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Neuroendocrine Center Bulletin; A newsletter with information regarding pituitary tumors and neuroendocrine diseases and conditions. Information on acromegaly, Cushing's disease or syndrome, prolactinoma, chromophobe or nonfunctioning pituitary adenoma, and thyrotroph adenomas.

The Mass General Neuroendocrine and Pituitary Tumor Clinical Center Bulletin shares the latest information for health care professionals about clinical and research topics in neuroendocrine conditions.

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A Pituitary Mass Presenting with Visual Loss During Pregnancy

Pituitary Home

Nicholas A Tritos
by Brooke Swearingen, MD

NEPTCC Newsletter Volume 22, Issue 1, Fall 2015 [PDF version]

A 30-year-old nulliparous woman presented to MGH at 34 weeks gestation with headaches and visual loss. She was referred by her ophthalmologist, who documented a bitemporal field defect with a dense left central scotoma. The visual loss was progressive, and had worsened in the week prior to presentation. She had developed headaches three months prior to admission. The pregnancy was otherwise uncomplicated. Menses were normal before her pregnancy, and she had no history of galactorrhea, polyuria/polydipsia or other endocrine symptoms. Medical history was notable for a deep venous thrombosis three years ago, and she was being treated with low molecular weight heparin during the pregnancy. She had a history of primary hypothyroidism and had been maintained on 100 μg of thyroxine daily for the past five years. She was otherwise healthy.

On exam, she did not appear acromegalic or Cushingoid. There was a dense left central and superior temporal defect, and a subtle right superior temporal defect. Acuity was 20/30 on the right and 20/800 on the left.

Endocrine testing revealed a 5am cortisol low at 2.3 ug/ dl, prolactin 33 ng/ml (normal <20), TSH 0.09 uIU/ml (normal 0.4-5.0), T4 7.8 ug/dl (normal 4.5-10.9), free T4 low at 0.7 ng/ml (normal 0.9-1.8). She was begun on steroid replacement with hydrocortisone and her thyroxine dose was increased.

A noncontrast (because of the pregnancy) MRI was performed. This demonstrated a 2.3 cm mass arising from the sella, extending into the suprasellar cistern, with significant chiasm compression. There was T1 hyperintensity within the central portion of the mass suggestive of hemorrhage. (Figure 1)

The presumptive diagnosis was pituitary adenoma with hemorrhage, perhaps related to her heparin requirement, leading to visual loss. She was seen by the high risk obstetrics service and the low molecular weight heparin was discontinued prior to planned surgery. Transsphenoidal exploration was performed after discontinuation of anticoagulation. At operation, a densely fibrotic mass was found and biopsied. The T1 hyperintensity seen on her preoperative MRI proved to be high-protein fluid within a Rathke’s cleft cyst. Frozen section pathologic analysis showed fibrotic anterior pituitary with a dense inflammatory infiltrate composed predominantly of lymphocytes, consistent with lymphocytic hypophysitis.

Postoperatively, her vision initially improved to 20/25 on the right and 20/50 on the left, with a smaller central scotoma. She was maintained on 20 mg of prednisone daily. Transient diabetes insipidus developed, which resolved spontaneously after a few days. At 2.5 weeks post biopsy, (35.5 weeks gestation), she reported worsening vision on the left despite steroid treatment, and return of the resolved visual defect on the right. The prednisone dose was increased to 60 mg daily. She was admitted for induction of labor, which was unsuccessful at 48 hours, and she therefore underwent cesarean section, with delivery of a healthy boy. Postpartum, a contrast-enhanced MRI was obtained (Figure 2) which showed the large densely enhancing sellar mass with persistent chiasm compression. The high dose prednisone was continued.

On post delivery day one, her vision had begun to improve, with acuity improved to 20/30 on the left and 20/20 on the right, and a smaller scotoma. The prednisone was tapered to 40 mg daily. By two weeks post delivery, formal neuroophthalmologic exam documented 20/15 vision bilaterally with full visual fields. A follow-up MRI was obtained which showed dramatic shrinkage in the inflammatory mass, without chiasmal compression. (Figure 3)

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Figure 1

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Figure 2

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Figure 3

Discussion

The differential diagnosis and management of pituitary disorders during pregnancy is complex. Possibilities in this case included a previously unrecognized nonfunctioning pituitary adenoma, with hemorrhage related to the anticoagulation, leading to rapid expansion and visual loss, enlargement of a previously unrecognized macro prolactinoma, or an inflammatory mass. A prolactinoma was considered unlikely, given the history of normal menses, absence of galactorrhea, and minimally elevated prolactin level on hormone testing, and we suspected hemorrhage into a previously existing tumor. Given the rapid progression and severity of presentation, a biopsy and possible resection was felt to be indicated. Pathology showed a dense inflammatory infiltrate composed predominantly of CD3 positive lymphocytes with scattered histiocytes and eosinophils, consistent with hypophysitis.

Hypophysitis can occur in a number of variants, including adenohypophysitis or infundibulohypophysitis. It is rare, with an incidence of one in 9 million person-years. It is most common in peri-partum women, but has been found in men, non-pregnant women and the elderly. A number of pathologic subtypes exist including lymphocytic, granulomatous, xanthomatous and IgG4. A subtype associated with the use of ipilimumab in the treatment of metastatic melanoma has been recently described. Symptoms typically include headaches and visual loss from the enlarging mass, as well as endocrine symptoms from hypopituitarism. As an autoimmune disorder, it is often associated with the presence of anti-pituitary antibodies and other autoimmune disease. Imaging typically demonstrates homogeneous enhancement in an enlarged pituitary, without a focal mass lesion. Indications for therapy vary with the severity of presentation; classic hypopituitarism without mass effect can be treated with replacement only. With symptomatic mass effect, definitive diagnosis and possible transsphenoidal debulking may be necessary, followed by immunosuppressive treatment with high-dose glucocorticoids and/or azathioprine. In our case, there was initial improvement with high-dose prednisone after biopsy, but dramatic improvement occurred only after delivery, with resolution of mass effect and improvement in vision.


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Physicians' Pituitary Information Service - Physicians with questions may contact Dr Biller or Dr Klibanski at 617.726.3965 or 1.888.429.6863 or via e-mail at - pituitary.info@partners.org :: Research Studies

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