33 year old male with a history of resected craniopharyngioma (12 years ago) presents after a seizure. Jess Hwang 9/27/12
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1 33 year old male with a history of resected craniopharyngioma (12 years ago) presents after a seizure Jess Hwang 9/27/12
2 Craniopharyngioma history In 2000, at age 22, he presented with headache and blurry vision was found to have a 3 x 3.2 x 3.4 cm suprasellar mass
3 Labs 2000 on presentation UA: SG Prolactin 30 (0-15) IGF ( ) FSH 2.1 LH 2.1 Total Testosterone 339 (RR ) 8AM Cortisol AM ACTH 45
4 MRI 2000 Large cystic/solid suprasellar mass exerting significant mass effect on the basal ganglia and thalamus. The mass extends into the 3 rd ventricle and herniates into the prepontine cistern.
5 Treatment Course /13/2000 Bifrontal craniotomy for resection of mass and ventriculostomy. Pathology- craniopharyngioma. Completed adjuvant EBRT w/fractionated stereotactic RT in 2001 Hormone replacement: hydrocortisone, LT4, desmopressin, testosterone
6 MRI 2008
7 HPI 9/2012 Seizure morning of admission. First episode ever. Denies headache, visual changes, fevers/chills, nausea/vomiting. No focal neurologic deficits. No altered mental status per wife. His energy level is fair. He drinks to thirst and does not endorse polyuria or polydypsia. Takes hydrocortisone 10mg q9am/10mg q9pm and synthroid 175mcg daily.
8 PMH Craniopharyngioma Pan-hypotuitarism Diet-controlled DM FHx No cancer Meds Hydrocortisone 10mg 9AM 10mg 9PM LT4 175 mcg daily SHx Lives with wife + son
9 Physical Exam Vitals: lb BMI / % HEENT: bicoronal scars, PERLLA, EOMI, visual fields grossly full, hearing intact Neck: no neck pain, +acanthosis nigricans CV: RRR, no murmurs Resp: CTAB GI: obese, soft, non-tender Neuro: A+Ox3, reflexes 2+, strength 5/5 BUE/BLE, CN II-XII grossly intact, sensation intact Psych: normal mood, affect
10 Differential Diagnosis New Seizure Structural Mass (recurrent CP, new mass) Hemorrhage Ischemic infarct Metabolic cause Hypoglycemia Electrolyte imbalance (low Na, low Ca) Infection (meningitis, encephalitis) Drugs (EtOH withdrawal)
11 Admission Labs
12 Admission Labs PM Cortisol 2.2, ACTH 13.8 LH < 0.01 FSH < 0.01 Free Testosterone 234 Prolactin (10-80) TSH 0.45 ( ) FT ( ) HbA1c 6.9 These labs were on Hydrocortisone and Levothyroxine
13 MRI Brain mm x 39 mm x 15 mm frontal mass with mass effect, overall appearance likely represents recurrence of craniopharyngioma
14 9/14/12 Bicoronal craniotomy for resection of a craniopharyngioma. Pathology- recurrent craniopharyngioma
15 Craniopharyngioma 6-8% pediatric, 1-4% adult brain tumors Peak age in adults is usually yo Second most frequent tumor in hypothalamic-pituitary axis in any age group Histologically benign, epithelial neoplasm but can have malignant course Recurrence reported in 25-70% patients
16 Clinical Questions Outcomes in childhood onset vs adult onset craniopharyngioma? Hormone deficits pre vs postop? Morbidity: Transcranial vs Transsphenoidal approach? Incidence of ectopic recurrence?
17 Eur J Endo 2005 CO vs AO European Journal of Endocrinology
18 Hormone Deficiency: Pre-op J Neurosurg 2011; 114:
19 Hormone Deficiency: Post-op J Neurosurg 2011; 114:
20 Transcranial vs Transsphenoidal Neurosurgery 2011 Sept 69(3):
21 Recurrence-free survival Absence of residual tumor Presence of residual tumor J Neurosurg 2011; 114:
22 Ectopic Recurrence Remote recurrence is rare 2 to 26 years post-surgical resection 2 patterns of spread Along the surgical tract Dissemination through CSF Retrospective analysis of 86 cases showed 4 patients (4.7%) of ectopic recurrence.
23 Post-op Hypernatremia 9/14 9/14 9/15 9/15 9/16 9/16 9/17 9/17 9/18 Na P osm U osm 217 ddavp (mg PO) IVF 0.9NS UOP PO NPO
24 Hospital course Post-op hypernatremia Discharge meds Lantus 18U qday, Novolog 6U qac Levothyroxine 175mcg daily Decadron 4mg BID taper 0.05 mg PO ddavp prn
25 Take home points Hormone deficiencies common both pre- and post-op Possible complications from TC vs TS approach Ectopic recurrence is rare but does happen so long-term follow-up of at least a decade or more of serial imaging is important to identify this early
26 References Kendall-Taylor P et al. The Clinical, Metabolic and Endocrine Features and the QOL in Adults w/childhoodonset Craniopharyngioma Compared with Adult-onset Craniopharyngioma. Eur J Endo : Erfurth EM et al. Mortality and Morbidity in Adult Craniopharyngioma. Pituitary 2012 September (epub). Elliott et al. Surgical Treatment of Ectopic Recurrence of Craniopharyngioma. J Neurosurg Pediatrics : Wang XY et al. Post-operative Implantation Metastasis of Craniopharyngioma: A Case Report. The J of Internat Medical Res : Elliott et al. Surgical Management of Craniopharyngiomas in Children: Meta-analysis and Comparison of Transcranial and Transsphenoidal Approaches. Neurosurg 2011; 69(3): Mortini et al. Neurosurgical Treatment of Craniopharyngioma in Adults and Children: Early and Long-term Results in a Large Case Series. J Neurosurg 2011; 114:
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