AJR 2005; 184:S97-S98
© American Roentgen Ray Society
Retroperitoneal Fibrosis Involving the Left Kidney in a Patient with a Remote History of Riedel's Thyroiditis
Todd M. Emch and
Margaret A. Miller
Department of Radiology, The Mercy Hospital of Pittsburgh, 1400 Locust
St., Pittsburgh, PA 15219.
Received January 20, 2004;
accepted after revision March 19, 2004.
Address correspondence to T. M. Emch
(te{at}neuucom.edu).
Introduction
Retroperitoneal fibrosis is characterized as a dense fibrotic plaque
surrounding the aorta and inferior vena cava, typically confined to the
retroperitoneum and starting at the level of the renal hila, with extension
into the pelvis along the iliac vessels
[1]. Nine cases have been
reported of atypical perinephric involvement, with four unilateral cases
[2].
Case Report
A 49-year-old man with a history of Riedel's thyroiditis and resultant
right thyroidectomy and isthmusectomy 12 years earlier presented to his
primary care physician with left flank discomfort and a 40-lb weight loss over
the previous 6 months. On physical examination, a firm mass was palpated in
the left side of the abdomen. Unenhanced CT revealed a large, slightly
hyperdense mass engulfing the left kidney
(Fig. 1A). On contrast-enhanced
CT, the mass showed minimal enhancement and was infiltrating a poorly
enhancing hydronephrotic left kidney
(Figs.1B and
1C). A left nephrectomy was
performed, with the mass described as "rock hard" and fixed in
position. Although not apparent on CT, adherence to the aorta had occurred at
the level of the kidneys, with extension to the mesentery of the left colon
and psoas muscle. Histologic examination revealed retroperitoneal fibrosis of
the left kidney, with genotype studies showing no evidence of malignancy.
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Fig. 1B. —49-year-old man with history of Riedel's thyroiditis. CT of
abdomen after contrast agent administration shows hydronephrosis of left
kidney that enhances to lesser degree than normal right kidney. There is
diffuse renal parenchymal infiltration by minimally enhancing mass.
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Fig. 1C. —49-year-old man with history of Riedel's thyroiditis. CT of
abdomen after contrast agent administration shows hydronephrosis of left
kidney that enhances to lesser degree than normal right kidney. There is
diffuse renal parenchymal infiltration by minimally enhancing mass.
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Discussion
Retroperitoneal fibrosis may present with dull pain in the abdomen or
flank, fever, weight loss, an abdominal mass, or with symptoms of ureteral
obstruction [3]. Middle-aged
men are affected twice as often as women
[2]. An association exists
between retroperitoneal fibrosis and the systemic fibroses, including Riedel's
thyroiditis, fibrosing mediastinitis, retroorbital fibrosis, and sclerosing
cholangitis. Retroperitoneal fibrosis in an aortic distribution is most
commonly idiopathic but may be secondary to abdominal aortic aneurysm,
malignancy, infection, radiation therapy, and the use of certain medications,
such as methysergide. One theory that may explain the relationship between
retroperitoneal fibrosis and the systemic fibroses involves ceroid that is
present in the atheromatous plaques of blood vessels that may act as an
antigen. When ceroid leaks through the arterial wall, an immune response can
occur that is supported by eosinophilia that may be present
[3].
Retroperitoneal fibrosis can be treated with surgery that is aimed at
obtaining a diagnosis and relieving obstruction
[1]. Corticosteroids, most
advantageous in the early inflammatory phase, are most often used alone or in
combination with other agents or surgery. One series combining steroids with
surgery decreased ureteral obstruction to 10% from 48%. Once steroids are
discontinued, retroperitoneal fibrosis can recur. Azathioprine can be used
when steroids are ineffective. Less data are available for the use of
methotrexate and cyclophosphamide. Promising results involving tamoxifen have
been reported [4].
Diagnosis requires clinical suspicion, multiple imaging techniques, and,
ultimately, tissue diagnosis. Excretory urography shows medial deviation of
the ureters with proximal dilatation and distal smooth compression
[5]. On unenhanced CT,
retroperitoneal fibrosis appears as a soft-tissue mass that is similar in
density to muscle, typically surrounding the aorta and the inferior vena cava.
Enhancement is greatest in the earlier inflammatory phase, with minimal
enhancement in the fibrotic phase. Although uncommon, extension to the kidney,
small bowel mesentery, colon, bladder, and spinal canal has been reported
[1,
2]. The findings with MRI are
those of fibrosis: low signal intensity on both T1 and T2-weighted images
[2]. The differential diagnoses
include lymphoma, sarcoma, retroperitoneal hematoma, metastatic disease, and,
in addition, in the case of isolated renal involvement, histiocytosis,
amyloidosis, and extramedullary hematopoiesis
[1,
2]. Malignant processes in
contrast to retroperitoneal fibrosis typically displace the ureters laterally,
anteriorly displace the aorta, and can result in bony destruction
[1]. Lymphoma involves the
kidney and the perirenal fat, while extramedullary hematopoiesis and amyloid
involve the perirenal fat [6].
Patterns of enhancement and attenuation values have not shown value in
differentiation of retroperitoneal fibrosis from other entities
[1].
Multiple biopsies must be obtained when performed percutaneously
[4,
7]. Marinoni et al.
[8] describe the correct
preoperative diagnosis of retroperitoneal fibrosis with the combination of
other techniques and CT-guided percutaneous biopsy in four of seven patients.
The remaining three were diagnosed during surgery (two patients) or during
autopsy (one patient). Fine-needle aspiration findings can be nonspecific,
with only two reports of retroperitoneal fibrosis diagnosed by this technique
[9].
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Introduction
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