Case Study 4 Gabrielle Yeaney, M.D.

Question 1 The patient is a 72-year-old male who presents with bitemporal hemianopsia without endocrine abnormalities. Being the astute student of neuropathology that you are, you review the patient's MRI results. Describe the MRI findings (including location).

Coronal T1

Coronal +Contrast

Sagittal +contrast

Answer Intrasellar and suprasellar enhancing multilobulated mass lesion with solid and cystic components (heterogeneous).

Question 2 Differential diagnosis based on MRI/age.

Answer Suprasellar lesions include:
Aneurysm
Dermoid/epidermoid cyst
Craniopharyngioma
Granular cell tumor
Hypophysitis
Hypothalamic neuronal hamartoma
Meningioma
Metastasis
Optic glioma
Pituitary macroadenoma
Rathke's cyst
Xanthogranuloma

Question 3 The surgeon performs an endoscopic endonasal biopsy, and an intraoperative evaluation is requested. Describe the cytologic features of the smear. Click here to view slide.

Answer Cohesive clusters and sheets of bland squamoid epithelium

Question 4 What is your intraoperative diagnosis (A. Neoplastic/Defer/Non-neoplastic, B. ________)

Answer Neoplasm Craniopharyngioma

Question 5 The patient undergoes resection. Review the permanent section. Describe the histologic features of this lesion. Click here to view slide.

Answer The lesion shows broad strands and bridges of stratified squamous epithelium with peripheral palisading of nuclei, an internal loose reticulum and microcyst formation.  There are nodules of compact "wet" keratin with dystrophic calcification.  The stromal background is vascular with chronic inflammation, fibrosis and scattered foamy macrophages.

Question 6 What is your final diagnosis?

Answer Craniopharyngioma, adamantinomatous-type

Question 7 True or False. This tumor is immunoreactive for EMA.

Answer True. Craniopharyngioma is positive for high- and low- molecular weight cytokeratins and EMA.

Question 8 What is the corresponding WHO grade of this lesion?

Answer WHO grade I

Question 9 What is a typical CT finding of this lesion that wasn't seen on MRI?

Answer Calcification(s)

Question 10 The surgeon makes a rare visit to neuropathology to review the slides and tells you that s/he extracted material from the cystic portion of the lesion.  How would you expect this material to appearly grossly?

Answer Thick brownish-yellow fluid, like machine oil, possibly with crumbly debris.

Question 11 From what embryologic structure is this tumor presumed to develop?

Answer Rathke pouch epithelium

Question 12 In regards to this tumor, which of the following is FALSE? Lesions > 5 cm in diameter carry a worse prognosis Recurrence rate is higher for incompletely excised lesions Brain invasion is correlated with higher recurrence Papillary-type may have a better prognosis than adamantinomatous > 60% of patients have a 10-year recurrence-free survival

Answer The correct answer is C. According to WHO 2000, "histologic evidence of brain invasion, more frequently documented in the adamantinomatous than in the papillary type, is not correlated with a higher recurrence rate in cases with gross surgical resection." p 246