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Therapeutic challenges of giant invasive cystic macroprolactinoma
S Mekni1,2, H Khiari1, S Bouali3
1Department of Endocrinology, Charles Nicolle Hospital, Tunis, Tunisia.
Summary:
Giant cystic prolactinomas are extremely rare, with only four cases previously reported in the literature. Herein, we report the case of a 59-year-old male who presented with an acute decline in visual acuity. An urgent brain MRI revealed a large intra- and suprasellar tumor, consisting of a solid (48 × 31 × 23 mm) and a cystic suprasellar (39.9 × 38.7 × 26.3 mm) component, with cavernous sinus invasion and optic chiasm compression. Hormonal workup revealed hyperprolactinemia (1,020 ng/mL) and panhypopituitarism. The patient was initially treated with bromocriptine. After 3 months, his serum prolactin levels increased despite adequate dosing, and no improvement was observed in his visual deficits. Consequently, he underwent transcranial surgery with subtotal tumor resection. Histopathological examination confirmed PRL-secreting pituitary adenoma. Postoperatively, despite sequential treatment with bromocriptine and cabergoline (maximum approved dose of 3 mg/week), prolactin levels failed to normalize. Furthermore, follow-up MRI revealed a persistent 29 × 21 × 33 mm intra- and suprasellar tumor. This report presents a case of a dopamine agonist (DA)-resistant giant cystic prolactinoma, highlighting the therapeutic challenges associated with this rare tumor subtype.
Learning Points:
Giant cystic prolactinomas are extremely rare. Dopamine agonists remain the first-line therapy for both giant and cystic prolactinomas. Early surgical intervention is indicated for vision-threatening conditions or in cases of dopamine agonist resistance.
Insights
Giant cystic prolactinomas are rare tumors. This case highlights a dopamine agonist-resistant giant cystic prolactinoma, emphasizing challenges in treatment and the need for surgical intervention when medical therapy fails.
Area of Science:
- Endocrinology
- Neurosurgery
- Oncology
Background:
- Giant cystic prolactinomas are exceptionally rare pituitary tumors.
- These tumors can cause significant visual impairment and hormonal dysfunction.
Purpose of the Study:
- To report a rare case of giant cystic prolactinoma.
- To highlight therapeutic challenges, particularly dopamine agonist resistance, in managing this rare subtype.
Main Methods:
- Case presentation of a 59-year-old male with visual decline.
- Diagnostic imaging (MRI) and hormonal workup (hyperprolactinemia, panhypopituitarism).
- Treatment involved bromocriptine, surgery, and subsequent cabergoline therapy.
Main Results:
- The patient presented with a large intra- and suprasellar tumor with optic chiasm compression.
- Initial bromocriptine treatment was ineffective.
- Despite surgery and maximal medical therapy (cabergoline), prolactin levels remained elevated, indicating dopamine agonist resistance.
Conclusions:
- Giant cystic prolactinomas can be resistant to dopamine agonist therapy.
- Surgical intervention is crucial for vision-threatening or treatment-resistant cases.
- This case underscores the complex management of rare, resistant prolactinomas.
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