Spitz Nevus
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Physicians have known for almost a century that some childhood lesions histologically diagnosed as melanomas did not show malignant behavior. The terms juvenile melanoma and prepubertal melanoma were used to describe such lesions.
Several attempts were made to establish objective criteria that would clearly delineate Spitz nevi and melanomas. The controversial classification of some cases as metastasizing Spitz nevi further confounded the issue; however, such cases illustrate the difficulty of accurately distinguishing some Spitz nevi from melanoma based on histological criteria alone. Even today, no set of criteria can be used to predict the clinical outcome of atypical Spitz tumors with absolute assurance.
A Spitz nevus can arise de novo or in association with an existing melanocytic nevus. Some may be fast growing. [1]
The cause of Spitz nevi is not known.
Exact data on incidence or prevalence are not available. Spitz nevi are estimated to represent less than 1% of all childhood melanocytic nevi.
Spitz nevi have been described most frequently in fair-skinned individuals. One study reviewed 130 cases in a Hispanic population, demonstrating that Spitz nevi are not restricted to white patients. [2]
Both sexes are equally affected. Some authors describe a slight female predominance.
About 50% of cases occur in children younger than 10 years; 70% of all cases are diagnosed during the first 2 decades of life.
The prognosis is good. Recurrences should be treated with re-excision. These lesions are clinically benign. A 2011 study reporting on 157 patients with Spitz-type melanotic lesions suggests that atypical Spitz tumors pose a minimal threat of mortality but have an increased risk of melanoma and a moderate risk of metastasis to regional nodes. Aggressive treatment is usually not needed, but monitoring for signs of relapse, as well as subsequent melanomas, is recommended. [3] Mitoses and inflammation are indicators of increased aggressiveness. [4] Using single morphologic features to determine prognosis has severe limitations. [5]
Educate patients about sun protection and self-examination of the skin. For patient education materials, see the Cancer and Tumors Center.
Pedrazini MC, Montalli VAM, Souza EM. The Fast Clinical Evolution of a Spitz Nevus: Three-Year Follow-Up of a Child. Rev Paul Pediatr. 2017 Oct-Dec. 35 (4):476-479. [Medline].
Berlingeri-Ramos AC, Morales-Burgos A, Sánchez JL, Nogales EM. Spitz Nevus in a Hispanic Population: A Clinicopathological Study of 130 Cases. Am J Dermatopathol. 2010 Jan 22. [Medline].
Sepehr A, Chao E, Trefrey B, et al. Long-term Outcome of Spitz-Type Melanocytic Tumors. Arch Dermatol. 2011 Oct. 147(10):1173-9. [Medline].
Luo S, Sepehr A, Tsao H. Spitz nevi and other Spitzoid lesions part I. Background and diagnoses. J Am Acad Dermatol. 2011 Dec. 65(6):1073-84. [Medline]. [Full Text].
Massi D, Tomasini C, Senetta R, Paglierani M, Salvianti F, Errico ME, et al. Atypical Spitz tumors in patients younger than 18 years. J Am Acad Dermatol. 2015 Jan. 72(1):37-46. [Medline].
Li CC, Harrist TJ, Noonan VL, Woo SB. Intraoral Spitz nevus: case report and literature review. Oral Surg Oral Med Oral Pathol Oral Radiol. 2013 Oct 16. [Medline].
Rossiello L, Zalaudek I, Ferrara G, Docimo G, Giorgio CM, Argenziano G. Melanoacanthoma simulating pigmented spitz nevus: an unusual dermoscopy pitfall. Dermatol Surg. 2006 May. 32(5):735-7. [Medline].
Moscarella E, Lallas A, Kyrgidis A, Ferrara G, Longo C, Scalvenzi M, et al. Clinical and dermoscopic features of atypical Spitz tumors: A multicenter, retrospective, case-control study. J Am Acad Dermatol. 2015 Nov. 73 (5):777-84. [Medline].
Fullen DR, Poynter JN, Lowe L, et al. BRAF and NRAS mutations in spitzoid melanocytic lesions. Mod Pathol. 2006 Oct. 19(10):1324-32. [Medline].
Gill M, Cohen J, Renwick N, Mones JM, Silvers DN, Celebi JT. Genetic similarities between Spitz nevus and Spitzoid melanoma in children. Cancer. 2004 Dec 1. 101(11):2636-40. [Medline].
van Dijk MC, Bernsen MR, Ruiter DJ. Analysis of mutations in B-RAF, N-RAS, and H-RAS genes in the differential diagnosis of Spitz nevus and spitzoid melanoma. Am J Surg Pathol. 2005 Sep. 29(9):1145-51. [Medline].
Requena C, Heidenreich B, Kumar R, Nagore E. TERT promoter mutations are not always associated with poor prognosis in atypical spitzoid tumors. Pigment Cell Melanoma Res. 2017 Mar. 30 (2):265-268. [Medline].
Yeh I, Tee MK, Botton T, Shain AH, Sparatta AJ, Gagnon A, et al. NTRK3 kinase fusions in Spitz tumours. J Pathol. 2016 Nov. 240 (3):282-290. [Medline].
Lazova R, Seeley EH, Kutzner H, et al. Imaging mass spectrometry assists in the classification of diagnostically challenging atypical Spitzoid neoplasms. J Am Acad Dermatol. 2016 Dec. 75 (6):1176-1186.e4. [Medline].
Da Forno PD, Fletcher A, Pringle JH, Saldanha GS. Understanding spitzoid tumours: new insights from molecular pathology. Br J Dermatol. 2008 Jan. 158(1):4-14. [Medline].
Valdebran M, Elbendary A, Chaitanya Arudra SK, Torres KM, Elattar I, Elston DM. Nuclear and cytoplasmic features in the diagnosis of banal nevi, Spitz nevi, and melanoma. J Am Acad Dermatol. 2016 Nov. 75 (5):1032-1037.e8. [Medline].
Zhao G, Lee KC, Peacock S, Reisch LM, Knezevich SR, Elder DE, et al. The utilization of spitz-related nomenclature in the histological interpretation of cutaneous melanocytic lesions by practicing pathologists: results from the M-Path study. J Cutan Pathol. 2017 Jan. 44 (1):5-14. [Medline].
Cho-Vega JH. A diagnostic algorithm for atypical spitzoid tumors: guidelines for immunohistochemical and molecular assessment. Mod Pathol. 2016 Jul. 29 (7):656-70. [Medline].
Egberts F, Kaehler KC, Brasch J, Schwarz T, Cerroni L, Hauschild A. Multiple skin metastases of malignant melanoma with unusual clinical and histopathologic features in an immunosuppressed patient. J Am Acad Dermatol. 2008 May. 58(5):880-4. [Medline].
Nojavan H, Cribier B, Mehregan DR. [Desmoplastic Spitz nevus: a histopathological review and comparison with desmoplastic melanoma]. Ann Dermatol Venereol. 2009 Oct. 136(10):689-95. [Medline].
Ko CJ, McNiff JM, Glusac EJ. Melanocytic nevi with features of Spitz nevi and Clark’s/dysplastic nevi (“Spark’s” nevi). J Cutan Pathol. 2009 Oct. 36(10):1063-8. [Medline].
Sabater Marco V, Escutia Muñoz B, Morera Faet A, Roig MM, Botella Estrada R. Pseudogranulomatous Spitz nevus: a variant of Spitz nevus with heavy inflammatory infiltrate mimicking a granulomatous dermatitis. J Cutan Pathol. 2012 Sep 18. [Medline].
Gelbard SN, Tripp JM, Marghoob AA, et al. Management of Spitz nevi: a survey of dermatologists in the United States. J Am Acad Dermatol. 2002 Aug. 47(2):224-30. [Medline].
Murphy ME, Boyer JD, Stashower ME, Zitelli JA. The surgical management of Spitz nevi. Dermatol Surg. 2002 Nov. 28(11):1065-9; discussion 1069. [Medline].
Caracò C, Mozzillo N, Di Monta G, Botti G, Anniciello AM, Marone U, et al. Sentinel lymph node biopsy in atypical Spitz nevi: is it useful?. Eur J Surg Oncol. 2012 Oct. 38(10):932-5. [Medline].
Rapini RP. Spitz nevus or melanoma?. Semin Cutan Med Surg. 1999 Mar. 18(1):56-63. [Medline].
Zoltan Trizna, MD, PhD Private Practice
Zoltan Trizna, MD, PhD is a member of the following medical societies: Texas Medical Association
Disclosure: Nothing to disclose.
Ronald P Rapini, MD Professor and Chair, Department of Dermatology, The University of Texas MD Anderson Cancer Center; Distinguished Chernosky Professor and Chair of Dermatology, Professor of Pathology, University of Texas McGovern Medical School at Houston
Ronald P Rapini, MD is a member of the following medical societies: American Academy of Dermatology, American Dermatological Association, American Medical Association, American Society for Dermatologic Surgery, American Society for MOHS Surgery, American Society of Dermatopathology, Association of Professors of Dermatology, Society for Investigative Dermatology, Texas Dermatological Society
Disclosure: Book royalties from Elsevier publishers.
Richard P Vinson, MD Assistant Clinical Professor, Department of Dermatology, Texas Tech University Health Sciences Center, Paul L Foster School of Medicine; Consulting Staff, Mountain View Dermatology, PA
Richard P Vinson, MD is a member of the following medical societies: American Academy of Dermatology, Texas Medical Association, Association of Military Dermatologists, Texas Dermatological Society
Disclosure: Nothing to disclose.
Rosalie Elenitsas, MD Herman Beerman Professor of Dermatology, University of Pennsylvania School of Medicine; Director, Penn Cutaneous Pathology Services, Department of Dermatology, University of Pennsylvania Health System
Rosalie Elenitsas, MD is a member of the following medical societies: American Academy of Dermatology, American Medical Association, American Society of Dermatopathology, Pennsylvania Academy of Dermatology
Disclosure: Received royalty from Lippincott Williams Wilkins for textbook editor.
Dirk M Elston, MD Professor and Chairman, Department of Dermatology and Dermatologic Surgery, Medical University of South Carolina College of Medicine
Dirk M Elston, MD is a member of the following medical societies: American Academy of Dermatology
Disclosure: Nothing to disclose.
Maureen B Poh-Fitzpatrick, MD Professor Emerita of Dermatology and Special Lecturer, Columbia University College of Physicians and Surgeons
Maureen B Poh-Fitzpatrick, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Dermatology, New York Academy of Medicine, New York Dermatological Society
Disclosure: Nothing to disclose.
Spitz Nevus
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