Chordoma : Clinical Features
Image Description
Chordoma - Clinical Presentation: Chordoma is an indolent but locally destructive tumor that tends to grow into large masses. The 7 cm fleshy, hemorrhagic tumor shown here arose in the sacrococcygeal region.
Symptoms depend upon the site of origin and are produced by compression of surrounding structures, vessels, and nerves. Spheno-occipital tumors present with headaches, visual field defects, diplopia, cranial neuropathies (especially VI cranial nerve), and radiculopathies. They can destroy pituitary gland and produce pituitary dysfunction. The tumor may also erode the bone inferiorly producing a nasal, paranasal, or a nasopharyngeal mass. Sacral tumors frequently cause pain at the tip of spinal column, constipation, incontinence, erectile dysfunction, radiculopathies, and motor weakness in lower extremities.
Skull base tumors are generally small (2-5 cm) because they produce symptoms early given their location. Sacral tumors can attain large sizes (> 10 cm) as they remain clinical silent in early stages and have room to grow. Metastases occur via hematogenous route in 30-40% of cases to sites such as lungs (most common), subcutaneous tissues, liver, lymph nodes, and cerebrospinal fluid. Cases presenting at young age, involving base of skull, or with multiple chordomas may be familial variants and should be investigated.
Chordomas in Children: Cases presenting before the age of 20 years make up only 5% of all chordomas. The average age at diagnosis in children is 10 years. However, cases have been reported in infants in association with tuberous sclerosis. Pediatric chordomas have a predilection for the base of skull and upper cervical spine. Some cases present with lung metastases as the initial manifestation.
Image courtesy of Dr. Bulent Celasun, Ankara, Turkey. Used with permission.
Symptoms depend upon the site of origin and are produced by compression of surrounding structures, vessels, and nerves. Spheno-occipital tumors present with headaches, visual field defects, diplopia, cranial neuropathies (especially VI cranial nerve), and radiculopathies. They can destroy pituitary gland and produce pituitary dysfunction. The tumor may also erode the bone inferiorly producing a nasal, paranasal, or a nasopharyngeal mass. Sacral tumors frequently cause pain at the tip of spinal column, constipation, incontinence, erectile dysfunction, radiculopathies, and motor weakness in lower extremities.
Skull base tumors are generally small (2-5 cm) because they produce symptoms early given their location. Sacral tumors can attain large sizes (> 10 cm) as they remain clinical silent in early stages and have room to grow. Metastases occur via hematogenous route in 30-40% of cases to sites such as lungs (most common), subcutaneous tissues, liver, lymph nodes, and cerebrospinal fluid. Cases presenting at young age, involving base of skull, or with multiple chordomas may be familial variants and should be investigated.
Chordomas in Children: Cases presenting before the age of 20 years make up only 5% of all chordomas. The average age at diagnosis in children is 10 years. However, cases have been reported in infants in association with tuberous sclerosis. Pediatric chordomas have a predilection for the base of skull and upper cervical spine. Some cases present with lung metastases as the initial manifestation.
Image courtesy of Dr. Bulent Celasun, Ankara, Turkey. Used with permission.