Sections

Spitz Nevus

Overview

Background

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.

Pathophysiology

A Spitz nevus can arise de novo or in association with an existing melanocytic nevus. Some may be fast growing.^[1]

Etiology

Molecular techniques have defined subtypes of Spitz tumors, including benign Spitz nevi and spitzoid melanoma.^{[2, 3, 4, 5, 6, 7, 8]}

Frequency

Exact data on incidence or prevalence are not available. Spitz nevi are estimated to represent less than 1% of all childhood melanocytic nevi.

Race

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.^[9]

Sex

Both sexes are equally affected. Some authors describe a slight female predominance.

Age

About 50% of cases occur in children younger than 10 years; 70% of all cases are diagnosed during the first 2 decades of life.

Prognosis

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.^[10] Mitoses and inflammation are indicators of increased aggressiveness.^[11]Using single morphologic features to determine prognosis has severe limitations.^[12]

Patient Education

Educate patients about sun protection and self-examination of the skin. For patient education materials, see the Cancer and Tumors Center.

Clinical Presentation

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. [QxMD MEDLINE Link].
Kim D, Khan AU, Compres EV, Zhang B, Sunshine JC, Quan VL, et al. BRAF Fusion Spitz Neoplasms, Clinical, Morphologic and Genomic Findings in 6 Cases. J Cutan Pathol. 2020 Aug 9. [QxMD MEDLINE Link].
Raghavan SS, Wang JY, Kwok S, Rieger KE, Novoa RA, Brown RA. PRAME expression in melanocytic proliferations with intermediate histopathologic or spitzoid features. J Cutan Pathol. 2020 Jul 23. [QxMD MEDLINE Link].
Hillen LM, Geybels MS, Spassova I, Becker JC, Gambichler T, Garmyn M, et al. A digital mRNA expression signature to classify challenging Spitzoid melanocytic neoplasms. FEBS Open Bio. 2020 May 19. [QxMD MEDLINE Link].
Goto K, Pissaloux D, Durand L, Tirode F, Guillot B, de la Fouchardière A. Novel three-way complex rearrangement of TRPM1-PUM1-LCK in a case of agminated Spitz nevi arising in a giant congenital hyperpigmented macule. Pigment Cell Melanoma Res. 2020 Sep. 33 (5):767-772. [QxMD MEDLINE Link].
Sainz-Gaspar L, Sánchez-Bernal J, Noguera-Morel L, Hernández-Martín A, Colmenero I, Torrelo A. Spitz Nevus and Other Spitzoid Tumors in Children. Part 2: Cytogenetic and Molecular Features. Prognosis and Treatment. Actas Dermosifiliogr. 2020 Jan 2. 111 (1):20-25. [QxMD MEDLINE Link].
Houlier A, Pissaloux D, Masse I, Tirode F, Karanian M, Pincus LB, et al. Melanocytic tumors with MAP3K8 fusions: report of 33 cases with morphological-genetic correlations. Mod Pathol. 2020 May. 33 (5):846-857. [QxMD MEDLINE Link].
Donati M, Kastnerova L, Martinek P, Grossmann P, Sticová E, Hadravský L, et al. Spitz Tumors With ROS1 Fusions: A Clinicopathological Study of 6 Cases, Including FISH for Chromosomal Copy Number Alterations and Mutation Analysis Using Next-Generation Sequencing. Am J Dermatopathol. 2020 Feb. 42 (2):92-102. [QxMD MEDLINE Link].
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. [QxMD MEDLINE Link].
Sepehr A, Chao E, Trefrey B, et al. Long-term Outcome of Spitz-Type Melanocytic Tumors. Arch Dermatol. 2011 Oct. 147(10):1173-9. [QxMD MEDLINE Link].
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. [QxMD MEDLINE Link]. [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. [QxMD MEDLINE Link].
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. [QxMD MEDLINE Link].
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. [QxMD MEDLINE Link].
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. [QxMD MEDLINE Link].
Fullen DR, Poynter JN, Lowe L, et al. BRAF and NRAS mutations in spitzoid melanocytic lesions. Mod Pathol. 2006 Oct. 19(10):1324-32. [QxMD MEDLINE Link].
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. [QxMD MEDLINE Link].
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. [QxMD MEDLINE Link].
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. [QxMD MEDLINE Link].
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. [QxMD MEDLINE Link].
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. [QxMD MEDLINE Link].
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. [QxMD MEDLINE Link].
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. [QxMD MEDLINE Link].
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. [QxMD MEDLINE Link].
Cho-Vega JH. A diagnostic algorithm for atypical spitzoid tumors: guidelines for immunohistochemical and molecular assessment. Mod Pathol. 2016 Jul. 29 (7):656-70. [QxMD MEDLINE Link].
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. [QxMD MEDLINE Link].
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. [QxMD MEDLINE Link].
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. [QxMD MEDLINE Link].
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. [QxMD MEDLINE Link].
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. [QxMD MEDLINE Link].
Murphy ME, Boyer JD, Stashower ME, Zitelli JA. The surgical management of Spitz nevi. Dermatol Surg. 2002 Nov. 28(11):1065-9; discussion 1069. [QxMD MEDLINE Link].
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. [QxMD MEDLINE Link].
Rapini RP. Spitz nevus or melanoma?. Semin Cutan Med Surg. 1999 Mar. 18(1):56-63. [QxMD MEDLINE Link].

Media Gallery

Spitz nevus on the ear of a child.
Microphotograph (low power).
Microphotograph (medium power).
Microphotograph (low power).
Microphotograph (medium power).

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Author

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.

Coauthor(s)

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.

Specialty Editor Board

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.

Chief 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.

Additional Contributors

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

Background

Pathophysiology

Etiology

Epidemiology

Frequency

Race

Sex

Age

Prognosis

Patient Education

References

Tables

Contributor Information and Disclosures

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