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1.  Coexisting Rathke Cleft Cyst and Pituitary Adenoma Presenting with Pituitary Apoplexy: Report of Two Cases 
Skull Base Reports  2011;1(2):99-104.
The authors report two cases of coexisting Rathke cleft cyst (RCC) and pituitary macroadenoma. Both patients presented at the university hospital with pituitary apoplexy symptoms of sudden-onset headache while undergoing treatment with Coumadin (warfarin). Magnetic resonance imaging was consistent with a pituitary adenoma in one case and RCC in the other. Intraoperative findings and pathological work-up identified RCC along with adenomatous tissue displaying hemorrhagic pituitary adenoma in one and hemorrhagic RCC in the other. Clinical symptoms of pituitary apoplexy were present in both cases, making pituitary and RCC apoplexy clinically indistinguishable. RCC and concomitant pituitary adenoma are a rare intraoperative finding that must be considered as a differential diagnosis in patients with symptoms of pituitary adenoma apoplexy.
PMCID: PMC3743596  PMID: 23984210
Rathke cleft cyst; pituitary adenoma; pituitary apoplexy; anticoagulation
2.  Spontaneous involution of a Rathke's cleft cyst in a patient with normal cortisol secretion 
Rathke's cleft cyst (RCC) is a lesion derived from maldeveloped remnants of a dorsal invagination of the stomodeal ectoderm (Rathke's pouch). Although commonly found on autopsy, these lesions rarely become symptomatic during an individual's lifetime. When symptoms occur, they most often include headaches, visual disturbances, and/or varying degrees of hypopituitarism. The natural history remains unclear. The current standard of care includes surgical drainage and biopsy of the cyst wall or surgical resection of symptomatic lesions; however, debate exists regarding the management of asymptomatic lesions. Rare reports of spontaneously resolving RCC can be found in the literature.
Case Description:
We describe the management of a case of RCC in an 8½-year-old girl who presented with a history of growth deceleration since 4 years of age and near-growth arrest since 7 years of age. Her parents also described a tendency towards polydipsia since she was 2 years of age. Endocrine evaluation revealed growth hormone deficiency, central hypothyroidism, and diabetes insipidus, but normal cortisol secretion. The patient experienced no symptoms characteristic of intracranial or sellar mass. Neurologic examination was normal; formal ophthalmologic examination revealed no deficits. The magnetic resonance imaging (MRI) was consistent with RCC. The patient was treated medically for her hormone deficiencies. Over the next year, her sellar mass spontaneously involuted. Twenty-seven months after her initial presentation to our clinic, imaging revealed no sellar mass; the patient remained on hormone replacement therapy.
Although the natural history of RCC requires further study, observation with serial MRI may be an acceptable management strategy in the absence of debilitating symptoms.
PMCID: PMC3347493  PMID: 22574251
Pituitary cyst; Rathke's cleft cyst; spontaneous involution; transsphenoidal surgery
3.  Symptomatic Rathke's cleft cyst with a co-existing pituitary tumor; Brief review of the literature 
Asian Journal of Neurosurgery  2013;8(4):183-187.
Pituitary adenomas and Rathke's cleft cysts (RCCs) share a common embryological origin. Occasionally, these two lesions can present within the same patient. We present a case of a 39-year-old male who was found to have a large sellar lesion after complaints of persistent headaches and horizontal nystagmus. Surgical resection revealed components of a RCC co-existing with a pituitary adenoma. A brief review of the literature was performed revealing 38 cases of co-existing Rathke's cleft cysts and pituitary adenomas. Among the cases, the most common symptoms included headache and visual changes. Rathke's cleft cysts and pituitary adenomas are rarely found to co-exist, despite having common embryological origins. We review the existing literature, discuss the common embryology to these two lesions and describe a unique case from our institution of a co-existing Rathke's cleft cyst and pituitary adenoma.
PMCID: PMC3912769  PMID: 24551002
Adenoma; development; neoplasm; pituitary; Rathke's cleft cyst; sella; tumor
4.  A Case of Ectopic Rathke's Cleft Cyst in the Prepontine Cistern 
A Rathke's cleft cyst (RCC) is a benign pituitary cyst derived from the remnant of Rathke's pouch, and usually presents as an intrasellar lesion with varying degrees of suprasellar extension. However, to date, a description of a primary prepontine RCC with no intrasellar component has not been reported. The author describes an exceptional case of a symptomatic RCC located behind the sella turcica in a 41-year-old woman who presented with severe headache. The author also provides an embryological hypothesis of the development of an ectopic RCC, with a special emphasis on radiologic characteristics.
PMCID: PMC3467375  PMID: 23091676
Prepontine cistern; Rathke's pouch; Rathke's cleft cyst; Sella turcica; Suprasellar cyst
5.  Pituitary Apoplexy Presenting as Addisonian Crisis 
Texas Heart Institute Journal  2002;29(3):193-199.
Addisonian crisis, also commonly referred to as adrenal crisis, occurs when the cortisol produced by the adrenal glands is insufficient to meet the body's acute needs. The symptoms are nonspecific and can mimic other processes, such as sepsis. Hypotension, lethargy, and fever can all be presenting signs. Secondary addisonian crisis can also result from pituitary apoplexy.
Pituitary apoplexy usually occurs as hemorrhagic or ischemic necrosis in the presence of a pre-existing pituitary adenoma, and is a rare sequela of surgery. The symptoms of pituitary apoplexy are typically impressive and are relieved by urgent transsphenoidal decompression. Hypopituitarism resulting from pituitary apoplexy can be treated with exogenous hormones.
The case presented herein illustrates occult pituitary apoplexy that occurred after on-pump coronary artery bypass grafting. In this patient, the initial signs of addisonian crisis were overlooked; however, once recognized, they were reduced dramatically with standard stress-dose cortisone. A suprasellar mass with a cystic component was found on magnetic resonance imaging. The hemorrhagic pituitary gland was treated by transsphenoidal decompression, which relieved the patient's bitemporal hemianopia and 6th-nerve palsy. (Tex Heart Inst J 2002;29:193–9)
PMCID: PMC124758  PMID: 12224722
Addisonian crisis; adenoma/diagnosis/complications; adrenal gland diseases/etiology; adrenal gland hypofunction/diagnosis/drug therapy/physio-pathology; coronary artery bypass; cardiopulmonary bypass/adverse effects; hemorrhage/etiology; pituitary apoplexy/etiology; pituitary neoplasms/diagnosis/complications; post-operative complications
6.  Diabetes insipidus as a presenting manifestation of Rathke's cleft cyst 
Rathke's cleft cysts (RCC) are cystic sellar and suprasellar lesions derived from remnants of Rathke's pouch, lined by cuboidal or columnar epithelium. RCC are usually asymptomatic but can present with headache, visual impairment, panhypopituitarism and hypothalamic dysfunction. Diabetes Insipidus as a presenting symptom of RCC is reported, but rare. We present a case of a 48-year-old male presenting with polyuria and on investigations found to have central diabetes insipidus due to a sellar RCC. Patient underwent transsphenoidal surgery with complete excision with resolution of his symptoms. His polyuria resolved post-surgery without vasopressin replacement, which has never been reported.
PMCID: PMC3830277  PMID: 24251131
Diabetes insipidus; Rathke cyst; panhypopituitarism
7.  Manifestation of a sellar hemangioblastoma due to pituitary apoplexy: a case report 
Hemangioblastomas are rare, benign tumors occurring in any part of the nervous system. Most are found as sporadic tumors in the cerebellum or spinal cord. However, these neoplasms are also associated with von Hippel-Lindau disease. We report a rare case of a sporadic sellar hemangioblastoma that became symptomatic due to pituitary apoplexy.
Case presentation
An 80-year-old, otherwise healthy Caucasian woman presented to our facility with severe headache attacks, hypocortisolism and blurred vision. A magnetic resonance imaging scan showed an acute hemorrhage of a known, stable and asymptomatic sellar mass lesion with chiasmatic compression accounting for our patient's acute visual impairment. The tumor was resected by a transnasal, transsphenoidal approach and histological examination revealed a capillary hemangioblastoma (World Health Organization grade I). Our patient recovered well and substitutional therapy was started for panhypopituitarism. A follow-up magnetic resonance imaging scan performed 16 months postoperatively showed good chiasmatic decompression with no tumor recurrence.
A review of the literature confirmed supratentorial locations of hemangioblastomas to be very unusual, especially within the sellar region. However, intrasellar hemangioblastoma must be considered in the differential diagnosis of pituitary apoplexy.
PMCID: PMC3197537  PMID: 21970745
8.  Pituitary Apoplexy due to Pituitary Adenoma Infarction 
Cause of pituitary apoplexy has been known as hemorrhage, hemorrhagic infarction or infarction of pituitary adenoma or adjacent tissues of pituitary gland. However, pituitary apoplexy caused by pure infarction of pituitary adenoma has been rarely reported. Here, we present the two cases pituitary apoplexies caused by pituitary adenoma infarction that were confirmed by transsphenoidal approach (TSA) and pathologic reports. Pathologic report of first case revealed total tumor infarction of a nonfunctioning pituitary macroadenoma and second case partial tumor infarction of ACTH secreting pituitary macroadenoma. Patients with pituitary apoplexy which was caused by pituitary adenoma infarction unrelated to hemorrhage or hemorrhagic infarction showed good response to TSA treatment. Further study on the predisposing factors of pituitary apoplexy and the mechanism of infarction in pituitary adenoma is necessary.
PMCID: PMC2588219  PMID: 19096606
Pituitary apoplexy; Pituitary adenoma infarction
9.  Successful Pregnancy in a Female with a Large Prolactinoma after Pituitary Tumor Apoplexy 
Pituitary apoplexy is a rare condition which may cause death of the patient in severe cases and many times leads to hypopituitarism. We report a case of apoplexy in a large prolactinoma resulting in empty sella syndrome followed by a successful pregnancy. Our patient is a 32-year-old female with a history of a macroprolactinoma for approximately 17 years who presented to our hospital with a history of severe headache, decreased level of consciousness, fever, nausea, vomiting, and diplopia of 12 hours duration. Magnetic resonance imaging done on admission showed an increase in the size of the pituitary adenoma with a subtle hemorrhage. The patient was admitted to the intensive care unit and treated conservatively. The condition of the patient improved within a few days. A few months later, she started having regular menstrual periods. A magnetic resonance imaging of the pituitary 1.5 years later was reported as empty sella syndrome, and approximately one year later she became pregnant. With the pituitary adenoma being resolved after developing pituitary apoplexy and continuing on cabergoline, the patient had a successful pregnancy with no recurrence of the adenoma after delivery and breastfeeding.
PMCID: PMC3806158  PMID: 24194992
10.  Pituitary Apoplexy Presenting as Isolated Third Cranial Nerve Palsy with Ptosis : Two Case Reports 
Pituitary apoplexy is a clinical syndrome caused by an acute ischemic or hemorrhagic vascular accident involving a pituitary adenoma or an adjacent pituitary gland. Pituitary apoplexy may be associated with a variety of neurological and endocrinological signs and symptoms. However, isolated third cranial nerve palsy with ptosis as the presenting sign of pituitary apoplexy is very rare. We describe two cases of pituitary apoplexy presenting as sudden-onset unilateral ptosis and diplopia. In one case, brain magnetic resonance imaging (MRI) revealed a mass in the pituitary fossa with signs of hemorrhage, upward displacement of the optic chiasm, erosion of the sellar floor and invasion of the right cavernous sinus. In the other case, MRI showed a large area of insufficient enhancement in the anterior pituitary consistent with pituitary infarction or Sheehan's syndrome. We performed neurosurgical decompression via a transsphenoidal approach. Both patients showed an uneventful recovery. Both cases of isolated third cranial nerve palsy with ptosis completely resolved during the early postoperative period. We suggest that pituitary apoplexy should be included in the differential diagnosis of patients presenting with isolated third cranial nerve palsy with ptosis and that prompt neurosurgical decompression should be considered for the preservation of third cranial nerve function.
PMCID: PMC2651557  PMID: 19274125
Third cranial nerve palsy; Ptosis; Pituitary apoplexy
11.  Collision sellar lesions: experience with eight cases and review of the literature 
Pituitary  2009;13(1):8-17.
The concomitant presence of a pituitary adenoma with a second sellar lesion in patients operated upon for pituitary adenoma is an uncommon entity. Although rare, quite a great variety of lesions have been indentified coexisting with pituitary adenomas. In fact, most combinations have been described before, but an overview with information on the frequency of combined pathologies in a large series has not been published. We present a series of eight collision sellar lesions indentified among 548 transsphenoidally resected pituitary adenomas in two Neurosurgical Departments. The histological studies confirmed a case of sarcoidosis within a non-functioning pituitary adenoma, a case of intrasellar schwannoma coexisting with growth hormone (GH) secreting adenoma, two Rathke’s cleft cysts combined with pituitary adenomas, three gangliocytomas associated with GH-secreting adenomas, and a case of a double pituitary adenoma. The pertinent literature is discussed with emphasis on pathogenetic theories of dual sellar lesions. Although there is no direct evidence to confirm the pathogenetic relationship of collision sellar lesions, the number of cases presented in literature makes the theory of an incidental occurrence rather doubtful. Suggested hypotheses about a common embryonic origin or a potential interaction between pituitary adenomas and the immune system are presented.
PMCID: PMC2807600  PMID: 19551516
Adenoma; Collision tumors; Nonadenomatous sellar lesions; Pituitary
12.  Radiological Apoplexy and Its Correlation with Acute Clinical Presentation, Angiogenesis and Tumor Microvascular Density in Pituitary Adenomas 
Pituitary apoplexy is life-threatening clinical syndrome caused by the rapid enlargement of a pituitary tumor due to hemorrhage and/or infarction. The pathogenesis of pituitary apoplexy is not completely understood. We analyzed the magnetic resonance imaging (MRI) of pituitary tumors and subsequently correlated the radiological findings with the clinical presentation. Additionally, immunohistochemistry was also performed to determine whether certain biomarkers are related to radiological apoplexy.
Thirty-four cases of pituitary adenoma were enrolled for retrospective analysis. In this study, the radiological apoplexy was defined as cases where hemorrhage, infarction or cysts were identified on MRI. Acute clinical presentation was defined as the presence of any of the following symptoms: severe sudden onset headache, decreased visual acuity and/or visual field deficit, and acute mental status changes. Angiogenesis was quantified by immunohistochemical expression of fetal liver kinase 1 (Flk-1), neuropilin (NRP) and vascular endothelial growth factor (VEGF) expression, while microvascular density (MVD) was assessed using Endoglin and CD31.
Clinically, fourteen patients presented with acute symptoms and 20 for mild or none clinical symptoms. Radiologically, fifteen patients met the criteria for radiological apoplexy. Of the fifteen patients with radiologic apoplexy, 9 patients presented acute symptoms whereas of the 19 patient without radiologic apoplexy, 5 patients presented acute symptoms. Of the five biomarkers tracked, only VEGF was found to be positively correlated with both radiological and nonradiological apoplexy.
While pituitary apoplexy is currently defined in cases where clinical symptoms can be histologically confirmed, we contend that cases of radiologically identified pituitary hemorrhages that present with mild or no symptoms should be designated subacute or subclinical apoplexy. VEGF is believed to have a positive correlation with pituitary hemorrhage. Considering the high rate of symptomatic or asymptomatic pituitary tumor hemorrhage, additional studies are needed to detect predictors of the pituitary hemorrhage.
PMCID: PMC3243828  PMID: 22200007
Pituitary adenoma; Pituitary apoplexy; Pituitary hemorrhage; Angiogenesis; Microvascular density; VEGF
13.  Pituitary Apoplexy Associated with Endocrine Stimulation Test: Endocrine Stimulation Test, Treatment, and Outcome 
Case Reports in Endocrinology  2012;2012:826901.
Pituitary apoplexy is a rare clinical syndrome attributable to hemorrhage or hemorrhagic infarction of pituitary tumors or pituitary glands. The features of pituitary apoplexy associated with the endocrine stimulation test remain to be elucidated and the importance of surgical treatment has not been discussed enough. We report two rare patients who were treated successfully by endoscopic endonasal transsphenoidal surgery within several hours after onset of pituitary apoplexy associated with the endocrine stimulation test. Their postoperative course was uneventful. We reviewed earlier reports on this clinical entity, document its features especially as related to the endocrine stimulation test, discuss the significance of immediate surgical treatment, and present our treatment outcomes. Performing only conservative treatment is not recommended. We suggest that the necessity of endocrine stimulation test should be assessed on a case-by-case basis and in patients subjected to the test, and neurosurgical support should be sought.
PMCID: PMC3424651  PMID: 22934202
14.  Pituitary Apoplexy Producing Internal Carotid Artery Compression: A Case Report 
Journal of Korean Medical Science  2008;23(6):1113-1117.
We report a case of pituitary apoplexy resulting in right internal carotid artery occlusion accompanied by hemiplegia and lethargy. A 43-yr-old man presented with a sudden onset of severe headache, visual disturbance and left hemiplegia. Investigations revealed a nodular mass, located in the sella and suprasellar portion and accompanied by compression of the optic chiasm. The mass compressed the bilateral cavernous sinuses, resulting in the obliteration of the cavernous portion of the right internal carotid artery. A border zone infarct in the right fronto-parietal region was found. Transsphenoidal tumor decompression following conservative therapy with fluid replacement and steroids was performed. Pathological examination revealed an almost completely infarcted pituitary adenoma. The patient's vision improved immediately after the decompression, and the motor weakness improved to grade IV+ within six months after the operation. Pituitary apoplexy resulting in internal carotid artery occlusion is rare. However, clinicians should be aware of the possibility and the appropriate management of such an occurrence.
PMCID: PMC2610651  PMID: 19119461
Pituitary Apoplexy; Cerebral Infarction; Cerebrovascular Disorders; Paresis
15.  An interesting case of Rathke's cleft cyst presenting as bilateral cryptorchidism 
Rathke's cleft cyst (RCC) are benign, epithelium-lined sellar and suprasellar cysts believed to originate from remnants of the Rathke pouch. Here, we report a rare case of entirely suprasellar RCC in a 4.5-year-old child causing hypopituitarism, who presented to us with bilateral cryptorchidism. Subsequently we discuss the distinct rarity of entirely suprasellar RCC presenting in this fashion and the relevant literature is also discussed.
PMCID: PMC3888040  PMID: 24470817
Cryptorchidism; hypopituitarism; Rathke's cleft cyst; testicular descent
16.  Pituitary Apoplexy Complicated by Chemical Meningitis and Cerebral Infarction 
Journal of Korean Medical Science  2007;22(6):1085-1089.
A 41-yr-old man was admitted with acute headache, neck stiffness, and febrile sensation. Cerebrospinal fluid examination showed pleocytosis, an increased protein level and, a decreased glucose concentration. No organisms were observed on a culture study. An imaging study revealed pituitary macroadenoma with hemorrhage. On the 7th day of the attack, confusion, dysarthria, and right-sided facial paralysis and hemiparesis were noted. Cerebral infarction on the left basal ganglia was confirmed. Neurologic deficits gradually improved after removal of the tumor by endoscopic transnasal transsphenoidal approach. It is likely that the pituitary apoplexy, aseptic chemical meningitis, and cerebral infarction are associated with each other. This rare case can serve as a prime example to clarify the chemical characteristics of pituitary apoplexy.
PMCID: PMC2694622  PMID: 18162729
Cerebral Infarction; Meningitis; Pituitary Apoplexy
17.  Partially Reversible Hypopituitarism in an Adolescent with a Rathke Cleft Cyst 
Rathke cleft cysts are remnants of the Rathke pouch. Most of them are asymptomatic, but sometimes they can grow enough to cause compression of structures within and/or close to the sella, thus eliciting symptoms such as visual disturbance, pituitary defects, and headache. Asymptomatic cysts can safely be followed up with serial imaging, while the standard treatment for symptomatic lesions is surgical removal. We describe a 14-yr-old boy, admitted for anorexia, fatigue, weight loss, recurrent headache and vomiting. Magnetic resonance imaging showed an intra- and suprasellar cystic lesion, which was surgically removed. Histology was consistent with Rathke’s cleft cyst. Diabetes insipidus and multiple anterior pituitary defects (GH, ACTH and TSH) were found preoperatively, and substitutive therapy was started. No additional hormonal defect appeared after surgery. After 4 yr of follow up, pituitary function was retested, and there were no confirmed GH or ACTH defects, allowing a partial withdrawal of replacement therapy. Our report confirms that pituitary defects, in patients with a Rathke cleft cyst, may recover even year after surgery. Thus, retesting of pituitary axes is indicated during long-term follow up.
PMCID: PMC3687652  PMID: 23926414
Rathke cleft cyst; pituitary; GH
18.  Spontaneous Rupture, Disappearance, and Reaccumulation of a Rathke's Cleft Cyst 
Case Reports in Endocrinology  2011;2011:549262.
Rathke's cleft cysts (RCCs) are benign epithelium-lined intrasellar cysts containing mucoid material and are believed to originate from the remnants of Rathke's pouch. Most are asymptomatic but may cause symptoms secondary to compression of adjacent structures such as visual disturbances and endocrinopathies, especially hypopituitary. Furthermore, inflammation such as an aseptic meningitis syndrome may be associated with these tumors, presumably resulting from leakage of cyst material into the subarachnoid space. We present a unique case of spontaneous rupture and complete disappearance of a known sella-suprasellar cyst associated with a severe headache syndrome, followed by cyst reaccumulation requiring surgery. Although this phenomenon is well accepted, to our knowledge, this is the first report of the complete disappearance of a Rathke's cyst presenting with the classic syndrome. Furthermore, it was remarkable how quickly it recurred and became symptomatic, providing evidence that an “empty sella syndrome” may indeed need clinical follow-up.
PMCID: PMC3420408  PMID: 22937285
19.  Precipitating factors in pituitary apoplexy 
Pituitary apoplexy is a rare but life threatening condition caused by sudden haemorrhage or infarction of the pituitary gland. Potential precipitating factors in the occurrence of acute pituitary apoplexy in 30 consecutive patients were identified and compared with the clinical characteristics and outcome of patients with and without associated factors. Six patients had a previously known pituitary adenoma. All patients complained of severe headaches, associated with neuro-ophthalmological symptoms and signs in 83% and altered mental status in 30%. Potential risk factors were identified in nine patients (30%). When there was an associated factor, the clinical presentation was no different than in patients without such factors although altered mental status may be more frequent in patients with associated diseases. In these patients, the visual prognosis was worse and the diagnosis was more difficult to establish. Acute pituitary apoplexy is unpredictable and should be considered in any patient with abrupt neuro-ophthalmological deterioration associated with headache. Patients with pituitary apoplexy often have an associated disease that confounds recognition and treatment despite a typical presentation.

PMCID: PMC1763528  PMID: 11561045
20.  Pituitary apoplexy induced by Gonadotropin-releasing hormone agonists for treating prostate cancer-report of first Asian case 
We present the first Asian case of a 77-year-old man who developed pituitary apoplexy (PA) soon after gonadotropin-releasing hormone agonist (GnRHa) (leuprorelin) injection to treat prostate cancer. Headache, ophthalmoplegia, visual field deficit, nausea, and vomiting are the typical characteristics of pituitary apoplexy. Though the occurrence rate is rare, the consequence of this condition can vary from mild symptoms such as headache to life-threatening scenarios like conscious change. Magnetic resonance imaging is the best imaging modality to detect PA and sublabial trans-sphenoid pituitary tumor removal can resolve most of PA symptoms and is so far the best solution in consensus. We also review 11 previous reported cases receiving GnRHa for androgen deprivation therapy of prostate cancer, and hope to alert clinicians to use GnRHa with caution.
PMCID: PMC3851712  PMID: 24088191
Gonadotropin-releasing hormone agonists; Pituitary apoplexy; Prostate cancer
21.  Ectopic salivary gland tissue in a Rathke’s cleft cyst 
The presence of salivary gland tissue in the sella turcica has rarely been reported, mainly after pituitary examination at autopsy. Only five symptomatic cases have previously been described, mainly associated with Rathke’s cleft cyst. We report a 17-year-old boy presenting with headaches and hyperprolactinemia. The MRI showed a 19 mm sellar mass that at surgery revealed as a cystic lesion filled with mucinous fluid. The histological examination documented the presence of ectopic salivary gland tissue in the wall of a Rathke’s cleft cyst. The present report focuses on the possible pitfalls when dealing with unusual sellar lesions, and the need of increased awareness of this rare condition.
PMCID: PMC3693212  PMID: 23826428
Salivary gland tissue; pituitary; Rathke’s cleft cyst
22.  Xanthogranuloma of the intrasellar region presenting in pituitary dysfunction: a case report 
Differentiation of cystic mass lesions of the sellar and parasellar regions may pose a diagnostic dilemma for physicians, neurosurgeons, radiologists and pathologists involved in treating patients with these entities. A considerable number of tumors previously identified as craniopharyngiomas may, in fact, have been xanthogranulomas. We report a case of pituitary dysfunction caused by xanthogranuloma of the intrasellar region.
Case presentation
A 47-year-old man of Japanese descent presented to our institution with a tumor located exclusively in the intrasellar region which manifested as severe hypopituitarism. MRI revealed a clearly defined intrasellar mass that was heterogeneously hyperintense on T1-weighted images and markedly hypointense on T2-weighted images. We preoperatively diagnosed the patient with Rathke's cleft cyst or non-functioning pituitary adenoma. Although the tumor was completely removed using a transsphenoidal approach, the improvement of the patient's endocrine function was marginal, and continued endocrine replacement therapy was needed. Postoperatively, a histological examination revealed the tumor to be a xanthogranuloma of the intrasellar region. His visual field defects and headache improved.
Because diagnosis depends on surgical intervention and xanthogranulomas of the intrasellar region are very rare, the natural history of xanthogranuloma is still unknown. Therefore, this entity is difficult to diagnose preoperatively. We suggest that xanthogranuloma should be included in the differential diagnosis, even in the case of sellar lesions, to formulate appropriate postoperative management and improve endocrine outcomes.
PMCID: PMC3353873  PMID: 22540975
xanthogranuloma; intrasellar region; craniopharyngioma; Rathke's cleft cyst; panhypopituitarism
23.  Risk factor for pituitary dysfunction in children and adolescents with Rathke's cleft cysts 
Korean Journal of Pediatrics  2010;53(7):759-765.
This study evaluated the clinical manifestations of and risk factors for pituitary insufficiency in children and adolescents with Rathke's cleft cysts.
Forty-four patients with Rathke's cleft cysts younger than 19 years who visited Seoul National University Children's Hospital between January 1995 and September 2009 were enrolled. Rathke's cleft cysts were confirmed histologically through an operation in 15 patients and by brain magnetic resonance imaging (MRI) in 29 patients. The clinical, hormonal, and imaging features were reviewed retrospectively.
The clinical presentation of symptomatic patients was as follows: headache (65%), endocrinopathy (61%), and visual disturbance (19%). Endocrinopathy included central precocious puberty (18%), diabetes insipidus (14%), general weakness (11%), and decreased growth velocity (7%). After surgery, hyperprolactinemia resolved in all patients, but growth hormone insufficiency, hypothyroidism, and diabetes insipidus did not improve. Pituitary insufficiency except gonadotropin abnormality correlated significantly with severe headache, visual disturbance, general weakness, and cystic size. Suprasellar extension of cysts and high signals in the T2-weighted image on brain MRI were related to hypothyroidism, hypocortisolism, and diabetes insipidus. Multivariable linear regression analysis showed that only general weakness was a risk factor for pituitary insufficiency (R2=0.549).
General weakness is a risk factor for pituitary insufficiency in patients with Rathke's cleft cysts. When a patient with a Rathke's cleft cyst complains of general weakness, the clinician should evaluate pituitary function and consider surgical treatment.
PMCID: PMC3004488  PMID: 21189952
Rathke's cleft cysts; Pituitary insufficiency
24.  Pituitary apoplexy 
Pituitary apoplexy is rare endocrine emergency which can occur due to infarction or haemorrhage of pituitary gland. This disorder most often involves a pituitary adenoma. Occasionally it may be the first manifestation of an underlying adenoma. There is conflicting data regarding which type of pituitary adenoma is prone for apoplexy. Some studies showed predominance of non-functional adenomas while some other studies showed a higher prevalence in functioning adenomas amongst which prolactinoma have the highest risk. Although pituitary apoplexy can occur without any precipitating factor in most cases, there are some well recognizable risk factors such as hypertension, medications, major surgeries, coagulopathies either primary or following medications or infection, head injury, radiation or dynamic testing of the pituitary. Patients usually present with headache, vomiting, altered sensorium, visual defect and/or endocrine dysfunction. Hemodynamic instability may be result from adrenocorticotrophic hormone deficiency. Imaging with either CT scan or MRI should be performed in suspected cases. Intravenous fluid and hydrocortisone should be administered after collection of sample for baseline hormonal evaluation. Earlier studies used to advocate urgent decompression of the lesion but more recent studies favor conservative approach for most cases with surgery reserved for those with deteriorating level of consciousness or increasing visual defect. The visual and endocrine outcomes are almost similar with either surgery or conservative management. Once the acute phase is over, patient should be re-evaluated for hormonal deficiencies.
PMCID: PMC3183518  PMID: 22029023
Apoplexy; hypopituitarism; pituitary
25.  Clinical Outcome of Cranial Neuropathy in Patients with Pituitary Apoplexy 
Pituitary apoplexy (PA) is described as a clinical syndrome characterized by sudden headache, vomiting, visual impairment, and meningismus caused by rapid enlargement of a pituitary adenoma. We retrospectively analyzed the clinical presentation and surgical outcome in PA presenting with cranial neuropathy.
Twelve cases (3.3%) of PA were retrospectively reviewed among 359 patients diagnosed with pituitary adenoma. The study included 6 males and 6 females. Mean age of patients was 49.0 years, with a range of 16 to 74 years. Follow-up duration ranged from 3 to 20 months, with an average of 12 months. All patients were submitted to surgery, using the transsphenoidal approach (TSA).
Symptoms included abrupt headache (11/12), decreased visual acuity (12/12), visual field defect (11/12), and cranial nerve palsy of the third (5/12) and sixth (2/12). Mean height of the mass was 29.0 mm (range 15-46). Duration between the ictus and operation ranged from 1 to 15 days (mean 7.0). The symptom duration before operation and the recovery period of cranial neuropathy correlated significantly (p = 0.0286). TSA resulted in improvement of decreased visual acuity in 91.6%, visual field defect in 54.5%, and cranial neuropathy in 100% at 3 months after surgery.
PA is a rare event, complicating 3.3% in our series. Even in blindness following pituitary apoplexy cases, improvement of cranial neuropathy is possible if adequate management is initiated in time. Surgical decompression must be considered as soon as possible in cases with severe visual impairment or cranial neuropathy.
PMCID: PMC2966721  PMID: 21082047
Pituitary apoplexy; Pituitary adenoma; Cranial neuropathy; Transsphenoidal approach

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