Malignant granular cell tumor of the median nerve: a case report with a literature review of 157 cases

Abstract

Malignant granular cell tumors are an extremely rare, high-grade sarcoma with a schwannian phenotype and are composed of malignant granular cells with cytoplasmic lysosomal inclusion. To date, 157 cases of malignant granular cell tumors have been reported. We report the first case of a malignant granular cell tumor arising from the digital nerve to the median nerve in the palm, and we review the 157 previously reported cases and summarize the clinical profile, treatment, and outcome of this tumor. The median age, tumor size, and follow-up periods were 51 years, 6 cm, and 24 months respectively. With respect to the oncological result, 53 patients (33.8%) had no evidence for disease, 31 (19.7%) were alive with the disease, and 51 (32.5%) died because of the disease. Our case report indicates that rare malignant tumors can arise from the digital nerve to the median nerve in the palm, an anatomical site that is usually affected by benign lesions. Exhaustive discussions between surgeons and pathologists are necessary for the treatment of this rare malignant tumor.

Metastasizing Malignant Granular Cell Tumor (Abrikossoff Tumor) of the Anterior Abdominal Wall, with Prolonged Survival

“…There were 99 females (63% of total cases) and 58 males (37%), with female to male ratio of 1.7:1. The commonest sites of the tumor were the trunk and the thighs [11]. Fanburg-Smith et al reviewed 46 cases of MGCTs from the Armed Forces Institute of Pathology, with a mean age of 40 years.…”

“…On the other hand, malignant granular cell tumors (MGCTs) are exceedingly rare high-grade malignant mesenchymal neoplasms, representing only 1%-2% of all GCTs [9]. In a study of 6,412 soft tissue sarcomas, 26 cases (0.4%) were diagnosed as MGCT [10], with 157 cases in various anatomical sites on record, documented in a review in 2018 [11]. When the tumor has malignant cytological features or when a benign-appearing tumor causes metastasis or death, it is considered malignant.…”

Malignant granular cell tumor of the lumbar region – a case report and review of the criteria for diagnosis

“…A wide excisional margin is optimal because of the infiltrative pattern of growth and the tendency to recur. It has been described that MGCTs can result from malignant transformation of benign GCT, so margins preservation is highly recommended also for benign--appearing lesions [6][7][8].…”

Bladder malignant granular cell tumor with EP300 gene mutation: a case report and literature review

“…Most of the clinical manifestations are painless masses in subcutaneous or deep soft tissue. Most of the cases occurred in the extremities, torso, head, neck, and breast [10][11][12][13][14][15][16]. To date, few cases of bladder malignant GCT have been reported [7,17].…”