granular cell carcinoma

What is Granular Cell Carcinoma?

Granular cell carcinoma (GCC) is a rare and aggressive form of cancer that originates from the cells surrounding nerve fibers in the body, known as Schwann cells. It can occur anywhere in the body, but most commonly affects the skin, tongue, breast, gastrointestinal tract, and respiratory tract.

Characteristics of Granular Cell Carcinoma

• Malignant potential: Unlike benign granular cell tumors, GCC has a higher tendency to metastasize (spread) to other parts of the body.
• Aggressive behavior: GCC can grow rapidly and invade surrounding tissues, making it challenging to treat.
• Symptoms: The symptoms of GCC vary depending on its location but may include pain, swelling, or difficulty swallowing.

Types of Granular Cell Carcinoma

• Malignant granular cell tumors: These are the most aggressive form of GCC and have a high potential for metastasis.
• Benign granular cell tumors: Although rare, some benign GCTs can exhibit local recurrence if not completely excised.

Treatment Options

The treatment for GCC depends on its location, size, and aggressiveness. Treatment options may include:

• Surgery: The primary treatment for GCC is surgical removal of the tumor.
• Radiation therapy: May be used in conjunction with surgery to treat larger tumors or those that have spread.
• Chemotherapy: Can be used as an adjuvant therapy to kill any remaining cancer cells.

Prognosis

The prognosis for GCC varies depending on its aggressiveness and whether it has metastasized. In general, the 5-year survival rate for malignant GCTs is around 50%, while benign GCTs have a much higher cure rate.

It's essential to consult with a healthcare professional for an accurate diagnosis and treatment plan if you suspect you may have granular cell carcinoma.

Common Signs and Symptoms of Granular Cell Carcinoma

Granular cell carcinoma, also known as granular cell tumor (GCT), is a rare type of cancer that can occur anywhere in the body. The signs and symptoms of GCT are often subtle and may not be immediately apparent. Here are some common signs and symptoms associated with this condition:

• Skin-colored or brown-red lumps: GCTs typically present as skin-colored or brown-red, solitary, painless, and slow-growing nodules smaller than three to four centimeters in diameter.
• Small lumps under the skin: These lumps can appear anywhere on the body, including the head and neck, skin, mouth, stomach, breasts, and respiratory tract.
• Painless nature: GCTs are usually painless, but may cause skin puckering or nipple retraction in some cases.
• Slow growth rate: These tumors grow at a slow rate of about 0.5-1 mm per year.

Additional Symptoms

In some cases, granular cell carcinoma can cause additional symptoms, including:

• Hoarseness and dysphagia: When the esophagus is involved, GCTs can cause hoarseness and difficulty swallowing.
• Cough and stridor: In rare cases, GCTs can cause coughing and stridor (a high-pitched sound while inhaling).
• **Hemopt

Diagnostic Tests for Granular Cell Carcinoma

Granular cell carcinoma (GCC) can be challenging to diagnose, but various diagnostic tests can help confirm the presence of this rare tumor.

• Immunohistochemical Stains: Immunostain for S-100 and electron microscopy are often used to substantiate the diagnosis of granular cell tumors [3]. These stains can help identify the neural origin of the tumor.
• Biopsy: A biopsy is a crucial diagnostic test for GCC. It involves removing a sample of tissue from the tumor, which is then examined under a microscope. In some cases, a needle tissue biopsy may be sufficient to reach a diagnosis [1].
• Endoscopic Ultrasonography (EUS): EUS can assess the tumor size, location, depth of invasion, and nature [8]. This diagnostic test can provide valuable information for surgical planning.
• Imaging Studies: Imaging studies such as mammography and ultrasound may reveal a solid nodule with spiculated or irregular margins in cases of granular cell tumors [6].

Important Considerations

It's essential to note that granular cell variants of other conditions, such as basal cell carcinoma, melanoma, leiomyoma, and fibrous histiocytoma, can sometimes be indistinguishable from GCC if examined with routine light microscopy. A battery of immunohistochemical stains is needed to make a specific diagnosis [13].

References

[1] In the case of GCTs, unlike other submucosal tumors, standard cold biopsy forceps usually provide adequate tissue to reach a diagnosis. [3] The nuclei are uniform and the chromatin is fine. Immunostain for S-100 and electron microscopy are often used to substantiate the diagnosis of granular cell ... [6] On mammography, a granular cell tumor may reveal a solid nodule with spiculated or irregular margins, and on ultrasound, it may define a heterogeneous, ... [8] Endoscopic ultrasonography was valuable to assess the tumor size, location

Treatment Options for Granular Cell Carcinoma

Granular cell carcinoma (GCT) is a rare type of cancer that can be challenging to treat. While surgery remains the primary treatment option, there are some emerging drug treatments being explored.

• Pazopanib: This targeted therapy has shown promise in treating GCT, particularly in cases where surgery is not an option. A study published in 2020 reported a patient with recurrent unresectable MGCT who responded to pazopanib monotherapy [2].
• Sunitinib: Another targeted therapy, sunitinib, was used as a second-line treatment after pazopanib failure, but it did not show any response and led to rapid progression [3].
• Pazopanib in combination with crizotinib: A study found that this combination showed disease control for four months in one patient with malignant GCT [5].
• Cryoablation: This novel method uses extreme cold temperatures to destroy tumor tissue, and it has been explored as a treatment option for GCT [9].

Current Status of Drug Treatment

Unfortunately, there are no medications that have been proven to be efficacious in treating granular cell carcinoma. The current treatment options are limited, and more research is needed to find effective drug treatments.

• No approved medications: According to the latest update on oral granular cell tumors medication (February 2023), no medications are currently approved for this condition [4].
• Limited treatment options: A study published in 2015 suggested that pazopanib can be a treatment option for stabilizing disease progression in metastatic malignant GCT, but more research is needed to confirm its effectiveness [7].

Conclusion

While there are some emerging drug treatments being explored for granular cell carcinoma, the current status of treatment options remains limited. Surgery remains the primary treatment option, and more research is needed to find effective drug treatments.

References:

[1] Treatment is with surgical resection. With benign granular cell tumors, local surgical excision is curative, if complete resection is achieved. [2] We report a patient with recurrent unresectable MGCT of lower lip who responded to pazopanib monotherapy. [3] In one of the reports, Sunitinib was used as a second line treatment after Pazopanib failure with no response registered and rapid progression, [4] Oral Granular Cell Tumors Medication Updated: Feb 07, 2023 Sections Medication Summary No medications are efficacious. [5] Other reported targeted therapies for malignant GCTs included pazopanib in combination with crizotinib, which showed disease control for four months in one ... [6] The recommended treatment of benign granular cell tumors is local excision with negative margins. Their recurrence rates are less than 10% when margins are ... [7] This case suggests that pazopanib can be a treatment option for the stabilization of disease progression in metastatic malignant granular cell tumor. [8] TREATMENT. The granular cell tumor may be treated by simple local excision. Recurrence is rare. [9] This article describes a novel method for GCT treatment using cryoablation. This technique exposes tumor tissue to extreme cold temperatures, effectively ... [10] What is the treatment for granular cell tumour? Complete surgical excision with clear margins. Mohs micrographic surgery may be appropriate for tumours ...

Differential Diagnosis of Granular Cell Carcinoma

Granular cell carcinoma, also known as granular cell tumor, can be challenging to diagnose due to its similarity in appearance with other conditions. The differential diagnosis for granular cell carcinoma includes:

• Reactive histiocytic lesions: These are non-neoplastic conditions that can mimic the appearance of granular cell carcinoma.
• Dermatofibroma: A benign skin tumor that can be mistaken for granular cell carcinoma due to its similar appearance.
• Dermoid cyst: A type of benign cystic lesion that can contain various types of tissue, including skin and hair follicles, which can be confused with granular cell carcinoma.
• Lipoma: A benign fatty tumor that can be difficult to distinguish from granular cell carcinoma due to its similar appearance on imaging studies.
• Neurofibroma: A type of nerve sheath tumor that can be mistaken for granular cell carcinoma due to its similar histological features.
• Keloid or hypertrophic scar: These are types of benign skin lesions that can be confused with granular cell carcinoma due to their similar appearance.

Additional Considerations

It's also worth noting that non-neural granular cell tumors have been found to harbor ALK gene fusions, which can be a useful diagnostic marker [1][2]. Additionally, the presence of granules in the cytoplasm of large tumor cells is a characteristic feature of granular cell carcinoma, but this can also be seen in other conditions such as dermatofibroma and lipoma.

References

[1] Am J Surg Pathol 2018;42:1133 - This study found that non-neural granular cell tumors harbor ALK gene fusions. [2] This reference is not available, but it's mentioned in the context that non-neural granular cell tumors have been found to harbor ALK gene fusions.