Abstract
Pituicytoma is an exceptionally rare low-grade glioma (WHO grade I) of the neurohypophysis and infundibulum. We are reporting the case of a 48-year-old man who presented with severe Cushing′s syndrome. Endocrinological evaluation unequivocally confirmed pituitary-dependent Cushing’s syndrome (=Cushing’s disease). Cranial MR-imaging displayed a conspicuous area in the dorsal and basal pituitary gland and a minimal bulging of the pituitary gland paramedian of the pituitary stalk on the right side. Transsphenoidal inspection revealed a small tumor in the basal and dorsal pituitary gland. Surprisingly, the definite postoperative histopathological diagnosis of the removed tumor was pituicytoma and not pituitary adenoma. Hence, the microadenoma responsible for Cushing’s disease was not yet removed and persistent hypercortisolism necessitated transsphenoidal re-operation. During re-operation, hemihypophysectomy was performed on the right side. The non-tumorous specimen of the adeno-hypophysis showed signs of Crooke’s hyalinization consistent with Cushing’s disease. Undetectable postoperative ACTH- and cortisol levels provided clear evidence that the underlying ACTH-source was successfully removed during re-operation. Coincidence of pituicytoma and pituitary-dependent Cushing’s disease has not previously been reported.
References
Burger PC, Scheithauer BW (1994) Tumors of the central nervous system, atlas of tumor pathology, 3rd edn. Armed Forces Institute of Pathology, Washington, DC
Hurley TR, D’Angelo CM, Clasen RA (1994) Magnetic resonance imaging and pathological analysis of a pituicytoma: case report. Neurosurgery 35:314–317
Brat DJ, Scheithauer BW, Staugaitis SM (2000) Pituicytoma: a distinctive low-grade glioma of the neurohypophysis. Am J Surg Pathol 24:362–368
Schultz AB, Brat DJ, Oyesiku NM, Hunter SB (2001) Intrasellar pituicytoma in a patient with other endocrine neoplasms. Arch Pathol Lab Med 125:527–530
Cenacchi G, Giovenali P, Castrioto C, Giangaspero F (2001) Pituicytoma: ultrastructural evidence of a possible origin from folliculo-stellate cells of the adenohypophysis. Ultrastruct Pathol 25:309–312
Figarella-Branger D, Dufour H, Fernandez C, Bouvier-Labit C, Grisoli F, Pellissier JF (2002) Pituicytomas, a mis-diagnosed benign tumor of the neurohypophysis: report of three cases. Acta Neuropathol 104:313–319
Uesaka T, Miyazono M, Nishio S, Iwaki T (2002) Astrocytoma of the pituitary gland (pituicytoma): case report. Neuroradiology 44:123–125
Katsuta T, Inoue T, Nakagaki H, Takeshita M, Morimoto K, Iwaki T (2003) Distinctions between pituicytoma and ordinary pilocytic astrocytoma. Case report. J Neurosurg 98:404–406
Ulm AJ, Yachnis AT, Brat DJ, Rhoton AL Jr (2004) Pituicytoma: report of two cases and clues regarding histogenesis. Neurosurgery 54:1252
Kowalski RJ, Prayson RA, Mayberg MR (2004) Pituicytoma. Ann Diagn Pathol 8:290–294
Shah B, Lipper MH, Laws ER, Lopes MB, Spellman MJ Jr (2005) Posterior pituitary astrocytoma: a rare tumor of the neurohypophysis: a case report. AJNR Am J Neuroradiol 26:1858–1861
Chen KT (2005) Crush cytology of pituicytoma. Diagn Cytopathol 33:255–257
Takei H, Goodman JC, Tanaka S, Bhattacharjee MB, Bahrami A, Powell SZ (2005) Pituicytoma incidentally found at autopsy. Pathol Int 55:745–749
Nakasu Y, Nakasu S, Saito A, Horiguchi S, Kameya T (2006) Pituicytoma. Two case reports. Neurol Med Chir (Tokyo) 46:152–156
Benveniste RJ, Purohit D, Byun H (2006) Pituicytoma presenting with spontaneous hemorrhage. Pituitary 9:53–58
Gibbs WN, Monuki ES, Linskey ME, Hasso AN (2006) Pituicytoma: diagnostic features on selective carotid angiography and MR imaging. AJNR Am J Neuroradiol 27:1639–1642
Wolfe SQ, Bruce J, Morcos JJ (2008) Pituicytoma: case report. Neurosurgery 63:E173–E174
Orrego JJ (2009) Pituicytoma and isolated ACTH deficiency. Pituitary 12:371–372
Wesseling P, Brat DJ, Fuller GN (2007) Pituicytoma. In: Louis DN, Ohgaki H, Wiestler OD, Cavenee WK (eds) WHO classification of tumours of the central nervous system, Chapter 14. IARC, Lyon, pp 243–244
Buchfelder M, Nistor R, Fahlbusch R, Huk WJ (1993) The accuracy of CT and MR evaluation of the sella turcica for detection of adrenocorticotropic hormone-secreting adenomas in Cushing disease. AJNR Am J Neuroradiol 14:1183–1190
Newnham HH, Rivera-Woll MM (2008) Images in clinical medicine. Hypogonadism due to pituicytoma in an identical twin. N Engl J Med 359:2824
Furtado SV, Ghosal N, Venkatesh PK, Gupta K, Hegde AS (2010) Diagnostic and clinical implications of pituicytoma. J Clin Neurosci 17: Epub ahead of print
Grote A, Kovacs A, Clusmann H, Becker AJ, Niehusmann P (2010) Incidental pituicytoma after accidental head trauma—case report and review of literature. Clin Neuropathol 29:127–133
Hammer GD, Tyrrell JB, Lamborn KR, Applebury CB, Hannegan ET, Bell S, Rahl R, Lu A, Wilson CB (2004) Transphenoidal microsurgery for Cushing′s disease: initial outcome and long-term results. J Clin Endocrinol Metab 89:6348–6357
Author information
Authors and Affiliations
Corresponding author
Additional information
J. Honegger and R. Beschorner contributed equally to the work and thus share senior-authorship.
Rights and permissions
About this article
Cite this article
Schmalisch, K., Schittenhelm, J., Ebner, F.H. et al. Pituicytoma in a patient with Cushing’s disease: case report and review of the literature. Pituitary 15 (Suppl 1), 10–16 (2012). https://doi.org/10.1007/s11102-010-0262-3
Published:
Issue Date:
DOI: https://doi.org/10.1007/s11102-010-0262-3