Rathke's cleft cyst with xanthogranulomatous change: Case report
Department of Neurosurgery, Kitasato University School of Medicine, 1-15-1 Kitasato, Minami-ku, Sagamihara, Kanagawa, Japan. Neurologia medico-chirurgica
(Impact Factor: 0.72).
01/2011; 51(10):740-2. DOI: 10.2176/nmc.51.740
A 58-year-old man presented with a rare case of Rathke's cleft cyst (RCC) manifesting as headache and visual disturbance. Goldman's perimeter showed he had bitemporal hemianopia. Magnetic resonance imaging revealed a solid intrasellar lesion. He underwent surgery via a right pterional approach. The lesion was red, solid, and fibrous. Histological examination revealed a few columnar epithelial cells with poor cilia in a xanthogranulomatous lesion. Only 13 cases of RCC with granulomatous change have been diagnosed by pathological examination, including 4 xanthogranulomatous cases. Xanthogranuloma of the sellar region is relatively rare. The etiology is still unknown. We speculate that xanthogranulomas of the sellar region may be caused by reaction to the presence of RCC.
Available from: Kosaku Amano
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ABSTRACT: Xanthogranuloma of the sellar region is a rare clinical observation. Although it was included in the World Health Organization (WHO) brain tumor classification in 2000, its clinical features and pathogenesis remain uncertain. We report herein seven cases of xanthogranuloma of the sellar region who underwent transsphenoidal surgery at Tokyo Women's Medical University between 2005 and 2011, and discuss the clinical characteristics of this tumor. Six out of these 7 patients (86 %) presented with endocrinological dysfunction, six (86 %) had headaches, five (71 %) had visual field disturbances, and three (43 %) had diplopia including two with unilateral ptosis. Pathological findings revealed cholesterol clefts, hemosiderin deposits, chronic inflammatory infiltrates, multinucleated giant cells, macrophages, and fibrous proliferation. Of these seven cases, components of Rathke's cleft cyst were observed for six. Xanthogranuloma in the sellar region is suspected of being a terminal stage resulting from a secondary reaction caused by repeated inflammatory change, hemorrhage, and degeneration of a Rathke's cleft cyst.
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ABSTRACT: This article reviews recent literature on sellar region masses that most closely mimic non-secretory pituitary adenomas: hypophysitis, pituicytoma, spindle cell oncocytoma, and granular cell tumor of neurohypophysis. Even today, these 4 entities often cannot be confidently distinguished from each other clinically or by preoperative neuroimaging features. Thus, they often come to biopsy/surgical resection and require tissue confirmation of diagnosis. Causes of secondary and primary hypophysitis will be discussed, including two newly-described types, IgG4 plasma cell hypophysitis and hypophysitis caused by anti-CTLA4 antibody therapy for cancer. For the neoplastic conditions, emphasis will be placed on literature that has emerged since these entities were first codified in the 2007 World Health Organization fascicle (38, 39, 119). The finding that immunohistochemical staining for thyroid transcription factor-1 (TTF-1) is shared by pituicytoma, spindle cell oncocytoma, and granular cell tumor of neurohypophysis suggests common lineage and explains why histological overlap can be encountered. We incorporate our own experiences over the last 30 years from two referral institutions with specialty practices in pituitary region masses.
Available from: Paul Dimitri
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ABSTRACT: Xanthogranulomatous hypophysitis (XGH) is a very rare form of pituitary hypophysitis that may present both clinically and radiologically as a neoplastic lesion. It may either be primary with an autoimmune aetiology and can occur in isolation or as a part of autoimmune systemic disease or secondary as a reactive degenerative response to an epithelial lesion (e.g. craniopharyngioma (CP), Rathke's cleft cyst, germinoma and pituitary adenomas) or as a part of a multiorgan systemic involvement such as tuberculosis, sarcoidosis or granulomatosis. It may also present with a variation of symptoms in children and adults. Our case series compares the paediatric and adult presentations of XGH and the differential diagnoses considered in one child and two adult patients, highlighting the wide spectrum of this condition. Endocrine investigations suggested panhypopituitarism in all three patients and imaging revealed a suprasellar mass compressing the optic chiasm suggestive of CP or Rathke's cleft cyst in one patient and non-functioning pituitary macroadenoma in two patients. Magnetic resonance imaging (MRI) demonstrated mixed signal intensities on T1- and T2-weighted sequences. Following endoscopic transsphenoidal surgery, histological analysis revealed necrotic material with a xanthogranulomatous reaction confirming XGH in two patients and a necrobiotic granulomatous chronic inflammatory infiltrate with neutrophils in one patient, which is not typical of current descriptions of this disorder. This case series describes the wide spectrum of XGH disease that is yet to be defined. Mixed signal intensities on T1- and T2-weighted MRI sequences may indicate XGH and diagnosis is confirmed by histology. Histological variation may indicate an underlying systemic process.
XGH is a rare form of pituitary hypophysitis with a wide clinical and histological spectrum and can mimic a neoplastic lesion.XGH primarily presents with growth arrest in children and pubertal arrest in adolescents. In adults, the presentation may vary.A combination of hypopituitarism and mixed signal intensity lesion on MRI is suggestive of XGH and should be considered in the differential diagnosis of sellar lesions.Radical surgery is the treatment of choice and carries an excellent prognosis with no recurrence.
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