bubble_chart Overview

Chordoma is a rare primary malignant tumor located within the vertebral bodies and intervertebral discs, rarely involving the presacral soft tissues. Most chordomas originate from residual notochordal tissue near the vertebrae rather than the intervertebral discs. It predominantly occurs in middle-aged and elderly individuals between 50 and 60 years old, but can also affect other age groups. Both sexes are equally susceptible, with no difference in incidence. The tumor grows slowly, often existing for over five years before symptoms appear. Approximately 50% of chordomas occur in the sacrococcygeal region, 35% in the spheno-occipital area, and the remaining cases are distributed in the cervical, thoracic, and lumbar spine. Sacrococcygeal tumors are most common in individuals aged 40 to 70, while spheno-occipital tumors are more frequently seen in children. In the sacral region, osteolytic destruction is commonly observed, with osteogenesis being rare. If the tumor invades the spine, spinal cord compression symptoms may occur due to direct infiltration, often involving retroperitoneal tissues. When the tumor grows large enough, it may cause intestinal lumen narrowing or invade the bladder. A rectal examination may reveal an extraluminal mass. Spheno-occipital chordomas can also extend into the nasopharynx, affecting various cranial nerves.

bubble_chart Pathological Changes

Gross examination: The tumor appears round or lobulated, soft and gelatinous. There may be local hemorrhage, necrosis, cystic changes, and calcification. In the early stages, it generally has a

membrane, and nearby areas often contain bone fragments and sequestra. Microscopic examination: The tumor tissue exhibits considerable variability, differing between cases and even within different regions of the same tumor. Poorly differentiated tissue shows tightly packed cells that are smaller in size with clear boundaries, and minimal intracellular and extracellular mucinous components. In contrast, well-differentiated tissue has sparsely arranged cells that are larger, spindle-shaped or polygonal, with prominent vacuoles in the cytoplasm. The tumor stroma contains fibrous septa and abundant mucinous {|###|} abdominal mass. In highly malignant cases, mitotic figures may be observed.

bubble_chart Clinical Manifestations

Pain is the earliest symptom, often caused by tumor expansion invading or compressing adjacent important tissues or organs. Tumors located in the sacrococcygeal region often cause coccygeal pain, followed by the appearance of a local mass that gradually enlarges, protruding subcutaneously. It may also extend into the pelvic cavity, compressing the bladder and rectum, leading to symptoms such as urinary incontinence, constipation, and sciatica. Tumors in the spheno-occipital region can compress the optic nerve and other cranial nerves, the pituitary gland, brainstem, etc., and in the late stage [third stage], may cause intracranial hypertension. When the spinal cord is compressed around the vertebral canal, it can lead to radicular pain, paraplegia, and major incontinence of urine.

X-ray findings: Skull chordomas are commonly found at the skull base, near the sella turcica, the spheno-occipital synchondrosis, and the petrous bone. The bone destruction has relatively clear boundaries, with possible residual small bone fragments and patchy calcified deposits. A soft tissue mass may protrude into the nasopharyngeal cavity, usually large with smooth edges. When the chordoma mass protrudes into the cranial cavity, it can displace the calcified pineal gland and occasionally cause intracranial hypertension. Cervical chordomas are often located in the upper cervical vertebrae, especially at the junction of the cervical vertebrae and occipital bone, mostly involving the vertebral body. The pedicle may occasionally be affected, and a soft tissue mass is often an early prominent manifestation. Thoracic chordomas are relatively rare.

Chordomas occurring in the spine are most commonly found in the sacrococcygeal region, often at the junction of the sacrum and coccyx. The affected bone is significantly expanded, with the normal internal structure disappearing, presenting a ground-glass shadow. The tumor shows osteolytic defects and may sometimes break through the cortical bone, extending into the buttocks and pelvic cavity, forming a well-defined tumorous soft tissue mass. There may be residual bone sclerosis or scattered amorphous calcification points within, with locally increased density. Contrast examination can reveal displacement of adjacent organs or tissues such as the rectum, bladder, and uterus due to compression.

Chordomas in the spine above the sacrum, when involving a single or 2–3 adjacent vertebral bodies, show osteolytic destruction, sometimes with scattered calcification points. In cases of single vertebral body damage, round or spotty areas of bone rarefaction may be observed.

bubble_chart Treatment Measures

The most effective method is complete resection of the tumor. However, if the tumor's location makes complete removal difficult, partial resection may be performed. Friedman suggests that radiotherapy is also effective for certain chordomas, with doses as high as 50–60 Gy (5000–6000 rads).

bubble_chart Prognosis

The probability of chordoma metastasis is not high. Chordomas located in the sacrococcygeal region are more likely to metastasize, while those in the skull base and spine above the sacrum rarely metastasize. Metastasis usually occurs more than 10 years after the tumor develops, often involving local lymph nodes first, followed by hematogenous spread to the lungs, liver, and abdominal membrane. The prognosis of chondroid chordoma in the occipital region, which contains abundant cartilage components, is generally better than that of typical chordoma.