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At least 19 recordsLinked to original sources
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↗
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↗
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↗
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↗
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↗
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↗
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↗
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↗
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↗
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↗