A Malignant Granular Cell Tumor Excised with Mohs Micrographic Surgery

Abstract

Malignant granular cell tumors are extremely rare, aggressive neoplasms displaying rapid growth and frequent associated metastatic disease. Excision and evaluation for metastatic disease are mandatory. We present a 54-year-old patient with a malignant granular cell tumor, treated with Mohs micrographic surgery. Cutaneous granular cell tumors are uncommon neoplasms, likely of perineural origin. Most follow a benign and uneventful course, with wide local excision being the treatment of choice (Enzinger, 1988). The malignant granular cell tumor is an extremely rare, aggressive variant, which provides a diagnostic challenge and management dilemma, especially with early presentation when it may be mistaken for other entities. There is also controversy regarding surgical management and follow-up of both benign and malignant granular cell tumors.

Histopathological Features of Granular Cell Tumor

“…The histologic criteria to differentiate the benign GCTs from their malignant counterpart include: presence of necrosis, spindling of the cells, vesicular nuclei with prominent nucleoli, nuclear pleomorphism, high N/C ratio and increased mitoses (>2/10 high power field). 4,8,9 GCTs with three or more of these criteria are classified as malignant and as atypical if they have two of these features. The presented case showed (focal) spindling of the cells but without any other criteria of malignancy.…”

Malignant Granular Cell Tumor of the Back: A Case Report and Review of the Literature

“…Currently, surgical resection remains the best possible option as no chemotherapy or radiation has been reported effective. Wide local resection is generally the treatment of choice; however, Mohs micrographic surgery has been utilized for malignant and benign granular cell tumors [21–26]. Although in this case, in addition to the wide local resection, Pazopanib was recently initiated as monotherapy.…”

Granular Cell Tumor of Brachial Plexus Mimicking Nerve Sheath Tumor: A Case Report

“…Although most of cases follow a benign course, 1-2% of granular cell tumors exhibit malignant behavior and behave like high-grade sarcomas with a high rate of metastases and short survival 2 , 4 , 5 , 18) . Tumors typically spread via lymphatic and hematogenous routes to the lungs, liver, and skeletal structures.…”

“…Primary tumors of the brachial plexus are rare and granular cell tumors of this region is an extremely rare disease 3 , 11 , 12) . It mostly presents as an asymptomatic solitary nodule and can be found in any kind of tissue 2 , 3 , 4 , 6 , 7 , 8 , 9 , 10 , 14 , 16 , 17) . It is often misdiagnosed because of its usually subtle presentation with histological examination setting the correct diagnosis subsequently.…”

“…Granular cell tumors behave in a benign fashion, but has a tendency to recur 4) . Surgical removal with wide margins is the treatment of choice as malignant changes have been reported after long-term follow-up 2) .…”