A Rare Case of Vulvar Superficial Angiomyxoma in a Pediatric Patient

Pediatric Vulvar Superficial Angiomyxoma: A Case Report With Clinical, Radiological, and Anatomopathological Characterization and a Comprehensive Review of the Literature

“…Superficial angiomyxoma is an extremely infrequent tumor in the pediatric population, with only 22 cases reported to date in the English scientific literature (including ours). 4,5,[9][10][11][12][13][14][15][16][17][18][19][20][21] A comprehensive review of the published cases and their clinical, radiological, and pathological features is shown in Table 1.…”

“…A total of 5 cases of pediatric vulvar superficial angiomyxoma have been reported in the literature. 5,9,10,11,17 Interestingly, the genital region is, along with the head and neck area, the most reported location for pediatric superficial angiomyxoma in the literature, with a total of 6 cases, 5 of them in females (all of them on the vulva/labia majora) and 1 in a male, appearing on the scrotum. 19 However, it should be considered that Neumann et al 4 recently reported 3 more pediatric cases of superficial angiomyxoma in which the anatomical location was not specified.…”

“…The same is true for desmin. 17,19,21 On the other hand, only one study reports immunohistochemistry for the estrogen and progesterone receptors, 10 which was negative. In our case, the characterization is consistent with these findings (with negative desmin and with focal positivity for actin), bringing as a novelty finding negativity for myogenin and Myo-D1.…”

“…6,7,8 Regarding the pediatric population, only 5 other cases of vulvar superficial angiomyxoma have been reported. 5,[9][10][11]…”

A tale of two vulvar angiomyxomas: Two cases and review of literature

“…( Goyal et al, 2022 ) Two phenotypes of angiomyxomas have been identified: Superficial (or Cutaneous) and Aggressive (or Deep). Superficial angiomyxoma is a noninvasive cutaneous tumor, first described in association with the Carney complex, a rare multiple neoplasia syndrome; although, it more often arises as a sporadic tumor ( Mehrotra et al, 2021 , O’Flynn O’Brien et al, 2020 ) Conversely, Aggressive angiomyxoma is a locally invasive soft tissue neoplasm. ( Steeper and Rosai, 1983 ).…”