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Malignant Melanoma Arising at the Site of a Previously Excised Giant Congenital Melanocytic Nevus FREE

Carrie C. Coughlin, MD1; M. Laurin Council, MD1; Alejandro A. Gru, MD2; Ryan C. Fields, MD3; Susan J. Bayliss, MD1
[+] Author Affiliations
1Division of Dermatology, Department of Medicine, Washington University School of Medicine, St Louis, Missouri
2Department of Pathology, Washington University School of Medicine, St Louis, Missouri
3Department Surgery, Washington University School of Medicine, St Louis, Missouri
JAMA Dermatol. 2014;150(1):100-101. doi:10.1001/jamadermatol.2013.5775.
Text Size: A A A
Published online

Patients with giant congenital nevi are at increased risk for malignant melanoma. Most reported cases involve development of melanoma during childhood, usually before age 6 years.13 Herein, we report a case of a man with a giant congenital nevus and satellite nevi who underwent multiple excisions and grafting procedures as a child and developed malignant melanoma more than 30 years later.

REPORT OF A CASE

A man in his 40s with a history of a giant truncal congenital nevus and many satellite nevi presented with concern for a tender nodule on his right posterior thigh (Figure 1). Notably, over a 4-year period in childhood, he underwent 10 to 11 serial surgical excisions with accompanying split-thickness graft reconstruction to attempt nevus removal. Histologic analysis showed the presence of nevus cells at the deep margin of the final excision. He had no family history of skin cancer.

Place holder to copy figure label and caption
Figure 1.
Clinical Images

A, Tender nodule (circled) on the upper right posterior thigh in a graft site. B, Upper right posterior thigh after wide local excision (the 0-50 scale represents millimeters).

Graphic Jump Location

At presentation, the nodule had been palpable for 2 months and had not improved with oral antibiotic treatment prescribed by his primary care physician. On physical examination, he had a 2.0 × 1.5-cm subcutaneous, tender nodule with an overlying well-healed skin graft of the upper right posterior thigh. There was no palpable lymphadenopathy. Biopsy showed a dermal-based atypical melanocytic proliferation with epithelioid nests of pleomorphic melanocytes with prominent nucleoli and infiltration into the subcutis. Multiple mitotic figures, focal lymphovascular space invasion, and necrosis were seen (Figure 2). These findings were consistent with metastatic melanoma; no epidermis was present in the specimen. The patient underwent positron emission tomography/computed tomography, and the findings were negative for metastatic disease.

Place holder to copy figure label and caption
Figure 2.
Histopathologic Findings

A. Malignant melanoma with epithelioid cells and nevoid features (left) immediately adjacent to a nevus with neurotization (right) (hematoxylin-eosin, original magnification ×400). B, Lymph node with metastatic melanoma (hematoxylin-eosin, original magnification ×200). C, Lymphovascular space invasion by the tumor cells (hematoxylin-eosin, original magnification ×400).

Graphic Jump Location

The patient underwent wide local excision and sentinel lymph node biopsy. Histologic examination revealed a nevoid melanoma, lymphovascular space invasion, and foci of residual benign congenital melanocytic nevus. Microscopic metastatic disease was present in 2 of 4 right inguinal sentinel lymph nodes. Completion lymph node dissection revealed no additional disease. His tumor was negative for a BRAF V600E mutation. The patient was referred to medical oncology for evaluation of his stage IIIC melanoma and underwent treatment with pegylated interferon.

DISCUSSION

The patient underwent several staged excisions and grafting procedures as a child to address his giant congenital melanocytic nevus (GCMN) and numerous congenital satellite nevi. His melanoma was diagnosed 34 years after the final stage of his GCMN excision. To our knowledge, melanoma in a site of excision and grafting has been reported only 4 times.2,46 It has also been reported after dermabrasion for congenital nevi.7 The risk for malignant melanoma in patients with GCMN has been variably reported but is agreed to be greater than that of patients without GCMN.1

As in this patient, it has been postulated that melanoma arises more deeply in patients with GCMN.1 Our patient’s age at presentation is older than average. In 205 patients in the New York University Large Congenital Melanocytic Nevus registry,3 70% of melanomas (7 of 10) developed in patients 3 years or younger, and 30% (3 of 10) developed in patients 35 years or older. Similarly, in a cohort of 1008 patients with large or multiple congenital melanocytic nevi, only 27% of melanomas (4 of 15) were diagnosed in patients older than 30 years.2 Though most melanomas arising in patients with GCMN have been reported in young patients,1 the risk for malignancy persists with age.

Congenital satellite nevi are known risk factors for neurocutaneous melanosis.1 Their impact on the risk for malignant melanoma is not as well established, but some studies point to a possible increased risk in patients with GCMN and satellite nevi.1,3 In summary, our patient is an example of the need for continued dermatologic follow-up and high index of suspicion for new nodules appearing in patients with a history of GCMN.

ARTICLE INFORMATION

Corresponding author: Carrie C. Coughlin, MD, Washington University School of Medicine, 660 S Euclid Ave, Campus Box 8123, St Louis, MO 63110 (ccoughli@dom.wustl.edu).

Published Online: November 27, 2013. doi:10.1001/jamadermatol.2013.5775.

Conflict of Interest Disclosures: None reported.

Additional Contributions: We are indebted to Lynn A. Cornelius, MD, Division of Dermatology, Department of Medicine, Washington University School of Medicine, St Louis, Missouri, for comments on a draft of this manuscript.

REFERENCES

Alikhan  A, Ibrahimi  OA, Eisen  DB.  Congenital melanocytic nevi, where are we now? part I, clinical presentation, epidemiology, pathogenesis, histology, malignant transformation, and neurocutaneous melanosis. J Am Acad Dermatol. 2012;67(4):e1-e17.
PubMed   |  Link to Article
Bett  BJ.  Large or multiple congenital melanocytic nevi: occurrence of cutaneous melanoma in 1008 persons. J Am Acad Dermatol. 2005;52(5):793-797.
PubMed   |  Link to Article
Hale  EK, Stein  J, Ben-Porat  L,  et al.  Association of melanoma and neurocutaneous melanocytosis with large congenital melanocytic naevi—results from the NYU-LCMN registry. Br J Dermatol. 2005;152(3):512-517.
PubMed   |  Link to Article
Rhodes  AR, Wood  WC, Sober  AJ, Mihm  MC  Jr.  Nonepidermal origin of malignant melanoma associated with a giant congenital nevocellular nevus. Plast Reconstr Surg. 1981;67(6):782-790.
PubMed   |  Link to Article
Streams  BN, Lio  PA, Mihm  MC, Sober  AJ.  A nonepidermal, primary malignant melanoma arising in a giant congenital melanocytic nevus 40 years after partial surgical removal. J Am Acad Dermatol. 2004;50(5):789-792.
PubMed   |  Link to Article
Shaw  MH.  Malignant melanoma arising from a giant hairy naevus. Br J Plast Surg. 1962;15:426-431.
PubMed   |  Link to Article
Dragieva  G, Hafner  J, Künzi  W,  et al.  Malignant melanoma in a large congenital melanocytic nevus 9 years after dermabrasion in childhood. Dermatology. 2006;212(2):208-209.
PubMed   |  Link to Article

Figures

Place holder to copy figure label and caption
Figure 1.
Clinical Images

A, Tender nodule (circled) on the upper right posterior thigh in a graft site. B, Upper right posterior thigh after wide local excision (the 0-50 scale represents millimeters).

Graphic Jump Location
Place holder to copy figure label and caption
Figure 2.
Histopathologic Findings

A. Malignant melanoma with epithelioid cells and nevoid features (left) immediately adjacent to a nevus with neurotization (right) (hematoxylin-eosin, original magnification ×400). B, Lymph node with metastatic melanoma (hematoxylin-eosin, original magnification ×200). C, Lymphovascular space invasion by the tumor cells (hematoxylin-eosin, original magnification ×400).

Graphic Jump Location

Tables

References

Alikhan  A, Ibrahimi  OA, Eisen  DB.  Congenital melanocytic nevi, where are we now? part I, clinical presentation, epidemiology, pathogenesis, histology, malignant transformation, and neurocutaneous melanosis. J Am Acad Dermatol. 2012;67(4):e1-e17.
PubMed   |  Link to Article
Bett  BJ.  Large or multiple congenital melanocytic nevi: occurrence of cutaneous melanoma in 1008 persons. J Am Acad Dermatol. 2005;52(5):793-797.
PubMed   |  Link to Article
Hale  EK, Stein  J, Ben-Porat  L,  et al.  Association of melanoma and neurocutaneous melanocytosis with large congenital melanocytic naevi—results from the NYU-LCMN registry. Br J Dermatol. 2005;152(3):512-517.
PubMed   |  Link to Article
Rhodes  AR, Wood  WC, Sober  AJ, Mihm  MC  Jr.  Nonepidermal origin of malignant melanoma associated with a giant congenital nevocellular nevus. Plast Reconstr Surg. 1981;67(6):782-790.
PubMed   |  Link to Article
Streams  BN, Lio  PA, Mihm  MC, Sober  AJ.  A nonepidermal, primary malignant melanoma arising in a giant congenital melanocytic nevus 40 years after partial surgical removal. J Am Acad Dermatol. 2004;50(5):789-792.
PubMed   |  Link to Article
Shaw  MH.  Malignant melanoma arising from a giant hairy naevus. Br J Plast Surg. 1962;15:426-431.
PubMed   |  Link to Article
Dragieva  G, Hafner  J, Künzi  W,  et al.  Malignant melanoma in a large congenital melanocytic nevus 9 years after dermabrasion in childhood. Dermatology. 2006;212(2):208-209.
PubMed   |  Link to Article

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