Diapositiva 1

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GIANT CELL GRANULOMATOUS HYPOPHYSITIS SECONDARY
TO INTRASELLAR RUPTURE OF RATHKES CLEFT CYST A
CASE REPORT. M. Bisceglia (1), D. Catapano (2),
G. Giannatempo (3), V.A. DAngelo
(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.
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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.
• Rathkes 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.
• Rathkes 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 Rathkes cleft, but squamous
  metaplasia is not uncommon.

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• 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 Rathkes 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 Rathkes cleft cyst.

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Case Report.

• A 53 years-old woman presented with acute
  diabetes insipidus and visual impairment.
• At imaging (MRI), there was an intense
  contrast-enhancing intra-supra-sellar mass with a
  large cystic component compressing the optic
  chiasm and the pituitary stalk.

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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, T1-weighted image with
contrast, showing ring enhancement around the
cyst. The pituitary stalk is thickened and
enhanced.
A
B
D
C
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• 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 Rathkes cyst wall partly lined
  by simple columnar and squamous metaplastic
  epithelium were found.

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CK-AE1-AE3
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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 Langerhans cell histiocytosis) and
  ruptured Rathkes cleft cyst.

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• 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 Rathkes 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.

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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.

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References

1. Nishikawa T, Takahashi JA, Shimatsu A, Hashimoto
   N. Hypophysitis caused by Rathke's cleft
   cyst. Case report. Neurol Med Chir (Tokyo).
   200747136-9.
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. 200831157-63.
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. 200629353-7.

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1. 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. 199843146-9
2. 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. 200418489-94.
3. Folkerth RD, Price DL Jr, Schwartz M, Black PM,
   De Girolami U. Xanthomatous hypophysitis. Am J
   Surg Pathol. 199822736-41.