Pediatric cellular neurothekeoma: Seven cases and systematic review of the literature

Abstract

Background/Objectives Neurothekeoma is a rare, benign, cutaneous neoplasm consisting of Schwann cells and perineural cells in myxoid stroma. Cellular neurothekeoma (CNT) was previously thought to represent a morphologic variant of neurothekeoma, but recent studies have shown that CNTs are unrelated to neurothekeomas and are more likely of histiocytic lineage. Methods Herein, we describe seven cases of CNT in pediatric patients. A comprehensive search of PubMed was performed, and 71 cases of cellular neurothekeoma in pediatric patients were reviewed. Results The clinical differential diagnosis for these lesions included Spitz nevi, keloid, juvenile xanthogranuloma, cutaneous lymphoid hyperplasia, and lymphomatoid papulosis. All cases were treated by excision or excisional biopsy. Histopathologically, all demonstrated multilobular, primarily intradermal neoplasms composed of plump spindled or epithelioid mononuclear cells with abundant eosinophilic pale‐staining cytoplasm. Immunophenotypic findings included CD68 and NKI/C3 positivity, and negative staining with cytokeratin, S‐100, Melan‐A, and SOX‐10. Conclusion Cellular neurothekeoma is distinguished from conventional neurothekeoma by increased cellularity, a lack of myxoid stroma, and a lack of neural expression with immunohistochemical stains. These uncommon neoplasms should be included in the differential diagnosis of dermal nodules in children. Accurate diagnosis of these lesions is essential, as they can be mistaken for malignancy leading to unnecessary treatment.

Cellular neurothekeoma occurring at the site of radiation therapy in a child with medulloblastoma

“…Clinical features of cellular neurothekeoma in children are similar to adults, except that unlike rare local recurrence described in adults, no local recurrence has been reported in children following surgical excision 1,3 . Atypical histological features, including nuclear atypia, increased mitoses, infiltrative borders, and perineural invasion may be present, but do not predict clinical behavior 4,5 .…”

“…Clinical features of cellular neurothekeoma in children are similar to adults, except that unlike rare local recurrence described in adults, no local recurrence has been reported in children following surgical excision. 1,3 Atypical histological features, including nuclear atypia, increased mitoses, infiltrative borders, and perineural invasion may be present, but do not predict clinical behavior. 4,5 Differential diagnoses of cellular neurothekeoma include nerve sheath myxoma, superficial angiomyxoma, JXG, melanocytic neoplasia, benign fibrous histiocytoma, and plexiform fibrohistiocytic tumor.…”

Neurothekeoma located in the hallux and axilla: Two case reports

“…Most tumours also express CD99. A few tumours express SMA and are negative for S-100, cytokeratin, Melan-A, and SOX-10 staining[ 11 , 12 ], indicating that NTKs are associated with scattered histiocytes.…”

The role of dermoscopy in the diagnosis of neurothekeoma – personal observations and review of previously reported cases

“…[4][5][6] Differential diagnosis of neurothekeoma includes but is not limited to basal cell carcinoma, epidermal cyst, dermatofibroma, adnexal skin tumors, neurofibroma, amelanotic melanoma, Spitz nevus, hemangioma, keloid, juvenile xanthogranuloma, cutaneous lymphoid hyperplasia, lymphomatoid papulosis, and insect bite. 2,5,9 Based on the current knowledge, histopathological examination remains the diagnostic standard. Future studies would be needed to precisely investigate the association between dermoscopic features and tumor location, histopathological type as well as skin phototype.…”