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PubMed1992

Primary appendiceal malignancies: rare but important.

The four main types of appendiceal neoplasms are carcinoid tumors (CT), mucinous cystadenocarcinomas, colonic adenocarcinomas (CAC), and adenocarcinoid tumors. They have different clinical features and require different therapy. They are rarely diagnosed before or during surgery and frequently require a secondary operation for proper treatment. A series of 14 patients treated between 1979 and 1989 at Harris Methodist Fort Worth Hospital are described. There are six important points of management. (1) A frozen section should be done whenever the appendiceal findings are atypical. Then a diagnosis of malignancy can be made during surgery and appropriate surgery can be done primarily. (2) All patients with appendiceal neoplasms should be followed because a second malignancy will develop in 15% to 20% of them. (3) Appendectomy is recommended for patients whose CT are less than or equal to 1.0 cm in diameter and for most patients whose tumors are between 1.0 and 2.0 cm in diameter. Right hemicolectomy is preferred for those patients whose CT are 2.0 cm or more in diameter or between 1.0 and 2.0 cm with heavy lymphatic and mesenteric invasion. (4) Mucinous cystadenocarcinomas are slowly progressive and are best treated by means of right hemicolectomy with aggressive debulking added if pseudomyxoma peritonei is present. (5) CAC require a right hemicolectomy and have the same prognosis as have other colon adenocarcinomas. (6) Adenocarcinoid tumors have a dual cell origin, a predilection for developing ovarian metastases, and a malignant potential between CT and CAC. They are best treated by means of right hemicolectomy with concomitant oophorectomy in postmenopausal women.

Adenocarcinoma↗

PubMed2002

Cancer of appendix as a presenting feature of Crohn's disease.

Appendiceal neoplasms are rare. Colonic malignancies, including appendiceal carcinomas, have been rarely reported in Crohn's disease patients. The involvement of the appendix in Crohn's disease is not often seen. We report an interesting case in which a caecal Crohn's disease patient presented for the first time with an appendiceal adenocarcinoma.

Adenocarcinoma, Mucinous↗

PubMedDate not supplied

Carcinoid tumors of the appendix. Prognostic factors and evaluation of indications for right hemicolectomy.

BACKGROUND/AIMS: Carcinoid tumors of the appendix are thought to be the most common type of appendiceal neoplasms. Although the vast majority of appendiceal carcinoids behave in a benign fashion, they are considered malignant because they all have the potential for invasion, metastasis and production of physiologically active substances. The aim of our study is to evaluate the gravity of the prognostic factors and the indications for extended surgical treatment. METHODOLOGY: Twenty-four patients, six male (aged from 18 to 59 years) and eighteen female (aged from 16 to 27 years) are included in our study. All these patients were managed and followed-up in our section during the last 17 years (follow-up range 10-17 years). Following confirmation of the diagnosis, secretion of 5-HLAA (5-Hydroxy-Inndole-Acetic Acid) was measured after a 24-hour collection of urine. Moreover, ultrasound (US) examination of the liver and computerized tomography (CT) scanning of the upper abdomen were performed after the operation in all patients. Also, patients with tumor size more than 1cm underwent a Somatostatin Receptor Scintigraphy (Octreoscan). The follow-up data of all patients included measurement of 5-HLAA and US examination every six months. RESULTS: The most common site for the tumors was the tip of the appendix (18/22). In the rest of the cases, the neoplasms were located in the base (4/22) and in the body (2/22), while in one patient the mesoappendix was invaded. In sixteen patients the tumor size was less than 1cm, in seven patients the tumor diameter was measured to be 1 to 2cm and in one patient the tumor was 3cm. Most of our patients (16/22) underwent only an appendicectomy, while in the rest of them (in the patients with tumor size between 1-2cm and in the patient with invasion of mesoappendix) a right hemicolectomy was performed. No patient was found to have metastatic disease during the operation, while the patient with invasion of the mesoappendix developed metastases in the lung, two years after the operation. CONCLUSIONS: Carcinoid tumors of the appendix, in most cases, are found incidentally during appendicectomies, especially in young females and usually are less than 1cm in size, which is probably the reason of the absence of metastases in all cases. Histological examination and size of the tumor are important factors that contribute to the selection of the surgical treatment and both must be estimated by the surgeons to make the final choice.

Adolescent↗

PubMed1985

Adenocarcinoid of the appendix presenting as bilateral Krukenberg's tumor of the ovaries. Immunohistochemical and ultrastructural studies and literature review.

A patient with an adenocarcinoid of the appendix presented with bilateral Krukenberg's tumors of the ovaries. Immunohistochemical and ultrastructural studies revealed a selective ability of the mucinous (goblet cell) component of the appendiceal neoplasm to metastasize. A review of the literature has revealed nine previously published cases of appendiceal adenocarcinoid metastatic to the ovaries. All showed involvement of both ovaries, but none provided unequivocal evidence of a metastatic proliferating carcinoid element. As the appendiceal lesion is often grossly inconspicuous, it may be overlooked in cases presenting initially with ovarian tumors. Routine appendectomy is therefore recommended in such patients where no grossly obvious primary tumor is evident.

Adenocarcinoma↗

PubMed1994

Laparoscopic management of appendiceal intussusception associated with villous adenocarcinoma.

The authors present a case of appendiceal intussusception, a rare finding that can be associated with appendiceal neoplasms. A 74-year-old woman with right lower quadrant abdominal pain was found to have an appendiceal intussusception associated with a villous adenocarcinoma and was managed with a laparoscopic assisted right hemicolectomy. This case is presented along with a review of the symptoms, diagnosis, classification, and appropriate management of this entity via laparoscopy.

Adenocarcinoma↗

PubMed2006

Surgical treatment of malignant carcinoid tumours of the appendix.

Since their first description in 1882, malignant neoplasms of the appendix still remain rare. Malignant carcinoid tumours are the most common accounting for 85% of all appendiceal neoplasms. Preoperative diagnosis is invariably difficult, and precise treatment protocols for these neoplasms remain unclear. We reviewed our experience and searched published evidence to produce management guidelines. A retrospective review of all malignant carcinoid tumours diagnosed in our hospital between April 1994 and December 2003 was performed. Patient demographics, operative details, histological types and clinical outcomes were retrieved from case notes supplemented by a questionnaire to the patient's General Practitioner. A literature search was then performed. Nine patients were identified with classical carcinoid tumours (CCT); (M = 3 and F = 6, mean age: 43, range 14-81) and two patients with goblet-cell morphology (F = 2, age 46 and 76). Mean follow-up was 63 months (range 1-125 months). Nine patients were alive at the end of follow-up. Appendicectomy was performed for acute appendicitis. Other appendiceal pathologies were identified following hemicolectomy and oophorectomy. CCT are the most common tumours and have the better prognosis. From our experience and subsequent review of the literature, we recommend right hemicolectomy as the treatment of choice for malignant carcinoid tumours. However, small CCTs less than 2 cm in diameter at the tip of the appendix, with a low proliferative index, without angiolymphatic or mesoappendiceal extension can be treated by appendicectomy. Following oncological assessment, further adjuvant therapy should be considered for patients with advanced disease.

Adolescent↗

PubMed2005

[Appendiceal mucinous cystadenoma].

BACKGROUND: Appendiceal neoplasms are rare. Patients may present with clinical symptoms suggestive of "acute appendicitis" or other unspecific abdominal complaints. An appendiceal mucinous neoplasia is sometimes diagnosed during a laparotomy performed on another indication. Frequently, the condition remains undiagnosed until the pathologists' examination. MATERIALS AND METHODS: We present the case of a 49-year-old woman who presented with symptoms suggestive of acute appendicitis. An appendectomy was performed. The appendectomy specimen revealed a mucinous cystadenoma with a diverticulum in the appendix. The case is discussed in the light of the current literature. RESULTS AND INTERPRETATION: Appendiceal tumours account for less than 0.4% of neoplasias in the gastrointestinal tract and are found in less than 1% of appendectomies. Mucinous lesions ("mucocele") are classified as mucosal hyperplasia, mucinous cystadenoma, or mucinous cystadenocarcinoma. However, there are reports of great variability in the biological behavior, especially concerning the development of pseudomyxoma peritonei. No current consensus exists as to diagnostic criteria or treatment. A radically removed appendix is curative in most cases of mucinous cystadenoma, whereas right hemicolectomy should be considered for patients with malignant mucinous lesions of the appendix.

Appendectomy↗

PubMed1980

Appendiceal carcinoma mimicking primary bladder cancer.

We report the first case of adenocarcinoma of the appendix invading the bladder. Because invasive bladder cancer was diagnosed intially the pelvis was irradiated preoperatively. At operation a primary appendiceal neoplasm invading the bladder was discovered and a right hemicolectomy and en block partial cystectomy were done. This treatment is recommended for appendiceal adenocarcinoma that invades the bladder.

Adenocarcinoma, Mucinous↗

PubMed2006

From krukenberg to today: the ever present problems posed by metastatic tumors in the ovary: part I. Historical perspective, general principles, mucinous tumors including the krukenberg tumor.

This review considers historical aspects of metastatic tumors to the ovary, general principles that aid in their evaluation, and metastatic mucinous tumors, including the Krukenberg tumor. The historical timeline on the Krukenberg tumor dates back to the legendary Sir James Paget and the story is followed through the well-known, albeit flawed, contribution of Friedrich Krukenberg and others who have contributed important papers over the years, including the overlooked contribution of the French investigator Gauthier-Villars. Knowledge of metastatic colorectal carcinoma is traced back to the famed British surgeon Sir John Bland-Sutton and followed through to more recent contributions, including the important one of Lash and Hart. Contributions on mucinous tumors conclude the historical perspective, note being made of the recent evidence suggesting that the long held contention of Dr Robert E. Scully that ovarian mucinous tumors in patients with pseudomyxoma peritonei usually originate from the appendix is correct. The section on general principles highlights the many clinical, gross, microscopic, and special techniques such as immunohistochemistry that may aid in determining that an ovarian tumor is metastatic with emphasis on the first 3 mentioned aspects. Problematic features such as a tendency for metastatic tumors to be cystic, even when the primary tumors are not, and for many metastatic tumors to mature in the ovary (so-called maturation phenomenon), are emphasized. Of the many helpful findings that resolve the problem, the characteristic features of surface implants are highlighted. The contribution on the Krukenberg tumor reviews the varied microscopy of this tumor pointing out that the well-known pattern of signet-ring cells in a cellular stroma, albeit characteristic, is often not striking and frequently overshadowed by other microscopic features. The latter include, in many cases, a rather unique microcystic pattern. The final portion of the essay reviews mucinous tumors of non-Krukenberg type, beginning with those that originate from the appendix. The appendiceal neoplasms have distinctive features in most cases being particularly well differentiated, and this is also seen in their ovarian metastases. Other mucinous tumors that commonly simulate closely metastatic neoplasms, include those from the pancreas in particular, but also diverse other sites, are then reviewed.

Adenocarcinoma, Mucinous↗

PubMed1995

Expression of cytokeratin 7 in simultaneous mucinous tumors of the ovary and appendix.

Cytokeratin 7 (CK-7) is a simple epithelial keratin that may be used to investigate the site of origin of adenocarcinomas. In fact, CK-7 is present in ovarian epithelial neoplasms but is generally absent in colonic carcinomas. This pattern of CK-7 expression may aid in elucidating the genesis of mucinous tumors occurring simultaneously in the ovary and appendix, accompanied by psuedomyxoma peritonei. Five such cases were immunostained with anti-CK-7, and all showed a concordant staining pattern of the appendiceal, ovarian, and peritoneal lesions. Two cases showed a negative reaction for CK-7 and thus would appear to represent ovarian and peritoneal metastases from an appendiceal primary tumor. Three cases were CK-7 positive, and the nature of these mucinous lesions remains open to debate; they may either represent independent primary tumors or originate from the appendix. For comparison, five Stage I mucinous borderline tumors of the ovary and their normal appendices were also stained with anti-CK-7. These ovarian tumors were all CK-7 positive, whereas the appendices were negative. It is concluded that CK-7 is capable of distinguishing a group of tumors that can reliably be classified as primary appendiceal neoplasms metastatic to the ovaries and peritoneum.

Adult↗

PubMed2002

Primary malignant neoplasms of the appendix: a population-based study from the surveillance, epidemiology and end-results program, 1973-1998.

BACKGROUND: Cancer of the appendix is an uncommon disease that is rarely suspected rarely before surgery. Although several case series of these tumors have been published, little research has been anchored in population-based data on cancer of the appendix. METHODS: This analysis included all actively followed cases of appendiceal neoplasms reported to the National Cancer Institute's Surveillance, Epidemiology and End-Results (SEER) program between 1973 and 1998. Tumors were classified as "colonic type" adenocarcinoma, mucinous adenocarcinoma, signet ring cell carcinoma, goblet cell carcinoid, and "malignant carcinoid" (SEER only collects data on carcinoids specifically classified as malignant). We compared incidence, overall survival and survival rates by extent of disease at diagnosis. RESULTS: Between 1973 and 1998, 2117 appendiceal malignancies were reported to the SEER program, of which 1645 cases were included in the analysis. Age-adjusted incidence of cancer of the appendix was 0.12 cases per 1,000,000 people per year. Demographic characteristics of patients with goblet cell carcinoid tumors were midway between those of patients with malignant carcinoid and all types of adenocarcinomas. After controlling for age and extent of disease at diagnosis, the overall survival rate for patients diagnosed between 1983 and 1997 (n = 1061) was significantly worse for those with signet ring cell carcinoma than for those with any other tumor type (P < 0.01). In addition, overall survival rates were better for patients with malignant carcinoid (P = 0.01). CONCLUSIONS: Demographic characteristics of patients with cancer of the appendix vary by histology. Except for signet ring cell carcinoma and malignant carcinoid, the extent of disease at time of diagnosis is a more important predictor of survival than histology.

Adenocarcinoma↗

PubMed2006

[Microhematuria secondary to mucocele and appendicular cystoadenoma].

Appendiceal mucocele is a rare entity consisting of cystic dilatation of the appendix as a result of increased mucus production. Most cases are related to the presence of a cystadenoma. This appendiceal neoplasm is frequently associated with other extraintestinal and colonic tumors and therefore adequate abdominal examination is necessary. The clinical presentation is nonspecific and varies from asymptomatic forms to abdominal discomfort and, less frequently, to urological manifestations. We describe the case of a woman with hematuria associated with an appendiceal mucocele. Only a few cases have been reported in the medical literature. The treatment of choice of cystadenoma is surgery and simple mucocele cannot be macroscopically differentiated from cystadenoma and cystadenocarcinoma. Survival in patients with appendiceal cystadenoma is excellent. The 5-year survival rate is reduced by at least half in patients with cystadenocarcinoma associated with pseudomyxoma peritonei.

Appendiceal Neoplasms↗

PubMed2001

Krukenberg tumor from an occult appendiceal adenocarcinoid: a case report and review of the literature.

Appendiceal neoplasms with ovarian metastasis are rare. A 35-year-old woman with a left ovarian tumor underwent left salpingo-oophorectomy, partial resection of the right ovary, and a total hysterectomy. Pathological diagnosis of both ovaries was typical, Krukenberg tumor with signet-ring cells, and the second laparotomy revealed an occult appendiceal tumor to be the primary lesion. The appendix showed no evidence of malignant change of the mucosa, but the tumor cells were observed infiltrating from the basiglandular region into the underlying stroma, associated with mucocele. Although, argentaffin and argyrophil staining were negative, a few tumor cells showed immunohistochemical positivity for Chromogranin A. Accordingly, the tumor was diagnosed as adenocarcinoid rather than adenocarcinoma of the appendix. A review of the literature showed less than 40 cases of metastatic ovarian tumor from appendiceal primary, one-third of which were occult and could be detected at the second laparotomy. Cisplatin-based chemotherapy may have partial effect in the treatment of patient with adenocarcinoid tumor.

Adult↗

PubMed2004

Incidence and predictors of appendiceal tumors in elderly males presenting with signs and symptoms of acute appendicitis.

BACKGROUND: Patients with appendiceal tumors (AT) frequently present with the same signs and symptoms as acute appendicitis (AA). The purpose of this study was to identify the incidence of AT in a population of patients greater than 60 years of age thought to have AA, and to identify presenting factors that can help differentiate the 2 disease processes. METHODS: An institutional review board-approved, retrospective review was performed identifying all patients greater than 60 years old who underwent either appendectomy or colectomy after presenting with signs and symptoms of AA from Janaury 1997 to April 2004. Patient records were examined for demographic variables, presenting signs and symptoms, and pathology. RESULTS: A total of 34 patients fit the entry criteria; 8 (24%) had AT. Average duration of symptoms was significantly longer (4.9 +/- 1.9 versus 2.3 +/- 0.3 days; P = 0.01) and hematocrit lower (36.5 +/- 2.3 versus 42.4 +/- 1.0%; P = 0.01) for patients with AT compared with patients with AP. No other significant differences in presenting signs and symptoms were noted. CONCLUSIONS: There is an increased incidence of appendiceal neoplasms among elderly patients presenting with signs and symptoms of AA. Distinction between AT and appendicitis is difficult preoperatively, but AT should be suspected when patients present with longer duration of symptoms or reduced hematocrit.

Abdomen, Acute↗

PubMed1998

The use of laparoscopy in a case of appendiceal cystadenoma presenting as pseudomyxoma peritonei in an inguinal hernial sac.

A case is reported of a patient with a benign appendiceal neoplasm presenting incidentally with mucin in an inguinal hernial sac. Only one such case of a benign tumour, and one such case of cystadenocarcinoma, have previously been reported. In neither of these cases was laparoscopy used in diagnosis or staging of the disease. We review the management of such cases in light of recent proposed changes to pathological classification.

Adult↗

PubMed1998

Goblet-cell mucinous epithelium lining the endometrium and endocervix: evidence of metastasis from an appendiceal primary tumor through the use of cytokeratin-7 and -20 immunostains.

Differential staining with cytokeratin (CK)-7 and CK-20, two members of a complex family of proteins in human epithelial cells, proved critical in showing that the extremely well-differentiated goblet-cell (intestinal) mucinous epithelium lining the surface of the endometrium and endocervix in two patients and the fallopian tube in one was identical to that of the coincident appendiceal neoplasms. One of these patients also had a large ovarian tumor that grossly and microscopically resembled a mucinous cystadenoma of borderline malignancy and would have been considered primary except for the CK stains (CK-20 positive and CK-7 negative), which suggested metastasis from the appendix, presumably by a transtubal route.

Appendiceal Neoplasms↗

PubMed1999

K-ras and p53 mutations in the pathogenesis of classical and goblet cell carcinoids of the appendix.

BACKGROUND: Mutations in the K-ras oncogene and the p53 tumor suppressor gene are present in approximately 50% of colonic adenocarcinomas. Goblet cell carcinoids (GCCs) are uncommon neoplasms of the appendix that appear to be intermediate between carcinoid tumors and adenocarcinomas, both histologically and biologically. The current study was undertaken to examine the role of p53 and K-ras mutations in the pathogenesis of GCCs and typical carcinoids (TCs) of the appendix. METHODS: Archival materials from 22 GCCs and 18 TCs were analyzed. K-ras mutations in codons 12, 13, and 61 were studied by a polymerase chain reaction (PCR) based designed restriction fragment length polymorphism method using mismatched nested primers. Mutations in exons 5-8 of the p53 tumor suppressor gene were analyzed in 16 GCCs and 18 TCs by PCR and single-strand conformational polymorphism followed by direct sequencing. Immunostains for p53 and chromogranin were performed in all cases. RESULTS: K-ras mutations and nuclear accumulation of p53 by immunohistochemistry were not detected in any of the GCCs or TCs. p53 mutations were found in 4 of 16 GCCs (25%) and 8 of 18 TCs (44%). Immunoreactivity for chromogranin was seen in the vast majority of GCCs and TCs. CONCLUSIONS: p53 mutations appear to play a role in the pathogenesis of some GCCs and in approximately 50% of TCs of the appendix, whereas mutations in the K-ras oncogene do not appear to be important in the development of these tumors. The minimal cytologic atypia, low incidence of metastases, and lack of K-ras mutations in goblet cell appendiceal neoplasms suggest that they are variants of carcinoid tumors. Our findings lend support to the recommendation that the therapeutic guidelines applied to TCs of the appendix should be the same for GCCs.

Appendiceal Neoplasms↗

PubMed2006

Management of goblet cell carcinoid.

BACKGROUND AND OBJECTIVES: Goblet cell carcinoid, a rare tumor of intermediate malignant potential, is known to account for a significant minority of appendiceal neoplasms. Sixteen new cases of gastrointestinal goblet cell carcinoid were reviewed to describe their presentation, treatment, and outcome. METHODS: A review of 16 cases from a single institution. RESULTS: Sixteen patients were diagnosed with goblet cell carcinoid between 1995 and 2005. Presenting diagnoses included appendicitis (n=8), abdominal or liver mass (n=5), uterine fibroids (n=1), ovarian mass (n=1), and Crohn's Disease exacerbation (n=1). Mean follow-up was 12 months with a mortality of 19% (n=3). Patients were divided into two groups: those where the diagnosis was an incidental finding at operation (Group 1) and those where the presentation was of an abdominal mass or metastatic disease (Group 2). Nine of ten patients in Group 1 initially received appendectomies. Group 2 included patients presenting with Krukenberg type lesions (n=2) and abdominal masses (n=4). CONCLUSIONS: Goblet cell carcinoid is a rare malignant tumor largely affecting the appendix. In patients presenting with appendicitis, our series does not support the recommendation of right hemicolectomy based on pathologic diagnosis alone and surgical intervention must be customized to the individual patient.

Adult↗