eMedicine Specialties > Dermatology > Benign Neoplasms

Spitz Nevus: Differential Diagnoses & Workup

Author: Zoltan Trizna, MD, PhD, Private Practice
Coauthor(s): Ronald P Rapini, MD, Professor and Chair, Department of Dermatology, Professor of Pathology, University of Texas Medical School, MD Anderson Cancer Center
Contributor Information and Disclosures

Updated: Feb 20, 2009

Differential Diagnoses

Basal Cell Carcinoma
Juvenile Xanthogranuloma (Nevoxanthoendothelioma)
Nevi, Melanocytic
Pyogenic Granuloma (Lobular Capillary Hemangioma)
Warts, Nongenital

Other Problems to Be Considered

Melanoma
Epidermal nevus
Granulomas
Histiocytoma
Reed nevus
Vascular tumors

Workup

Imaging Studies

  • Dermoscopy: A starburst pattern is observed (ie, pigmented streaks symmetrically distributed at the periphery of the lesion).1

Other Tests

  • Histopathologic evaluation of a suspected Spitz nevus is indicated.
  • The value of including individual proliferation index and histopathological parameters (eg, Ki-67, Pi-21, fatty acid synthetase) into models of predictive probabilities is uncertain, but a panel of markers including Ki-67, HMB-45, and S100A6 can be helpful in establishing a histologic diagnosis. Ki-67 staining is usually absent in dermal nuclei of benign Spitz nevi. HMB-45 staining demonstrates a gradient, and S100A6 diffusely stains the lesion.
  • Analysis of mutations of BRAF, NRAS, and HRAS were promising in distinguishing Spitz nevi from melanomas, but BRAF mutations do not separate all Spitz nevi from spitzoid melanomas and other melanocytic proliferations.2,3,4

Procedures

  • Surgical excision (generally with local anesthesia) and histopathologic evaluation of the margins of the specimen is recommended.
  • Sentinel node biopsy: In a series of 12 cases with atypical Spitz nevi showed nodal micrometastases in one third of the patients, suggesting a yet not understood metastatic potential.

Histologic Findings

Most Spitz nevi are predominantly compound, although junctional and intradermal lesions are also observed. The sine qua non of the diagnosis is the presence of large and/or spindle-shaped melanocytes, usually in nests. The nests are composed of an admixture of spindle cells and/or epithelioid cells, although frequently, the spindle-shaped cells predominate (see Media Files 2-5).

The spindle cells are usually observed in a fascicular arrangement, and typically have a vertical orientation. These cells have abundant cytoplasm and contain a vesicular nucleus with a conspicuous nucleolus. The epithelioid cells have prominent nucleoli. Tumors composed only of epithelioid cells, those that lack dispersion at the base, those that are grossly asymmetrical, those with deep mitoses, and those in older individuals are more likely to represent malignant melanoma.5

Striking symmetry, sharp lateral demarcation, absent (or rare) mitoses, absence of atypical mitoses, presence of eosinophilic and periodic acid-Schiff (PAS)–positive globules (Kamino bodies) and nondisruptive (single-file like) infiltration of collagen are important features indicating the diagnosis of Spitz nevi. Single-file melanocytes may also be observed in the reticular dermis located at the base of the lesion (dispersion).

Another important feature is the maturation of cellular elements toward the dermis. Pagetoid spread of the melanocytes is usually confined to the center of the lesion; when present, it can cause confusion with melanoma.

The epidermis is hyperkeratotic and acanthotic. A cleavage artifact of fixation is commonly noticed above the nests and around superficial dermal elements.

The histologic distinction between Spitz nevi and melanomas is equivocal in up to 8% of cases. Immunohistochemistry and comparative genomic hybridization have proved helpful.  Spitz nevi stain diffusely with S100A6, demonstrate no deep Ki-67 positive cells and show a gradient with HMB-45 staining.  Melanomas show the opposite patterns.6

More on Spitz Nevus

Overview: Spitz Nevus
Differential Diagnoses & Workup: Spitz Nevus
Treatment & Medication: Spitz Nevus
Follow-up: Spitz Nevus
Multimedia: Spitz Nevus
References
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References

  1. Rossiello L, Zalaudek I, Ferrara G, Docimo G, Giorgio CM, Argenziano G. Melanoacanthoma simulating pigmented spitz nevus: an unusual dermoscopy pitfall. Dermatol Surg. May 2006;32(5):735-7. [Medline].

  2. Fullen DR, Poynter JN, Lowe L, et al. BRAF and NRAS mutations in spitzoid melanocytic lesions. Mod Pathol. Oct 2006;19(10):1324-32. [Medline].

  3. Gill M, Cohen J, Renwick N, Mones JM, Silvers DN, Celebi JT. Genetic similarities between Spitz nevus and Spitzoid melanoma in children. Cancer. Dec 1 2004;101(11):2636-40. [Medline].

  4. 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. Sep 2005;29(9):1145-51. [Medline].

  5. Da Forno PD, Fletcher A, Pringle JH, Saldanha GS. Understanding spitzoid tumours: new insights from molecular pathology. Br J Dermatol. Jan 2008;158(1):4-14. [Medline].

  6. 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. May 2008;58(5):880-4. [Medline].

  7. Gelbard SN, Tripp JM, Marghoob AA, et al. Management of Spitz nevi: a survey of dermatologists in the United States. J Am Acad Dermatol. Aug 2002;47(2):224-30. [Medline].

  8. Murphy ME, Boyer JD, Stashower ME, Zitelli JA. The surgical management of Spitz nevi. Dermatol Surg. Nov 2002;28(11):1065-9; discussion 1069. [Medline].

  9. Boer A, Wolter M, Kneisel L, Kaufmann R. Multiple agminated Spitz nevi arising on a cafe au lait macule: review of the literature with contribution of another case. Pediatr Dermatol. Nov-Dec 2001;18(6):494-7. [Medline].

  10. Dal Pozzo V, Benelli C, Restano L, Gianotti R, Cesana BM. Clinical review of 247 case records of Spitz nevus (epithelioid cell and/or spindle cell nevus). Dermatology. 1997;194(1):20-5. [Medline].

  11. Ferrara G, Argenziano G, Soyer HP, et al. The spectrum of Spitz nevi: a clinicopathologic study of 83 cases. Arch Dermatol. Nov 2005;141(11):1381-7. [Medline].

  12. Harvell JD, Bastian BC, LeBoit PE. Persistent (recurrent) Spitz nevi: a histopathologic, immunohistochemical, and molecular pathologic study of 22 cases. Am J Surg Pathol. May 2002;26(5):654-61. [Medline].

  13. Kapur P, Selim MA, Roy LC, Yegappan M, Weinberg AG, Hoang MP. Spitz nevi and atypical Spitz nevi/tumors: a histologic and immunohistochemical analysis. Mod Pathol. Feb 2005;18(2):197-204. [Medline].

  14. Orchard DC, Dowling JP, Kelly JW. Spitz naevi misdiagnosed histologically as melanoma: prevalence and clinical profile. Australas J Dermatol. Feb 1997;38(1):12-4. [Medline].

  15. Rapini RP. Spitz nevus or melanoma?. Semin Cutan Med Surg. Mar 1999;18(1):56-63. [Medline].

  16. Shimek CM, Golitz LE. The golden anniversary of the Spitz nevus. Arch Dermatol. Mar 1999;135(3):333-5. [Medline].

  17. Song JY, Kwon JA, Park CJ. A case of Spitz nevus with multiple satellite lesions. J Am Acad Dermatol. Feb 2005;52(2 Suppl 1):48-50. [Medline].

  18. Spatz A, Calonje E, Handfield-Jones S, Barnhill RL. Spitz tumors in children: a grading system for risk stratification. Arch Dermatol. Mar 1999;135(3):282-5. [Medline].

  19. Urso C, Borgognoni L, Saieva C, et al. Sentinel lymph node biopsy in patients with "atypical Spitz tumors." A report on 12 cases. Hum Pathol. Jul 2006;37(7):816-23. [Medline].

  20. Vollmer RT. Patient age in Spitz nevus and malignant melanoma: implication of Bayes rule for differential diagnosis. Am J Clin Pathol. Jun 2004;121(6):872-7. [Medline].

  21. Vollmer RT. Use of Bayes rule and MIB-1 proliferation index to discriminate Spitz nevus from malignant melanoma. Am J Clin Pathol. Oct 2004;122(4):499-505. [Medline].

  22. Weedon D, Little JH. Spindle and epithelioid cell nevi in children and adults. A review of 211 cases of the Spitz nevus. Cancer. Jul 1977;40(1):217-25. [Medline].

  23. Zaenglein AL, Heintz P, Kamino H, Zisblatt M, Orlow SJ. Congenital Spitz nevus clinically mimicking melanoma. J Am Acad Dermatol. Sep 2002;47(3):441-4. [Medline].

  24. Zatterstrom U, Thor A, Nordgren H. Cervical metastasis from Spitz nevus of the buccal mucosa. Melanoma Res. Feb 2008;18(1):36-9. [Medline].

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Further Reading

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Keywords

Spitz nevus, Spitz nevi, Spitz's nevus, Spitz's nevi, spindle and epithelioid cell nevus, juvenile melanoma, benign juvenile melanoma, nevus of large spindle and/or epithelioid cells, prepubertal melanoma

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Contributor Information and Disclosures

Author

Zoltan Trizna, MD, PhD, Private Practice
Zoltan Trizna, MD, PhD is a member of the following medical societies: American Academy of Dermatology, American Medical Association, and Texas Medical Association
Disclosure: Nothing to disclose.

Coauthor(s)

Ronald P Rapini, MD, Professor and Chair, Department of Dermatology, Professor of Pathology, University of Texas Medical School, MD Anderson Cancer Center
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, Society for Investigative Dermatology, Southern Medical Association, and Texas Medical Association
Disclosure: Nothing to disclose.

Medical Editor

Maureen B Poh-Fitzpatrick, MD, Professor Emerita of Dermatology and Special Lecturer, Columbia University; Professor of Medicine (Dermatology), University of Tennessee
Maureen B Poh-Fitzpatrick, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Dermatology, and New York Academy of Medicine
Disclosure: Nothing to disclose.

Pharmacy Editor

Richard P Vinson, MD, Assistant Clinical Professor, Department of Dermatology, Texas Tech University 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, Association of Military Dermatologists, Texas Dermatological Society, and Texas Medical Association
Disclosure: Nothing to disclose.

Managing Editor

Rosalie Elenitsas, MD, Herman Beerman Associate 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 and American Society of Dermatopathology
Disclosure: Nothing to disclose.

CME Editor

Catherine Quirk, MD, Clinical Assistant Professor, Department of Dermatology, Brown University
Catherine Quirk, MD is a member of the following medical societies: Alpha Omega Alpha and American Academy of Dermatology
Disclosure: Nothing to disclose.

Chief Editor

Dirk M Elston, MD, Director, Department of Dermatology, Geisinger Medical Center
Dirk M Elston, MD is a member of the following medical societies: American Academy of Dermatology
Disclosure: Nothing to disclose.

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