British Journal of Radiology (2005) 78, 848-850
© 2005 British Institute of Radiology
doi: 10.1259/bjr/62356738
An unusual case of ureteric obstruction
C Messiou, FRCR
A G Chalmers, FRCR
and
S Dexter, FRCS
Radiology Department, Leeds General Infirmary, Great George Street, Leeds, W Yorkshire LS1 3EX, UK
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Abstract
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We report a case of a 33-year-old female presenting with right sided abdominal pain, a right iliac fossa mass and right hydronephrosis. The mass was subsequently shown to represent an isolated desmoid tumour compromising the adjacent ureter. The patient had no predisposing factors for this pathology. This is an uncommon occurrence and we discuss the imaging features and differential diagnosis.
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Introduction
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Intra-abdominal desmoids are uncommon tumours, which occur sporadically or in association with familial adenomatous polyposis (FAP). They have been linked with previous abdominal surgery, oral contraceptives and pregnancy. Although they do not metastasise and are histologically benign, they are locally aggressive with a propensity to invade local structures and recur after resection. Isolated mesenteric desmoids are uncommon and we present an unusual case of ureteric obstruction secondary to an isolated desmoid tumour.
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Case report
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A 33-year-old caucasian female presented with a several week history of right-sided abdominal pain and frequency of bowel habit. There was no relevant past medical history and the patient was taking no medication. On examination, the patient was apyrexial with a normal white cell count. A mass was palpable in the right iliac fossa.
An abdominal and pelvic CT was requested which demonstrated a 4 cm solid mass in the right iliac fossa which was poorly marginated and associated with some prominence of the adjacent mesenteric vasculature (Figure 1
). The mass was compromising the right ureter causing a hydronephrosis (Figure 2) with reduced renal cortical enhancement. A normal terminal ileum was identified separate from the mass and the adjacent colon appeared unremarkable. The remainder of the abdominal and pelvic examination revealed no evidence of a generalized inflammatory process and the appendix could not be identified as a separate structure. The radiological differential diagnosis suggested an organizing appendicitis, a complicated Meckel's, carcinoid, along with the more uncommon inflammatory and neoplastic pathologies of the right iliac fossa.
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Figure 1. CT shows a 4 cm mass in the right iliac fossa (large arrow) with prominence of surrounding vasculature. The mass is seen to encase the right ureter (small arrow).
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Figure 2. (a, b) Coronal CT multiplanar reconstructions that show hydronephrosis of the right kidney (dilated ureter shown by arrow) subsequent to distal compression of the ureter by the right iliac fossa mass (star).
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The patient was admitted for resection of the mass and at operation was found to have a fixed retroperitoneal mass of uncertain origin involving the mesentery of the ileocolic angle. The ureter and right ovarian vessels were found to run through the mass and required resection en block. A right hemicolectomy was performed and the mass was resected off the iliac vessels. The locoregional nodes were cleared. The ureter was repaired by primary anastomosis and a covering J-J ureteric stent was inserted at the same time.
Histologically, the mass was shown to represent a desmoid tumour, which was encasing and superficially infiltrating the ureteric wall. Infiltration was also present within the muscularis propria of the terminal ileum. Tumour encased and distorted the appendix. Resection was considered complete but with only a margin of 1–2 mm. The colon itself was unremarkable.
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Discussion
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The association of mesenteric desmoids and FAP is well established and approximately 10% of patients with FAP will develop desmoid tumours [1]. Few cases of isolated mesenteric desmoids have been reported and cases of ureteric obstruction secondary to an isolated desmoid are very rare. We can find only one other case in the English language literature [2].
Mesenteric desmoids most commonly arise at or close to the root of the small bowel mesentery and CT typically demonstrates an irregular, infiltrating, non enhancing mass [3]. These radiological features bring carcinoid and the more mass like manifestations of sclerosing mesenteritis into the differential diagnosis. Lymphoma and lymphosarcoma can also produce diffuse desmoplastic changes [4].
On MR desmoid tumours are hypointense to muscle on T1 weighted imaging but of variable signal on T2 weighted imaging. There are no specific imaging features on MR or CT to distinguish desmoid tumours from other solid masses [5].
A small series of 23 patients followed up over 13 years demonstrated differences in desmoid tumours in patients with FAP and those which occur sporadically [6]. It showed that desmoids associated with FAP were more likely to be multiple and to involve the mesentery and abdominal wall, whereas isolated desmoids were singular and located in the retroperitoneum and pelvis. Desmoids related to FAP tended to be smaller than idiopathic desmoids with a mean size of 4.8 cm and 13.8 cm, respectively. This particular series also showed post operative recurrences to occur only in the patients with FAP.
Regarding prognosis, the series reported by Smith et al [7] showed apparent cure in the majority of cases with sporadic desmoids who underwent surgical resection. Mortality and morbidity was largely related to cases where small bowel resection was required. Non surgical options included antioestrogens, non-steroidal anti-inflammatory drugs (NSAIDs), chemotherapy and radiotherapy of which there are a few small series and anecdotal reports of success. The authors concluded that in patients with minor symptoms, a trial of observation combined with minimally cytotoxic therapy (i.e. antioestrogens, suldinac) may be preferable to resection, especially in those with FAP and when vascular involvement is a factor. They had also encountered a few anecdotal cases of spontaneous regression which added further doubt as to the usefulness of surgery as a first line therapy. They suggest that surgery should be reserved for the palliation of complications. A more recent trial [8] of neoadjuvant chemoradiotherapy prior to surgical resection has shown promising results, although the current study numbers are small.
In conclusion, a specific diagnosis of an isolated desmoid tumour is rarely made on radiological grounds alone, but should be considered in the differential diagnosis of an infiltrating mass within the right iliac fossa. The main role of CT appears to be in the planning of surgical resection rather than a precise diagnosis.
Received for publication February 1, 2005.
Accepted for publication April 4, 2005.
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References
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- Gurbuz AK, Giardello FM, Peterson GM, et al. Desmoid tumours in familial adenomatous polyposis. Gut 1994;35:377–81.[Abstract/Free Full Text]
- Hailemariam S, Jaeger P, Goebel N, Grant JW. Mesenteric fibromatosis with ureteric stenosis. Postgrad Med J 1988;64:79–81.[Abstract/Free Full Text]
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- Casillas J, Sais GJ, Greve JL, Iparraguirre MC, Morillo G. Imaging of intra- and extraabdominal desmoid tumours. Radiographics 1991;11:959–68.[Abstract]
- Kawashima A, Goldman SM, Fishman EK, et al. CT of intraabdominal desmoid tumours: is the tumour different in patients with Gardners disease. AJR Am J Roentgenol 1994;162:339–42.[Abstract/Free Full Text]
- Smith AJ, Lewis JJ, Merchant NB, Leung DH, Woodruff JM, Brennan MF. Surgical management of intra-abdominal desmoid tumours. Br J Surg 2000;87:608–13.[CrossRef][Medline]
- Baliski CR, Temple WJ, Arthur K, Schachar N. Desmoid tumours: a novel approach for local control. J Surg Oncol 2002;80:96–9.[CrossRef][Medline]
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Abstract
Introduction
