Atypical/Borderline and Malignant Spitz Tumors

Atypical/Borderline and Malignant Spitz Tumors

David Cassarino, MD, PhD

Clinical photograph shows an atypical compound Spitz tumor in a child. The tumor is large and somewhat nodular appearing. The focal epidermal defect image represents a biopsy site. (Courtesy J. Wu, MD.)

Spitzoid melanoma in a young adult patient shows very marked atypia and pleomorphism, as well as prominent pagetoid scatter image extending throughout the full thickness of the epidermis.



  • Atypical Spitz tumor (AST)


  • Atypical spitzoid melanocytic proliferation

  • Spitz tumor of borderline/uncertain malignant potential

  • Spitzoid melanoma


  • Atypical/borderline Spitz tumor: Severely atypical melanocytic proliferation with features intermediate between benign Spitz nevus and spitzoid melanoma

  • Spitzoid melanoma: Malignant melanocytic proliferation with spitzoid features



  • Typically occur in young patients without any known risk factors for melanoma

  • Sun exposure may be a risk factor in some cases

Possible Genetic Role

  • Suggested by chromosome 11p amplification in a minority of cases



  • Incidence

    • Very rare tumors

  • Age

    • Mostly young patients, range: 2-30 years

    • Malignant tumors more common in post-puberty age group


  • Dermal papule or nodule


  • Surgical approaches

    • Complete and wide excision is standard therapy

      • Typically ≥ 5 mm clinical margins for atypical/borderline tumors

      • Spitzoid melanomas should be treated with same margins as invasive melanomas of similar Breslow depth

    • Sentinel lymph node biopsy is highly controversial

      • Should be considered a prognostic rather than therapeutic procedure at this time

      • Likely should not be routinely used (especially for atypical/borderline tumors) until validated by extensive clinical studies


  • Difficult to predict

    • Most patients have relatively good prognosis, with only a minority of cases progressing to death

    • Even patients with lymph node involvement have a relatively good prognosis, as most tumors do not disseminate further

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Jul 8, 2016 | Posted by in PATHOLOGY & LABORATORY MEDICINE | Comments Off on Atypical/Borderline and Malignant Spitz Tumors

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