Clinical presentation and preoperative work up Nonfunctioning tumors are rare in childhood and children mainly present with endocrinological symptoms. [pediatricneurosciences.com]

With ISG visual disturbances were present in 47% of cases at presentation. In front of SGPA, patient present most frequently acromegalic manifestations (67%), followed by visual manifestations in 29%. [atlasofscience.org]

In children, hypopituitarism commonly presents with delayed puberty or impairment of growth. Diabetes insipidus is rarely a presenting feature but may occur following surgery for a pituitary adenoma. [patient.info]

Hormonal imbalance due to overproduction tends to present earlier and tumors are thus usually small at presentation. This mode of presentation is discussed in the article on pituitary microadenomas. [radiopaedia.org]

Case Presentation A 12-year-old child presented with headaches for 3 months, gradual onset of blurred vision, and strabismus in the right eye. The patient had bitemporal hemianopia and severe vision loss in the right eye on exam. [jaocr.org]

• Short Stature

  Common complaints include headache, increased intracranial pressure, vision changes, short stature and delayed puberty in children, decreased libido, amenorrhea, diabetes insipidus or behavioral changes from tumor expansion and mass effect. [healio.com]

  Clinical presentation and preoperative work up They often present with features of raised intracranial tension (ICT) and endocrine dysfunction (delayed puberty, short stature) in children apart from visual impairment which is more common presentation [pediatricneurosciences.com]

  Growth hormone-secreting adenomas can increase ICP in a fashion similar to those in children taking growth hormone for short stature or patients with Turner syndrome who develop idiopathic intracranial hypertension. [eyewiki.aao.org]

• Anosmia

  Cerebrospinal fluid rhinorrhea or anosmia can occasionally occur, especially if excessive retraction of the frontal lobe is indicated. 19 Schmidek and Sweet Operative Neurosurgical Techniques Indication [clinicalgate.com]

  Anosmia and hyponosmia is a frequent and under recognized minor complication. Fibrosis of neuroepithelium, poor aeration of olfactory receptors, and sinusitis likely contribute. [thamburaj.com]

  An undue superior dissection can cause anosmia (44, 92) and can injure the cribriform plate and cause a CSF leak(54). Vertical orientation is best achieved with televised fluoroscopy (30, 36, 52). [pituitary.org]

• Osteoporosis

  A prolactinoma is the most common pituitary tumor and may cause amenorrhea, irregular periods, galactorrhea, infertility in women and osteoporosis. It may cause hypogonadism, loss of libido, and impotence in men. [emedicine.medscape.com]

  Osteoporosis is present in nearly 40% of patients with Cushing's disease and, pathological fractures are not uncommon. Therefore, care should be taken while positioning these patients. [jnaccjournal.org]

  Cortisol and other glucocorticoids have many systemic effects, which include immunosuppression, diabetes due to increased gluconeogenesis, hypertension caused by partial mineralocorticoid effects, osteoporosis from inhibition of bone formation and calcium [eyewiki.aao.org]

  Clinical features of Cushing's syndrome Feature Frequency (%) Obesity 92 Moon facies 90 Hypertension 85 Glucose intolerance 80 Menstrual and sexual dysfunction 74 Hirustism and acne 70 Striae 70 Proximal muscle weakness 67 Osteoporosis 55 Easy bruising [glowm.com]

• Diplopia

  Patients will often complain that they have trouble with anteroposterior tasks such as cutting fingernails, threading needles, and working with precision tools. [23] Diplopia Diplopia may be due to cranial nerve paresis from lateral extension of tumors [eyewiki.aao.org]

  Visual symptoms and signs include vision loss, diplopia, and multiple cranial nerve palsies secondary to rapid expansion of the tumor into the adjacent cavernous sinuses, with cranial nerve III the most commonly affected. [webeye.ophth.uiowa.edu]

  […] of below the knee amputation 3 months prior for Ewing's sarcoma [Figure 1], presented to her primary care physician, with left hemicranial headache and rapidly progressive diminution of vision in the left eye along with drooping of eyelid, binocular diplopia [ruralneuropractice.com]

  Other features Visual symptoms are generally related to compression of visual pathways and include bitemporal visual-field loss, which is denser from the superior to inferior than in other orientations, color desaturation, diplopia, and ophthalmoplegia [emedicine.medscape.com]

  However, the lack of a binocular temporal field may also cause difficulties with depth perception for near vision and diplopia. [jnnp.bmj.com]

• Hemianopsia

  His vision postoperatively showed a persistent bitemporal hemianopsia, but with significant improvement in visual acuity to 20/40 in the right eye and 20/20 in the left eye. [healio.com]

  Humphrey visual field 24-2 showing a bitemporal hemianopsia. Figure 2. Goldmann visual field showing a bitemporal hemianopsia. [27] Neuroimaging The pituitary gland is best seen on coronal sections and normally enhances with contrast. [eyewiki.aao.org]

  Visual field analysis demonstrated bitemporal hemianopsia. [pituitary.mgh.harvard.edu]

  The anatomy of this structure causes pressure on it to produce a defect in the temporal visual field on both sides, a condition called bitemporal hemianopsia. [en.wikipedia.org]

  PRL levels were relatively low (90.7 ng/ml) and visual field perimetry confirmed temporal hemianopsia in the left eye. [hormones.gr]

• Unilateral Proptosis

  Ewing's sarcoma of the orbit with intracranial extension: A rare cause of unilateral proptosis. J Pediatr Neurosci 2011;6:36-9. [Full text] 3. Weins AL, Hattab EM. The pathological spectrum of solid CNS metastases in the pediatric population. [ruralneuropractice.com]

• Loss of Libido

  Signs and symptoms of prolactinoma include oligomenorrhea or amenorrhea, reduced fertility, loss of libido, and erectile dysfunction. [fortherecordmag.com]

  It may cause hypogonadism, loss of libido, and impotence in men. Tumors that secrete excess GH cause gigantism in children and acromegaly in adults. [emedicine.medscape.com]

  Loss of libido. Erectile dysfunction. Galactorrhea in the estrogen-primed female breast. [cancer.gov]

• Aggressive Behavior

  An event associated with neoplastic progression and aggressive behavior. Am. J. Pathol. 151, 769–784 (1997). 89. Gadelha, M. R., Trivellin, G., Hernandez Ramirez, L. C. & Korbonits, M. Genetics of pituitary adenomas. Front. Horm. [nature.com]

• Headache

  They present with headaches, visual field defects and hypopituitarism (including growth failure, as often present in childhood and adolescence). Other causes of headache, visual field defects, visual disturbance and endocrine dysfunction. [patient.info]

  Amongst the types of headaches experienced are both chronic and episodic migraine, and more uncommonly various unilateral headaches; primary stabbing headache, [6] short-lasting unilateral neuralgiform headache attacks with conjunctival injection and [en.wikipedia.org]

  A 45-year-old man was seen for urgent evaluation of worsening headaches and loss of vision. He first noticed his symptoms 6 months before the onset of recurrent headaches with blurry and blank spots in his vision. [healio.com]

  Headaches associated with Rathke′s cleft cyst. Headache 2006;46:1580-6. 6. Billeci D, Marton E, Tripodi M, Orvieto E, Longatti P. Symptomatic Rathke′s cleft cysts: A radiological, surgical and pathological review. Pituitary 2004;7:131-7. 7. [mjdrdypu.org]

  Case Presentation A 12-year-old child presented with headaches for 3 months, gradual onset of blurred vision, and strabismus in the right eye. The patient had bitemporal hemianopia and severe vision loss in the right eye on exam. [jaocr.org]

• Bitemporal Hemianopsia

  His vision postoperatively showed a persistent bitemporal hemianopsia, but with significant improvement in visual acuity to 20/40 in the right eye and 20/20 in the left eye. [healio.com]

  Visual field analysis demonstrated bitemporal hemianopsia. [pituitary.mgh.harvard.edu]

  Humphrey visual field 24-2 showing a bitemporal hemianopsia. Figure 2. Goldmann visual field showing a bitemporal hemianopsia. [27] Neuroimaging The pituitary gland is best seen on coronal sections and normally enhances with contrast. [eyewiki.aao.org]

  The anatomy of this structure causes pressure on it to produce a defect in the temporal visual field on both sides, a condition called bitemporal hemianopsia. [en.wikipedia.org]

  Visual diminution and bitemporal hemianopsia is seen in 85-90% patients. They also present with endocrinopathies (hypopituitarism or precocious puberty); features of raised ICT may be present due to hydrocephalus. [pediatricneurosciences.com]

• Neurologic Manifestation

  The neurological manifestations of hypothyroidism include carpal tunnel syndrome, slowing of cerebration, and, less commonly, myotonia and myopathy. [jnnp.bmj.com]

[…] massive MRI: High signal intensity on T1 weighted images Heterogeneous enhancement Fluid levels consistent with cystic components CT: Better than MRI at showing calcifications Laboratory Same for both types of craniopharyngiomas Full pituitary endocrine workup [pathologyoutlines.com]

While a majority of patients with endogenous Cushing syndrome have thyrotrophic adenomas (called Cushing Disease - see below), other tumors should also be considered as part of the endocrinologic workup, including adrenal tumors and especially ACTH-secreting [eyewiki.aao.org]

It is important to note that while a sellar mass may present with bitemporal hemianopia, the combination of findings described above describing a junctional scotoma also localizes to the sellar area, and additional workup is indicated. [webeye.ophth.uiowa.edu]

Dynamic pituitary MRI has high sensitivity and specificity for the diagnosis of mild Cushing's syndrome and should be part of theinitial workup. Horm Metab Res. 2007;39(6):451-456. Simonetta AB. Imaging of suprasellar and parasellar tumors. [appliedradiology.com]

[…] in its presentation as a rare case of sellar metastasis of Ewing's sarcoma in pediatric age group which was initially indistinguishable radiologically from pituitary macroadenoma [20], [23] and highlights the need of appropriate medical oncological workup [ruralneuropractice.com]

Genimatas”, Athens, Greece Abstract OBJECTIVE: Dopamine agonists (DA) are the treatment of choice in patients with macroprolactinomas. Brain and optic chiasm herniation are unusual complications following treatment with DA. [hormones.gr]

[PUBMED Abstract] Pituitary Carcinomas Treatment Standard Treatment Options for Pituitary Carcinomas Standard treatment options for pituitary carcinomas include the following: Surgery. [cancer.gov]

Rapidly consult with trusted authorities thanks to new expert-opinion treatment strategies and recommendations. Zero in on the most relevant and useful references with the aid of a more focused, concise bibliography. [books.google.com]

Is surgery the best path of treatment at this time? Thank you again for your support. M. Montoya, M.D. [endotext.org]

Treatment Because these cysts are often asymptomatic only patients with clear neurological and endocrinological indications require treatment. [skullbaseinstitute.com]

Prognosis Surgical good prognosis and the recurrence rate has been reported to be less than 5%. The postoperative outcome of most patients is resolution or improvement of their symptoms. [skullbaseinstitute.com]

In view of this, emergency decompression was planned; however, patients relatives refused surgery due to expected poor prognosis. The patient finally died of cardiorespiratory failure. [ruralneuropractice.com]

For a general discussion, including epidemiology, treatment, and prognosis, please refer to the article on pituitary adenomas. Patients typically present with symptoms of local mass effect on adjacent structures (especially optic chiasm ). [radiopaedia.org]

Prognosis Remission can be obtained in up to 90% of patients with microadenomas and in about 50% to 60% of those with macroadenomas. [ 1 ] [patient.info]

Pituitary apoplexy: evaluation, management, and prognosis. Curr Opin Ophthalmol, 20 (6):456-461. Nawa, R, et al. (2008). Pituitary tumor apoplexy: a review. J Intens Care Med, 23 (2):75-90. Mindermann, T, & Wilson, CB. (1994). [webeye.ophth.uiowa.edu]

T2 shows a large aneurysm characterized by flow void is seen in the right cavernous sinus 343. 2-Ectatic ICA : -See (Intrasellar masses) 344. 3-Cavernous Sinus Thrombosis : a) Etiology b) Radiographic features 345. a) Etiology : -Most commonly results [slideshare.net]

More common than papillary craniopharyngioma (even in adults) Higher frequency in Africans and Far East Asians Sites Suprasellar: frequently extend into neighboring structures Rarely intranasal, sphenoid sinus, cerebellopontine angle and pineal region Etiology [pathologyoutlines.com]

Hypogonadism is detected in up to 96% of patients with pituitary macroadenomas (> 1 cm) and is usually associated with inappropriately normal or decreased serum gonadotropin levels, indicating central hypogonadism The etiology of hypogonadism in this [pituitary.mgh.harvard.edu]

The differential diagnosis of a cystic suprasellar mass in a child includes more common etiologies such as craniopharyngioma and Rathke’s cleft cyst, as well as less common entities such as a necrotic pituitary adenoma, cystic glioma, and suprasellar [jaocr.org]

Identification of normal structures, such as the pituitary gland, in relation to the pathology can be helpful to determine the etiology. [appliedradiology.com]

Epidemiology of central nervous system tumors in childhood and adolescence based on the new WHO classification. Childs Nerv Syst. 2001; 17 (9): 503-11[ DOI ][ PubMed ] 2. Keene DL, Johnston DL. 2015; : 9-12[ DOI ] 3. [iranjradiol.com]

For a general discussion, including epidemiology, treatment, and prognosis, please refer to the article on pituitary adenomas. Patients typically present with symptoms of local mass effect on adjacent structures (especially optic chiasm ). [radiopaedia.org]

Always WHO Grade I Tumor with palisading epithelium, "wet" keratin and stellate reticulum associated with surrounding gliosis and Rosenthal fibers Terminology Epithelial neoplasm resembling ameloblastoma or keratinizing and calcifying odontogenic cyst Epidemiology [pathologyoutlines.com]

Epidemiology [ 1 ] Pituitary tumours account for 10% to 25% of all intracranial neoplasms. Adenomas account for the largest portion of pituitary neoplasms (estimated at approximately 17%). Only a minority of adenomas are symptomatic. [patient.info]

Figure 7 : Non-contrast head CT, axial view, showing layered hemorrhages within bilateral lateral ventricles Diagnosis Compressive optic neuropathy/junctional scotoma secondary to pituitary adenoma EPIDEMIOLOGY Age and gender predilection varies depending [webeye.ophth.uiowa.edu]

Pathophysiology Hematogenous metastases to this region most frequently go to the pituitary gland, infundibulum, and tuber cinereum, because these structures lie outside the blood-brain barrier. [radiologykey.com]

The exact pathophysiology of this phenomenon is unknown; however, as most of these lesions involve the chiasm and/or diencephalon, many have proposed that disruption of visual and vestibular input contribute to this pathology. [eyewiki.aao.org]

A thorough understanding of the pathophysiology of the pituitary disease is necessary for the anaesthesiologist to be better equipped to manage these patients in the perioperative period. [jnaccjournal.org]

Nelson syndrome: comprehensive review of pathophysiology, diagnosis, and management. Neurosurg. Focus 23, E13 (2007). 120. Wagenmakers, M. A. et al. [nature.com]

It also suggests, however, that pituitary adenomas may arrive by different pathophysiologic pathways. Non-hormone-secreting pituitary tumors tend to grow slowly and insidiously. [glowm.com]

8055-8051-9 (Print) eISBN: 978-3-318-01300-9 (Online) Abstract The principles in the surgical management of sellar and suprasellar tumors are to relieve mass effect, normalize pituitary hypersecretion, preserve or restore normal pituitary function, prevent [karger.com]

At the end of surgery, the anaesthesiologist has to ensure that there is smooth emergence from anaesthesia to prevent formation of haematoma in the surgical bed as well as to prevent dislodgement of the fat graft. [jnaccjournal.org]

Meticulous surgical technique with careful hemostatis is also critical in preventing the obscuring of important anatomic details. [pituitary.org]

If an extension of tumor hooks around the optic nerve or carotid artery, this extension prevents descent of the tumor, and the tumor extension above the carotid artery or chiasm is inaccessible. 19 The anterior extension is particularly troublesome for [clinicalgate.com]

A parasellar meningioma is a potential mimic of the pituitary adenoma, but has a comparatively harder texture, preventing it from compressing at the diaphragma sella, and therefore no “snowman” appearance. [webeye.ophth.uiowa.edu]