Granular cell tumors of the urethra.

Granular cell tumors (GCTs) are a rare type of mesenchymal tumors that are histologically derived by Schwann cells and rise within soft tissues such as skin and mucosal surfaces. Differentiation between benign and malignant GCTs is often difficult and relies on their biological behavior and metastatic potential. While there are no standard guidelines for management, upfront surgical resection, whenever feasible, is key as a definitive measure. Systemic therapy is often limited by poor chemosensitivity of these tumors; however, accumulating knowledge of their underlying genomic landscape has opened some opportunities for targeted approaches, for example, the vascular endothelial growth factor tyrosine kinase inhibitor pazopanib, which is already in clinical use for the treatment of many types of advanced soft tissue sarcomas.

Isolated limb infusion as a treatment option for malignant granular cell tumour.

A 54-year-old man presented with a painful lesion on the right posterior calf with MRI identifying a 5 cm lesion in the medial head of the gastrocnemius. He underwent wide local excision of the tumour, and the final pathology was consistent with atypical granular cell tumour. Three years later, he developed a recurrent right popliteal mass. Complete staging workup also identified multiple lung nodules and a caecal polyp that were consistent with metastatic granular cell tumour. He was started on pazopanib and deemed a poor candidate for palliative resection due to encasement of the popliteal vessels. The patient refused above-the-knee amputation (AKA) at that point and was evaluated for isolated limb infusion as an alternative. He received three cycles of isolated limb infusion within a 2-year period and achieved good response from the first two cycles. He underwent AKA 4 years after his diagnosis of malignant granular cell tumours and is currently doing well.

Malignant granular cell tumor of the breast; literature review.

Malignant granular cell tumor (MGCT) is rare tumors that comprise 1-2% of all granular cell tumors. They commonly arise on lower extremity, nuchal region, chest wall, gastrointestinal tract, head, and neck but very rarely in breast. We report a case of a MGCT of breast with review of literature. The patient had noticed a breast mass 4 years back which was operated, and wide local excision was done. The tumor was diagnosed as MGCT. The tumor fulfilled 3 of the 6 criteria of Fanburg-Smith et al. The patient received 8 cycles of chemotherapy thereafter with 4 cycles of antharacycline and 4 of taxanes. However, the tumor reoccurred 4 years after resection and grew rapidly. Contrast-enhanced computed tomography done showed a large lobulated breast mass with axillary lymph node metastasis. She underwent Modified Radical Mastectomy with axillary clearance. The histopathology this time also revealed similar malignant tumor. To the best of our knowledge, only 7 cases have been reported in indexed English literature occurring primarily in breast.

Uterine cervical malignant granular cell tumor.

Malignant granular cell tumor is relatively uncommon, constituting only 1-2% of all granular cell tumors. It is a rare and unusual tumor, especially in non-typical sites, such as the uterine cervix, and grows more rapidly than benign granular cell tumor. It can be treated with surgical excision, but recurrence is possible and prognosis can be poor. A malignant granular cell tumor in the uterine cervix of a 37-year-old woman was incidentally diagnosed. The patient has a history of irregular vaginal bleeding. Uterine cervical biopsy under colposcope revealed a malignant granular cell tumor. After isophosphamide, etoposide, and cisplatin neoadjuvant chemotherapy, surgery was performed on the lesion, which approximately involved half the depth of cervical stroma. Computed tomography examination showed no local recurrence or distant metastasis during the 26-month follow-up period.

Malignant bronchial Abrikossoff's tumor and small cell lung cancer: a case report and review.

A 61-year-old male Caucasian smoker patient underwent chest radiography and CT scan for persistent non-inflammatory cough, which showed a left bronchial unresectable mass. Bronchoscopy showed an endobronchial mass; washing cytology was negative and histology findings suggested diagnosis of granular cell tumor (GCT), also called Abrikossoff's tumor. After 3 weeks a new washing cytology test revealed the presence of small cell lung cancer (SCLC). A CT-scan and chest radiography showed a 30% increase in the maximum diameter of the lesion, clinically defining the primary neoplasm as malignant. The patient was referred to our institution and started chemotherapy with cisplatin and etoposide. After 6 cycles of treatment, the CT scan showed complete, disappearance of the neoplasm and bronchoscopy examination showed no endobronchial lesion, defining the mucosal surface as normal. We have reviewed and summarized the international literature with regard to bronchial localization of malignant granular cell tumor and its association with SCLC, therefore concluding that our case is the first malignant endobronchial GCT linked to SCLC.