Figure 1
Teaching Case
SectionAbdominal imaging
Case TypeClinical Cases
AuthorsGherarducci G, Ceccarelli A, Bemi P, Cappelli C, Mazzeo S, Bartolozzi C
Connected authors
52 years, male
A 52-year-old man was admitted to the hospital for right lower abdominal pain, prickling of the right leg, intermittent diarrhoea lasting for 1 month and a single episode of enterorrhagia.
Laboratory data showed the presence of neutrophilia (13.200/mm3) and normal tumour markers levels (CEA, CA 19.9).
Laboratory data showed the presence of neutrophilia (13.200/mm3) and normal tumour markers levels (CEA, CA 19.9).
Primary mucinous cystadenocarcinoma of the appendix
Acute appendicitis
Colon cancer
A colonoscopy was initially performed and it revealed an exuberant and slightly friable appendiceal mass, no other intraluminal bowel pathology was seen.
The patient underwent ultrasound (US) examination (both baseline and post contrast agent administration) that revealed the presence of a thickened wall of the appendix with both fluid and solid intraluminal components. After contrast agent administration (SonoVue) a vegetating intraluminal lesion originating from the appendix base was better highlighted, clearly enhancing and characterised by a central arterious vascular structure in the arterial phase (Fig. 1, 2).
The patient underwent a computed tomography (CT) scan with iv contrast agent administration and oral hyperhydration with an isotonic solution.
Findings included a broad appendiceal mass with thickened walls showing contrast enhancement and diffuse thickening of the surrounding adipose tissue, extended for 10 cm and reaching the pelvic region, where it adhered to the sigma (Fig. 3-5).
The patient underwent ultrasound (US) examination (both baseline and post contrast agent administration) that revealed the presence of a thickened wall of the appendix with both fluid and solid intraluminal components. After contrast agent administration (SonoVue) a vegetating intraluminal lesion originating from the appendix base was better highlighted, clearly enhancing and characterised by a central arterious vascular structure in the arterial phase (Fig. 1, 2).
The patient underwent a computed tomography (CT) scan with iv contrast agent administration and oral hyperhydration with an isotonic solution.
Findings included a broad appendiceal mass with thickened walls showing contrast enhancement and diffuse thickening of the surrounding adipose tissue, extended for 10 cm and reaching the pelvic region, where it adhered to the sigma (Fig. 3-5).
Neoplastic lesions of the appendix are rare (0,08% of resected appendixes) and preoperative diagnosis is notoriously difficult. Histopathological reviews of appendectomy specimens have shown that the most common malignant neoplasms of the appendix are carcinoid tumors followed by adenocarcinomas. According to the surgery literature the mean age of patients with primary mucinous cystadenocarcinoma is 56 years.
Although the clinical presentation and natural history of appendiceal adenocarcinoma are not well elucidated because of its rarity, the most common presentation is acute right lower abdominal pain, palpable mass and ascites, which is suggestive of acute appendicitis, so that most reported patients had the diagnosis made only several days after the surgery, at the time of histopathologic examination.
Mucinous cystadenocarcinoma is a well-differentiated, slowly progressive neoplasm, that shows mass effect of adjacent organs rather than infiltrating them, so that usually lymph nodes, liver and lungs are not involved with metastasis.
Colonoscopic features of neoplastic lesions of the appendix have been described but are not specific, because in some patients precise endoscopic definition of the site of origin (caecal or appendiceal) may be difficult. Right hemicolectomy is the treatment of choice for all malignant appendiceal neoplasms, except for small classical carcinoids, less than 2 cm in diameter, that are located at the tip of the appendix with a low proliferative index, without angiolymphatic or mesoappendiceal extension.
The most fearful complication is pseudomyxoma peritonei secondary to spontaneous or iatrogenic rupture of the appendix and consequent spillage of neoplastic cells and mucin into the peritoneal cavity.
Our patient was subjected to right hemicolectomy including excision of the mass, partial resection of the terminal ileum, pericaecal mesenterectomy and seriated peritoneal biopsies (Fig. 6).
Histology stated the diagnosis of cystadenocarcinoma of the appendix involving one lymph node but not the peritoneum. The patient received chemotherapy treatment.
The risk of neoplastic epithelial change in the appendix is likely to be similar to the colon because of the biological characteristics of both epithelia are also similar, but the true risk of colon cancer in patients with appendiceal neoplasia is not known. It has been estimated that the risk of synchronus colon neoplasia is 3%, with a further 2% to 3% risk of metachronous cancer in patients with colorectal cancer.
Considering this regular colonoscopic surveillance for metachronous tumor is recommended in patients with primary neoplastic appendiceal diseases, including carcinoid tumors.
Although the clinical presentation and natural history of appendiceal adenocarcinoma are not well elucidated because of its rarity, the most common presentation is acute right lower abdominal pain, palpable mass and ascites, which is suggestive of acute appendicitis, so that most reported patients had the diagnosis made only several days after the surgery, at the time of histopathologic examination.
Mucinous cystadenocarcinoma is a well-differentiated, slowly progressive neoplasm, that shows mass effect of adjacent organs rather than infiltrating them, so that usually lymph nodes, liver and lungs are not involved with metastasis.
Colonoscopic features of neoplastic lesions of the appendix have been described but are not specific, because in some patients precise endoscopic definition of the site of origin (caecal or appendiceal) may be difficult. Right hemicolectomy is the treatment of choice for all malignant appendiceal neoplasms, except for small classical carcinoids, less than 2 cm in diameter, that are located at the tip of the appendix with a low proliferative index, without angiolymphatic or mesoappendiceal extension.
The most fearful complication is pseudomyxoma peritonei secondary to spontaneous or iatrogenic rupture of the appendix and consequent spillage of neoplastic cells and mucin into the peritoneal cavity.
Our patient was subjected to right hemicolectomy including excision of the mass, partial resection of the terminal ileum, pericaecal mesenterectomy and seriated peritoneal biopsies (Fig. 6).
Histology stated the diagnosis of cystadenocarcinoma of the appendix involving one lymph node but not the peritoneum. The patient received chemotherapy treatment.
The risk of neoplastic epithelial change in the appendix is likely to be similar to the colon because of the biological characteristics of both epithelia are also similar, but the true risk of colon cancer in patients with appendiceal neoplasia is not known. It has been estimated that the risk of synchronus colon neoplasia is 3%, with a further 2% to 3% risk of metachronous cancer in patients with colorectal cancer.
Considering this regular colonoscopic surveillance for metachronous tumor is recommended in patients with primary neoplastic appendiceal diseases, including carcinoid tumors.
References
[1] HJ Freeman, DL Webber, AT Meneghetti (2009) An appendiceal neoplastic lesion:case report and implications for colonoscopic screening and surveillance. Can J Gastroenterology 23:379-381 (PMID: 19440570)
[2] Lim HK, Lee WJ, Kim SH, Kim B, Cho JM, Byun JY (1999) Primary Mucinous Cystadenocarcinoma of the appendix, CT findings. AJR 173:1071-1074 (PMID: 10511181)
[3] Ng SS, Lee JF (2009) Mucinous cystadenoma of the appendix. Can J Surgery 52:158-159 (PMID: 19399213)
[4] KW Ma, NH Chia, HW Yeung, MT Cheung (2010) If not appendicitis, then what else can it be?A retrospective review of 1492 appendectomies. Hong Kong Med J 16:12-7 (PMID: 20124568)
Case information
| URL: | https://www.eurorad.org/case/9105 |
| DOI: | 10.1594/EURORAD/CASE.9105 |
| ISSN: | 1563-4086 |
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