Extramammary Paget’s Disease and Melanoma: 2 Cases of Double Cancers

Abstract

Extramammary Paget's disease (EMPD) is a rare intraepidermal neoplastic disease. There is a well-known relationship between EMPD and underlying malignancy. However, only a few cases of EMPD and cutaneous melanoma have been reported previously. In this case report we present 2 cases of such double cancers: one as a collision tumor, the other at separate sites. We discuss the pathogenesis, treatment, and importance of a thorough clinical and radiological examination and review the literature.

Depigmentation of the vulvar area: Is it an alarming sign?

“…However, so far, there is no precise explanation of the exact hereditary mechanisms concerning this association. 9 The family history of malignant melanoma and the early presentation of EMPD in our patient may highlight the possibility of a common genetic association between the two neoplasms that needs further studies for confirmation.…”

A Rare Collison of Vulvar Melanoma and Extramammary Paget Disease: Case Report and Review of the Literature

“…Other than MM, no one had a history of another malignancy. 6,11,12 The diagnostic challenges of MM and EMPD collision tumors are similar clinical presentation to include pruritis, pain, bleeding, discoloration, and overlapping histopathologic features to include pagetoid spread and concentration near the dermal-epidermal interface. 13,14 It was notable in the literature review of MM and EMPD collision tumors that many cases clinically presented with heterogeneously discolored lesions, with darkly pigmented areas corresponding to MM, and erythematous or white areas to EMPD.…”

“…1 Because the most common collision tumor is composed of the most common skin tumors, a melanocytic nevus, and BCC, the leading theory is neoplastic heterogenicity produced by sheer coincidence. 3 A search on PubMed for "(melanoma) AND (extramammary Paget disease)" within the English literature found 5 instances of MM and EMPD occurring within the same lesion ("collision tumors") 6-10 and 3 instances of them occurring at different anatomic regions ("double tumors") 6,11,12 (Table 1). Our report would be the sixth example of a collision tumor of MM and EMPD.…”

“…A search on PubMed for “(melanoma) AND (extramammary Paget disease)” within the English literature found 5 instances of MM and EMPD occurring within the same lesion (“collision tumors”) 6–10 and 3 instances of them occurring at different anatomic regions (“double tumors”) 6,11,12 (Table 1). Our report would be the sixth example of a collision tumor of MM and EMPD.…”