A rare case of a non-neural granular cell tumor presenting as an enlarging right chest wall nodule in a 4-year-old male and review of the literature

Abstract

Non-neural granular cell tumor was first described in 1991 as an unusual primitive, polypoid variant of the conventional granular cell tumor. To date, this neoplasm remains a rare entity and the cell of origin is uncertain. While the histological features are similar to the conventional granular cell tumor, it represents a distinct entity that is negative for S100 and lacks true nerve sheath differentiation. Here, we describe a case of a 4-year-old male who presented with a painless, soft nodule on his right chest wall that was slowly increasing in size. The mass was excised and sent for pathologic analysis. Microscopic examination reveals spindle and epithelioid cells with vesicular nuclei and prominent granular eosinophilic cytoplasm. Immunohistochemical analysis shows negative staining for S100 and AE1/AE3/PCK26 but is positive for CD68. A diagnosis of a non-neural granular cell tumor was made. We report a rare and diagnostically challenging case in a pediatric patient.

Granular Cell Tumor of Soft Tissue – A Rare Non-Neural Variant

“…Cells are polygonal, oval or spindle with abundant eosinophilic cytoplasm. The diagnosis of certainty is made by histopathological and immunohistochemical examination [4] . These are usually negative for S-100, SOX10 which differentiate PNNGCT from conventional GrCT.…”

“…Primitive non-neural granular cell tumor(PNNGCT) is a variant of GrCT which is even more rare, low grade neoplasm of mesenchymal cells of unknown lineage [2,3] . These tumors are most common in children and young adults, and often involves back and extremities [4][5][6] . Less than 100 cases of PNNGCT have been reported so far and these are usually benign.…”

An update on cutaneous granular cell tumours for dermatologists and dermatopathologists

“…Whereas GCTs are most common in the skin, oral cavity and gastrointestinal tract, PNGCTs most commonly arise on the trunk. 33 The behaviour of PNGCTs is similar to that of benign GCTs, with complete excision being curative. One report exists of a single PNGCT lymph node metastasis, for which lymphadenectomy was curative.…”

“…PNGCTs have a similarly wide age range (5–83 years) but more commonly present in younger patients (median age 16 years). Whereas GCTs are most common in the skin, oral cavity and gastrointestinal tract, PNGCTs most commonly arise on the trunk 33 …”

Polypoid non-neural granular cell tumor of the oral cavity

“…We found 15 articles in the English language literature that document cases or case series of NN-GCT (3)(4)(5)(6)(7)(8)(9)(10)(11)(12)(13)(14)(15)(16)(17) and only six describe cases involving the oral cavity 3,4,[6][7][8][9] . This study reports a case of oral NN-GCT with atypical histopathological characteristics.…”