The Diagnosis and Management of Lymphocytic Hypophysitis

Sherry L Iuliano; Edward R Laws

Disclosures

Expert Rev Endocrinol Metab. 2011;6(6):777-783. 

In This Article

Case Examples

In a 3-year period, we encountered seven cases initially diagnosed as lymphocytic hypophysitis (Table 1). We have included two cases that were initially diagnosed as hypopyhsitis to demonstrate the difficulties of accurate preoperative diagnosis in some cases.

Case One

This 31-year-old man presented with the spontaneous onset of diabetes insipidus prior to being evaluated. Further evaluation revealed partial hypopituitarism, with a decreased testosterone level. His visual examination was normal. Laboratory studies showed negative markers for germ cell tumor and sarcoidosis. The MRI demonstrated a 9 × 8 × 8 cm sellar lesion with suprasellar extension and thickening of the pituitary stalk. He was treated with trans-sphenoidal surgery with a subtotal removal of the lesion. The histological diagnosis was lymphocytic hypophysitis. Immunostains were positive for CD3 and CD20. Immunostains for acid-fast bacilli, PLAP and OCT3/4 were negative. The patient remains asymptomatic, maintained on pituitary replacement therapy including DDAVP.

Case Two

This 64-year-old woman had a history of Sjögren's syndrome, and presented with headache, mildly elevated prolactin, hypopituitarism and diabetes insipidus. The headache persisted despite treatment with steroids. Her visual examination was normal.

The MRI revealed an intrasellar mass 1.3 cm in maximum diameter, with thickening of the pituitary stalk. She was treated with trans-sphenoidal surgery and partial removal of the lesion. The histological diagnosis was lymphocytic hypophysitis. Immunostains for CD3 and CD20 confirmed the presence of T cell and B cell lymphocytes. She improved following surgery, with relief of her headache. Subsequently she has been treated for a granulomatous mycobacterial pulmonary infection.

Case Three

This 49-year-old man presented with chronic headache, present since childhood, chronic progressive fatigue and decreased libido. He was diagnosed with Klinefelter's syndrome and hypopituitarism, and was treated with prednisone, synthroid and androgel. He had no visual symptoms or signs, and did not have diabetes insipidus. The MRI showed a sellar lesion with enlargement and heterogeneous involvement of the pituitary gland and a thickened pituitary stalk. Initially, this was diagnosed as a pituitary adenoma. He was treated with trans-sphenoidal surgery and partial removal of the lesion. The histopathology revealed lymphocytic hypophysitis. Immunostains for CD3, CD20 and CD138 confirmed the presence of T cells, B cells and plasma cells. There was significant fibrosis in the specimen. The patient has done relatively well, except for persistent chronic headaches, and is being treated for persistent hypopituitarism.

Case Four

This 36-year-old woman developed postpartum amenorrhea and galactorrhea, persisting for a year after childbirth. Symptoms included headache, fatigue, dizziness and sensitivity to light. She had a moderately elevated prolactin level and evidence of partial hypopituitarism without diabetes insipidus. The MRI demonstrated a large intrasellar partially cystic mass extending into the cavernous sinuses with thickening of the pituitary stalk (Figure 1). She was treated with trans-sphenoidal surgery and partial removal of the lesion. The histopathology was positive for lymphocytic hypophysitis. Markers for T cells and B cells were positive; CD3 and CD20 and synaptophysin were positive in scattered cells. Immunostains for OCT3/4 and PLAP were negative. The patient remains asymptomatic on pituitary replacement therapy.

Case Five

This 40-year-old woman presented with amenorrhea, galactorrhea, hyperprolactinemia, headache, fatigue and visual blurring. She was found to have pan-hypopituitarism and diabetes insipidus and was treated with pituitary replacement therapy including DDAVP. The MRI revealed a large cystic lesion involving the entire pituitary fossa extending superiorly along the infundibulum. The patient underwent a trans-sphenoidal operation for radical removal of the lesion. The histopathology was positive for pituitary granuloma with necrotic features, staining negative for acid-fast bacilli, periodic acid-Schiff and MSS gene. Diabetes insipidus persisted postoperatively; the headaches and visual symptoms improved.

Case Six

This 24-year-old man presented 6 years previously with diabetes insipidus, fatigue and hypopituitarism. The MRI demonstrated a sellar and suprasellar mass involving the pituitary stalk. The presumptive diagnosis of lymphocytic hypophysitis was made. He was treated with corticosteroids, with an initial favorable response, but the lesion recurred and continued to enlarge. He was subsequently treated with methotrexate, again with a transient response. Progressive recurrence and enlargement of the suprasellar mass prompted a trans-sphenoidal biopsy. The histopathology revealed a germinoma, with positive staining for OCT3/4, NANOG and PLAP. He was subsequently referred for chemotherapy and radiation therapy, and had an excellent response. He remains on full pituitary replacement therapy and DDAVP.

Case Seven

This is a 61-year-old woman with a history of hypothyroidism controlled with levothyroxine. She represented with fatigue and severe headache. A post-contrast MRI demonstrates a homogenous enhancing enlarged pituitary gland with thickening of the pituitary stalk. The sella is mildly expanded, and there is compression of the optic chiasm. There was also invasion of the right cavernous sinus (Figure 2). The patient underwent a trans-sphenoidal operation for the resection of the mass. The postoperative course was uneventful and the patient's headaches resolved on postoperative day one. A diagnosis of lymphocytic hypophysitis was confirmed on pathology. A mononuclear inflammatory infiltrate was identified consisting of mature T and B lymphocytes, as confirmed by positive staining for CD3 and CD20.

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