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Spitzoid melanocytic neoplasms (Spitz nevus and atypical Spitz tumors)

Raymond L Barnhill, MD
Jinah Kim, MD
Section Editor
Hensin Tsao, MD, PhD
Deputy Editor
Rosamaria Corona, MD, DSc


Spitz tumor is an uncommon melanocytic lesion composed of large epithelioid and/or spindled cells. It typically presents in childhood or adolescence as a sharply circumscribed, dome-shaped, pink-red papule most commonly located on the face or lower extremities (picture 1). The clinical relevance of Spitz tumor lies in its close histologic resemblance to melanoma. In some cases, differentiating Spitz tumors from melanoma may be difficult or impossible even for the expert pathologist. Spitzoid lesions are often not classified in any standardized way, evoke uncertainty in diagnosis by pathologists, and elicit variability in treatment recommendations [1].

The classification, epidemiology, clinical manifestations, diagnosis, and management of Spitz tumors will be discussed here. Congenital nevi, acquired melanocytic nevi, and atypical nevi are discussed separately. (See "Congenital melanocytic nevi" and "Acquired melanocytic nevi (moles)" and "Atypical (dysplastic) nevi".)


Spitz tumors were first described by Darier and Civatte in 1910 [2]. In 1948 Sophie Spitz articulated for the first time criteria for this unusual group of childhood melanocytic lesions that histologically resembled melanomas but did not demonstrate the typical aggressive clinical behavior commonly associated with adult melanomas [3]. Of interest, one of Spitz's original 13 cases resulted in metastasis and death of the patient. These lesions were termed "juvenile melanomas" to distinguish them from adult melanoma and benign nevi of childhood. Subsequently, various terms have been used for this group of lesions including "Spitz nevus," "Spitz tumor," and "spindle and epithelioid cell nevus."

Atypical variants of Spitz tumors have been termed "atypical Spitz tumors," "spindle and epithelioid cell nevus with atypia and metastasis," "melanocytic tumor of unknown malignant potential," or "spitzoid tumor of uncertain malignant potential" [4-6].

In this topic we will use the term "Spitz tumor" as a general term for all types of Spitz lesions. Spitz lesions with one or more atypical features and those with indeterminate biologic behavior will be referred to as "atypical Spitz tumors." (See 'Classification' below.)

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Literature review current through: Oct 2017. | This topic last updated: Aug 10, 2017.
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