Case 4

Diagnosis – “An unusual Primitive Neuroectodermal Tumour of the Kidney”

Mr. B.R, 28 years of age consulted me with history of pain in right lumbar region for last 4 – 5 weeks. This was associated with gross total painless intermittent hematuria without clots once in 2 – 3 weeks. He also complained of fever about 2 months back and his hematuria has been persisting for the last 2 days. He does not suffer from any other medical or surgical illness.

Physical Examination was essentially normal except for the large ballotable mass was felt in the right hypochondrium and right lumbar region.  This was non tender. Routine Biochemical Parameters were within normal limits.  His urine revealed significant microscopic hematuria with urinary tract infection. 

His ultrasonography revealed a hypoechoic mass in the lower pole of the right kidney.  CT scan of the abdomen and pelvis revealed a large mass lesion involving the right kidney with non visualization of the right ureter.  The lesion abuts the duodenum, head of pancreas, psoas muscles and ascending colon with stranding of the adjacent fat planes.  Neoplastic etiology is likely? RCC.  Centimeter sized retroperitoneal nodes are noted.  X-ray chest was normal.

Subsequently Mr. B.R was admitted to hospital.  He was subjected to Laparoscopic Right Radical Nephrectomy under general anesthesia.  Multiple ports were used and right colon was mobilized medially. Duodenum was kocherised.  The hilum was approached and the renal artery was dissected free.  It was cut between the ligatures.  Renal vein was stapled.  The ureter was mobilized and was clipped.  The gonadal veins were also clipped.  The specimen was freed and was removed through an extended transverse pfannensteil like incision and the kidney was removed and sent for histopathology examination.

The histopathology revealed: The Right Kidney – Malignant, Poorly Differentiated Round Cell Tumor – Primitive Neuroectodermal Tumor (PNET).  Tumor invades Ureter.  Tumor invades Adrenal (not separately identified from tumor) Peri renal fat invaded. Gerota’s Fascia not involved.  Renal Hilac vessels – not involved.

Mr. B.R. recovered from the procedure well and was discharged from the hospital.

Further, the histopathology slides were sent for another opinion which revealed Primitive Neuroectodermal Tumor of the Kidney.  The tumor cells express Mic2.  They are negative for CK & vimentin.

Post operatively he was also subjected to whole body PET CT scan which shows minimally active left para aortic nodes.  Uptake in the left adrenal ? significance.

Mr. B.R has further been referred to a medical Oncologist for further directions. In the meanwhile skin staples were removed and wound was healthy. Medical Oncologist has advised him to take chemotherapy for Primitive Neuroectodermal Tumor with ICE x 6 such.  He is pursuing the treatment with medical oncologist.

Mr. B.R. is now doing well and is fit to resume his normal activities.

Dilip Raja