Pituitary carcinoma is a rare entity requiring the presence of metastasis to confirm its malignant potential. We report a case of pituitary carcinoma and discuss the diagnosis and management of this lesion in relation to the existing literature.
The patient is a 51-year-old woman with Cushing’s disease and intact adrenal glands who was diagnosed with metastatic pituitary carcinoma to the liver, 29 months after initial resection of an ACTH-secreting primary atypical pituitary adenoma (APA). Prior to detection of this metastasis the patient underwent repeat resection and radiotherapy for residual cavernous sinus disease. The metastatic lesion was detected by interval surveillance of serum ACTH and 24-hour urine cortisol, which despite stable pituitary MRI, were significantly elevated. These abnormalities prompted a PET scan that demonstrated hypermetabolic liver parenchyma, which was suspicious for metastasis on abdominal MRI. An ultrasound-guided liver biopsy demonstrated nests of moderately-differentiated cells with intermediate-sized, monotonous nuclei, distinct nucleoli, and abundant basophilic cytoplasm, confirmed by immunohistochemistry to represent metastatic pituitary carcinoma. The liver lesion was subsequently successfully removed by wedge resection. One year later, the patient’s residual cavernous sinus disease grew markedly, and she was placed on dual-agent chemotherapy consisting of oral temozolomide and capecitabine, with stabilization of her intracranial disease to present, although liver metastases recurred.
Pituitary carcinoma is a rare entity impossible to recognize as a primary tumor because its diagnosis by definition requires the presence of metastasis. Maintaining awareness of the entity and its precursor lesion APA is essential for its accurate pathologic diagnosis and appropriate management.
Joehlin-Price AS, Arnold CA, Kirschner LS, Prevedello DM, Lehman NL. ACTH-secreting pituitary carcinoma metastatic to the liver in a patient with a history of atypical pituitary adenoma and Cushing’s syndrome. Neuro Oncol. 2015;15 Suppl 5:v204.
Queiroz LS, Facure NO, Facure JJ, Modesto NP, de Faria JL. Pituitary carcinoma with liver metastases and Cushing syndrome. Arch Pathol. 1975;99:32–5.
Papotti M, Limone P, Riva C, Gatti G, Bussolati G. Malignant evolution of an ACTH-producing pituitary tumor treated with intrasellar implantation of 90-Y. Appl Pathol. 1984;2:10–21. PubMed
Shastri BR, Nanda A, Fowler M, Levine SN. Adrenocorticotropic hormone-producing pituitary carcinoma with intracranial metastases. World Neurosurg. 2013;79:404.E11–16. CrossRef
Lloyd RV, Kovacs K, Young Jr WF, et al. Tumors of the pituitary. In: DeLellis RA, Heitz P, Lloyd RV, Eng C, editors. WHO classification of tumors of the endocrine organs: Pathology and genetics of endocrine organs. Lyon: IARC Press; 2004. p. 10–45.22.
Riss D, Jin L, Qian Z, Bayliss J, Scheithauer BW, Young WF, et al. Differential expression of galectin-3 in pituitary tumors. Cancer Res. 2003;63:2251–5. PubMed
Scheithauer BW, Kurtkaya-Yapicier O, Kovacs KT, Young Jr WF, Lloyd RV. Pituitary carcinoma: a clinicopathologic review. Neurosurgery. 2005;56:1066–74.
Doniach I. Pituitary carcinoma. Clin Endocrinol. 1992;37:194–5. CrossRef
Hansen TM, Batra S, Lim M, Gallia GL, Burger PC, Salvatori R, et al. Invasive adenoma and pituitary carcinoma: a SEER database analysis. Neurosurgery. 2014;Rev. 37:279–86.
Seltzer J, Carmichael JD, Commins D, Liu CS, Omura E, Chang E, et al. Prolactin-Secreting Pituitary Carcinoma with Dural Metastasis: Diagnosis, Treatment, and Future Directions. World Neurosurg. 2016;91:676.e23–8. CrossRef
Zacharia BE, Gulati AP, Bruce JN, Carminucci AS, Wardlaw SL, Siegelin M, et al. High response rates and prolonged survival in patients with corticotroph pituitary tumors and refractory Cushing disease from capecitabine and temozolomide (CAPTEM): a case series. Neurosurgery. 2014;74:E447–55. CrossRefPubMed
Halevy C, Whitelaw BC. How effective is temozolomide for treating pituitary tumours and when should it be used? Pituitary. 2016. Epub ahead of print. doi: 10.1007/s11102-016-0745-y.
- Case report: ACTH-secreting pituitary carcinoma metastatic to the liver in a patient with a history of atypical pituitary adenoma and Cushing’s disease
Amy S. Joehlin-Price
Douglas A. Hardesty
Christina A. Arnold
Lawrence S. Kirschner
Daniel M. Prevedello
Norman L. Lehman
- BioMed Central