Chordoma represents a rare type of cancer that originates in the bone, specifically from remnants of the notochord, a structure present during embryonic development. This malignant tumor can appear anywhere along the spine, though it most frequently affects the sacrum (the triangular bone at the base of the spine) or the coccyx (tailbone). This condition is uncommon, affecting approximately one in one million people worldwide each year, with around 300 new diagnoses in the United States annually.
What is Coccygeal Chordoma
Chordoma arises from notochord remnants, cells that guide spinal formation during fetal development. While these cells typically disappear after birth, residual pieces can sometimes remain. For reasons not fully understood, these leftover cells can transform into a cancerous tumor.
The coccyx is a common site for chordomas because notochordal tissue is often found in this region. This tumor is slow-growing, meaning it can take years for symptoms to appear. Despite its slow growth, chordoma is locally aggressive, tending to invade and damage surrounding bone and soft tissues.
Chordomas are malignant tumors, often diagnosed in individuals between 50 and 70 years of age, though they can occur at any age. Their unique cellular origin and growth patterns set them apart from other bone malignancies.
Symptoms and Diagnosis
Symptoms associated with coccygeal chordoma often progress slowly, making early detection challenging. A common symptom is persistent pain in the tailbone area, which may be mistaken for more common back issues. The tumor’s growth can compress nearby nerves, leading to symptoms like numbness, tingling, or weakness in the legs.
As the tumor expands, it can also affect bowel and bladder function, causing changes or difficulties in control. These neurological symptoms arise from the tumor pressing on nerves that supply the lower extremities and pelvic organs.
The diagnostic process begins with a physical examination and a review of the patient’s medical history. Imaging techniques are then employed to locate and characterize the tumor.
Magnetic Resonance Imaging (MRI) is often used to assess the tumor’s size and its relationship to surrounding structures. Computed Tomography (CT) scans can help evaluate bone involvement and determine if the tumor has spread.
A definitive diagnosis is established through a biopsy, where a small tissue sample is taken from the tumor. This sample is then examined by a pathologist under a microscope to confirm the presence of chordoma cells, which have a unique bubbly appearance and contain brachyury protein.
Treatment Options
Surgical resection is considered the primary treatment for coccygeal chordoma. The objective of surgery is to achieve complete tumor removal while preserving as much function as possible. Due to the tumor’s location near delicate nerves and structures, achieving clear margins—removing all cancerous tissue—can be challenging.
Even with complete removal, there is a possibility of microscopic tumor cells remaining, which is why radiation therapy often follows surgery. Radiation therapy, particularly proton beam therapy, is frequently used as an adjuvant treatment. This type of radiation delivers a precise dose to the tumor area, minimizing damage to surrounding healthy tissues and helping to control local recurrence.
Chemotherapy and targeted therapies have a more limited role in the treatment of chordoma. These systemic treatments are generally reserved for cases where the tumor has spread or for situations where surgery and radiation are not sufficient. Ongoing research continues to explore new therapeutic approaches, including immunotherapy, which aims to harness the body’s immune system to fight the cancer.
Long-Term Outlook
The long-term outlook for patients with coccygeal chordoma is influenced by several factors, including the completeness of surgical removal and the tumor’s initial size. Despite aggressive treatment, local recurrence is a concern, emphasizing the importance of regular follow-up imaging, such as MRI scans. This ongoing monitoring helps detect any re-growth early.
Patients undergo surveillance imaging at regular intervals, every 6 to 12 months, for several years following treatment. A multidisciplinary approach to care, involving surgeons, radiation oncologists, and other specialists, is beneficial for managing this complex disease. Continued research efforts are aimed at improving treatment strategies and enhancing the long-term prognosis for individuals with chordoma.