Case report of a laparoscopic biopsy found to trigger spontaneous tumor lysis syndrome in pediatric Burkitt lymphoma

Introduction

Burkitt lymphoma (BL) was first described in 1958 by Denis Burkitt in Uganda as a childhood tumor frequently involving the jaws and multiple anatomical sites (1). BL is an extremely aggressive non-Hodgkin lymphoma characterized by a short doubling time and rapid tumor proliferation, which frequently involves abdominal organs. While intensive chemotherapy has achieved excellent outcomes with 5-year event-free survival rates of 80–90% in children (2), the disease’s high tumor burden and chemosensitivity also predispose patients to a life-threatening complication: tumor lysis syndrome (TLS). TLS is caused by the massive release of intracellular contents from lysed tumor cells and results in severe metabolic disturbances, potentially leading to renal failure, cardiac arrhythmias, and multi-organ dysfunction (3).

Notably, TLS occurring in the immediate postoperative period is exceptionally rare, with only a few cases documented worldwide. This rarity, coupled with potential diagnostic challenges, highlights a significant lack of clinical awareness. Herein, we present a detailed case of TLS developing within 24 hours following laparoscopic surgery in a pediatric BL patient. Through this report and a concise review of the literature, we aim to raise awareness of this unusual clinical scenario and inform prevention and management strategies. We present this article in accordance with the CARE reporting checklist (available at https://acr.amegroups.com/article/view/10.21037/acr-2025-217/rc).

Case presentation

A 5-year-old girl presented with abdominal distension accompanied by paroxysmal pain for 2 weeks. Prior to admission, she was evaluated at a community clinic and underwent an ultrasound scan that identified multiple abdominal masses. Additionally, she had respiratory symptoms, including cough with sputum production, as well as nasal obstruction and rhinorrhea, all of which had developed over the preceding week. The patient had no significant past medical history and denied fever, headache, nausea, vomiting, diarrhea, or constipation. Upon admission, the patient’s overall condition was satisfactory, and all vital signs fell within normal ranges. Abdominal palpation revealed the presence of a mass in the left upper abdomen region. The abdomen was diffusely distended but soft, with no tenderness or rebound tenderness noted. Hyperactive bowel sounds were noted.

Thoracoabdominal computed tomography (CT) revealed neoplastic lesions in the retroperitoneal space, the liver, and the portocaval area. Diffuse thickening of the pleura, peritoneum, and intestinal walls was also observed, accompanied by pleural effusions and ascites (Figure 1). Her laboratory evaluation showed an elevated white blood cell (WBC) count, elevated lactic acid, and hypokalemia (Table 1). Other significant laboratory data included carbohydrate antigen 125 of 545.4 U/mL, uric acid of 646 µmol/L, and lactate dehydrogenase of 1,689 U/L. A bone marrow biopsy was not performed.

Figure 1 Preoperative CT with intravenous contrast, axial view. (A) An irregular, hypodense retroperitoneal mass with poorly defined margins was seen, measuring 123 mm × 105 mm × 61 mm (long arrow), and a hypodense lesion was noted in hepatic segment VI (short arrow). (B) Left pleural thickening with focal nodularity was observed (arrow). The preoperative clinical diagnosis was pancreatic blastoma with multiple metastases. CT, computed tomography.

Shortly after admission, the patient developed progressive dyspnea. A chest X-ray revealed moderate effusion. After a multidisciplinary discussion, the decision was made to perform a right-sided thoracostomy and diagnostic laparoscopy. Approximately 350 mL of pleural fluid was aspirated for testing. Laparoscopic exploration revealed that a dominant mass was located in the left retroperitoneum, involving the tail of the pancreas and multiple lymph nodes. Tumor samples were obtained for pathological examination. The uterus seemed normal, but both ovaries were enlarged. A subcapsular nodule measuring 2.5 cm × 1.5 cm was identified on the right hepatic lobe. The omentum showed multiple miliary white nodules that were friable. The procedure was uneventful. The patient was then transferred to the pediatric intensive care unit (PICU) for advanced life support, including mechanical ventilation.

Upon arrival in the PICU, her heart rate was 144 breaths per minute (bpm), respiratory rate was 35 bpm, and all other vital signs were stable. The oxygen saturation level was kept above 98%. Two hours later, the patient developed a fever of 40.7 ℃ and was treated with physical cooling measures and ibuprofen. Given a suspected infection, antimicrobial therapy was initiated. Despite these interventions, the fever persisted. Subsequent laboratory tests revealed hyperuricemia and hypocalcemia (Table 2). A diagnosis of laboratory TLS (LTLS) was considered, and consequently, the patient was treated with intravenous fluids, diuretics, and urate-lowering therapy. Nine hours later, the patient developed cardiac arrest with oxygen saturation plummeting to 0%. An arterial blood gas analysis revealed acidosis (pH 7.17), hyperkalemia (potassium 9.1 mmol/L), elevated lactate (4.9 mmol/L), and critically low ionized calcium (0.54 mmol/L). After cardiopulmonary resuscitation, ventilator parameter optimization, and vasopressor support, spontaneous circulation was restored. Repeated administration of sodium bicarbonate, calcium gluconate, insulin-dextrose, and furosemide failed to correct the homeostatic imbalance. Due to persistent hyperkalemia and a lack of improvement in renal function (Table 2), continuous renal replacement therapy (CRRT) was initiated. According to the marked elevation of WBC and C-reactive protein (CRP), the antibiotic was changed to meropenem. With the use of CRRT, her serum potassium and uric acid levels gradually reduced to normal range. Following the resolution of metabolic derangements and restoration of renal function (Table 2), CRRT was discontinued after 48 hours of therapy. Eight days later, the patient had recovered well, so she was weaned off the ventilator. The final pathological diagnosis confirmed BL (Figure 2).

Figure 2 H&E-stained section showing medium-sized tumor cells (white arrow) and macrophages engulfing nuclear fragments of tumor cells (black arrow), demonstrating the characteristic “starry sky pattern” in the retroperitoneal mass biopsy. Original magnification ×100. H&E, hematoxylin and eosin.

On hospital day 15, the patient was moved to the hematology-oncology department to receive chemotherapy. Unexpectedly, bilateral lower limb motor impairment occurred at the same time. Spine MRI demonstrates enhancement of the anterior nerve roots below the T11 level. The cerebrospinal fluid examination revealed immature lymphocytes. In the absence of other causes of paraplegia, she was considered to have secondary intramedullary spinal cord involvement from BL. The patient was discharged after completing the first cycle of BFM-95 chemotherapy. Her treatment timeline is shown in Figure 3. She subsequently underwent six cycles of chemotherapy and tolerated them well. During the 3-year follow-up period, she completed the full course of chemotherapy with good tolerance and showed no evidence of tumor recurrence or metastasis. However, her lower limb motor function has not fully recovered, although it has improved significantly compared to before. Currently, she ambulates independently but demonstrates abnormal postures, including a steppage gait.

Figure 3 Timeline summarizing the events. CRRT, continuous renal replacement therapy; ECPR, extracorporeal cardiopulmonary resuscitation; LTLS, laboratory tumor lysis syndrome; MV, mechanical ventilation; PICU, pediatric intensive care unit.

The study was approved by the Guangzhou Women and Children’s Medical Center Institutional Review Board (approval No. 280A01). All procedures performed in this study were in accordance with the Declaration of Helsinki and its subsequent amendments. Written informed consent was obtained from the patient’s legal guardian for publication of this case report and any accompanying images. A copy of the written consent is available for review by the editorial office of this journal.

Discussion

TLS is an oncologic emergency that typically manifests after the initiation of chemotherapy, resulting from massive lysis of tumor cells. Spontaneous TLS (STLS) refers to TLS occurring in the absence of cytotoxic therapy. The incidence of STLS is extremely low, with a reported rate of only 1.08% in patients with hematologic malignancies (3). To the best of our knowledge, this is the first case describing the development of STLS following laparoscopic biopsy in BL patients. The exact etiology of STLS is mostly unknown. Studies indicate that risk factors such as tumor extension, high initial tumor burden, bulky tumor size, extensive metastasis, and highly proliferative tumor cells may be linked to STLS in solid tumors (4). The patient reported in this case had all these factors mentioned above. Moreover, certain external factors, such as infection, anesthesia, and surgery, are recognized as triggers that may precipitate tumor cell death (5). Recently, Pan et al. reported a case of BL in which STLS occurred during surgical biopsy (6). Although TLS in that case occurred intraoperatively, it shares several features with our patient, including high tumor burden, hyperthermia, and biopsy performed under general anesthesia. This suggests that surgical biopsy is one of several potential triggers for STLS, but not the only factor. Potential contributing mechanisms could include direct physical disruption of the tumor and indirect effects mediated by systemic stress responses. Besides that, our patient was exposed to a suspected infection, which is considered an acute stressor. The course of our patient lends clinical support to the hypothesis proposed by Chapman-Fredricks et al. that endogenous glucocorticoids may contribute to STLS in patients with high lymphoid tumor burden (7).

The internationally validated 2010 Cairo-Bishop criteria stratify TLS risk into low (<1%), intermediate (1–5%), and high (>5%) categories to guide prophylaxis (8). Prior to surgery, the patient showed serum LDH exceeding twice upper limit of normal with radiographic evidence of a large solitary mass and multi-organ involvement. It would be reasonable to classify this as high-risk TLS even before histopathological verification, allowing timely preventive interventions. Unfortunately, risk stratification for TLS was not performed upon the patient’s admission to the PICU.

The Cairo-Bishop criteria define two distinct classifications of TLS (9): LTLS and clinical TLS (CTLS). In this case, the patient received appropriate management immediately after being diagnosed with LTLS. However, her condition progressed from LTLS to CTLS, with worsening electrolyte disturbances going unrecognized due to insufficient monitoring. Hyperkalemia was not detected on biochemical testing until after cardiac arrest occurred. In the National Inpatient Sample study, the development of LTLS did not affect mortality during induction therapy, but CTLS was associated with a significantly higher mortality rate and posed challenges to clinical management (10). This case underscores that timely recognition and management of LTLS prior to progression to CTLS are critical.

After the patient had been stabilized from cardiogenic shock, severe metabolic derangement remained uncorrected. In particular, hyperkalemia is a potentially fatal metabolic complication that has been noted for its unresponsiveness to conventional management in TLS (11). While no guideline exists for when to provide CRRT in TLS, this case demonstrates that urgent CRRT can be a lifesaving intervention when encountering refractory hyperkalemia. CRRT not only rapidly stabilized the child’s internal milieu but also shortened the duration of acute kidney injury. Our experience provides invaluable lessons for clinicians managing patients with BL complicated by STLS.

After experiencing STLS, this patient developed lower extremity weakness, which was a rare complication of BL. CNS involvement is a severe complication of NHL, with poor long-term survival in previous literature (12,13). In this case, the child has remained in complete remission and has shown slow but progressive improvement in functional independence over the three years of post-chemotherapy follow-up. Compared to adult patients, our patient has a better prognosis, which may be related to the higher sensitivity of children to chemotherapeutic drugs.

Conclusions

In view of the high mortality associated with TLS, there is a necessity for risk factor assessment and patient stratification in cases of bulky malignancies. Patients at risk of TLS require prophylaxis during the perioperative period. The potential role of endogenous glucocorticoid release triggered by surgical biopsy and fever in STLS remains unclear but warrants further study. In pediatric patients exhibiting LTLS, it is essential to monitor more frequently as needed, in addition to providing aggressive management. This report contributes to the limited literature on STLS, emphasizing the need for screening and early intervention in high-risk populations. Additionally, for severe hyperkalemia caused by TLS, the timely use of CRRT is extremely important for improving outcomes.

Acknowledgments

None.

Footnote

Reporting Checklist: The authors have completed the CARE reporting checklist. Available at https://acr.amegroups.com/article/view/10.21037/acr-2025-217/rc

Peer Review File: Available at https://acr.amegroups.com/article/view/10.21037/acr-2025-217/prf

Funding: This study was supported by the Guangzhou Science and Technology Bureau (No. 2024A03J1170, awarded to X.L. and X.S.).

Conflicts of Interest: All authors have completed the ICMJE uniform disclosure form (available at https://acr.amegroups.com/article/view/10.21037/acr-2025-217/coif). X.L. and X.S. report that this study was supported by the Guangzhou Science and Technology Bureau (No. 2024A03J1170). The other authors have no conflicts of interest to declare.

Ethical Statement: The authors are accountable for all aspects of the work in ensuring that questions related to the accuracy or integrity of any part of the work are appropriately investigated and resolved. The study was approved by the Guangzhou Women and Children’s Medical Center Institutional Review Board (approval No. 280A01). All procedures performed in this study were in accordance with the Declaration of Helsinki and its subsequent amendments. Written informed consent was obtained from the patient’s legal guardian for publication of this case report and any accompanying images. A copy of the written consent is available for review by the editorial office of this journal.

Open Access Statement: This is an Open Access article distributed in accordance with the Creative Commons Attribution-NonCommercial-NoDerivs 4.0 International License (CC BY-NC-ND 4.0), which permits the non-commercial replication and distribution of the article with the strict proviso that no changes or edits are made and the original work is properly cited (including links to both the formal publication through the relevant DOI and the license). See: https://creativecommons.org/licenses/by-nc-nd/4.0/.

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