Skip to main content
Top
Published in: World Journal of Surgical Oncology 1/2009

Open Access 01-12-2009 | Review

ACTH-producing carcinoma of the pituitary with refractory Cushing's Disease and hepatic metastases: a case report and review of the literature

Authors: Scott N Pinchot, Rebecca Sippel, Herbert Chen

Published in: World Journal of Surgical Oncology | Issue 1/2009

Login to get access

Abstract

Background

Pituitary carcinomas are rare neuroendocrine tumors affecting the adenohypophysis. The hallmark of these lesions is the demonstration of distant metastatic spread. To date, few well-documented cases have been reported in the literature.

Case presentation

Here, we report the case of a fatal pituitary carcinoma evolving within two years from an adrenocorticotrophic hormone (ACTH)-secreting macroadenoma and review the global literature regarding this rare neuroendocrine tumor.

Conclusion

Pituitary carcinomas are extremely rare neoplasms, representing only 0.1% to 0.2% of all pituitary tumors. To date, little is understood about the molecular basis of malignant transformation. The latency period between initial presentation of a pituitary adenoma and the development of distal metastases marking carcinoma is extremely variable, and some patients may live well over 10 years with pituitary carcinoma.
Appendix
Available only for authorised users
Literature
1.
go back to reference Asa S, Ezzat S: The cytogenesis and pathogenesis of pituitary adenomas. Endocr Rev. 1998, 19: 798-827. 10.1210/er.19.6.798.PubMed Asa S, Ezzat S: The cytogenesis and pathogenesis of pituitary adenomas. Endocr Rev. 1998, 19: 798-827. 10.1210/er.19.6.798.PubMed
2.
go back to reference Landman R, Horwith M, Peterson R, Khandji A, Wardlaw S: Long-term survival with ACTH-secreting carcinoma of the pituitary: a case report and review of the literature. J Clin Endocrinol Metab. 2002, 87: 3084-3089. 10.1210/jc.87.7.3084.CrossRefPubMed Landman R, Horwith M, Peterson R, Khandji A, Wardlaw S: Long-term survival with ACTH-secreting carcinoma of the pituitary: a case report and review of the literature. J Clin Endocrinol Metab. 2002, 87: 3084-3089. 10.1210/jc.87.7.3084.CrossRefPubMed
3.
go back to reference Ragel B, Couldwell W: Pituitary carcinoma: a review of the literature. Neurosurg Focus. 2004, 16: E7-10.3171/foc.2004.16.4.8.PubMed Ragel B, Couldwell W: Pituitary carcinoma: a review of the literature. Neurosurg Focus. 2004, 16: E7-10.3171/foc.2004.16.4.8.PubMed
4.
go back to reference Pernicone P, Scheithauer B, Sebo T, Kovacs K, Horvath E, Young WJ, Lloyd R, Davis D, Guthrie B, Schoene W: Pituitary carcinoma: a clinicopathologic study of 15 cases. Cancer. 1997, 79: 804-812. 10.1002/(SICI)1097-0142(19970215)79:4<804::AID-CNCR18>3.0.CO;2-3.CrossRefPubMed Pernicone P, Scheithauer B, Sebo T, Kovacs K, Horvath E, Young WJ, Lloyd R, Davis D, Guthrie B, Schoene W: Pituitary carcinoma: a clinicopathologic study of 15 cases. Cancer. 1997, 79: 804-812. 10.1002/(SICI)1097-0142(19970215)79:4<804::AID-CNCR18>3.0.CO;2-3.CrossRefPubMed
5.
go back to reference Scheithauer B, Kovacs K, Laws EJ, Randall R: Pathology of invasive pituitary tumors with special reference to functional classification. J Neurosurg. 1986, 65: 733-744.CrossRefPubMed Scheithauer B, Kovacs K, Laws EJ, Randall R: Pathology of invasive pituitary tumors with special reference to functional classification. J Neurosurg. 1986, 65: 733-744.CrossRefPubMed
6.
go back to reference Della Casa S, Corsello S, Satta M, Rota C, Putignano P, Vangeli V, Colosimo C, Anile C, Barbarino A: Intracranial and spinal dissemination of an ACTH secreting pituitary neoplasia. Case report and review of the literature. Ann Endocrinol (Paris). 1997, 58: 503-509. Della Casa S, Corsello S, Satta M, Rota C, Putignano P, Vangeli V, Colosimo C, Anile C, Barbarino A: Intracranial and spinal dissemination of an ACTH secreting pituitary neoplasia. Case report and review of the literature. Ann Endocrinol (Paris). 1997, 58: 503-509.
7.
go back to reference Kemink S, Wesseling P, Pieters G, Verhofstad A, Hermus A, Smals A: Progression of a Nelson's adenoma to pituitary carcinoma; a case report and review of the literature. J Endocrinol Invest. 1999, 22: 70-75.CrossRefPubMed Kemink S, Wesseling P, Pieters G, Verhofstad A, Hermus A, Smals A: Progression of a Nelson's adenoma to pituitary carcinoma; a case report and review of the literature. J Endocrinol Invest. 1999, 22: 70-75.CrossRefPubMed
8.
go back to reference Brown R, Muzzafar T, Wollman R, Weiss R: A pituitary carcinoma secreting TSH and prolactin: a non-secreting adenoma gone awry. Eur J Endocrinol. 2006, 154: 639-643. 10.1530/eje.1.02141.CrossRefPubMed Brown R, Muzzafar T, Wollman R, Weiss R: A pituitary carcinoma secreting TSH and prolactin: a non-secreting adenoma gone awry. Eur J Endocrinol. 2006, 154: 639-643. 10.1530/eje.1.02141.CrossRefPubMed
9.
go back to reference Koyama J, Ikeda K, Shose Y, Kimura M, Obora Y, Kohmura E: Long-term survival with non-functioning pituitary carcinoma – case report. Neurol Med Chir (Tokyo). 2007, 47: 475-478. 10.2176/nmc.47.475.CrossRef Koyama J, Ikeda K, Shose Y, Kimura M, Obora Y, Kohmura E: Long-term survival with non-functioning pituitary carcinoma – case report. Neurol Med Chir (Tokyo). 2007, 47: 475-478. 10.2176/nmc.47.475.CrossRef
10.
go back to reference Guastamacchia E, Triggiani V, Tafaro E, De Tommasi A, De Tommasi C, Luzzi S, Sabbà C, Resta F, Terreni M, Losa M: Evolution of a prolactin-secreting pituitary microadenoma into a fatal carcinoma: a case report. Minerva Endocrinol. 2007, 32: 231-236.PubMed Guastamacchia E, Triggiani V, Tafaro E, De Tommasi A, De Tommasi C, Luzzi S, Sabbà C, Resta F, Terreni M, Losa M: Evolution of a prolactin-secreting pituitary microadenoma into a fatal carcinoma: a case report. Minerva Endocrinol. 2007, 32: 231-236.PubMed
11.
go back to reference Brown R, Wollman R, Weiss R: Transformation of a pituitary macroadenoma into to a corticotropin-secreting carcinoma over 16 years. Endocr Pract. 2007, 13: 463-471.CrossRefPubMed Brown R, Wollman R, Weiss R: Transformation of a pituitary macroadenoma into to a corticotropin-secreting carcinoma over 16 years. Endocr Pract. 2007, 13: 463-471.CrossRefPubMed
12.
go back to reference Manahan M, Dackiw A, Ball D, Zeiger M: Unusual case of metastatic neuroendocrine tumor. Endocr Pract. 13: 72-76. Manahan M, Dackiw A, Ball D, Zeiger M: Unusual case of metastatic neuroendocrine tumor. Endocr Pract. 13: 72-76.
13.
go back to reference Tena-Suck M, Salinas-Lara C, Sánchez-García A, Rembao-Bojórquez D, Ortiz-Plata A: Late development of intraventricular papillary pituitary carcinoma after irradiation of prolactinoma. Surg Neurol. 2006, 66: 527-533. 10.1016/j.surneu.2006.02.039. discussion 533CrossRefPubMed Tena-Suck M, Salinas-Lara C, Sánchez-García A, Rembao-Bojórquez D, Ortiz-Plata A: Late development of intraventricular papillary pituitary carcinoma after irradiation of prolactinoma. Surg Neurol. 2006, 66: 527-533. 10.1016/j.surneu.2006.02.039. discussion 533CrossRefPubMed
14.
go back to reference Fadul C, Kominsky A, Meyer L, Kingman L, Kinlaw W, Rhodes C, Eskey C, Simmons N: Long-term response of pituitary carcinoma to temozolomide. Report of two cases. J Neurosurg. 2006, 105: 621-626. 10.3171/jns.2006.105.4.621.CrossRefPubMed Fadul C, Kominsky A, Meyer L, Kingman L, Kinlaw W, Rhodes C, Eskey C, Simmons N: Long-term response of pituitary carcinoma to temozolomide. Report of two cases. J Neurosurg. 2006, 105: 621-626. 10.3171/jns.2006.105.4.621.CrossRefPubMed
15.
go back to reference Kumar K, Wilson J, Li Q, Phillipson R: Pituitary carcinoma with subependymal spread. Can J Neurol Sci. 2006, 33: 329-332.CrossRefPubMed Kumar K, Wilson J, Li Q, Phillipson R: Pituitary carcinoma with subependymal spread. Can J Neurol Sci. 2006, 33: 329-332.CrossRefPubMed
16.
go back to reference Sivan M, Nandi D, Cudlip S: Intramedullary spinal metastasis (ISCM) from pituitary carcinoma. J Neurooncol. 2006, 80: 19-20. 10.1007/s11060-006-9156-7.CrossRefPubMed Sivan M, Nandi D, Cudlip S: Intramedullary spinal metastasis (ISCM) from pituitary carcinoma. J Neurooncol. 2006, 80: 19-20. 10.1007/s11060-006-9156-7.CrossRefPubMed
17.
go back to reference Mamelak AN, Carmichael JD, Park P, Bannykh S, Fan X, Bonert HV: Atypical pituitary adenoma with malignant features. Pituitary. 2008, Mamelak AN, Carmichael JD, Park P, Bannykh S, Fan X, Bonert HV: Atypical pituitary adenoma with malignant features. Pituitary. 2008,
18.
go back to reference Siddiqui A, Bashir S: Giant pituitary macroadenoma at the age of 4 months: case report and review of the literature. Childs Nerv Syst. 2006, 22: 290-294. 10.1007/s00381-005-1179-4.CrossRefPubMed Siddiqui A, Bashir S: Giant pituitary macroadenoma at the age of 4 months: case report and review of the literature. Childs Nerv Syst. 2006, 22: 290-294. 10.1007/s00381-005-1179-4.CrossRefPubMed
19.
go back to reference Garrão A, Sobrinho L, Pedro-Oliveira , Bugalho M, Boavida J, Raposo J, Loureiro M, Limbert E, Costa I, Antunes J: ACTH-producing carcinoma of the pituitary with haematogenic metastases. Eur J Endocrinol. 1997, 137: 176-180. 10.1530/eje.0.1370176.CrossRefPubMed Garrão A, Sobrinho L, Pedro-Oliveira , Bugalho M, Boavida J, Raposo J, Loureiro M, Limbert E, Costa I, Antunes J: ACTH-producing carcinoma of the pituitary with haematogenic metastases. Eur J Endocrinol. 1997, 137: 176-180. 10.1530/eje.0.1370176.CrossRefPubMed
20.
go back to reference Gaffey T, Scheithauer B, Lloyd R, Burger P, Robbins P, Fereidooni F, Horvath E, Kovacs K, Kuroki T, Young WJ: Corticotroph carcinoma of the pituitary: a clinicopathological study. Report of four cases. J Neurosurg. 2002, 96: 352-360.CrossRefPubMed Gaffey T, Scheithauer B, Lloyd R, Burger P, Robbins P, Fereidooni F, Horvath E, Kovacs K, Kuroki T, Young WJ: Corticotroph carcinoma of the pituitary: a clinicopathological study. Report of four cases. J Neurosurg. 2002, 96: 352-360.CrossRefPubMed
21.
go back to reference Zafar M, Mellinger R, Chason J: Cushing's disease due to pituitary carcinoma. Henry Ford Hosp Med J. 1984, 32: 61-66.PubMed Zafar M, Mellinger R, Chason J: Cushing's disease due to pituitary carcinoma. Henry Ford Hosp Med J. 1984, 32: 61-66.PubMed
22.
go back to reference Zhao D, Tomono Y, Nose T: Expression of P27kip1 and Ki-67 in pituitary adenomas: an investigation of marker of adenoma invasiveness. Acta Neurochir (Wien). 1999, 141: 187-192. 10.1007/s007010050285.CrossRef Zhao D, Tomono Y, Nose T: Expression of P27kip1 and Ki-67 in pituitary adenomas: an investigation of marker of adenoma invasiveness. Acta Neurochir (Wien). 1999, 141: 187-192. 10.1007/s007010050285.CrossRef
23.
go back to reference Vaquero J, Herrero J, Cincu R: Late development of frontal prolactinoma after resection of pituitary tumor. J Neurooncol. 2003, 64: 255-258. 10.1023/A:1025672617249.CrossRefPubMed Vaquero J, Herrero J, Cincu R: Late development of frontal prolactinoma after resection of pituitary tumor. J Neurooncol. 2003, 64: 255-258. 10.1023/A:1025672617249.CrossRefPubMed
24.
go back to reference Zahedi A, Booth G, Smyth H, Farrell W, Clayton R, Asa S, Ezzat S: Distinct clonal composition of primary and metastatic adrencorticotrophic hormone-producing pituitary carcinoma. Clin Endocrinol (Oxf). 2001, 55: 549-556. 10.1046/j.1365-2265.2001.01322.x.CrossRef Zahedi A, Booth G, Smyth H, Farrell W, Clayton R, Asa S, Ezzat S: Distinct clonal composition of primary and metastatic adrencorticotrophic hormone-producing pituitary carcinoma. Clin Endocrinol (Oxf). 2001, 55: 549-556. 10.1046/j.1365-2265.2001.01322.x.CrossRef
25.
go back to reference Matoso A, Zhou Z, Hayama R, Flesken-Nikitin A, Nikitin A: Cell lineage-specific interactions between Men1 and Rb in neuroendocrine neoplasia. Carcinogenesis. 2008, 29: 620-628. 10.1093/carcin/bgm207.CrossRefPubMed Matoso A, Zhou Z, Hayama R, Flesken-Nikitin A, Nikitin A: Cell lineage-specific interactions between Men1 and Rb in neuroendocrine neoplasia. Carcinogenesis. 2008, 29: 620-628. 10.1093/carcin/bgm207.CrossRefPubMed
26.
go back to reference Harvey M, Vogel H, Lee E, Bradley A, Donehower L: Mice deficient in both p53 and Rb develop tumors primarily of endocrine origin. Cancer Res. 1995, 55: 1146-1151.PubMed Harvey M, Vogel H, Lee E, Bradley A, Donehower L: Mice deficient in both p53 and Rb develop tumors primarily of endocrine origin. Cancer Res. 1995, 55: 1146-1151.PubMed
27.
go back to reference Williams B, Schmitt E, Remington L, Bronson R, Albert D, Weinberg R, Jacks T: Extensive contribution of Rb-deficient cells to adult chimeric mice with limited histopathological consequences. EMBO J. 1994, 13: 4251-4259.PubMedCentralPubMed Williams B, Schmitt E, Remington L, Bronson R, Albert D, Weinberg R, Jacks T: Extensive contribution of Rb-deficient cells to adult chimeric mice with limited histopathological consequences. EMBO J. 1994, 13: 4251-4259.PubMedCentralPubMed
28.
go back to reference Hinton D, Hahn J, Weiss M, Couldwell W: Loss of Rb expression in an ACTH-secreting pituitary carcinoma. Cancer Lett. 1998, 126: 209-214. 10.1016/S0304-3835(98)00013-5.CrossRefPubMed Hinton D, Hahn J, Weiss M, Couldwell W: Loss of Rb expression in an ACTH-secreting pituitary carcinoma. Cancer Lett. 1998, 126: 209-214. 10.1016/S0304-3835(98)00013-5.CrossRefPubMed
29.
go back to reference Thapar K, Scheithauer B, Kovacs K, Pernicone P, Laws EJ: p53 expression in pituitary adenomas and carcinomas: correlation with invasiveness and tumor growth fractions. Neurosurgery. 1996, 38: 765-770. 10.1097/00006123-199604000-00027. discussion 770–761CrossRefPubMed Thapar K, Scheithauer B, Kovacs K, Pernicone P, Laws EJ: p53 expression in pituitary adenomas and carcinomas: correlation with invasiveness and tumor growth fractions. Neurosurgery. 1996, 38: 765-770. 10.1097/00006123-199604000-00027. discussion 770–761CrossRefPubMed
30.
go back to reference Sonino N, Zielezny M, Fava G, Fallo F, Boscaro M: Risk factors and long-term outcome in pituitary-dependent Cushing's disease. J Clin Endocrinol Metab. 1996, 81: 2647-2652. 10.1210/jc.81.7.2647.PubMed Sonino N, Zielezny M, Fava G, Fallo F, Boscaro M: Risk factors and long-term outcome in pituitary-dependent Cushing's disease. J Clin Endocrinol Metab. 1996, 81: 2647-2652. 10.1210/jc.81.7.2647.PubMed
31.
go back to reference Locatelli M, Vance M, Laws E: Clinical review: the strategy of immediate reoperation for transsphenoidal surgery for Cushing's disease. J Clin Endocrinol Metab. 2005, 90: 5478-5482. 10.1210/jc.2004-2436.CrossRefPubMed Locatelli M, Vance M, Laws E: Clinical review: the strategy of immediate reoperation for transsphenoidal surgery for Cushing's disease. J Clin Endocrinol Metab. 2005, 90: 5478-5482. 10.1210/jc.2004-2436.CrossRefPubMed
32.
go back to reference Nelson D, Meakin J, Dealy Jj, Matson D, Emerson Kj, Thorn G: ACTH-producing tumor of the pituitary gland. N Engl J Med. 1958, 259: 161-164.CrossRefPubMed Nelson D, Meakin J, Dealy Jj, Matson D, Emerson Kj, Thorn G: ACTH-producing tumor of the pituitary gland. N Engl J Med. 1958, 259: 161-164.CrossRefPubMed
33.
go back to reference Assié G, Bahurel H, Coste J, Silvera S, Kujas M, Dugué M, Karray F, Dousset B, Bertherat J, Legmann P, Bertagna X: Corticotroph tumor progression after adrenalectomy in Cushing's Disease: A reappraisal of Nelson's Syndrome. J Clin Endocrinol Metab. 2007, 92: 172-179. 10.1210/jc.2006-1328.CrossRefPubMed Assié G, Bahurel H, Coste J, Silvera S, Kujas M, Dugué M, Karray F, Dousset B, Bertherat J, Legmann P, Bertagna X: Corticotroph tumor progression after adrenalectomy in Cushing's Disease: A reappraisal of Nelson's Syndrome. J Clin Endocrinol Metab. 2007, 92: 172-179. 10.1210/jc.2006-1328.CrossRefPubMed
34.
go back to reference Jenkins P, Trainer P, Plowman P, Shand W, Grossman A, Wass J, Besser G: The long-term outcome after adrenalectomy and prophylactic pituitary radiotherapy in adrenocorticotropin-dependent Cushing's syndrome. J Clin Endocrinol Metab. 1995, 80: 165-171. 10.1210/jc.80.1.165.PubMed Jenkins P, Trainer P, Plowman P, Shand W, Grossman A, Wass J, Besser G: The long-term outcome after adrenalectomy and prophylactic pituitary radiotherapy in adrenocorticotropin-dependent Cushing's syndrome. J Clin Endocrinol Metab. 1995, 80: 165-171. 10.1210/jc.80.1.165.PubMed
35.
go back to reference Thompson S, Hayman A, Ludlam W, Deveney C, Loriaux D, Sheppard B: Improved quality of life after bilateral laparoscopic adrenalectomy for Cushing's disease: a 10-year experience. Ann Surg. 2007, 245: 790-794. 10.1097/01.sla.0000251578.03883.2f.PubMedCentralCrossRefPubMed Thompson S, Hayman A, Ludlam W, Deveney C, Loriaux D, Sheppard B: Improved quality of life after bilateral laparoscopic adrenalectomy for Cushing's disease: a 10-year experience. Ann Surg. 2007, 245: 790-794. 10.1097/01.sla.0000251578.03883.2f.PubMedCentralCrossRefPubMed
36.
go back to reference Evers BM, Ishizuka J, Townsend CM, Thompson JC: The human carcinoid cell line, BON. A model system for the study of carcinoid tumors. Ann N Y Acad Sci. 1994, 733: 393-406. 10.1111/j.1749-6632.1994.tb17289.x.CrossRefPubMed Evers BM, Ishizuka J, Townsend CM, Thompson JC: The human carcinoid cell line, BON. A model system for the study of carcinoid tumors. Ann N Y Acad Sci. 1994, 733: 393-406. 10.1111/j.1749-6632.1994.tb17289.x.CrossRefPubMed
Metadata
Title
ACTH-producing carcinoma of the pituitary with refractory Cushing's Disease and hepatic metastases: a case report and review of the literature
Authors
Scott N Pinchot
Rebecca Sippel
Herbert Chen
Publication date
01-12-2009
Publisher
BioMed Central
Published in
World Journal of Surgical Oncology / Issue 1/2009
Electronic ISSN: 1477-7819
DOI
https://doi.org/10.1186/1477-7819-7-39

Other articles of this Issue 1/2009

World Journal of Surgical Oncology 1/2009 Go to the issue