Malignant Eccrine Spiradenoma of the Face

Abstract

Malignant eccrine spiradenoma, or spiradenocarcinoma, is an exceedingly rare sweat-gland tumor, with only 102 reported cases. Low-grade carcinomas are especially rare with only a few cases reported. Because of the limited number of case reports, the biologic behavior of low-grade malignant eccrine spiradenoma is poorly understood and no evidence-based therapeutic approach is established. Here, the authors report a 29-year-old woman who presented with a history of left-sided facial lesions present since the age of 2 months. Histopathologic examination revealed multiple benign spiradenomas, several of which showed foci of low-grade malignant transformation evidenced by loss of the characteristic 2-cell population seen in the benign tumor component. Included are the clinical presentation, histopathologic description, and surgical decision making in an effort to guide recognition of this rare entity.

Spiradenocarcinoma: A Comprehensive Data Review

“…Reports of tumors consisting of a combination of different skin adnexal carcinomas (21), duplicate reports (11), and studies in which data concerning SCs could not be distinguished from other skin adnexal tumors (7) were excluded. One hundred twenty patients were presented in 71 case reports 3,[6][7][8][9][10][11][12] and 3 case series. 5,14,81 Each case report was analyzed for patient'sage, sex, and ethnicity.…”

“…However, in the recent literature a more favorable clinical course in cases with low-grade histomorphological features has been reported. [4][5][6] The eccrine spiradenoma is considered the primary skin lesion from which an SC evolves, but de novo appearances have been reported as well. [7][8][9] Accelerated growth of a long-standing lesion, pain, and ulceration are the most common symptoms that lead to referral.…”

Challenges in diagnosis and management of a spiradenocarcinoma: a comprehensive literature review

“…Computed tomography was the preferred modality, used in 49.1% of these cases, followed by magnetic resonance imaging (MRI) (32.7%), X-ray (30.9%), bone scan (23.6%), ultrasound (20%), positron emission tomography (PET)/computed tomography (CT) (14.5%) and finally mammography (10.9%). [4][5][6][8][9][10][11][54][55][56][57][58][60][61][62][63][67][68][69][73][74][75][77][78][79] Staiger et al have recently recommended that patients presenting with confirmed malignant spiradenoma be evaluated with an MRI and fluorodeoxyglucose (FDG)-PET/CT. 74 Details regarding a fineneedle aspirate or punch biopsy was provided for 76 case reports and case series, representing 86 (61.9%) cases.…”

“…This pre-operative test was performed 40.7% of the time, with the majority of surgeons electing to proceed firstly with excisional biopsy or wide local excision. [4][5][6][7][8][9][10][11][12][18][19][20][21][22][24][25][26][27][28][29][54][55][56][57][58][60][61][62][63][64][65][66][67][68][69][70][71][72][73][74][75][77][78][79][80][81][82][83][84][85][86][8...…”

“…Five cases underwent excision of the primary lesion with sentinel lymph node biopsy (3.7%). 1,[54][55][56][57][58][59][60][61][62] After developing one or more recurrences, four patients had amputations. 26,51,84 Avraham's retrospective analysis reported 39 (90.7%) of 43 cases that had surgical resection of spiradenocarcinoma, however, details regarding any lymph node dissection or biopsy were not provided.…”