Ewing sarcoma is an aggressive high grade small cell tumour with mostly bony involvement. However, in very rare circumstances, they may have extra-skeletal manifestations as in PNET (Primitive Neuro-ectodermal tumour), thoracic, pulmonary involvement or simply extra-skeletal Ewing’s sarcoma.[1] Incidence of bone cancers in young adults is around (15-24yr old) 5.7%, whereas average age of patients with extra-osseous Ewing sarcoma is usually higher than those compared to bone cancer. [2],[3] Thereby, Ewing sarcoma with renal involvement that too in paediatric age group is extremely rare and is associated with an aggressive course with poor prognosis. In many instances, they may further present with a thrombus encroaching inferior vena cava or extending to right atrium at the time of presentation. Thus, strong clinical suspicion, timely diagnosis and removal of the tumour remain the main treatment for the disease. Till date as low as 48 cases have been reported and so knowledge regarding the anaesthetic management and the role of preoperative neoadjuvant chemotherapy for this rare disease, especially in paediatric age group remains limited.[4] Anticipatory major blood loss with fluid shifts leading to probable hemodynamic changes in the paediatric age group remains the major challenges in the anaesthetic management for this rare disease when they are scheduled for surgery.

Here we present the anaesthetic challenges and the benefit of neoadjuvant chemotherapy in a 2-year-old male child weighing 12 kg who had presented with left renal Ewing’s Sarcoma being posted for Left Nephrectomy under general anaesthesia. In this child history dates to six months back when her mother noticed a swelling in left flank of abdomen. An ultrasonic evaluation then revealed a large SOL with peripheral calcification in upper pole of left kidney measuring (3cm×3.5cm). Further evaluation with MRI and biopsy from the mass confirmed the diagnosis of left renal Ewing’s sarcoma. A PET scan was done where no distant metastasis or vena caval extension was noted. Thereafter, the child received 4 cycles of chemotherapy at the interval of every 1 month. Incidentally and most likely due to renal involvement the child developed hypertension which was managed conservatively with Tab. Amlodipine 2.5mg once daily dosage. At the time of diagnosis with the disease the child’s blood pressure was around 160/110 mmHg with complains of frequent headaches. Later, the blood pressure had optimised to 110/70 mmHg. The patient was treated with vincristine, doxorubicin and cyclophosphamide every three weekly alternated by etoposide and ifosfamide. Following completion of these four cycles of chemotherapy the child underwent a thorough pre-operative anaesthetic evaluation and was posted for the surgery. The child’s parents were then counselled regarding the chances of probable surgical and anaesthetic complications associated with the disease. Standard ASA monitors were attached and baseline readings were recorded. General anaesthesia was induced with Inj. Propofol 25mg, Inj. Fentanyl 25mcg as analgesic and Inj. Hydrocortisone 25mg. Muscle relaxation was achieved with Inj. Atracurium 6mg and after loss of all the four twitches in TOF (Train of Four) airway was secured with 4.5mm ID Endotracheal Tube, following which IPPV was continued. The child was then positioned into right lateral kidney position. Anaesthesia was maintained with sevoflurane, oxygen, air and intermittent bolus doses of Atracurium at 0.1mg/kg body weight. Intra-operative hemodynamic changes were closely monitored with SpO2, ECG, Urine output and NIBP. Additional monitors included temperature probe, ETCO2, neuromuscular monitoring and a pre-cordial stethoscope. The child had a peripherally inserted central cannula (PICC) preoperatively which was used for chemotherapy. This PICC line was kept in situ and 1 unit of PRBC was kept in reserve in view of anticipated perioperative blood loss. Goal directed fluid therapy was administered. Analgesia was further supplemented with IV Paracetamol 180mg and local skin infiltration with lignocaine 2 %. Inj. Tramadol 25 mg was used as rescue analgesic Though we were prepared for anticipated major blood loss, surgery went uneventful without any major hemodynamic instability. The child was reversed and extubated after surgery. Thereafter child was shifted to postoperative recovery unit with stable vitals. The patient had a pain score of <3 according to Wong Baker pain scale at any point of time post-operatively without any supplemental opioid. Intra-operative blood loss was less than 50 ml and hence no transfusion was required. The child was discharged on 4th post-operative day.

Extra-osseous Ewing’s sarcoma is rare and its overall incidence is 1 % of all sarcomas.[5] Among the various extra-osseous presentation, involvement of kidney or adrenal glands are extremely rare and they may present with thrombus extension to inferior vena cava or right atrium [6]. Total 48 cases of extra-osseous Ewing’s sarcoma have been reported so far, are mostly in young adults and to the best of our knowledge not many cases are reported in the paediatric age group.[4] Laparotomy with or without thoracotomy along with removal of tumour is the primary treatment. Such surgical procedure along with anticipated hemodynamic instability happens to be a major significant challenge for anaesthesia and this may be more pronounced in the paediatric age group. Massive bleeding and tumour thrombus could lead to an unusual burden on the heart which may produce acute coronary syndrome or even cardiac deaths. Besides, Ewing’s sarcoma can secrete a range of pro-inflammatory cytokines which may lead to peri-operative shock. [7] In this particular patient, we did not encounter the anticipated risks probably due to pre-operative chemotherapy. Extensive chemotherapy over a period of 4 month not only did shrink the size of the tumour but also kept it more confined, prevented any thrombus extension and probably diminished the vascularity of the tumour. PET Scan also confirmed it, as this benefit of chemotherapy was probably indicated by inactive residual calcified necrotic hypodense soft tissue lesion. Metastatic disease is very common in Ewing’s Sarcoma of kidney and nearly 40%have overt metastasis with the commonest sites being lung, bones, lymph nodes and liver. [8] In this patient however, there was no such indication of visceral metastasis. Chemotherapy to the paediatric age group may have lots of adverse effect as well. Chemotherapeutic agents like- vincristine, doxorubicin, cyclophosphamide all cause significant cardiotoxic effects which presents as congestive heart failure, haemorrhagic myocarditis, pericardial necrosis, IHD, dysrrythmia, nonspecific ST-T changes, QRS depression and QT prolongation. Cyclophosphamide also causes liver toxicity, pulmonitis with or without fibrosis and renal toxicity. [9] It not only reduces the immune status of an individual but also renders difficulty in securing an IV access. Though in this child a pre-operative PICC line was established, these adverse effects of chemotherapy may be detrimental in the paediatric age group. Despite all these problems in the absence of neoadjuvant chemotherapy major blood loss with fluid shifts leading to acute kidney injury, major cardiac events or DIC could have happened. Thus, neoadjuvant chemotherapy probably prevented the chances of developing these adverse events and hence these were avoided. Thereby, pre-operative chemotherapy not only has a surgical benefit but also it offers a great help to our domain as well.

To conclude Renal Ewing’s sarcoma in the paediatric age group is a rare and may lead to a challenging scenario in the absence of neoadjuvant chemotherapy. Though chemotherapy may be associated with various adverse effects, it may have a beneficial role in preventing the life-threatening complications that are associated with massive blood loss. This is of a great advantage where the hemodynamic reserve is compromised.

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