Papillary Craniopharyngioma Presenting as a Purely Intraventricular Lesion in an Adult EE-21

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Presented by: Robert D. Stoffey, D.O.¹ Clark W. Sitton, M.D.¹ Roy F. Riascos, M.D.¹ Meenakshi B. Bhattacharjee, M.D., MBBS² ¹ University of Texas at Houston Department of Neuroradiology ² University of Texas at Houston Department of Neuropathology

American Society of Neuroradiology Washington, D.C. 54th Annual Meeting 23-26 May 2016

We Present a Case of a Pathologically-Proven Papillary Craniopharyngioma Presenting as a Purely Intraventricular Lesion in an Adult Male.

Case History: A 35-year old male presented to an outside ER reportedly with a two-day history of falls and confusion.

Upon transfer to our institution further history indicated a two-year course of progressive short-term memory loss and a general decline in cognitive functions. More recently he had developed worsening positional headaches, which he rated as 10 out of 10. The headaches were associated with nausea and vomiting.

Neuro Cross-Sectional Imaging including both CT & MRI were performed.

MRI Findings: MRI demonstrated a 51 x 34 x 35 mm solid mass arising within the Third Ventricle and extending through the foramina of Monro into both lateral ventricles. Coronal T2 imaged is displayed on the right.

The mass was felt to originate from the lateral anterior margin of the third ventricle. The third ventricle was markedly expanded and there was obstructive hydrocephalus, likely at the foramina of Monro, resulting in effacement of the basilar cisterns and downward translation of the posterior fossa structures.

T2 Weighted Images Coronal Axial

Pre-Contrast Post-Contrast The Third Ventricular Mass Enhanced Homogeneously as demonstrated on these Axial T1 Weighted Images Pre-Contrast Post-Contrast

T1 Weighted Post-Contrast Images Again Demonstrate Avid & Homogeneous Enhancement Coronal Sagittal

Sagittal T1 Weighted Images Pre-Contrast Post-Contrast

The Papillary Craniopharyngioma demonstrated No Restricted Diffusion DWI ADC

Scattered Areas of Susceptibility were Noted on the GRE Sequences

Axial Sag-Reformatted Non-Contrast Head CT Demonstrated a Heterogeneous, Intraventricular Mass Axial Sag-Reformatted

The patient underwent successful transcranial resection of his third ventricular mass and had a relatively uneventful post-operative recovery.

The final tissue diagnosis was: Papillary Craniopharyngioma WHO Grade I of the Third Ventricle.

Histopathology of Papillary Craniopharyngioma: Characterized by Proliferation of non-keratinizing squamous epithelium in a papillary configuration with fibrovascular cores. Does NOT demonstrate Calcifications, Nodules of Wet Keratin, or Stellate Retinaculum, all of which are characteristic of the more common Adamantinomatous subtype of Craniopharyngioma.

Histopathology of Papillary Craniopharyngioma

While as many as 40% of Papillary Craniopharyngiomas may involve the third ventricular cavity secondarily. Primary origin from the third ventricle is rare, accounting for less than 1% of such masses in adults. Nonetheless Papillary Craniopharyngiomas should be considered in the differential diagnosis of adult third ventricular masses when a homogeneously enhancing mass, demonstrating no restricted diffusion, is encountered.

Craniopharyngiomas are benign, slow growing neoplasms felt to arise from epithelial remnants of Rathke’s pouch. 75% are primarily suprasellar. 25% have at least a small intrasellar component. Craniopharyngiomas are nearly equally common in children as in adults and demonstrate a Bimodal Age Range of approximately 5-10 & 50-60 years of age.

Craniopharyngiomas: Two Histologic Subtypes: 90%: Adamantinomatous 10%: Papillary Craniopharyngiomas demonstrate a Propensity to recur following surgery, particularly in larger lesions and in cases where subtotal resection is not possible.

The Differential Diagnosis of Adult Intraventricular Masses includes a number of neoplasms including but not limited to: -Ependymoma ( our presumed diagnosis) -Central Neurocytoma -Subependymoma -Meningioma -Choroid Plexus Papillomas/Carcinomas -Germ Cell Tumors -Metastases

References: - Mortini P, Losa M, Pozzobon G, et al References: - Mortini P, Losa M, Pozzobon G, et al. Neurosurgical treatment of craniopharyngioma in adults and children: early and long-term results in a large case series. J Neurosrug 2011; 114: 1350-1359. - Acta Cytol. 2005. Jul-Aug; 49(4): 43-4. Intraventricular squamous papillary craniopharyngioma: Report of a case with intraoperative imprint cytology. Madhaven M1, P JG, Abdullah Jafri J, Idris Z. - Neurol Med Chir (Tokyo). 1992 Dec; 32(13): 972-5. Papillary craniopharyngioma of the third ventricle – case report. Tada M1, Aida T, Koiwa M, Chono Y, Kashiwaba T, Abe H.

References (continued): - Glastonbury CM, Osborn AG, Salzman KL References (continued): - Glastonbury CM, Osborn AG, Salzman KL. Masses and Malformations of the Third Ventricle: Normal Anatomic Relationships and Differential Diagnoses. Radiographics 2011; 31: 1889-905. - Crotty TB, Scheithauer BW, Young WF, et al. Papillary craniopharyngioma: a clinocopathological study of 48 cases. J Neurosurg 1995; 83: 206-14.