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Hypothalamic neuronal hamartoma and adenohypophyseal neuronal choristoma: their association with growth hormone adenoma of the pituitary gland.

Hypothalamic neuronal hamartomas and neuronal choristomas of the anterior pituitary are rare lesions; either may be associated with endocrinopathy. We describe a case of each with associated growth hormone-producing pituitary adenomas and clinical acromegaly, both well documented and studied by immunocytochemistry and electron microscopy. That a functional relationship exists between the neuronal malformation and the pituitary neoplasm remains speculative. We suggest that a growth hormone-releasing factor-like substance may have been elaborated by hypothalamic-type neurons, which, by a trophic effect, may have resulted in the production of an adenohypophyseal neoplasm. Our study supports the concept that secretory neurons, either outside or within the sella, may induce adenomas.

Acromegaly↗

SUNCT syndrome secondary to prolactinoma.

Short-lasting unilateral neuralgiform headache attacks with conjunctival injection and tearing (SUNCT) syndrome is a rare form of primary headache disorder, although secondary causes, particularly posterior fossa abnormalities, are well known. We report a case of SUNCT syndrome secondary to a prolactinoma. Administration of dopamine agonists led to complete resolution of the SUNCT attacks. This case, together with other similar case reports in the literature, highlight the importance of excluding a diagnosis of pituitary adenoma in all suspected cases of SUNCT syndrome, especially as the headache can precede more classical pituitary symptoms by a considerable period of time. Clinicians managing patients with suspected SUNCT syndrome should elicit a history of symptoms associated with pituitary neoplasms, perform a magnetic resonance imaging scan of the brain and pituitary, and screen for serum hormonal abnormalities.

Adenoma↗

From prolactin cell to prolactinoma.

The pathogenesis, diagnosis and treatment of common pituitary neoplasms is based on the understanding of the differentiated hormone expression by specific pituitary cell types and of the central hypothalamic, peripheral and local control of their hormonal secretion (endocrine, autocrine, paracrine regulation). Tumorigenesis is the result of a complex multistep process. It is likely that a molecular genetic alteration initially leads to cell transformation. Nevertheless the transformed cell is dependent on hormonal and/or growth factor stimulation for tumor progression. This cascade is responsible for autonomic hormone secretion, and cell proliferation. PRL cells do not behave identically in terms of cell function. Functional diversity among PRL cells is documented in vitro, in terms of basal and synthetic capacity and in respect to responsiveness to dopamine and oestrogen regulation. These in vitro observations can be extended to the in vivo situation. In the intact pituitary, PRL cells differ in their relative content of PRL and their response to secretagogues. These differences relate to their topographical localization within the pituitary. Prolactinoma are mainly composed of cells with a high basal synthetic and secretory capacity and arise preferentially in the peripheral parts of the hypophysis. It is therefore tempting to postulate that the "in situ milieu" within the pituitary is relevant for the development of prolactinoma. By stimulating growth in certain areas, there is an increased probability of mutation. At the same time growth of a mutated PRL cell could be stimulated.

Cell Division↗

Giant pituitary adenomas: clinical characteristics and surgical results.

Although most pituitary neoplasms are benign, some grow rapidly, spreading to extrasellar tissues. Definition of these 'giant' pituitary adenomas (PAs) is not clear. In order to clarify this question, we studied all pituitary macroadenomas operated in our hospital during the last 20 years, differentiating those with diameter > or = 3 cm (n = 43) and attempting to identify their typical features and prognosis. The reason for consultation was local in 58.3% of giant PAs, hormonal in 22.9% and tumour recurrence in 16.7%. Surgery was performed via a trans-sphenoidal approach in 92.5% of cases and succeeded in completely removing the tumour in only 27% of cases. In conclusion, we found that giant PAs are not a special type but the extreme case in the gradient of invasiveness of tumours of adenohypophyseal origin. They are characterized by a higher frequency of neuro-ophthalmological symptoms and hormonal deficits, and poorer response to surgical treatment.

Adenoma↗

Pituitary carcinoma: a review of the literature.

Pituitary carcinomas, defined as distant metastases of a pituitary neoplasm, are rare; fewer than 140 reports exist in the English literature. The initial presenting pituitary tumor is usually a secreting, invasive macroadenoma, with adrenocorticotropic hormone (ACTH)--and prolactin (PRL)--secreting tumors being the most common. The latency period between the diagnosis of a pituitary tumor and the diagnosis of a pituitary carcinoma is 9.5 years for ACTH-producing lesions and 4.7 years for PRL-secreting tumors. Survival after documentation of metastatic disease is poor; 66% of patients die within 1 year. Treatment options include additional surgery, radiotherapy, and chemotherapy, all of which are associated with poor results. Future studies will focus on identifying those invasive pituitary tumors most likely to metastasize and treating them aggressively before they progress to pituitary carcinomas.

Adrenocorticotropic Hormone↗

Bilateral and simultaneous inferior petrosal sinus sampling in the early diagnosis of an ACTH-producing pituitary microadenoma and its detection by magnetic resonance one year later.

This study describes a case of pituitary-dependent Cushing's syndrome where standard biochemical and radiological techniques were discordant in localizing the origin of the autonomous adrenocorticotropic hormone (ACTH) hypersecretion in the pituitary. Hormonal evaluation suggested a pituitary genesis for the disease, but both sellar computed tomography and cranial magnetic resonance (MR) were unable to give clear-cut evidence for a pituitary neoplasm. Simultaneous and bilateral inferior petrosal sinus sampling (SBIPS) correctly identified the left side of the pituitary gland as the source of autonomous ACTH production. One year later, the shaded signs of a pituitary lesion in the left side of the gland were seen with MR imaging, and a 0.5-cm in height adenoma was surgically removed. At the 2-year follow-up the patient's symptoms had completely disappeared, and her menses were restored. In this case, SBIPS correctly diagnosed the presence of an ACTH-secreting pituitary microadenoma one year before shaded signs of the pituitary lesion appeared with MR imaging. This is a clear-cut demonstration of the accuracy of the SBIPS technique in localizing small pituitary lesions.

Adenoma↗