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Neurofibromatosis type 1 and masses of the appendix: a case report.

BACKGROUND: Neurofibromatosis type 1 is an autosomal dominant disease with high penetrance, affecting 1:3,000 pregnancies. Meningiomas and other benign central nervous system tumors, such as ependymomas, are common features. CASE: A patient with neurofibromatosis underwent cesarean section due to intrauterine growth restriction and nonreassuring fetal heart rate patterns. Examination of the abdominal cavity and intestines revealed a large (diameter, 12 cm), rubbery, fibrin-coated appendicular mass. The appendix was removed, and pathologic analysis confirmed the diagnosis of neuroma of the appendix. CONCLUSION: The case stresses the importance of careful inspection and evaluation of the abdominal cavity during surgery on patients with neurofibromatosis for the detection of possible associated masses.

Adult↗

Sciatic hernia with an early-stage adenocarcinoma of the appendix: report of a case.

Sciatic hernia and early-stage appendiceal adenocarcinoma are rare disorders. We report herein a case of an early stage of appendiceal adenocarcinoma found incidentally during an operation for sciatic hernia. An 80-year-old woman was admitted to our hospital with abdominal distension. Abdominal computed tomography showed a small bowel obstruction and a small bowel loop through the right sciatic foramen outside the pelvic cavity. During an operation for the repair of the sciatic hernia, a deformed appendix was found incidentally and a simple appendectomy was also performed. Histological examination of the excised appendix showed well-differentiated adenocarcinoma confined to the mucosal layer in a tubulo-villous adenoma. At 13-month follow-up, the patient is doing well without evidence of recurrence or metastasis.

Adenocarcinoma↗

[Benign mucus-secreting tumors of the appendix. Six cases].

The authors report six cases of mucus-secreting tumor of the vermicular appendix, in four women and two men (mean age 59 years). All were benign. Pain in the right lower quadrant was the initial sign in 4 cases, while the mucus-secreting tumor was an incidental finding in the other two cases. A mass was palpated in the right lower quadrant in one case. A barium enema was performed in four cases and showed an extrinsic compression of the caecal base in one case, and of the right colon in another case. Four appendectomies, one associated with removal of the adjacent caecal tissues and two right colectomies were performed. Analysis of these six cases and a review of the literature allow us to recall the histologic classification of these tumors, which determines the prognosis and to emphasize the sonographic and CT scan findings which might lead to earlier preoperative diagnosis in the future. Management depends upon the circumstances under which the tumor is discovered, the local anatomy, and the type of tumor. Appendectomy is the treatment most often performed. Faced with a tumor of the appendix, without histologic proof or when the local anatomic conditions increase the risk of opening an abscess, right colectomy is recommended.

Aged↗

[Mucus-secreting tumors of the appendix: a preoperative diagnosis is possible. Apropos of 2 cases].

The authors present two cases of pseudo tumoral type Mucus Secreting Tumours (MST) of the appendix. In this type (32% of the cases), the clinical presentation, barium enema, ultrasonography and most importantly CT scan generally confirm the preoperative diagnosis. We found three advantages this preoperative diagnosis. 1) Primarily it allows selection of a large surgical approach that permits full exploration of the abdominal cavity especially the ovaries (2-18% associated lesions); 2) also it allows a histopathological examination (frozen section) of the appendix to be performed during the operation; 3) and finally the surgical treatment will depend on the operative findings and the result of the histopathological examination: in the benign forms simple appendicectomy will be sufficient but the malignant forms (12% of the cases) should be treated by right hemicolectomy.

Appendectomy↗

Primary mucinous adenocarcinoma of the appendix with bilateral Krukenberg ovarian tumors.

After experiencing intermittent episodes of abdominal pain for two years, a 28-year-old woman developed partial small bowel obstruction. Barium enema and colonoscopy revealed the source of obstruction to be an apparent cecal carcinoma. At exploratory laparotomy a primary adenocarcinoma of the appendix with bilateral Krukenberg ovarian metastases was found. This is a rare occurrence and, to our knowledge, the first well-documented case in the English literature. These case also demonstrates difficulties in the preoperative diagnosis of adenocarcinoma of the appendix.

Adenocarcinoma, Mucinous↗

Goblet cell carcinoid tumor of the appendix. Report of five cases and review of the literature.

Five cases of goblet cell carcinoid tumor of the appendix showed characteristic histologic features that justified classification of these lesions as mucinous variants of carcinoid tumor. The tumor has low-grade malignancy, and metastases are uncommon. Resemblance to mucinous adenocarcinoma of the appendix is striking, and the features that help to differentiate the two lesions are delineated.

Adenocarcinoma, Mucinous↗

Deciduosis of the appendix.

A clinicopathologic and immunohistochemical study of six cases of deciduosis of the appendix is presented. The patients were pregnant women aged 18-40 years. In four cases, the patients presented with signs and symptoms of acute appendicitis during their 26th to 31st week of pregnancy; in the other two, the lesions were discovered incidentally in appendectomy specimens obtained during cesarean section and tubal ligation at term. Histologically, the lesions were characterized by the presence of multiple, irregularly distributed submesothelial deposits of decidualized cells in the serosa of the appendix, without any associated evidence of endometriosis. By immunohistochemistry, the decidualized cells strongly labelled with vimentin antibodies, consistent with their stromal origin. In two cases, coexpression of vimentin and desmin intermediate filaments was observed, suggestive of myoid differentiation. The possible relationship of the lesions with the development of symptoms of peritoneal irritation in these patients is discussed along with a review of the literature on the abdominal complications of ectopic decidua.

Adolescent↗

[A primary malignant lymphoma of the appendix].

A malignant lymphoma (ML) of the gastrointestinal tract occurs most frequently in the stomach, and ML of the appendix is very rare, with only 16 cases reported in the Japanese literature. Reported is a case of a primary ML the appendix in a 48 year old male, in whom an ileocecal resection was carried out. This case was pathohistologically diagnosed as a diffuse large lymphoma of the B cell type, according to the LSG classification. Therapeutic problems were encountered because of the difficulty in making an early diagnosis.

Antineoplastic Combined Chemotherapy Protocols↗

Adenocarcinoma of the appendix.

Sixteen instances of adenocarcinoma of the appendix treated at Roswell Park Memorial Institute between 1964 and 1986 were reviewed. Thirteen of 15 were mucinous and ten were well differentiated. Synchronous metastases were present in 11 patients, with carcinomatosis being the most common pattern of metastases (nine patients). Seven of eight women had ovarian metastases. Nine of the 11 patients with metastatic disease presented with complaints referrable to the metastatic site. The five year survival rate was 50 per cent in the eight patients who underwent right hemicolectomy and 25 per cent in the four patients who underwent appendectomy. The data from this review support right hemicolectomy as the treatment of choice for adenocarcinoma of the appendix. The prolonged survival time of patients with metastatic disease and the symptomatic nature of that disease support the application of palliative oophorectomy in patients with ovarian metastases.

Adenocarcinoma, Mucinous↗

[A primary adenocarcinoma of the appendix, accompanied with 5 ileocolonic fistulae].

A primary adenocarcinoma of the appendix is a rare lesion, and this paper reports a case of a primary adenocarcinoma of the appendix, but with the inclusion of 5 fistulae. According to the classification by Uihlein & McDonald, this histology is of the colonic type. The first known case accompanied with a fistula was reported by Haldane (1862) in the English-language literature and by Shiraishi et al. (1954) in Japan. Since then, 21 cases have been found in the Japanese literature. The patient underwent an operation and his prognosis has been considered good. A review of some the relevant literature is discussed with regard to the pathology, diagnosis, and treatment.

Adenocarcinoma, Mucinous↗

[Adenocarcinoma of the cecal appendix. Presentation of 2 cases and a review of the literature].

Two cases of adenocarcinoma of the vermiform appendix are described. The literature on these rare cancers which are almost always diagnosed at routine histology and whose prognosis is related to Duke grading and staging, is reviewed. The stable cure observed in one patient after 13 years after straightforward caecal resection for cancer of the appendix extending beyond the base of the implant (which today would be handled with hemicolectomy) and then treated for multiple polyps, two of them in a stage of malignant degeneration, suggests a difference of biological behaviour compared to tumours of the other colon districts. In the second case, stress is laid on the unusual extension of the tumour-related inflammation to the skin of the gluteal region.

Adenocarcinoma↗

[Adenocarcinoma of the appendix. A multicenter study from AURC].

Thirty-two patients with primary adenocarcinoma of the appendix were studied in this collective review. Diagnosis was never suspected preoperatively. Immediate operation was performed in 21 patients with a diagnosis of acute appendicitis. At operation, tumor of the appendix was only identified in 50 percent of the 32 patients. One patient with an obstructing tumor and peritoneal involvement died postoperatively. Estimated survival rate was 46% at five years. Probability of survival was unrelated to the histologic tumor type but significantly correlated with the extent of tumor spread. Right hemicolectomy led to a significant increase in survival compared with appendectomy alone and to a significant decrease in risk of recurrence. In Dukes' B2 and C patients, differences in the survival curves were in favor of right hemicolectomy. In patients with pseudomyxoma peritonei, long-term survival was obtained by repeated laparotomy with resection of mucinous material. Our results indicate that, in good risk patients, right hemicolectomy performed as first or second operation, provides better results than appendectomy alone.

Adenocarcinoma↗

Goblet cell carcinoid of the appendix.

Goblet cell carcinoid of the appendix is a relatively uncommon clinical condition. Although its histogenesis remains controversial, its histological characteristics are distinctive enough not to pose a serious problem for diagnosis. The clinical behaviour of the tumor appears to lie between the typical behaviour of an ordinary carcinoid and that of a well differentiated adenocarcinoma of the appendix. Clinical diagnosis of this condition is seldom made pre-operatively, most patients presenting with signs and symptoms of an acute appendicitis. In a number of patients the tumor is identified incidentally during an operation performed for some unrelated entity. Treatment of this tumor is generally by simple appendectomy, although in some patients a more radical procedure is indicated. Prognosis for the most part is quite favorable with the exception of those few patients who present with the more virulent form of the disease.

Aged↗

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↗

[Wound infection following appendectomy. Metronidazole vs ornidazole as single-dose prophylaxis in non-perforated appendix].

In an open prospective randomized study, the postoperative wound infection rate following removal of an unperforated appendix was evaluated in 187 patients who received either metronidazole (1 g suppositories) or ornidazole (500 mg iv or 500 mg suppositories) in a single dose preoperatively. The overall postoperative infection rate was 2.1%. Metronidazole and ornidazole suppositories are a cheap method of preventing postoperative infection in cases of unperforated appendix.

Adolescent↗

Primary adenocarcinoma of the appendix and pseudomyxoma peritonei.

Two cases of primary adenocarcinoma of the appendix are reported. Both patients presented with a clinical picture resembling acute appendicitis. One case was complicated by a pseudomyxoma peritonei. The other patient additionally had several colonic adenomatous polyps and a malignant neoplasm of the ascending colon. Primary adenocarcinoma of the appendix is rare and only some 250 cases have been reported. It usually presents as acute appendicitis in the 6th or the 7th decade. Prognosis and histology closely resemble that of colonic adenocarcinoma. Resection of the ileocecal segment is the first choice treatment in all stages except Dukes A. In Dukes A appendectomy alone is sufficient treatment. After ileocecal resection the 5 year survival is better than after appendectomy alone for Dukes' stages B and C. Clinical presentation and treatment of the adenocarcinoma and a remarkable complication, the pseudomyxoma peritonei, will be discussed.

Adenocarcinoma↗

Crohn's disease limited to the vermiform appendix.

Thirteen cases of Crohn's disease confined to the vermiform appendix were seen during a 12-year period. They constituted 16.9% of patients with primary resection of the bowel for Crohn's disease in the same period, but only 0.4% of the cases of acute appendicitis. In 10 of the 13 cases there was marked fibrous thickening of the appendiceal wall, and in 11 there were epithelioid cell granulomas. Appendectomy was performed in all cases. None had postoperative fistula or later manifestations of the disease within the observation time averaging 6.3 years. The recurrence rate was previously believed to approach that of recurrence after resection in other parts of the intestines. Collective review of this and three other relatively large case series gave an estimated recurrence rate of 3.5%. We conclude that in Crohn's disease initially confined to the appendix the course appears to be indolent.

Acute Disease↗