Seymour Matthew, Robertson Thomas, Papacostas Jason, Morris Kirk, Gillespie Jennifer, Norris Debra, Duncan Emma L
Department of Endocrinology and Diabetes, Royal Brisbane and Women's Hospital, Brisbane, Australia.
Faculty of Medicine, The University of Queensland, Brisbane, Australia.
Endocrinol Diabetes Metab Case Rep. 2021 May 1;2021. doi: 10.1530/EDM-20-0100.
A 34-year-old woman presented 18 months post-partum with blurred vision, polyuria, amenorrhoea, headache and general malaise. Comprehensive clinical examination showed left superior temporal visual loss only. Initial investigations revealed panhypopituitarism and MRI demonstrated a sellar mass involving the infundibulum and hypothalamus. Lymphocytic hypophysitis was suspected and high dose glucocorticoids were commenced along with desmopressin and thyroxine. However, her vision rapidly deteriorated. At surgical biopsy, an irresectable grey amorphous mass involving the optic chiasm was identified. Histopathology was initially reported as granulomatous hypophysitis. Despite the ongoing treatment with glucocorticoids, her vision worsened to light detection only. Histopathological review revised the diagnosis to partially treated lymphoma. A PET scan demonstrated avid uptake in the pituitary gland in addition to splenic involvement, lymphadenopathy above and below the diaphragm, and a bone lesion. Excisional node biopsy of an impalpable infraclavicular lymph node confirmed nodular lymphocyte-predominant Hodgkin lymphoma. Hyper-CVAD chemotherapy was commenced, along with rituximab; fluid-balance management during chemotherapy (with its requisite large fluid volumes) was extremely complex given her diabetes insipidus. The patient is now in clinical remission. Panhypopituitarism persists; however, her vision has recovered sufficiently for reading large print and driving. To the best of our knowledge, this is the first reported case of Hodgkin lymphoma presenting initially as hypopituitarism.
Lymphoma involving the pituitary is exceedingly rare and, to the best of our knowledge, this is the first reported case of nodular lymphocyte-predominant Hodgkin lymphoma presenting as hypopituitarism. There are myriad causes of a sellar mass and this case highlights the importance of reconsidering the diagnosis when patients fail to respond as expected to appropriate therapeutic intervention. This case highlights the difficulties associated with managing panhypopituitary patients receiving chemotherapy, particularly when this involves large volumes of i.v. hydration fluid.
一名34岁女性在产后18个月出现视力模糊、多尿、闭经、头痛和全身不适。全面的临床检查仅显示左上颞侧视力丧失。初步检查发现全垂体功能减退，MRI显示鞍区有一肿块，累及漏斗和下丘脑。怀疑为淋巴细胞性垂体炎，开始使用高剂量糖皮质激素以及去氨加压素和甲状腺素治疗。然而，她的视力迅速恶化。手术活检时，发现一个无法切除的灰白色无定形肿块累及视交叉。组织病理学最初报告为肉芽肿性垂体炎。尽管持续使用糖皮质激素治疗，她的视力仍恶化至仅能感知光。组织病理学复查将诊断修订为部分治疗的淋巴瘤。PET扫描显示垂体有活跃摄取，此外还有脾脏受累、膈上下淋巴结肿大以及一处骨病变。对一个不可触及的锁骨下淋巴结进行切除活检，证实为结节性淋巴细胞为主型霍奇金淋巴瘤。开始进行Hyper-CVAD化疗并联合利妥昔单抗；鉴于她患有尿崩症，化疗期间（需要大量液体）的液体平衡管理极其复杂。患者目前处于临床缓解期。全垂体功能减退仍然存在；然而，她的视力已恢复到足以阅读大字和驾驶的程度。据我们所知，这是首例最初表现为垂体功能减退的霍奇金淋巴瘤报告病例。
累及垂体的淋巴瘤极为罕见，据我们所知，这是首例报告的以垂体功能减退表现的结节性淋巴细胞为主型霍奇金淋巴瘤。鞍区肿块有多种病因，该病例凸显了在患者对适当治疗干预未按预期反应时重新考虑诊断的重要性。该病例凸显了管理接受化疗的全垂体功能减退患者所面临的困难，尤其是当化疗涉及大量静脉补液时。
