frozen section analysis of an appendicial mass resulted as borderline tumor of appendix vermiformis, and right hemicolectomy, as is advised in invasive colon tumors, was not carried out.
CONCLUSION: Borderline mucinous tumor of the appendix vermiformis should be kept in mind in patients who have pseudomyxoma peritonei during surgery. Tumoral resection may be the definitive therapy in these patients.


Iversen LH, Rasmussen PC, Laurberg S
Value of laparoscopy before cytoreductive surgery and hyperthermic intraperitoneal chemotherapy for peritoneal carcinomatosis.
Br J Surg. 2013; 100(2):285-92 [PubMed]
BACKGROUND: The outcome of cytoreductive surgery combined with hyperthermic intraperitoneal chemotherapy (HIPEC) depends on the extent of peritoneal carcinomatosis. The role of laparoscopy in the preoperative assessment of extent of peritoneal carcinomatosis in potential candidates for cytoreductive surgery and HIPEC was evaluated in a consecutive series.
METHODS: Patients with peritoneal carcinomatosis from colorectal cancer or appendiceal cancer, pseudomyxoma peritonei or peritoneal mesothelioma referred to a single, national HIPEC centre were included prospectively between June 2006 and January 2012. From September 2010, preoperative evaluation also included a laparoscopy in patients deemed amenable to cytoreductive surgery and HIPEC after radiological evaluation, apart from those with pseudomyxoma peritonei with massive amounts of mucin.
RESULTS: In the period before laparoscopic evaluation, 70 patients underwent laparotomy of whom 39 (56 per cent) completed cytoreductive surgery and HIPEC. After the introduction of laparoscopic assessment, diagnostic laparoscopy was planned in 45 patients and successful in 43. The laparoscopic evaluation excluded 18 patients from surgery because of extensive disease, among other reasons. Laparoscopy was uneventful and associated with no deaths. Twenty-seven patients were considered amenable to cytoreductive surgery and HIPEC based on laparoscopic findings, of whom 17 completed this treatment; the disease was unresectable in the remaining ten patients. Of 13 patients who were not eligible for laparoscopic evaluation and were subjected to cytoreductive surgery plus HIPEC, 11 completed the procedure. The overall completion rate of cytoreductive surgery and HIPEC increased to 70 per cent (28 of 40) after the introduction of laparoscopic evaluation.
CONCLUSION: Diagnostic laparoscopy was valuable in preoperative evaluation of the extent of peritoneal carcinomatosis, and improved patient selection for cytoreductive surgery and HIPEC.


Lin JE, Seo S, Kushner DM, Rose SL
The role of appendectomy for mucinous ovarian neoplasms.
Am J Obstet Gynecol. 2013; 208(1):46.e1-4 [PubMed]
OBJECTIVE: To determine how frequently the appendix harbors pathology in women having surgery for mucinous neoplasms of the ovary and assess the associated morbidity.
STUDY DESIGN: A retrospective chart review of patients operated on at our institution with the diagnosis of a mucinous neoplasm of the ovary or appendix.
RESULTS: A total of 327 cases were identified. Of the 309 women with mucinous ovarian neoplasms, 197 (64%) were benign, 68 (22%) low malignancy potential, and 44 (14%) were invasive. Of 155 appendectomies performed, only 1 metastatic low grade mucinous appendiceal tumor was found, but this appendix was grossly abnormal. There was no association between wound complications and appendectomy.
CONCLUSION: When a grossly normal appendix is removed during surgery for a mucinous ovarian neoplasm without evidence of pseudomyxoma peritonei, no primary or metastatic mucinous appendiceal tumors are found.


Shankar S, Ledakis P, El Halabi H, et al.
Neoplasms of the appendix: current treatment guidelines.
Hematol Oncol Clin North Am. 2012; 26(6):1261-90 [PubMed]
The purpose of this article is to update the medical community on the current management of patients with appendiceal neoplasms. The authors discuss clinical evidence of medical and surgical treatment with emphasis on presentation, diagnosis, pathology, and surgical technique. Current available clinical evidence on the use of systemic chemotherapy is included. The authors describe in detail management of peritoneal carcinomatosis arising from tumors of the appendix with cytoreductive surgery and hyperthermic intraperitoneal therapy as standard of care.


Nezhat CH, Dun EC, Wieser F, Zapata M
A rare case of primary extranodal marginal zone B-cell lymphoma of the ovary, fallopian tube, and appendix in the setting of endometriosis.
Am J Obstet Gynecol. 2013; 208(1):e12-4 [PubMed]
Extranodal marginal zone B-cell lymphomas are uncommon. Most occur in the gastrointestinal tract. Marginal zone B-cell lymphomas of the female genital tract are rare, and few cases exist of marginal zone B-cell lymphomas of the uterus, cervix, and fallopian tubes. We report the first marginal zone B-cell lymphoma of the ovary, fallopian tube, and appendix arising in endometriosis.


Vassos N, Agaimy A, Günther K, et al.
A novel complex KIT mutation in a gastrointestinal stromal tumor of the vermiform appendix.
Hum Pathol. 2013; 44(4):651-5 [PubMed]
Gastrointestinal stromal tumors of the vermiform appendix are rare. To date, only 11 cases have been reported in the English literature. Here, we present a new case of appendiceal gastrointestinal stromal tumor associated with complete situs inversus. A 48-year-old man was operated on due to appendicitis-like symptoms. Laparotomy revealed a ruptured conglomerate tumor in the lower abdomen associated with extensive peritoneal adhesions. Histology showed a spindle cell gastrointestinal stromal tumor with prominent sclerosis and calcification without low mitotic activity. The tumor cells expressed strongly CD117 and CD34. The mutation analysis revealed a heterozygous deletion/insertion involving exon 11 of KIT (pK558_V559delNNins). Because the tumor was ruptured intraoperatively, a high risk was assigned according to the revised National Institute of Health criteria and adjuvant therapy with imatinib mesylate was recommended. The patient is currently alive without evidence of progression 27 months after surgery.


Poiana C, Carsote M, Trifanescu R, et al.
Case study of appendiceal carcinoid during pregnancy.
J Med Life. 2012; 5(3):325-8 [PubMed] Article available free on PMC after 05/03/2014
The carcinoid tumor of the appendix is one of the most common tumors of the appendix, but one of the rarest anatomic locations of carcinoids. The prognosis is very good, as most tumors are incidentally discovered during surgery for acute or sub-acute appendicitis. The diagnosis is exceptional when combined with pregnancy. We present such a case of a 27-year-old female patient. An emergency appendectomy was performed, and soon after, pregnancy was confirmed. The patient had a tumor smaller than 1 cm in diameter, at the tip of the appendix. The immunochemistry revealed the neuroendocrine profile by positive reaction for chromogranin A and synaptophysin, with a ki-67 profile at an undetectable level. After surgery, the neuroendocrine markers and the octreoscan were negative, consequently indicating a favorable prognosis. Further follow-up is necessary, even though not all the authors recommend it (considering the low index to the associated metastases, especially for small appendiceal tumors). A short review of the literature is presented, starting with this case report.


Votanopoulos KI, Shen P, Stewart JH, Levine EA
Current status and future directions in appendiceal cancer with peritoneal dissemination.
Surg Oncol Clin N Am. 2012; 21(4):599-609 [PubMed]
This article focuses on the use of intraperitoneal hyperthermic chemotherapy for the treatment of peritoneal dissemination from appendiceal primary tumors. The first part of the article details patient selection criteria used at the Wake Forest University School of Medicine and the use of preoperative imaging and endoscopic evaluation in the management of this cohort of patients. The second part of the article focuses on clinical outcomes for patients undergoing hyperthermic intraperitoneal perfusion for peritoneal dissemination from appendiceal tumors. Finally, future challenges for the use of hyperthermic intraperitoneal perfusion for appendiceal primary tumors are explored.


Yildirim U, Ozaydin I, Erdem H, Uzunlar AK
Intussusception of the appendix induced by sessile serrated adenoma: a case report.
Turk Patoloji Derg. 2012; 28(3):290-2 [PubMed]
Intussusception of the appendix vermiformis is a rare condition. It occurs mainly in infants and children. Here, we report an intussusception case that occurred in a 65-year-old male presenting with repeated periumbilical pain, nausea, vomiting and febrile sensation. The appendix was seen to be intussuscepted at laparoscopy. The invaginated segment was reducted and simple appendicectomy was carried out. Histopathologic examination revealed a sessile serrated adenoma at the wall of the appendix, suggesting it as the cause of the intussusception.


Fajardo AD, Tan B, Reddy R, Fleshman J
Delayed repeated intraperitoneal chemotherapy after cytoreductive surgery for colorectal and appendiceal carcinomatosis.
Dis Colon Rectum. 2012; 55(10):1044-52 [PubMed]
BACKGROUND: Delayed repeated intraperitoneal chemotherapy after cytoreductive surgery for carcinomatosis may be an alternative to intraoperative hyperthermic infusion.
OBJECTIVE: The aim of this study was to evaluate the safety and feasibility of delayed repeated intraperitoneal chemotherapy after cytoreduction of colorectal and appendiceal carcinomatosis and pseudomyxoma peritonei.
DESIGN: This study constitutes a retrospective case series.
SETTING: This study was conducted at a single institution.
PATIENTS: A total of 31 patients with peritoneal carcinomatosis (23) and pseudomyxoma peritonei (8) were included.
INTERVENTIONS: Cytoreduction was followed by placement of an adhesion barrier and intraperitoneal catheters. Peritoneal scintigraphy preceded biweekly intraperitoneal 5-fluorouracil and systemic combination chemotherapy with leucovorin, fluorouracil, and oxaliplatin (FOLFOX).
MAIN OUTCOME MEASURES: The primary outcomes measured are safety, feasibility, and short-term survival.
RESULTS: Cytoreduction to a score of 0 to 1 was possible in 25 patients (80%). Complications occurred in 16 patients (51.6%) and were confined to grades I to III. There were no deaths, and no digestive fistulae occurred. Port malfunction or complication resulted in removal in 5 patients (16.1%). Intraperitoneal chemotherapy was possible in 83.8% of patients; 55% completed the full course. Peritoneal scintigraphy demonstrated free diffusion of tracer in 18 patients (58%), 4 (12.9%) had diffusion in each gutter with limited communication, 5 (16.1%) had limited diffusion around each catheter without communication, and 2 (6.5%) had no diffusion on scintigraphy. Overall survival for peritoneal carcinomatosis was 44.5% at 3 years (95% CI = 23%-65%).
LIMITATIONS: The nonrandomized nature of this study and the early experience are limitations.
CONCLUSIONS: Delayed repeated intraperitoneal and systemic chemotherapy after cytoreduction is feasible and has acceptable morbidity rates. Delayed intraperitoneal chemotherapy is possible in 83% of patients.


Boxberger N, Redlich A, Böger C, et al.
Neuroendocrine tumors of the appendix in children and adolescents.
Pediatr Blood Cancer. 2013; 60(1):65-70 [PubMed]
BACKGROUND: Neuroendocrine tumors (NET) of the appendix in children and adolescents are rare and mostly detected postoperatively by a histopathological examination. Since the malignant potential of these tumors remains unclear, therapeutic recommendations are not evidence based. The Society of Pediatric Oncology and Hematology (GPOH) has prospectively registered and followed children with appendical NET since 1997 (GPOH-MET trial). The objective of this study was to critically evaluate the therapeutic recommendations for appendical NET in children.
PROCEDURE: Clinical data of 237 children with appendical NET were prospectively analyzed.
RESULTS: The mean age at presentation was 13.0 years, while the mean follow-up time was 2.9 (0.0-12.8) years. The majority of tumors (70.9%) were located at the apex, and were smaller than or equal to 10 mm (72.7%). Tumor size was directly correlated with age. A second operation or primary lymph node sampling was done in 25.3% (N = 60) of the patients, and infiltration of the lymph nodes by tumor cells was found in nine of these patients (15%). All 237 patients are alive without tumor symptoms.
CONCLUSIONS: ROC analysis and subsequent calculations identified a tumor size of >15 mm as the optimal cut-off point for the prediction of metastatic spread into the lymph system, with a sensitivity of 77.8% and a specificity of 66.7%. Therefore, secondary right hemicolectomy in completely removed appendical NET is recommended only in tumors >15 mm in size. For incompletely removed tumors ≤15 mm a local follow-up resection with lymph node sampling is recommended.


Majumdar K, Sakhuja P, Kaur S, et al.
Inflammatory myofibroblastic tumor appendix with concomitant mucosal dysplasia, simulating pseudomyxoma on preoperative aspiration cytology.
J Cancer Res Ther. 2012 Apr-Jun; 8(2):317-9 [PubMed]
Inflammatory myofibroblastic tumor (IMT) has been described as a pseudosarcomatous proliferation of spindled myofibroblasts admixed with lymphoplasmacytic cells. The various terminologies like inflammatory pseudotumor, plasma cell granuloma, and inflammatory myofibrohistiocytic proliferation, used to describe this entity, highlight the controversial etiopathogenesis of this relatively indolent neoplasm. IMT has now been described in different anatomic locations. However, cases occurring in the gastrointestinal tract are rare with very few cases described in the appendix. We present a case of inflammatory myofibroblastic tumor appendix with mucosal dysplasia in a 41-year-old male, presenting with abdominal pain and lump in the right iliac fossa. Aspiration cytology yielded few atypical epithelial cells and spindle cells in a mucinous background, suggesting the possibility of pseudomyxoma peritonei. Awareness of IMT appendix with rare presence of mucosal dysplasia may help in preventing overzealous resection, especially in situations that on preoperative evaluation may suggest malignancy.


Guo AT, Li YM, Wei LX
Pseudomyxoma peritonei of 92 Chinese patients: clinical characteristics, pathological classification and prognostic factors.
World J Gastroenterol. 2012; 18(24):3081-8 [PubMed] Article available free on PMC after 05/03/2014
AIM: To assess the clinicopathologic features and its relationship with prognosis of pseudomyxoma peritonei (PMP) in Chinese patients.
METHODS: The clinicopathologic features and follow-up data of 92 patients with PMP were reviewed and retrospectively analyzed. The cases were categorized into three groups: disseminated peritoneal adenomucinosis (DPAM), peritoneal mucinous carcinomatosis (PMCA), and peritoneal mucinous carcinomatosis with intermediate or discordant features (PMCA-I/D). The log-rank test was used to analyze survival for each group and various clinicopathological parameters. Multivariate Cox proportional-hazard models were constructed to determine the important factors associated with survival.
RESULTS: The median age at diagnosis was 51.9 years (range: 22-76 years). The median follow up was 124 mo. The 3-, 5- and 10-year survival rates were 74.0%, 67.4% and 49.1%, respectively. There were 49 (53.2%) patients with DPAM, 26 (28.3%) with PMCA-I and 17 (18.5%) with PMCA. Patients with DPAM, PMCA-I/D and PMCA exhibited statistically significant difference in survival (P = 0.001). The 3 year survival for DPAM, PMCAI/D and PMCA was 97.0%, 80.0% and 67.0%, respectively; the 5 year survival was 80.0%, 67.0% and 50.0%, respectively; and the 10 year survival was 65.0%, 28.0% and 14.0%, respectively. Survival rate was significantly lowest in patients < 40 age years of age (P = 0.011). Appendiceal tumor and extra-ovarian parenchymal organ involvement were significantly related to overall survival. Patients with appendiceal mucinous adenocarcinoma (MACA) showed the significantly poorer prognosis (P = 0.011). Multivariate analysis showed that pathological classification, age, appendiceal tumor were significant related to overall survival.
CONCLUSION: The clinical process "PMP" should be pathologically classified into DPAM, PMCA and PMCA-I/D. Pathological classification, age, appendiceal MACA are survival independent predictors in Chinese patients with PMP.


Hsu C, Rashid A, Xing Y, et al.
Varying malignant potential of appendiceal neuroendocrine tumors: importance of histologic subtype.
J Surg Oncol. 2013; 107(2):136-43 [PubMed]
BACKGROUND: Neuroendocrine tumors (NETs) of the appendix include malignant carcinoid tumor (MCT), goblet cell carcinoid (GCT), and composite goblet cell carcinoid-adenocarcinoma (CGCC-A).
METHODS: We compared characteristics and outcomes of these histologic subtypes. Patients with appendiceal NETs were identified from the National Cancer Database (1998-2007). Descriptive statistics were used to compare cohorts and associations between clinicopathologic factors and overall survival (OS) were examined using Cox proportional hazards models.
RESULTS: A total of 2,812 patients with appendiceal NETs were identified. The most common histologic subtype was GCT (59.6%), followed by MCT (32.1%), CGCC-A (6.9%), and others (1.4%). CGCC-A had a significantly higher incidence of lymph node metastases (odds ratio [OR], 3.2; 95% confidence interval [CI], 2.1-4.8) and distant metastases (OR, 6.0; 95% CI = 3.8-9.3) than GCT. The 5-year OS was 86.3% (95% CI, 81.4-89.9) for MCT, 77.6% (95% CI, 74.0-80.8) for GCT, and 56.3% (95% CI, 42.1-68.4) for CGCC-A (P < 0.0001).
CONCLUSION: Appendiceal NETs represent a spectrum of disease with varying malignant potential: MCT (low), GCT (intermediate), and CGCC-A (high). GCTs represent the most common subtype, whereas CGCC-As place the patient at highest risk for regional and distant metastases and have the worst prognosis.


Chua TC, Chong CH, Liauw W, et al.
Inflammatory markers in blood and serum tumor markers predict survival in patients with epithelial appendiceal neoplasms undergoing surgical cytoreduction and intraperitoneal chemotherapy.
Ann Surg. 2012; 256(2):342-9 [PubMed]
BACKGROUND: The study examines the role inflammatory and tumor markers as biomarkers to preoperatively predict outcome in patients with epithelial appendiceal neoplasm undergoing cytoreduction and intraperitoneal chemotherapy.
METHODS: Associations between baseline variables, tumor markers [CEA (carcinoembyronic antigen], CA125, CA199), inflammatory markers including neutrophils-lymphocyte ratio (NLR), platelet-lymphocyte ratio (PLR), and C-reactive protein (CRP) with progression-free survival (PFS) and overall survival (OS) were examined in patients undergoing surgical cytoreduction and intraperitoneal chemotherapy for epithelial appendiceal neoplasm.
RESULTS: A total of 174 patients with epithelial appendiceal neoplasm (low-grade pseudomyxoma, n = 117; appendiceal cancer, n = 57) underwent cytoreduction. On univariate analysis, all 3 inflammatory and tumor markers predicted for both PFS and OS, respectively; NLR ≤ 2.6 (P = 0.01, P = 0.002), PLR ≤ 166 (P = 0.006, P = 0.016), CRP ≤ 12.5 (P = 0.001, P = 0.008), CEA (P < 0.001, P = 0.001), CA125 (P = 0.004, P < 0.001), CA199 (P < 0.001, P < 0.001). On multivariate analysis, there were no independent predictors of OS. PFS was independently associated with the presence of lymph node metastasis (P = 0.02), CA199 > 37 (P = 0.003), and a CRP > 12.5 (P = 0.013). A higher peritoneal cancer index (PCI > 24) was associated with elevation in CEA > 12, CA125 > 39, CA199 > 37, PLR > 166 and CRP > 12. The tumor histologic subtype was associated with CA 199 levels.
CONCLUSIONS: The results from this investigation suggest that preoperative inflammatory markers in blood and serologic tumor markers may predict outcomes and are associated with tumor biology in patients with epithelial appendiceal neoplasm undergoing cytoreduction and intraperitoneal chemotherapy treatment.


El Halabi H, MacDonald R, Studeman K, et al.
Delay of cytoreductive surgery and heated intraperitoneal chemotherapy in patients with appendiceal neoplasm.
Am Surg. 2012; 78(7):745-8 [PubMed]
Cytoreductive surgery/heated intraperitoneal chemotherapy (CRS/HIPEC) has been shown to be effective for selected patients with advanced appendiceal cancer. We propose that delaying CRS/HIPEC leads to disease progression and affects outcome. A retrospective analysis of a prospective database was carried out. Patients were divided into two groups based on time from diagnosis to CRS/HIPEC (less than 6 months = early, greater than 6 months = delayed). Comparison was made of Peritoneal Cancer Index (PCI), Prior Surgery Score (PSS), complete cytoreduction (CC), and lymph node status. Overall survival (OS) was calculated using Kaplan-Meier estimates. Of 127 patients, 50 had disseminated peritoneal adenomucinosis and 77 had peritoneal mucinous carcinomatosis (PMCA). Of patients with PMCA, 41 had early CRS/HIPEC and 36 delayed. PCI was less than 20 in 46 and 17 per cent (P = 0.007) of the early and delayed groups, respectively. CC was achieved in 88 and 61 per cent (P = 0.009) of the early and delayed groups, respectively. PSS was (2 of 3) in 51 and 91 per cent (P = 0.001) of the early and delayed groups, respectively. Five-year OS was 54 per cent for the early group and 45 per cent for the delayed group (P = 0.2). Delaying CRS/HIPEC was associated with higher tumor load and lower chance for complete cytroreduction. Longer follow-up and larger numbers are needed to determine if OS difference will reach statistical significance.


Jiang Y, Long H, Li T, et al.
Schistosomiasis may contribute to goblet cell carcinoid of the appendix.
J Parasitol. 2012; 98(3):565-8 [PubMed]
Abstract : To investigate whether schistosomiasis can contribute to appendiceal goblet cell carcinoid, appendix samples were obtained from 3 patients with combined appendiceal schistosomiasis and goblet cell carcinoid (CSG), 6 patients with goblet cell carcinoid only (GCC), 12 patients with appendiceal schistosomiasis only (ASO), and 12 cases with normal appendix (NA), all of similar gender ratio and age distributions. Hematoxylin and eosin-(H&E) stained sections were studied in 3 CSGs and 12 ASOs to diagnose schistosomiasis by detecting schistosome eggs. H&E and alcian blue/PAS-stained sections and immunohistochemistry of CgA and CEA were employed to establish the diagnosis of GCC in the 3 CSGs and 6 GCCs. Then, to determine whether schistosomiasis can contribute to GCC, immunostaining patterns of CgA and Ki67 in mucosal crypt epithelia were investigated and compared among all 33 cases. Our results revealed typical histological and immunohistochemical phenotypes of GCC in the 3 CSGs and 6 GCCs and schistosome egg deposits in 3 CSGs and 12 ASOs. We found that the expression levels of both CgA and Ki67 in mucosal crypt epithelia were significantly higher in CSG than in GCC (P < 0.05  =  0.013 and P  =  0.004, respectively). Moreover, high expression levels of both CgA and Ki67 in mucosal crypt epithelia favor ASO as compared to NA (P < 0.001  =  3.4 × 10(-6) and 3.1 × 10(-5), respectively). Our findings suggest that appendiceal schistosomiasis was associated with increased proliferation and neuroendocrine differentiation of mucosal pluripotent crypt cells and that it may contribute to GCC, which is documented to originate from mucosal pluripotent crypt cells in mucosal crypt epithelia.


Veerapong J, Helm CW, Solomon H
Resection of tumor from the supragastric lesser sac with peritonectomy.
Gynecol Oncol. 2012; 127(1):256 [PubMed]
This 48 yr old lady underwent laparotomy for primary appendiceal carcinoma metastatic within the peritoneal cavity including the lesser omentum (LO) and supragastric lesser sac (Fig. 1). The left triangular ligament was divided allowing retraction of the left lobe of the liver. The stomachwasmanually pulled to stretch out the LO and facilitate resection. The left gastric, common hepatic and left hepatic arteries and the vagal nerves of Latarjet running along the lesser curve of the stomachwere avoided. Tumorwasmobilized frombetween the left liver and anterior caudate lobe and from behind the pont hepatique. Care was taken to avoid damage to a branch of the left hepatic artery running in the roof of the lesser sac. The stomach was elevated and the caudate lobe carefully retracted to expose the posterior surface of the supragastric lesser sac formed by a single layer of peritoneum. This was stripped off and then detached from the caudate lobe. Tumor was then stripped or wiped off the anterior surface of the caudate lobe. Residual visible tumor was ablated. At the end of the procedure there was no visible disease. The patientwas then treatedwith hyperthermic intraperitoneal chemotherapy with mitomycin for 90min. The postoperative course was uncomplicated apart from short-term ileus and urinary retention.


Mathur A, Steffensen TS, Paidas CN, et al.
The perforated appendiceal carcinoid in children: a surgical dilemma.
J Pediatr Surg. 2012; 47(6):1155-8 [PubMed]
The decision for aggressive reoperation after discovery of an appendiceal carcinoid is generally based upon criteria such as size, grade, degree of involvement of the mesoappendix or the appendiceal base, lymphovascular invasion, and the presence of goblet cell or adenocarcinoid features. No guidelines currently exist for the management of perforated appendiceal carcinoids. We present a case of perforated appendiceal carcinoid that was subsequently treated with right hemicolectomy, and we review the pertinent literature.


Chua TC, Moran BJ, Sugarbaker PH, et al.
Early- and long-term outcome data of patients with pseudomyxoma peritonei from appendiceal origin treated by a strategy of cytoreductive surgery and hyperthermic intraperitoneal chemotherapy.
J Clin Oncol. 2012; 30(20):2449-56 [PubMed]
PURPOSE: Pseudomyxoma peritonei (PMP) originating from an appendiceal mucinous neoplasm remains a biologically heterogeneous disease. The purpose of our study was to evaluate outcome and long-term survival after cytoreductive surgery (CRS) and hyperthermic intraperitoneal chemotherapy (HIPEC) consolidated through an international registry study.
PATIENTS AND METHODS: A retrospective multi-institutional registry was established through collaborative efforts of participating units affiliated with the Peritoneal Surface Oncology Group International.
RESULTS: Two thousand two hundred ninety-eight patients from 16 specialized units underwent CRS for PMP. Treatment-related mortality was 2% and major operative complications occurred in 24% of patients. The median survival rate was 196 months (16.3 years) and the median progression-free survival rate was 98 months (8.2 years), with 10- and 15-year survival rates of 63% and 59%, respectively. Multivariate analysis identified prior chemotherapy treatment (P < .001), peritoneal mucinous carcinomatosis (PMCA) histopathologic subtype (P < .001), major postoperative complications (P = .008), high peritoneal cancer index (P = .013), debulking surgery (completeness of cytoreduction [CCR], 2 or 3; P < .001), and not using HIPEC (P = .030) as independent predictors for a poorer progression-free survival. Older age (P = .006), major postoperative complications (P < .001), debulking surgery (CCR 2 or 3; P < .001), prior chemotherapy treatment (P = .001), and PMCA histopathologic subtype (P < .001) were independent predictors of a poorer overall survival.
CONCLUSION: The combined modality strategy for PMP may be performed safely with acceptable morbidity and mortality in a specialized unit setting with 63% of patients surviving beyond 10 years. Minimizing nondefinitive operative and systemic chemotherapy treatments before definitive cytoreduction may facilitate the feasibility and improve the outcome of this therapy to achieve long-term survival. Optimal cytoreduction achieves the best outcomes.


Nikolic O, Djurdjevic S, Stojanovic S, et al.
Pseudomyxoma peritonei--case report.
Eur J Gynaecol Oncol. 2012; 33(2):227-9 [PubMed]
The syndrome pseudomyxoma peritonei is rare, present in only 2/10,000 laparotomies. We report the case of a 58-year-old woman with a primary tumor of the appendix, and secondary involvement of other structures and organs of the abdominal cavity. In our case, we performed maximal surgical reduction of the tumor, with remaining implants on diaphragmatic domes and liver, as we did not have technical conditions to safely perform prolonged surgery which would have included a surgical procedure on the liver and administration of intraoperative chemotherapy. The patient underwent six series of parenteral chemotherapy, but refused the second-look surgery. Even though our patient did not receive intraperitoneal chemotherapy, maximal surgical tumor reduction, and refused second-look surgery, she is still alive and without any major complaints two years after the surgery.


Addison AB, Miller K, Hammouch D, et al.
Appendiceal metastasis 10 years following 'curative' resection for low-grade primary endometrial carcinoma.
BMJ Case Rep. 2012; 2012 [PubMed]
Metastasis of primary endometrial adenocarcinoma to unusual sites has been occasionally reported. However, the authors believe this to be the first case report of metastasis to the appendix. This occurred more than 10 years after curative resection, and presented as sepsis with an intra-abdominal focus.


Hayashi K, Takamura M, Sato Y, et al.
Primary malignant mesothelioma of the appendix.
Intern Med. 2012; 51(9):1027-30 [PubMed]
We report a case of primary malignant mesothelioma of the appendix. A 35-year-old man without any history of asbestos exposure was admitted to our hospital for further examination following the discovery of multiple liver tumors, an ileocecal tumor, and abdominal lymph node swelling. An ultrasound-guided liver tumor biopsy revealed malignant mesothelioma. Despite receiving systemic chemotherapy, he died 3 months after the initial diagnosis. At autopsy, a diagnosis of multiple organ metastases from a malignant biphasic mesothelioma of the appendix was made. To our knowledge, this is only the second reported case of primary malignant mesothelioma of the appendix.


McDonald JR, O'Dwyer ST, Rout S, et al.
Classification of and cytoreductive surgery for low-grade appendiceal mucinous neoplasms.
Br J Surg. 2012; 99(7):987-92 [PubMed]
BACKGROUND: Low-grade appendiceal mucinous neoplasm (LAMN) is a precursor lesion for pseudomyxoma peritonei (PMP), which, if treated suboptimally, may later disseminate throughout the abdominal cavity. The role of cytoreductive surgery for these relatively early lesions is unclear.
METHODS: Clinicopathological details and treatment outcomes of patients with a LAMN and disease limited to the appendix or immediate periappendiceal tissues, referred to a national treatment centre between 2002 and 2009, were evaluated prospectively.
RESULTS: Of 379 patients with a diagnosis of PMP, 43 (median age 49 years) had LAMNs localized to the appendix and periappendiceal tissue. Thirty-two patients initially presented with symptoms of acute appendicitis or right iliac fossa pain. Two distinct lesions were identified: type I (disease confined to the appendiceal lumen) and type II (mucin and/or neoplastic epithelium in the appendiceal submucosa, wall and/or periappendiceal tissue, with or without perforation). Type I lesions were managed by a watch-and-wait surveillance policy with serial measurement of tumour markers and computed tomography in 14 of 16 patients. Seventeen of 27 patients with type II lesions underwent risk-reducing cytoreductive surgery and hyperthermic intraperitoneal chemotherapy with low morbidity. After a median follow-up of 40 months, there was no disease progression in either treatment pathway.
CONCLUSION: This study identified two LAMN subtypes. Type II lesions have pathological features of increased risk for dissemination and should be considered for risk-reducing cytoreductive surgery.


Kallenbach K, Hjorth SV, Engel U, et al.
Significance of acquired diverticular disease of the vermiform appendix: a marker of regional neoplasms?
J Clin Pathol. 2012; 65(7):638-42 [PubMed]
AIM: To assess the prevalence of acquired diverticulum of the appendix (DA), including incipient forms and its possible significance as a marker of local/regional neoplasms.
MATERIALS AND METHODS: The pathology database at Hvidovre Hospital was searched for appendix specimens, received between 2001 and 2010, coded for DA or for a space-occupying lesion. Slides were reviewed to determine DA status and the nature of lesions possibly causing DA.
RESULT: Among 4413 appendix specimens, DA were identified in 39 (0.9%, CI 0.6% to 1.2%) cases, 17 (43.6%, 28.0% to 59.2%) of which additionally harboured an appendiceal neoplasm/neoplastic precursor, whereas this figure was 1.2% (CI 0.9% to 1.6%) for non-DA specimens (p<0.0001). Six of the 39 DA specimens comprised incipient DA, three of which coexisted with appendiceal neoplasms. In addition, local/regional non-neoplastic lesions (six cases) and colorectal carcinomas (four cases) coexisted with DA.
CONCLUSION: DA has significance as a putative marker of local/regional neoplasms. Therefore, a DA specimen proved significantly more likely to harbour a neoplastic growth than a non-DA counterpart. Submission for microscopy of the entire DA specimen, whether transmural or only incipient, and a comment in the pathology report on the occasional concurrence of local/regional neoplasms in this setting seem appropriate. The observation of DA may thus provide a valuable contribution in the diagnostic process.


This page last updated: 22nd May 2013
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