Radiology
HOME HELP FEEDBACK SUBSCRIPTIONS ARCHIVE SEARCH TABLE OF CONTENTS
QUICK SEARCH:   [advanced]


     

DOI: 10.1148/radiol.2352030088
This Article
Right arrow Figures Only
Right arrow Full Text (PDF)
Right arrow Alert me when this article is cited
Right arrow Alert me if a correction is posted
Right arrow Citation Map
Services
Right arrow Email this article to a friend
Right arrow Similar articles in this journal
Right arrow Similar articles in PubMed
Right arrow Alert me to new issues of the journal
Right arrow Download to citation manager
Right arrow reprints & permissions
Citing Articles
Right arrow Citing Articles via HighWire
Right arrow Citing Articles via Google Scholar
Google Scholar
Right arrow Articles by Szarf, G.
Right arrow Articles by Bluemke, D. A.
Right arrow Search for Related Content
PubMed
Right arrow PubMed Citation
Right arrow Articles by Szarf, G.
Right arrow Articles by Bluemke, D. A.
(Radiology 2005;235:829-832.)
© RSNA, 2005

Diagnosis Please

Case 83: Multifocal Fibrosclerosis with Mediastinal-Retroperitoneal Involvement1

Gilberto Szarf, MD and David A. Bluemke, MD, PhD

1 From the Russell H. Morgan Department of Radiology and Radiological Sciences, Johns Hopkins University School of Medicine, 600 N Wolfe St, Baltimore, MD 21287. Received January 10, 2003; revision requested March 20; revision received August 27; accepted October 14. Address correspondence to D.A.B. (e-mail: dbluemke@jhmi.edu).


   History
TOP
 History
Imaging Findings
 Discussion
 REFERENCES
 
A 72-year-old man with a history of congestive heart failure, malignant hypertension, and peripheral and coronary vascular disease was admitted to the hospital 4 days after onset of acute dyspnea and lower-extremity edema. He underwent coronary catheterization; after this procedure, his serum creatinine levels increased from 1.6 to 5.9 mg/dL (141.4–521.6 µmol/L). He was subsequently referred for renal and aortic magnetic resonance (MR) imaging to allow more comprehensive evaluation of renal and vascular disease.

Prior to admission, the patient had undergone coronary artery bypass graft placement for triple-vessel coronary artery disease (5 years previously) and right carotid endarterectomy (1 year previously). Pathologic specimens of the carotid artery showed dense fibrous tissue and chronic inflammation surrounding the vessel wall.


   Imaging Findings
TOP
 History
 Imaging Findings
Discussion
 REFERENCES
 
T1- and T2-weighted MR images of the abdomen demonstrated intermediate soft-tissue signal intensity in the subcapsular and perirenal space that circumferentially envelops the kidneys (Figs 1, 2). There was also tissue with the same MR imaging characteristics surrounding the abdominal aorta, left renal artery (Fig 3), and aortic arch (Fig 4).



View larger version (119K):
[in this window]
[in a new window]
[Download PPT slide]
  		Figure 1. Transverse T1-weighted MR image (repetition time msec/echo time msec, 650/10) of the abdomen at the level of the kidneys. Abnormal slightly hypointense tissue (white arrows) is seen in the perirenal space (compared with the paravertebral muscles) and adjacent to the aorta (black arrow).

 


View larger version (123K):
[in this window]
[in a new window]
[Download PPT slide]
  		Figure 2. Transverse single-shot T2-weighted MR image (single shot/99.4) of the abdomen at the level of the kidneys. Abnormal tissue in the perirenal space has low signal intensity (thick white arrows). Tissue with similar characteristics surrounds the aorta (thin white arrow).

 


View larger version (131K):
[in this window]
[in a new window]
[Download PPT slide]
  		Figure 3. Transverse fat-saturated T2-weighted MR image (1767/91.6 [effective]) of the abdomen at the level of the kidneys. Abnormal hypointense tissue is seen in the perirenal space (thick white arrows). Tissue with similar characteristics surrounds the aorta (arrowhead) and the left renal artery (thin white arrow).

 


View larger version (122K):
[in this window]
[in a new window]
[Download PPT slide]
  		Figure 4. Transverse electrocardiographically gated double inversion-recovery fast spin-echo MR image (1000/18.4 [effective]) of the thorax shows slightly hypointense tissue (arrows) surrounding the aortic arch (compared with the paravertebral muscles).

 

   Discussion
TOP
 History
 Imaging Findings
 Discussion
REFERENCES
 
Multifocal fibrosclerosis is a rare syndrome of unknown cause that is characterized by fibrosis involving multiple organ systems. Comings et al (1) reported the occurrence of different combinations of retroperitoneal fibrosis (RPF), mediastinal fibrosis, Riedel thyroiditis, orbital pseudotumor, and sclerosing cholangitis in two brothers who were the offspring of a first-cousin marriage, and these researchers suggested that the microscopic pathologic findings of all of those disorders were similar. Subsequently, numerous cases were reported showing two or more of these entities, as well as involvement of the kidneys, lacrimal glands, choroid plexus, stomach, pancreas, small bowel mesentery, and pelvic mesocolon (2–12).

Depending on the involved organ systems, different clinical and radiologic manifestations are observed in patients with multifocal fibrosclerosis. In the case presented, there is extensive soft-tissue proliferation, most extensively surrounding the kidneys, but there is also involvement of the abdominal aorta, renal arteries, and thoracic aorta. This patient additionally had soft-tissue fibrosis around the carotid arteries (not shown, but stated in the history). While the abdominal findings alone are typical of perirenal fibrosis and RPF, their combination with the same pattern of disease in the mediastinal and cervical areas denotes generalized systemic involvement; this is termed multifocal fibrosclerosis. These findings were confirmed by means of core biopsy of the tissue surrounding the kidneys and examination of the endarterectomy specimen. In this patient, symptoms of congestive heart failure and coronary artery disease were caused by atherosclerotic disease; atherosclerotic narrowing of the renal ostia also likely contributed to the patient’s renal failure and hypertension.

Mediastinal fibrosis in multifocal fibrosclerosis is characterized by hypointense tissue on T1- and T2-weighted MR images in the mediastinum, especially around the aorta, as in this case. Mediastinal fibrosis can also be associated with superior vena cava obstruction, stenosis of the pulmonary veins, tracheal obstruction (eg, symptoms similar to asthma), esophageal stricture, pulmonary artery obstruction with cor pulmonale, and coronary artery occlusion (1,4). Histologically, it has a similar appearance to that observed in patients with RPF (1,13). Cross-sectional imaging findings are those of an infiltrative soft-tissue process, with variable degrees of deformation of the mediastinal anatomy (2,4).

RPF was a prominent component of multi-focal fibrosclerosis in the case presented, and RPF is the most common manifestation of multifocal fibrosclerosis. RPF is characterized by fibrous tissue proliferation surrounding the aorta and extending into periaortic tissue as a plaque-like infiltrative soft-tissue process (14). In most cases, it is localized around the lower aorta and common iliac arteries in the retroperitoneal space (12), but the pelvic cavity can also be involved (5). About two-thirds of the cases are considered idiopathic; in the remaining one-third, an inciting factor (eg, methysergide, malignancies, radiation therapy, surgery, hemorrhage, retroperitoneal infection, autoimmune diseases) can be identified (15). It has also been suggested that RPF could be secondary to a local autoallergic reaction to components of atherosclerotic plaques, occurring in areas in which the aorta has severe atherosclerotic plaque (13). There is a male-to-female ratio of approximately 3:1. In 70% of patients, the age at diagnosis is between 30 and 60 years, but the disease has been described in children (16).

Two histologic patterns of RPF have been described. One pattern consists of chronic fibrosis that is relatively avascular, acellular, and often calcified. The second pattern demonstrates collagen bundles interspersed with an equal or greater volume of inflammatory cells and mucopolysaccharide. In the later group, small blood vessels are numerous, and the infiltrative process is pleomorphic, consisting of lymphocytes, plasma cells, eosinophils, Russell bodies, fibroblasts, and mast cells. Neutrophils are absent (12). It is hypothesized that the more active type of inflammation matures into the more chronic histologic pattern.

The diagnosis of retroperitoneal fibrosis is often suggested by imaging studies performed to enable the evaluation of nonspecific clinical signs and symptoms. Medial deviation and narrowing of the ureters near the lower lumbar spine (17) demonstrated by excretory urography represents a classic radiologic presentation of RPF. Renal failure may be profound, despite low-grade obstructive uropathy. One proposed mechanism of obstruction is interference with ureteral peristaltic activity rather than mechanical obstruction (18).

Sonographic findings of RPF consist of hydronephrosis and visualization of fibrotic tissue as a hypoechoic mass in the para-aortic region (19). Computed tomography (CT) shows retroperitoneal plaque-like, infiltrative soft-tissue surrounding vessels and ureter. The soft-tissue attenuation seen on CT scans is similar to that of muscle (20). Enhancement is variable after intravenous administration of iodinated contrast material. MR images show an infiltrative soft-tissue process with homogeneous low signal intensity compared with that of the adjacent psoas muscle seen on T1-weighted images (21). However, RPF can show moderate to high signal intensity on T2-weighted images, depending on the degree of associated inflammatory response. Extensive inflammation early in the disease process results in increased T2 signal intensity (21,22). In a series of 17 patients, malignant RPF was characterized by ill-defined margins, increased signal intensity on T2-weighted images, and heterogeneous MR signal intensity (23). MR imaging characteristics, however, are not specific for distinguishing benign from malignant cause. Therefore, biopsy is essential to confirm the diagnosis. Fluorodeoxyglucose positron emission tomography can play a role in establishing the diagnosis and sites of involvement in patients with multifocal fibrosclerosis, and it could be helpful in evaluating disease activity and patient response to corticoid therapy by showing high fluorodeoxyglucose uptake in involved areas (likely caused by active inflammation) (24).

Localized extensions of RPF (19,22,25–32) may be associated with more typical presentations (eg, periaortic disease) that may lead to the correct imaging diagnosis. In the case presented, subtle periaortic abnormalities were present and suggest the diagnosis of RPF.

The primary diagnostic consideration in this case would be treated lymphoma. The other differential diagnoses usually considered for RPF, including sarcoma, metastatic lymphadenopathy, amyloidosis, and extramedullary hematopoiesis (22,33,34), would not appear as hypointense soft tissue on T1- and T2-weighted images around the kidneys and thoracic and abdominal aorta. A particular characteristic of RPF is the tendency to infiltrate and envelop surrounding structures without displacing them. Malignancies, such as lymphomas and sarcomas, typically cause displacement of surrounding structures, especially the aorta (21,35). In addition, the anterior margin of RPF is clearly delineated, respecting peritoneal boundaries, while the posterior margin is poorly defined and not easily separated from subjacent structures (20).

Therapy for multifocal fibrosclerosis includes removal of any identifiable inciting agents, including a search for occult tumors, and suppression of inflammatory process. Steroid therapy is of value in most cases when there is no associated malignancy. In steroid-resistant cases, other forms of immunosuppression may be useful. Medroxyprogesterone acetate, progesterone, and tamoxifen have been described as alternative forms of treatment. Surgical intervention is considered for steroid-resistant cases and for patients who do not tolerate steroids. Long-term follow-up is indicated, as recurrences are unpredictable and may occur from 3 months to more than 10 years after the initial diagnosis and treatment (15).


   ACKNOWLEDGMENTS
 
The authors gratefully acknowledge the assistance provided by Tracy L. McCracken, RN, CCRC, in the preparation of the manuscript.


   FOOTNOTES
 
Part one of this case appeared 4 months previously and may contain larger images.


   REFERENCES
TOP
 History
 Imaging Findings
 Discussion
 REFERENCES
 

  1. Comings DE, Skubi KB, Van Eyes J, Motulsky AG. Familial multifocal fibrosclerosis: findings suggesting that retroperitoneal fibrosis, mediastinal fibrosis, sclerosing cholangitis, Riedel’s thyroiditis, and pseudotumor of the orbit may be different manifestations of a single disease. Ann Intern Med 1967; 66:884-892.
  2. De Backer TL, Mollet P, Clement DL. Images in cardiology: two brothers with mediastinal-retroperitoneal fibrosis. Clin Cardiol 2001; 24:633.[Medline]
  3. Klisnick A, Fourcade J, Ruivard M, et al. Combined idiopathic retroperitoneal and mediastinal fibrosis with pericardial involvement. Clin Nephrol 1999; 52:51-55.[Medline]
  4. Graal MB, Lustermans FA. A patient with combined mediastinal, mesenteric and retroperitoneal fibrosis. Neth J Med 1994; 44:214-219.[Medline]
  5. Stewart TW, Jr, Friberg TR. Idiopathic retroperitoneal fibrosis with diffuse involvement: further evidence of systemic idiopathic fibrosis. South Med J 1984; 77:1185-1187.[CrossRef][Medline]
  6. Oguz KK, Kiratli H, Oguz O, Cila A, Oto A, Gokoz A. Multifocal fibrosclerosis: a new case report and review of the literature. Eur Radiol 2002; 12:1134-1138.[CrossRef][Medline]
  7. Ozgen A, Cila A. Renal involvement in multifocal fibrosclerosis: CT and MRI. J Comput Assist Tomogr 1999; 23:937-938.[CrossRef][Medline]
  8. Owen K, Lane H, Jones MK. Multifocal fibrosclerosis: a case of thyroiditis and bilateral lacrimal gland involvement. Thyroid 2001; 11:1187-1190.[CrossRef][Medline]
  9. Morris WR, Haik BG, Osborn D, Fleming JC. Intraocular involvement in multifocal fibrosclerosis. Ophthalmology 2000; 107:962-966.[CrossRef][Medline]
  10. Fung Y, Yee J, Simpson WL, Glajchen N, Harpaz N. CT appearance of gastric involvement in multifocal idiopathic fibrosclerosis. J Comput Assist Tomogr 2001; 25:239-241.[CrossRef][Medline]
  11. Levey JM, Mathai J. Diffuse pancreatic fibrosis: an uncommon feature of multifocal idiopathic fibrosclerosis. Am J Gastroenterol 1998; 93:640-642.[CrossRef][Medline]
  12. Mitchinson MJ. The pathology of idiopathic retroperitoneal fibrosis. J Clin Pathol 1970; 23:681-689.[Abstract/Free Full Text]
  13. Mitchinson MJ. Retroperitoneal fibrosis revisited. Arch Pathol Lab Med 1986; 110:784-786.[Medline]
  14. Amis ES, Jr. Retroperitoneal fibrosis. AJR Am J Roentgenol 1991; 157:321-329.[Abstract/Free Full Text]
  15. van Bommel EF. Retroperitoneal fibrosis. Neth J Med 2002; 60:231-242.[Medline]
  16. Brooks MT, Magill HL, Hanna SL, et al. Case of the day. Pediatric. Idiopathic retroperitoneal fibrosis. RadioGraphics 1990; 10:1096-1100.
  17. Ormond J. Bilateral ureteral obstruction due to envelopment and compression by an inflammatory retroperitoneal process. J Urol 1948; 59:1072-1079.
  18. Lalli AF. Retroperitoneal fibrosis and inapparent obstructive uropathy. Radiology 1977; 122:339-342.[Abstract]
  19. Barrett RL, Horrow MM, Gubernick JA, Rosenberg HK. US case of the day: retroperitoneal fibrosis with perirenal involvement. RadioGraphics 1995; 15:1024-1026.[Medline]
  20. Fagan CJ, Larrieu AJ, Amparo EG. Retroperitoneal fibrosis: ultrasound and CT features. AJR Am J Roentgenol 1979; 133:239-243.[Abstract]
  21. Mulligan SA, Holley HC, Koehler RE, et al. CT and MR imaging in the evaluation of retroperitoneal fibrosis. J Comput Assist Tomogr 1989; 13:277-281.[Medline]
  22. Bechtold RE, Dyer RB, Zagoria RJ, Chen MY. The perirenal space: relationship of pathologic processes to normal retroperitoneal anatomy. RadioGraphics 1996; 16:841-854.[Abstract]
  23. Arrive L, Hricak H, Tavares NJ, Miller TR. Malignant versus nonmalignant retroperitoneal fibrosis: differentiation with MR imaging. Radiology 1989; 172:139-143.[Abstract/Free Full Text]
  24. Drieskens O, Blockmans D, Van den Bruel A, Mortelmans L. Riedel’s thyroiditis and retroperitoneal fibrosis in multifocal fibrosclerosis: positron emission tomographic findings. Clin Nucl Med 2002; 27:413-415.[CrossRef][Medline]
  25. Yancey JM, Kaude JV. Diagnosis of perirenal fibrosis by MR imaging. J Comput Assist Tomogr 1988; 12:335-337.[Medline]
  26. Triantopoulou C, Rizos S, Bourli A, Koulentianos E, Dervenis C. Localized unilateral perirenal fibrosis: CT and MRI appearances. Eur Radiol 2002; 12:2743-2746.[Medline]
  27. Rominger MB, Kenney PJ. Perirenal involvement by retroperitoneal fibrosis: the usefulness of MRI to establish diagnosis. Urol Radiol 1992; 13:173-176.[Medline]
  28. Ayuso JR, Garcia-Criado A, Caralt TM, Ayuso C, Torras A, Ribalta T. Atypical retroperitoneal fibrosis: MRI findings. Eur Radiol 1999; 9:937-939.[CrossRef][Medline]
  29. Cerisier A, Brulport V, Cathebras P, et al. Periaortic fibrosis with pericardial and perirenal involvement: apropos of a case. Rev Med Interne 1996; 17:154-156.[Medline]
  30. Naudts P, Coolsaet BR, Weinberg R, Coppens A. Perirenal localized form of retroperitoneal fibrosis. J Belge Radiol 1984; 67:421-424.[Medline]
  31. Inaraja L, Franquet T, Caballero P, Encabo B, Humbert P. CT findings in circumscribed upper abdominal idiopathic retroperitoneal fibrosis. J Comput Assist Tomogr 1986; 10:1063-1064.[Medline]
  32. Warkentin JH. An unusual manifestation of idiopathic retroperitoneal fibrosis. Radiology 1969; 93:1313-1314.[Medline]
  33. Sterzer SK, Herr HW, Mintz I. Idiopathic retroperitonial fibrosis misinterpreted as lymphoma by computed tomography. J Urol 1979; 122:405-406.[Medline]
  34. Rapezzi D, Racchi O, Ferraris AM. Perirenal extramedullary hematopoiesis in agnogenic myeloid metaplasia: MR imaging findings. AJR Am J Roentgenol 1997; 168:1388-1389.[Medline]
  35. Degesys GE, Dunnick NR, Silverman PM, Cohan RH, Illescas FF, Castagno A. Retroperitoneal fibrosis: use of CT in distinguishing among possible causes. AJR Am J Roentgenol 1986; 146:57-60.[Abstract/Free Full Text]

Congratulations to the 20 individuals and one resident group that submitted the most likely diagnosis (multifocal fibrosclerosis with mediastinal-retroperitoneal involvement) for Diagnosis Please, Case 83. The names and locations of the individuals and of the resident group, as submitted, are as follows:
Individual responses
Albert J. Alter, Madison, Wis
Juan Ramón Ayuso, Barcelona, Spain
Richard Beedie, Auckland, New Zealand
Michael P. Buetow, MD, Okemos, Mich
Antonio Cavalcanti, MD, São Paulo, Brazil
Ann S. Fulcher, MD, Richmond, Va
Myeong-Jin Kim, MD, PhD, Seoul, Korea
Yu-Ting Kuo, MD, Kaohsiung, Taiwan
David A. Lisle, Brisbane, Australia
Manabu Minami, MD, Ibaraki, Japan
Eduardo Mondello, MD, Buenos Aires, Argentina
Mizuki Nishino, MD, Boston, Mass
Michael T. O’Loughlin, MD, West Hartford, Conn
Taro Shimono, MD, Osaka, Japan
Kouichi Sugiyama, Hamamatsu, Japan
Norio Takahashi, MD, Fukui, Japan
Hiroyuki Ueda, Kyoto, Japan
P. M. Vos, Vancouver, British Columbia, Canada
Satoru Yoshida, MD, Muroran City, Japan
Joe Yut, Olathe, Kan
Resident group response
Oregon Health and Science University Radiology Residents, Portland, Ore




This article has been cited by other articles:


Home page
 RadioGraphicsHome page
V. R. Surabhi, C. Menias, S. R. Prasad, A. H. Patel, A. Nagar, and N. C. Dalrymple
Neoplastic and Non-neoplastic Proliferative Disorders of the Perirenal Space: Cross-sectional Imaging Findings
RadioGraphics, July 1, 2008; 28(4): 1005 - 1017.
[Abstract] [Full Text] [PDF]

Home page
 ICVTSHome page
C. Bahler, Z. Hammoud, and C. Sundaram
Mediastinal fibrosis in a patient with idiopathic retroperitoneal fibrosis
Interactive CardioVascular and Thoracic Surgery, April 1, 2008; 7(2): 336 - 338.
[Abstract] [Full Text] [PDF]

Home page
 ThoraxHome page
V Cottin, P-Y Brillet, F Combarnous, F Duperron, H Nunes, and J-F Cordier
Syndrome of pleural and retrosternal "bridging" fibrosis and retroperitoneal fibrosis in patients with asbestos exposure
Thorax, February 1, 2008; 63(2): 177 - 179.
[Abstract] [Full Text] [PDF]

Home page
 Am. J. Roentgenol.Home page
Y. K. Lee, J. B. Seo, S. S. Kim, and T.-H. Lim
Thoracic Periaortic Fibrosis Mimicking Malignant Tumor: CT and 18F-FDG PET Findings
Am. J. Roentgenol., February 1, 2007; 188(2): 345 - 347.
[Full Text] [PDF]

Home page
 RadiologyHome page
A. V. Proto
Diagnosis please certificates of recognition awarded to tammam nehme, MD, and to international and north american radiology resident groups.
Radiology, November 1, 2006; 241(2): 331 - 333.
[Full Text] [PDF]

Home page
 RadiologyHome page
A. V. Proto
Diagnosis Please Certificate of Recognition Awarded to Taro Shimono, MD, PhD
Radiology, September 1, 2005; 236(3): 747 - 747.
[Full Text] [PDF]

This Article
Right arrow Figures Only
Right arrow Full Text (PDF)
Right arrow Alert me when this article is cited
Right arrow Alert me if a correction is posted
Right arrow Citation Map
Services
Right arrow Email this article to a friend
Right arrow Similar articles in this journal
Right arrow Similar articles in PubMed
Right arrow Alert me to new issues of the journal
Right arrow Download to citation manager
Right arrow reprints & permissions
Citing Articles
Right arrow Citing Articles via HighWire
Right arrow Citing Articles via Google Scholar
Google Scholar
Right arrow Articles by Szarf, G.
Right arrow Articles by Bluemke, D. A.
Right arrow Search for Related Content
PubMed
Right arrow PubMed Citation
Right arrow Articles by Szarf, G.
Right arrow Articles by Bluemke, D. A.

HOME HELP FEEDBACK SUBSCRIPTIONS ARCHIVE SEARCH TABLE OF CONTENTS
RADIOLOGY RADIOGRAPHICS RSNA JOURNALS ONLINE