Claudia da Costa Leite, MD, PhD Thomas Bouldin, MD

NEURORADIOLOGY-NEUROPATHOLOGY CONFERENCE

CASE 1

21 y-o female Partial epilepsy since 6 years old with poor seizure

control in the last 5 months Frontal mass diagnosed for 6 years, unchanged on

imaging studies

Ganglioglioma, WHO grade I (not large bi nucleus neuroons (arrow) and glial cells (arrow).

Ganglioglioma

Intracranial tumor composed of dysplastic neurons and neoplastic glial cells Most of them are WHO grade I and located in the

temporal lobe Often circunscribed, cortical or cortico/subcortical lesion Classical imaging: combination of intracortical cysts, and

hyperintensity on FLAIR with enhancement. Calcifications can occur in 1/3 of cases.

Urbach H. Seminars in Ultrasound CT and MRI 2008; 29: 40-46.

CASE 2

13 y-o female with diabetes insipidus,

panhypopituitarism, hypocortisolism, growth hormone deficiency and hypothyroidism

Xanthogranuloma

Tumors of pituitary and sellar region

Pituitary adenoma 82% Atypical adenoma 3.0 Pituitary carcinoma 0.1 Craniopharyngioma 3.2

Adamantinomatous 2.9 Papillary 0.3

Rathke’s cyst 2.0 Meningioma 1.0 Chordoma 0.5 Metastasis 0.6 Lymphocytic hypophysitis 0.3 Other lesions 4.1

Saeger W, Ludecke DK, Buchfelder M, Fahlbusch R, Quabbe H-J, and Petersenn S. Pathohistological classification of pituitary tumors: 10 years of experience with the German Pituitary Tumor Registry. European Journal of Endocrinology (2007) 156 203–216

Xanthogranuloma of sellar region

Rare, also know as cholesterol granuloma

Relatively more common in childhood but seen at all ages

May have suprasellar extension

Better prognosis than craniopharyngioma or Rathke cleft cyst

Etiology unclear—from craniopharyngioma, Rathke cleft cyst, or de novo

Amano K, et al. Brain Tumor Pathol. 2013;30(4):233-41.

Jung CS, et al. Acta Neurochir (Wien) (2006) 148: 473–477.

Muller HL, et al. J Clin Endocrinol Metab 97: 3935–3943, 2012.

CASE 3

79 y-o female, progressive altered mental status for 3 months

Diabetes mellitus and high blood pressure

Gliomatosis

Gliomatosis Rare, diffuse infiltrative glioma involving at least 3

cerebral lobes, usually bilateral involving cerebral hemispheres and/or deep gray matter and extending to brain stem, cerebellum and even spinal cord Can be de novo or secondary. It is a heterogenous

disease Can occur in any age Imaging ill infiltrative lesion, mass effect and variable

enhancement

Rudà et al. Curr Treat Options Neurol 2014; 16:273

CASE 4

64 y-o male with back pain and lower extremity weakness for 4-5 months Symptoms have been progressing lately

Ependymoma grade II

Ependymal tumors 54%

Ependymoma 42%

Myxopapillary ependymoma 9

Subependymoma 3

Astrocytic tumors 23

Pilocytic astrocytoma 12

Astrocytoma, grade 2 5

Anaplastic Astrocytoma 3

GBM 1

Other gliomas 2

Ganglioglioma 2

Hemangioblastoma 12

Epidermoid 5

Dermoid 2

Melanocytoma 2

Adenal cortical adenoma 1

102 intramedullary tumors at Duke between 1998-2009

Karikari, IO et al. Neurosurgery 68:188–197, 2011.

TABLE 3: Histological type of 430 primary tumors of the spinal cord, spinal meninges, or cauda equina

Histological Type No. of Tumors (%)

astrocytoma* 41 (9.5) glioblastoma 8 (1.9) oligodendroglioma & mixed oligoastrocytoma 4 (0.9) other glioma 14 (3.3) ependymoma 102 (23.7) lymphoma & plasmacytoma 18 (4.2) PNET 4 (0.9) schwannoma 91 (21.2) chordoma 7 (1.6) neurofibroma & other nerve sheath tumor 6 (1.4) meningioma 105 (24.4) hemangioblastoma 7 (1.6) teratoma, dermoid, lipoma & other benign 8 (1.9) other and unclassified 15 (3.5)

* WHO Grades I–III.

Engelhard HH, et al. J Neurosurg Spine 13:67–77, 2010

Case 5

45 y-o male with right side hearing loss. He also recently

developed headaches, double and blurry vision, and balance and gait difficulties

Plasmacytoma

Cranial plasmacytoma

Plasma cell tumors include solitary plasmacytoma, multiple plasmacytoma and multiple myeloma (MM). Plasmacytoma may convert to MM. Plasmacytoma may be intramedullary or extramedullary Bone or soft tissue mass arising from cranial vault skull

base, orbits, paranasal sinus Meningeal involvement is usually due to extension of

bone/soft tissue lesions Intraaxial brain lesions without osseous or dural contact

have been sporadicaly described

Cerase A, et al. Neuroradiology 2008; 50: 665-674.

Cranial plasmacytoma

CT: hyperdense mass, with bone erosions MRI: iso to hyperintense on T1-WI and iso-hypointense

on T2-WI Restricted diffusion is described in some cases

Cerase A, et al. Neuroradiology 2008; 50: 665-674.