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
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
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
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
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.
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
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
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
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.
Cerebral Infarction; Meningitis; Pituitary Apoplexy
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 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
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.
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.
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.
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
We report a rare case of symptomatic Rathke's cleft cyst with thick calcified wall. Brain CT scans revealed a large cystic mass with round thick calcified wall. In this case, we selected the pterional approach instead of transsphenoidal approach due to the possibility of cystic craniopharyngioma. Histopathologically, it was calcified Rathke's cleft cyst with focal epithelial metaplasia. This case illustrates that calcification of the suprasellar cyst does not always suggest craniopharyngioma and the calcification pattern of Rathke's cleft cyst is different from that of the craniopharyngioma.
Rathke's cleft cyst; Calcification
Background and Purpose. To explore strategies for the diagnosis and treatment of Rathke's cleft cyst (RCC). Methods. The medical records of 24 patients with sellar RCC were retrospectively reviewed. Two patients had concomitant pituitary adenoma, 2 underwent transcranial surgery, and 22 underwent transsphenoidal surgery. The clinical features, especially the findings of intracystic nodules on MRI, were evaluated and compared with the pathological findings. Results. Preoperatively, only 2 patients were diagnosed with RCC or suspected RCC. Pre- and postoperative MRI images revealed 10 intracystic nodules in 9 (37.5%) patients. Two nodules had bull's eyelike changes. The signal intensity of the intracystic nodules varied on T1- and T2-weighted images. Not all nodules on T2-weighted images were visualized. Postoperative MRI revealed recurrence or residual lesion in 5 patients; none had new symptoms and a second surgery was not required. Conclusions. Identifying intracystic nodules is important in patients with sellar cystic lesions. Bull's eyelike change in an intracystic nodule on MRI, which is reported here for the first time, potentially might have value for confirming the diagnosis.
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.
Salivary gland tissue; pituitary; Rathke’s cleft cyst
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
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 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.
Apoplexy; hypopituitarism; pituitary
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.
Pituitary apoplexy; Pituitary adenoma; Cranial neuropathy; Transsphenoidal approach
Our aim is to describe a case of acromegaly that was associated with symptomatic Rathke's cyst. We describe a young male student without any significant family history who presented with clinical and biochemical features consistent with growth hormone excess, which was confirmed with dynamic testing. He also described a persistent headache predating symptoms of growth hormone excess by 4 years. Magnetic resonance imaging (MRI) of the pituitary showed a large sellar mass which was thought to be a somatotroph adenoma. Trans-sphenoidal surgery was performed; however, a colloid lesion was identified by the neurosurgeon that proved to be a Rathke's cyst. The association of acromegaly with Rathke's cyst is very rare, with less than 10 cases found to be reported on review of literature. This is the first report from India.
Acromegaly; colloid cyst; growth hormone excess; pituitary adenoma; pituitary tumor; Rathke's cyst
Collision tumors of the sellar region are relatively uncommon and consist mainly of more than one type of pituitary adenoma or a cyst or cystic tumor. The association of a pituitary adenoma and a craniopharyngioma is particularly rare. This study describes a rare occurrence in which a pituitary adenoma and a craniopharyngioma coexisted in the sellar region. The case involves a 47-year-old woman who underwent transsphenoidal surgery with subtotal tumor resection and reoperation using an interhemispheric transcallosal approach for total microsurgical resection of the tumor because the visual acuity in her left eye had re-deteriorated. Histopathological and immunohistochemical examinations of the excised tissue revealed a pituitary adenoma in the first operation and a craniopharyngioma in the second operation. Retrospective analysis found the coexistence of a pituitary adenoma and a craniopharyngioma, known as a collision tumor. Instead of the transsphenoidal approach, a craniotomy should be performed, to explore the suprasellar region.
Collision tumor; Craniopharyngioma; Pituitary adenoma; Sellar region
A patient with panhypopituitarism and visual field defects due to a Rathke's cleft cyst is presented. These cysts are commonly found in random pituitaries examined at necropsy, but rarely produce symptoms. Subtle endocrine deficiencies, however, may now be uncovered more frequently with modern diagnostic techniques. An unusual and unreported feature of this cyst was an apudamyloid stroma. This would imply that cells derived from the neural crest participate in the formation of the cyst. Recognition of these cysts at the time of operation is important in avoiding confusion with pituitary adenomas and unnecessarily aggressive treatment.