Sreedhara Padmini, Czerwinski Vincent, Alexander Ethan, Choudhary Chitra
Internal Medicine, University of Kansas School of Medicine, Kansas City, KS 66204, USA.
Division of Endocrinology, Metabolism, Diabetes, Clinical Pharmacology, University of Kansas School of Medicine, Kansas City, KS 66204, USA.
JCEM Case Rep. 2023 Sep 1;1(5):luad097. doi: 10.1210/jcemcr/luad097. eCollection 2023 Sep.
Primary pituitary T-lymphoblastic lymphoma is a rare clinical entity. A 45-year-old woman presented with headache, left-eye blurry vision, diplopia, ophthalmoplegia, and ptosis. Magnetic resonance imaging of the brain showed a sellar mass most likely consistent with a pituitary macroadenoma. Laboratory evaluation disclosed secondary hypothyroidism, secondary adrenal insufficiency, and hyperprolactinemia. The mass was removed by transsphenoidal resection, and subsequent immunophenotyping revealed T-cell lymphoblastic lymphoma. Secondary workup confirmed lymphomatous confinement to the central nervous system. Following resection, the patient's headaches improved, but she experienced persistent visual deficits and palsies of cranial nerves III, IV, and VI. The chemotherapy regimen consisted of high-dose methotrexate, followed by alternating cycles of cyclophosphamide, vincristine sulfate, doxorubicin hydrochloride (Adriamycin), dexamethasone (cyclophosphamide, mesna, vincristine sulfate, doxorubicin hydrochloride, dexamethasone), and methotrexate/cytarabine. Since receiving chemotherapy, there has been an improvement in numbness, ptosis, left orbital pressure, and headaches. This case represents only the eighth example of T-cell primary pituitary lymphoma, and the youngest patient to receive the diagnosis.
原发性垂体T淋巴细胞母细胞淋巴瘤是一种罕见的临床实体。一名45岁女性出现头痛、左眼视力模糊、复视、眼肌麻痹和上睑下垂。脑部磁共振成像显示蝶鞍区有一肿块,很可能符合垂体大腺瘤。实验室检查发现继发性甲状腺功能减退、继发性肾上腺功能不全和高催乳素血症。通过经蝶窦切除术切除肿块,随后的免疫表型分析显示为T细胞淋巴母细胞淋巴瘤。进一步检查证实淋巴瘤局限于中枢神经系统。切除术后,患者的头痛有所改善,但仍存在持续性视力缺陷以及动眼神经、滑车神经和展神经麻痹。化疗方案包括大剂量甲氨蝶呤,随后交替使用环磷酰胺、硫酸长春新碱、盐酸多柔比星(阿霉素)、地塞米松(环磷酰胺、美司钠、硫酸长春新碱、盐酸多柔比星、地塞米松),以及甲氨蝶呤/阿糖胞苷。自接受化疗以来,麻木、上睑下垂、左眼眶压力和头痛均有所改善。该病例是T细胞原发性垂体淋巴瘤的第八个病例,也是确诊时最年轻的患者。