.
This commit is contained in:
@@ -0,0 +1,291 @@
|
||||
---
|
||||
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 <img src='img/arrows/CO.png'/>.*
|
||||
|
||||

|
||||
*Sagittal T1 C+ MR in a 65-year-old woman shows an enhancing suprasellar mass <img src='img/arrows/CO.png'/>.*
|
||||
|
||||

|
||||
*Coronal T1 C+ MR in the same patient shows the suprasellar enhancing mass <img src='img/arrows/CO.png'/> contacting the optic chiasm <img src='img/arrows/WS.png'/>. 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 <img src='img/arrows/WO.png'/> with superior displacement of the optic chiasm <img src='img/arrows/WS.png'/>. There is a hyperintense focus <img src='img/arrows/CC.png'/> along the inferior aspect of the mass.*
|
||||
|
||||

|
||||
*Sagittal T1 C+ MR in the same patient shows peripheral enhancement <img src='img/arrows/WO.png'/> of the mass. The region of T1 hyperintensity is also seen, resembling an intracystic nodule <img src='img/arrows/CC.png'/>, 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 <img src='img/arrows/CO.png'/>. Granular cell tumor was diagnosed at resection.*
|
||||
|
||||

|
||||
*Coronal T1 C+ MR shows a large sellar and suprasellar enhancing mass contacting the left optic chiasm <img src='img/arrows/WO.png'/>. Granular cell tumor was diagnosed at resection. Imaging mimics the much more common pituitary macroadenoma.*
|
||||
|
||||
Reference in New Issue
Block a user