ABSTRACT
A 38-years-old woman presented to our hospital 2 years ago with 5-year history of reduced vision, chronic cephaleea and secondary amenorrhea. Her baseline prolactin level was 46504 ?IU/mL (reference range=127-637mUI/ml) with secondary hypogonadism, and pituitary magnetic resonance imaging revealed a giant prolactinoma (4 × 2.2 × 2.6 cm3) with suprasellar extension, optic chiasma and right cavernous sinus compression. She was initially treated with cabergoline in order to reduce the prolactin level and tumoral mass, but after 6 months of medical treatment she proceed to transsphenoidal tumor debulking surgery. Prolactin level dropped in the normal range only after the addition of cabergoline treatment after surgery (1 mg/day 3 times/per week). However, the extensive tumour was not completely resectable so she remained amenorrheic requiring hormone replacement therapy as well as thyroxine replacement due to the development of TSH deficiency (free T4 6.03 pmol/L)
ABSTRACT
El prolactinoma gigante es un adenoma pituitario poco frecuente caracterizado por su gran invasión local. Se reporta el caso de un varón de 15 años de edad con dolor retroocular izquierdo y exoftalmos ipsilateral de 4 meses de evolución secundario a un tumor en la base del cráneo que invadía la órbita. Los estudios hormonales revelaron prolactina sérica de 6913,7 ng/ml (valor normal < 20), que confirmó el diagnóstico de prolactinoma gigante. El paciente inició un tratamiento con el agonista dopaminérgico cabergolina en dosis crecientes. Luego de 7 meses de seguimiento, la prolactina había descendido a 349,8 ng/ml y el volumen del tumor se redujo un 70%, sin efectos adversos al tratamiento. El paciente se encontraba asintomático y había reiniciado la pubertad. La rápida remisión de los síntomas sin necesidad de tratamientos invasores subraya la importancia de considerar el diagnóstico de prolactinoma entre los posibles diagnósticos diferenciales de tumor de la base del cráneo.
Giant prolactinomas are rare pituitary adenomas characterized by their great local invasion. In this paper, we report a 15-year-old male with left retro-ocular pain and ipsilateral exophthalmos of 4 months of evolution, secondary to a tumour in the base of the skull that invaded the orbit. Hormonal studies revealed serum prolactin of 6913,7 ng/ml (normal value < 20), confirming the diagnosis of giant prolactinoma. The patient started treatment with the dopaminergic agonist cabergoline in increasing doses. After 7 months of follow-up the prolactin had decreased to 349.8 ng/ml and the tumor volume was reduced by 70%, without presenting adverse effects to the treatment. The patient was asymptomatic and had restarted puberty. The rapid remission of symptoms without the need for invasive treatments underlines the importance of considering the diagnosis of prolactinoma among the possible differential diagnoses of tumor of the skull base.
Subject(s)
Humans , Male , Adolescent , Pituitary Neoplasms/diagnosis , Prolactinoma/diagnosis , Pituitary Neoplasms/pathology , Prolactinoma/pathologyABSTRACT
Pituitary tumors occur in 15-50% of patients with multiple endocrine neoplasia of type 1 (MEN1). To the best of our knowledge, no MEN1 case in which the initial lesion was an invasive giant prolactinoma has been reported from Korea. We describe a patient in whom a skull-base tumor involved the sellar and parasellar spaces. A 49 year-old female presented with headache and diplopia. The tumor was ultimately identified as a giant prolactinoma; the serum prolactin concentration increased from 155.6 ng/mL to 3,234.3 ng/mL after cranial irradiation. She was evaluated in terms of incidental hypercalcemia and was found to have parathyroid hyperplasia. Genetic analysis revealed a missense mutation in the MEN1 gene (c.643G>A, p.Val215Met). Two years of treatment with a dopamine agonist reduced, but did not normalize, the serum prolactin concentration. We highlight the aggressive behavior of the giant skull-base tumor, and the diagnostic delay caused by a high-dose hook effect of the MEN1-related prolactinoma.
Subject(s)
Female , Humans , Cranial Irradiation , Diplopia , Dopamine Agonists , Headache , Hypercalcemia , Hyperplasia , Korea , Multiple Endocrine Neoplasia Type 1 , Multiple Endocrine Neoplasia , Mutation, Missense , Pituitary Neoplasms , Prolactin , ProlactinomaABSTRACT
The prevalence of pituitary among adolescents is 0.1 per million and the most common type is prolactinoma. We report two adolescents with pituitary adenomas. A 15 years old female presenting with a progressive reduction of visual acuity, headache and galactorrhea. Magnetic resonanceshowed a sellar tumor with suprasellar expansion. She was subjected to transcraneal surgery and the pathological study of the piece disclosed a prolactinoma. A 23 years old male presenting with weight gain, headache and decreased visual acuity of the left eye. Magnetic resonance demonstrateda tumor with sellar and suprasellar involvement. He was subjected to transsphenoidal surgery and the pathological study of the surgical piece disclosed a prolactinoma.
Subject(s)
Humans , Male , Female , Adolescent , Pituitary Neoplasms/surgery , Pituitary Neoplasms/diagnosis , Prolactinoma/surgery , Prolactinoma/diagnosis , Biopsy , Immunohistochemistry , Magnetic Resonance Imaging , Pituitary Neoplasms/pathology , Prolactinoma/pathology , Vision Disorders/etiologyABSTRACT
Although cabergoline is effective in the treatment of micro- and macro-prolactinoma, little is known about its efficacy in the treatment of invasive giant prolactinoma. We investigated the efficacy and safety of cabergoline in 10 male patients with invasive giant prolactinoma. Before treatment, mean serum prolactin level was 11,426 ng/mL (range, 1,450-33,200 ng/mL) and mean maximum tumor diameter was 51 mm (range, 40-77 mm). Three months after initiation of cabergoline treatment, serum prolactin concentrations decreased more than 97% in 9 patients; at last follow-up (mean treatment duration, 19 months), the mean decrease in serum prolactin concentrations was 98%, with 5 patients having normal serum prolactin levels. At first MRI follow-up (3-12 months after initiation of cabergoline), the mean reduction in tumor size was 85+/-4% (range, 57-98%). Cabergoline treatment for more than 12 months caused a greater reduction in tumor size compared to the treatment for less than 12 months (97+/-1% vs. 78+/-7%, P<0.05). These findings indicate that cabergoline treatment led to a significant and rapid reduction in serum prolactin concentrations and tumor size in patients with giant prolactinoma. Therefore, cabergoline represents an effective and well-tolerated treatment for invasive giant prolactinoma.