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.
Rathke cleft cyst; pituitary adenoma; pituitary apoplexy; anticoagulation
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)
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
Pituitary apoplexy (PA) is a rare and potentially fatal clinical condition presenting acute headache, vomiting, visual impairment, ophthalmoplegia, altered mental state and possible panhypopituitarism. It mostly occurs in patients with haemorrhagic infarction of the pituitary gland due to a pre-existing macroadenoma. Although there are pathological and physiological conditions that may share similar imaging characteristics, both clinical and imaging features can guide the radiologist towards the correct diagnosis, especially using magnetic resonance imaging (MRI). In this review, we will describe the main clinical and epidemiological features of PA, illustrating CT and MRI findings and discussing the role of imaging in the differential diagnosis, prognosis and follow-up.
• Headache, ophtalmoplegia and visual impairment are frequent symptoms of pituitary apoplexy.
• CT is often the first imaging tool in PA, showing areas of hyperdensity within the sellar region.
• MRI could confirm haemorrhage within the pituitary gland and compression on the optic chiasm.
• Frequent simulating conditions are aneurysms, Rathke cleft cysts, craniopharingioma and mucocele.
• The role of imaging is still debated and needs more studies.
Pituitary apoplexy; Pituitary adenoma; MRI; Pituitary haemorrhage; Macroadenoma
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.
Pituitary adenoma; Pituitary apoplexy; Pituitary hemorrhage; Angiogenesis; Microvascular density; VEGF
A ruptured aneurysm associated with a pituitary apoplexy is rare. We present the first case report of the coexistence of a ruptured posterior communicating aneurysm with a surgically discovered pituitary apoplexy where the pituitary apoplexy had not been diagnosed by a pre-operative computerized tomography scan.
A 31-year-old right-handed Chinese woman began to experience severe headache, vomiting and blurred vision which continued for two days. On admission to the hospital, a brain computerized tomography scan demonstrated a small amount of increased signal in the basal cisterns; no evidence of intrasellar and suprasellar lesions was seen. The appearance of her brain suggested aneurysmal subarachnoid hemorrhage. She had nuchal rigidity and reduced vision. There was no extra-ocular palsy and no other neurological deficit. Our patient had no stigmata of Cushing’s syndrome or acromegaly. During an interview for further history, she reported normal menses and denied reduced vision.
Cerebral digital subtraction angiography was subsequently performed, which revealed a 6mm left posterior communicating aneurysm. Urgent left pterional craniotomy was performed. The left ruptured posterior communicating artery aneurysm was completely dissected prior to clipping. At surgery, a suprasellar mass was discovered, the tumor bulging the diaphragma sella and projecting anteriorly under the chiasm raising suspicion of a pituitary tumor. The anterior part of the tumor capsule was opened and a necrotic tumor mixed with dark old blood was removed. The appearance suggested pituitary apoplexy.
Histopathology revealed pituitary adenoma with evidence of hemorrhagic necrosis. Our patient made a good recovery.
Our case report proves that pituitary apoplexy can be coexistent with the rupture of a posterior communicating aneurysm. This association should be considered when evaluating any case of aneurysm. A normal computerized tomography scan does not exclude pituitary apoplexy. Pre-operative magnetic resonance imaging interpretation is required if a pituitary apoplexy is suspected. Craniotomy allows a coexisting aneurysm and pituitary apoplexy to be simultaneously treated.
Aneurysm; Pituitary apoplexy; Pituitary adenoma; Subarachnoid hemorrhage
Cystic sellar lesions are a rare cause of hypopituitarism and extremely rare in the pediatric age group. The differential diagnosis is large and includes both primary pituitary abscesses and cystic components on pre-existing lesions, such as adenoma, craniopharyngioma, Rathke's cleft cyst, leukemia, granulomatous disease and lymphocytic hypophysitis. In the absence of a definitive diagnosis, treatment can be challenging. We report a case of a 15-year-old female, who presented with headaches, altered consciousness and diplopia after a molar extraction, for which she had received oral antibiotics. Broad-spectrum i.v. antibiotics were given for presumed meningitis. Blood cultures failed to identify pathogens. Cerebral magnetic resonance imaging showed a pituitary cystic lesion. Endocrine studies revealed abnormal pituitary function. In the absence of a therapeutic response, the patient underwent a transsphenoidal biopsy of the pituitary gland, which yielded a purulent liquid, but cultures were negative. Histopathology showed lymphocytic infiltrates but no neutrophils, compatible with an inflammation of autoimmune or infectious origin. High-dose glucocorticoid therapy was started and pursued, along with i.v. antibiotics, for 6 weeks, leading to clinical and radiological improvement but with persistence of endocrine deficits. In conclusion, this is a case of secondary panhypopituitarism due to a cystic pituitary lesion, with a differential diagnosis of lymphocytic hypophysitis vs abscess in a context of decapitated meningitis. Combination therapy with antibiotics and glucocorticoids is a legitimate approach in the face of diagnostic uncertainty, given the morbidity, and even mortality, associated with these lesions.
It is not always easy to differentiate primary cystic sellar lesions (such as a primary infectious pituitary abscess) from cystic components on pre-existing lesions (such as adenoma, craniopharyngioma, Rathke's cleft cyst, leukemia or lymphocytic hypophysitis).Because of the absence of specific symptoms and of immunohistochemical and serum markers, response to glucocorticoids can be the only way to differentiate lymphocytic hypophysitis from pituitary lesions of another origin. In addition, microbiological cultures are negative in 50% of cases of primary infectious sellar abscesses, thus the response to antibiotic treatment is often the key element to this diagnosis.A short course of high-dose glucocorticoids combined with antibiotics is not harmful in cases where there is no diagnostic certainty as to the origin of a cystic sellar mass, given the morbidity and mortality associated with these lesions.This approach may also diminish inflammation of either infectious or autoimmune origin while ensuring that the most likely pathogens are being targeted.
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.
Adenoma; development; neoplasm; pituitary; Rathke's cleft cyst; sella; tumor
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.
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.
Pituitary cyst; Rathke's cleft cyst; spontaneous involution; transsphenoidal surgery
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.
Rathke's cleft cysts; Pituitary insufficiency
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.
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.
Pituitary Apoplexy; Cerebral Infarction; Cerebrovascular Disorders; Paresis
Pituitary apoplexy is an uncommon phenomenon typically characterized by vascular insufficiency or acute hemorrhage into a pituitary adenoma. The overall incidence of pituitary apoplexy ranges between 1 and 25% of all pituitary adenomas. With the widespread use of MRI technology, the diagnosis of asymptomatic intratumoral hemorrhage is closer to 10%. The authors report a case of a 27-year-old female in her 36th week of pregnancy who presented with severe onset headache and acute left-sided vision loss. MRI of the brain revealed a large hemorrhagic mass occupying the sella turcica. The patient underwent an emergent endoscopic endonasal transsphenoidal resection for pituitary apoplexy. Postoperatively, the patient's neurologic deficit resolved. Minimally invasive endoscopic endonasal transsphenoidal resection of pituitary apoplexy can be safely utilized in third trimester pregnant women presenting with acute severe neurologic deficits.
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.
Third cranial nerve palsy; Ptosis; Pituitary apoplexy
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.
Prepontine cistern; Rathke's pouch; Rathke's cleft cyst; Sella turcica; Suprasellar cyst
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.
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.
Enlargement of intracerebral hematoma without rebleeding in chronic phase is a rare but well-known clinical condition, and is well-described as chronic expanding intracerebral hematoma. However, chronic enlargement of pituitary hematoma without rebleeding after pituitary apoplexy is extremely rare.
We report a case of chronic expanding pituitary hematoma without rebleeding after pituitary apoplexy. A 29-year-old male presented with sudden onset of headache and vomiting. Magnetic resonance imaging (MRI) demonstrated a pituitary mass lesion with hematoma, consistent with pituitary apoplexy. Neuro-ophthalmological examination revealed no visual field defect, and endocrinological evaluations showed an elevated prolactin level. Pituitary apoplexy due to a prolactinoma was the most likely diagnosis. He was conservatively treated because he exhibited no visual disturbance. Three weeks after the onset, he gradually began to complain of blurred vision and neuro-ophthalamological examination revealed bitemporal upper quadrant hemianopsia. MRI showed enlargement of the pituitary hematoma without any finding suggestive of rebleeding. This enlarged mass lesion compressed the chiasm. The patient was operated on via transsphenoidal approach. After dural opening, xanthochromic fluid spouted out, but no fresh clot could be detected within the cyst. After the operation, the visual field disturbance resolved completely. The possible mechanism of hematoma enlargement is considered to be expansion due to the serum exudation from capillaries of the hematoma capsule. This pathogenetic mechanism is common in enlargement of chronic subdural hematoma.
This case is the first report of chronic expanding pituitary hematoma without rebleeding after pituitary apoplexy.
Chronic expanding hematoma; pituitary apoplexy; pituitary adenoma; prolactinoma; transsphenoidal surgery
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.
Diabetes insipidus; Rathke cyst; panhypopituitarism
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.
Pituitary apoplexy; Pituitary adenoma infarction
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.
Adenoma; Collision tumors; Nonadenomatous sellar lesions; Pituitary
Pituitary apoplexy is a rare event in pregnancy. A 41-year-old woman with a known pituitary microadenoma presented with visual disturbance and headache during the second trimester of pregnancy. Magnetic resonance imaging (MRI) demonstrated pituitary apoplexy with chiasmal compression. After treatment with corticosteroid therapy, she underwent transsphenoidal excision of the pituitary adenoma. Visual abnormalities were completely restored and pituitary function preserved. There was no evidence of impact on the foetus. The literature on the subject is reviewed with emphasis on the management of the apoplectic patient with mild and stable neuro-ophthalmological signs.
There are no clear guidelines on the management of pituitary apoplexy in pregnancy. A multidisciplinary approach can minimise morbidity and mortality.Pituitary apoplexy has an unpredictable clinical course and determining which clinical situations warrant early surgery needs to take into consideration the presence and severity of neurological signs and their stability.The management of conscious apoplectic patients with absent or mild and stable neuro-ophthalmological signs is controversial.
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.
Rathke cleft cyst; pituitary; GH
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.
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.
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.
xanthogranuloma; intrasellar region; craniopharyngioma; Rathke's cleft cyst; panhypopituitarism
We report and discuss the rare case of a pituitary abscess forming within a Rathke's cleft cyst (RCC). A 66-year-old gentleman presented with visual deterioration and symptoms suggestive of hypopituitarism. The patient underwent transsphenoidal debulking of the lesion whereupon purulent material was discovered. Histological examination was suggestive of RCC together with numerous neutrophils characteristic of abscess. Microbiological culture of the material grew Staphylococcus aureus. The patient was treated for a RCC abscess and received antibiotics and endocrine replacement therapy. The patient has been followed up for 2 years without recurrence. Although uncommon, we recommend the consideration of RCC abscess as a differential diagnosis of a pituitary mass lesion as clinical presentation and radiological assessment are not specific in identifying these lesions preoperatively.
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.