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At least 19 recordsLinked to original sources
PubMed2006

Management of an unexpected appendiceal neoplasm.

BACKGROUND: Appendiceal neoplasms are rare and most present unexpectedly as acute appendicitis. The classification and management are confusing, and there are few substantial reports in the literature. METHODS: A systematic literature review was performed to access relevant publications on the presentation, pathology and management of appendiceal tumours. RESULTS: Appendiceal tumours account for 0.4 to 1 per cent of all gastrointestinal tract malignancies and are found in 0.7 to 1.7 per cent of appendicectomy specimens. Carcinoid tumours are most common. Most are cured by simple appendicectomy if the tumour is less than 2 cm in size and does not involve the resection margin or mesoappendix. Epithelial tumours may present with, or in time develop, pseudomyxoma peritonei, the optimal management of which involves complete tumour resection and intraperitoneal chemotherapy, usually available only in specialized centres. CONCLUSION: Suggested algorithms for the management of unexpected appendiceal tumours are provided. Recommendations are made for follow-up of patients with a perforated appendiceal epithelial tumour.

Appendectomy↗
PubMed2007

Early results of surgery in 123 patients with pseudomyxoma peritonei from a perforated appendiceal neoplasm.

PURPOSE: Epithelial appendiceal tumors are uncommon but can present as an emergency simulating appendicitis, or unexpectedly at laparotomy, laparoscopy, or on cross-sectional imaging. Occult rupture with features of pseudomyxoma peritonei may be encountered. We report the operative findings, pathologic assessment, and early outcomes in 123 consecutive patients with a perforated appendiceal neoplasm presenting as pseudomyxoma peritonei. METHODS: From March 1994 to March 2004, 292 patients were referred to a peritoneal malignancy surgical treatment center. Complete tumor removal (cytoreduction) was attempted in selected patients and, if achieved, surgery was combined with intraoperative, intraperitoneal mitomycin C (10 mg/m(2)). RESULTS: In total, 123 patients (52 males; 41 percent) underwent laparotomy for a perforated appendiceal malignancy presenting as pseudomyxoma peritonei. The median age was 52 (range 30-77) years. Complete cytoreduction was achieved in 83 of 123 patients (67 percent), major palliative resection in 34 patients (28 percent), and 6 patients (5 percent) were inoperable. Postoperative mortality was 6 of 123 patients (5 percent). Kaplan-Meier analysis of the 83 patients who had complete tumor removal predicted 75 percent disease-free survival at five years. CONCLUSIONS: A perforated appendiceal epithelial tumor most frequently presents as pseudomyxoma peritonei. This treatment strategy, involving surgery and intraperitoneal chemotherapy, can result in good outcomes in this rare and otherwise fatal disease.

Adult↗
PubMed2004

Pseudomyxoma peritonei and selected other aspects of the spread of appendiceal neoplasms.

The varied aspects of spread of appendiceal neoplasms are reviewed with emphasis on the often clinically dramatic phenomenon known as pseudomyxoma peritonei, a term mostly used to describe grossly evident mucin within the peritoneal cavity. The majority of cases of pseudomyxoma peritonei result from tumors primary in the appendix, which are usually low-grade. On microscopic examination pseudomyxoma peritonei is typically characterized by large aggregates of mucin which may be relatively acellular or cellular containing strips of mucinous epithelium, mucinous epithelium encircling glands and cysts, or aggregates of mucinous epithelium lying within mucin pools. High-grade adenocarcinoma of the appendix may spread to the omentum and peritoneal surfaces without grossly striking mucin deposition and resemble spread of other high-grade gastrointestinal adenocarcinomas. In many cases of pseudomyxoma peritonei in females there is involvement of one, or more often, both ovaries. The size of the ovarian neoplasms characteristically dwarfs the often relatively unremarkable appearing appendix in these cases. The ovaries are typically multilocular, although one locule may dominate, and in cases in which the primary is a low-grade appendiceal mucinous neoplasm often have a "jelly-like" consistency. In cases of spread of frank adenocarcinomas the ovarian metastases typically have a more solid, albeit still somewhat gelatinous consistency. Microscopic examination of the ovaries typically shows surface involvement, a characteristic of spread to the ovaries in general, and the glands and cysts that replace most or all of the parenchyma are typically lined by tall mucin-rich cells with, in many cases, relatively bland microscopic features. In cases of frank adenocarcinoma, the tumors may mimic closely a primary mucinous adenocarcinoma of the ovary. Spread to the ovaries may also be seen in cases of frank intestinal-type adenocarcinoma primary in the appendix and the uncommon signet ring cell carcinoma of the appendix, the latter being one cause of the Krukenberg tumor. Occasional cases are reported in the literature of ovarian spread of goblet cell carcinoid tumor of the appendix, but in our opinion most of the primary tumors in those cases are better classified as adenocarcinomas, usually dominantly of signet-ring cell type, albeit sometimes with focal neuroendocrine differentiation. Other interesting aspects of spread of appendiceal neoplasms include to the lining of the uterus and the fallopian tube. In yet other cases the tumors may present clinically as incidentally discovered mucinous aggregates within hernia sac specimens or as a scrotal mass.

Appendiceal Neoplasms↗
PubMed2006

Appendiceal neoplasms with peritoneal dissemination: outcomes after cytoreductive surgery and intraperitoneal hyperthermic chemotherapy.

BACKGROUND: Appendiceal neoplasms frequently present with peritoneal dissemination (PD) and have a clinical course marked by bowel obstruction and subsequent death. Few data have correlated outcome with appendiceal histology after cytoreductive surgery and intraperitoneal hyperthermic chemotherapy (IPHC). We have reviewed our experience with cytoreductive surgery and IPHC for PD from the appendix. METHODS: A total of 110 cases of PD from proven appendiceal neoplasms treated with IPHC were identified from a prospectively managed database. Tumor samples were classified on pathologic review as disseminated peritoneal adenomucinosis (n = 55), peritoneal mucinous carcinomatosis (PMCA) with intermediate features (n = 18), PMCA (n = 29), or high-grade nonmucinous lesions (n = 8). A retrospective review was performed with long-term survival as the primary outcome measure. RESULTS: A total of 116 IPHCs were performed on 110 patients for appendiceal PD between 1993 and 2004. The 1-, 3-, and 5-year survival rates for all cases were 79.9% +/- 4.1%, 59.0% +/- 5.7%, and 53.4% +/- 6.5%, respectively. When stratified by histology, disseminated peritoneal adenomucinosis and intermediate tumors had better 3-year survival rates (77% +/- 7% and 81% +/- 10%) than PMCA and high-grade nonmucinous lesions (35% +/- 10% and 15% +/- 14%; P = .0032 for test of differences between groups). Age at presentation (P = .0134), performance status (P < .0001), time between diagnosis and IPHC (P = .0011), resection status (P = .0044), and length of hyperthermic chemoperfusion (P = .0193) were independently associated with survival. CONCLUSIONS: The data show that long-term survival is anticipated in most patients who are treated with cytoreduction and IPHC for appendiceal PD. The findings presented herein underscore the important prognostic characteristics that predict outcome after IPHC in patients with PD. In all, this work establishes a framework for the consideration of IPHC in future trials for appendiceal PD.

Adenocarcinoma, Mucinous↗
PubMed1986

Mucinous adenocarcinoma of the appendix presenting as an ovarian cystadenocarcinoma: case report and review of appendiceal neoplasms with ovarian metastases.

Primary adenocarcinoma of the vermiform appendix is a rare clinical entity that is virtually never diagnosed preoperatively. A case of mucin-producing adenocarcinoma of the appendix manifesting as a pelvic mass is presented. The ultrasonographic finding of a multilocular cystic lesion with thick septa and solid components was consistent with an ovarian cystadenocarcinoma. A review of primary appendiceal neoplasms with ovarian metastases is given.

Adenocarcinoma, Mucinous↗
PubMed1993

Primary appendiceal neoplasms.

A retrospective review of the files of 1,740 appendectomies performed during a 10-year period disclosed 13 patients (0.7%) with primary appendiceal tumors. Adenocarcinoma of the appendix was diagnosed in 6 patients (0.3%), which is a two- to four-fold higher incidence than reported in the literature. The other seven patients had benign tumors and only appendectomy was performed. The female to male ratio of adenocarcinoma was 5:1. All six adenocarcinoma patients, classified histologically as Dukes' B stage, underwent right hemicolectomy, and were disease free following a mean follow-up of 35 months. The results of our policy, also recommended by others, confirmed the benefit of right hemicolectomy in all patients with confirmed primary appendiceal carcinomas.

Adenocarcinoma↗
PubMed2006

New standard of care for appendiceal epithelial neoplasms and pseudomyxoma peritonei syndrome?

Appendiceal mucinous neoplasms sometimes present with peritoneal dissemination, which was previously a lethal condition with a median survival of about 3 years. Traditionally, surgical treatment consisted of debulking that was repeated until no further benefit could be achieved; systemic chemotherapy was sometimes used as a palliative option. Now, visible disease tends to be removed through visceral resections and peritonectomy. To avoid entrapment of tumour cells at operative sites and to destroy small residual mucinous tumour nodules, cytoreductive surgery is combined with intraperitoneal chemotherapy with mitomycin at 42 degrees C. Fluorouracil is then given postoperatively for 5 days. If the mucinous neoplasm is minimally invasive and cytoreduction complete, these treatments result in a 20-year survival of 70%. In the absence of a phase III study, this new combined treatment should be regarded as the standard of care for epithelial appendiceal neoplasms and pseudomyxoma peritonei syndrome.

Antineoplastic Combined Chemotherapy Protocols↗
PubMed2006

The natural history, gross pathology, and histopathology of appendiceal epithelial neoplasms.

AIM: To report the pathology of appendiceal epithelial neoplasms a review of the Washington Cancer Institute experience and a PubMed literature search was performed. FINDINGS: Epithelial appendiceal neoplasms present a significant oncologic challenge. The clinical and pathologic features are unusually varied as compared to other gastrointestinal cancers and require expert clinical judgment for proper management. CONCLUSIONS: Appendiceal epithelial neoplasms should be regarded as a distinct clinical entity with special clinical treatments used for optimal management.

Adenocarcinoma, Mucinous↗
PubMed2003

Appendiceal mucinous neoplasms: a clinicopathologic analysis of 107 cases.

The classification of appendiceal mucinous tumors is controversial and terminology used for them inconsistent, particularly when they lack overtly malignant features but are associated with extra-appendiceal spread. We reviewed 107 appendiceal mucinous neoplasms and classified them as low-grade appendiceal mucinous neoplasm (LAMN) (n = 88), mucinous adenocarcinomas (MACAs) (n = 16), or discordant (n = 3) based on architectural and cytologic features. LAMNs were characterized by a villous or flat proliferation of mucinous epithelium with low-grade atypia. Thirty-nine tumors were confined to the appendix, but 49 had extra-appendiceal tumor spread, including 39 with peritoneal tumor characterized by mucin pools harboring low-grade mucinous epithelium, usually dissecting in a hyalinized stroma. Eight of the 16 MACAs lacked destructive invasion of the appendiceal wall and eight showed an infiltrative pattern of invasion. Extra-appendiceal tumor spread was present in 12 MACAs (four peritoneum, seven peritoneum and ovaries; one ovaries only). In MACAs with an infiltrative pattern, peritoneal tumor consisted of glands and single cells in a desmoplastic stroma. The peritoneal tumor in the remaining cases consisted of mucin pools that contained mucinous epithelium with high-grade atypia and, in some cases, increased cellularity compared with that seen in peritoneal spread in cases of LAMN. Three cases were classified as discordant because the appendiceal tumors were LAMNs but the peritoneal tumors were high-grade. Follow-up was available for 49 LAMNs, 15 MACAs, and 2 discordant cases. None of the patients with LAMNs confined to the appendix experienced recurrence (median follow-up 6 years). LAMNs with extra-appendiceal spread were associated with 3-, 5-, and 10-year survival rates of 100%, 86%, and 45%, respectively. Patients with MACA had 3- and 5-year survival rates of 90% and 44%, respectively (p = 0.04). The bulk of peritoneal disease correlated with prognosis among patients with MACA (p = 0.04) and, to a lesser degree, among patients with LAMNs (p = 0.07). We conclude that: 1) appendiceal mucinous neoplasms can be classified as either low-grade mucinous neoplasms or mucinous adenocarcinoma based on architectural and cytologic features; 2) tumors that can be confidently placed in the low-grade group (which requires rigorous pathologic evaluation of the appendix) and are confined to the appendix are clinically benign in our experience to date; 3) low-grade tumors confined to the appendix are morphologically identical to those with extra-appendiceal spread (except for the usual identification of breach of the wall in the latter cases) and the same designation is appropriate for the appendiceal neoplasia in each situation; 4) the long-term outlook for patients with low-grade tumors and peritoneal spread is guarded with just over half dying of disease after 10 years; 5) appendiceal mucinous tumors with destructive invasion of the appendiceal wall, complex epithelial proliferations, or high-grade nuclear atypia generally pursue an aggressive clinical course and should be classified as mucinous adenocarcinomas; 6) peritoneal tumor can be classified as involvement by LAMN or MACA, and this distinction is of prognostic significance; 7) bulky peritoneal tumor worsens prognosis; and 8) LAMNs associated with high-grade peritoneal tumor behave as adenocarcinoma.

Adenocarcinoma, Mucinous↗
PubMed2004

Defective mismatch repair in the pathogenesis of low-grade appendiceal mucinous neoplasms and adenocarcinomas.

Defective DNA mismatch repair has been proposed as a second pathway for colonic carcinogenesis, particularly in tumors arising in the right colon. We investigated whether tumors arising in the appendix are associated with defective DNA mismatch repair using immunohistochemistry for mismatch repair enzymes hMLH-1, hMSH-2, hMSH-6, and hPMS-2. These immunoassays have been shown to be highly sensitive and specific for defective DNA mismatch repair in sporadic and familial adenocarcinomas. Sporadic adenocarcinomas with defective DNA mismatch repair essentially always show loss of hMLH-1, while loss of hMSH-2, hMSH-6, or hPMS-2 is almost always due to germline mutation. In all, 35 cases of appendiceal epithelial neoplasms were evaluated, comprising 18 low-grade appendiceal mucinous neoplasms confined to the appendix; eight low-grade appendiceal mucinous neoplasms with extra-appendiceal spread (five peritoneum and ovaries, two peritoneum, one ovaries only); and nine invasive adenocarcinomas (three with metastatic disease). All immunohistochemical slides were reviewed by two pathologists. One (11%) invasive adenocarcinoma showed absent expression of hMSH-2 and hMSH-6, but preserved hMLH-1 and hPMS-2 expression. This case was a 26-year-old female with a history of synovial sarcoma who presented with acute appendicitis and appendiceal perforation (median age for other invasive carcinomas, 62 years; range 38-76 years). The appendiceal tumor was a moderately differentiated, colonic-type adenocarcinoma without significant extracellular mucin or tumor-infiltrating lymphocytes. The remaining invasive carcinomas and low-grade appendiceal mucinous neoplasms demonstrated preserved expression of all mismatch repair enzymes, including the seven cases in which extra-appendiceal tumor was also evaluated. We conclude that defective DNA mismatch repair does not play a role in the pathogenesis of low-grade appendiceal mucinous neoplasms. Defective DNA mismatch was found in 11% of invasive carcinomas, likely due to a germline mutation. These findings suggest that sporadic appendiceal neoplasia rarely arises through the defective DNA mismatch repair (mutator) pathway.

Adaptor Proteins, Signal Transducing↗
PubMedDate not supplied

Primary neoplasms of the appendix: radiologic spectrum of disease with pathologic correlation.

Although uncommon, primary appendiceal neoplasms often result in clinical symptoms that may lead to abdominal imaging. Acute appendicitis from luminal obstruction is the most common manifestation for most tumor types. Other manifestations include intussusception, a palpable mass, gastrointestinal bleeding, increasing abdominal girth (from pseudomyxoma peritonei), and secondary genitourinary complications. Asymptomatic appendiceal neoplasms may be discovered incidentally. Mucoceles from either benign or malignant mucinous neoplasms represent the majority of appendiceal tumors detected at imaging but are the least likely to manifest as appendicitis. Pseudomyxoma peritonei is a common manifestation of mucinous adenocarcinoma. Colonic-type (nonmucinous) adenocarcinoma of the appendix is much less common than mucinous tumors and typically manifests as a focal mass without mucocele formation. Carcinoid tumor is the most common appendiceal neoplasm but is less often detected radiologically because it is typically small and relatively asymptomatic. Goblet cell carcinoid tumor and non-Hodgkin lymphoma of the appendix are rare and usually infiltrate the entire appendix. Cross-sectional imaging, particularly computed tomography (CT), is effective in the evaluation of these neoplasms. CT appears to be the modality of choice whenever an appendiceal mass is suspected. CT will help rule out or confirm an appendiceal tumor and may suggest a more specific diagnosis.

Appendiceal Neoplasms↗
PubMed2000

Epithelial cells and other cytologic features of pseudomyxoma peritonei in patients with ovarian and/or appendiceal mucinous neoplasms: a study of 12 patients including 5 men.

BACKGROUND: Pseudomyxoma peritonei (PP) is a rare condition. Cytologic evaluation of peritoneal fluid often is an initial diagnostic test for possible ovarian and/or appendiceal primary tumors. Previous studies suggest that patients with PP who have epithelial cells (ECs) in their peritoneal fluid usually have a less favorable prognosis than patients with acellular PP. To the authors' knowledge, few reports of PP in the cytologic literature cite the presence of ECs. METHODS: Twelve cases of PP diagnosed by cytologic examination at the University of Texas M. D. Anderson Cancer Center over 15 years were identified. In all cases, primary tumors were confirmed histologically. All available cytologic smears and cell block sections were reviewed for cytomorphology, with particular attention given to the presence of ECs. A correlation between the presence of ECs and patient outcome also was sought (median follow-up, 26 months). RESULTS: Two patients had ovarian neoplasms, six patients (one female and five males) had appendiceal neoplasms, and four patients had synchronous ovarian and appendiceal tumors. Cytologic features included mucin pools (12 of 12 patients), ECs (11 of 12 patients), mesothelial or mesothelial-like cells (10 of 12 patients), histiocytes (11 of 12 patients), and fibroblast-like or spindle cells (6 of 12 patients). ECs were columnar with mucinous features in the majority of cases, and the number of ECs in each case was variable, ranging from 1+ (rare) to 3+ (many). Of the 11 patients with available follow-up data, 6 had recurrent disease, 4 had persistent disease, and 1 patient with acellular PP was alive without clinical evidence of disease after 24 months of follow-up. CONCLUSIONS: Unlike previous PP cytology reports, the current study frequently identified ECs (92%). Because of the potential prognostic implication of ECs in patients with PP, a diligent search for ECs is warranted. Indication of the presence or absence of ECs in the cytology report may be useful when PP is diagnosed.

Adenocarcinoma, Mucinous↗
PubMed2006

Cytoreductive surgery and perioperative intraperitoneal chemotherapy for pseudomyxoma peritonei from appendiceal mucinous neoplasms.

BACKGROUND: Cytoreductive surgery (CRS) combined with perioperative intraperitoneal chemotherapy (PIC) has been used to treat pseudomyxoma peritonei. The aim of this prospective study was to evaluate survival outcome and treatment-related prognostic markers in patients who underwent CRS and PIC for pseudomyxoma peritonei from appendiceal mucinous neoplasms. METHODS: Survival data and 12 clinicopathological and treatment-related prognostic variables for survival were obtained prospectively in 50 consecutive patients (23 men). Univariate analysis was used to determine their prognostic significance for overall survival, determined from the time of CRS. RESULTS: The mean(s.d.) age was 52(12) years. Eighteen patients had moderate complications, and six patients had severe complications that required operation or intensive care support. Two patients died after surgery. The actuarial 5-year survival rate was 69 per cent. Univariate analysis demonstrated that the extent of previous surgery (P = 0.045) and Ronnett's histopathological classification (P < 0.001) were significantly related to overall survival. CONCLUSION: CRS combined with PIC was associated with improved survival in patients with less extensive previous surgery and diffuse peritoneal adenomucinosis histopathological type.

Adenocarcinoma, Mucinous↗
PubMed2002

Primary neoplasms of the appendix manifesting as acute appendicitis: CT findings with pathologic comparison.

PURPOSE: To evaluate the computed tomographic (CT) findings in patients with acute appendicitis related to an underlying appendiceal neoplasm. MATERIALS AND METHODS: Clinical and pathologic data obtained over a 10-year period in 65 patients with primary appendiceal neoplasms were reviewed. Preoperative CT studies in patients who presented clinically with symptoms of acute appendicitis were analyzed retrospectively and in consensus by three radiologists. The appendix was evaluated on CT scans for morphology, location, presence of calcification, maximal diameter, and wall thickness. RESULTS: Twenty-six (40%) of the 65 patients with appendiceal neoplasms had presented with symptoms of acute appendicitis. Preoperative CT studies available in 22 patients showed increased appendiceal diameter, wall thickening, and periappendiceal fat stranding in 22 (100%), 22 (100%), and 21 (95%) patients, respectively. The appendiceal diameter was greater than 15 mm (mean diameter, 2.9 cm) in 19 patients (86%). Morphologic changes of concern for neoplasm were present in 19 patients (86%) and included cystic dilatation in nine patients and presence of a soft-tissue mass without dilatation in 10 patients. An appendiceal diameter greater than 15 mm and/or a morphologic abnormality were present in 21 of 22 cases (95%). CONCLUSION: CT findings strongly suggest the presence of underlying neoplasm in the majority of patients with secondary appendicitis.

Acute Disease↗
PubMed2001

Second-look surgery in patients with peritoneal dissemination from appendiceal malignancy: analysis of prognostic factors in 98 patients.

OBJECTIVE: To establish selection criteria for reoperation in patients with peritoneal dissemination from appendiceal malignancy. SUMMARY BACKGROUND DATA: The outcome of patients with mucinous appendiceal neoplasms with peritoneal surface dissemination has changed as a result of a better understanding of the clinical and pathologic features of this disease. This knowledge, combined with aggressive strategies for the use of perioperative intraperitoneal chemotherapy and peritonectomy procedures, has resulted in long-term disease-free survival in many of these patients. However, some of the patients develop progressive disease, and a question regarding additional surgery is appropriate. A critical analysis of the results of second-look surgery should assist in the management of patients with recurrent peritoneal surface dissemination of an appendiceal neoplasm. METHODS: Three hundred twenty-one patients with epithelial peritoneal surface malignancy of appendiceal origin underwent surgery during a 12-year period. Ninety-eight of these patients (30.5%) underwent a second-look procedure. A database of selected clinical features regarding these 98 patients was gathered from the clinical records. A critical statistical analysis of these clinical features and their prognostic impact was performed using survival as an endpoint. All patients were managed by a treatment regimen that used cytoreductive surgery and intraperitoneal chemotherapy. RESULTS: The overall 5-year survival rate of these 98 patients was 73.6%. This compared favorably with a 68% survival rate of 223 patients who did not undergo reoperation. Survival based on the number of cytoreductive surgeries and the free interval between them showed no significant difference. Patients who had second-look surgery with bowel obstruction as a symptom and those in whom the amount of tumor was increased or minimally decreased at the first and second cytoreductions had a significantly inferior 5-year survival rate. A complete second cytoreduction was associated with an improved 5-year survival rate. CONCLUSIONS: Follow-up of patients treated for peritoneal dissemination from neoplasms of appendiceal origin is indicated. Selected patients in whom recurrence develops are candidates for repeat cytoreductive surgery plus intraperitoneal chemotherapy with curative intent.

Adenocarcinoma, Mucinous↗