Giant sacrococcygeal chordoma presenting with isolated urinary symptoms: A case report

Background: Chordomas are rare low-grade malignant tumors arising from remnants of the primitive notochord. They most commonly affect the sacrum and typically present with lower back pain and radiculopathy. Genitourinary or gastrointestinal symptoms usually occur later in the disease course and are commonly associated with neurologic deficits. Case description: We report the case of an 83-year-old Latin American male who presented with a 1-year history of intermittent dysuria, urinary frequency, nocturia, tenesmus, and dyschezia, without back pain or neurologic symptoms. Initial urologic and infectious evaluations were unrevealing. Subsequent abdominal and pelvic imaging revealed a giant presacral mass originating from S4, measuring 13.5 cm, consistent with sacral chordoma. The patient underwent partial surgical resection; however, due to the highly vascular nature of the tumor and intraoperative complications, he developed multiorgan failure and died shortly after surgery. Conclusion: Sacral chordomas may present atypically with isolated urinary or gastrointestinal symptoms in the absence of neurologic complaints. In patients with persistent pelvic or lower urinary tract symptoms and a negative urologic workup, early cross-sectional imaging should be considered. Awareness of these rare presentations may facilitate earlier diagnosis and improve outcomes.