Abstract
Malignant granular cell tumors are rare, intensely aggressive entities. This paper presents a case of a large rapidly recurrent malignant granular cell tumor with regional and distal metastases on the back of a 54-year-old Cuban man. The primary tumor recurred within six months of the original wide local excision and with satellite lesions apparent at twelve months, and the mass was diagnosed using the histological criteria established by Fanburg-Smith et al. for malignant granular cell tumors. By fifteen months, right axillary lymphadenopathy, multiple satellite lesions, pulmonary nodules, and distant metastasis in the right thigh were present. At sixteen months, wide local excision of recurrent mass and local satellite masses along with right axillary dissection and placement of Integra with subsequent split-thickness skin graft were performed by surgical oncology and plastic surgery teams. The surgical specimen measured 32.0 × 13.5 × 5.5 cm, containing multiple homogeneous masses with the largest mass 22.0 × 9.0 × 4.6 cm. Following surgery, patient was started on Pazopanib 800 mg/day based on phase III randomized trial data in the treatment of soft tissue sarcomas showing this as a potential novel therapy for malignant granular cell tumors.
Highlights
Granular cell tumors, first described by Abrikossoff in 1926 as myoblastomas [1], are tumors of Schwannian cell origin that may be classified as either benign or malignant
While both benign and malignant granular cell tumors present in a similar age range of 30–50 years, Malignant granular cell tumors (MGCT) are more likely to affect African-Americans than whites and are two times more likely to occur in females than males [5]
Local recurrence and metastasis are relatively common in malignant granular cell tumors, with 32% rate of recurrence and 50% metastasis in the Fanburg-Smith analysis
Summary
First described by Abrikossoff in 1926 as myoblastomas [1], are tumors of Schwannian cell origin that may be classified as either benign or malignant. Granular cell tumors represent 0.5% of all soft tissue tumors [4], and the Malignant granular cell tumors (MGCT) are exceedingly rare representing less than 1-2% of all granular cell tumors [5, 6]. Involves a large rapidly recurrent malignant granular cell tumor of the skin and subcutaneous tissue of the back with regional and distant metastases, which demonstrates the capacity of these tumors to recur and to metastasize within a very limited amount of time.
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