Tumor lysis syndrome (TLS) is an oncologic emergency characterized by a combination of metabolic derangements (hyperuricemia, hyperkalemia, hyperphosphatemia, and hypocalcemia) caused by rapid turnover from cell destruction in certain cancers. These metabolic derangements can lead to seizures, cardiac arrhythmias, renal failure, and death. TLS is usually seen after the initiation of chemotherapy for hematologic malignancies. TLS occurring spontaneously, without initiation of chemotherapy, is rare and its occurrence in solid tumors is rarer still. We report a case of spontaneous TLS in a patient with leiomyosarcoma of the uterus, with metastasis to lung. Such a case has never been reported before.
Tumor lysis syndrome (TLS) is an oncologic emergency resulting from lysis of tumor cells with release of intracellular contents into the circulation [
TLS is most commonly seen during the initiation of chemotherapy in aggressive hematologic malignancies, such as acute myeloid leukemia, acute lymphoblastic leukemia, and non-Hodgkin’s lymphoma [
A 58-year-old Caucasian female with no significant past medical history presented from a community hospital with a 3-week history of abdominal distension and constipation. She also complained of nausea, fatigue, shortness of breath with exertion, and decreased appetite. On examination, the patient had an extremely firm and distended lower abdomen with tenderness in the right lower quadrant. The rest of her examination was otherwise unremarkable. A computed tomogram (CT) scan of the abdomen showed a large heterogeneous, partially necrotic abdominal-pelvic mass, 16.3 cm by 20.2 cm in size (Figure
CT scan of the abdomen showing a large heterogeneous, partially necrotic abdominal-pelvic mass, 16.3 cm by 20.2 cm in size.
CT scan of chest showing a moderate right-sided pleural effusion, as well as bilateral pulmonary nodules with hilar adenopathy.
Because of the size of her tumor, she was subsequently transferred to a tertiary health center for further evaluation and possible surgery. As seen in Table
Progression of the patient’s electrolyte derangements.
Patient disposition | Potassium (mg/dL) | Phosphorus (mg/dL) | Ionized calcium (mmol/L) | Uric acid (mg/dL) | Creatinine (mg/dL) | Bicarbonate (mg/dL) | LDH (mg/dL) | Lactate (mmol/L) |
---|---|---|---|---|---|---|---|---|
Admission to community hospital | 3.2 | 3.6 | N/A | N/A | 1 | 28.2 | 774 | 3.8 |
Prior to transfer to tertiary care center | 6.2 | 9.8 | N/A | 15.1 | 2.3 | 19.4 | N/A | 2.3 |
Admission to tertiary care center | 6.2 | 9.8 | 1 | 15.1 | 3 | 9 | N/A | 11.1 |
On ICU admission | 6.5 | 11.5 | 1.07 | 16.1 | 2.8 | 14 | 1243 | 5.5 |
Prior to death | 4.3 | 6.5 | 1.14 | 7 | 2.3 | 17 | 1066 | 5 |
The patient’s electrolytes worsened, with potassium of 6.5 mg/dL, phosphorus of 11.1 mg/dL, and a uric acid of 16.1 mg/dL. She also had a lactic acid level of 11.1 mg/dL. She was transferred to the ICU due to hypotension (blood pressure of 62/49 mm Hg), requiring vasopressor support, and increased work of breathing due to her profound acidosis (for which she was placed on rescue noninvasive bilevel positive airway pressure)
Final pathology from the biopsies of the abdominal mass and lung nodules taken at the community hospital was consistent with leiomyosarcoma with metastases to the lungs. The patient however was deemed a poor surgical candidate for debulking as well as a poor candidate for dialysis. She was additionally unable to be weaned off of vasopressor support. Given the patient’s poor prognosis, the patient opted for comfort care. She was placed on hospice and expired subsequently.
The Cairo-Bishop laboratory criteria of TLS, established in 2004, define laboratory TLS as meeting two of the following: uric acid > 8 mg/dL, potassium > 6.5 mg/dL, phosphorus > 4.5 mg/dL in adults (or a 25% increase from baseline in all), and calcium < 7 mg/dL (or a 25% decrease from baseline) [
TLS primarily occurs after initiating chemotherapy in hematologic malignancies. Aggressive non-Hodgkin’s lymphoma (such as Burkitt’s lymphoma), acute myeloid leukemia, and acute lymphoblastic leukemia have the highest risk of TLS [
According to a risk-stratification system, for TLS, high-risk group was defined as >5% risk for developing TLS [
Prophylactic uric acid lowering therapy is important in the prevention of TLS in malignancies that have the potential to develop it. Intravenous hydration promotes the excretion of uric acid; additionally, allopurinol blocks the production of uric acid by inhibiting xanthine oxidase. In malignancies with a high risk of developing TLS, aggressive intravenous hydration as well as rasburicase (although controversial for prophylactic use), which converts uric acid to a readily excreted metabolite, should be given prophylactically [
Intravenous hydration and correction of other electrolyte derangements such as hyperkalemia are crucial in the management for TLS. Correction of hyperkalemia can be achieved via kayexalate as well as dialysis [
Spontaneous tumor lysis syndrome makes up for only 15% of TLS cases and is typically seen in highly aggressive hematologic malignancies [
Additionally, TLS occurring in vaginal, vulvar, and ovarian cancers has been previously reported [
The mechanism of occurrence of spontaneous TLS is hypothesized to be the consequence of necrosis developing in a large tumor as vascular supply is compromised by tumor growth and necrosis of tumor cells progress to tumor lysis from necrotic tumor cell contents being released into circulation [
This case illustrates a presentation of spontaneous TLS in a gynecologic solid tumor which has not been previously reported. This case demonstrates that TLS may occur in large solid tumors even without the initiation of chemotherapy. Patients with large tumors should be closely monitored for metabolic derangements that may indicate the development of spontaneous TLS.
An earlier version of this work was presented as an abstract at CHEST 2016 Annual Meeting Abstracts.
The authors declare that they have no conflicts of interest.