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A 38 Year Old Man with Headaches and Blurred Vision Presented by Dr A 38 year old man with headaches and blurred vision presented by dr keshavarzi 00-06-01 1 • A 38 year old man after workup for headaches and blurred vision, was referred to endocrinologist because of abnormal MRI. • His headaches and blurred vision worsened during last mounth; He first noticed his symptoms 1 year ago • NO(weakness,sexual dysfunction, weight changes,polyuria,polydipsia,vertigo) • The remainder of his review of systems was otherwise negative. 2 • He has 2y old child • PH/E: BMI 19.8 • NO (plethora, buffalo hump ,striae, proximal muscle weakness, centripetal obesity,hyperpigmentation, acanthosis nigricans ,no enlargement of jaws or hands or feet,skin rashes 3 00-04-19 4 5 6 7 8 9 10 1400-05 1400-05 ACE EVEL 50U/L (8-65) U/L NA 148 IgG4 162 mg/l (39-864) mg/l k 4.3 AFP 2.9 IU/ml (<6.9) IU/ml Urine SG 1020 B-HCG <0.1 mIu/ml ESR 3 T4 8.9 CR 1 TSH 2.43 LH 9 (1.7-8.6)IU/L TES 7.87 (2.5-8.4)ng/ml CORTISOL 15.1 mcg/dl ACTH 60.2 Pg/ml(7.2-64) GH 0.09 ng/ml IGF1 164 ng/ml 11 00-04 Right Left 12 13 Brain CT 00-05 14 • Pituitary region mass suprasellar/parasellar/intrasellar masses : ütumors • pituitary adenoma • pituitary lymphoma • meningioma • craniopharyngioma • hypothalamic astrocytoma/glioma • chiasmatic astrocytoma • optic nerve glioma • germinoma • chordoma • dermoid (CNS)/epidermoid/intracranial teratoma • pituitary metastases(breast, lung, kidney, gastrointestinal tract and nasopharynx) • pilocytic astrocytoma of the neurohypophysis (infundibuloma) • granuloma (TB, syphilis, giant cell granuloma, eosinphilic granuloma) Pituitary region masses,Dr Mostafa El-Feky 15 ücellular infiltrates • Langerhans cell histiocytosis • neurosarcoidosis • lymphocytic hypophysitis • IgG4-related hypophysitis üother lesions • anterior circulation berry aneurysm • hamartoma (tuber cinereum hamartoma) • Rathke cleft cyst • intracranial lipoma • sphenoid sinus mucocoele • pituitary abscess • carotido-cavernous fistula 16 • Evaluation of Parasellar Lesions • differential diagnosis: vascular, inflammatory, and infectious • processes, patient age and sex, relevant clinical history and • symptoms, and other comorbid conditions can often help narrow down the differential diagnosis. 17 • Most pituitary masses are adenomas. • Most commonly encountered nonadenomatous lesions include Rathke cleft cyst, craniopharyngioma, and meningioma,with Rathkecysts accounting for up to 40% of all such masses. • For parasellar masses of uncertain origin, histologic examination of surgically excised tissue may be the best approach to yield an accurate diagnosis. 18 Key Laboratory and Imaging Tests • MRI: • Meningiomas enhance brightly and homogeneously after contrast administration. • Lesions predominantly affecting the infundibulum/stalk can be inflammatory (hypophysitis), infectious, cystic (craniopharyngiomas or Rathke’s cleft cysts), or neoplastic (germ cell tumors, meningiomas, gliomas, lymphomas, metastases from a lung or breast primary malignancy) 19 • Both hCG and alpha fetoprotein (AFP), measured in serum and CSF, diagnostic value in patients with suspected germ cell tumors, absence of markers does not exclude germ cell tumors, as many are nonsecreting. • Serum ACE levels can be measured in serum and CSF of patients with suspected sarcoidosis (elevated in about 65% of these patients). • Serum IgG4 levels are helpful in IgG4-related hypophysitis. • ANCA, including c-ANCA, are very helpful in patients with suspected Wegener granulomatosis (elevated in >90% of patients). 20 Meningiomas • second most common in the sellar and parasellar region • Sellar meningiomas are usually well circumscribed • headache and progressive visual disturbances accompanied by optic atrophy. • On MRI meningiomas are isodense on both T1-weighted and T2,in contrast to other parasellar lesions, which are usually hyperdense on T2-weighted imaging. • Dural calcification may be evident on CT scanning. 21 Craniopharyngiomas • 3% of all intracranial tumors and up to 10% of childhood brain tumors. • bimodal age distribution, between 5 and 14 years and from 50 to 74 years. • tend to recur and invade local structures and adhere to surrounding structures,including vessels. typically suprasellar and may extend into the sella. They can have cystic and solid components. Rim or nodular calcification is present . Calcification is more common in children. • Adamantinomatous • craniopharyngiomas have a greater propensity to 22 • Increased intracranial pressure results in headache, projectile vomiting, papilledema, • Only about one-third of patients are over 40 years of age, and they commonly present with asymmetric visual disturbances, including papilledema, optic atrophy, and field deficits. • On CT imaging, most children and about half of all adults exhibit characteristic flocculent or convex calcifications. 23 craniopharyngioma • diabetes insipidus is often the earliest feature • develop partial or complete pituitary deficiency. GH deficiency with short stature, diabetes insipidus,and gonadal failure are common. • There are two craniopharyngioma subtypes: adamantinomatous and papillary. • Adamantinomatous : most common , occur at all ages,predominantly young children.heterogeneous solid-cystic masses,The cysts may be multilobulated and contain liquid,Calcification is common • The papillary subtype is almost seen exclusively in adults ,predominantly solid in with a minor cystic component on imaging. 24 Germinomas • According to the 2016 (WHO) classification:GCTs(germinomas, non-germinal germ cell tumors (NGGCT) and suprasellar with male prominent). • Most patients are 10 to 21 y at diagnosis, • male to female 3:1. • Typically in the midsagittal plane;(pineal gland and the suprasellar area). • Other locations, such as the foramen of Monro and the cerebellum,are less common. • Common symptoms of GCTs : headache,dizziness, vomiting, thirst, heavy drinking, and precocious puberty. • Because of GCTs arising in the pineal region could compress the interventricular foramen with consequence obstructive hydrocephalus Pelletier J, Bartlett K. Fever, Vomiting, and Altered Mental Status in a 17-year-old Boy 25 Germinomas • Tumors in the suprasellar region interrupt axonal projections from the hypothalamus to the pituitary gland and result in endocrinopathies. • Tumor markers, including AFP and HCG, are typically nearly normal in germinomas and are often drastically elevated in NGGCTs. • Brain biopsy is required for definitive diagnosis. • Patients should undergo MRI of the brain and spine and diagnostic LP at the time of diagnosis for staging purposes. Pelletier J, Bartlett K. Fever, Vomiting, and Altered Mental Status in a 17-year-old Boy 26 Germinomas Treatment and Prognosis • Patients with more than 1 site of tumor,positive CSF cytologic findings,or outside of the CNS are considered metastatic disease. • Germinomas are exquisitely radiosensitive. • Patients with metastatic disease receive craniospinal radiotherapy; those with localized disease may undergo local radiotherapy. • Neoadjuvant chemotherapy with bleomycin, etoposide, and cisplatin or carboplatin may be used. • Survival is excellent, with 98% 5-year event-free survival among patients with metastatic germinoma. Pelletier J, Bartlett K. Fever, Vomiting, and Altered Mental Status in a 17-year-old Boy. 27 Rathke cleft cysts • Rathke cleft cyst • non-neoplastic, sellar or suprasellar epithelium-lined cysts arising from the embryologic remnants of Rathke pouch in the pituitary gland. • rare in childhood; mostly seen in adults female to male ratio~2:1 • Radiographic features • non-enhancing midline cyst within the sella arising between the anterior and intermediate lobes of the pituitary. 40% are purely intrasellar and 60% have suprasellar extension. Purely suprasellar location, although reported, is rare. Dr Mohamed Saber and Associate Professor Donna D'Souza 28 • Plain radiograph • On lateral skull x-ray, it can rarely cause sellar enlargement if reaches large size. • non-contrast CT • typically non-calcified and of homogeneous low attenuation • uncommonly it may be of mixed iso- and low-attenuation, or contain small curvilinear calcifications in the wall (seen in 10-15% of cases) • postcontrast • The signal characteristics vary according to the cyst composition, which may be mucoid or serous. 29 • MRI T1 • 50% are hyperintense (high protein content),50% are hypointense • T2 • 70% are hyperintense ,30% are iso or hypointense • T1 + (Gd) • no contrast enhancement of the cyst is seen; however, a thin enhancing rim of surrounding compressed pituitary tissue may be apparent • Treatment and prognosis • Surgical treatment is indicated for symptomatic cases. • A case series showed 18% recurrence rate after surgical resection, 30 31 • A 45-year-old man was seen for urgent evaluation of worsening headaches and loss of vision. recent weight gain and decreased libido,bitemporal hemianopsia, • MRI 2.7 cm x 2.8 cm x 3.6 cm heterogeneous mass in the suprasellar cistern and extending up to the third ventricle,mildly lobulated, predominantly hyperintense on T2, hypointense on T1and mildly hyperintense with FLAIR images, relatively intense enhancement after gadolinium with multifocal areas of hypo- enhancement seen suggestive of focal areas of cystic necrosis . Suprasellar tumor with rapid decline in vision,endocrinology/20121116/10_3928_1081_597x_20120101_03_951830 32 • Imaging and biochemical testing was consistent with a large nonfunctioning suprasellar tumor with MRI characteristics concerning for a craniopharyngioma (suprasellar origin, heterogeneous
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