Chordoma: a rarity in South-east Nigeria

Abstract

Chordomas originate from embryonic remnant of the notochord. They are primary bone neoplasms located on the midline from the skull base to the sacrum. Chordomas are slow growing, locally aggressive tumor with predilection for the spheno-occipital region of the skull base and sacral regions. The usual clinical manifestations of chordoma are insidious and nonspecific, making late presentation of patients inevitable. Chordomas arising in the sacrum may appear as a large retrorectal or sacrococcygeal mass. We report a case of a 45-year-old woman with radicular symptoms, unsteady gait and urinary incontinence associated with a sacrococcygeal mass chordoma. Histologic sections showed aggregates of cells separated into lobules by an extensive amount of mucoid intercellular tissue and thin fibrous septa. Some of the tumor cells appearing physaliferous, were extremely large, with vacuolated cytoplasm, hyperchromatic and vesicular nuclei with prominent nucleoli. Other tumor cells were small, with inconspicuous nuclei and mitoses are sparse. The tumor showed positive reactivity to S100, EMA and AE1/AE3 antibodies, and negativity to GFAP, CK-7, CK-20 and Desmin. To the best of our knowledge, chordoma has not shown to have occurred in Southeast Nigeria with an over 20 million population as evidenced by dearth of documentations in the literature.