A rare case of isolated IgG4-related hypophysitis with Rathke’s cleft cyst presenting as panhypopituitarism
We report a rare case of biopsy-proven isolated immunoglobulin G4 (IgG4)-related hypophysitis and Rathke’s cleft cyst (RCC) presenting as panhypopituitarism. A 54-year-old Caucasian female presented with symptoms of slurred speech, altered mental status, polyuria and polydipsia and was found to have...
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| Format: | Article |
| Language: | English |
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Bioscientifica
2023-04-01
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| Series: | Endocrinology, Diabetes & Metabolism Case Reports |
| Online Access: | https://edm.bioscientifica.com/view/journals/edm/2023/1/EDM22-0359.xml |
| _version_ | 1797842773584379904 |
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| author | Hiba Z Hashmi Dinkar Rupakula Rekha Magar H Brent Clark Amir Moheet |
| author_facet | Hiba Z Hashmi Dinkar Rupakula Rekha Magar H Brent Clark Amir Moheet |
| author_sort | Hiba Z Hashmi |
| collection | DOAJ |
| description | We report a rare case of biopsy-proven isolated immunoglobulin G4 (IgG4)-related hypophysitis and Rathke’s cleft cyst (RCC) presenting as panhypopituitarism. A 54-year-old Caucasian female presented with symptoms of slurred speech, altered mental status, polyuria and polydipsia and was found to have panhypopituitarism. Brain MRI showed a suprasellar mass with suspected intralesional hemorrhage. She underwent trans-sphenoidal resection due to MRI evidence of compression of the optic chiasm and left optic nerve. Preoperatively, she was started on hydrocortisone, levothyroxine and desmopressin. Histopathology demonstrated a RCC with adjacent lymphoplasmacytic hypophysitis with numerous IgG4- immunoreactive plasma cells. Hydrocortisone was stopped at 10 months after confirming hypothalamic-pituitary-adrenal (HPA)-axis recovery and desmopressin was stopped at 1 year. There was recurrence of a cystic mass at 1 year follow-up. Over 4 years of follow-up, she continued to require levothyroxine, and the mass remained stable in size. In order to begin to understand how this case’s unique histopathological presentation influences clinical presentation, pituitary imaging and prognosis, we present an accompanying literature review. |
| first_indexed | 2024-04-09T16:53:41Z |
| format | Article |
| id | doaj.art-661e9953485a4ed486460ae28fa90af5 |
| institution | Directory Open Access Journal |
| issn | 2052-0573 |
| language | English |
| last_indexed | 2024-04-09T16:53:41Z |
| publishDate | 2023-04-01 |
| publisher | Bioscientifica |
| record_format | Article |
| series | Endocrinology, Diabetes & Metabolism Case Reports |
| spelling | doaj.art-661e9953485a4ed486460ae28fa90af52023-04-21T07:47:35ZengBioscientificaEndocrinology, Diabetes & Metabolism Case Reports2052-05732023-04-01111510.1530/EDM-22-0359A rare case of isolated IgG4-related hypophysitis with Rathke’s cleft cyst presenting as panhypopituitarismHiba Z Hashmi0Dinkar Rupakula1Rekha Magar2H Brent Clark3Amir Moheet4Division of Endocrinology, Diabetes and Metabolism, University of Minnesota, Minneapolis, Minnesota, USADivision of Endocrinology, Diabetes and Metabolism, University of Arizona, Tucson, Arizona, USADivision of Endocrinology, Diabetes and Metabolism, University of Minnesota, Minneapolis, Minnesota, USADepartment of Laboratory Medicine and Pathology, University of Minnesota, Minneapolis, Minnesota, USADivision of Endocrinology, Diabetes and Metabolism, University of Minnesota, Minneapolis, Minnesota, USAWe report a rare case of biopsy-proven isolated immunoglobulin G4 (IgG4)-related hypophysitis and Rathke’s cleft cyst (RCC) presenting as panhypopituitarism. A 54-year-old Caucasian female presented with symptoms of slurred speech, altered mental status, polyuria and polydipsia and was found to have panhypopituitarism. Brain MRI showed a suprasellar mass with suspected intralesional hemorrhage. She underwent trans-sphenoidal resection due to MRI evidence of compression of the optic chiasm and left optic nerve. Preoperatively, she was started on hydrocortisone, levothyroxine and desmopressin. Histopathology demonstrated a RCC with adjacent lymphoplasmacytic hypophysitis with numerous IgG4- immunoreactive plasma cells. Hydrocortisone was stopped at 10 months after confirming hypothalamic-pituitary-adrenal (HPA)-axis recovery and desmopressin was stopped at 1 year. There was recurrence of a cystic mass at 1 year follow-up. Over 4 years of follow-up, she continued to require levothyroxine, and the mass remained stable in size. In order to begin to understand how this case’s unique histopathological presentation influences clinical presentation, pituitary imaging and prognosis, we present an accompanying literature review.https://edm.bioscientifica.com/view/journals/edm/2023/1/EDM22-0359.xml |
| spellingShingle | Hiba Z Hashmi Dinkar Rupakula Rekha Magar H Brent Clark Amir Moheet A rare case of isolated IgG4-related hypophysitis with Rathke’s cleft cyst presenting as panhypopituitarism Endocrinology, Diabetes & Metabolism Case Reports |
| title | A rare case of isolated IgG4-related hypophysitis with Rathke’s cleft cyst presenting as panhypopituitarism |
| title_full | A rare case of isolated IgG4-related hypophysitis with Rathke’s cleft cyst presenting as panhypopituitarism |
| title_fullStr | A rare case of isolated IgG4-related hypophysitis with Rathke’s cleft cyst presenting as panhypopituitarism |
| title_full_unstemmed | A rare case of isolated IgG4-related hypophysitis with Rathke’s cleft cyst presenting as panhypopituitarism |
| title_short | A rare case of isolated IgG4-related hypophysitis with Rathke’s cleft cyst presenting as panhypopituitarism |
| title_sort | rare case of isolated igg4 related hypophysitis with rathke s cleft cyst presenting as panhypopituitarism |
| url | https://edm.bioscientifica.com/view/journals/edm/2023/1/EDM22-0359.xml |
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