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Unusual presentation of carcinoma of the vermiform appendix: a report of two cases.

Two unusual presentations of perforated mucoid adenocarcinoma of the appendix are described. One patient presented with a large periappendicular abscess that extended into the gluteal region, the other with a retroperitoneal abscess and abscesses in the right flank and groin. Recognition of the source of the abscess may be difficult but is essential for the prevention and elimination of the sepsis which may be life-threatening. The presence of mucin in the drained pus is highly suggestive for perforated bowel carcinoma. A high index of suspicion and the performance of prompt diagnostic procedures may bring early surgical treatment and better results.

Abscess↗

Adenocarcinoma of the vermiform appendix.

Primary adenocarcinoma of the appendix is rare. Eleven patients with this rare neoplasm have been evaluated at our institution over a 50-year period. We have reviewed the presentation and clinical course of these patients, and have compared them with those described in the literature. The presenting signs and symptoms, physical findings, and treatment were similar to those described in the literature. However, the 5-year survival of 20% is lower than most series, and reflects the advanced stage of disease at the time of diagnosis in this group of patients. Despite the low 5-year survival, we feel that aggressive therapy (right hemicolectomy) is necessary to obtain long-term survival.

Adenocarcinoma↗

Functional organization of the corticofugal system from visual cortex to lateral geniculate nucleus in the cat (with an appendix on geniculo-cortical mono-synaptic connections).

1. In the cat visual cortex (VC), electrophoretic glutamate application at a depth corresponding to layer VI may have excitatory or inhibitory effects on relay cells of the lateral geniculate nucleus (LGN). Corticofugal excitation was seen, if the receptive field centers (RFCs) of the VC neurons recorded at the application site were within 2.3 degrees of the RFCs of the LGN neurons under test. Inhibitory effects were seen if the RFCs of both cells were further apart up to 3.1 degrees. Glutamate application at more superficial cortical sites had no effect on LGN-neuron activity. 2. Cross-correlation analysis between spontaneous activities of simultaneously recorded VC and LGN neurons revealed excitatory cortico-geniculate connections in 18 pairs with RFCs separated by less than 1.7 degrees. In 15 pairs the peak latency of the excitation was 2--5 msec (3.4 msec in the average), 3 pairs showed long cortico-geniculate latencies (13--18 msec). The existence of a fast and slow cortico-geniculate system is suggested. 3. Inhibitory cortico-geniculate interaction was demonstrated with cross-correlation analysis in 8 pairs of which 4 had RFCs separated by more than 1.7 degrees. The onset latency of the inhibition was 2--7 msec except for 2 pairs with about 20 msec latency. 4. Most of the LGN neurons which were affected by cortical glutamate application or which showed an excitatory or inhibitory connection with a VC neurons were sustained cells, while the majority of VC neurons which were recorded in the effective glutamate application sites or which showed a significant interaction with LGN neurons in the cross-correlogram were binocularly driven and complex, with mostly large RFCs (mean diameter 3.5 degrees). They responded briskly to moving small spots as well as to moving slits. 5. It is concluded that the corticofugal excitatory effect is transmitted through monosynaptic links from VC neurons located in layer VI (complex cell) to LGN relay neurons (mostly sustained-cell) and this system is organized in a precise topographical manner. 6. In an Appendix neuron pairs which showed a positive correlation in the geniculo-cortical direction were described. The findings may support the view that complex as well as simple cells are drive monosynaptically from geniculo-cortical afferents of the sustained or transient type.

Animals↗

Adenocarcinoid of the vermiform appendix.

Four cases of adenocarcinoid of the appendix were studied. Two tumours were found among 28 cases primarily diagnosed as appendiceal carcinoids. They showed characteristic histological structures with features of both a conventional carcinoid tumour and a mucinproducing adenocarcinoma with goblet cells. All tumours were small and ill-defined; three were associated with fibrous obliteration of the appendiceal lumen. All were diagnosed incidentally by the pathologist in appendices removed "en passant" or because of acute appendicitis. Three of the tumours appeared well differentiated with a low degree of malignancy similar to that of the conventional carcinoid tumour. In one case however, the tumour was less differentiated with atypical foci and a high mitotic count and had metastasised to peritoneum and both ovaries.

Adenocarcinoma↗

Endometriosis of the cecum and appendix: two case reports.

Two cases of endometriosis of the cecal area are reported. In one case, the endometriosis was located in the appendix causing intussusception into the cecum. Both cases were associated with localizations of endometriosis in the rectosigmoid.

Adult↗

Primary malignant lymphoma of the appendix.

A 70-year-old Japanese woman with primary malignant lymphoma in the appendix was treated. The diagnosis was established after surgery. Histologically, the tumor was malignant lymphoma, lymphocytic, well differentiated, according to the Rappaport's classification which is a good prognostic type of extranodal malignant lymphoma. The patient was treated by ileocecal resection and without radiochemotherapy. The 36-month follow-up revealed neither evidence of recurrence nor metastases.

Aged↗

Intussusception with incarceration of a cystadenoma of the appendix: case report and review of the complications of appendiceal adenomas.

Fifty benign epithelial neoplasms were encountered during the examination of more than 30,000 appendectomy specimens in the Laboratory of Surgical Pathology, Columbia University. In 44 cases, the neoplasms were papillary cystadenomas; in 11 of these cases, there were complications, all thought to have been caused by abnormal accumulations of mucus. We have reported a unique case of an incarcerated intussusception, and attempted to explain the pathogenesis of this unusual complication. We have also summarized the existing medical literature on the subject of intussusception of adenomatous lesions of the appendix.

Adenolymphoma↗

Surgical management for carcinoid tumors of small bowel, appendix, colon, and rectum.

Carcinoid tumors occur most frequently in the gastrointestinal tract. Despite their ability to produce hormones, most of the midgut and hindgut carcinoids covered in this study are clinically silent, and the diagnosis is often not made before emergency surgery or evaluation for liver metastases. Because the rate of lymph node involvement and the prognosis of carcinoid tumors depend on their site and size, surgery refers to these two factors too. Lymph node metastases are most commonly found with small bowel carcinoids (20-45%), providing the rationale for an extended resection including the adjacent lymph node drainage area. Carcinoid tumors of the appendix < 1 cm in diameter rarely metastasize, simply requiring appendectomy for treatment. Lesions > 2 cm should be treated by right hemicolectomy because of their approximately 30% risk of lymph node metastases. Resection should always be done for carcinoid tumors of the colon resection as for adenocarcinomas. Rectal carcinoids < 2 cm rarely metastasize, directing the conclusion that for these smaller lesions local excision is sufficient; for lesions >2 cm a standard cancer resection should be performed provided distant metastases are absent. In general, the younger the patient or the larger the primary tumor, the more aggressive the treatment should be.

Age Factors↗

Tumors of the appendix and colon.

This collective review includes all available case reports of smooth muscle (stromal) tumors of the appendix and large intestine in the world literature. When compiling this review, we endeavored to examine cumulative as well as recently collected data on both benign and malignant smooth muscle tumors spanning the period 1875 to 1996. In total, there were reports of 331 leiomyomas (LMs) and 263 leiomyosarcomas (LMSs). The peak age of incidence of LM was 30 to 39 years, and the peak age of incidence of LMSs was 50 to 59 years. The female/male ratio was slightly higher for LM, and the male/female ratio was higher for LMS. The descending colon and sigmoid colon were the most common sites of both benign and malignant smooth muscle tumors. The growth of LMs most often occurred extraluminally, whereas LMSs tended to grow within the lumen of the colon. With both tumor types pain was the most frequent presenting complaint, followed less commonly by complaints of a palpable mass or gastrointestinal bleeding. LMSs tended to be larger at diagnosis than LMs, though the duration of symptoms for both types of tumor was most often reported to be between 1 month and 1 year. Finally, LMSs were found to metastasize mo

Abdominal Pain↗

[Adenocarcinoid of the appendix vermiformis].

Adenocarcinoids are rare tumors with histological features of both carcinoid tumor and adenocarcinoma. They show a more aggressive biological behaviour than conventional carcinoids. We report a case of a 64-years-old female patient with a diffuse infiltration of the appendicular wall by an adenocarcinoid. Due to the positive surgical margin and the tumor expansion a hemicolectomy was performed. There are no precise criteria to direct the operative choice between appendectomy and hemicolectomy. It is thought that appendectomy is sufficient in case of small tumors in the tip of the appendix. Patients with diffuse appendicular involvement require a more aggressive surgical therapy.

Adenocarcinoma↗

[Serous cystadenofibroma of the epiploic appendix. A tumor of the secondary müllerian system: case report and review of the literature].

We present a case of serous cystadenofibroma 2 cm in diameter in the epiploic appendix of the sigmoid as incidental finding in a 72-year-old patient who underwent hysterectomy and oophorectomy for endometrial carcinoma. The tumor showed the same histology as analogous tumors of the ovary and was associated with endosalpingiosis. Further findings were large adhesions between the epiploic appendices of the sigmoid and the parietal peritoneum and atypical cells in the peritoneal washings. Both may be explained by occult peritoneal endosalpingiosis. The histogenesis, histology, and locations of extraovarian müllerian tumors are reviewed.

Adenofibroma↗

Appendix tumors in the era of laparoscopic appendectomy.

BACKGROUND: The safety of laparoscopic appendectomy for the management of incidentally discovered appendiceal tumors has not yet been established. METHODS: Appendiceal tumor cases managed by laparoscopy or laparotomy over a 10-year period were reviewed. RESULTS: The pathological diagnoses were 23 carcinoid and 20 cancerous lesions. The median patient ages were 36 and 69 years, respectively, for carcinoid and other tumors (p < 0.05). Acute appendicitis was present in 70% of carcinoid cases and 35% of other tumors (p < 0.05). Eight patients with carcinoid tumors were operated on by laparoscopy, whereas 15 underwent laparotomy. Laparoscopic and open procedures were performed in three and 17 patients with cancerous lesions, respectively. Invaded surgical margins were seen after laparoscopy in 20% of patients and open surgery in 6%. Synchronous colon carcinoma was detected in 14% of the patients with an appendix neoplasm. The 5-year survival rates were similar after both laparoscopic and open appendectomy for either carcinoid or other tumors. CONCLUSION: Laparoscopic appendectomy for appendiceal tumors seems to have a slightly higher rate of inadequate resection. However, it is not associated with a significantly worse patient prognosis than open appendectomy.

Adenocarcinoma↗

Laparoscopic versus open resection for appendix carcinoid.

BACKGROUND: Since an increasing number of appendectomies are performed via laparoscopy, it is crucial to determine the impact of this approach on appendix carcinoid (AC) outcome. The goal of this study was to compare results of laparoscopic (LAP) versus open (OP) appendectomy for AC according to intend to treat approach. METHODS: A retrospective review (1991-2003) identified 39 patients (median age, 36 years; range, 12-83) treated by laparoscopy (LAP) or laparotomy (OP) for AC in a single institution. Follow-up was complete for all patients (median, 67 months; range, 4-132). RESULTS: Most cases had associated acute appendicitis (64%). Median carcinoid size was 1.1 cm (range, 0.3-5) and 0.4 cm (range, 0.2-3) in the LAP and OP groups, respectively. LAP and OP were performed in 21 (54%) and 18 (46%) patients, respectively. Surgical margins were positive in two patients in the LAP group and one patient in the OP group (p = 0.6). Right colectomies were performed for AC >2 cm in five patients after LAP and in four patients after OP (p = 0.9). Actuarial 5-year survival rates were 100 and 94% in the LAP and OP groups, respectively (p = 0.2). Two patients died in the OP group, one due to metastatic carcinoid and the other due to metachronous colorectal cancer. Synchronous or metachronous colorectal carcinomas developed in six patients (15%). CONCLUSION: Laparoscopic appendectomy is a safe procedure for AC, with carcinologic and long-term results similar to those of conventional appendectomy. Thus, pre- or per-operative suspicion of AC is not a contraindication to LAP. Prognosis of AC appears more dependent on carcinoid malignant potential or associated tumors. Risk for developing colorectal adenocarcinoma is high in AC patients and warrants follow-up of all patients with colonoscopic screening.

Adolescent↗

Malignancies of the appendix: beyond case series reports.

PURPOSE: A comprehensive analysis was performed for five histologic types of appendiceal tumors to compare incidence, clinicopathologic features, survival, and appropriateness of surgery. METHODS: All patients diagnosed with mucinous adenocarcinoma (n = 951), adenocarcinoma (n = 646), carcinoid (n = 435), goblet (n = 369), and signet-ring cell (n = 113) in the Surveillance, Epidemiology, and End Results database (1973-2001) were analyzed. Evaluation of incidence, stage, and five-year relative survival were determined for each histology. The appropriateness of the operative procedure (i.e. , appendectomy vs. colectomy) was examined by tumor type and size. RESULTS: Tumor incidence, patient demographics, survival outcomes, and appropriateness of surgery varied significantly among the different appendiceal tumor histologies. The most common appendiceal tumors were mucinous. With regard to patient demographics, carcinoids presented at an earlier mean age of 41 years and 71 percent were female (P < 0.001 for both). Overall five-year survival was highest for carcinoid (83 percent) and lowest for signet ring (18 percent). Although current guidelines specify that a right hemicolectomy (rather than an appendectomy) be performed for all noncarcinoid tumors and carcinoid tumors >2 cm, we found that 30 percent of noncarcinoids underwent appendectomy. Similarly, 28 percent of carcinoids >2 cm under-went appendectomy, which is a lesser resection than is indicated. CONCLUSIONS: This study provides a population-based analysis of epidemiology, tumor characteristics, survival, and quality of care for appendiceal carcinomas. This characterization provides a novel description of the presentation and outcomes for malignancies of the appendix and highlights that a substantial number of patients with appendiceal tumors may not be receiving appropriate surgical resection.

Adenocarcinoma, Mucinous↗

Surgical debulking and intraperitoneal chemotherapy for established peritoneal metastases from colon and appendix cancer.

BACKGROUND: Aggressive treatment of peritoneal metastases from colon cancer by surgical cytoreduction and infusional intraperitoneal (IP) chemotherapy may benefit selected patients. We reviewed our institutional experience to assess patient selection, complications, and outcome. METHODS: Patients having surgical debulking and IP 5-fluoro-2'-deoxyuridine (FUDR) plus leucovorin (LV) for peritoneal metastases from 1987 to 1999 were evaluated retrospectively. RESULTS: There were 64 patients with a mean age of 50 years. Primary tumor sites were 47 in the colon and 17 in the appendix. Peritoneal metastases were synchronous in 48 patients and metachronous in 16 patients. Patients received IP FUDR (1000 mg/m2 daily for 3 days) and IP leucovorin (240 mg/m2) with a median cycle number of 4 (range, 1-28). The median number of complications was 1 (range, 0-5), with no treatment related mortality. Only six patients (9%) required termination of IP chemotherapy because of complications. The median follow-up was 17 months (range, 0-132 months). The median survival was 34 months (range, 2-132); 5-year survival was 28%. Lymph node status, tumor grade, and interval to peritoneal metastasis were not statistically significant prognostic factors for survival. Complete tumor resection was significant on multivariate analysis (P = .04), with a 5-year survival of 54% for complete (n = 19) and 16% for incomplete (n = 45) resection. CONCLUSIONS: Surgical debulking and IP FUDR for peritoneal metastases from colon cancer can be accomplished safely and has yielded an overall 5-year survival of 28%. Complete resection is associated with improved survival (54% at 5 years) and is the most important prognostic indicator.

Adenocarcinoma↗