Syringomatous adenoma of the nipple: A case series and systematic review

Abstract

Syringomatous adenoma of the nipple (SAN) is known to be a rare benign breast neoplasm. With a few cases documented in the literature, preoperatively diagnosing this tumor is a challenge, which often leads to invasive procedure such as mass excision with nipple removal. This study was aimed at presenting a case report of SAN and to conduct a review of published cases. Literature search was conducted through PubMed databases. Articles published from year 1983 to March of 2022 were included. Only histologically confirmed cases of SAN were included. The review was performed according to the PRISMA guidelines. Twenty-eight cases, How to cite this article: Park SK, Samat SH, Whitelock CM, Fortes T. Syringomatous adenoma of the nipple: A case series and systematic review.

Nipple Adenoma: Systematic Review of Literature

“…Although a recognized association exists, the definitive link between NA and subsequent breast cancer development or a direct causal relationship for NA transforming into breast cancer has not been established but cannot be ruled out. 1,2,6 In our study, 10.34% of cases presented with a malignant or premalignant co-diagnosis, a statistically significant occurrence. All cases of malignant disease during follow-up were identified in the co-diagnosis group.…”

“…Previous studies reported diverse clinical presentations, including skin growth, rash, nipple discharge, erosive lesion, or other symptoms. 2,6 Our findings highlight a nodule or mass as the predominant clinical symptom, occurring more frequently in the co-diagnosis group. Symptoms like erythema, pain, and nipple deformity were more prevalent in the CONA, whereas ulceration and discharge were more common in the ONA group.…”

“…Excision remains the predominant treatment, 1,2,6 with more than half of the patients undergoing local excision. Notably, local excision demonstrated a local recurrence rate of 2.01%.…”

“…9 This rarity might be attributed to the historical challenge in pathologically distinguishing NA from other nipple neoplasms. 6 Advancements in immunohistochemistry have addressed this challenge, but no current estimation of its incidence was found. 10 Reports on NA are scarce, with most being described in case reports, 2 as supported by the results of our systematic review.…”

“…NAs are rare and benign breast tumors. On average, a breast pathology unit encounters only one case per year, 2,6,7 a yearly incidence rate of 0.0025%, 8 or a diagnosis rate of 0.45% among all benign cases. 9 This rarity might be attributed to the historical challenge in pathologically distinguishing NA from other nipple neoplasms.…”