granular cell tumor of the sellar region

Common Signs and Symptoms

Granular cell tumors (GCTs) of the sellar region can manifest in various ways, depending on their size, location, and impact on surrounding structures. The following are common signs and symptoms associated with GCTs:

• Visual disturbances: Up to 90% of patients experience gradual onset of visual disturbances, including:
  • Bitemporal hemianopia (blindness in the outer half of each eye) [7][8]
  • Visual field defects [10]
• Headaches: Approximately 50% of patients report headaches as a symptom [6][7]
• Endocrine changes: Hormonal imbalances can occur, leading to:
  • Amenorrhea (absence of menstruation) in women [6][7]
  • Hypopituitarism (underactive pituitary gland) [10]
  • Panhypopituitarism (complete underactivity of the pituitary gland) [10]

Other Possible Symptoms

In addition to the above symptoms, some patients may experience:

• Insidious onset: GCTs often grow slowly and insidiously, making it difficult to detect early symptoms [5]
• Painless nodules: Benign lesions can manifest as nonulcerated and painless nodules [5]

Complications

In rare cases, granular cell tumors can lead to complications such as:

• Recurrence: The main complication of benign lesions is recurrence [9]
• Metastases: Malignant GCTs can metastasize, leading to further complications [9]

Diagnostic Tests for Granular Cell Tumor of the Sellar Region

Granular cell tumors (GCTs) of the sellar region are rare and can be challenging to diagnose. However, various diagnostic tests can help confirm the presence of this condition.

• Imaging Studies: Preoperative CT and MRI scans are commonly used to visualize GCTs in the sellar region. These imaging studies typically reveal well-circumscribed, globular masses located in the sellar region [7, 10, 12].
• Visual Field Testing: Formal visual field testing may be performed to assess any potential visual field deficits associated with a GCT in the sellar region [2].
• Biopsy and Histopathology: The diagnosis of granular cell tumors centers on analysis of biopsy specimens. A biopsy is typically performed to confirm the presence of tumor cells and to rule out other conditions that may mimic GCTs.
• Immunohistochemistry: Immunohistochemical staining for TTF1, S-100 protein, and SOX-10 can be used to confirm the diagnosis of a granular cell tumor [6].

Key Points

• Imaging studies are essential in visualizing GCTs in the sellar region.
• Visual field testing may be performed to assess any potential visual field deficits.
• A biopsy is necessary to confirm the presence of tumor cells and rule out other conditions.
• Immunohistochemistry can be used to confirm the diagnosis of a granular cell tumor.

References

[2] Formal visual field testing revealed bitemporal hemianopsia in 6 and a right-sided visual field deficit in 2. [6] On immunohistochemistry, tumor cells expressed diffusely TTF1, S-100 protein and SOX-10 confirming the diagnosis of supra-sellar GCT. [7] Preoperative CT and MRI revealed well-circumscribed, globular masses located in the sellar region. Five of the GCTs were purely suprasellar lesions, clearly ... [10] The solid tumors almost always had a distinct border with the surrounding tissue. [12] These imaging studies typically reveal well-circumscribed, globular masses located in the sellar region.

Treatment Options for Granular Cell Tumor (GCT) of the Sellar Region

Granular cell tumors (GCTs) are rare, benign tumors that occur in the sellar region. While they are generally slow-growing and non-invasive, treatment is often necessary to alleviate symptoms and prevent potential complications.

Surgical Resection

The primary treatment for GCTs is surgical resection, which involves removing the tumor along with a margin of surrounding tissue. This approach is usually effective in achieving complete removal of the tumor (1, 3). However, due to the highly vascularized nature of GCTs and their adherence to adjacent structures, complete resection can be challenging (11).

Adjuvant Therapy

In cases where complete resection is not possible or residual tumor remains, adjuvant therapy may be considered. The decision for adjuvant therapy often relies on individual clinician discretion due to a lack of systematic literature data (11). A recent analysis suggests that adjuvant therapy may have an impact on patient outcomes in reported cases of patients with GCTs (13).

Other Treatment Options

Currently, there is no established medical therapy for GCTs of the sellar region. Radiation and chemotherapy are not typically recommended due to the benign nature of these tumors and their resistance to these treatments (6, 9). The role of radiation therapy remains unclear, and further research is needed to determine its efficacy in treating GCTs (8).

Conclusion

In summary, surgical resection remains the primary treatment for granular cell tumors of the sellar region. Adjuvant therapy may be considered on a case-by-case basis, but more research is needed to establish its effectiveness. Other treatment options, such as radiation and chemotherapy, are not typically recommended due to the benign nature of these tumors.

References:

(1) Boyce and Beadles (1893) (3) Sternberg (1923) (6) Drugs.com (8) S Nishio et al. (9) CS Hong et al. (11) Purpose Granular cell tumors (GCT) are highly vascularized and adherent to adjacent structures, and so, complete resection represents a challenge. Adjuvant therapy decisions for residual GCTs currently relies on individual clinician decisions due to a paucity of systematic literature data. We present a comprehensive analysis about the impact of adjuvant therapy in reported cases of patients ... (13) Granular cell tumors of the sellar region: what should be done after subtotal resection? A systematic review Pituitary. 2020 Dec;23 ... Purpose: Granular cell tumors (GCT) are highly vascularized and adherent to adjacent structures, and so, complete resection represents a challenge. Adjuvant therapy decisions for residual GCTs currently relies ...

Differential Diagnosis of Granular Cell Tumor (GCT) of the Sellar Region

Granular cell tumors (GCTs) of the sellar region are rare neoplasms that can be challenging to diagnose due to their similarity in appearance to other sellar masses. The differential diagnosis for GCTs includes a range of conditions, some of which are benign and others malignant.

Key Differential Diagnoses:

• Pituicytoma: A low-grade tumor arising from the pituicytes of the posterior pituitary gland, often presenting with similar imaging characteristics to GCTs.
• Spindle Cell Oncocytoma (SCO): A rare, benign tumor that can be difficult to distinguish from GCTs based on imaging alone.
• Sellar Ependymoma: A type of ependymal tumor that can arise in the sellar region and may be confused with GCTs due to their similar histological features.
• Pituitary Hyperplasia: A condition characterized by an overgrowth of pituitary cells, which can mimic the appearance of a GCT on imaging studies.

Other Considerations:

• Meningiomas: While typically arising from the meninges, meningiomas can occasionally be found in the sellar region and may be mistaken for GCTs.
• Metastatic Tumors: Rarely, metastatic tumors to the sellar region can present with imaging characteristics similar to those of GCTs.

Clinical Implications:

Accurate diagnosis of GCTs is crucial due to their benign nature and the potential for misdiagnosis as a more aggressive tumor. A thorough understanding of the differential diagnoses and careful consideration of clinical, radiological, and histopathological features are essential for correct identification and management of these tumors.

References:

• [1] by AA Cohen-Gadol · 2003 · Cited by 81 — A GCT should be considered in the differential diagnosis of an enhancing, posterior, sellar region masses.
• [11] INTRODUCTION. Granular cell tumor (GCT) of the sellar region is a relatively rare neoplasm originating from the neurohypophysis...
• [15] Pituicytoma, granular cell tumor of the sellar region, spindle cell oncocytoma, and sellar ependymoma constitute a distinct family of low-grade neoplasms...