Intradural, extramedullary spinal cord granular cell tumor: A case report and clinicopathologic review of the literature

Abstract

Granular cell tumor (GCT) of the spine is uncommon, with intradural extramedullary location being exceptionally rare. The non-specific clinical presentation and variable histologic patterns can make recognition of this tumor challenging. Two previous reports of GCT of the spine were reviewed (Medline 1960-2009) and analyzed with respect to this case report. The patients included two women and one man (mean age, 28.7 years). Patients presented with 3 to 4 months of lower back pain and/or lower extremity radiculopathy. The lesions appeared radiographically to be intradural and extramedullary or intramedullary. The tumors were found at T10 or L1-L2 space. Radiographically, all tumors enhanced homogenously on T1 post-gadolinium imaging with a mean tumor size of approximately 1.6 cm. Histologically, the tumors were composed of large, polygonal granular cells. The abundant cytoplasm was fine or coarsely granular, surrounding small, pale-staining nuclei, which were eccentrically located in the cell. The tumor cells were periodic acid Schiff positive, diastase resistant, and were positive with S-100 protein, CD68, inhibin, and neuron-specific enolase immunohistochemistry. The clinical and histologic differential diagnosis includes schwannoma, neurofibroma, meningioma, astrocytoma, melanocytoma, and metastatic tumors. Patients were managed with excision. One patient had symptomatic and radiographic local recurrence that was subsequently treated with radiation, resulting in stabilization of disease and symptoms. Intradural GCTs of the spine are rare and radiographically indistinguishable from tumors that more commonly arise in this location. Histologic recognition of this rare tumor is important because the subsequent clinical course of the disease differs from other similar lesions.

Intradural spinal granular cell tumor

“…[234567] Brain and pituitary are another uncommon site for GCT within CNS. Average age of patients with intraspinal GCT is 23 years, with a typical age range of 13 to 49 years and striking female preponderance.…”

“…[9] Although the intradural tumors described in thus far have been benign histologically, they can cause serious morbidity if not diagnosed and treated correctly due to the anatomic confines of the canal. [34567]…”

Granular Cell Tumor of the Intradural Extramedullary Spinal Cord : Report of Two Cases with Respect to Radiological Differential Diagnosis

“…The tumor clusters consist of small, hyperchromatic to vesicular nuclei and background fibrous connective tissue. The islands of the tumors are separated by delicate fibrovascular tissue12). Case 1 showed fibrosis and vacuole changes, and Case 2 demonstrated hyalinizing fibrosis and lymphocytic infiltration.…”

“…GrCTs can affect any part of the body but are most commonly found in the skin, tongue, ovaries, brain, and breast tissue5,6,9). Spinal intradural extramedullary (IDEM) GrCTs are exceptionally rare, and only 6 cases have been reported in the literature2,5,8,10-12).…”

Recurrent granular cell tumor: a case report and review of literature

“…Complete surgical excision is the treatment of choice (19,20). However, incomplete excision requires regular clinico-radiological follow-up and adjuvant radiotherapy.…”