Hypopituitarism is a rare presentation of
Burkitt's lymphoma (BL). The purpose of this report is to present a case of BL presenting with
panhypopituitarism and to review other case reports of
lymphoma presenting with pituitary dysfunction to highlight the distinguishing features of these cases from other benign aetiologies of pituitary dysfunction such as non-functioning
pituitary adenomas. We reviewed a total of 11 cases of
lymphoma presenting with pituitary dysfunction published from 1998 to 2013 including the present case. The demographics, clinical presentations, laboratory features, radiological findings, histological diagnosis, treatment administered and outcomes were described. Of the total number of patients, 45.5% of the cases had
diffuse large B-cell lymphoma while 27.3% had BL. Anterior pituitary dysfunction was more common than posterior pituitary dysfunction at presentation. The other common associated presenting symptoms were
painful ophthalmoplegia,
cranial nerve palsies and constitutional symptoms. Hypothalamic-pituitary abnormalities were often demonstrated radiologically to be associated with cavernous sinus and/or stalk involvement. All patients who completed immunochemotherapy responded haematologically. Pituitary dysfunction also improved in most cases although the recovery tended to be partial. In conclusion, a high index of suspicion of underlying
malignancy, such as
lymphoma, should be present in patients presenting with acute pituitary dysfunction associated with
painful ophthalmoplegia, rapidly evolving neurological features, radiological features atypical of a
pituitary adenoma and constitutional symptoms. An early diagnosis is essential as prompt initiation of definitive
therapy will induce disease remission and recovery of pituitary dysfunction.
LEARNING POINTS:
Hypopituitarism may be the presenting symptom of
lymphoma in the absence of associated overt symptoms or signs of a haematological
malignancy resulting in delay in diagnosis and institution of treatment.Pituitary dysfunction due to tumour infiltration has a greater tendency to involve the posterior pituitary and infundibulum resulting in
diabetes insipidus and
hyperprolactinaemia compared with a non-functioning
pituitary adenoma.The common associated symptoms of
hypopituitarism due to
lymphoma infiltration of the hypothalamic-pituitary system include
painful ophthalmoplegia,
cranial nerve palsies and constitutional symptoms.Radiological abnormalities of the hypothalamic-pituitary region are usually present and often associated with cavernous sinus or stalk involvement.With early institution of definitive treatment, both haematological response and improvement of pituitary dysfunction are expected although the reversal of
hypopituitarism tends to be partial and delayed.A high index of suspicion of underlying
malignancy such as
lymphoma should be present in patients presenting with acute pituitary dysfunction associated with
painful ophthalmoplegia, radiological features atypical of
pituitary adenomas and constitutional symptoms to enable early diagnosis and prompt initiation of definitive
therapy.