Melanotic hamartoma (“retinal anlage tumour”) of the epididymis

Melanotic Neuroectodermal Tumor of Infancy in the Epididymis

“…MNTI in regions other than the head and neck rarely occurs. Since the first description of MNTI of the epididy mis by Eaton and Ferguson [14], only 23 such cases have been reported [6][7][8][9][10][11][12][14][15][16][17][18][19][20][21][22][23][24][25]. The clinical backgrounds of the patients are summarized in table 1.…”

“…Of the 16 patients with epididymal MNTI in whom the surgical procedure was clearly described [6, 8-12, 14-16, 18, 20, 22-24], 1 patient underwent tumor excision [15], while the remaining 15 patients underwent orchiectomy. Three of them proved to be malignant; 2 patients devel oped métastasés [8,11] and 1 showed invasion of lym phatics by tumor cells [12].…”

“…MNTI has also been referred to as congenital melanocarcinoma [13], retinoblastic teratoma [14], melanotic hamartoma [15,18,23] and retinal anlage tumor [16]. In 1971, the World Health Organization designated this neo plasm 'melanotic neuroectodermal tumor of infancy' [26], While the genesis of MNTI is uncertain, several investiga tors found evidence of a neuroectodermal (neural crest) origin by biochemical, histochemical, ultrastructural and immunohistochemical analyses [1][2][3][4][5][6][7][8][9][10][11][12].…”

“…The clinical backgrounds of the patients are summarized in table 1. Sixteen patients were diagnosed below 1 year of age [8-10, 12, 14, 16, 17, 19-22, 24], 3 were between the ages of 1 and 2 years [11,15,18], 1 was 3 years old [23], and 1 was 8 years old [6].…”

“…A neural crest origin has been suggested by the published histochemical, ultrastruc tural and immunohistochemical studies [1][2][3][4][5][6][7][8][9][10][11][12], Since its first description by Krompechcr [13] in 1918, about 200 cases of MNTI have been published. This tumor usually affects the lower parts of the face and skull, particularly the maxilla, although it has also originated in other sites, including the epididymis [6][7][8][9][10][11][12][14][15][16][17][18][19][20][21][22][23][24][25], thigh [4], femur [3], and foot [5]. The 4-month-old boy described here represents the 23rd reported case of MNTI involving the epididymis.…”