and 6 other(s)
Introduction. Syringoid eccrine carcinoma (SEC) is an extremely rare malignant adnexal tumor with eccrine differentiation first described by Freeman and Wilkemann in 1969 as eccrine epithelioma. Its name originates from histological pattern which resembles that one of a syringoma by showing ductal, cystic and comma-like epithelial components in a fibrocollagenous matrix. Fewer than 50 cases have been reported under the name of SEC and its synonysms. Due to limited availability of literature, there is a lack of a universally accepted staging system and the diagnosis and management of these tumors are difficult. Objectives. The aim of this study is to report the extremely rare case of a syringoid eccrine carcinoma of the vulva occurred in a 33 years old woman who referred to our Gynecology Unit on January 2017, and to review other recent cases described in literature speculating on differential diagnosis, prognostic criteria, management and follow up. Methods. Accepted criteria on haematoxylin and eosin sections were used to make the diagnosis. Two pathologists evaluated formalin-fixed tissue sections independently, reaching the same conclusions. Fourteen manuscripts about various locations' syringoid eccrine carcinoma were rewieved. Results. The patient underwent a right anterior hemivulvectomy with minor labrum preservation, without complications and next day discharge. Resection margins were tumor-free, obtaining oncologic radicality (FIGO stage: IB, Nx). Since SEC has commonly a local recurrence disposition and a potential for distant metastasis, the patient underwent a clinical gynecological evaluation and ultrasonographic bilateral inguinal lymph nodes examination in June 2017, which were found to be negative. The following control in 3 months was negative, too. Discussion. Despite the low number of cases described in literature, it is unanimously acknowledged that the recommended treatment of all subtypes of sweat gland carcinomas is wide surgical excision associated with regional lymph node dissection in case of clinically positive nodes because of SEC potential for destructive local tissue infiltration and regional as well as distant metastasis. On one hand local reccurrences of these tumors may be massive; on the other, metastases rarely occur, yet they are not impossible. In literature, there are reports indicating both single-agent and combination chemotherapy have been used infrequently and, at best, have shown only a temporary benefit. Sweat gland carcinomas are commonly considered radio resistant. Prognostic factors are difficult to identify because of the small number of reported cases. Size, histological type, lymph node involvement above all and distant metastases are those considered more relevant. Several features are associated with a poor prognosis: tumor depth > 7 mm, more than 14 mitotic figures on high powered magnification, infiltrative margins; an acute change to a rapid growing lesion could be suggestive for a transition to malignancy. Survival rates are substantially unknown due to the lack of data. For what concerns apocrine adenocarcinomas, patients without lymph nodes involvement have a 56% 10-year disease-free survival rate, which decrease to 9% if nodes are involved. Conclusion. SEC should be taken into consideration in vulvar cancer differential diagnosis. A frequent follow up is essential to detect early recurrence or distant metastases.
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