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A 54-year-old man presented with a history of several weeks of fatigue and weight loss. He also reported atrophy of the testes and loss of secondary hair that had begun one and a half years earlier. The results of neurologic and ophthalmologic examinations were normal. Evaluation of serum hormone levels revealed a slightly increased prolactin level (20.9 ng per milliliter) and decreased levels of follicle-stimulating hormone (0.2 IU per milliliter), luteinizing hormone (<0.7 IU per milliter), and testosterone (<0.1 ng per milliliter [0.347 nmol per liter]). The levels of growth hormone, thyrotropin, and corticotropin were within normal limits. Contrast-enhanced, T1-weighted magnetic resonance imaging showed a tumor measuring approximately 3 by 3 by 3.5 cm in the sellar region (Panel A, arrow). As shown in Panel B, the tumor reached the internal carotid arteries (arrows) and the optic chiasm (arrowhead), without compressing the chiasm. Furthermore, there was massive involvement of the skull base.
The patient underwent total resection of the tumor, by way of a transsphenoidal approach. After surgery, his neurologic status remained normal, and he received supplementary testosterone. Histologic examination revealed features consistent with a diagnosis of chondroid clival chordoma. This rare subtype of chordoma has been associated with longer survival than that associated with conventional chordoma. It is also characterized by local recurrence; metastases into the spinal canal may occur. During the next two years, this patient presented twice with local tumor recurrence and both times was treated with surgery.
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