Manual Sellar and Parasellar Tumors: Diagnosis, Treatments, and Outcomes

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Rob Louis, an expert in endoscopic and minimally invasive treatment of benign and malignant brain tumors, sellar and parasellar tumors and skull base tumors. Louis for many of these complex cases.

Sellar and Parasellar Metastatic Tumors

His particular expertise lies in endoscopic sinus and skull base surgery. Louis, Duma, and Kelley often work together, combining their skills and expertise to ensure the best possible outcome for patients. The multidisciplinary team also includes endocrinologists, neuro-radiologists, ophthalmologists, neurologists, pathologists and oncologists. The nurse navigator for the program is Lori Berberet, R. Physician Spotlight feature by the Pituitary Network Association, which is the largest pituitary patient support group in the country.

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All Rights Reserved. Buy As Gift. Overview Focusing on the variety of pathological processes that arise in the sellar and parasellar regions of the central skull base, Sellar and Parasellar Tumors: Diagnosis, Treatments, and Outcomes is a state-of-the-art reference written by renowned leaders in the field. It takes a disease-oriented approach to complex pathologies. The work includes contributions by multispecialty teams and details technical advances and management options for all clinicians treating patients with parasellar and sellar pathology.

Product Details About the Author. Average Review. The pituitary gland is bulky, although the stalk is not thickened. Tumours arise from the diaphragma sella, tuberculum sella, medial lesser wing of sphenoid, anterior clinoid, clivus cavernous sinus or optic nerve sheath Smith There is a strong association between meningiomas, NF multiple tumours and ionising radiation, depending on the dose applied Hartmann et al. However, malignant behaviour, although very rare, may occur with any grade of meningioma Bruna et al. Imaging features of meningiomas frequently allow pre-operative diagnosis, distinguishing them from other parasellar tumours Smith Lesions arise from the dura and have a broad attachment to the dura or fill and expand the cavernous sinus.

There may be a linear, enhancing dural tail extending along the dura away from the lesion. Bone reaction, with bone thickening and sclerosis, or expansion of the sphenoid sinus air space, may also be seen Smith Meningiomas typically are similar to grey matter in CT density and T 1 - and T 2 -weighted MR image signal intensity, enhancing homogeneously and brightly with occasional areas of diffuse calcification Smith In contrast to other parasellar tumours, meningiomas encase blood vessels and tend to narrow the lumen Young et al.


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Surgical excision, particularly in the presence of symptomatic or growing lesions, remains the treatment of choice WHO I. Malignant histology is generally associated with a poor prognosis with a survival less than 2 years Hartmann et al. The mass lies above the pituitary which is normal , involves the chiasm and extends anteriorly along the planum sphenoidale. Coronal enhanced T 1 -weighted image of a meningioma in the left cavernous sinus. The meningioma enhances avidly, expands the cavernous sinus and is causing some narrowing of the left cavernous carotid artery, which is of smaller calibre than the right carotid.

Although the majority has a major intrasellar component, about a third can extend into the parasellar region; occasionally they can be entirely suprasellar Mukherjee et al. Atypical presentations include haemorrhages into the cyst and abscess formation rarely can coexist with pituitary adenomas Mukherjee et al. They are distinguished from craniopharyngiomas histologically by having walls composed of columnar or cuboidal epithelium FitzPatrick et al.

Although no specific radiological features have been identified, Rathke's cysts appear as discrete cystic and non-enhancing lesions on MRI with variable signal intensity on either T 1 - or T 2 -weighted images; these lesions need to be differentiated from craniopharyngiomas and arachnoid cysts Mukherjee et al.

Treatment involves drainage of the fluid with or without resection of the cystic wall Mukherjee et al. Although these lesions tend not to recur, repeated resections may be required in as many as a third of patients; it has been suggested that many relapsing lesions may contain overlapping histological features with craniopharyngiomas Mukherjee et al. The role of RT for recurrent lesions has not been clearly defined Mukherjee et al. The cyst is seen to be non-enhancing and lying on the superior aspect of the pituitary gland that appears flattened.

It is causing elevation of the optic chiasm. On CT scan, the cyst contents are very similar to CSF; both lesions are often hypointense on T 1 - and hyperintense on T 2 -weighted MR images and do not enhance with contrast. Hypothalamic hamartomas are of neuronal origin and represent congenital heterotopias usually located within the tuber cinereum and mostly affecting children causing precocious puberty and epileptic seizures Judge et al. This is seen as a pedunculated mass hanging from the undersurface of the hypothalamus.

It does not enhance and has the same signal as the grey matter of the brain parenchyma. Paragangliomas are rare, usually encapsulated and benign neoplasms WHO grade I , that arise in specialised neural crest cells associated with segmental or collateral autonomic ganglia Freeman et al. Although the majority develop in the carotid body and jugular glomus, paraganglionic cells have been demonstrated in the pituitary gland and adjacent structures Steel et al.

Intrasellar and parasellar tumors - concomitant symptoms and clinical syndromes

Only seven cases of intrasellar, two cases of suprasellar and four cases of parasellar non-malignant paragangliomas have been described Boari et al. Symptoms of pituitary hormonal deficiency and mass effects are the main presenting symptoms Steel et al. Surgical excision, when possible, remains the treatment of choice; there are no data regarding the prophylactic effect of RT on tumour recurrence.

Lipomas are benign fatty tumours, derived from remnants of maldevelopment of the primitive meninx Smith ; Fig. In the sellar region, they occur as lesions adherent to the surface of the infundibulum, floor of the third ventricle or adjacent cranial nerves Smith They are usually discovered incidentally but rarely may enlarge and produce symptoms. Lipomas are all well-circumscribed, homogeneous lesions that appear identical to fat on CT and all MRI sequences, do not enhance and usually exhibit rim calcification Smith The high signal mass containing fat is seen lying on the undersurface of the hypothalamus in the interpeduncular cistern.

There is an enhancing mass expanding the left cavernous sinus which followed the course of the trigeminal nerve on other images. These are rare tumours originating from either the neurohypophysis or infundibulum and include myoblastomas, choristomas and infundibulomas Cone et al. Their firm and vascular nature, along with the lack of an obvious dissection plane between the tumour and normal brain, prohibits gross total resection Cohen-Gadol et al. Although radiation therapy has been used, the recurrence rate in those who underwent RT was not different compared with those who did not, 4.

This enhancing mass lesion involves the pituitary stalk and the median eminence.


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Gangliocytoma WHO grade I and ganglioglioma WHO grades I—II are well-differentiated, slow-growing neuroepithelial tumours comprised of neoplastic, mature ganglion cells, either alone gangliocytoma or in combination with neoplastic glial cells ganglioglioma. The correlation of anaplasia with clinical outcome is inconsistent Day , Zee et al.

Ependymomas are glial neoplasms that very rarely can develop in the parasellar region with only four cases described up to date; surgery with or without RT is the treatment of choice Karim et al. Pituitocytoma is a primary tumour of the neurohypophysis, also located at the pituitary stalk, presenting with headache and hypopituitarism. Although histologically benign, the location and vascular nature of the tumour makes surgical resection difficult Glezer et al.

Other tumours such as plasmatocytomas, brown cell tumours and melanocytic tumours have been described as cases reports Glezer et al. A number of non-neoplastic processes can also involve the parasellar region and present in a similar manner to parasellar neoplasms Table 1. Their diagnosis is usually established in the presence of relevant clinical settings and if necessary histologically. Abscesses in the region can develop following direct extension from the sphenoid sinus, cavernous sinus and CSF or secondary to bacteraemia; masses of the sella can become secondarily infected Freda et al.

Sarcoidosis can also present in a similar manner with varying degrees of hypopituitarism, DI and cranial neuropathy that in a minority can occur without evidence of sarcoidosis elsewhere FitzPatrick et al.

Neurosurgery | Sellar and Parasellar Tumors

Other granulomatous diseases that can involve the parasellar region include giant cell granulomatous hypophysitis, Wegener's granulomatosis and the Tolosa—Hunt syndrome Glezer et al. Aneurysms originating from the cavernous sinus or circle of Willis can project into the parasellar region and their appearance is mostly affected by the amount of calcification and thrombosis present within the aneurysms Zee et al.

The parasellar region can be affected by a variety of tumours. The presentation of these tumours can be similar to that of large non-functioning pituitary tumours with extensive extrasellar extension and other non-neoplastic lesions involving the same region. Although symptoms of mass effect to adjacent structures and anterior pituitary endocrine deficits do not distinguish parasellar tumours from non-functioning pituitary tumours, the presence of DI directs towards a non-pituitary aetiology.

Considering the specific morphological characteristics of MRI and CT, a presumptive diagnosis is possible in many cases of parasellar lesions. However, in the majority of cases their diagnosis involves a multidisciplinary effort including detailed endocrinological, ophthalmological, neurological and imaging procedures.

In cases of doubt, a histological diagnosis may still be required to allow appropriate treatment planning.

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Treatment involves a joint effort requiring the collaboration of different specialties. Specific hormonal replacement therapy of established or evolving endocrine deficits is required to maintain the patient's quality of life. Multidisciplinary teams for the management and support of patients with these tumours are required for better long-term results. Tumour-specific therapies are required and need to be addressed on the basis of tumoral biology. The central registration of patients seems necessary as it may provide correlates between forms of treatments and outcomes and establish prognostic factors at the pathological or molecular level.

These authors declare that there is no conflict of interest that could be perceived as prejudicing the impartiality of the research reported. This research did not receive any specific grant from any funding agency in the public, commercial or not-for-profit sector. Introduction The area immediately around the pituitary, the sellar and parasellar region, is an anatomically complex area that represents a crucial crossroads for important adjacent structures Ruscalleda Anatomical considerations The parasellar region includes, laterally, the dural walls of the cavernous sinus, a multi-lobulated venous structure containing the intracavernous portion of the internal carotid artery, cranial nerves III, IV, VI and the V 1 and V 2 branches of the trigeminal nerve Smith ; Fig.

Download Figure Download figure as PowerPoint slide. Clinical presentation Non-pituitary adenomatous parasellar lesions do not present with hypersecretory syndromes but rather with hypopituitarism or symptoms of mass effect due to compression of nearby vital surrounding structures, the severity of which depends on the location, size and growth potential of the tumours Glezer et al.

Imaging Radiological imaging of the parasellar region is challenging since the sella is a small volume region in close proximity to many complex structures Ruscalleda Variable enhancement on post-contrast images Rarely calcifications, haemorrhages solid lesions. Occasionally small cystic or necrotic components in large legions. Most cases show strong homogenous enhancement.

Cysts are present in the majority of craniopharyngiomas that tend to predominate in children containing highly proteinaceous fluid. The fluid is usually very bright on pre-contrast T 1 -weighted MRI. In adults the solid components are larger and the cysts tend to be small or multiloculated.

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MR images show a heterogeneous mass with internal septations and heterogeneous enhancement. Differ from pituitary adenomas due to differential uniform enhancing pattern. There is no contrast enhancement or surrounding oedema.

They appear to have the density of CSF, with low intensity on T 1 and high intensity on T 2 -weighted images. Calcification of the cyst is usually absent and this may help differentiate them from craniopharyngiomas.