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Granular Cell Tumor da976b04-85a3-4bf8-ac81-e579f081293e
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8d5254e9-8dda-478b-8f08-bdee97a32c79 Karen L. Salzman, MD, FACR
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Brain 16196366-3704-4511-b91f-73ac70ac21c1 6 08/05/20 Granular Cell Tumor Brain, Diagnosis, Anatomy-Based Diagnoses, Sella and Pituitary, Neoplasms, Granular Cell Tumor Granular Cell Tumor | STATdx Granular Cell Tumor DX true
Brain
Diagnosis
Anatomy-Based Diagnoses
Sella and Pituitary
Neoplasms
Granular Cell Tumor

title: "Granular Cell Tumor" docid: "da976b04-85a3-4bf8-ac81-e579f081293e" authors:

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  • "Brain"
  • "Diagnosis"
  • "Anatomy-Based Diagnoses"
  • "Sella and Pituitary"
  • "Neoplasms"
  • "Granular Cell Tumor"

KEY FACTS

  • Terminology

    • Neoplasms that arise from pituicytes, specialized glial cells of neurohypophysis or infundibulum
    • Rare low-grade, nonendocrine neoplasms of sellar region
    • Formerly called pituicytoma; granular cell tumor of neurohypophysis
    • Part of 2017 WHO spectrum of thyroid transcription factor 1 (TTF-1) expressing pituitary tumors of posterior lobe

  • Imaging

    • Enhancing, well-circumscribed sellar/suprasellar or infundibular mass
    • 1.5-6.0 cm
    • CT: Sellar/suprasellar mass with hyperattenuation - Rarely calcification may be present
    • Best imaging tool: C+ MR with high-resolution imaging through sellar region
    • Consider granular cell tumor if sellar/suprasellar mass appears separate from anterior pituitary gland

  • Top Differential Diagnoses

    • Pituitary macroadenoma
    • Lymphocytic hypophysitis
    • Pituicytoma
    • Spindle cell oncocytoma
    • Rathke cleft cyst

  • Pathology

    • WHO grade 1

  • Clinical Issues

    • Commonly asymptomatic (small lesions)
    • Visual field deficit related to optic chiasm compression is most common presenting feature
    • Less common symptoms: Panhypopituitarism, galactorrhea, amenorrhea, decreased libido, neuropsychological changes
    • Typically present in adulthood, 5th-6th decades
    • Generally benign clinical course

TERMINOLOGY

  • Abbreviations

    • Granular cell tumor (GCT)

  • Synonyms

    • Formerly called pituicytoma; granular cell tumor of neurohypophysis

  • Definitions

    • Neoplasms that arise from pituicytes, specialized glia of neurohypophysis or infundibulum
    • Rare low-grade, nonendocrine neoplasms of sellar region
    • Part of 2017 WHO spectrum of thyroid transcription factor1 (TTF-1) expressing pituitary tumors of posterior lobe

IMAGING

  • General Features

    • Best diagnostic clue

      - Enhancing, well-circumscribed sellar/suprasellar or infundibular mass

    • Location

      - Sellar and suprasellar or infundibular mass

    • Size

      - 1.5-6.0 cm

    • Morphology

      - Lobulated and well circumscribed

  • CT Findings

    • NECT

      - Sellar/suprasellar mass with hyperattenuation
- Rarely calcification may be present

  • MR Findings

    • T1WI

      - Sellar/suprasellar mass isointense to gray matter

    • T1WI C+

      - Enhancement may be homogeneous or heterogeneous

  • Imaging Recommendations

    • Best imaging tool

      - C+ MR with high-resolution imaging through sellar region

DIFFERENTIAL DIAGNOSIS

  • Pituitary Macroadenoma
    • Sellar and suprasellar enhancing mass
    • Arises from adenohypophysis
    • May be indistinguishable
  • Lymphocytic Hypophysitis
    • May be indistinguishable from macroadenoma
    • May present as infundibular mass
    • Typically pregnant or postpartum females
  • Pituicytoma
    • May be intrasellar or suprasellar mass
    • May be separate from adenohypophysis
  • Spindle Cell Oncocytoma
    • Imaging mimics macroadenoma
    • Enhancing sellar and suprasellar mass
  • Rathke Cleft Cyst
    • Nonenhancing cystic sellar &/or suprasellar lesion
    • Intracystic nodule in up to 75%

PATHOLOGY

  • General Features

    • Associated abnormalities

      - Granular cell tumors have been found in associated with adenomas
- Small granular cell clusters have been found in up to 17% of autopsy series

  • Staging, Grading, & Classification

    • WHO grade 1

  • Gross Pathologic & Surgical Features

    • Lobulated, well-circumscribed mass, soft but rubbery
    • More firm than pituitary adenoma

  • Microscopic Features

    • Densely packed polygonal cells with abundant granular eosinophilic cytoplasm
    • Electron microscopy: Cytoplasm is filled with phagolysosomes containing electron-dense material and membranous debris

CLINICAL ISSUES

  • Presentation

    • Most common signs/symptoms

      - Commonly asymptomatic (small lesions)
- Visual field deficit related to optic chiasm compression

    • Other signs/symptoms

      - Panhypopituitarism, galactorrhea, amenorrhea, decreased libido, neuropsychological changes
- Rarely diabetes insipidus

  • Demographics

    • Age

      - Typically present in adulthood, 5th-6th decades

    • Gender

      - F:M = 2:1

  • Natural History & Prognosis

    • Rare (~ 150 reported cases)
    • Generally benign clinical course

  • Treatment

    • Surgical resection

DIAGNOSTIC CHECKLIST

  • Image Interpretation Pearls

    • Consider granular cell tumor if sellar/suprasellar mass appears separate from anterior pituitary gland

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References

Selected References

  1. Borg A et al: Tumors of the neurohypophysis: One unit's experience and literature review. World Neurosurg. 134:e968-e978, 2020
  2. Guerrero-Pérez F et al: Posterior pituitary tumours: the spectrum of a unique entity. A clinical and histological study of a large case series. Endocrine. 63(1):36-43, 2019
  3. Guerrero-Pérez F et al: Posterior pituitary tumours: the spectrum of a unique entity. A clinical and histological study of a large case series. Endocrine. 63(1):36-43, 2019
  4. Shibuya M: Welcoming the new WHO classification of pituitary tumors 2017: revolution in TTF-1-positive posterior pituitary tumors. Brain Tumor Pathol. 35(2):62-70, 2018
  5. Ahmed AK et al: Extent of surgical resection and tumor size predicts prognosis in granular cell tumor of the sellar region. Acta Neurochir (Wien). 159(11):2209-16, 2017
  6. Jian F et al: Surgical biopsies in patients with central diabetes insipidus and thickened pituitary stalks. Endocrine. 47(1):325-35, 2014
  7. Shizukuishi T et al: Granular cell tumor of the neurohypophysis with optic tract edema. Jpn J Radiol. 32(3):179-82, 2014
  8. Mete O et al: Spindle cell oncocytomas and granular cell tumors of the pituitary are variants of pituicytoma. Am J Surg Pathol. 37(11):1694-9, 2013
  9. Saiegh L et al: Granular cell tumor of the neurohypophysis: case report and review of the literature. Neuro Endocrinol Lett. 34(5):331-8, 2013
  10. Covington MF et al: Pituicytoma, spindle cell oncocytoma, and granular cell tumor: clarification and meta-analysis of the world literature since 1893. AJNR Am J Neuroradiol. 32(11):2067-72, 2011
  11. Mumert ML et al: Cystic granular cell tumor mimicking Rathke cleft cyst. J Neurosurg. 114(2):325-8, 2011
  12. Menon G et al: Symptomatic granular cell tumour of the pituitary. Br J Neurosurg. 22(1):126-30, 2008
  13. Fuller GN et el: Granular cell tumour of the neurohypophysis In Louis, DN et el: WHO Classification of Tumors of the Central Nervous System. 4th Ed. Lyon: IARC. 241-2, 2007

Images

Selected Images

Sagittal T1 C+ MR in a 65-year-old woman shows an enhancing suprasellar mass . Sagittal T1 C+ MR in a 65-year-old woman shows an enhancing suprasellar mass .

Sagittal T1 C+ MR in a 65-year-old woman shows an enhancing suprasellar mass . Sagittal T1 C+ MR in a 65-year-old woman shows an enhancing suprasellar mass .

Coronal T1 C+ MR in the same patient shows the suprasellar enhancing mass contacting the optic chiasm . Imaging mimics the much more common pituitary macroadenoma. Granular cell tumors are rare low-grade, nonendocrine neoplasms arising from the infundibulum or neurohypophysis. These tumors are part of the 2017 WHO spectrum of TTF-1 expressing pituitary tumors of the posterior lobe. Coronal T1 C+ MR in the same patient shows the suprasellar enhancing mass contacting the optic chiasm . Imaging mimics the much more common pituitary macroadenoma. Granular cell tumors are rare low-grade, nonendocrine neoplasms arising from the infundibulum or neurohypophysis. These tumors are part of the 2017 WHO spectrum of TTF-1 expressing pituitary tumors of the posterior lobe.

Sagittal T1 MR in a 31-year-old woman with headache and visual complaints shows a sellar and suprasellar mass with superior displacement of the optic chiasm . There is a hyperintense focus along the inferior aspect of the mass. Sagittal T1 MR in a 31-year-old woman with headache and visual complaints shows a sellar and suprasellar mass with superior displacement of the optic chiasm . There is a hyperintense focus along the inferior aspect of the mass.

Sagittal T1 C+ MR in the same patient shows peripheral enhancement of the mass. The region of T1 hyperintensity is also seen, resembling an intracystic nodule , typically seen in a Rathke cleft cyst. Granular cell tumor of the neurohypophysis was diagnosed at resection. Sagittal T1 C+ MR in the same patient shows peripheral enhancement of the mass. The region of T1 hyperintensity is also seen, resembling an intracystic nodule , typically seen in a Rathke cleft cyst. Granular cell tumor of the neurohypophysis was diagnosed at resection.

Additional Images

Sagittal T1 C+ MR shows an enhancing mass along the superior infundibulum and anterior 3rd ventricle . Granular cell tumor was diagnosed at resection. Sagittal T1 C+ MR shows an enhancing mass along the superior infundibulum and anterior 3rd ventricle . Granular cell tumor was diagnosed at resection.

Coronal T1 C+ MR shows a large sellar and suprasellar enhancing mass contacting the left optic chiasm . Granular cell tumor was diagnosed at resection. Imaging mimics the much more common pituitary macroadenoma. Coronal T1 C+ MR shows a large sellar and suprasellar enhancing mass contacting the left optic chiasm . Granular cell tumor was diagnosed at resection. Imaging mimics the much more common pituitary macroadenoma.

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