A seven-year old girl presented to emergency room with 5 months history of intermittent fever, joint pain, severe anemia, and distended abdomen. She was treated outside by alternative medicine (‘naturopathy’) based on severe diet restrictions consisting of few pieces of bread and vegetables for several months. She was also misdiagnosed, hence treated wrongly as a case of congestive heart failure. Bilateral renal enlargement was mistaken for congested hepatosplenomegaly. No proper evaluation for abdominal distention was made at this time.
At admission to our hospital, physical examination revealed an emaciated child with severe anemia and bilateral palpable kidneys. Clinically they were symmetrical, non-cystic, and non-tender. Routine laboratory evaluation showed a blood urea nitrogen level of 16 mg/dl, serum creatinine 0.9 mg/dl, and uric acid was 7.8 mg/dl. Creatinine clearance was 52 ml per minute. Her hemoglobin was 3.2 g/dl, total leukocyte count 7900/mm3, and platelet count 2.53 × 104/mm3. Peripheral smear showed moderate anisocytosis with macrocytes, polychromatic cells, normocytes, and few microcytic normochromic cells suggesting hemolytic anemia. Reticulocyte count was 4.5 percent. Serum calcium was 10.2 mg/dl, phosphorous 3.1 mg/dl, and uric acid 9.8 mg/dl. Liver function tests were normal. The erythrocyte sedimentation rate was 38 mm/h and C-reactive protein was 1.4 mg/dl. Tuberculin skin test and serological assays for EB virus and virus were negative. Serum compliment and quantitative estimation of immuno-globulins were non-contributory. Urinalysis revealed trace protein but no active sediments. Repeated urine cytology revealed no malignant cells. Ultrasound of abdomen revealed marked bilateral renal enlargement with homogenous cortical echogenicity. The kidneys measured 17.9 × 6.8 × 7.1 and 16.6 × 6.8 × 7.1 cm. There was mild hepatomegaly without evidence of any focal lesion. Intrahepatic vascular and biliary radicles were normal. A plain computed tomography (CT) scan of abdomen confirmed bilateral diffused renal enlargement with decreased attenuation suggestive of diffuse infiltrative disease. There was mild hepatomegaly but no evidence of hydronephrosis or intra-abdominal lymphadenopathy . Chest X-ray showed normal lung parenchyma and a normal size heart without any mediastinal lymphadenopathy, which was confirmed by CT scan.
CT scan showing bilateral diffuse renal enlargement
Percutaneous renal biopsy showed extensive infiltration of interstitium by monomorphic neoplastic cells, some of which were round with round nuclei and scanty cytoplasm. Tumor cells were arranged in a tubular pattern at places. Tumor infiltration was found confined to interstitium without involving glomeruli or tubule [Figure and ]. CD45 showed surface positivity in all cells . Bone marrow biopsy was unremarkable, as were the head and chest CT scans. In view of angulation of all nucleoli, mucosa-associated lymphoid tissue (maltoma), a form of PRL was entertained.
(a,b) Needle biopsy of kidney low and high magnification showing tumor infiltration confined to interstitium. (c) Immunohistochemistry with CD45 marker study
She was put on combination chemotherapy consisting cyclophosphamide, intravenous vincristine 2 mg/M2and shifted to a medical oncology unit where she was started on combination chemotherapy with CHOP (cyclophosphamide, doxorubicin, vincristine, and prednisolone). She developed serious leucopenia (total leukocyte count of 900/cmm) on sixth postchemotherapy day, needing temporary suspension of therapy and institution of broad spectrum antibiotics.