Appendiceal Lesion Research Articles

Intestinal metaplasia and colonization of endometriosis in a case of an appendiceal mucinous neoplasm

Intestinal endometriosis is reported in 15–37 % of patients with pelvic endometriosis [1]. Making a correct diagnosis can be challenging for clinicians as well as for pathologists as this disease is able to mimic other pathologies such as inflammatory bowel disease, infectious etiologies, or neoplasms. The appendix is less commonly involved than the small or large intestine. Endometriosis of the appendix is often asymptomatic and discovered as an incidental finding, but it may also present as an acute or chronic appendicitis, occasionally with the formation of an inflammatory mass, mimicking a neoplasm [1]. A few case reports describe the development of obstructive mucocoeles secondary to endometriosis of the appendix. These mucin-filled cystic dilatations of the appendix were lined by normal epithelium without hyperplastic or neoplastic changes [2]. We report the case of an appendiceal mucinous neoplasm in association with endometriosis in a 48-yearold woman. The appendiceal lesion was an incidental finding in this patient who underwent imaging for an episode of pancreatitis. Surgery was performed. Microscopic examination demonstrated that the cystic lesion was covered by a mucosa with serrated and dilated crypts that assumed Land inverted T-shapes (Fig. 1). The crypts were lined by columnar epithelial cells with low-grade atypia. With the presence of extra-appendiceal acellular mucin, this lead to the diagnosis of a low-grade mucinous neoplasm with low risk of recurrence [3]. In addition, the wall of the appendix showed numerous foci of endometriosis consisting of endometrial glands embedded in variable amounts of endometrial stroma, confirmed with ER and CD10 immunohistochemistry (not shown). Interestingly, some of the glandular structures that were embedded in the endometrial stroma were lined by mucinous epithelium, very similar to the epithelium observed in the appendiceal mucinous neoplasm. Immunohistochemistry demonstrated CK7 expression in the normal endometrial epithelium and CK20 expression in the mucinous epithelium. Two theories could explain this finding. Firstly, the epithelium of the appendiceal mucinous neoplasm colonizes the endometrial glands of the appendiceal endometriosis. In intestinal endometriosis, endometrial glands can merge with the surface epithelium and be continuous with it [1, 4]. Secondly, the endometrial glands undergo metaplastic mucinous changes. Mucinous metaplasia in endometriosis is a well-known phenomenon, although it more often mimics endocervical epithelium. Intestinal-type mucinous metaplasia with goblet cells and Paneth cells has been described, but is rare [5]. In our opinion, both explanations can account for the observations in this case. In a few glands, both types of epithelium were continuous. The transition between the normal endometrial epithelium and the mucinous epithelium was very abrupt. Focally, some Paneth cells were present. The glands were dilated and showed serrated growth pattern. Similar cytological and architectural characteristics as recognized in the neoplasm and the sharp demarcation between both types of epithelium suggest that the mucinous changes of the epithelium are due to colonization of the endometrial glands (Figs. 2, 3, 4, and 5). In other areas, the L. Libbrecht :K. Geboes : C. Cuvelier : L. Ferdinande (*) Department of Pathology, Ghent University Hospital, De Pintelaan 185, 9000 Ghent, Belgium e-mail: Liesbeth.Ferdinande@UGent.be

Pseudomyxoma Peritonea: Uncommon Presentation

Pseudomyxoma peritonei is rare being characterized by intraperitoneal accumulation of mucinous ascites produced by neoplastic cells, mostly originating from a perforated appendiceal adenoma. The clinical presentatation of the disease is variable, and preoperative diagnosis is often difficult. We describe the clinical case of a 60-year-old patient who presentated predominantly with urological symptoms. CECT revealed an appendiceal lesion infiltrating and projecting into the urinary bladder. Surgical cytoreduction was performed and patient remains symptomatically better on follow up.

Appendiceal carcinoid at a large tertiary center: pathologic findings and long-term follow-up evaluation

Background Appendiceal carcinoid tumor is the most common type of primary appendiceal malignant lesion. Nonetheless; it is an exceedingly rare diagnosis; found in only about .3% to .9% of appendectomy specimens. We report the clinical and pathologic characteristics of carcinoids found with long-term follow-up evaluation. Methods Data of patients who were histologically reported to have carcinoid tumor of the appendix; in a single center; during a 16-year period were collected retrospectively. In addition; patients were contacted for current follow-up information. Results A total of 44 patients were diagnosed with appendiceal carcinoid during the study period. The median follow-up period for the cohort was 7 years. No evidence of any recurrent disease or other noncarcinoid neoplasm was found. Conclusions Our data confirm the good prognosis as seen in earlier reports. The long-term significance of having a carcinoid; and whether this poses a risk for future neoplasms; need even longer-term studies.

Mucocèles appendiculaires, pseudomyxomes péritonéaux et tumeurs mucosécrétantes du carrefour iléocæcal : apport de l’imagerie dans la stratégie thérapeutique et le choix de la voie d’abord laparoscopique ou classique

The treatment of pseudomyxoma peritonei (PMPs) and appendiceal mucocele (AM) has changed radically. To assess the contribution of preoperative imaging to the treatment strategy and choice of approach, a surgeon and a radiologist different from the initial radiologist examined the files of all patients treated for PMP or AM in four facilities in one district from January 1, 1996, through December 31, 2008. The study included 27 patients (20 men and seven women, mean age: 63+/-13 years). Eleven patients had an intact AM, seven synchronous PMP (malignant appendiceal lesion in two of seven), six metachronous PMP (five with peritoneal mucinous carcinomatosis and one with diffuse peritoneal adenomucinosis) and three a ruptured AM but not PMP. The incidence of mucin-secreting tumors observed (27 cases in 12 years in a region of 500 000 inhabitants) corresponds to a prevalence of approximately five cases per year per million inhabitants. Acute clinical pictures (7/27) were significantly more frequent for the malignant forms (5/7) (p<0,02). The overall sensitivity of computed tomography (CT) for all the criteria studied was 93%. The predictive value for AM rupture of visualization of thick calcifications was 100%. On the other hand, rupture never occurred when the CT showed an AM under pressure, with thin walls and septa. The predictive value for PMP of "scalloping" was 100%. The diagnostic accuracy of the initial reading was 25/27 for the imaging overall and 25/25 for the CT. Preoperative visualization of the exact size of the intact AM or of diagnostic information about ruptured AM and PMP helped to select an appropriate approach in 25 of 27 cases.

An Appendiceal Neoplastic Lesion: Case Report and Implications for Colonoscopic Screening and Surveillance

A 65-year-old man with a history of previously resected colonic adenomas had an apparent cecal lesion detected during colonoscopy. The polyp proved to be a tubulovillous adenoma with high-grade dysplasia involving most of the body of the appendix along with the base of the cecum. The appendiceal mucosa is biologically similar to the colonic mucosa, yet remains relatively 'hidden' in screening and surveillance studies, which suggests important implications for evolving detection strategies in the follow-up of patients with a previous colon polyp or cancer resections. Although endoscopic removal of the appendix has been reported, treatment of these localized appendiceal lesions requires a wide surgical excision.

Small cell carcinoma of the appendix

BackgroundAn extrapulmonary small cell carcinoma is a rare condition. It has similar histological features to pulmonary small cell carcinoma and is equally aggressive.Case presentationWe present the case of a 60-year-old woman who presented with right upper quadrant pain. Computerised tomography revealed an appendiceal lesion and multiple liver metastases. Exploratory laparotomy and right hemicolectomy was performed with histopathological analysis confirming a primary small cell carcinoma of her appendix.ConclusionThis is the first reported case of a pure extrapulmonary carcinoma arising from the appendix.

Appendiceal neoplasms and pseudomyxoma peritonei: A population based study

Background Pseudomyxoma peritonei (PMP) is a rare disease with an estimated incidence of 1 per million per year, and is thought to originate usually from an appendiceal mucinous epithelial neoplasm. However it is not known exactly how often these neoplasms lead to PMP. The aim of this study is to investigate the incidence of both lesions and their relation. Methods The nationwide pathology database of the Netherlands (PALGA) was searched for the incidence of all appendectomies, the incidence of primary epithelial appendiceal lesions and the incidence and pathology history of patients with PMP. All regarded the 10-year period of 1995–2005. Results In the 10-year period 167,744 appendectomies were performed in the Netherlands. An appendiceal lesion was found in 1482 appendiceal specimens (0.9%). Nine percent of these patients developed PMP. Coincidentally, an additional epithelial colonic neoplasm was found in 13% of patients with an appendiceal epithelial lesion. A mucinous epithelial neoplasm was identified in 0.3% (73% benign, 27% malignant) of appendiceal specimens and 20% of these patients developed PMP. For mucocele and non-mucinous neoplasm the association with PMP was only 2% and 3%, respectively. From the nationwide database 267 patients (62 men and 205 women) with PMP were identified, which demonstrates an incidence of PMP in the Netherlands approaching 2 per million per year. The primary site was identified in 68% and dominated by the appendix (82%). Conclusions Primary epithelial lesions of the appendix are rare. One third of these lesions are mucinous epithelial neoplasms and especially these tumours may progress into PMP. The incidence of PMP seems to be higher than thought before. Furthermore there is a considerable risk of an additional colonic epithelial neoplasm in patients with an epithelial neoplasm at appendectomy.

Ulcerative colitis and appendiceal lesion

Ulcerative colitis and appendiceal lesion

Molecular Genetic Evidence Supporting the Clonality and Appendiceal Origin of Pseudomyxoma Peritonei in Women

Pseudomyxoma peritonei (PMP) is a poorly understood condition characterized by mucinous ascites and multifocal peritoneal mucinous tumors. Women with PMP often have mucinous tumors involving both the appendix and the ovaries. Several previous histopathological and immunohistochemical studies of PMP have suggested that most, if not all, cases of PMP in women are derived from mucinous adenomas of the appendix rather than from primary ovarian tumors. A few studies of the molecular genetics of PMP have been recently reported. However, these studies analyzed only a small number of cases and some included a heterogeneous group of mucinous tumors, including both benign and malignant appendiceal and ovarian tumors. We analyzed K-ras mutations and allelic losses of chromosomes 18q, 17p, 5q, and 6q in a substantial number of morphologically uniform cases of PMP with synchronous ovarian and appendiceal tumors as well as in appendiceal mucinous adenomas (MAs) and ovarian mucinous tumors of low malignant potential (MLMPs) unassociated with PMP. Each of the 16 PMP cases (100%) analyzed demonstrated identical K-ras mutations in the appendiceal adenoma and corresponding synchronous ovarian tumor. K-ras mutations were identified in 11 of 16 (69%) appendiceal MAs unassociated with PMP and in 12 of 16 (75%) ovarian MLMPs unassociated with PMP. Two PMP cases showed identical allelic losses in the matched ovarian and appendiceal tumors. A discordant pattern of allelic loss between the ovarian and appendiceal tumors at one or two of the loci tested was observed in six PMP cases. In all but one instance, LOH was observed in the ovarian tumor, whereas both alleles were retained in the matched appendiceal lesion, suggesting tumor progression in a secondary (metastatic) site. Our findings strongly support the conclusion that mucinous tumors involving the appendix and ovaries in women with PMP are clonal and derived from a single site, most likely the appendix.

Appendiceal Involvement in Patients with Ulcerative Colitis

Abstract: There have been few case reports of ulcerative colitis with appendiceal involvement because the appendix has generally received little attention in ulcerative colitis patients. We encountered an inflammatory appendiceal lesion in a patient with ulcerative colitis, which piqued our interest in endoscopic findings of the appendix in these patients. Subsequently, we carefully observed the appendiceal orifice during colonoscopy in patients with ulcerative colitis. From December 1994 to December 1996, 44 patients with ulcerative colitis underwent colonoscopy in Nagaoka Red Cross Hospital. Among these 44, there were three in whom it had not been possible to observe the cecum. During this period, we encountered inflammatory appendiceal lesions in eight cases. Therefore, 20% (8/41) of patients with ulcerative colitis undergoing colonoscopy had appendiceal involvement. Five of these eight patients showed a colonoscopically normal cecum, such that appendiceal involvement thought to be a colonoscopic skip lesion was seen in five (12%: 5/41). There was only one case who had an appendiceal lesion without a microscopically diseased cecum. Appendiceal involvement may be frequent in ulcerative colitis. We thus recommend that endoscopists meticulously examine the appendiceal orifice during colonoscopy in patients with ulcerative colitis.

PRIMARY ADENOCARCINOMA OF THE APPENDIX-A CASE REPORT AND A REVIEW OF 132 CASES IN JAPAN-

This paper describes a case of primary adenocarcinoma of the appendix in a 53-year-old woman, together with an analysis of 132 cases seen in the Japanese literature from 1985 to 1995. The patient was admitted to the hospital because of right lower quadrant pain lasting for about one month when the pain was repeatedly intensified and relieved. With abdominal ultrasonography and computed tomography, a preoperative diagnosis of ileocecal abscess associated with appendicitis was made. An ileocecal resection including a two-third portion of the oral side ascending colon was performed. Histologically it was adenocarcinoma of the appendix. In a review of 132 cases reported in Japan in a recent decade, the chief complaints were unspecific such as right lower quadrant pain in 40%, abdominal mass in 14% and genitourinary complaint in 11% of the cases. Twenty-one patients had no symptoms caused by the appendiceal lesion. Preoperative diagnosis was made as acute appendicitis in 26%, cecal cancer in 6%, cancer of the ascending colon in 4%, ileocecal mass in 3% and cancer of the sigmoid colon in 2%, whereas cancer or tumor of the appendix in 20%. Another review of Japanese cases before 1987 shows that the correct preoperative diagnosis was made in much fewer cases than those of the last 10 years. In our review the number of the patients diagnosed as appendiceal carcinoma or appendiceal tumor, who preoperatively underwent ultrasonography, computed tomography, barium enema, or colon fiberscopy, was double of those whose preoperative diagnoses were incorrect. This fact shows that these methods should be tried to improve the preoperative correct diagnosing rate.

Intussusception of the appendix: A report of three cases with different clinical and pathologic features

Three cases of intussusception of the appendix (IA) with distinctive pathologic changes were reported. All patients were women with different clinical presentations. Grossly, a complete intussusception was found in one case (case 1), while the others (cases 2 and 3) showed a partial intussusception. In case 1, almost the total segment of the appendix bearing the villous adenoma with focal malignant transformation became completely telescoped into the cecum. In case 2, no underlying appendiceal lesion was disclosed. In case 3, appendiceal endometriosis was found as the point of traction. Awareness of such a rare complication associated with various appendiceal lesions provides a clue for making an accurate diagnosis and selecting appropriate surgical management.

Adenocarcinoma of the Appendix in Older Patients

Acute inflammation of the appendix secondary to luminal obstruction is the chief reason for appendectomy. The rare association of a malignant neoplastic process with the inflammatory process is usually an unexpected finding and is often not diagnosed until the histologic study has been completed. Two patients with adenocarcinoma of the appendix are presented. They exemplify the diverse situations of intraoperative recognition with immediate definitive treatment, or surgical management after appendectomy alone. A review of the literature confirms the rarity of appendiceal adenocarcinoma in older patients. In this report, the pathophysiology of appendiceal carcinoma and guidelines for therapy are outlined. Emphasis is placed on the possible occurrence of a malignant appendiceal lesion in the elderly patient with acute appendicitis.

Unusual roentgenographic manifestations of mucocele of the appendix.

Acute appendicitis occurs with such frequency that other lesions of this organ are often forgotten, overlooked, or not considered by the radiologist. This is particularly true when a soft-tissue mass is identified within the abdomen, in an unusual or atypical position for an appendiceal lesion. One such case is presented in this paper. Woodruff and MacDonald (3), in reviewing 146 cases out of approximately 43,000 appendectomies at the Mayo Clinic, found that simple mucoceles of the appendix may occur at almost any age. Apparently, however, they are more frequent in the fourth, fifth, and sixth decades of life, with a slightly higher incidence in women. Cystocarcinomas are less common than simple mucoceles of the appendix and are the result of malignant change in the wall of the benign mucocele. Mucoceles of the appendix are reputed to displace the cecum medially and may on occasion contain calcium in the wall or substance of the mass (1). A 49-year-old white male was admitted through the emergency room because of abdominal pain which had been present for one week. The patient stated that he had had lower abdominal pain, fever, and constipation about two months previously. He had also noticed firmness in the left flank, which he thought was possibly muscles enlarging as a result of exercise. Because of an enlarging abdomen he had his trouser waistline widened. His wife noticed his eyes were yellow one week before admission, and he observed that his urine had been brown. Physical examination revealed a well nourished patient. A large hard mass, estimated to measure 30 × 15 cm, occupied the left half of the abdomen, crossing the midline in the suprapubic region. It could not easily be demarcated by palpation, however. The liver edge was palpable 3 finger-breadths below the costal margin. X-ray examination of the abdomen performed on admission demonstrated a large soft-tissue mass in the left midabdomen (Fig. 1). The following day excretory urography showed no abnormality, but a barium enema disclosed lateral displacement of the medial wall of the descending colon (Fig. 2). On exploratory laparotomy, a large cystic mass which had omentum tightly adherent to its surface was excised. Pathologic examination revealed mucocele of the appendix with malignant change in the wall. The patient's postoperative course was uneventful, but after several months he died. At autopsy widespread metastases were found, including pseudomyxoma peritonei. Summary An unusual case of mucocele of the appendix with malignant change is presented. This is not a rare pathological entity and should be considered in the differential diagnosis of abdominal masses.

Hemorrhage into hydatid of morgagni simulating acute appendicitis

These cases illustrate the similarity of the clinical picture of twisted hemorrhagic hydatid of Morgagni to that of an acute appendiceal lesion. The former however gives a history of a menstrual irregularity and dysmenorrhea (our attack occurred before the onset of menstruation), with only a few of the signs of an acute abdomen.

CYSTS OF THE VERMIFORM APPENDIX.

Cysts of the vermiform appendix have until recently received but little attention. These cysts are without doubt inflammatory in origin. The inflammation results in destruction of a portion of the mucous lining and obliteration of the corresponding canal. The secretion of undestroyed portion of membrane forms a cyst. These cysts are usually of small size, although Virchow has reported one which attained to the size of the fist. Van Hook reports thirty-two cases. The writer removed one from a pregnant woman, in which there was marked hyperesthesia over the right inguinal and lumbar regions, more marked, indeed, than seemed justified by the local lesion. The patient miscarried the day following. Microscopic examination disclosed staphylococci in the contents of the cyst. It has always been with me a question as to how much the symptoms were due to the appendiceal lesion, and how much to the occurrence of an attack of