An eighteen-year-old male presented with a three-month history of progressive dull constant left flank pain/fullness, intermittent total hematuria, nausea/vomiting, occasional low-grade fevers, and anorexia. Furthermore, he noticed progressive weight loss over the two weeks before the presentation. In the period leading up to his presentation, this patient had been erroneously diagnosed with black water fever, a complication of malaria, and was treated with IV Artesunate 120 mg q12hrs for three doses. He was also treated erroneously for pyelonephritis with IV Ceftriaxone 2g q24hrs for five days when his symptoms persisted. Despite these treatments, the patient's condition deteriorated, with worsening hematuria, fever, left flank pain, and weight loss over the last two weeks to his presentation prompting his consultation at a referral hospital. He had an uneventful birth, medical, social, and family history, and had never undergone a major surgical intervention before.
Clinical examination revealed a mildly ill and afebrile patient who was neither pale nor jaundiced and also had no lymphadenopathy. He had high blood pressure (right arm BP 152/112 mmHg, left arm BP 148/112 mmHg); a tender palpable firm and relatively fixed left flank mass that extended 4cm below left subcostal margin; and non-tender worm-like swellings within the left scrotum.
A diagnosis of the left renal tumor was made. The differential diagnoses included renal cell carcinoma, Wilm's tumor, metanephric adenoma, renal polycystic disease, renal tuberculosis, hydronephrosis, tropical splenomegaly syndrome, and pyelonephritis.
Based on the left flank pain, nausea/vomiting, anorexia and high blood pressure, the patient received PO morphine 10mg q6hrs PRN, PO amlodipine 5mg q24hrs, IV ondansetron 4mg q12hrs PRN and PO ferrous sulfate 100mg q12hrs. These significantly improved his symptoms and normalized his blood pressure.
Abdominal ultrasound revealed an enlarged left kidney with irregular margins and multiple ill-defined mixed echogenic masses. An abdominal/chest CT scan conducted later revealed a predominantly hypodense heterogeneous solid intra-renal mass (claw sign, size 16.05 x 10.46 cm) with patchy contrast-enhancement distorting the renal parenchyma and collecting system, breeching the renal capsule but not invading the surrounding tissue (transparent fat plane between mass and surrounding organs, e.g., spleen). There was also an inferior vena cava thrombus, a mass effect that displaced adjacent structures. Still, there was neither encasement/elevation of the aorta and renal artery nor a tumor crossing the midline. There were para-aortic lymph nodes and bilateral sub-pleural lung base nodules. However, the CT appearances of the right kidney and ureter, liver, gall bladder, pancreas, spleen, and bones were normal (Fig. 1). A chest x-ray showed bilateral nodular opacities in the lower lung zones and a nodular opacity in the right upper zone (Fig. 2).
Figure 1. NECT & CECT of the Abdomen and Lower Thorax.
A - Non-enhanced CT: Large left heterogeneous solid renal mass (16.05 x 10.46 cm) (blue arrow). B - A contrast-enhanced image shows a sizeable mixed-density mass of 15x12x9 cm involving the entire left kidney with patchy enhancement (orange arrow). C - IVC thrombus (blue arrow); claw sign (green arrow); para-aortic lymph node (orange arrow). D - Bilateral lung base nodules adjacent to pleura (sub-pleural) (blue arrow).
Figure 2. PA Chest X-ray
Bilateral nodular opacities in the lower lung zones. Right upper zone nodular opacity (green arrow)
Laboratory investigations were done for completion and ruled out liver metastasis (mild rise in transaminases, SGPT 64, SGOT 79), bone metastasis (normal alkaline phosphatase), TB nephritis (negative urine LAM), pyelonephritis (normal leucocyte count, white cell count 5.92), malaria-associated splenomegaly (negative blood smear for malaria) and normal kidney function test (Urea 10 mg/dl, Creatinine 0.9 mg/dl).
Worthy of note is that the patient could not afford Contrast-enhanced MRI, which is the most accurate imaging modality for detecting local and vascular extension, nephroblastomatosis at initial diagnosis, or contralateral affectation.
A presumptive diagnosis of renal cell carcinoma with metastasis to the lungs was made and the prognosis at this point was poor. The differential diagnosis was an adult-onset Wilms tumor.
The patient was managed surgically by conducting a left radical nephrectomy and was expected to continue with palliative adjuvant chemotherapy and localized radiotherapy to the lungs and left flank area. Intraoperatively, a left subcostal incision was made and we found a left renal tumor with Gerota fascia fixed to the parietal peritoneum indicating tumor infiltration. The left kidney containing the tumor and the suprarenal gland encapsulated by the Gerota fascia were separated off the lateral edge of the psoas major muscle and this revealed testicular vessels, renal vessels, and infiltrated upper half of the ureters. The upper half of the ureter, testicular vessels, and renal vessels were ligated and divided and the inferior vena cava tumor thrombose was carefully removed in its entirety. The kidney was mobilized off its attachment to the lower edge of the pancreas, and removed along with most of the Gerota fascia and the perirenal fat (Fig. 3). Hemostasis was achieved, the left retroperitoneal cavity was washed with normal saline, and the left flank abdominal wall was closed in layers.
Figure 3. Tumor-involved left kidney and suprarenal gland after removal.
Postoperatively, the patient was admitted to the intensive care unit for advanced respiratory and cardiovascular support after sustaining a hypertensive emergency (BP 166/105 mmHg) with flash pulmonary edema. The patient was discharged from intensive care on postoperative day three fully conscious and alert with normal blood pressure (BP 113/72 mmHg).
The patient continued to improve in the ward as left incisional flank pain progressively reduced, hematuria resolved and the patient’s blood pressure normalized (BP 119/87 mmHg). The patient reported better appetite, no longer feeling nausea/vomiting, and generally felt better. The rest of the postoperative follow-up in the ward was uneventful.
The histopathological examination of the specimen revealed the following findings:
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Morphology: well encapsulated brown tissue 15x9x7cm; cut surface heterogeneous grey-brown solid and firm mass measuring 15x7x5cm—rim of tissue with extensive necrosis and areas of hemorrhages.
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Microscopy: H&E sections comprise round blue tumor cells (blastema), intact capsule, and no lymphovascular invasion.
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The conclusion was a blastemal-type Wilms tumor. It was staged as Wilms Tumor Society stage IV (metastatic disease) with lung and para-aortic lymph node metastasis.
The patient reported feeling much better with little incisional pain and was discharged on postoperative day 9. He was expected to report to the cancer treatment center for adjuvant palliative chemotherapy and localized radiotherapy to the lungs and left flank area. The patient, unfortunately, died six weeks after surgery as he was lost to follow-up because he could not afford adjuvant chemotherapy or radiotherapy.