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Case Study 45

This case study examines the radiologic appearance of a suprasellar mass in a patient with hypopituitarism, the differential diagnosis, and the interpretation of frozen section, smear preparation, and permanent section. Stains and features distinguishing pituicytoma from other tumors are also discussed.

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Case Study 45

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  1. Case Study 45 Julia Kofler, M.D.

  2. Question 1 Clinical history: 41 year old male with a 2 year history of progressive hypopituitarism, headache and bitemporal hemianopsia. Describe the lesion on the following CT scan (no MRI images available due to pacemaker).

  3. Question 1 CT with contrast

  4. Answer Diffusely contrast-enhancing suprasellar mass

  5. Question 2 What is your differential diagnosis based on the radiologic appearance and location of the lesion?

  6. Answer • Pituitary adenoma • Craniopharyngioma • Pituicytoma • Granular cell tumor • Meningioma • Pilocytic astrocytoma • Germ cell tumor

  7. Question 3 An endoscopic endonasal resection was performed. An intraoperative consultation was requested. What is your interpretation of the following frozen section and smear preparation? According to the surgeon, the mass extended around the pituitary stalk and appeared highly vascular Click here to view frozen section slide.Click here to view smear preparation.

  8. Answer • Low-grade spindle cell tumor • A pituicytoma was favored over other spindle cell neoplasms

  9. Question 4 Describe the findings on the permanent section. Click here to view slide.

  10. Answer Moderately cellular neoplasm Comprised of mildly pleomorphic spindle cells with variably distinct cell borders, irregular vesicular nuclei and light eosinophilic cytoplasm with a fibrillar quality Cells are arranged in groups and haphazardly interwoven fascicles The fascicles are separated by very thin, compressed vascular channels No mitoses are seen No Rosenthal fibers, eosinophilic granular bodies, Herring bodies or oncocytic change is seen

  11. Question 5 What is your differential diagnosis and which stains may be useful to support your diagnosis?

  12. Answer Pituicytoma, normal infundibulum, pilocytic astrocytoma, spindle cell oncocytoma, granular cell tumor PAS, S100, GFAP, Neurofilament, EMA, Synaptophysin

  13. Question 6 What is your interpretation of the following stains? Click here to view PAS slideClick here to view S100 slideClick here to view GFAP slideClick here to view neurofilament slide

  14. Answer PAS is negative in tumor cells S100 shows strong nuclear and cytoplasmic reactivity GFAP is negative in the tumor cells (may be variably positive in pituicytomas) Neurofilament highlights rare infundibular axons at the margin of the specimen The tumor was also positive for vimentin and negative for synaptophysin and EMA

  15. Question 7 Name a few features that distinguish pituicytoma from normal infundibulum.

  16. Answer Normal infundibular tissue is usually less cellular than a pituicytoma (but cellularity may overlap) Normal tissue is looser in texture and contains axons and perivascular fibrillar zones Normal tissue contains Herring bodies (PAS positive focal axonal swellings) Normal tissue is diffusely positive for synaptophysin and neurofilament; pituicytomas are negative

  17. Question 8 Name a few features that distinguish pituicytoma from pilocytic astrocytoma.

  18. Answer • Pilocytic astrocytomas commonly occur in children, whereas pituicytomas are usually seen in adults • Pituicytomas lack Rosenthal fibers and eosinophilic granular bodies that are commonly seen in pilocytic astrocytomas • Pilocytic astrocytomas usually exhibit a biphasic growth pattern and more variability (compact, piloid, microcystic patterns)

  19. Question 9 Name a few features that distinguish pituicytoma from spindle cell oncocytoma.

  20. Answer • Spindle cell oncocytomas are composed of interlacing fascicles of spindled to epithelioid cells with eosinophilic to oncocytic cytoplasm • Ultrastructurally, numerous mitochondria are seen • Spindle cell oncocytomas are usually positive for vimentin, EMA, S100 and galectin-3 • They are negative for pituitary hormones, GFAP and synaptophysin

  21. Question 10 What is your final diagnosis?

  22. Answer Pituicytoma

  23. Question 11 Name a transcription factor that has recently been shown to be expressed in human fetal and adult pituicytes as well as in a variety of sellar masses (pituicytoma, granular cell tumor, spindle cell oncocytoma)?

  24. Answer Thyroid transcription factor 1, which was also positive in our pituicytoma (see image below) Reference: Lee EB et al. J Neuropath Exp Neurol 2009;68:482

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