Pituitary and systemic autoimmunity in a case of intrasellar germinoma

Germinomas arising in the sella turcica are difficult to differentiate from autoimmune hypophysitis because of similar clinical and pathological features. This differentiation, nevertheless, is critical for patient care due to different treatments of the two diseases. We report the case of an 11-yea...

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Main Authors: Gutenberg, Angelika, Bell, Jennifer J., Lupi, Isabella, Tzou, Shey-Cherng, Landek-Salgado, Melissa A., Kimura, Hiroaki, Su, Jack, Karaviti, Lefkothea P., Salvatori, Roberto, Caturegli, Patrizio
Format: Online
Language:English
Published: Springer US 2009
Online Access:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3291822/
id pubmed-3291822
recordtype oai_dc
spelling pubmed-32918222012-03-21 Pituitary and systemic autoimmunity in a case of intrasellar germinoma Gutenberg, Angelika Bell, Jennifer J. Lupi, Isabella Tzou, Shey-Cherng Landek-Salgado, Melissa A. Kimura, Hiroaki Su, Jack Karaviti, Lefkothea P. Salvatori, Roberto Caturegli, Patrizio Article Germinomas arising in the sella turcica are difficult to differentiate from autoimmune hypophysitis because of similar clinical and pathological features. This differentiation, nevertheless, is critical for patient care due to different treatments of the two diseases. We report the case of an 11-year-old girl who presented with diabetes insipidus and growth retardation, and was found to have an intra- and supra-sellar mass. Initial examination of the pituitary biopsy showed diffuse lymphocytic infiltration of the adenohypophysis and absent placental alkaline phosphatase expression, leading to a diagnosis of hypophysitis and glucocorticoid treatment. Because of the lack of clinical and radiological response, the pituitary specimen was re-examined, revealing this time the presence of scattered c-kit and Oct4 positive germinoma cells. The revised diagnosis prompted the initiation of radiotherapy, which induced disappearance of the pituitary mass. Immunological studies showed that the patient’s serum recognized antigens expressed by the patient’s own germinoma cells, as well as pituitary antigens like growth hormone and systemic antigens like the Sjögren syndrome antigen B and alpha-enolase. The study first reports the presence of pituitary and systemic antibodies in a patient with intrasellar germinoma, and reminds us that diffuse lymphocytic infiltration of the pituitary gland and pituitary antibodies does not always indicate a diagnosis of autoimmune hypophysitis. Springer US 2009-05-26 2011-12 /pmc/articles/PMC3291822/ /pubmed/19466616 http://dx.doi.org/10.1007/s11102-009-0187-x Text en © Springer Science+Business Media, LLC 2009
repository_type Open Access Journal
institution_category Foreign Institution
institution US National Center for Biotechnology Information
building NCBI PubMed
collection Online Access
language English
format Online
author Gutenberg, Angelika
Bell, Jennifer J.
Lupi, Isabella
Tzou, Shey-Cherng
Landek-Salgado, Melissa A.
Kimura, Hiroaki
Su, Jack
Karaviti, Lefkothea P.
Salvatori, Roberto
Caturegli, Patrizio
spellingShingle Gutenberg, Angelika
Bell, Jennifer J.
Lupi, Isabella
Tzou, Shey-Cherng
Landek-Salgado, Melissa A.
Kimura, Hiroaki
Su, Jack
Karaviti, Lefkothea P.
Salvatori, Roberto
Caturegli, Patrizio
Pituitary and systemic autoimmunity in a case of intrasellar germinoma
author_facet Gutenberg, Angelika
Bell, Jennifer J.
Lupi, Isabella
Tzou, Shey-Cherng
Landek-Salgado, Melissa A.
Kimura, Hiroaki
Su, Jack
Karaviti, Lefkothea P.
Salvatori, Roberto
Caturegli, Patrizio
author_sort Gutenberg, Angelika
title Pituitary and systemic autoimmunity in a case of intrasellar germinoma
title_short Pituitary and systemic autoimmunity in a case of intrasellar germinoma
title_full Pituitary and systemic autoimmunity in a case of intrasellar germinoma
title_fullStr Pituitary and systemic autoimmunity in a case of intrasellar germinoma
title_full_unstemmed Pituitary and systemic autoimmunity in a case of intrasellar germinoma
title_sort pituitary and systemic autoimmunity in a case of intrasellar germinoma
description Germinomas arising in the sella turcica are difficult to differentiate from autoimmune hypophysitis because of similar clinical and pathological features. This differentiation, nevertheless, is critical for patient care due to different treatments of the two diseases. We report the case of an 11-year-old girl who presented with diabetes insipidus and growth retardation, and was found to have an intra- and supra-sellar mass. Initial examination of the pituitary biopsy showed diffuse lymphocytic infiltration of the adenohypophysis and absent placental alkaline phosphatase expression, leading to a diagnosis of hypophysitis and glucocorticoid treatment. Because of the lack of clinical and radiological response, the pituitary specimen was re-examined, revealing this time the presence of scattered c-kit and Oct4 positive germinoma cells. The revised diagnosis prompted the initiation of radiotherapy, which induced disappearance of the pituitary mass. Immunological studies showed that the patient’s serum recognized antigens expressed by the patient’s own germinoma cells, as well as pituitary antigens like growth hormone and systemic antigens like the Sjögren syndrome antigen B and alpha-enolase. The study first reports the presence of pituitary and systemic antibodies in a patient with intrasellar germinoma, and reminds us that diffuse lymphocytic infiltration of the pituitary gland and pituitary antibodies does not always indicate a diagnosis of autoimmune hypophysitis.
publisher Springer US
publishDate 2009
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3291822/
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