The pathology of idiopathic retroperitoneal fibrosis

Mitchinson, M. J.

doi:10.1136/jcp.23.8.681

Cited by 194 publications

(96 citation statements)

References 34 publications

(28 reference statements)

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“…7 The histologic appearance of retroperitoneal fibrosis consists of variable proportions of fibroblastic proliferation and mononuclear inflammatory infiltrate, depending on the stage of the lesion. 8 The diagnosis of idiopathic retroperitoneal fibrosis is typically strongly suggested by radiographic findings. However, biopsy may be necessary when an atypical clinical presentation is encountered or when a neoplastic process is suspected.…”

mentioning

confidence: 99%

Detection of MDM2 gene amplification or protein expression distinguishes sclerosing mesenteritis and retroperitoneal fibrosis from inflammatory well-differentiated liposarcoma

et al. 2009

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Inflammatory liposarcoma is a variant of well-differentiated liposarcoma/atypical lipomatous tumor that consists of a mixture of lymphocytes, histiocytes, scattered atypical stromal cells, mature adipocytes, and rarely lipoblasts. When the inflammatory infiltrate predominates, the morphological features overlap with various fibroinflammatory disorders including sclerosing mesenteritis and retroperitoneal fibrosis, making the diagnosis difficult. Well-differentiated liposarcoma/atypical lipomatous tumor and dedifferentiated liposarcoma have characteristic molecular markers in the form of giant marker and ring chromosomes consisting of amplicons of 12q13-15, which includes MDM2. MDM2 immunohistochemistry (IHC) (Zymed; clone IF2) and dual color fluorescence in situ hybridization utilizing MDM2 (12q15) and chromosome 12 centromeric probes were performed on formalin-fixed and paraffin-embedded specimens from inflammatory well-differentiated liposarcoma (17 cases), sclerosing mesenteritis (14 cases), and idiopathic retroperitoneal fibrosis (10 cases). MDM2 expression as detected by IHC is a very sensitive tool in recognizing inflammatory well-differentiated liposarcoma (17 of 17); however, 21% (3 of 14) and 10% (1 of 10) of sclerosing mesenteritis and retroperitoneal fibrosis, respectively, displayed weak MDM2 immunoexpression. The MDM2 fluorescence in situ hybridization assay was very specific for inflammatory well-differentiated liposarcoma as 15 of 17 (88%) cases showed MDM2 amplification, whereas none of the cases of sclerosing mesenteritis or idiopathic retroperitoneal fibrosis showed amplification. Five cases of retroperitoneal fibrosis were noncontributory secondary to autofluorescence, potentially limiting the usefulness of the assay in certain situations such as inappropriate fixation. Increased MDM2 expression and/or MDM2 amplification can be employed to aid discrimination of inflammatory well-differentiated liposarcoma from fibroinflammatory mimics. MDM2 fluorescence in situ hybridization is a very specific method (100%), but less sensitive (88%), whereas MDM2 expression by IHC is very sensitive (100%), but less specific (83%). Therefore, a positive screen of difficult cases with MDM2 IHC would require confirmation by the fluorescence in situ hybridization. However, lack of MDM2 immunoexpression would rule out the possibility of inflammatory well-differentiated liposarcoma.

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confidence: 99%

Detection of MDM2 gene amplification or protein expression distinguishes sclerosing mesenteritis and retroperitoneal fibrosis from inflammatory well-differentiated liposarcoma

et al. 2009

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“…[6] Steroidler antienflamatuvar etkileri, fibrotik doku matürasyonunu inhibe etmeleri ve son yıllarda RPF'nin etyolojisinde rolü gösterilen immüniteyi baskılamaları nedeniyle RPF tedavisinde kullanılmaktadır. [11] Higgins ve arkadaş-ları [12] 18 yıl içinde steroid ile tedavi ettikleri idyopatik RPF'li 17 olgunun 13'ünde başarılı sonuç almış-lar, obstrüksiyonun yedi veya onuncu günde düzeldi-ğini bildirmişlerdir. Prednizolon 1 mg/kg/gün 6 hafta verilmesi önerilmektedir.…”

Section: Discussionunclassified

“…Prednizolon 1 mg/kg/gün 6 hafta verilmesi önerilmektedir. [11][12][13] Steroid tedavisinin yan etkileri doz ayarlaması yapılarak azaltılabilir. Biz de hastamıza öncelikle 60 mg/gün steroid tedavisi baş-ladık.…”

Section: Discussionunclassified

Bilateral Hydronephrosis Related To Retroperitoneal Fibrozis and Response To Steroid Therapy

Güllü¹,

Dağel²,

Doğan³

et al. 2011

Istanbul Tip Derg

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ÖZETRetroperitoneal fibrozis (RPF) steroid tedavisine sıklık-la yanıt veren kronik enflamatuvar hastalıktır. Bu yazıda, anüri nedeni ile başvuran, RPF'ye bağlı bilateral hidronefrozu tespit edilen ve steroide çok hızlı yanıt veren bir olgu sunuldu. Altmış beş yaşında erkek hasta, üç gündür idrar yapamama yakınması ile başvurdu. Laboratuvar testlerinde üre: 156 mg/dl, kreatinin: 15 mg/dl idi. Karın bilgisayarlı tomografisinde (BT) retroperitenal mesafede paraaortik alanlarda yaygın yumuşak doku yoğunluk artışı ve bilateral üreteral dilatasyon izlendi ve retroperitoneal fibrozis olarak yorumlandı. Hastaya duble J (DJ) kateter takıldı ve metilprednizolon 60 mg/gün başlandı. İşlem sonrası 7. gün üre 32 mg/dl, kreatinin 1.1 mg/dl ölçüldü. İki ay sonra BT kontrolünde RPF'nin gerilediği izlendi. Hastanın DJ kateteri çekildi ve steroid tedavisi azaltılarak, altıncı ayda kesildi. RPF, obstrüktif üropatinin etyolojik sebepleri arasında akılda tutulmalıdır.Anahtar sözcükler: Böbrek yetmezliği; hidronefroz; retroperitoneal fibrozis; metilprednizolon. SUMMARY Retroperitoneal fibrosis (RPF) is a chronic inflammatory dis-

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“…Depending on involvement of other vessels, claudication, and other manifestations of vascular impairment may accompany this disorder. Chronic periaortitis has been hypothesized to be a consequence autoantibodies reacting against antigens like oxidized low-density lipoproteins and ceroid in atherosclerotic plaque [6][7][8].…”

Section: Discussionmentioning

confidence: 99%

Isolated periaortitis in a 53-year-old male

Nambiar¹,

Kannoth²,

Dae³

2017

J Med Therap

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Chronic periaortitis includes a specific group of rare diseases which is characterized by the presence of abnormal fibroinflammatory tissue in the periaortic retroperitoneum. We describe a case of nonaneurysmal form of chronic periaortitis, recognized as isolated periaortitis, in a male patient who presented with low back pain. We report this case to highlight the fact that periaortitis can present as an isolated primary condition and can be reported without aortic aneurysm or retroperitoneal fibrosis. Case reportA 53-year-old male presented with low backache of 3 month's duration. The pain was of moderate intensity, unaffected by motion, and not relieved by bed-rest. He had lost approximately nine kilograms over the last 6 months. He did not give any history of fever. Physical examination revealed blood pressure of 200/110 mm Hg in upper limbs and 180/110 mm Hg in both lower limbs. The remainder of physical examination was essentially normal. Blood investigations revealed elevated erythrocyte sedimentation rate of 130 mm and elevated C-reactive protein. Renal and liver function tests were essentially normal. Autoimmune (ANA, ANCA) and infectious work-up (serological testing for syphilis) were negative. Chest and lumbar spine radiographs were normal. A computerized tomography (CT) scan of the lumbar spine was undertaken to exclude any inflammatory process which showed retroperitoneal soft tissue thickening. Hence, CT angiography of aorta (Figure 1) was done which revealed extensive abdominal aortic thickening without any abnormal dilatation. In addition, there was a retroperitoneal concentric periaortic soft tissue mass (rind of periaortic tissue) causing luminal narrowing of the abdominal aorta and significant ostial stenosis of the left renal artery. Infectious workup for vasculitis was also negative. There was no ureteric obstruction or hydronephrosis. This presentation was consistent with the diagnosis of isolated periaortitis confined to abdominal aorta. He was started on antihypertensives and steroids (prednisone 60 mg daily). On follow-up after 12 months, his blood pressure was controlled (130/80 mm Hg). Repeated CT scans showed progressive resolution of the periaortic rind with no evidence of abdominal aorta aneurysm.

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The pathology of idiopathic retroperitoneal fibrosis

Cited by 194 publications

References 34 publications

Detection of MDM2 gene amplification or protein expression distinguishes sclerosing mesenteritis and retroperitoneal fibrosis from inflammatory well-differentiated liposarcoma

Detection of MDM2 gene amplification or protein expression distinguishes sclerosing mesenteritis and retroperitoneal fibrosis from inflammatory well-differentiated liposarcoma

Bilateral Hydronephrosis Related To Retroperitoneal Fibrozis and Response To Steroid Therapy

Isolated periaortitis in a 53-year-old male

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