Retroperitoneal fibrosis associated with cervical cancer
When a lack of classical findings can be misleading
Franco C, Shastry S, Thati N
Department of Internal Medicine, Wayne State University, Detroit Medical Center.
Detroit, Michigan, 48201
Retroperitoneal fibrosis was first described in 1905. It is a rare disease with an incidence of 1/200,000. Most cases are idiopathic, nonetheless some cases are associated to aortic aneurysms, malignancy, ureteric injury, drugs, radiation therapy and chemotherapy. It is suggested it could be an autoimmune reaction to a lipid called ceroid.
There is no racial preponderance. It is more commonly seen in males than in females.
The disease is most commonly diagnosed between the age of 40 and 60.
Radiologically it is described as a fibrous tissue involving the retroperitoneal organs.
Patients present with symptoms depending of the organ involved. (Blood vessels, Intestines, Biliary duct, Ureter, spine).
The prognosis depends of the underlying disease as well as progression of the fibrosis. Typically described as poor for malignancy associated retroperitoneal fibrosis and good for nonmalignant causes.
Treatment is difficult. It is centered in resolving the complications, stopping any offending drug and treating the underlying process. Steroids, azathioprine, mycophenolate mofetil, cyclophosphamide and tamoxifen were tried with different degree of success.
A 63 year old Caucasian female with history of hypertension, tobacco use and dyslipidemia presented to the hospital with a 7 day history of weakness, anuria and right sided flank pain.
PMH: Hypertension, dyslipidemia
Medications: Metoprolol 50 mg po bid, Nifedipine extended release 60 mg po qd, fluvastatin 40 mg po qd, Hydrochlorothiazide 25 mg po qd
Family History: Negative
Social History: 25 pack year smoking history, social alcohol drinker.
Review of systems: weakness, anuria, weight loss.
Physical Examination: VITALS: T 97.3, P 76, BP 124/80, R 18 BMI 18
GENERAL: Well nourished female, in no acute distress
HEENT: PERRL, no scleral pallor, oral mucosa moist, neck supple, thyroid non palpable. No lymphadenopathy.
CHEST: Lungs with bilateral symmetric air entry, no accessory muscle usage, clear to auscultation; Heart with no displacement of PMI, regular rhythm and rate, S1, S2 normal, no murmur.
ABDOMEN: Slightly distended, bowel
sounds present, positive for right flank tenderness. Bimanual pelvic examination elicited a pelvic mass involving the cervix and upper third of the vagina. Rectal examination demonstrated a mass in the anterior rectal wall.
EXTREMITIES-SKIN: Bilateral LE edema up to the knees, no cyanosis/clubbing. No purpura/petechiae.
NEUROLOGICAL:Alert/Oriented x 3, Cranial nerves
intact, no focal deficits, Strength and sensory intact
Labs: sodium 121, potassium 5.6, BUN 110, creatinine 17.7, hemoglobin 11.7, white count 9.1, platelet count 329. Urinalysis: RBC >100, Blood 3+, Protein 2+, no crystals, no casts.
Radiology: CT scan of the abdomen: extensive fat stranding/inflammatory changes involving the mesentery, pericolic gutter, peritoneal and retroperitoneal fascia with mild bilateral hydroureter and hydronephrosis. No evidence of lithiasis.
MRI: Irregular and nodular enhancement of the cervix and bladder base with soft tissue infiltration of the fat planes.
The patient was admitted with a diagnosis of Acute Renal Failure. IV Fluids were started. US of the kidneys showed no evidence of hydronephrosis. Microscopic analysis did not show casts in the urine.
CT scan of the abdomen was obtained.
Bilateral nephrostomy tubes were placed by Interventional Radiology. Good amount of urine output through the tube. Creatinine was trending down dramatically.
The patient was discharged home with a creatinine of 0.9
Post-discharge update: Pt underwent cervical biopsy. It showed differentiated adenosquamous carcinoma of the cervix.
Further work up showed mestastatic disease. Chemotherapy started with carboplatin and taxol.
Bilateral ureteral stents were placed by Urology, creatinine remains around 0.9
Unlike idiopathic RPF, a distinctly poor prognosis is associated with malignant RPF, which occurs in response to metastatic tumor cells in the retro peritoneum. The average survival is 3-6 months. No sex predominance is noted in malignant RPF. There are few cases described in the literature about the association of retroperitoneal fibrosis and cervical cancer.
Acute renal failure with anuria is seen in RPF cases and is usually due to obstruction with no evidence of hydronephrosis.
The clinician should be aware about these findings. A delay in the diagnosis and treatment (relieving of the obstruction) may lead to permanent renal damage and increased morbidity and mortality.
Treatment consists in nephrostomy tube or ureteral stent placement to relieve the obstruction.
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First Published May 2008
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