A prolactin-secreting tumor in a patient with Klinefelter’s syndrome: A case report

Abstract

We report the case of a patient with Klinefelter's syndrome who developed a prolactin (PRL)-secreting tumor. The patient developed headaches, visual alterations and also symptoms of hypogonadism despite appropriate testosterone (T) replacement therapy. The diagnosis of hyperprolactinemia was then suspected. The laboratory findings confirmed the hypothesis, showing high levels of serum PRL. The patient was initially treated with oral bromocriptine, and afterwards with the injectable form. There was a marked decrease in PRL levels and in tumor size. Although some neoplasms, like breast carcinoma and germ cell tumors, are known to occur more frequently in patients with Klinefelter's syndrome, an association with PRL-secreting tumor has not been reported yet. In conclusion, symptoms of hypogonadism in patients with Klinefelter's syndrome receiving appropriate T replacement therapy can suggest the presence of hyperprolactinemia.

Further Evidence of the Clinical, Hormonal, and Genetic Heterogeneity of Klinefelter Syndrome: A Study of 216 Infertile Egyptian Patients

“…Although PRL‐secreting tumors have been reported in KS patients (Kumanov, 1995; Pinto et al, 1996), hyperprolactinemia was of mild severity in our cases, and computed tomography scans of the pituitary were found to be normal. It is reasonable to assume that because of loss of the negative feedback effect, chronic stimulation of the lactotroph cell type would result in hyperplasia, which in turn might transform into adenoma (Samaan et al, 1979).…”

“…It is reasonable to assume that because of loss of the negative feedback effect, chronic stimulation of the lactotroph cell type would result in hyperplasia, which in turn might transform into adenoma (Samaan et al, 1979). Therefore, it remains to be elucidated whether hyperplasia of the pituitary occurs secondary to gonadal failure (Samaan et al, 1979; Pinto et al, 1996) or is caused by a genetic abnormality linked to KS (Scheithauer et al, 2005). Although unlikely, the hyperplasia observed in KS patients might have been an incidental finding, having no causal relationship with hypogonadism.…”

Hyperprolactinemia as a Side Effect of Long-Acting Injectable Risperidone Therapy or a Symptom of Klinefelter Syndrome – A Diagnostic and Therapeutic Dilemma

“…Our patient was also diagnosed with hyperprolactinemia and gynecomastia. A few case reports and studies have been published regarding the hyperprolactinemia in Klinefelter syndrome (Xu et al 2011, Pinto et al 1996, Kumanov 1995, Takeuchi et al 1999. A number of studies have tried to find a possible underlying disorder in the hypothalamo-pituitary-thyroid axis in patients with Klinfelter syndrome.…”

“…Nuclear magnetic resonance was used to diagnose a pituitary adenoma with a permanent prolactin hypersecretion in our patient. Prolactin-secreting tumors have been reported in Klinefelter syndrome patients (Kumanov et al 1995, Pinto et al 1996. It is reasonable to assume that because of the loss of negative feedback effect, chronic stimulation of lactotroph cell type would result in hyperplasia, which in turn may transform into adenoma.…”

Tumores não hipofisários da região selar

“…Dentre estas situações, pela sua prevalência, devemos chamar a atenção para o hipotireoidismo primário, que provoca sintomas insidiosos, incluindo alterações menstruais e galactorréia, e está associado ao aumento de volume hipofisário que pode alcançar proporções de macroadenoma. Assim, a avaliação de função tireoidiana deve fazer parte da avaliação rotineira dos estados de hiperprolactinemia e aumento de volume selar (34). O diagnóstico diferencial das lesões selares é extenso.…”