Adenohypophyseal hyperfunction syndromes and posterior pituitary tumors: prevalence, clinical characteristics, and patho
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REVIEW
Adenohypophyseal hyperfunction syndromes and posterior pituitary tumors: prevalence, clinical characteristics, and pathophysiological mechanisms Pedro Iglesias
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Fernando Guerrero-Pérez3 Carles Villabona3 Juan José Díez1,2,4 ●
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Received: 13 April 2020 / Accepted: 20 June 2020 © Springer Science+Business Media, LLC, part of Springer Nature 2020
Abstract Posterior pituitary tumors are rare nonneuroendocrine neoplasms originating in the neurohypophysis that lack hormonal secretory capacity. Surprisingly, these tumors are relatively frequently associated with adenohypophyseal syndromes of hormonal hypersecretion such as Cushing’s disease and acromegaly. Fifteen cases of posterior pituitary tumor associated with hypercortisolism have been reported to date, 13 of them were pituicytomas (Pi) and 2 were granular cell tumors (GCT). Six patients with posterior pituitary tumor associated with acromegaly have been reported (4 Pi and 2 GCT). The main forms of clinical presentation and the possible pathophysiological mechanisms of this association are reviewed. Keywords Cushing’s disease Acromegaly Hyperprolactinemia Posterior pituitary tumor Pituicytoma Granular cell tumor ●
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Introduction Posterior pituitary tumors (PPTs) are very rare neoplasms, with less than 300 cases reported to date [1, 2]. They are low-grade nonneuroendocrine sellar/suprasellar tumors originating from pituicytes, specialized glial cells of the neurohypophysis [3]. Four pathological types have been reported: pituicytoma (Pi), granular cell tumor (GCT), spindle cell oncocytoma (SCO), and sellar ependymoma (SE). These tumors are immunohistochemically characterized by expression of thyroid transcription factor-1 (TTF-1) as described in fetal and adult pituicytes but not in neurosecretory cells of the adenohypophysis [4]. As evidenced by
* Pedro Iglesias [email protected] 1
Departments of Endocrinology, Hospital Universitario Puerta de Hierro Majadahonda, Madrid, Spain
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Instituto de Investigación Sanitaria Puerta de Hierro Segovia de Arana (IDIPHISA), Majadahonda, Madrid, Spain
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Hospital Universitario Bellvitge l’Hospitalet de Llobregat, Barcelona, Spain
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Department of Medicine, Universidad Autónoma de Madrid, Madrid, Spain
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a negative immunohistochemical staining for all adenohypophyseal hormones and ultrastructural microscopic characteristics, pituicytes do not have the ability to synthesize and secrete hormones. PPTs are usually diagnosed in the 5th decade of life without sex predilection. Pi is the most common histological type (approximately half of the cases), followed by GCT (~25%), SCO (18%), and SE (3%) [1]. They usually manifest as visual disturbances, headaches with or without hypopituitarism. The prevalence of diabetes insipidus is very low at clinical presentation [1, 5]. However, a nonnegligible percentage of patients have been associated with hypercortisolism [5–17] or acromegaly [5, 11, 12, 17, 18]. This association is relatively high given the low incidence of both dis
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