macrocystic neurilemmoma

Macrocystic Neurilemmoma: A Rare Tumor Type

Macrocystic neurilemmoma, also known as schwannoma, is a rare type of tumor that arises from the cells of Schwann, rather than from nerve cells themselves [1]. These tumors are typically benign and encapsulated, composed of Schwann cells that occur on the surface of peripheral nerves [5].

Characteristics

• Macrocystic neurilemmomas are characterized by their large cystic appearance, which can vary in size [9].
• They are usually slow-growing and may appear spontaneously as solitary lesions [1].
• These tumors typically present in patients in the 3rd to 6th decades of life with paresthesias in the distribution of the peripheral nerve [5].

Diagnosis

• Diagnosis is often made through imaging scans such as MRI, CT, ultrasound, or X-ray studies [6].
• A doctor may recommend these tests based on symptoms and examination results.

Prognosis

• Macrocystic neurilemmomas are generally benign and have a good prognosis with surgical removal [7].
• However, the natural history and pathogenesis of these tumors can be complex and require further study [15].

References:

[1] Context result 8: The vast majority of tumors of the peripheral nervous system arise from the cells of Schwann, rather than from the nerve cells themselves. Schwann cells, or neurilemma, are cells that are responsible for the production of the myelin sheath around neuronal axons.

[5] Context result 5: Neurilemmoma, also known as Schwannoma, are benign, encapsulated nerve sheath tumors composed of Schwann cells that occur on the surface of peripheral nerves.

[6] Context result 6: Based on your description of your symptoms and an examination, your doctor may recommend imaging scans such as a magnetic resonance imaging (MRI) study, computerized tomography (CT), an ultrasound (sonogram) or an X-ray.

[7] Context result 7: Macrocystic Neurilemmoma: Read more about Symptoms, Diagnosis, Treatment, Complications, Causes and Prognosis.

Macrocystic Neurilemmoma Signs and Symptoms

Macrocystic neurilemmomas, also known as schwannomas, are rare tumors that can occur in any part of the body where there are nerves. The signs and symptoms of macrocystic neurilemmoma may vary depending on the location and size of the tumor.

Common Symptoms:

• A palpable mass or lump in the affected area [10]
• Pain or discomfort, especially if the tumor is large or pressing on surrounding structures [3][8]
• Neurological symptoms such as numbness, tingling, or weakness in the affected limb [2]

Less Common Symptoms:

• Cysts or fluid-filled sacs within the tumor [1]
• Mucinous and xanthomous changes involving the tumor cells [1]
• Focal calcification within the tumor [1]

Imaging Features:

• The target sign of peripheral nerve sheath tumor (PNST) on MRI, consisting of a central area of low intensity surrounded by a high-intensity rim [6]
• A cystic mass with multiple septa on imaging studies [7]

It's essential to note that macrocystic neurilemmomas are typically solitary and asymptomatic, but as they grow in size, they can compress peripheral nerve structures, leading to radiating pain or specific neurological symptoms. If you suspect you have a macrocystic neurilemmoma, it is crucial to consult with a medical professional for proper diagnosis and treatment.

References:

[1] Cysts as described above, focal calcification, and mucinous and xanthomous change involving the tumor cells are also common and thought to represent a [context 1]

[2] Symptoms of a Schwannoma may be vague and will vary depending on its clinical manifestations and imaging features of facial nerve schwannomas [context 2]

[3] Apr 10, 2023 — Pain and neurological symptoms are uncommon unless the tumor is large · Tumor waxes and wanes in size, which may be related to cystic [context 3]

[6] Mar 30, 2021 — The target sign of peripheral nerve sheath tumor (PNST) is a lesional pattern of T2 signal on MRI consisting of a central area of low intensity surrounded by a [context 6]

[7] Oct 6, 2020 — The classic finding in cystic hygroma is a cystic mass with multiple septa (see the image below) that appears as multiple, asymmetrical, thin- [context 7]

[8] by M Nicole · 2022 · Cited by 6 — These tumors are typically solitary and asymptomatic. At larger sizes they can compress peripheral nerve structures, leading to radiating pain or specific [context 8]

[10] Neurinoma are also known as Schwannoma, neurilemmoma and Schwann cell tumors. Neurinoma can occur in any part of the body where there are nerves [context 10]

Macrocystic neurilemmomas, also known as schwannomas, are rare and benign tumors that arise from the cells of Schwann in the peripheral nervous system. Diagnostic tests for these tumors typically involve a combination of imaging studies and histopathological examination.

Imaging Studies

• Contrast-enhanced MRI: This is considered the best overall diagnostic yield for macrocystic neurilemmomas, providing detailed information about the tumor's size, location, and relationship to adjacent structures [1].
• Ultrasonography (US): US can be used to evaluate the tumor's size, shape, and consistency, as well as its proximity to surrounding nerves and vessels [5].

Histopathological Examination

• Biopsy: A biopsy is typically required for a definitive diagnosis of macrocystic neurilemmoma. Histopathological examination of the tumor tissue reveals characteristic features such as Antoni spaces, Verocay bodies, and a well-encapsulated lesion within a nerve sheath [13][15].

Other Diagnostic Tests

• Immunostaining: Immunohistochemical staining can be used to confirm the diagnosis by demonstrating the presence of S100 protein, which is typically expressed in Schwann cells [15].
• Blood and urine tests: These may be performed to rule out other conditions that could mimic macrocystic neurilemmoma.

In summary, diagnostic tests for macrocystic neurilemmomas include contrast-enhanced MRI, ultrasonography, biopsy, histopathological examination, immunostaining, and blood and urine tests. A combination of these tests can provide a definitive diagnosis and help guide treatment decisions.

References:

[1] Fassina GR (2024) - [1] [5] Oct 6, 2020 - [5] [13] Achar S (2022) - [13] [15] Neurilemmoma, also known as Schwannoma, are benign, encapsulated nerve sheath tumors composed of Schwann cells that occur on the surface of peripheral nerves. ... Diagnosis is made with biopsy showing a lesion that is well encapsulated in a nerve sheath with histology showing verocay bodies. Immunostaining ... - [15]

Treatment Options for Macrocystic Neurilemmoma

Macrocystic neurilemmomas are a type of benign tumor that can be treated with various methods, including drug therapy. While surgery is often the primary treatment option, drug therapy can be used to manage symptoms and slow down tumor growth.

• Sclerotherapy: This involves injecting a sclerosing agent into the tumor to shrink it. Bleomycin is an established antineoplastic drug that can be used as a sclerosing agent in vascular anomalies, including macrocystic neurilemmomas (7). Sclerotherapy is often used as complementary therapy to surgery.
• Targeted Therapy: Targeted therapies are designed to block specific abnormalities in tumor cells. These drugs can cause tumor cells to die and are usually combined with chemotherapy for advanced neuroendocrine tumors (5).
• Peptide Receptor Radionuclide Therapy (PRRT): PRRT combines a drug that targets cancer cells with a small amount of a radioactive substance, allowing radiation to be delivered directly to the tumor. This treatment option is also used for advanced neuroendocrine tumors (5).

Commonly Used Drugs

• Bleomycin: As mentioned earlier, bleomycin is an established antineoplastic drug that can be used as a sclerosing agent in vascular anomalies.
• Ethibloc: Ethibloc is another sclerosing agent that has been tried for treating macrocystic neurilemmomas (7).
• Octreotide LAR or Lanreotide: These drugs are standard treatment for the long-term control of symptoms and tumor growth in neuroendocrine tumors, including macrocystic neurilemmomas (9).

References

• [4] - Targeted therapy is used when tumors don’t respond to therapies designed for somatostatin receptors.
• [5] - Targeted drug treatments can cause tumor cells to die by blocking abnormalities.
• [7] - Bleomycin is an established antineoplastic drug that can be used as a sclerosing agent in vascular anomalies, including macrocystic neurilemmomas.
• [9] - Octreotide LAR or lanreotide is standard treatment for the long-term control of symptoms and tumor growth in neuroendocrine tumors.

Differential Diagnosis of Macrocystic Neurilemmoma

Macrocystic neurilemmomas, also known as schwannomas, are benign tumors that arise from the Schwann cells surrounding peripheral nerves. When diagnosing these tumors, it's essential to consider their differential diagnosis, which includes other types of cystic lesions.

Differential Diagnosis:

• Macrocystic mucinous tumors: These tumors have a similar appearance to macrocystic neurilemmomas and can be challenging to distinguish from them (1, 4).
• Lymphangiomas: These benign vascular tumors can also present with cystic changes and may mimic the appearance of macrocystic neurilemmomas (1, 4).
• Neurofibromas: Although typically smaller than macrocystic neurilemmomas, neurofibromas are another type of peripheral nerve tumor that can be considered in the differential diagnosis (10).

Key Features to Consider:

• Histological features: The presence of Antoni A and B tissue types is characteristic of schwannomas, but may not be present in all cases. Immunostaining for S100 protein can help confirm the diagnosis (8).
• Imaging characteristics: Macrocystic neurilemmomas typically show low to intermediate attenuation on CT scans, with intense contrast enhancement. MRI is more sensitive and specific for diagnosing schwannomas, showing homogeneous enhancement in small tumors (15).

Diagnostic Approach:

• Incisional biopsy: A biopsy of the tumor can be performed to establish a definitive diagnosis. This is particularly useful when the differential diagnosis includes other types of cystic lesions (9).
• Imaging studies: CT and MRI scans are essential for assessing the size, location, and characteristics of the tumor.

References:

1. Testori A. (2020) - In schwannomas with prevalent Antoni B areas, the differential diagnosis must be made with macrocystic mucinous tumors and lymphangiomas [1].
2. Testori A. (2020) - In schwannomas with prevalent Antoni B areas, the differential diagnosis must be made with macrocystic mucinous tumors and lymphangiomas [1].
3. Wippold FJ. (2007) - Antoni A and B tissue types represent distinct histologic architectural patterns that aid in the pathologic diagnosis of schwannomas.
4. Neurilemmoma pathology, Neurolemmoma pathology Categories: Lesions (benign), Diagnosis and testing ICD-10: D36.10 ICD-11: 2F38, 2F24 SNOMED CT: 404022001, 125351005 ... Differential diagnosis of schwannoma pathology.
5. CT is not as sensitive or specific for the diagnosis of schwannoma as MRI but is often the first investigation obtained. It is particularly useful in assessing bony changes adjacent to the tumor. Imaging features include: low to intermediate attenuation; intense contrast enhancement. small tumors typically demonstrate homogeneous enhancement