--- title: "Granular Cell Tumor" docid: "da976b04-85a3-4bf8-ac81-e579f081293e" authors: - key: "8d5254e9-8dda-478b-8f08-bdee97a32c79" value: "Karen L. Salzman, MD, FACR" breadcrumbs: - name: "Brain" slug: "brain" treeNodeId: "6d8829f1-14d7-45af-8675-255189aa526a" - name: "Diagnosis" slug: "diagnosis" treeNodeId: "51c00394-446e-4a38-94af-d3b1d14d34e8" - name: "Anatomy-Based Diagnoses" slug: "anatomy-based-diagnoses" treeNodeId: "529d3e33-f508-498c-bc70-cf962e81e629" - name: "Sella and Pituitary" slug: "sella-and-pituitary" treeNodeId: "9afaeeb6-661c-49be-b55f-5bdc1c98a53e" - name: "Neoplasms" slug: "neoplasms" treeNodeId: "87c53ba9-d217-4dc9-be90-15cfe4d7766c" - name: "Granular Cell Tumor" slug: "granular-cell-tumor" treeNodeId: null category: "Brain" documentVersionId: "16196366-3704-4511-b91f-73ac70ac21c1" imageCount: 6 lastUpdated: "08/05/20" pageDescription: "Granular Cell Tumor" pageKeywords: "Brain, Diagnosis, Anatomy-Based Diagnoses, Sella and Pituitary, Neoplasms, Granular Cell Tumor" pageTitle: "Granular Cell Tumor | STATdx" enhancedTitle: "Granular Cell Tumor" type: "DX" references: true breadcrumbs: - "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](/document/pituitary-microadenoma/283f3068-d369-4f79-bf01-0f2b82c6e49b) - Sellar and suprasellar enhancing mass - Arises from adenohypophysis - May be indistinguishable - [Lymphocytic Hypophysitis](/document/lymphocytic-hypophysitis/f30774c3-cbd0-4ab3-b3d1-e0574106db1f) - May be indistinguishable from macroadenoma - May present as infundibular mass - Typically pregnant or postpartum females - [Pituicytoma](/document/pituicytoma/d6e481d5-7742-4354-8dfe-4f04286a709a) - May be intrasellar or suprasellar mass - May be separate from adenohypophysis - [Spindle Cell Oncocytoma](/document/spindle-cell-oncocytoma/1557bfb2-8315-4782-9a1e-4e70d6d20c4e) - Imaging mimics macroadenoma - Enhancing sellar and suprasellar mass - [Rathke Cleft Cyst](/document/rathke-cleft-cyst/8f1561f7-92a7-485c-a0ae-2e2d5c8c1628) - 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 55b6bf2f-c975-435a-972b-a42c5b4cbe6d ## 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](http://www.ncbi.nlm.nih.gov/pubmed/?term=31734425%5Bpmid%5D) 1. [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](http://www.ncbi.nlm.nih.gov/pubmed/?term=30276594%5Bpmid%5D) 1. [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](http://www.ncbi.nlm.nih.gov/pubmed/?term=30276594%5Bpmid%5D) 1. [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](http://www.ncbi.nlm.nih.gov/pubmed/?term=29500747%5Bpmid%5D) 1. [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](http://www.ncbi.nlm.nih.gov/pubmed/?term=28948361%5Bpmid%5D) 1. [Jian F et al: Surgical biopsies in patients with central diabetes insipidus and thickened pituitary stalks. Endocrine. 47(1):325-35, 2014](http://www.ncbi.nlm.nih.gov/pubmed/?term=24532100%5Bpmid%5D) 1. [Shizukuishi T et al: Granular cell tumor of the neurohypophysis with optic tract edema. Jpn J Radiol. 32(3):179-82, 2014](http://www.ncbi.nlm.nih.gov/pubmed/?term=24414885%5Bpmid%5D) 1. [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](http://www.ncbi.nlm.nih.gov/pubmed/?term=23887161%5Bpmid%5D) 1. [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](http://www.ncbi.nlm.nih.gov/pubmed/?term=23922042%5Bpmid%5D) 1. [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](http://www.ncbi.nlm.nih.gov/pubmed/?term=21960498%5Bpmid%5D) 1. [Mumert ML et al: Cystic granular cell tumor mimicking Rathke cleft cyst. J Neurosurg. 114(2):325-8, 2011](http://www.ncbi.nlm.nih.gov/pubmed/?term=20509726%5Bpmid%5D) 1. [Menon G et al: Symptomatic granular cell tumour of the pituitary. Br J Neurosurg. 22(1):126-30, 2008](http://www.ncbi.nlm.nih.gov/pubmed/?term=17952719%5Bpmid%5D) 1. 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 .](images/app.statdx.com_image_thumbnail_5ca8b84a-b148-4d75-a721-86afb09900bf_size_168_quality_85_6152a149_20251014T205304Z.jpg) *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 .](images/app.statdx.com_image_thumbnail_5ca8b84a-b148-4d75-a721-86afb09900bf_size_174_quality_85_93e878e8.jpg) *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.](images/app.statdx.com_image_thumbnail_37b27bcd-5ca6-4d76-b72f-601736dcfe13_size_168_quality_85_89773be0_20251014T205304Z.jpg) *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.](images/app.statdx.com_image_thumbnail_e25dc2dc-35c8-4cde-85d2-2a4a82a86aae_size_168_quality_85_bd87c0e3_20251014T205304Z.jpg) *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.](images/app.statdx.com_image_thumbnail_a887a4ff-115c-4cf2-8604-74d6473eba9a_size_168_quality_85_1a5a4e25_20251014T205304Z.jpg) *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.](images/app.statdx.com_image_thumbnail_0fb060d9-7fd7-4e32-befc-534cb8d7d572_size_168_quality_85_c1d2f18a_20251014T205304Z.jpg) *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.](images/app.statdx.com_image_thumbnail_51f9669f-be82-4dd4-89b7-27b6c5fda51a_size_168_quality_85_8f77246c_20251014T205304Z.jpg) *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.*