Abstract
BackgroundPrimary central nervous system lymphoma (PCNSL) is a rare but well-known extra-nodal lymphoma, which usually presents with non-Hodgkin B-cell lymphomas. PCNSL is generally located around the ventricle and is often detected as multiple lesions. It is rarely seen in the area of the hypothalamus.Case presentationWe report the case of a 48-year-old Caucasian woman with progressive short-term memory deterioration, headache, mental confusion, diabetes insipidus (DI) and hypopituitarism. Early findings were suggestive of a pituitary apoplexy. The results of tests performed during the initial admission at the tertiary health center revealed hypernatremia, hypopituitarism and DI. Intravenous hydrocortisone treatment was initiated for the secondary adrenal insufficiency, and 75 mcg/day of levothyroxine was started for the secondary hypothyroidism on the fourth day following hydrocortisone treatment. A daily dose of 120 mg desmopressin melt tablet was started twice a day for polyuria/polydipsia after the patient’s volume status was balanced. A brain magnetic resonance imaging scan revealed a mass lesion in the hypothalamic area, which was surrounded by marked edema. Anti-edema treatment was initially started considering the suggestion by our neurosurgery team. The patient’s clinical and laboratory findings improved after the initiation of the anti-edema therapy. Afterwards, a biopsy was performed, which diagnosed a malignant diffuse large B-cell lymphoma. Subsequently, intravenous high-dose methotrexate-based therapy was started; however, after the second cycle of chemotherapy, the patient died due to sepsis.ConclusionIn this report, we present a case of hypopituitarism that developed due to the mass effect of hypothalamic lymphoma with clinical findings of pituitary apoplexy. Intracranial masses may cause obvious endocrinological findings related to hypopituitarism, while vague findings may also be observed due to partial failure. Therefore, it is important to perform a comprehensive endocrinological examination at the time of diagnosis in patients with intracranial masses.
Highlights
Primary central nervous system lymphoma (PCNSL) is a rare but well-known extra-nodal lymphoma, which usually presents with non-Hodgkin B-cell lymphomas
In this report, we present a case of hypopituitarism that developed due to the mass effect of hypothalamic lymphoma with clinical findings of pituitary apoplexy
Intracranial masses may cause obvious endocrinological findings related to hypopituitarism, while vague findings may be observed due to partial failure
Summary
PCNSL complicated by hypopituitarism is a rare condition; it attracts attention due to the lack of specific clinical findings and the possibility of misdiagnosis due to pituitary adenoma or many other causes. Since the differential diagnosis of primary hypothalamic lesions is extensive, this case highlights the difficulty in making a diagnosis. In patients with sellar or parasellar masses accompanied by pituitary dysfunction, the presence of a CNS lymphoma should be evaluated. As described in our case report, an urgent biopsy may be life-saving when laboratory and imaging methods are not sufficient for a definitive diagnosis. Appropriate timing of treatment is vital in hypopituitarism
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