Retroperitoneal fibrosis

1. RETROPERITONEAL FIBROSIS
Dept of Urology
Govt Royapettah Hospital and Kilpauk Medical College
Chennai

2. Moderators:
Professors:
 Prof. Dr. G. Sivasankar, M.S., M.Ch.,
 Prof. Dr. A. Senthilvel, M.S., M.Ch.,
Asst Professors:
 Dr. J. Sivabalan, M.S., M.Ch.,
 Dr. R. Bhargavi, M.S., M.Ch.,
 Dr. S. Raju, M.S., M.Ch.,
 Dr. K. Muthurathinam, M.S., M.Ch.,
 Dr. D. Tamilselvan, M.S., M.Ch.,
 Dr. K. Senthilkumar, M.S., M.Ch.
Dept of Urology, GRH and KMC, Chennai.
2

3. Introduction:
 First reported -French Urologist Albarran(1905)
 SYNONYMS:
❖ Ormond's Disease( Idiopathic )

❖ Periureteritis Fibrosa
❖ Periureteritis Plastica

❖ Chronic Periureteritis,
❖ Sclerosing Retroperitoneal Granuloma,
❖ Fibrous Retroperitonitis
3
Dept of Urology, GRH and KMC, Chennai.

4.  Characterized by the development of extensive fibrosis
throughout the retroperitoneum.
 Grossly-- fibrous, whitish plaque rembling woody mass
centers around the distal aorta at l4 to s1
 Encases the aorta, inferior vena cava, and their major
branches, and also the ureters, other retroperitoneal
structures, and, at times, intraperitoneal structures
including the gastrointestinal tract.
4
Dept of Urology, GRH and KMC, Chennai.

5.  Most prominent -- Fourth lumbar vertebra to the first sacral
vertebra
 Medial deviation of the middle third of the ureters (50 -
75%)
 Hydronephrosis and can cause varying degrees of renal
failure
 Great vessel involvement
 Common ; Do not often leading to vascular obstruction.
 Arterial obstruction - Rarely significant
 Venous obstruction - Lower extremity edema
5
Dept of Urology, GRH and KMC, Chennai.

6. Retroperitoneal fibrosis:
 Incidence: 1 : 200,000 population.
 Race: no difference between ethnic groups.
 Sex: male : female = 2 to 3:1.
 Age: the peak incidence is between 40-60 years.
6
Dept of Urology, GRH and KMC, Chennai.

7. Types:
 Idiopathic: with severe atherosclerosis of the
aorta. – 70%
 Secondary:
Prolonged use of drugs: methysergide, beta-
adrenergic blockers, methyldopa, amphetamines, phenacetin
and cocaine.
Autoimmune disorders: RA, SLE, PBC, autoimmune
thyroiditis, panhypopituitarism.
Malignancy: lymphoma, multiple myeloma, carcinoid,
pancreatic cancer, sarcoma and carcinomas of the stomach,
colon, bladder, prostate, and cervix.
Abdominal surgery, irradiation.
Infectiouscauses:Tuberculosis,Actinomyces,gonorrhe
a,or schistosomiasis
7
Dept of Urology, GRH and KMC, Chennai.

8. Malignant retroperitoneal fibrosis:
 7.9% incidence
 Three types of malignant processes
❖ periureteral metastasis with fibrosis
❖ primary retroperitoneal tumor
❖ serotonin production by carcinoid tumor.
 Biopsy at the time of surgical exploration to differentiate
idiopathic from malignant retroperitoneal fibrosis
 Malignant retroperitoneal fibrosis is associated with poor
prognosis, and most patients have an average survival of
approximately 3-6 months.
8
Dept of Urology, GRH and KMC, Chennai.

9.  Periureteral Fibrosis - 1% Incidence
 Clinically apparent several years after radiation therapy.
 Pathology:
▪ Fibrous tissue has a characteristic appearance, exhibiting large
collagen fibers and extensive hyalinization.
▪ Blood vessels show evidence of endarteritis obliterans
9
Dept of Urology, GRH and KMC, Chennai.

10. Pathogenesis: Unknown
 Development of vasculitis in the adventitial vessels
of the aorta and perioaortic small vessels.
 Release of antigens from atheromatous plaque
such as a complex lipoprotein
 Autoimmune antigenic response.
 Induce local inflammation resulting in varying degrees
of fibrosis
10
Dept of Urology, GRH and KMC, Chennai.

11. Genetic factors:
Association with other connective tissue diseases.
Reported familial occurrence.
 As part of multifocal fibrosclerosis, a rare syndrome
characterized by fibrosis involving multiple organ
systems
presentation may include RPF, sclerosing
mediastinitis, sclerosing cholangitis, orbital
pseudotumor, and Riedel's thyroiditis ( Dehner and
Coffin, 1998 ; Özgen and Cila, 2000 ).
11
Dept of Urology, GRH and KMC, Chennai.

12. Pathology:
 RPF appears as an exuberant mass of smooth, flat, tan-colored
white, woody, fibrous tissue covering the retroperitoneal
structures such as the aorta, vena cava, ureters, and psoas
muscle.
 The fibrous tissue may bifurcates and follows the common iliac
arteries.
 Rarely, the fibrous process extends into the root of the
mesentery or passes through the crura of the diaphragm to
continue as fibrous mediastinitis.
12
Dept of Urology, GRH and KMC, Chennai.

13.  Fibrous component – Myofibroblasts and type-1 collagen
deposition
 A nonspecific inflammatory reaction is often present. The
cellular infiltrate includes PNL, lymphocytes, eosinophils or
plasma cells.
 The infiltrate is both perivascular and diffuse.
 In the chronic phase, the only finding may be an acellular
fibrosis, consisting of sheets of hypocellular collagen.
13
Dept of Urology, GRH and KMC, Chennai.

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Dept of Urology, GRH and KMC, Chennai.

15. Clinical presentations:
 Most patients present with nonspecific symptoms of less than
12 months duration.
 In the early stage, signs and symptoms originate from the
disease process (inflammatory process).
 In the advanced stage, clinical features represent the effects of
entrapment of the retroperitoneal structures by fibrosis.
15
Dept of Urology, GRH and KMC, Chennai.

16. Early presentations:
Pain (92%): most common presentation, dull aching,
poorly localized, noncolicky pain in the flank, back, scrotum or
lower abdomen relieved with asprin.
Other presentations: fever, weight loss, nausea,
vomiting, anorexia and malaise.
Urinary symptoms: nocturia, oliguria, urinary frequency
and hematuria.
Hypertension: a common clinical feature- renal vein
encasement.
16
Dept of Urology, GRH and KMC, Chennai.

17. Late presentations:
Urinary system: obstructive uropathy and renal impairment.
Aorta and branches: claudications, arterial insufficiency.
IVC: bilateral lower limbs oedema, thrombophlebitis, DVT.
Lymphatic: hydrocele, ascites.
Pancreatic duct: obstructive jaundice.
Colon: constipation, intestinal obstruction.
17
Dept of Urology, GRH and KMC, Chennai.

18. Investigations:
•ESR and CRP: elevated.
•Normocytic normochromic anemia.
•Raised urea and creatinine levels (50-75%).
•Polyclonal hypergammaglobulinemia
•Alkaline phosphatase: elevated.
•Urinalysis:
– Usually normal.
– Rarely, microscopic hematuria or pyuria.
18
Dept of Urology, GRH and KMC, Chennai.

19. Abdominal ultrasonography:
 RPF may appear as a well-defined, smooth-marginated,
hypoechoic, retroperitoneal soft-tissue mass encasing the aorta
and its branches and inferior vena cava.
 Ultrasonography may demonstrate dilatation of the
pelvocalyceal system and the ureters.
 Ultrasonography is not a sensitive examination, as compared
with CT and MRI. It is used to diagnose and to perform follow-
up studies.
19
Dept of Urology, GRH and KMC, Chennai.

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Dept of Urology, GRH and KMC, Chennai.

21.  IVP - hydronephrosis with medial deviation of
the proximal ureter and midureter
 a smoothly tapered ureter at the level of
obstruction.
 Urinary obstruction is usually bilateral, but
unilateral cases have been described
21
Dept of Urology, GRH and KMC, Chennai.

22. CT abdomen: The best diagnostic modality
RPF may appear as soft tissue encasing the
aorta and IVC extending between the renal
hilum and sacral promontory. Laterally,
spreading to involve the ureters, causing
varying degrees of obstruction and usually
medial displacement of the ureters.
The fat plane between the mass and the psoas
muscle may be obliterated and even invasion of
the muscle.
Certain CT features can help in differentiating
benign masses from malignant masses: e.g.
lymphadenopathy.
22
Dept of Urology, GRH and KMC, Chennai.

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Dept of Urology, GRH and KMC, Chennai.

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Dept of Urology, GRH and KMC, Chennai.

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Dept of Urology, GRH and KMC, Chennai.

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Dept of Urology, GRH and KMC, Chennai.

27. Other investigations:
MRI of the abdomen.
 Retrograde pyelo-ureterography.
Biopsy can be performed under CT guidance to
differentiate benign masses from malignant
retroperitoneal masses.
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Dept of Urology, GRH and KMC, Chennai.

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Dept of Urology, GRH and KMC, Chennai.

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Dept of Urology, GRH and KMC, Chennai.

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Dept of Urology, GRH and KMC, Chennai.

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Dept of Urology, GRH and KMC, Chennai.

32. Management:
The aims of management are:
Preserve renal function.
Prevent other organ involvement.
Treatment of the cause in 2ry RPF.
Relieve symptoms.
The treatment of retroperitoneal fibrosis depends
on the stage of the disease at diagnosis.
Treatment options includes medical and surgical
intervention for uretric obstruction.
32
Dept of Urology, GRH and KMC, Chennai.

33. Initial Management
 Patients with hydronephrosis and uremia -
emergently decompressed by either
percutaneous nephrostomy or indwelling
ureteral stents.
33
Dept of Urology, GRH and KMC, Chennai.

34. Medical treatment:
Empirical therapy includes corticosteroids, tamoxifen and
azathioprine.
 experimental therapy includes cyclophosphamide,
mycophenolate-mofetil, cyclosporin, medroxyprogesterone
acetate and progesterone.
Glucocorticoids and azathioprine are most useful in patients
with signs of inflammation.
34
Dept of Urology, GRH and KMC, Chennai.

35.  prednisolone - oral dose of 60 mg on alternate
days for 2 months, tapered to 5 mg daily over
the next 2 months
 continued for a total duration of 2 years
35
Dept of Urology, GRH and KMC, Chennai.

36. 36
Dept of Urology, GRH and KMC, Chennai.

37. SERM
 Tamoxifen, a nonsteroidal antiestrogen
 Alter TGF-β, potentially limiting fibrosis
 dose was 20 mg/day, and duration of therapy
varied
37
Dept of Urology, GRH and KMC, Chennai.

38. Surgical
 undertaken if medical therapy fails or if
the patient is not a candidate for medical
therapy
Bilateral ureterolysis -recommended, even
in the setting of unilateral disease.
 A biopsy of the fibrotic area should be
repeated
ureteral stents can generally be removed 6
to 8 weeks after
38
Dept of Urology, GRH and KMC, Chennai.

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Dept of Urology, GRH and KMC, Chennai.

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Dept of Urology, GRH and KMC, Chennai.

41.  Laparoscpic approach
41
Dept of Urology, GRH and KMC, Chennai.

42.  The most common primary medical
management of idiopathic RPF has been
corticosteroid therapy.
 ▪ In surgical bilateral ureterolysis, the
ureters need to be protected by
intraperitonealization or omental wrapping.
 Both open and laparoscopic techniques
may be applied successfully. If ureterolysis is
impossible to perform, renal
autotransplantation may be performed.
42
Dept of Urology, GRH and KMC, Chennai.

43. Pelvic Lipomatosis
43
Dept of Urology, GRH and KMC, Chennai.

44.  Rare, benign condition marked by exuberant pelvic
overgrowth of nonmalignant but infiltrative adipose
tissue.
 Engels- first described the condition in 1959
44
Dept of Urology, GRH and KMC, Chennai.

45.  Mean age at presentation was 48 years.
 Racial and gender differences were noted, with 67%
of the patients black and 33% white
 18:1 male-to-female ratio ( Heyns, 1991 ).
45
Dept of Urology, GRH and KMC, Chennai.

46. Etiology of this disorder is unknown.
 Obesity has been proposed to play
a role, as radiographic improvement
and worsening have been noted in
response to weight loss and gain (
Sacks et al, 1975 ).
 In addition, obesity has been noted
in over one half of the patients
afflicted ( Heyns, 1991 ).
46
Dept of Urology, GRH and KMC, Chennai.

47. Genetic etiology
occurrence of pelvic lipomatosis in
two brothers ( Tong et al, 2002
 An abnormality in the chromatin-
regulating high mobility group A
(HMGA) proteins- possible causative
factor in pelvic lipomatosis
 transgenic mice with truncated
HMGA developed pelvic
lipomatosis
47
Dept of Urology, GRH and KMC, Chennai.

48. Clinical presentation
 Lower urinary tract symptoms- 50%
 Bowel symptoms- 25% typically constipation.
 Suprapubic, back, flank, or perineal discomfort
can also be an initial clinical manifestation.
48
Dept of Urology, GRH and KMC, Chennai.

49. Pysical findings may include a
suprapubic mass, a high-riding
prostate, and an indistinct pelvic
mass.
Hypertension has been reported in as
many as one third of patients ( Klein
et al 1988 ; Heyns, 1991 ).
Assosiation with Ca bladder and
cystitis cystica
49
Dept of Urology, GRH and KMC, Chennai.

50.  On plain film, increased pelvic lucency may be noted
 On excretory urography, bladder characteristically
assumes a pear or gourd shape, extrinsically
compressed and elongated, and the bladder base is
frequently elevated .
50
Dept of Urology, GRH and KMC, Chennai.

51. 51
Dept of Urology, GRH and KMC, Chennai.

52. 52
Dept of Urology, GRH and KMC, Chennai.

53.  CT - quite helpful in establishing the
diagnosis as it readily demonstrates
pelvic fat.
 Extrinsic compression of the rectum may
also be demonstrated ( Susmano and
Dolin, 1979 ).
 Liposarcoma should be suspected if
there is tissue heterogeneity, areas of
positive attenuation coefficients,
enhancement with contrast, and poor
margination ( Andac et al, 2003 ).
53
Dept of Urology, GRH and KMC, Chennai.

54. 54
Dept of Urology, GRH and KMC, Chennai.

55.  MRI can also be used to make the diagnosis as it
permits characterization of fat deposits ( Demas et al,
1988 ).
55
Dept of Urology, GRH and KMC, Chennai.

56. Evaluation should include cystoscopy
- proliferative cystitis in 75% of patients
including cystitis glandularis in up to
40% ( Heyns, 1991 ).
 Continued cystoscopic surveillance is
recommended in those with cystitis
glandularis as there have been
reports of the development of
adenocarcinoma of the bladder in
this cohort ( Sozen et al, 2004 ).
56
Dept of Urology, GRH and KMC, Chennai.

57. Elongation of the prostatic urethra,
elevation of the bladder neck, and
pelvic fixation may impair
cystoscopic access to the bladder. In
Heyns' review (1991) evaluation of
the bladder was difficult in 24% and
impossible in 18%.
Hence, flexible cystoscopy may be
required if anatomic distortion
precludes rigid cystoscopy.
57
Dept of Urology, GRH and KMC, Chennai.

58.  Klein and associates (1988)- two clinically
separate groups of patients
 young, stocky men with irritative lower
urinary tract symptoms, vague pelvic
complaints, hypertension, and
proliferative cystitis.
 older men with incidentally discovered
pelvic lipomatosis - a more indolent
course.
58
Dept of Urology, GRH and KMC, Chennai.

59. Management
 Medical – not so effective
 Corticosteroids, immunomodulators statins –
attempted
 Radiotherapy ?
59
Dept of Urology, GRH and KMC, Chennai.

60. Surgical options
Obstructive uropathy - ureteral
stenting, percutaneous
nephrostomy, ureteral
reimplantation, and urinary
diversion.
Pelvic exploration should be
approached cautiously, as there is
obliteration of normal anatomic
planes and increased vascularity
within the fatty mass
60
Dept of Urology, GRH and KMC, Chennai.

61. THANK YOU