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Year : 2006  |  Volume : 16  |  Issue : 4  |  Page : 473-475
Case report: Primary appendiceal malignancy- an unusual case report and review of literature

Department of Radio-diagnosis, Gujarat Cancer and Research Institute, Asarwa, Ahmedabad - 380016 Gujarat State, India

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Date of Submission08-Jul-2006
Date of Acceptance10-Oct-2006

Keywords: Appendiceal Malignancy, USG, CT Scan

How to cite this article:
Soni H C, Patel S B, Goswami K G, Gohil Y. Case report: Primary appendiceal malignancy- an unusual case report and review of literature. Indian J Radiol Imaging 2006;16:473-5

How to cite this URL:
Soni H C, Patel S B, Goswami K G, Gohil Y. Case report: Primary appendiceal malignancy- an unusual case report and review of literature. Indian J Radiol Imaging [serial online] 2006 [cited 2021 Mar 1];16:473-5. Available from:

   Introduction Top

Primary appendiceal malignancy makes up only about 0.5% of all intestinal tumors. They are important because they are rarely diagnosed pre-operatively or per-operatively. Usually patient present with non-specific abdominal pain or sign of acute appendicitis1. The main types of appendiceal neoplasm are carcinoid and adeno-carcinoma. Primary adeno-carcinoma of the appendix is rare. We report a case of appendiceal adeno-carcinoma of colo-rectal type.

   Case History Top

75 years old female patient presented with complain of discomfort at right iliac fossa since one month. Patient has no history of bowel complains, fever or vomiting. On examination there is presence of mild tenderness at right iliac fossa with no evidence of rebound tenderness or fever. Pathological study reveals normal leucocyte count and ESR. Plain X-ray abdomen of patient was normal. USG of abdomen was performed with 7.5 MHz linear array transducer on RT 3200 Advantage II machine using the approach described by Rioux2. On USG there is presence of hypoechoic mass with central bright echoes noted in relation with medial wall of caecum below ileo-caecal junction involving appendix. The whole lesion is surrounded by echogenic fat. Light compression with probe produces only mild discomfort. There is no evidence of ascites or collection near the lesion. No evidence of walled of the lesion by omentum. Liver, Gall bladder, Spleen, Both kidneys and para-aortic region appears normal. Subsequent CT scan of patient done which reveals presence of irregular minimally in-homogeneously enhancing mass lesion medial to caecum involving base of appendix region.

On USG we put the diagnosis of malignant lesion of appendix with appendicolith. CT scan of patient reveals same findings as of USG. Patient was operated and our findings were confirmed per-operatively. Histopathology report of lesion reveals Adeno-carcinoma of appendix.

   Discussion Top

Neoplasm of the appendix is rare. The most common appendiceal malignancy is carcinoid, which comprises up to 35% of all appendiceal tumor3. Second most common tumor is adeno-carcinoma.

Carcinoid tumors are known to be distributed more commonly at the tip then at the base of the appendix, because they probably arise from sub-epithelial neuroendocrine cells deep within lamina propria that are more abundant in the distal appendix4. Since 80% of appendiceal carcinoid tumors are less than 1cm in size, the diagnosis is usually not made preoperatively on radiological examination. Contrast studies are not usually useful in the diagnosis of primary appendiceal carcinoid, although they may demonstrate non-filling of the appendix or in some cases mass effect of the caecum. On USG there is hypoechoic mobile mass at the tip of the appendix indicating carcinoid tumor. CT cannot usually visualize small appendiceal masses, it is useful for evaluation of possible metastasis. The primary site of metastasis is the liver. Carcinoid metastasis is hypervascular and is best visualized on scans performed with intravenous contrast and imaged in the arterial phase of liver enhancement.

Adenocarcinoma of the appendix occurs in 0.1% of appendices. The two main types of adenocarcinoma of the appendix are [1] cystadenocarcinoma or the [2] colorectal type adenocarcinoma. Since almost 70% of patients with adenocarcinoma of the appendix present with signs and symptoms suggesting acute appendicitis, the diagnosis is rarely made pre-operatively by imaging modalities. Most of the diagnosis is made during surgery or after examination of the pathologic specimen. In cases of appendiceal adenocarcinoma, USG is one of the most sensitive modality. The contrast enema cannot distinguish between acute appendicitis and adenocarcinoma of the appendix. Likewise, the findings on CT can also be identical, consisting of inflammation and soft tissue thickening of appendix and adjacent fat.

Cystadeno-carcinomas typically demonstrate abundant extra cellular mucin. Mucocele of the appendix is a condition caused by an abnormal accumulation of mucus in a dilated appendix. Preoperative diagnosis of mucocele of the appendix is crucial to attempt to avoid rupture at surgery. The term mucocele of the appendix is a descriptive term indicating that the appendix is distended by mucin. This can occur from hyperplasia, a benign mucinous cystadenoma or a mucinous cyst adenocarcinoma. The etiology of the mucocele is determined at pathology and cannot typically be determined on radiological examinations.

Barium enemas in patients with mucocele of the appendix classically demonstrate extrinsic compression on the caecum or adjacent small bowel. Also, there is non-filling of the appendix. However, these findings are very nonspecific. On ultrasound, a mucocele typically appears as a cystic mass in the right lower quadrant adjacent to the caecum. Ultrasound, usually demonstrates good through-transmission and posterior enhancement. On ultrasound, mucocele wall is typically thin which helps distinguish a mucocele from appendiceal wall thickening in appendicitis5. On CT, the normal appendix appears as a tubular

Structures with thin walls and should not measure more than 6mm in diameter. A mucocele of the appendix appears as a low attenuation well-defined mass in the right lower quadrant adjacent to the caecum. Mucocele are typically retrocaecal, because 65% of appendixes are retrocaecal in location. The CT may also demonstrate mass effect5. A key differential point is the lack of appendiceal inflammation. This helps distinguish mucocele from acute appendicitis. On CT, there is enhancement of the wall of the mucocele. The wall may either be thick or thin. The thickness of the wall does not help distinguish between neoplastic and non-neoplastic causes of mucocele of the appendix. The presence of an intramural nodule may help suggest the diagnosis of cystadenocarcinoma as the cause of the mucocele. Almost 50% of patients with cystadenocarcinoma of the appendix will develop pseudomyxoma peritonei. This can be well visualized on CT. Pseudomyxoma peritonei appears as multiple foci of gelatinous material throughout the peritoneum.

Colorectal type adenocarcinoma is less common. Most occurs near base of appendix and appears as a firm nodular or diffuse swelling that can occlude lumen of appendix and lead to appendicitis. On USG it appears as hypo-echoic lesion near base of appendix or replacing whole of the appendix. Lymph nodes at meso-appendix or mesentery may get enlarged and appears hypo-echoic indicating secondary spread.

Metastasis to the appendix has been reported with ovarian cancer, breast cancer, stomach cancer, and lung cancer6. Other malignant tumors of the appendix have been reported but are extremely rare. These include malignant lymphoma, smooth muscle tumors, nerve sheath tumors, ganglioneuromas, and Kaposi sarcoma.

Patient with appendix malignancy should undergo right hemicolectomy, but appendicetomy can be tried for carcinoid tumors less then one centimeter in size. All patients with an appendiceal malignancy should be followed since 15-20% of them develop a second malignancy.

   Conclusion Top

Neoplasm of the appendix are rare and continue to be a diagnostic challenge to the clinician, advancements in radiological technology such as improvement in enteroclysis techniques, newer more sensitive USG machines, the development of Multislice CT, and CT enteroclysis helps lot to improved diagnosis and preoperative staging of these tumors[6].

   References Top

1.Jonsson T. Johansson JH, Hallgrimsson JG: Carcinoid tumors of the appendix in children younger then 16 years. Acta Chir Scand 155:113-116,1989  Back to cited text no. 1    
2.Rioux M: Sonographic detection of the normal and abnormal appendix. AJR Am J Roentgenology 158:773-778,1992.  Back to cited text no. 2    
3.Godwin JD: Carcinoid Tumours: An analysis of 2837 cases. Cancer 36:560- 569, 1975  Back to cited text no. 3    
4.Shaw PAV: Carcinoid tumours of the appendix are different. J Pathol 162:189-190, 1990  Back to cited text no. 4    
5.Kim SH et al. Mucocele of the Appendix: Ultrasonographic and CT findings. Abdominal Imaging 23, 292-296, 1998  Back to cited text no. 5    
6.Wolf C et al. Metastasis to the Appendix: Sonographic Appearance and Review of the Literature. J Ultrasound Med 18: 23-25, 1999   Back to cited text no. 6    

Correspondence Address:
H C Soni
Department of Radio-diagnosis, Gujarat Cancer & Research Institute, Asarwa, Ahmedabad - 380016 Gujarat State
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/0971-3026.32249

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  [Figure - 1], [Figure - 2], [Figure - 3], [Figure - 4]

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