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Granular Cell Tumor (GCT) of the Lung

Granular cell tumor (GCT) of the lung is a rare neoplasm that arises from the mesenchymal cells and predominantly affects the bronchus, trachea, and lung. As a leading company specializing in drug and therapy development, our company is committed to advancing the field of GCT therapy.

Introduction to Granular Cell Tumor (GCT) of the Lung

Granular cell tumor (GCT) of the lung, also known as Abrikossoff tumor or pulmonary granular cell tumor, is a rare neoplasm characterized by the proliferation of large polygonal cells with eosinophilic granular cytoplasm. It was first described by Abrikossoff in 1926 and primarily affects the bronchus, trachea, and lung. GCTs account for approximately 0.1% of all lung tumors and are relatively rare.

Pathogenesis of Granular Cell Tumor (GCT) of the Lung

The exact etiology of granular cell tumor (GCT) of the lung remains unclear. However, several hypotheses have been proposed, including neural, myogenic, and histiocytic origins. It is believed that GCTs arise from Schwann cells, fibroblasts, or histiocytes due to their expression of S-100 protein, which is a commonly used marker in the immunohistochemical diagnosis of GCTs. However, further research is needed to validate these hypotheses and gain a deeper understanding of the underlying mechanisms.

Fig.1 Tumor cells infiltrated the bronchial walls (A, HE, 20×) and the surrounding nerves (arrow) (B, HE, 200×).Fig.1 Tumor cells infiltrated the bronchial walls (A, HE, 20×) and the surrounding nerves (arrow) (B, HE, 200×). (Zhang, Lin, et al., 2022)

Diagnostics of Granular Cell Tumor (GCT) of the Lung

Histopathological analysis is essential to confirm the diagnosis of GCT. Microscopic examination reveals large polygonal cells with eosinophilic granular cytoplasm, absence of mitotic figures, and positive immunohistochemical staining for S-100 protein. Additional markers, such as CD68, vimentin, and neuron-specific enolase (NSE), may be used to support the diagnosis.

Targeted Therapy Development of Granular Cell Tumor (GCT) of the Lung

Developing targeted therapies for granular cell tumors (GCT) of the lung relies on identifying specific molecular targets involved in tumor growth and progression. Although the research in this area is still evolving, potential therapeutic targets have been identified. These include CD68, S-100 protein, vimentin, NSE, and other markers expressed by GCT cells. By targeting these markers, novel therapies may be developed to selectively eliminate tumor cells and improve outcomes.

Table 1 Mutations of unknown significance were detected by NGS. (Zhang, Lin, et al., 2022)

Gene Variant type Exon cDNA change Amino acid change Allelic fraction
CSF1R Missense 4 c.704T>G p.Val235Gly 19.99%
DDR2 Missense 10 c.964C>T p.Leu322Phe 24.19%
FAT3 Missense 9 c.6671A>G p.Asp2224Gly 11.43%
GRIN2A Missense 6 c.1196G>C p.Cys399Ser 15.37%
KMT2A Missense 27 c.7262T>A p.Met2421Lys 26.05%
NOTCH4 Missense 6 c.1037G>A p.Gly346Asp 17.05%
NOTCH4 Missense 6 c.944T>A p.Val315Glu 21.24%
NSD1 Missense 13 c.4945G>C p.Ala1649Pro 25.50%
RAD50 Missense 8 c.1232C>G p.Ala411Gly 12.86%
RPA1 Missense 14 c.1540A>G p.Met514Val 25.51%
WRN Missense 32 c.3755T>C p.Ile1252Thr 27.88%
ZNRF3 Missense 8 c.1903G>C p.Ala635Pro 21.61%

Our Services

Due to the rarity of GCTs, there is a lack of standardized therapeutic protocols, underscoring the need for further research and therapeutic advancements. We offer a comprehensive range of services to support the development of diagnostics and novel therapeutic approaches.

Therapy Development Platforms

Furthermore, our company provides preclinical development services, including the development of animal models for GCT of the lung. Animal models play a crucial role in studying tumor biology, evaluating therapeutic efficacy, and assessing safety profiles. In addition, we offer comprehensive drug development services, including pharmacokinetic and pharmacodynamic studies.

Animal Model Development Services

  • GEMs (ATP6AP1 and ATP6AP2)
  • Others

If you are interested in our services, please feel free to contact us for more details and quotation information of related services.


  • Zhang, Hai, et al. "Clinicopathological characteristics and gene analysis of pulmonary granular cell tumor in three cases and a systematic review." Translational Cancer Research 9.8 (2020): 4834.
  • Ishaq, Mohammed, et al. "Granular cell tumor of the bronchus: A case report." International Journal of Surgery Case Reports 90 (2022): 106704.
  • Houcine, Yoldez, et al. "Granular cell tumor of the lung and tracheobronchial tree: Two case-presentation with a review of the literature." Rare Tumors 15 (2023): 20363613231187822.

All of our services and products are intended for preclinical research use only and cannot be used to diagnose, treat or manage patients.

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