title: Pituicytoma and Cushing's Disease in a 7-Year-Old Girl: A Mere Coincidence?
abstract: Pituicytoma is a tumor extremely rare in childhood, with only 4 cases reported in literature. It is thought to arise from the specialized glial elements called "pituicytes." The association of pituicytoma and Cushing's disease (CD) has been described only once so far, in an adult patient. A 7-year-old girl was referred for clinical signs of hypercortisolism, and a diagnosis of CD was made. MRI revealed 2 pathologic areas in the pituitary gland. The patient underwent surgery, with microscopic transsphenoidal approach, and a well-circumscribed area of pathologic tissue was identified and removed. Surprisingly, histologic and immunohistochemical study provided unequivocal evidence of pituicytoma. No pituitary adenoma could be identified. For persistent hypercortisolism, the patient necessitated transsphenoidal endoscopic reintervention and 2 other lesions were removed. By immunohistological examination, these lesions were confirmed to be corticotropin-secreting adenoma. Unfortunately, there was no postoperative decrease in corticotropin and cortisol levels, and the patient underwent bilateral laparoscopic adrenalectomy. Considering that we report a second case of association of pituicytoma and corticotropin-secreting adenoma, that CD is infrequent, and pituicytoma is extremely rare in childhood, the coexistence of these 2 tumors should not be considered a mere coincidence. To date, there is no conclusive evidence about the origin of these different subtypes of pituitary tumors. This case supports the hypothesis that these tumors share a common progenitor cell, which could be the folliculostellate cell.
authors.affiliation: Endocrinology and Diabetology Unit, University Department of Pediatrics.
authors.forename: Paola
authors.initials: P
authors.lastname: Cambiaso
authors.affiliation: Immune and Infectious Diseases Unit, University Department of Pediatrics, University of Rome Tor Vergata, don.amodio85@hotmail.it.
authors.forename: Donato
authors.initials: D
authors.lastname: Amodio
authors.affiliation: Neurosurgery Unit, Department of Neuroscience.
authors.forename: Emidio
authors.initials: E
authors.lastname: Procaccini
authors.affiliation: Neuroradiology Unit, Department of Imaging, and.
authors.forename: Daniela
authors.initials: D
authors.lastname: Longo
authors.affiliation: Neuroradiology Unit, Department of Imaging, and.
authors.forename: Stefania
authors.initials: S
authors.lastname: Galassi
authors.affiliation: Pathology Unit, Department of Laboratories, Bambino Gesù Children's Hospital, IRCCS, Rome, Italy.
authors.forename: Francesca Diomedi
authors.initials: FD
authors.lastname: Camassei
authors.affiliation: Endocrinology and Diabetology Unit, University Department of Pediatrics.
authors.forename: Marco
authors.initials: M
authors.lastname: Cappa
mesh_terms: D049913:ACTH-Secreting Pituitary Adenoma / Q000150:complications / Q000175:diagnosis; D000236:Adenoma / Q000150:complications / Q000175:diagnosis*; D002648:Child; D005260:Female; D006801:Humans; D047748:Pituitary ACTH Hypersecretion / Q000175:diagnosis / Q000209:etiology*; D010911:Pituitary Neoplasms / Q000150:complications / Q000175:diagnosis*