Sellar chordoma presenting as pseudo-macroprolactinoma with unilateral third cranial nerve palsy

Abstract

We described a 61-year-old female with a sellar chordoma, which presented as pseudo-macroprolactinoma with unilateral third cranial nerve palsy. Physical examination revealed that her right upper lid could not be raised by itself, right eyeball movement limited to the abduction direction, right pupil dilated to 4.5 mm with negative reaction to light, and hemianopsia in bitemporal sides. CT scanning showed a hyperdense lesion at sellar region without bone destruction. Magnetic resonance imaging (MRI) revealed the tumor was 2.3 cm×1.8 cm×2.6 cm, with iso-intensity on T1WI, hyper-intensity on T2WI and heterogeneous enhancement on contrast imaging. Endocrine examination showed her serum prolactin level increased to 1,031.49 mIU/ml. The tumor was sub-totally resected via pterional craniotomy under microscope and was histologically proven to be a chordoma. Postoperatively, she recovered uneventfully but ptosis and hemianopsia remained at the 6th month.

Extensive sphenoid chordoma mimicking a prolactinoma

“…Pseudohyperprolactinemia is a frequent situation which pose a diagnostic challenge. Only four cases of pseudohyperprolactinoma have been reported previously where chordoma was associated with increased prolactin values [1-3]. The mechanism underlying the higher level of serum prolactin is not fully understood.…”

“…The mechanism underlying the higher level of serum prolactin is not fully understood. Pituitary talk compression syndrome was proposed to explain this phenomenon [3]. Chordomas are hardly distinguished from pituitary adenomas.…”

Immunophenotypic features of dedifferentiated skull base chordoma: An insight into the intratumoural heterogeneity

“…Contemporary research in chordoma biology and pathogenesis is scarce. Hence, accurate radiologic diagnosis and better understanding of the pathology of skull base chordomas are of valuable prognostic significance [ 4 , 55 , 56 ]. Nonetheless, the exact correlation of molecular data with specific pathological features in chordoma subtypes is not well understood.…”

“…The differentiation between typical and atypical chordomas is relevant because the tumoural microenvironment and prognosis are partially different. According to recent studies, the prognosis of patients with dedifferentiated chordoma is poor, and the majority of patients will experience local recurrence, or metastases, and will die of local progression within 6–12 months after diagnosis [ 2 , 17 , 34 , 37 , 42 , 49 , 54 , 55 ].…”

Prolactin-producing pituitary adenoma with atypical spindle cell morphology: a case report

“…In instances where an intracranial mass and hyperprolactinemia coexist, dysfunction of the hypophyseal stalk due to the mass effect of various tumors (i.e., chordoma, schwannoma, paraganglioma, or mixed germ cell tumor) [ 6 ] and tumors arising near sella, other than prolactinoma, must be considered. Differential diagnosis was required since the histological findings in this case were different from that of typical pituitary adenoma.…”