Surgery provides the best chance of meeting these goals for most patients with pituitary masses with the exception of patients with presumed prolactinomas, which generally respond well to medical therapy. However, persistent or recurrent disease may occur postoperatively, requiring life-long follow-up imaging and laboratory testing. The recurrence risk may vary significantly depending on the underlying pathology, size, and location of the sellar lesion and surgical expertise.
Patients with functioning sellar lesions, including acromegaly and Cushing disease, have increased mortality risk. However, remission of these conditions appears to mitigate the excess mortality associated with growth hormone or cortisol excess, respectively.
The presence of central diabetes insipidus occurring in a patient with a sellar mass prior to any pituitary surgery strongly suggests that the sellar mass is not a pituitary adenoma. Changes in facial appearance or features may provide subtle, yet important clues to the presence of growth hormone or cortisol excess. Central hypoadrenalism should always be sought and either ruled out or treated with physiologic replacement doses of glucocorticoids, before thyroid hormone replacement is advised.
This is essential to avert precipitating adrenal crisis, which may occur by introducing thyroid hormone replacement in a patient with untreated central adrenal insufficiency.
Unreplaced glucocorticoid and thyroid hormone deficiencies decrease free water clearance by the kidneys and may prevent the development of polyuria in patients with ADH deficiency. As a consequence, the institution of glucocorticoid and thyroid hormone replacement therapies in patients with panhypopituitarism may unmask previously latent central diabetes insipidus, precipitating the onset of hyposthenuric polyuria.
Regular, pituitary protocol MRI examinations are advised in all patients with sellar masses. The frequency of MRI examinations depends on the underlying pathology. It may be noted that the optimal frequency of follow-up imaging has not been determined in prospective studies.
Incidental sellar lesions thought to represent nonfunctioning adenomas and are being followed expectantly may be imaged at 6 months and annually thereafter for years, with less frequent imaging thereafter, if stable. Patients undergoing pituitary surgery should generally have postoperative MRI examinations at weeks, with subsequent MRI studies obtained at 6 months, and then annually thereafter for years.
Subsequently, MRI examinations may be performed every years in patients with stable appearance of the sella. After undergoing pituitary surgery, patients should be monitored including assessment of fluid balance, serum sodium, and urine specific gravity or osmolality to detect the possible development of diabetes insipidus, and should have frequent monitoring of serum sodium for about 2 weeks after surgery in order to detect the possibility of hyponatremia occurring as a result of the syndrome of inappropriate ADH secretion, hypoadrenalism, or hypothyroidism.
Preemptively instituted glucocorticoid replacement is advised after pituitary surgery, until sufficiency of pituitary-adrenocortical function is assured. Full evaluation of pituitary function should be performed postoperatively generally around 6 weeks after surgery to examine whether pituitary function is intact, and establish if biochemical remission of disorders associated with hormone excess has occurred.
Patients with functioning pituitary adenomas require life-long periodic evaluation to detect possible biochemical recurrences. Patients with persistent or recurrent sellar masses after initial surgery may require reoperation, radiation therapy or medical therapy as already outlined. J Neurosurg. A case series of patients with incidentally found nonfunctioning sellar masses presumed macroadenomas , which includes data on the development of pituitary apoplexy during follow-up.
Endocrinol Metab Clin North Am. This is a helpful review of lesions showing T1 hyperintensity, focusing on radiographic differential diagnosis. Eur Radiol. Presse Med. Buurman, H, Saeger, W. Eur J Endocrinol. This study presents autopsy data on the prevalence of incidentally found sellar masses.
This report focuses on the radiographic appearance of the pituitary in patients with ipilimumab-induced hypophysitis. Clin Radiol. Donovan, LE, Corenblum, B. Arch Intern Med. This is a very helpful systematic review of the prevalence of pituitary adenomas, including incidental lesions. J Clin Endocrinol Metab. Clin Endocrinol Oxf. This article includes the Endocrine Society guidelines on the evaluation and management of incidentally found sellar lesions. This document includes a thoughtful discussion of the limitations of available data.
Endocrinol Metab ClinNorth Am. Ann Intern Med. This is an attempt to define a cost-effective approach in the evaluation and management of incidentally found sellar lesions [microincidentalomas]. Klibanski, A. N Engl J Med. This is a comprehensive, thoroughly updated review of prolactinomas. This is an interesting report on a relatively novel entity [IgG4-related hypophysitis]. To emphasize the significance of endocrine assessment, and to identify lesions amenable to hormonal treatment.. A retrospective review of the records of children under 14 years of age referred to our center for sellar lesions during a period of 12 years.
Data collected included sex, age, nature of lesion, clinical presentation, size, treatment, and endocrine abnormalities.. Forty-five patients 25 females aged 7. Follow-up time was 6. Lesion nature was known in 39 cases, 4 of which were successfully treated at the Endocrinology Department: 3 prolactinomas with dopamine agonist and one thyrotroph cell hyperplasia with levothyroxine. Duration of endocrine and neuro-ophthalmic symptoms was Management of sellar lesions requires a multidisciplinary effort.
Endocrine tests are indispensable to identify lesions amenable to hormonal treatment. Endocrine disorders usually occurred before neurological and ophthalmological symptoms, and their identification may therefore allow for earlier diagnosis. Hormone assessment should be regularly performed during follow-up.. Craniopharyngioma is the most common lesion in the first two decades of life, and should be treated with surgery.
Prolactinoma is rare at these ages, but this does not mean that it should not be considered. Both prolactinoma and pituitary hyperplasia require medical treatment. Surgery is also not required for other lesions in this area, such as germinal tumors, for which chemotherapy and radiation therapy are the treatment of choice, and optic tract gliomas, which often require no treatment at all.
Although most lesions are histologically benign, they are associated to high morbidity rates because of their proximity to vital structures. They are usually diagnosed based on visual or neurological changes caused by a mass effect.
In addition, they are associated to endocrine conditions including hypothalamic and pituitary disease due to either hyposecretion or hypersecretion of some hormone. These are very often the first manifestations of the lesion. Our study objectives are to describe the presentation forms of sellar area lesions in children and adolescents, to analyze the presence of endocrine diseases at presentation and during their course, and to emphasize the importance of endocrine assessment, identifying lesions amenable to hormone therapy.
A retrospective study was conducted of patients under 14 years of age referred to the pediatric endocrinology unit of a tertiary hospital for a sellar mass during a year period — Lesions had been diagnosed by magnetic resonance imaging MRI. The following variables were collected from the clinical history of each patient: sex, age at diagnosis, nature of the lesion, clinical presentation symptoms and their onset time, diameter of the longest axis of the mass at diagnosis and in MRI, primary treatment, and presence of endocrine diseases at diagnosis and during the follow-up period.
Clinically presented symptoms and signs were defined as those that were first related to the lesion leading to seek medical help, but all signs and symptoms reported or seen before diagnosis were also collected.
Presentation forms were categorized depending on whether signs and symptoms were due to neuropathy headache, signs of cranial hypertension, seizures, focal signs , ophthalmopathy decreased acuity or visual field, strabism, nystagmus , or endocrine diseases. The prior period with clinical signs and symptoms was defined as time from the start of symptoms attributable to the lesion even if they did not lead to seek medical help to lesion diagnosis.
Endocrine diseases included those of hypothalamic origin obesity and eating, thirst, sleep or temperature disorders and pituitary conditions due to hyposecretion of any hormone from the anterior pituitary gland growth hormone [GH] deficiency, hypocortisolism, central hypothyroidism and hypogonadism or the posterior pituitary gland central diabetes insipidus , or due to hormone hypersecretion hyperprolactinemia, early puberty, and gigantism.
To diagnose obesity, age- and sex-specific thresholds of body mass index were used. Standard of care clinical and laboratory assessments were performed in the patients at lesion diagnosis except in three patients with craniopharyngioma, prolactinoma, and dermoid cyst respectively, in whom they were performed after surgical resection and every six months thereafter.
Prior periods with neuro-ophthalmological and endocrine clinical signs were compared using a Wilcoxon test, and the proportions of patients with endocrine disease in each group by type of lesion were compared using a Chi-square test.
Forty-five patients 25 females with age at diagnosis ranging from 3 months to The greater lesion diameter was longer than 20 mm in 29 patients. Lesion nature was known in 39 patients: 13 optic tract gliomas 5 in patients previously diagnosed with neurofibromatosis type 1 , 10 germinal tumors, 6 craniopharyngiomas, 4 prolactinomas, 4 histiocytic granulomas, one dermoid cyst, and a case of pituitary hyperplasia secondary to congenital primary hyperparathyroidism diagnosed very late, at 9 years of age.
Table 1 shows the variables at diagnosis in the total cohort and by type of lesion. Variables of sellar lesions at diagnosis in the complete cohort and by type. Four patients were successfully treated at the endocrinology department: 3 prolactinomas with the dopamine agonist cabergoline, while the patient with pituitary hyperplasia received levothyroxine. A year-old boy complaining of headache underwent surgery with no prior hormone testing because neuroimaging revealed a lesion suspected to be craniopharyngioma because of its cystic appearance and large suprasellar extension Fig.
The pathological laboratory diagnosed prolactinoma, and the cystic area was due to tumor apoplexy. The three patients with medically treated prolactinomas, all of them aged 13 years, were two pubertal girls complaining of isolated galactorrhea and with adenomas less than 10 mm in size, and a boy reporting headache for the past few months and who had another cystic macroadenoma.
Medical history of males included no evidence of endocrine disease that could have allowed for earlier diagnosis; they had not started puberty, and one of them showed mild bilateral gynecomastia 15 mm in diameter, which could be considered physiological at this age.
MRI with gadolinium contrast showing an intrasellar and suprasellar expansive mass with a cystic area. Surgery was performed for suspected craniopharyngioma, and pathological examination found a prolactinoma with a necrotic area inside. Only 11 patients required surgery for the mass, and two of these subsequently needed chemotherapy and radiotherapy. Hormone testing also revealed four tumors secreting chorionic gonadotropin.
Diagnosis of germinoma was therefore established without the need for biopsy, and these patients were directly referred to the oncology department with no prior surgery. Reasons for consultation were that the symptoms related to neuropathy and ophthalmopathy in 25 patients, and symptoms were related to endocrine disease in 13 patients: diabetes insipidus in 6, early puberty in 3, galactorrhea in 2, low growth in one, and weight gain in one.
Seven lesions were subclinically diagnosed by neuroimaging, including 2 in workup study for neurofibromatosis type 1 and 5 that may be considered as incidentalomas, because problems not related to sellar disease epilepsy, psychomotor retardation, syncope were being investigated. Mean duration of prior endocrine symptoms was Half of the 24 patients with endocrine disorders at diagnosis 13 patients in whom they led to consultation and 11 patients in whom they were discovered by directed history had more than one endocrine disorder.
The seven endocrine conditions which developed over time occurred in 3—9 years 5 cases of early puberty and one each of central hypothyroidism and central diabetes insipidus. All patients had a single endocrine disease, except for the only patient who had received radiotherapy, who had both central early puberty and hypothyroidism after 8 and 9 years respectively. Presence of endocrine disease at diagnosis of sellar lesions, one year later, and at the end of the follow-up period in the complete cohort and by type of lesion.
Complete anterior and posterior pituitary deficiency was found in 9 of 10 germinal tumors. All these lesions were greater than 20 mm in size in neuroimaging at diagnosis. A single patient, who consulted for isolated diabetes insipidus and in whom was found pituitary stalk enlargement in neuroimaging, developed anterior deficiency three years later, consistent with tumor growth, before chemotherapy and radiotherapy were started.
Panhypopituitarism including central diabetes insipidus was found at diagnosis in 3 of the 6 craniopharyngiomas. Panhypopituitarism occurred immediately after surgery in the other 3 cases Fig. Patient complained of headache and vomiting, and directed clinical history found polyuria lasting longer than 2 years. Our data emphasize the importance of adequate identification of sellar lesions amenable to hormone treatment in children and adolescents and recognition of the symptoms and signs of endocrine disease to allow for early diagnosis and adequate management.
Although adenomas are rare in children, prolactinoma should mandatorily be ruled out because it responds to medical treatment.
Prolactinoma mainly occurs in children older than 12 years, but has also been reported in prepubertal children. Thus, when any sellar or parasellar mass is found, even in prepubertal children and regardless of radiographic appearance, prolactin levels should be measured before considering surgery. Although the former are usually solid and the latter cystic, long-standing adenomas may grow and have cystic areas due to tumor apoplexy tumor necrosis or hemorrhage. Dopamine agonists, mainly cabergoline, are the first choice for the treatment of prolactinoma because they significantly decrease tumor size in more than half the cases and normalize prolactin levels and restore gonadal function in more than three-quarters of the patients, preserving the remaining pituitary function.
Surgery is therefore reserved for patients intolerant or resistant to medication. Chorionic gonadotropin is another hormone which should be tested before deciding lesion management. Almost half of the hypothalamic germinal tumors secrete chorionic gonadotropin, together with alpha-fetoprotein. Chemotherapy and radiotherapy are the treatment of choice for these tumors, and presence of these markers makes therefore biopsy unnecessary.
As seen in our series, they usually cause hypopituitarism early, particularly central diabetes insipidus, often associated to anterior pituitary gland deficiencies. The few patients who have no panhypopituitarism at diagnosis experience it after oncological treatment.
After surgery, virtually all the patients experience complete panhypopituitarism. In a recently reported large series, two-thirds of the patients reported endocrine symptoms within a mean of 6 months of the start with space-occupying symptoms. It should be noted that many patients in our series had very long-standing 1—5 years central diabetes insipidus and had not been referred for workup by their physicians, and in whom diabetes mellitus and renal or electrolyte disorders had only been ruled out.
They are usually pilocytic astrocytomas of low-grade malignancy which often do not progress and even regress. They have usually no associated endocrine conditions at baseline, except in previously undiagnosed patients who attended the clinic for central early puberty or weight gain but no pituitary deficiencies.
They may however develop over time, more probably if chemotherapy or radiotherapy has been administered, but also when no treatment has been administered. One-fourth of the patients younger than 18 years with histiocytosis have pituitary deficiencies over the first 10 years. Sheehan's syndrome. Hyperprolactinemia is the most common manifestation. They can be clinically silent and detected incidentally on MRI performed for other reasons.
Patients may have symptoms due to compression of adjacent structures, particularly the optic chiasm and optic nerves. Upon immediate imaging after contrast enhancement, they are typically hypointense on T1-weighted imaging relative to the remaining normal pituitary tissue. These lesions are isointense to gray matter on T1- and T2-weighted imaging and demonstrate homogenous enhancement. Adenomas can expand cephalad into the suprasellar cistern and compress the optic chiasm and optic nerves, caudally to erode the sella turcia, and laterally to compress or invade the cavernous sinuses.
Craniopharyngiomas arise from remnants of Rathke's pouch along the path of the craniopharyngeal canal. They can be seen in children peak at years and adults fifth through seventh decades.
Craniopharyngiomas can cause symptoms such as visual disturbances, endocrine abnormalities, motor deficits and increased intracranial pressure. The main histologic subtypes are adamantinomatous and squamous papillary. Adamantinomatous subtypes are more often encountered in the suprasellar region in children.
They are predominantly cystic, lobulated calcifications and they are highly likely to recur. The squamous papillary subtype presents more frequently in adults. These craniopharyngiomas are predominantly solid, spherical and they occur in either the intrasellar or suprasellar regions. Enhancement can be solid or nodular Figure 8. Meningiomas in this region originate from the tuberculum sella, anterior clinoid processes, diaphragma sellae, planum sphenoidale and upper clivus Figure 9.
They can cause osseous changes including hyperostosis and erosion. As meningiomas in other locations, they are isointense to slightly hypointense on T1-weighted imaging. They are isointense to slightly hyperintense on T2-weighted imaging and they enhance homogenously and intensely.
The vascular supply to these lesions is from the carotid meningeal and ophthalmic arteries. The infundibulum has a funnel-shaped contour 3 to 3. A variety of inflammatory such as giant-cell granuloma and Langerhans cell histiocytosis and neoplastic lesions can involve the infundibulum.
These lesions often involve the hypothalamus and can result in diabetes inspidus. Langerhans cell histiocytosis LCH is the most common pediatric infundibular tumor Figure Neoplastic processes that can enlarge the stalk include lymphoma Figure 11 , metastases, germinoma and teratoma.
They may have a dumbbell morphology from invasion through the diaphragma sella. Primary brain tumors such as medulloblastoma, pineoblastoma and germinoma, and lymphoma and leukemia may spread to the infundibular or suparsellar region via cerebrospinal fluid CSF seeding.
Germinomas Figure 13 are more commonly seen in the pediatric population, presenting with diabetes inspidus, hypopituitarism and optic chiasm compression. There may be coexistent pineal masses, and there may be subarachnoid spread. Ectopic neurohypophysis can be caused by trauma or neoplastic processes, which disrupt the transport of hormones from the hypothalamus to the neurohypophysis. Congential ectopic neurohypophysis is associated with other midline abnormalties such as septo-optic dysplasia Figure The expected posterior pituitary bright spot is frequently located in the suprasellar region.
This can lead to growth hormone deficiency. In the extreme manifestation of this abnormality, i. The posterior pituitary bright spot may be absent.
Patients may present with visual field impairment and headache due to mass effect on adjacent structures. Given their significant prevalence in the general population, more studies are needed to evaluate the natural history of these masses and to help allocate appropriate resources for their management. Keywords: pituitary adenomas; pituitary tumo. Abstract Background: Sellar masses SM are mostly benign growths of pituitary or nonpituitary origin that are increasingly encountered in clinical practice.
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