Tahir Muhammad, Samman Abdulhadi, Shalin Sara, Knowles Kurt, Herrera Guillermo A, Liles Joe S, Phung Thuy
Pathology and Laboratory Medicine, University of South Alabama College of Medicine, Mobile, USA.
Basic Medical Sciences, Pathology Division, College of Medicine, University of Jeddah, Jeddah, SAU.
Cureus. 2024 Sep 17;16(9):e69594. doi: 10.7759/cureus.69594. eCollection 2024 Sep.
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.
神经嵴性皮肤错构瘤（NCH）是一种罕见的良性肿瘤性皮肤病变，其特征是神经外胚层和中胚层成分相结合。临床上，NCH通常表现为无症状、边界清晰且隆起的皮肤结节。组织病理学上，其特征为色素沉着的黑素细胞巢以及不同程度的纤维化和胶原沉积。NCH的确切病因仍未明确；然而，据推测它起源于神经间充质的异常发育。由于其有模仿其他色素沉着性黑素细胞疾病的可能，准确的鉴别诊断对于防止管理不当至关重要。手术切除是首选的治疗方式，通常预后良好且复发率低。相反，恶性皮肤神经嵴错构瘤（MCNH）是NCH极其罕见的恶性变体，带来了显著不同的临床挑战。本综述重点关注MCNH的诊断标准、临床表现和管理策略，强调需要与其他类似皮肤病变进行鉴别。我们报告了一例56岁女性MCNH的详细病例，突出其组织病理学和免疫组化特征，以提供对这种极其罕见恶性肿瘤的诊断和治疗方法的见解。
