Rapid decrease of a pituitary mass with gonadotrophic and thyrotrophic insufficiency the case for lymphocytic hypophysitis?

Background:Lymphocytic hypophysitis is an autoimmune condition that commonly presents in women of childbearing age as hypopituitarism and a sellar mass.Case report:A 66-year-old woman presented with anterior pituitary dysfunction. Computed tomography imaging revealed a small hypodensity that was not felt to be the cause of the pituitary dysfunction. Eight years later, her vision rapidly deteriorated and MRI showed a pituitary mass lesion causing optic chiasm compression. Histological examination of the partially resected gland revealed evidence of lymphocytic hypophysitis.Conclusion:Our patient is an example of the variable presentation and course of lymphocytic hypophysitis. Such a long latent period between the initial presentation of adenohypophysial hypofunction and optic chiasm compression due to an enlarging pituitary mass has not been reported.

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SAT-243 Lymphocytic Hypophysitis Mimicking Pituitary Apoplexy in a 27-Year-Old Woman

Journal of the Endocrine Society ◽

10.1210/jendso/bvaa046.799 ◽

2020 ◽

Vol 4 (Supplement_1) ◽

Author(s):

Danielle C Brooks ◽

Richard S Haber

Keyword(s):

Pituitary Gland ◽

Pituitary Apoplexy ◽

Optic Chiasm ◽

Lymphocytic Hypophysitis ◽

Surgical Pathology ◽

Bright Spot ◽

Inflammatory Disorder ◽

Pituitary Mass ◽

Ct Angiogram ◽

Wide Range

Abstract Introduction: Lymphocytic hypophysitis is an uncommon inflammatory disorder of the pituitary gland. Peripartum women are most often affected, but it can be seen in a wide range of patients. Patients can present with pituitary dysfunction or mass effect symptoms, making it difficult to differentiate from a pituitary adenoma. Pituitary apoplexy is an acute syndrome due to hemorrhage into the pituitary gland causing headache, visual deficits, and hypoadrenalism. We present a case of a woman with a pituitary mass presenting with symptoms mimicking pituitary apoplexy, in whom surgical pathology later revealed lymphocytic hypophysitis without evidence of hemorrhage. Clinical Case: A 27-year-old non-pregnant female presented with severe headaches, nausea, vomiting, and diplopia for four days. A presumed pituitary macroadenoma was diagnosed two years previously during a workup for irregular menses. An emergency evaluation with a CT angiogram of the head showed enhancement at the superior aspect of and surrounding the pituitary gland. MRI of the pituitary revealed an enhancing 17 x 17 mm sellar and suprasellar mass compressing the optic chiasm, without evidence of the normal pituitary posterior bright spot. Visual field testing was normal. She was referred for transsphenoidal resection of the pituitary mass due to persistent headaches, optic chiasm impingement, and suspicion of pituitary apoplexy. Intraoperatively, there was no evidence of hemorrhage. Central diabetes insipidus and hypothyroidism developed in the post-operative period. She was discharged on hydrocortisone replacement therapy (20 mg in the morning, 10 mg in the afternoon). An outpatient random cortisol level of 1.2 mcg/dL (reference: 6.7 - 22.6 mcg/dL) while off hydrocortisone for twenty-four hours confirmed hypocortisolism. Surgical pathology showed moderate lymphocytic infiltrate and focal germinal centers in the adenohypophysis consistent with lymphocytic hypophysitis. Conclusion: Although lymphocytic hypophysitis is well-described in the literature, its association with symptoms suggesting pituitary apoplexy has been reported only rarely. This atypical case shows that lymphocytic hypophysitis can present with acute symptoms mimicking pituitary apoplexy. The case highlights the difficulties in recognizing lymphocytic hypophysitis prior to surgery and emphasizes the need to consider the diagnosis in patients presenting with pituitary masses and/or symptoms of pituitary apoplexy.

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Lymphocytic Hypophysitis Successfully Treated with Stereotactic Radiosurgery: Case Report and Review of the Literature

Journal of Neurological Surgery Part A Central European Neurosurgery ◽

10.1055/s-0037-1604079 ◽

2017 ◽

Vol 79 (01) ◽

pp. 077-085 ◽

Cited By ~ 5

Author(s):

Vojislav Bogosavljevic ◽

Selcuk Peker ◽

Mirjana Doknic ◽

Dragana Miljic ◽

Marko Stojanovic ◽

...

Keyword(s):

Pituitary Gland ◽

Stereotactic Radiosurgery ◽

Artery Occlusion ◽

Lymphocytic Hypophysitis ◽

Review Of The Literature ◽

Pituitary Mass ◽

Inflammatory Infiltration ◽

Aggressive Form ◽

Secondary Hypothyroidism ◽

Short Period

AbstractLymphocytic hypophysitis (LH) is an autoimmune inflammatory infiltration of the pituitary gland, usually with a benign evolution. In rare circumstances the inflammatory process may extend beyond the pituitary and infiltrate the surrounding structures. We present a 42-year-old woman affected by an aggressive form of LH with extension to the cavernous sinus causing internal carotid artery occlusion and right sixth cranial nerve palsy. Prednisone therapy caused severe iatrogenic Cushing's syndrome, and the patient underwent transsphenoidal decompression. The histopathology report was consistent with LH. The patient was symptom free for a short period with reappearance of severe headache, diplopia, and hearing loss (middle ear inflammation) 3 months after surgery. Corticosteroids were reintroduced with the addition of azathioprine, but there was no regression of the pituitary mass. The patient was referred for stereotactic radiosurgery (SRS) using Gamma Knife (15 Gy to the margin). After 26 months, azathioprine was stopped, and the dose of prednisone was gradually tapered to 7.5 mg/day. Sellar magnetic resonance imaging showed regression of the pituitary mass. After follow-up for > 3 years after SRS, there was no clinical or radiologic evidence of the disease, but carotid arteries remained occluded. The patient developed secondary hypothyroidism and hypogonadism as consequences of SRS. An aggressive form of LH extending beyond the pituitary gland infiltrating surrounding structures is described. It was successfully treated with SRS after failure of transsphenoidal surgery and combined immunosuppressive therapy (prednisone, azathioprine). The review of the literature presents timely information concerning treatment with azathioprine and SRS of patients with an aggressive form of LH.

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Lymphocytic hypophysitis

Journal of Neurosurgery ◽

10.3171/jns.1994.80.5.0925 ◽

1994 ◽

Vol 80 (5) ◽

pp. 925-927 ◽

Cited By ~ 15

Author(s):

Ramachandra G. Naik ◽

Ariachery Ammini ◽

Pankaj Shah ◽

Chitra Sarkar ◽

Veer Singh Mehta ◽

...

Keyword(s):

Rectus Muscle ◽

Symptomatic Treatment ◽

Lymphocytic Hypophysitis ◽

Mass Lesion ◽

Pituitary Mass ◽

Content Type ◽

Lateral Rectus Muscle ◽

Peripartum Period ◽

The Right ◽

Glandular Enlargement

✓ A case of lymphocytic hypophysitis is described in a patient presenting with panhypopituitarism 8 years after her last childbirth. The patient developed headache, vomiting, and diplopia (due to palsy of the right lateral rectus muscle) 7 months after delivery of her last baby. The diplopia disappeared after a few days with symptomatic treatment, and the headache and vomiting decreased in intensity with analgesic therapy. Eight years later the patient developed symptoms suggestive of hypoadrenalism, hypothyroidism, and amenorrhea. Investigations revealed panhypopituitarism with a pituitary mass lesion. Repeat evaluation 1 year later demonstrated no change in the size of the pituitary gland. The patient underwent transsphenoidal surgery with a provisional diagnosis of pituitary adenoma. Histological examination of the resected gland revealed evidence of lymphocytic hypophysitis. Symptoms suggestive of a pituitary mass lesion were noted during the peripartum period, but features of hypopituitarism developed much later. Such a long latent period has not been reported before. This report also highlights the fact that glandular enlargement may persist for many years after the onset of lymphocytic hypophysitis.

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Parasellar T2 dark sign on magnetic resonance imaging to differentiate lymphocytic hypophysitis from pituitary adenoma

Surgical Neurology International ◽

10.25259/sni_338_2020 ◽

2020 ◽

Vol 11 ◽

pp. 239

Author(s):

Amit Agarwal ◽

Girish Bathla

Keyword(s):

Magnetic Resonance Imaging ◽

Magnetic Resonance ◽

Imaging Modality ◽

Inflammatory Cells ◽

Endoscopic Biopsy ◽

Lymphocytic Hypophysitis ◽

Tumor Board ◽

Resonance Imaging ◽

Pituitary Mass ◽

Dark Signal

Background: Pituitary adenomas are the most common sellar masses in adults with magnetic resonance imaging (MRI) being the imaging modality of choice. Inflammatory pituitary lesions such as lymphocytic hypophysitis (LH) can mimic pituitary macroadenoma on imaging and are often misdiagnosed as such. Although the imaging appearance on most of the sequences on MRI has similar findings, LH has a characteristic dark signal on T2 images (called dark T2 sign) which can be very helpful to reliably differentiate the two conditions. Case Description: A 68-year-old woman diagnosed with a “pituitary mass” on the MR study done at an outside facility was referred to our neurosurgery department. The case was discussed at our multidisciplinary tumor board, where the possibility of an inflammatory condition mimicking tumor was considered, given the very dark signal on T2-weighted sequences. Transsphenoidal endoscopic biopsy revealed a firm rubbery mass, which histopathology demonstrated fibrous connective tissue with inflammatory cells consistent with LH. Conclusion: Dark T2 signal on MR imaging can be very helpful in demarcating inflammatory pituitary conditions like LH from pituitary macroadenomas.

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MON-240 Histologically Proven Lymphocytic Hypophysitis with Marked Improvement on Glucocorticoid Therapy

Journal of the Endocrine Society ◽

10.1210/jendso/bvaa046.1230 ◽

2020 ◽

Vol 4 (Supplement_1) ◽

Author(s):

Priyanka Mathias ◽

Vafa Tabatabaie

Keyword(s):

Soft Tissue ◽

Hormone Replacement ◽

Fibrous Tissue ◽

Lymphocytic Hypophysitis ◽

Glucocorticoid Therapy ◽

Laboratory Evaluation ◽

High Dose ◽

Decompression Surgery ◽

Pituitary Mass ◽

Suprasellar Region

Abstract Background: Lymphocytic hypophysitis (LH) is a rare autoimmune disorder characterized by lymphocytic infiltration of the pituitary gland. The disease predominantly affects women, with >50% of cases presenting during pregnancy or postpartum.1 LH is often associated with other autoimmune conditions, primarily thyroiditis, and adrenalitis.2 Clinical case: A 27-year-old female presented with secondary amenorrhea for eight months. Workup revealed hyperprolactinemia (PRL 65 ng/mL) and a heterogenous pituitary mass measuring 3.3 cm in the largest dimension. Cabergoline was initiated for a presumed prolactinoma. Laboratory evaluation was significant for hypogonadotropic hypogonadism (estradiol <50 pg/mL, progesterone <1 ng/mL, FSH 2.9 mIU/mL, LH 0.45 mIU/mL) despite normalization in prolactin. She was also found to have Hashimoto’s thyroiditis (FT4 0.7 ng/dL, TSH 8.2 uU/mL with positive TPO antibodies) and was started on levothyroxine. Repeat imaging demonstrated a 2.4 cm heterogenous expanding sellar mass with soft tissue extension to the dorsum sella concerning for a meningioma. Visual field testing was intact without evidence of chiasmal compression. She underwent trans-sphenoidal pituitary decompression surgery which was terminated prematurely due to the presence of extensive fibrous tissue in the sella. Pathology was consistent with LH. Immunohistochemical staining was positive for lymphocytic markers CD3 and CD20, confirming marked infiltration of inflammatory B-cells and T-cells. Her postoperative course was notable for panhypopituitarism. In view of the pathological findings of LH, she was started on a high dose of 40mg of prednisone daily. Within two months, sellar magnetic resonance imaging revealed a homogenous normal-appearing pituitary with a reduction in soft tissue mass in the sellar and suprasellar region. Oral contraceptive therapy was initiated for sex hormone replacement with the resumption of menses. Prednisone was gradually tapered to 5mg/day, and she was subsequently transitioned to maintenance hydrocortisone for central adrenal insufficiency. Discussion: LH is a rare chronic inflammatory disease that should be considered in the differential diagnosis of a non-secreting pituitary mass, especially if occurring in young women presenting during pregnancy or postpartum. The condition is associated with preferential destruction of corticotroph and thyrotroph cells.3 Appropriate management remains controversial. High dose glucocorticoid therapy, to which our patient responded to dramatically, has been shown to be beneficial in reducing mass effect. Optimal treatment involves surgical resection of the pituitary mass to decompress surrounding structures.3 References: 1. JCEM, Volume 100, Issue 10, October 2015, Pages 3841-3849 2. JCEM, Volume 80, Issue 8, August 1995, Pages 2302-2311 3. Horm Res, 2007;68 Supplement 5:145-50

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The Ultrastructure of Lymphocytic Hypophysitis

Proceedings, annual meeting, Electron Microscopy Society of America ◽

10.1017/s0424820100098824 ◽

1981 ◽

Vol 39 ◽

pp. 466-467

Author(s):

S.L. Asa ◽

K. Kovacs ◽

J. M. Bilbao ◽

R. G. Josse ◽

K. Kreines

Keyword(s):

Electron Microscopy ◽

Plasma Cells ◽

Secretory Granules ◽

Sella Turcica ◽

Uranyl Acetate ◽

Lymphocytic Hypophysitis ◽

Pituitary Cells ◽

Transmission Electron ◽

Ultrathin Sections ◽

Mass Lesions

Seven cases of lymphocytic hypophysitis in women have been reported previously in association with various degrees of hypopituitarism. We report two pregnant patients who presented with mass lesions of the sella turcica, clinically mimicking pituitary adenoma. However, pathologic examination revealed extensive infiltration of the anterior pituitary by lymphocytes and plasma cells with destruction of the gland. To our knowledge, the ultrastructural features of lymphocytic hypophysitis have not been studied so far.For transmission electron microscopy, tissue from surgical specimens was fixed in glutaraldehyde, postfixed in OsO4, dehydrated and embedded in epoxy-resin. Ultrathin sections were stained with uranyl acetate and lead citrate and examined with a Philips 300 electron microscope.Electron microscopy revealed adenohypophysial cells of all types exhibiting varying degrees of injury. In the areas of most dense inflammatory cell infiltration pituitary cells contained large lysosomal bodies fusing with secretory granules (Fig. 1), as well as increased numbers of swollen mitochondria, indicating oncocytic transformation (Fig. 2).

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