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Rathke's cleft cyst (RCC) is a congenital, benign, epithelial tumor and mainly occurs in sellar region and occasionally in suprasellar region; ectopic RCC is exceedingly rare. We report an uncommon RCC in cerebellopontine angle (CPA) associated with RCC apoplexy and investigated the possible hypothesis of its origin. A 12-year-old female student was admitted to hospital for 3-month history of vertigo, headache, nausea, and vomiting and aggravated for 1 week. Magnetic resonance imaging (MRI) revealed a space-occupying lesion with short T1 and long T2 signals in the left CPA and an intracystic floating nodule with hypointensity on T1- and T2-weighted imaging. The patient underwent the total tumor removal via the retrosigmoid approach with a good recovery. Primary RCC was confirmed by pathology. Follow-up MRI showed no recurrence 3.5 years after craniotomy. Primary RCC can occur in CPA and present special neuroimaging features associated with RCC apoplexy. We presumed that a mimicking mechanism of ectopic craniopharyngioma in CPA leads to the formation in the present case. Microsurgical resection is the optimal strategy for management. Further research and longer follow-up are helpful to better understanding the pathogenesis and development history of RCC in CPA.
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PMID:Primary Rathke's cleft cyst in the cerebellopontine angle associated with apoplexy. 2071 84

Pituitary apoplexy is defined as a sudden loss of blood supply to the pituitary gland, leading to tissue necrosis and hemorrhage. Its clinical symptoms are characterized by sudden onset of headache, nausea, vomiting, ophthalmic symptoms and hormonal dysfunction. A 65-year-old woman presented with left-sided ptosis and blurred vision. These ophthalmic symptoms gradually worsened for one month without headache, visual acuity and field deficit. Neuro-ophthalmic examination revealed left oculomotor nerve palsy. Magnetic resonance imaging (MRI) revealed a round mass lesion in the left cavernous sinus, which was initially suspected as thrombosed cerebral aneurysm or hemorrhagic Rathke's cleft cyst. The mass lesion was finally diagnosed as pituitary apoplexy. The patient underwent trans-sphenoidal surgery and oculomotor nerve palsy improved after the surgery. Early diagnosis and treatment including surgical decompression are crucially important in patients with oculomotor nerve palsy in pituitary apoplexy, but the symptoms of pituitary apoplexy may slowly progress. It should be noted that pituitary apoplexy could be misdiagnosed as cerebral aneurysm or Rathke's cleft cyst.
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PMID:Pituitary apoplexy presenting atypical time course of ophthalmic symptoms. 2257 5

The occurrence of symptomatic pituitary hemorrhage into a Rathke's cleft cyst (RCC) is extremely rare. The author reports an interesting case of intra- and suprasellar RCC presented with features of pituitary apoplexy. This 62-year-old woman suffered acute headache, mental confusion, and partial hypopituitarism. The characteristics of the magnetic resonance imaging seemed most compatible with a hemorrhagic pituitary adenoma. Transsphenoidal drainage of the cyst contents confirmed the diagnosis of hemorrhagic RCC and resolved the symptoms. All published data on this rare clinical entity are extracted and reviewed.
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PMID:A Rathke's Cleft Cyst Presenting with Apoplexy. 2313 32

A prospective study was performed on 80 cases of different types of sellar lesions during the period between January 2007 and December 2010. The binasal approach was used in all patients. Functioning lesions were diagnosed in 59 patients. The most common secretory tumors were prolactinomas (37 patients) followed by adrenocorticotropic hormone producing tumors (11 patients) and growth hormone producing tumors (11 patients). Nonfunctioning lesions included: 14 pituitary adenomas, 2 chordomas, 2 metastatic lesions (ovarian carcinoma and thyroid carcinoma), and 1 case of sarcoidosis, Rathke's cleft cyst, and craniopharyngioma. Seven nonfunctioning cases developed apoplexy. Among the 59 patients with functioning pituitary lesions, postoperative endocrinological control was achieved in 51 of them (86.5%) 3 months postoperatively with normalization of pituitary functions and complete tumor resection as proved by postoperative magnetic resonance imaging. Postoperative visual field defect improvement was achieved in 15 out of 23 patients (65.2%), including the cases with apoplexy, with complete tumor resection. Postoperative discomfort such as headache, vomiting, or dizziness was minimal and hospitalization period was 2 to 3 days. Postoperative leak continued in one patient in whom revision surgical repair was performed 7 days later. The merits, outcome, and limitations of the approach were discussed.
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PMID:Binasal endoscopic approach to the sellar region: experience and outcome analysis of 80 cases. 2390 6

Pituitary infarction or apoplexy with spontaneous cure of the underlying pituitary adenoma is rare. In the paediatric population, we found only a few reported cases. We report a rare case of pituitary infarction progressing to CSF-sella syndrome (or empty sella) in an 11-year-old girl. She presented with sudden onset vomiting, moderate headaches, lethargy, weight loss, and tall stature above her mid-parental height. She did not have any severe symptoms of apoplexy. Her clinical and radiological findings suggested infarction of a pituitary lesion, such as a pituitary adenoma or infarction of a cystic lesion, such as a Rathke's cleft cyst. In this report, we discuss her case of probable infarction of a growth hormone secreting adenoma with a phase of accelerated growth ending up with total anterior pituitary insufficiency. The differential diagnosis and review of the rare cases of paediatric pituitary infarction in the literature will be discussed.
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PMID:A rare case of pituitary infarction leading to spontaneous tumour resolution and CSF-sella syndrome in an 11-year-old girl and a review of the paediatric literature. 2485 15

Hemorrhagic complications of optic pathway diseases are extremely rare causes of acute visual loss associated with dengue fever. In this paper we report a patient presenting with dengue fever and bilateral acute visual loss caused by chiasmal compression due to Rathke's cleft cyst apoplexy. Considering the importance of early diagnosis and treatment to visual recovery, apoplexy of sellar and suprasellar tumors should be considered in the differential diagnosis of patients with acute visual loss and dengue fever.
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PMID:Bilateral acute visual loss from Rathke's cleft cyst apoplexy in a patient with dengue fever. 2549 83

Rathke's cleft cyst (RCC) apoplexy is a rare clinical entity. We report a case of apoplexy of an RCC followed by cerebral infarction. A 67-year-old woman was found lying on the street unconscious. She had fallen from her motorbike. On referral to our hospital she gradually regained consciousness and presented with no neurological deficits. CT showed a round and slightly hyperdense area in the suprasellar region. However, the attending physician did not find this abnormal finding on CT and the patient was discharged the same day. Thirteen days after the first emergency visit she developed left hemiparesis and dysarthria. CT showed a round hypodense area in the suprasellar region. The change of the density in the suprasellar region on CT suggested the pituitary apoplexy. CT also showed a low density area in the territory of the right middle cerebral artery, which indicated the cerebral infarction. MR angiography revealed poor visibility and stenotic changes of right middle cerebral arteries. Transsphenoidal surgery was performed. Histopathological findings confirmed a hemorrhagic RCC. Postoperative MR angiography showed that the visibility and stenosis of right middle cerebral arteries were recovered. This is the rare case of apoplexy of an RCC followed by cerebral infarction.
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PMID:A Case of Apoplexy of Rathke's Cleft Cyst Followed by Cerebral Infarction. 2581 Sep 33


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