Intracranial metastasis from a sacrococcygeal chordoma

Abstract

Chordoma is a locally invasive tumor of low metastatic potential. Only six cases of chordoma that metastasized to the brain are found in the English literature. Most of these lesions were clinically silent and all were associated with extraneural metastases. The authors report a case of symptomatic brain metastasis from a sacrococcygeal chordoma in the absence of other metastases. The incidence, sites, and factors predictive of chordoma metastasis are discussed.

Brain Metastasis in Bone and Soft Tissue Cancers: A Review of Incidence, Interventions, and Outcomes

“…Only about 70 metastasizing cases have been reported, characterizing chordomas as more locally invasive and less susceptible to distant metastasis [51]. There is a male predominance overall, though sacrococcygeal chordomas are more frequent in females [53]. No age preference is apparent, with children as young as 2 and adults in their 70 s afflicted [50].…”

“…It is estimated 25–43% of sacrococcygeal chordomas will present with subsequent metastasis [51], most often to lymph nodes, liver, and lungs [54]. Metastasis presenting in the brain remains rare [51], and complete resection of the primary is vital for prevention and overall patient survival [53]. Table 3 outlines characteristics from published case reports on BM from chordoma [49, 51–56].…”

Multicentric Chordoma

“…[4][5][6] Metastatic chordomas were once thought to be relatively rare; however, in recent series, metastases have been reported in 10% to 48% of cases. 7 Reported sites of metastases have been lymph nodes, lungs, pancreas, brain, bone, adrenal glands, pituitary gland, skin, and bone, including the vertebral bodies. 6,7 Metastases to the neuraxis are rare, however, and often do not occur without significant systemic spread.…”

“…7 Reported sites of metastases have been lymph nodes, lungs, pancreas, brain, bone, adrenal glands, pituitary gland, skin, and bone, including the vertebral bodies. 6,7 Metastases to the neuraxis are rare, however, and often do not occur without significant systemic spread. 8 Very few cases of multicentric chordomas have been reported in the literature.…”

Solitary lymph node metastasis without local recurrence of primary chordoma

“…Organs which are frequently sites of metastases are: lung (48%), bones (26%) and sometimes liver. The sporadic cases of metastases in CNS [14], lymphatic organs [2,5], breast [27] and skin and subcutaneous tissue [18] have been reported. Some cases of unusual clinical presentations of chordoma have also been emphasized [1,19].…”