TY - JOUR
T1 - Highly Aggressive and Radiation-Resistant, "atypical" and Silent Pituitary Corticotrophic Carcinoma
T2 - A Case Report and Review of the Literature
AU - Flores, Laura
AU - Sleightholm, Richard
AU - Neilsen, Beth
AU - Baine, Michael
AU - Drincic, Andjela
AU - Thorell, William
AU - Shonka, Nicole
AU - Oupicky, David
AU - Zhang, Chi
N1 - Publisher Copyright:
© 2019 The Author(s). Published by S. Karger AG, Basel.
PY - 2019/1/1
Y1 - 2019/1/1
N2 - Background: Pituitary tumors typically remain silent unless interaction with nearby structures occurs. Rare subsets of pituitary tumors display aggressive phenotypes: highly mitotic, locally invasive, metastatic, chemotherapy and radiation resistant, etc. Disease progression and response to therapy is ill-defined in these subtypes, and their true prognostic potential is debated. Thus, identifying tumor characteristics with prognostic value and efficacious treatment options remains a challenge in aggressive pituitary tumors. Case Presentation: A 45-year-old female presented with a nonfunctioning corticotropic pituitary macroadenoma with biomarkers suggestive of an "atypical" subtype: Ki-67 of 8-12%, increased mitosis, and locally invasive. Despite resections and radiation, growth continued, eventually affecting her vision. Although histologically ACTH positive, the patient remained clinically asymptomatic. Twelve months later, an episode of Cushing's disease-induced psychosis prompted a PET-CT scan, identifying sites of metastasis. Temozolomide was added to her medical regimen, and her metastatic liver lesions and boney metastases were treated with radiofrequency ablation and stereotactic body radiation therapy, respectively. Systemic treatment resulted in a drop in her ACTH levels, with her most recent scans/labs at 12 months following RFA suggesting remission. Conclusions: This is a unique presentation of a pituitary tumor, displaying characteristics of both clinically silent corticotropic and "atypical" macroadenoma subtypes. Although initially ACTH positive while clinically silent, the patient's disease ultimately recurred metastatically with manifestations of Cushing's disease and psychosis. With the addition of temozolomide to her treatment plan, her primary and metastatic sites have responded favorably to radiation therapy. Thus, the addition of temozolomide may be beneficial in the treatment of aggressive pituitary tumors.
AB - Background: Pituitary tumors typically remain silent unless interaction with nearby structures occurs. Rare subsets of pituitary tumors display aggressive phenotypes: highly mitotic, locally invasive, metastatic, chemotherapy and radiation resistant, etc. Disease progression and response to therapy is ill-defined in these subtypes, and their true prognostic potential is debated. Thus, identifying tumor characteristics with prognostic value and efficacious treatment options remains a challenge in aggressive pituitary tumors. Case Presentation: A 45-year-old female presented with a nonfunctioning corticotropic pituitary macroadenoma with biomarkers suggestive of an "atypical" subtype: Ki-67 of 8-12%, increased mitosis, and locally invasive. Despite resections and radiation, growth continued, eventually affecting her vision. Although histologically ACTH positive, the patient remained clinically asymptomatic. Twelve months later, an episode of Cushing's disease-induced psychosis prompted a PET-CT scan, identifying sites of metastasis. Temozolomide was added to her medical regimen, and her metastatic liver lesions and boney metastases were treated with radiofrequency ablation and stereotactic body radiation therapy, respectively. Systemic treatment resulted in a drop in her ACTH levels, with her most recent scans/labs at 12 months following RFA suggesting remission. Conclusions: This is a unique presentation of a pituitary tumor, displaying characteristics of both clinically silent corticotropic and "atypical" macroadenoma subtypes. Although initially ACTH positive while clinically silent, the patient's disease ultimately recurred metastatically with manifestations of Cushing's disease and psychosis. With the addition of temozolomide to her treatment plan, her primary and metastatic sites have responded favorably to radiation therapy. Thus, the addition of temozolomide may be beneficial in the treatment of aggressive pituitary tumors.
KW - Cushing's disease
KW - Endocrine
KW - Pituitary adenoma
KW - Radiotherapy
KW - Temozolomide
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UR - http://www.scopus.com/inward/citedby.url?scp=85061633199&partnerID=8YFLogxK
U2 - 10.1159/000496019
DO - 10.1159/000496019
M3 - Review article
C2 - 31043952
AN - SCOPUS:85061633199
SN - 1662-6575
VL - 12
SP - 139
EP - 146
JO - Case Reports in Oncology
JF - Case Reports in Oncology
IS - 1
ER -