Case 1
A 52-year-old woman was admitted to our nephrology department for
management of renal failure. She was operated on for a renal tumor of
the left kidney a month ago. Histological examination showed
tubulo-papillary carcinoma (Fig1, 2). Several non necrotizing granulomas
were seen around the tumor. Granulomas formation did not contain
malignant cell. Specific stain for mycobacterial organisms (Ziel-Neelson
stains) was negative.
Otherwise, the patient had complained of unexplained cough one year ago.
Clinical examination revealed erythema nodosum. Laboratory tests showed
lymphopenia, high serum calcium level, normal serum parathyroid hormone
level, renal failure (creatinemia: 2.3 mg/dl), high CRP levels (65 mg/l)
and elevated serum angiotensin-converting levels). Chest computed
tomography (CT) did not suggested tuberculosis but it revealed
mediastinal lymph node enlargement. The broncho-alveolar lavage with
cell count revealed 20 % of lymphocytes with a ratio CD4/CD8 lymphocyte
ratio equal to 3.6. The diagnosis of active sarcoidosis with renal, lung
and skin involvement was made. After initiation of 1 mg/kg/day of
prednisone, clinical manifestations gradually disappeared. Laboratory
tests have shown a drop in serum calcium and CRP levels. Chronic renal
failure has remained stable.
Case 2- A 17-year-old female with a clinical history of
sarcoidosis with skin, lung, renal and neurological involvement was
referred to nephrology department for progressive renal failure and
uncontrolled hypertension under corticosteroid therapy. Clinical
examination showed hepatomegaly, erythema nodosum, multiple peripheral
lymphadenopathy and peripheral facial paralysis. Her blood pressure was
of 180/ 90 mmHg. Laboratory tests showed lymphopenia. Serum creatinine
level was 3.6 mg/dl and proteinuria was 1.2 g/24 hours. She had
pulmonary involvement with bilateral hilar lymphadenopathy on chest CT.
The bronchoalveolar lavage revealed a lymphocyte ratio CD4:CD8 equal to
3.7. Cerebral MRI showed hyperintensity of white matter consistent with
neurological involvement of sarcoidosis. Renal ultrasound revealed
kidney asymmetry with a smaller and hyper echogenic right kidney. A
CT-guided needle biopsy of the left kidney demonstrated several
non-necrotizing epithelioid granulomas (Fig 3). Despite
immunosuppressive therapy (Azathioprine at a dose of 100 mg a day and a
prednisone at a dose of one mg/kg/day) kidney function gradually
worsened. On the other hand, hypertension remained uncontrolled in spite
of antihypertensive therapy and salt free diet.
As part of etiological assessment of hypertension, retrograde
urethro-cystography was performed without abnormalities. Doppler
ultrasonography of the renal artery didn’t show any stenosis. DMSA renal
scintigraphy revealed impaired relative right renal function (14% in
right kidney vs 86% in left kidney). Aldosterone/direct Renin
concentration ratio was < 30, consisting with secondary
hyperaldosteronism. A total right nephrectomy was performed.
Histological examination of the nephrectomy specimen showed a multifocal
tubulocystic carcinoma without sarcoïd granuloma (Fig4). Despite
well-managed medical treatment, the patient progressed to end-stage
renal failure. At 5-years follow-up, the patient was on chronic dialysis
without recurrence of neoplasia.