Advanced testicular cancer presenting with phlegmasia cerulea dolens

Abstract

The results of treating germ cell cancer continue to improve. A recent review at the Men's Cancer Unit at the Royal Hospitals Trust showed 97% overall survival in a cohort treated from 1989 to 1994.¹ There was a lack of major impact of delay on survival in this cohort. In patients delaying initial presentation by less than three months survival was 98% and in those who delayed for greater than 12 months 94% still survived. This leads some to question the current practice requiring all testicular masses to be considered as an emergency. As two thirds present with delay of more than two months there is disagreement overthe necessity for surgery on the next operating list. It is obvious in the minority of patients with choriocarcinoma, a condition that can be screened for on the basis of a positive urinary pregnancy test,² that delay should be avoided as it can double every six days and kill in six weeks. However, experiencing a patient who died within a week of first presentation with venous infarction induced gangrene and rhabdomyolysis induced renal failure is a reminder of the fatal problems that can develop from delay in less malignant cases.

Case report

A 45 year old man presented to the casualty department describing a two week history of painless left testicular swelling and a two day history of leg muscle cramp, weakness, stiffness, and oedema. He had attended his general practitioner the previous day but he had not performed a general or testicular examination. The patient had no past history of hypertension, renal disease, scrotal surgery, or testicular trauma. He lived in a separate unit within a house with his uncle. Three years previously he had lost both parents and shortly afterwards he was made redundant from work as a security guard. He had become moderately reclusive but did not drink alcohol.

On examination he was drowsy but rousable. He was apyrexial and had no palpable lymphadenopathy. There was a large indistinct central mass in his abdomen and a 10 cm² firm, non-tender scrotal swelling. Both legs were grossly oedematous with peripheral discoloration, reduced capillary refill, and impalpable peripheral pulses.

A urinary pregnancy test was positive. Ultrasound examination revealed a right hydronephrotic kidney, and bilateral femoral vein thromboses secondary to the large abdominal mass. After resuscitation the patient was transferred for urgent right nephrostomy as his serum urea concentration measured 15.4 mmol/l with a creatinine of 419 mol/l. Overnight he became oliguric and his temperature rose to 38°C.

The next morning blood investigations showed a rising urea concentration of 21.3 mmol/l and creatinine 541 mol/l, with urate 902 mmol/l, lactate dehydrogenase 1642 IU/l, creatine kinase 53 IU/l, and β human chorionic gonadotrophin 530 U/l. Doppler pulses were absent in the lower limbs and a diagnosis of phlegmasia cerulea dolens was made: a fulminating form of deep vein thrombosis.³ Computed tomography revealed no abnormalities within the chest, liver, or spleen. A small left kidney was noted. The right kidney was atrophic and compatible with obstruction. There was a large heterogeneous mass measuring 10 cm² overlying the bifurcation of the inferior vena cava extending into the right pelvis, infiltrating the left iliac vein and inferior vena cava (fig 1). The venous thrombosis was secondary to venous compression and invasion.

Figure 1

Contrast enhanced computed tomography immediately caudal to the aortic bifurcation.

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The patient became hypotensive and anuric despite insertion of a right nephrostomy tube. His renal failure was initially treated medically with dopamine. The sepsis was treated with intravenous antibiotics. An intravenous heparin infusion was started in view of the venous thrombosis. He received high dose dexamethasone to reduce peritumoral inflammation and allopurinol to counteract potential tumour lysis syndrome. Emergency chemotherapy was given in an attempt to shrink the tumour mass and relieve pressure on the inferior vena cava. Because of the poor renal function he received a small dose of carboplatin (100 mg), etoposide (250 mg), and subcutaneous bleomycin (15 units).

His condition deteriorated progressively and haemodyalysis started on the third day. Despite these measures ischaemia and gangrene progressed rendering surgical salvage impossible.⁴ Further dialysis was withheld and he died six days after his initial presentation.

Discussion

This patient's reclusive personality undoubtedly resulted in delayed presentation. He consulted his general practitioner on the day before presenting to hospital, and had he been diagnosed his life might have been saved as renal failure due to rhabdomyolysis is reversible if the cause is resolved.

Vena caval invasion is a well recognised but relatively rare complication of testicular tumours. Vena caval invasion more frequently occurs with right sided tumours, as the sentinel lymph node on this side is usually the intercaval node at the level of the right renal artery.⁵ Such patients may present with pulmonary emboli, although thrombus in the vena cava can extend and cause the Budd-Chiari syndrome with hepatorenal failure. The iliac node involvement in this patient is more commonly seen after scrotal surgery. Literature review failed to identify a report of phlegmasia cerulea dolens in testis cancer patients. In our records of more than 300 cases with metastatic testis cancer there are six cases with vena caval invasion and one with Budd-Chiari syndrome. Despite hepatorenal failure that patient responded to low dose chemotherapy similar to that given in this case and proceeded to conventional treatment two weeks after resolution of the thrombus.² He is alive at 10 year follow up.

This case illustrates that there is still a need for public education about testis cancer as late presentation may reduce survival. A critical lesson for all health care professionals is to be vigilant for testicular cancer in men with non-specific symptoms.

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