Malignant Cutaneous Neurocristic Hamartoma With Features of Melanoma: A Rare Entity

Abstract

Neurocristic cutaneous hamartoma (NCH) is a rare, benign neoplastic skin lesion characterized by a combination of neuroectodermal and mesodermal components. Clinically, NCH typically presents as asymptomatic, well-circumscribed, and elevated cutaneous nodules. Histopathologically, it is characterized by nests of pigmented melanocytes and varying degrees of fibrosis and collagen deposition. The precise etiology of NCH remains undetermined; however, it is hypothesized to arise from the aberrant development of neuromesenchyme. Due to its potential to mimic other pigmented melanocytic disorders, accurate differential diagnosis is crucial to prevent mismanagement. Surgical excision is the preferred treatment modality, offering a generally favorable prognosis and low recurrence rate. Conversely, malignant cutaneous neurocristic hamartoma (MCNH), an exceedingly rare malignant variant of NCH, poses a significantly different clinical challenge. This review focuses on the diagnostic criteria, clinical presentation, and management strategies for MCNH, emphasizing the need for differentiation from other similar cutaneous lesions. We present a detailed case report of MCNH in a 56-year-old female, highlighting its histopathological and immunohistochemical features to provide insights into the diagnosis and therapeutic approach for this exceptionally rare malignancy.