Transcript Diapositiva 1

GIANT CELL GRANULOMATOUS HYPOPHYSITIS
SECONDARY TO INTRASELLAR RUPTURE OF
RATHKE’S CLEFT CYST – A CASE REPORT.
M. Bisceglia (1), D. Catapano (2), G. Giannatempo
(3), V.A. D’Angelo (2).
Department of Pathology – Division of Anatomic
Pathology (1), Department of Neurosciences Division of Neurosurgery (2), and Department of
Imaging – Division of Radiology (3), IRCCS ”Casa
Sollievo della Sofferenza” Hospital, San Giovanni
Rotondo, Italy.
Introduction.
• Rathke ‘s pouch closes in early embryonic life, but its
apical extremity persists into postnatal life as a cleft
between the pars distalis and the pars nervosa of the
pituitary.
• Rathke’s cleft is a frequent incidental finding in the normal
pituitary in post-mortem specimens. It is lined by cuboidal
or columnar epithelium, often ciliated in places and which
may include mucous goblet cells.
• Rathke’s cleft cysts are tumor-like lesions which may
derive from distension of clefts by gelatinous material, but
may remain asymptomatic.
• They are typically intrasellar, but dumb-bell intra- and
supra-sellar, and entirely suprasellar examples are
described.
• The epithelial lining of these cysts is similar to that of
Rathke’s cleft, but squamous metaplasia is not
uncommon.
• Large cysts may become symptomatic either due to
compression of the pituitary gland, optic chiasm, or
hypothalamus, or to inflammation of the surrounding
pituitary tissue with or without rupture of the cyst
capsule.
• Hypophysitis associated with Rathke’s cleft cyst is rarely
reported.
• Both non-granulomatous lymphocytic hypophysitis (1,2)
and giant cell foreign-body type granulomatous
hypophysitis (3) have been described, triggered by
rupture of the cyst capsule.
We describe an additional case of
giant cell granulomatous hypophysitis,
caused by a ruptured Rathke’s cleft cyst.
Case Report.
• A 53 years-old woman presented with acute
diabetes insipidus and visual impairment.
• At imaging (MRI), there was an intense contrastenhancing intra-supra-sellar mass with a large
cystic component compressing the optic chiasm
and the pituitary stalk.
Fig. 1. A and B, MRI T1-weighted image. A sellar-suprasellar, partly cystic, lesion is
visible. The sella is enlarged and the sphenoidal bone eroded. C and D, T1weighted image with contrast, showing ring enhancement around the cyst. The
pituitary stalk is thickened and enhanced.
A
C
B
D
• Laboratory chemical analyses were normal.
• The patient underwent trans-sphenoidal surgery.
• Intraoperatively a firm tumor capsule was detected
surrounding a cyst cavity.
• Inspissated fluid was aspirated and the tumor
capsule witht the surrounding inflammed tissue were
totally removed.
Light microscopy revealed giant cell
granulomatous hypophysitis.
Fragments of Rathke’s cyst wall partly lined by
simple columnar and squamous metaplastic
epithelium were found.
Cyst and inflammatory
tissue of cyst wall.
Cytokeratins, including CK5/6
Squamous cell nests
Squamous cell nests from which Rathke’s cyst
is widley believed tooriginate.
CK-AE1-AE3
CK-AE1-AE3
Discussion.
• All the above findings strongly indicated that the ruptured
cyst had caused the giant cell granulomatous
hypophysitis.
• Mucins produced by cells lining the cyst are believed to
be the direct stimulus for the giant cell response (4).
• Giant cell granulomatous hypophysitis accounts for less
than 1% of all pituitary disorders.
• Two forms of giant cell granulomatous hypophysitis are
distinguished, idiopathic (5) and secondary, the latter
having varying etiologies, including autoimmune
disorders, systemic infectious and non-infectious
granulomatous diseases (e.g. syphilis, tuberculosis,
sarcoidosis, histiocytoses, including Langerhan’s cell
histiocytosis) and ruptured Rathke’s cleft cyst.
• Histologically different is another type of hypophysitis
known as xanthomatous hypophysitis, in which anterior
pituitary is heavily infiltrated by xanthomatous
histiocytes with macrophagic immunoprofile (6).
• Todate only 7 cases of granulomatous hypophysitis
secondary to ruptured Rathke’s cleft cysts are recorded
in the world literature.
• All have occurred in women (3).
• All but one patient were not pregnant (3).
We have reported herein an additional such example,
also in a non-pregnant female.
Conclusion.
• Although a definite preoperative diagnosis is
almost impossible, particularly the challenging
distinction from a non-functioning pituitary
adenoma with cystic degeneration, the diagnosis
should be suspected in the presence of a sellar
mass with a cystic area.
• Anticipation of the diagnosis preoperatively or
intraoperatively is important for the correct
management of this lesion.
References
1. Nishikawa T, Takahashi JA, Shimatsu A, Hashimoto N.
Hypophysitis caused by Rathke's cleft cyst. Case report.
Neurol Med Chir (Tokyo). 2007;47:136-9.
2. Schittenhelm J, Beschorner R, Psaras T, et al. Rathke's
cleft cyst rupture as potential initial event of a secondary
perifocal lymphocytic hypophysitis: proposal of an
unusual pathogenetic event and review of the literature.
Neurosurg Rev. 2008;31:157-63.
3. Sonnet E, Roudaut N, Meriot P, Besson G, Kerlan V.
Hypophysitis associated with a ruptured Rathke's cleft
cyst in a woman, during pregnancy. J Endocrinol Invest.
2006;29:353-7.
4.
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6.
Roncaroli F, Bacci A, Frank G, Calbucci F.
Granulomatous hypophysitis caused by a ruptured
intrasellar Rathke's cleft cyst: report of a case and
review of the literature. Neurosurgery. 1998;43:146-9
Bhansali A, Velayutham P, Radotra BD, Pathak A.
Idiopathic granulomatous hypophysitis presenting as
non-functioning pituitary adenoma: description of six
cases and review of literature. Br J Neurosurg.
2004;18:489-94.
Folkerth RD, Price DL Jr, Schwartz M, Black PM, De
Girolami U. Xanthomatous hypophysitis. Am J Surg
Pathol. 1998;22:736-41.