Endocrine Abstracts

A 31-year-old female patient without previous history of pituitary diseases, at 16 weeks of 4th spontaneous pregnancy complained about severe headache; diplopia and right ptosis developed shortly, nausea and vomiting appeared. Brain MRI showed the mass lesion 30×19×14 mm (frontal×vertical×sagittal) with supra-laterosellar extension, deforming and displacing optic chiasm; signs of pansinusitis, pathological contents of the right mastoid cells, vascular malformation in the right temporal lobe. Hormonal investigation showed low concentrations of TSH, free T4, free?3, cortisol and ACTH so hypopituitarism was diagnosed, and appropriate replacement therapy started. According to clinical symptoms, patient required higher doses of glucocorticosteroids to stop signs of adrenal insufficiency. She also received the antibiotic therapy and repeated surgical interventions due to purulent pansinusitis and secondary meningoencephalitis with the formation of an abscess of the left frontal lobe. However, the condition worsened despite the cavities and abscess sanitation: headaches persisted, a progressive decrease in vision (bilateral amaurosis) and bilateral ptosis were observed. Also poliuria up to 6–9 l/daily without hypernatremia or a decrease in the specific gravity of urine were revealed. An antidiuresis and hyponatremia 124 mmol/l were detected on the background of single-dose desmopressin 30 μg. Hyponatremia was self-recovered, however, low-normal values of sodium (130–135 mmol/l), potassium (3.6–4.3 mmol/l) and glucose (3.5–4.1 mmol/l) remained in dynamics. The MRI control every 2 weeks showed the rapid unexpectable growth of pituitary lesion with both cavernous sinus invasion: 47×21×18 mm – 60×24×21 mm (frontal×vertical×sagittal×). The diagnosis of pituitary adenoma was doubtful, the presence of hypophysitis or other sellar mass’s etiology were assumed and pituitary biopsy was performed to clarify the diagnosis. The extranodal T/NK cell nasal lymphoma was diagnosed according to histological and immunohistochemical studies (Ki-67 up to 90%). Cesarean section was performed at 23 weeks of gestation for life indications to start chemotherapy. This clinical observation shows the difficulties of differential diagnosis of rare extranodal T/NK-cell lymphoma of the nasal type with CNS and pituitary lesion in a pregnant women with hypopituitarism and optic nerve atrophy. Such rude progression of pituitary lesion with predominant increase of frontal size is not characteristic for pituitary tumors and/or hypophysitis so rare and malignant disorders should be suspected.