A Case of Syringocystadenocarcinoma Papilliferum In situ Occurring Partially in Syringocystadenoma Papilliferum

Abstract

We report a case of syringocystadenocarcinoma papilliferum in situ associated with syringocystadenoma papilliferum. The patient was a 64-year-old man with a red tumor that arose on top of his head two years before he consulted our department. The histological findings revealed a papillomatous growth on the epidermis forming several invaginations. Numerous papillary projections, lined by a two-layered epithelium with a benign appearance and decapitations on the luminal surface of the cells, extended into the lumens of the invaginations. Some projections showed a disorderly arrangement of multilayered cells with atypical nuclei. No differences between findings in the syringocystadenocarcinoma papilliferum in situ and those in the syringocystadenoma papilliferum were observed histochemically (PAS) or immunohistochemically (cytokeratin, CEA, CA 19-9, S-100, gross cystic disease fluid protein, lysozyme and Leu M1).

Syringocystadenocarcinoma Papilliferum In Situ: Case Report with Immunohistochemical Observations

“…Thus, their findings are in discrepancy to ours. In cases of SPCIS, Arai et al [12] found positive staining for CK AE1, variable for CEA, and negative for S-100 protein whereas Ishida-Yamamoto et al case [11] was positive for EMA, partly positive for S-100 protein and negative for CEA in accordance with our findings. However, classification of the latter tumor as an SPCIS must be questioned since the authors state that "double-layered cuboidal and columnar epithelia typical of syringocystadenoma papilliferum were not seen in this tumor."…”

“…However, it also revealed an intracystic proliferation of atypical cells without infiltration of the dermis in conformity with lesions previously reported as "syringocystadenocarcinoma papilliferum in situ" (SPCIS) [5,12,13]. There is probably a continuum from a benign SP via various grades of atypia to an infiltrating syringocystadenocarcinoma papilliferum (SPCA), but criteria for grading of these rare tumors have to our knowledge not been determined.…”

“…Benign SPs have been reported to be positive for CEA [11,12,15,16], CK AE1 [12], and EMA [11], but variable for S-100 protein [11,12]. Ansai et al [8] employed a large panel of antibodies in a case of SPCA comparing the staining properties of the upper noninfiltrating and the deeper infiltrating parts of the tumor.…”

“…About 40% are associated with nevus sebaceous in the same area, some of them developing a basal cell carcinoma as well [1]. There are only few reports of transformation of an SP into an infiltrating syringocystadenocarcinoma papilliferum (SPCA) [2][3][4][5][6][7][8][9][10] and even fewer cases showing an SPCA in situ (SPCIS) [4,5,[11][12][13]. The latter tumors are characterized by a disorganized proliferation of atypical cells in the papillary structures of an SP but without tumor infiltration of the surrounding dermis.…”

Giant Naevoid Syringocystadenoma Papilliferum

“…To date only six cases of SCAP transforming to SCACP have been described (7). Published cases of malignant transformation of SCAP have described intervals of between 20 and 50 years from the time the lesions were first noted (7).…”