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Thread: Periappendiceal Carcinoma Pictures - Atlas of Colon and Ileum

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    Default Periappendiceal Carcinoma Pictures - Atlas of Colon and Ileum

    The diverticular disease of vermiform appendix is a rare condition [1, 2]. It may cause inflammation (diverticulitis) and rupture and is associated with pseudomyxoma peritonei, malignant tumors, and other complications [3]. However, appendiceal diverticulitis clinically masquerading as appendiceal carcinoma is very rare.

    Case Report
    A 62-year-old Japanese woman with HCV-related cirrhosis and small hepatocellular carcinoma presented with abdominal pain. The small hepatocellular carcinoma had been treated with microwave coagulation therapy and cured. A blood laboratory test revealed elevation of cirrhosis-related enzymes, bilirubin, and AFP. No leukocytosis or increased C-reactive protein was present. CA125 was elevated. US and CT showed a tumor measuring 5 × 4 × 4 cm in the vermiform appendix (Figure 1). Colon endoscopy showed mucosal elevation and irregularity in the orifice of vermiform appendix (Figure 2). A biopsy of the appendiceal mucosa showed no significant changes. Clinical diagnosis was appendiceal carcinoma and wide excision of the terminal ileum, appendix, cecum, and ascending colon was performed.

    The appendiceal diverticulum has been classified into congenital and acquired ones The former has complete appendiceal walls, while the latter lacks muscular walls. The present case appears congenital in origin. About half of patients with appendiceal diverticulosis are asymptomatic, but it can cause diverticulitis, abscess formation, rupture, perforation, and hemorrhage and is associated with pseudomyxoma peritonei and malignant transformation The incidence of appendiceal diverticulosis ranges from 0.27% to 3.56% in surgical cases and from 0.20% to 1.99% in autopsy series In recent years, the incidence has been reported in 1.7% (22/1361) , 1.7% (10/575) and 2% (67/3343) of surgical cases. Only about 15% of appendiceal diverticula develop diverticulitis Therefore, appendiceal diverticulitis is very rare disease condition.

    It is interesting that the present case was clinically diagnosed as appendiceal carcinoma. In a review of the English literature, the author could not find cases of appendiceal diverticulitis clinically masquerading as appendiceal carcinoma. The lack of blood inflammatory changes and positive mass formation in the imaging modalities of appendix led to the erroneous clinical diagnosis of appendiceal carcinoma in the current case. Therefore, it must be stressed that clinically appendiceal tumor may be due to appendiceal diverticulosis or diverticulitis. In recent years, relationship between appendiceal diverticulosis and appendiceal tumors or tumor-like conditions has been advocated.

    Dupre et al. described that appendiceal diverticulosis is associated with appendiceal carcinoids, adenoma, mucinous tumors, and adenocarcinoma. Lamps et al.described that appendiceal diverticulosis is associated with low grade mucinous neoplasms and pseudomyxoma peritonei. The causal relationship between appendiceal diverticula and tumor formation is unclear, but it may be possible that the presence of tumors causes increased luminal pressure of the appendix, thus giving rise to the development of diverticulosis. It is also possible that the presence of appendiceal diverticulosis may cause tumor formation. This issue remains to be elucidated.
    In summary, when they encounter an appendiceal mass, clinicians should consider appendiceal diverticulitis as a differential diagnosis.
    Periappendiceal Carcinoma Pictures Atlas Colon attachment.php?s=7efb2aa409c32e18be3d114d000d2bb2&attachmentid=2049&d=1442168156

    Acute appendicitis is typically a disease of children and young adults with peak incidence in the 2nd to 3rd decades of life .

    Clinical presentation
    Classically presentation consists of periumbilical pain (referred) which within a day or later localizes to McBurney's point and is associated with fever, nausea and vomiting 2. This progression is only seen in a minority of cases, and is unhelpful in children who often present with vague and nonspecific signs and symptoms. It also relies on the appendix being in a 'normal' position, which is not the case in a large number of cases (see below).

    Plain film
    Plain radiography is infrequently able to give the diagnosis, however is useful in identifying free gas, and may show an appendicolith in 7-15% of cases 1. In the right clinical setting, finding an appendicolith makes the probability of acute appendicitis up to 90%.
    If an inflammatory phlegmon is present, displacement of caecal gas with mural thickening may be evident.
    Small bowel obstruction pattern with small bowel dilatation and air-fluid levels is seen in approximately 40% of perforations.

    Ultrasound with its lack of ionizing radiation should be the investigation of choice in young patients, and is effective in competent hands in identifying abnormal appendixes, especially in thin patients. However, the identification of a normal appendix is more problematic, and in many instances, appendicitis cannot be ruled out.

    Pseudomyxoma peritonei is a term used to describe mucinous ascites or mucin deposits within the peritoneal cavity. Pseudomyxoma peritonei consists of organizing pools of mucin within peritoneal fat or on the serosal surfaces of the viscera, which contain variable numbers of neoplastic epithelial cells. Most cases reflect dissemination of an appendiceal mucinous neoplasm, in which case, mucin pools that contain scant strips and clusters of low-grade neoplastic epithelial cells are typical (Figure 2, D), similar to those present in the appendiceal mucosa (Figure 2, A and B). However, pseudomyxoma peritonei is not synonymous with a neoplasm nor are all neoplastic cases derived from the appendix. Ruptured diverticula of the appendix and colon may spill mucin into the peritoneal cavity, and mucin-producing carcinomas of the colon, pancreas, and other organs may disseminate throughout the peritoneum in the form of gelatinous ascites. Unfortunately, much of the earlier literature is of limited value in assessing the biologic potential of appendiceal mucinous neoplasms and pseudomyxoma peritonei. Most reports before 2000 did not distinguish between neoplastic and nonneoplastic etiologies for pseudomyxoma peritonei, appendiceal and nonappendiceal neoplasms, or cytologically low- and high-grade tumors in the peritoneal cavity.1 In our practice, we generally avoid using pseudomyxoma peritonei as a diagnostic term and limit its inclusion in pathology reports to a comment aimed at facilitating communication with clinicians.
    Periappendiceal Carcinoma Pictures Atlas Colon attachment.php?s=7efb2aa409c32e18be3d114d000d2bb2&attachmentid=2050&d=1442168169

    The combined results of several studies published in theearly 1990s implicated the appendix as a likely site of origin in most of nearly 100 patients with pseudomyxoma peritonei but also suggested that some cases may be derivedfrom either the ovary or peritoneum. Prayson etal2 evaluated a series of men and women with pseudomyxoma peritonei and found that 94% of patients had appendiceal mucinous neoplasms, including all of the women with ovarian tumors. Seidman et al3 found a high frequency of bilateral ovarian mucinous tumors, or right-sided ovarian mucinous tumors in unilateral cases, among women with concomitant mucinous tumors of the appendix, leading them to conclude that pseudomyxoma peritonei may be derived from either site or represent a primary peritoneal neoplasm. Young et al4 studied 22 appendiceal mucinous neoplasms associated with mucinous ovarian tumors and noted that tumors in both sites were histologically similar. They also found ovarian disease to be bilateral in most cases, whereas unilateral ovarian tumors showed a right-sided predominance. Unlike Seidman et al,3 however, they concluded that these features suggested a primary appendiceal origin with secondary ovarian involvement.

    Molecular data further support the notion that pseudomyxoma peritonei is usually derived from the appendix, rather than the ovary or peritoneum. Cautrecasas et al5 analyzed KRAS mutational status in 6 synchronous mucinous tumors of the appendix and ovary, 5 (83%) of which showed identical KRAS mutations in codon in tumors from both sites. Chuaqui et al6 analyzed loss of heterozygosity on chromosomes 5 and 17 in synchronous appendiceal and ovarian mucinous neoplasms obtained from 12 patients. They found either loss of heterozygosity at the same locus or no loss of heterozygosity in paired tumors in 75% of cases. Subsequent studies have shown progressive loss of heterozygosity in ovarian mucinous tumors, when compared with synchronous appendiceal neoplasms, leading most investigators to conclude that most pseudomyxoma peritonei cases are derived from appendiceal mucinous neoplasms, whereas an ovarian origin is considered a rare occurrence.

    Not uncommonly, surgical pathologists encounter appendiceal resection specimens with mucin deposits on the appendiceal serosa, within the mesoappendix, or limited to the right lower quadrant (Figure 3, A and B). Extra-appendiceal mucin deposits limited to the periappendiceal area are usually devoid of neoplastic epithelial cells (acellular), although one may infrequently observe strips or clusters of neoplastic mucinous epithelial cells in periappendiceal mucin, similar to cases with widespread peritoneal disease (Figure 3, C and D). Yantiss et al9 evaluated the prognostic implications of peritoneal mucin localized to the right lower quadrant in 65 patients with appendiceal mucinous neoplasms. Fifty patients (77%) had acellular extra-appendiceal mucin, and 15 (23%) had periappendiceal mucin deposits that contained scant mucinous epithelium with low-grade cytologic features. At follow-up, 48 (96%) patients without extra-appendiceal epithelium were disease free (mean, 52 months), whereas 33% of patients with any neoplastic epithelium outside the appendix developed widespread peritoneal tumor deposits,
    Periappendiceal Carcinoma Pictures Atlas Colon attachment.php?s=7efb2aa409c32e18be3d114d000d2bb2&attachmentid=2051&d=1442168192

    including one patient who died of disease (mean, 38 months). Notably, 2 patients with acellular periappendiceal mucin developed disseminated peritoneal disease, but neither of those appendices was submitted entirely for histologic examination. These results emphasize the importance of adequate sampling in determining the prognosis of patients with appendiceal mucinous neoplasms and indicate that tumors without extra-appendiceal neoplastic epithelium are associated with an excellent prognosis.

    Appendicitis | Radiology Reference Article |
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    Appendiceal Diverticulitis Clinically Masquerading as an Appendiceal Carcinoma

    Last edited by Medical Photos; 09-13-2015 at 06:16 PM.

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