Lymphocytic hypophysitis [Neurosurgery Wiki]

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====== Lymphocytic hypophysitis ====== 

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[[Lymphocytic adenohypohysitis]] and [[lymphocytic infundibulo neurohypophysitis]] are rare [[autoimmune]] mediated diseases of the anterior and [[posterior pituitary]], respectively. 
Lymphocytic [[hypophysitis]] is a relatively rare [[autoimmune disease]] defined by lymphocytic infiltration to the [[pituitary]].

It is closely related to other inflammatory conditions in the region, namely orbital pseudotumor and [[Tolosa Hunt syndrome]].

====Epidemiology====

Lymphocytic hypophysitis is seen most frequently in women (strong female predilection with a M:F of ~ 9:1), and often in the post-partum period or in the third trimester of pregnancy.

====Clinical presentation====

Clinical presentation is varied depends of part of the pituitary affected and on the size of the lesion. Lymphocytic hypophysitis can thus be classified as:

anterior pituitary: [[Lymphocytic adenohypohysitis]] (LAH)

most common

mimics a pituitary neuroendocrine tumor

endocrine hormone deficits are common

mass effects on adjacent structures (e.g. optic chiasm)

posterior pituitary: lymphocytic Infundibular neurohypophysitis (LINH)

rare

diabetes insipidus

both anterior and posterior pituitary: lymphocytic Infudibular panhypophysitis (LIPH)

Occasionally lymphocytic hypophysitis may be associated with auto-immune conditions such as:

autoimmune thyroiditis

pernicious anaemia

====Pathology====

It is characterised by infiltration of the pituitary stalk with lymphocytes (as the name would suggest).

Radiographic features

CT

Coronal CT and multiplanar reconstructions are able to visualise the pituitary region reasonably well. Lymphocytic hypophysitis appears as an enhancing soft tissue mass involving the pituitary and extending into the suprasellar region.

MRI

MRI, as is the case with other pituitary lesions, is the best modality for assessing this condition which appears as a pituitary region mass.

T1
affected region is isointense with slight signal heterogeneity
normal posterior pituitary bright spot may be absent 
T1 C+ (Gd)
can variably enhance, usually homogeneously 
dural enhancement may be present 
infundibulum may be thickened 

T2
[[Hypointensity]] in parasellar region can be present and may be useful in differentiating from a pituitary neuroendocrine tumor 

==== Treatment and prognosis ====


Lymphocytic hypophysitis is usually self limiting and spontaneous recovery can occur. Corticosteroids are sometimes given and deficient hormones can be replaced 8

====Differential diagnosis====

The differential diagnosis is essentially that of other pituitary region masses. Considerations include:

pituitary neuroendocrine tumor

craniopharyngioma (papillary type)

suprasellar meningioma

pituitary metastasis

Granulomatous hypophysitis (idiopathic of secondary to systemic illness e.g sarcoidosis, [[syphilis]], and tuberculosis).


====Case reports====
===2017===
Pekic S, Bogosavljevic V, Peker S, Doknic M, Miljic D, Stojanovic M,
Skender-Gazibara M, Gacic EM, Popovic V, Petakov M. Lymphocytic Hypophysitis
Successfully Treated with Stereotactic Radiosurgery: Case Report and Review of
the Literature. J Neurol Surg A Cent Eur Neurosurg. 2017 Jul 25. doi:
10.1055/s-0037-1604079. [Epub ahead of print] PubMed PMID: 28743133
((Pekic S, Bogosavljevic V, Peker S, Doknic M, Miljic D, Stojanovic M,
Skender-Gazibara M, Gacic EM, Popovic V, Petakov M. Lymphocytic Hypophysitis
Successfully Treated with Stereotactic Radiosurgery: Case Report and Review of
the Literature. J Neurol Surg A Cent Eur Neurosurg. 2017 Jul 25. doi:
10.1055/s-0037-1604079. [Epub ahead of print] PubMed PMID: 28743133.
)).