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Year : 2015  |  Volume : 6  |  Issue : 7  |  Page : 53-55  

Dermatopathology quiz: A dome-shaped papule on the cheek

1 Ackerman Academy of Dermatopathology, New York, USA
2 Aurora Diagnostics Twin Cities Dermatopathology, Plymouth, MN, USA
3 Department of Pathology, Memorial Sloan Kettering Medical Center, New York, USA
4 Department of Dermatology and Pathology, Medical University of South Carolina, Charleston, USA

Date of Web Publication4-Dec-2015

Correspondence Address:
Dirk Elston
Ackerman Academy of Dermatopathology, 145 East 32nd Street, 10th Floor, New York, NY 10016
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/2229-5178.171039

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How to cite this article:
Elbendary A, Jacobson E, Busam K, Elston D. Dermatopathology quiz: A dome-shaped papule on the cheek. Indian Dermatol Online J 2015;6, Suppl S1:53-5

How to cite this URL:
Elbendary A, Jacobson E, Busam K, Elston D. Dermatopathology quiz: A dome-shaped papule on the cheek. Indian Dermatol Online J [serial online] 2015 [cited 2021 May 11];6, Suppl S1:53-5. Available from: https://www.idoj.in/text.asp?2015/6/7/53/171039

The 43-year-old woman was evaluated for a 0.4 cm erythematous, dome shaped papule on her left cheek. Clinically, an irritated or atypical nevus was suspected, and a shave biopsy of the lesion was performed.

Histopathological sections revealed a compound, biphasic melanocytic neoplasm composed of both banal appearing nevus cells [Figure 1], and a second population of epithelioid melanocytes forming large dermal aggregates [Figure 2] and [Figure 3]. While the conventional component of the neoplasm demonstrated evidence of maturation and dispersion with descent into the dermis, the epithelioid component was monomorphic throughout and showed no evidence of maturation. An Elastic Van Gieson (EVG) stain showed retained elastic fibers within the epithelioid nests. A BRCA1 associated protein-1 (BAP1) immunohistochemical stain mirrored the dichotomous morphology, with nuclear staining in the conventional-appearing nevus cells and the absence of nuclear staining in the epithelioid cells [Figure 4]. Strong p16 staining was noted in the epithelioid component [Figure 5].
Figure 1: A large nodule of epithelioid melanocytes is embedded among small banal nevus cells

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Figure 2: The epithelioid nodule is sharply circumscribed and the transition between the two populations is abrupt

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Figure 3: The nuclei of the epithelioid cells are hyperchromatic and pleomorphic. They are unlike the vesicular nuclei with prominent central nucleoli noted in Spitz nevi

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Figure 4: Nuclear BRCA1 Associated Protein-1 is expressed normally in the banal nevus population, but lost in the epithelioid population

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Figure 5: The epithelioid cells strongly express p16

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The most likely diagnosis is:

  1. Malignant melanoma arising in a congenital nevus
  2. Epithelioid Spitz nevus
  3. Wiesner nevus
  4. Metastatic, familial uveal melanoma involving a nevus
  5. Balloon-cell change in a congenital nevus.

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   References Top

Busam KJ, Wanna M, Wiesner T. Multiple epithelioid Spitz nevi or tumors with loss of BAP1 expression: A clue to a hereditary tumor syndrome. JAMA Dermatol 2013;149:335-9.  Back to cited text no. 1
Murali R, Wiesner T, Scolyer RA. Tumours associated with BAP1 mutations. Pathology 2013;45:116-26.  Back to cited text no. 2
Wiesner T, Obenauf AC, Murali R, Fried I, Griewank KG, Ulz P, et al. Germline mutations in BAP1 predispose to melanocytic tumors. Nat Genet 2011;43:1018-21.  Back to cited text no. 3
Wiesner T, Murali R, Fried I, Cerroni L, Busam K, Kutzner H, et al. A distinct subset of atypical Spitz tumors is characterized by BRAF mutation and loss of BAP1 expression. Am J Surg Pathol 2012;36:818-30.  Back to cited text no. 4
Pouryazdanparast P, Haghighat Z, Beilfuss BA, Guitart J, Gerami P. Melanocytic nevi with an atypical epithelioid cell component: Clinical, histopathologic, and fluorescence in situ hybridization findings. Am J Surg Pathol 2011;35:1405-12.  Back to cited text no. 5
Gammon B, Traczyk TN, Gerami P. Clumped perinuclear BAP1 expression is a frequent finding in sporadic epithelioid Spitz tumors. J Cutan Pathol 2013;40:538-42.  Back to cited text no. 6
Barnhill RL. The spitzoid lesion: The importance of atypical variants and risk assessment. Am J Dermatopathol 2006;28:75-83.  Back to cited text no. 7
Kamino H, Tam S, Roses D, Toussaint S. Elastic fiber pattern in regressing melanoma: A histochemical and immunohistochemical study. J Cutan Pathol 2010;37:723-9.  Back to cited text no. 8
Vollmer RT. Use of Bayes rule and MIB-1 proliferation index to discriminate Spitz nevus from malignant melanoma. Am J Clin Pathol 2004;122:499-505.  Back to cited text no. 9


  [Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5]


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