Two Cases of anti-PIT-1 Hypophysitis Exhibited as a Form of Paraneoplastic Syndrome not Associated With Thymoma.

Abstract

Anti-pituitary-specific transcription factor 1 (PIT-1) hypophysitis (anti-PIT-1 antibody syndrome) is a thymoma-associated autoimmune disease characterized by acquired growth hormone (GH), prolactin (PRL), and thyrotropin (TSH) deficiencies due to autoimmunity against PIT-1. Ectopic expression of PIT-1 in the thymoma plays a causal role in development of the disease. Here, we report 2 cases of anti-PIT-1 hypophysitis exhibiting as a form of paraneoplastic syndrome with conditions other than thymoma. A 79-year-old woman (case 1) and an 86-year-old man (case 2) were referred with a suspicion of anti-PIT-1 hypophysitis because of acquired GH, PRL, and TSH deficiencies. Case 1 was complicated by diffuse large B-cell lymphoma (DLBCL) of the bladder and case 2 was diagnosed with malignancy with multiple metastases of unknown origin. Because circulating anti-PIT-1 antibody was detected, both patients were diagnosed with anti-PIT-1 hypophysitis. Circulating PIT-1-reactive T cells were detected in case 1 via enzyme-linked immunospot (ELISPOT) assay. Interestingly, the PIT-1 protein was ectopically expressed in the DLBCL cells of case 1, whereas DLBCL tissues derived from patients without anti-PIT-1 hypophysitis were negative for PIT-1. In case 2, the materials were not available because of best supportive care was under way. These data show that anti-PIT-1 hypophysitis is associated not only with thymoma but also with other malignancies. Additionally, the ectopic expression of PIT-1 in the DLBCL tissues and presence of PIT-1-reactive T cells suggested that the underlying mechanisms were similar to those observed in thymoma. Thus, anti-PIT-1 hypophysitis is defined as a form of paraneoplastic syndrome.

• Authors: Keitaro Kanie, Genzo Iguchi, Megumi Inuzuka, Kentaro Sakaki, Hironori Bando, Shin Urai, Hiroki Shichi, Yasunori Fujita, Ryusaku Matsumoto, Kentaro Suda, Masaaki Yamamoto, Hidenori Fukuoka, Takao Taniguchi, Wataru Ogawa, Yutaka Takahashi
• Journal: Journal of the Endocrine Society (J Endocr Soc) Vol. 5 Issue 3 Pg. bvaa194 (Mar 01 2021) ISSN: 2472-1972 [Electronic] United States
• PMID: 33506159 (Publication Type: Case Reports)
• Copyright: © The Author(s) 2020. Published by Oxford University Press on behalf of the Endocrine Society.