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Pituitary Gland Pathology J W Ironside 561 J Clin Pathol: first published as 10.1136/jcp.56.8.568 on 30 July 2003. Downloaded from Best Practice No 172 Pituitary gland pathology J W Ironside ............................................................................................................................. J Clin Pathol 2003;56:561–568 This article reviews published evidence on the diagnosis lesions that are encountered from within and and classification of pituitary gland tumours and the around the pituitary gland, to assess current diagnostic pathological techniques, and to ex- relevance of histological and genetic features to plore the expanding evidence base for the classi- prognosis. Much of the literature is devoted to the fication of pituitary adenomas and the clinical histological, ultrastructural, and immunocytochemical relevance of these approaches. classification of pituitary adenomas (extensively OVERVIEW OF PITUITARY REGION supported by multicentre studies), with little consensus PATHOLOGY AND CLINICAL on the identification of prognostic features in adenomas, SYMPTOMATOLOGY particularly in relation to invasion. There is a lack of The pituitary gland can be affected by a wide range of lesions, which may arise either within correspondence between clinical and pathological the gland itself or its surrounding structures criteria to identify and classify invasion, and a need to (table 1).25712 These lesions all tend to present reassess the nomenclature and diagnostic criteria for with clinical symptoms and signs that relate either to endocrine dysfunction of the pituitary invasive adenomas and carcinomas. Recent cytogenetic, gland, the local effects of an expanding mass in genetic, and molecular biological studies have identified the region of the pituitary gland on adjacent no consistent abnormalities in relation to pituitary tumour structures (particularly the optic nerves and chiasm), or the non-specific effects of an expand- progression, although many genes are likely to be ing intracranial mass associated with raised 24 involved. In light of these uncertainties, an approach to intracranial pressure. The clinical symptoms are http://jcp.bmj.com/ the diagnosis and classification of pituitary adenomas is of course also dependent on the underlying nature of the lesion, its size and extension beyond suggested, based on robust criteria from earlier studies the sella turcica, and the consequent effects on and incorporating provisional data that require adjacent structures beyond this region. reassessment in large prospective studies with an adequate clinicopathological database. “Larger lesions can produce signs and symptoms of raised intracranial pressure, on September 25, 2021 by guest. Protected copyright. .......................................................................... of which headache is the most common manifestation” ntracranial lesions arising in the region of the Small lesions confined to the pituitary gland pituitary gland are relatively common; the most may be asymptomatic (see below for “silent” frequent of these are pituitary adenomas, I adenomas), or in the case of functioning adeno- which account for 6–10% of all symptomatic mas will present with signs and symptoms of intracranial tumours.1–3 However, the pituitary endocrine dysfunction (either excess or reduced gland can be involved by a wide range of other production of one or several anterior pituitary pathological processes arising both within the hormones), particularly in younger patients.124 gland itself and in the surrounding structures: the Clinical biochemistry laboratories in centres with skull in the region of the sella turcica, dura mater, an endocrine clinic are able to provide rapid and blood vessels, cranial nerves, and brain.4–6 Ad- comprehensive assays for pituitary hormones and ....................... vances in neuroimaging have increased the sensi- will also help with pituitary suppression or tivity of detection of small lesions in the pituitary Correspondence to: 2 6–8 stimulation tests. Larger lesions with suprasellar Professor J W Ironside, region, which are now readily accessible to Division of Pathology, neurosurgeons following recent developments in School of Clinical and trans-sphenoidal surgery, including microsurgery ................................................. Molecular Medicine, and endoscopic resection of small intrasellar Abbreviations: ACTH, adrenocorticotrophic hormone; University of Edinburgh, 2 9–11 CI, computerised tomography; FGF, fibroblast growth Western General Hospital, lesions. Consequently, pathologists now en- factor; GH, growth hormone; MEN-1, multiple endocrine Edinburgh EH4 2XU, UK; counter an increasing number of lesions for diag- [email protected] nosis both within and around the pituitary gland, neoplasia type 1; MRI, magnetic resonance imaging; PRL, prolactin; ptd-FGFR-4, pituitary tumour derived N Accepted for publication which comprise an ever widening spectrum of terminally truncated isoform of fibroblast growth factor 512 5 May 2003 pathological processes. The aim of this article is receptor-4; PTTG, pituitary tumour transforming gene; TSH, ....................... to provide a brief review of the clinical features of thyroid stimulating hormone www.jclinpath.com 562 Ironside lesions as small as 2–3 mm can be routinely detected.8 Table 1 Overview of pituitary region tumours Because the incidence of apparently clinically “silent” J Clin Pathol: first published as 10.1136/jcp.56.8.568 on 30 July 2003. Downloaded from Tumours within the pituitary gland pituitary microadenomas has been estimated to be around Adenoma 20% in the general population (rising to over 30% in the eld- Metastatic carcinoma erly), a continuing increase in the detection of these small Carcinoma Secondary lymphoma/leukaemia lesions can be anticipated, although their clinical manage- 1–3 Craniopharyngioma ment is controversial. MRI is also useful for studies of mac- Germ cell tumours (particularly germinoma) roadenomas and other large lesions, because it is better able Granular cell tumour/spindle cell granular oncocytoma to identify changes in the size of the gland, distortion of the Gangliocytoma/ganglioglioma pituitary stalk, elevation of the dura mater comprising the Pituicytoma Meningioma diaphragma sellae, and the position of the carotid arteries, 78 Lymphoma the optic chiasm, and the cavernous sinus. The extent of the Non-neoplastic lesions involving the pituitary gland suprasellar and parasellar extensions is also more clearly Nodular hyperplasia visualised. Epidermoid, dermoid, and Rathke cleft cysts This brief summary of clinical and investigative features Abscess Tuberculoma indicates that it is essential to obtain a full history for any Sarcoidosis patient who is undergoing surgery for resection of an Histiocytosis (Langerhans and non-Langerhans) intrasellar or parasellar mass, particularly if a pituitary Lymphocytic hypophysitis adenoma is suspected. The clinical and radiological features Tumours arising from adjacent structures should be supplemented with the results of biochemical Optic chiasm/nerve: pilocytic astrocytoma, meningioma Cranial nerves: schwannoma investigations for pituitary hormones in the blood, because Hypothalamus: gangliocytoma, pilocytic astrocytoma these can obviously provide much useful information Sella turcica: meningioma, paraganglioma, chordoma/ concerning the probable nature of a pituitary region tumour chondroma/chondrosarcoma before a pathology consultation request, particularly for intraoperative diagnosis. It is also essential to obtain details of medical treatment of the adenoma before surgery, because treatment can cause considerable changes in the morpho- extensions often present with visual field defects, such as logical appearances of the tumour—for example, the bitemporal haemianopia caused by pressure on the optic chi- changes seen in a prolactinoma after treatment with 14 asm, particularly the decussating fibres.25Larger lesions can bromocryptine. produce signs and symptoms of raised intracranial pressure, of which headache is the most common manifestation. Cranial nerve palsies may occasionally result from a large INTRAOPERATIVE DIAGNOSIS AND SPECIMEN expanding sellar mass, and can vary from an isolated third HANDLING nerve palsy to combined fourth, fifth, and sixth cranial nerve Although many pituitary adenomas can be diagnosed after palsies if the cavernous sinus is invaded.5 An upward exten- clinical, biochemical, and radiological investigations (and sion from an expanding sellar mass can cause distortion and small intrasellar tumours producing PRL or growth hormone compression of the pituitary stalk and/or hypothalamus, (GH) may be treated medically if there are no other http://jcp.bmj.com/ 2 which in turn compromises the release of dopaminergic pro- indications for surgery ), neurosurgical management will lactin (PRL) inhibition, resulting in an increase in serum PRL often require intraoperative diagnosis to confirm the presence (although usually not above 150 ng/ml).12 This may be the of a tumour in the resected tissue. This can help at the time only endocrine abnormality in patients with large non- of surgery to distinguish adenomatous from non- functioning adenomas. Distortion and invasion of the adenomatous pituitary tissue, and to ensure that for larger pituitary stalk may also compromise the portal blood supply lesions extending beyond the sella turcica no other type of neoplasm is present for which further neurosurgical proce- to the anterior pituitary gland, resulting in infarction
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