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.


TERMINOLOGY


Abbreviations



  • Atypical Spitz tumor (AST)


Synonyms



  • Atypical spitzoid melanocytic proliferation


  • Spitz tumor of borderline/uncertain malignant potential


  • Spitzoid melanoma


Definitions



  • 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


ETIOLOGY/PATHOGENESIS


Unknown



  • 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


CLINICAL ISSUES


Epidemiology



  • Incidence



    • Very rare tumors


  • Age



    • Mostly young patients, range: 2-30 years


    • Malignant tumors more common in post-puberty age group


Presentation



  • Dermal papule or nodule


Treatment



  • 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


Prognosis



  • 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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