Clival prolactinoma masquerading as a chordoma: a case report

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Abstract

Ectopic pituitary adenomas are a rare clinical entity and are frequently mistaken for other base of skull lesions on imaging. We report the clinical presentation and management of a woman presenting with an ectopic prolactinoma located in the clivus. A 66-year-old female presented with a 6-month history of headaches and light-headedness. Anatomical imaging demonstrated a clival lesion most suspicious for chordoma. Endocrinological assessment revealed modestly increased prolactin level with lower-than-expected gonadotrophins levels for her age. Surgical resection confirmed an ectopic prolactinoma.

A skull base lesion in a patient with hormonal derangement should lend to a high clinical suspicion of an EPA as they may be treated with medications before surgery. Guidelines could assist clinicians investigating skull-based lesions to identify the rare, but important diagnosis of ectopic pituitary adenomas.

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Pituitary adenomas: an overview.

Cathleen M. Lake, Andrea V Cruz, L Krook (2013)

Prolactinomas and nonfunctioning adenomas are the most common types of pituitary adenomas. Patients with pituitary adenomas may present initially with symptoms of endocrine dysfunction such as infertility, decreased libido, and galactorrhea, or with neurologic symptoms such as headache and visual changes. The diagnosis may also be made following imaging done for an unrelated issue in an asymptomatic patient; this is termed a pituitary incidentaloma. Oversecretion of hormones from a dysfunctional pituitary gland may result in classic clinical syndromes, the most common of which are hyperprolactinemia (from oversecretion of prolactin), acromegaly (from excess growth hormone), and Cushing disease (from overproduction of adrenocorticotropic hormone). In the diagnostic approach to a suspected pituitary adenoma, it is important to evaluate complete pituitary function, because hypopituitarism is common. Therapy for pituitary adenomas depends on the specific type of tumor, and should be managed with a team approach to include endocrinology and neurosurgery when indicated. Dopamine agonists are the primary treatment for prolactinomas. Small nonfunctioning adenomas and prolactinomas in asymptomatic patients do not require immediate intervention and can be observed.

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Radiosurgery for chordomas and chondrosarcomas of the skull base.

Juan J Martin, V. J. Martin, L. Lunsford … (2007)

Chordomas and chondrosarcomas of the skull base are aggressive and locally destructive tumors with a high tendency for local progression despite treatment. The authors evaluated the effect of stereotactic radiosurgery (SRS) on local tumor control and survival. Twenty-eight patients with histologically confirmed chordomas (18) or chondrosarcomas (10) underwent Gamma Knife SRS either as primary or adjuvant treatment. Their ages ranged from 17 to 72 years (median 44 years). The most common presenting symptom was diplopia (26 patients, 93%). In two patients, SRS was the sole treatment. Twenty-six patients underwent between one and five additional surgical procedures. Two underwent an initial transsphenoidal biopsy. The average tumor volume was 9.8 cm3. The median dose to the tumor margin was 16 Gy. No patient was lost to follow-up. Transient symptomatic adverse radiation effects developed in only one patient. The actuarial local tumor control for chondrosarcomas at 5 years was 80 +/- 10.1%. For chordomas both the actuarial tumor control and survival was 62.9 +/- 10.4%. Stereotactic radiosurgery is an important option for skull base chordomas and chondrosarcomas either as primary or adjunctive treatment. Multimodal management appears crucial to improve tumor control in most patients.

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Chordomas and chondrosarcomas of the skull base: comparative analysis of clinical results in 30 patients.

Ki Kim, Jun Cho, Shin Khang … (2007)

Chordomas and chondrosarcomas occur rarely in the skull base and have been often considered commonly with respect to the management. The aim of this study was to investigate the clinical results in each series of these tumors and analyze comparative outcome. Between 1991 and 2005, 30 consecutive patients with pathological diagnosis of chordoma (n=19) or chondrosarcoma (n=11) of the skull base were managed by multimodal treatment combining surgical resection with conventional photon radiotherapy and/or gamma knife radiosurgery. A retrospective analysis was conducted on these patients (aged 3 to 69 years; mean age of 37.3 years; 17 females and 13 males; mean follow-up of 56.1 months). Four deaths occurred among the patients with chordoma. The progression-free survival rate at 3 and 5 years was 61.5 and 40.0% in chordoma. In contrast, no mortalities were observed, and there was only one recurrence among the patients with chondrosarcoma. The progression-free survival rate at 3 and 5 years was 88.9 and 80.0% in chondrosarcoma. Among 15 survivors with chordoma, four patients were suffering from severe disability with progressive disease. On the other hand, most patients with chondrosarcoma harbored stable disease and less disabling symptoms except one instance of recurrence. As the biological behavior of chordoma is much more aggressive than that of chondrosarcoma in the skull base, one should make a distinction between these entities to plan an optimal treatment strategy.

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Quynh Truong

Journal

Journal ID (nlm-ta): Radiol Case Rep

Journal ID (iso-abbrev): Radiol Case Rep

Title: Radiology Case Reports

Publisher: Elsevier

ISSN (Electronic): 1930-0433

Publication date PMC-release: 01 April 2021

Publication date Collection: June 2021

Publication date (Electronic): 01 April 2021

Volume: 16

Issue: 6

Pages: 1320-1324

Affiliations

[a ]Internal Medicine Services, Royal Brisbane and Women's Hospital, Brisbane, Queensland, Australia

[b ]Department of Endocrinology & Diabetes, Royal Brisbane and Women's Hospital, Brisbane, Queensland, Australia

[c ]Department of Medical Imaging, Royal Brisbane and Women's Hospital, Brisbane, Queensland, Australia and Faculty of Medicine, University of Queensland, Herston, Queensland, Australia

[d ]Department of Neurosurgery, Royal Brisbane and Women's Hospital, Brisbane, Queensland, Australia

[e ]Department of Endocrinology & Diabetes, Royal Brisbane and Women's Hospital, Brisbane, Queensland, Australia

Author notes

[* ]Corresponding author. quynh.truong@ 123456health.qld.gov.au

Article

Publisher Item ID: S1930-0433(21)00134-5

DOI: 10.1016/j.radcr.2021.02.071

PMC ID: 8053778

SO-VID: e9bca0e0-f1c7-40ba-b216-374fd4aca6d9

License:

This is an open access article under the CC BY-NC-ND license (http://creativecommons.org/licenses/by-nc-nd/4.0/).

History

Date received : 8 February 2021

Date revision received : 28 February 2021

Date accepted : 28 February 2021

Clival prolactinoma masquerading as a chordoma: a case report

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Abstract

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Most cited references 20

Pituitary adenomas: an overview.

Radiosurgery for chordomas and chondrosarcomas of the skull base.

Chordomas and chondrosarcomas of the skull base: comparative analysis of clinical results in 30 patients.

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