Enigmatic entity in childhood: clival chordoma from a tertiary center’s perspective

Abstract

Clival chordomas are challenging tumors in neurosurgical practice. A multidisciplinary approach is warranted in each patient. Today, the best management strategy seems to be surgical resection followed by radiotherapy. Chemotherapy should be considered in selective and preference basis. Sharing institutional experiences will provide future insights in prognosis of these rare tumors. Implementing newer surgical instruments, endoscope in particular, is encouraged in management of the clival chordomas.

A clinical approach to parasellar lesions in the transition age

“…Chordomas represent approximately 0.2% of primary brain tumours 227 . The cumulative incidence is 1:1,000,000, with different sex predominance among the series 227‐229,233‐241 . Chordomas usually occur in Caucasian 228,229 adults between the ages of 30 and 70 years.…”

“…Clinical manifestations and biochemical alterations depend on the localisation of the tumour mass and its proximity to critical structures, reflecting the specific sites of extension from the clivus 234 . The most frequent presenting feature of chordomas of the cranial base are cranial nerve dysfunction, 233,234,236,242,250,251 diplopia or isolated headaches (55% and 42%, respectively) 234,237,252‐254 (usually present in almost half of cases 251,253,255 ); neck pain, 254 snoring and nasal congestion 242 ; hypopituitarism is seen if the sella turcica is involved.…”

“…CT is essential for evaluating bony integrity and destruction. The classic appearance on HR‐CT is that of a centrally‐located, heterogeneous, well‐circumscribed, expansive soft tissue mass arising from the clivus and associated with extensive lytic bone destruction 233,234,236,242 . The heterogeneity results from the interspersion of isodense tumour tissue with hypodense areas of necrosis or myxoid cysts.…”

“…The latter are frequently associated with the chondroid variant 235,243 . There is moderate to marked enhancement following administration of iodinated contrast material 236 . Low‐attenuation areas, sometimes seen within the soft‐tissue mass, probably represent the myxoid and gelatinous material usually found on histology 256 .…”

Pediatric sinonasal and skull base lesions

“…Optimal outcomes often require a combination of surgical resection followed by radiotherapy. Chemotherapy may be an option in select cases 50 . Radiation or radiation with chemotherapy may be considered in recurrent or persistent disease 51 .…”