Malignant Cutaneous Adnexal Tumors and Role of SLNB.

BACKGROUND: Malignant cutaneous adnexal tumors (MCATs) are rare and their natural history is poorly understood. Available literature indicates aggressive behavior with a significant risk of metastasis. STUDY DESIGN: Retrospective review of our institutional surgical oncology databases was performed for patients diagnosed with MCATs (2001-2020). We hypothesized that most patients have a low risk of lymph node involvement, recurrence, and death. Kaplan-Meier statistical analysis was used to assess risk of recurrence and 5-year survival. RESULTS: We identified 41 patients diagnosed with MCATs (median age 59 years, 68% were men). Most patients had long-standing cutaneous lesions (median 24 months) and no palpable adenopathy. Most patients had stage I or II disease (98%). Primary tumors were treated with wide local excision (n = 28 [68%]), Mohs surgery (n = 5 [12%]), or amputation (n = 8 [19%]). Of 25 patients who underwent SLNB (61%), 1 had lymphatic metastasis. These include apocrine carcinoma (1 of 3), digital papillary adenocarcinoma (0 of 8), porocarcinoma (0 of 4), and additional MCAT sub-types (0 of 10). Three patients (7%) had disease recurrence at a median interval of 3.6 years (interquartile range 1.5 to 4.4 years). Five patients (12%) died at a median interval of 7 years (interquartile range 6.7 to 9.2 years), but only 1 patient was known to have succumbed to MCAT. Overall 5-year survival rate was 96% (95% CI, 75% to 99%). CONCLUSIONS: Despite the historical impression that MCATs have a high metastatic potential, most patients have low recurrence rates and excellent 5-year survival rates. Lymphatic disease identified after SLNB in early-stage tumors is rare and the value of this staging procedure in MCAT remains unclear.

Behavior of Cutaneous Adnexal Malignancies: a Single Institution Experience.

Cutaneous adnexal malignancies are biologically and pathologically diverse, and associated with a range of clinical outcomes. Given their rarity, the prognosis and optimal treatment of these neoplasms remains unclear. A single institution database from a tertiary care cancer center of patients treated for malignant cutaneous adnexal tumors was retrospectively analyzed. Clinicopathologic variables and outcome measures were analyzed in patients undergoing wide excision with or without sentinel node biopsy. 103 patients were analyzed; the majority of tumors were of eccrine sweat gland derivation (n = 69, 70%), and these exhibited a higher rate of nodal involvement and overall worse outcome. Sixteen patients (16%) demonstrated nodal metastasis, which included 10 (10%) with nodal disease at presentation and 6 who developed nodal metastasis during followup. 20 patients underwent sentinel node biopsy, and 2 (10%) had a positive sentinel node. 62% of nodal metastases occurred in patients with porocarcinoma. Seven patients died of disease (7%) with a median time from diagnosis to death of 48 months (range, 10-174). After a median follow up of 44.7 months, age > 70 years and larger tumor size were significantly associated with worse overall survival. Adnexal malignancies are rare tumors, and there is a paucity of information to guide the clinician in determining optimum surgical and medical treatment. Tumors of eccrine derivation, especially porocarcinomas, have a high risk of nodal involvement and may be considered for sentinel node biopsy.

Surgery and Adjuvant Radiation for High-risk Skin Adnexal Carcinoma of the Head and Neck.

OBJECTIVES: Skin adnexal carcinoma (SAC) is a rare cutaneous malignancy that arises from sebaceous and sweat glands. These carcinomas are believed to behave more aggressively than cutaneous squamous cell carcinomas (SCC) with a propensity for local recurrence. The role of adjuvant radiotherapy in SAC is undefined. METHODS: We retrospectively reviewed all cases of head and neck SAC treated with surgery and adjuvant radiation from 2000 to 2012 at a single institution. RESULTS: Nine cases were identified. Median age was 67 (range, 52 to 88) years. The histologies were: adnexal carcinoma (n=1), adnexal carcinoma with sebaceous differentiation (n=1), adnexal carcinoma with squamous differentiation (n=1), skin appendage carcinoma (n=1), sclerosing sweat duct carcinoma (n=1), mucinous carcinoma (n=1), ductal eccrine adenocarcinoma (n=1), porocarcinoma (n=1), and trichilemmal carcinoma (n=1). All tumors were reviewed by a dermatopathologist to confirm the SAC diagnosis.All patients had undergone surgery. Indications for adjuvant radiation included involved lymph nodes (n=4), perineural invasion (n=2), nodal extracapsular extension (n=2), positive margin (n=1), high-grade histology (n=6), multifocal disease (n=2), and/or recurrent disease (n=5). Radiation was delivered to the primary site alone (n=3), to the draining lymphatics alone (n=2), or to both (n=4). One patient received concurrent cisplatin. Median dose to the primary site was 60 Gy and to the neck was 50 Gy.Median follow-up was 4.0 years (range, 0.6 to 11.4 y). Locoregional control was 100%. Five-year progression-free survival was 89%. There was 1 acute grade 3 toxicity and no greater than or equal to grade 2 late toxicities were recorded. CONCLUSIONS: Surgery and adjuvant radiation for high-risk SAC offers excellent locoregional control with acceptable toxicity.

The risk of lymph node involvement in malignant cutaneous adnexal tumors.

Malignant cutaneous adnexal tumors (MCATs) are rare neoplasms that do not have a well-studied treatment algorithm. They are generally treated by excision alone. Given its successful application in other cutaneous malignancies, sentinel lymph node biopsy (SLNB) has been advocated by some for use in MCATs. A retrospective chart review was performed. Clinicopathological factors, recurrence patterns, and long-term follow-up were documented. Survival analysis was performed. Forty-eight subjects were identified. Mean age was 69 years with locations on the face (52%), including periocular sites (16%), extremities (25%), and all other sites (7%). Histologic distribution was sebaceous carcinoma (56%), porocarcinoma (17%), eccrine carcinoma (13%), adenocarcinoma (10%), and hidradenocarcinoma (4%). Mean follow-up was 3.9 years (range, 0 to 17 years). Nine subjects (18.8%) recurred locally, and recurrence was inversely associated with age (P = 0.04). Four (8.3%) demonstrated lymph node involvement, none without first developing local recurrence. Five-year disease-specific survival was 97 per cent (confidence interval, 81.4 to 99.6%). Despite being one of the largest reported analyses of MCATs, this study is limited by the small number of subjects. MCATs seemingly develop nodal recurrence only after demonstrating aggressive local biology. SLNB may best be applied selectively, possibly only in patients with local recurrence.

Survival after resection of cutaneous adnexal carcinomas with eccrine differentiation: risk factors and trends in outcomes.

BACKGROUND: Current staging systems do not specifically address cutaneous adnexal carcinomas with eccrine differentiation. Due to their rarity, prognosis and management strategies are not well established. A population-based study was performed to determine prognostic factors and survival. METHODS: Patients diagnosed with cutaneous adnexal carcinomas with eccrine differentiation were identified using the surveillance, epidemiology, and end results population-based cancer registry. Associations between risk factors, treatment modalities, and survival were calculated using logistical regression, Kaplan-Meier estimates and log-rank analysis. RESULTS: The incidence of distinct eccrine subtypes was determined within 1,045 patients with cutaneous adnexal tumors containing eccrine differentiation. All-cause 5-year survival (OS) was 82%, while age-adjusted survival was 94%. Patients with microcystic adnexal carcinoma had improved OS (90%) compared to patients with hidradenocarcinoma (74%), spiradenocarcinoma (77%), porocarcinoma (79%), and eccrine adenocarcinoma (81%). The majority of patients were treated with surgical excision and a small subset with surgery plus radiation, with similar OS. Patients with well-to-moderately differentiated tumors demonstrated improved OS compared to those with poorly differentiated/anaplastic disease. CONCLUSIONS: Histological subtype and grade were associated with survival, and should be specified in biopsies and excised specimens. Surgical excision is appropriate, and the addition of adjuvant radiation may not be associated with survival. These results highlight survival data and high-risk prognostic factors that warrant prospective validation, and may augment current staging systems.

Rare tumors through the looking glass: an examination of malignant cutaneous adnexal tumors.

OBJECTIVE: To identify prognostic factors related to malignant cutaneous adnexal tumors. DESIGN: Population-based study using the Surveillance, Epidemiology, and End Results database from January 1, 1988, through December 31, 2006. SETTING: Seventeen population-based cancer registries. PARTICIPANTS: A total of 4032 patients with malignant cutaneous adnexal tumors. MAIN OUTCOME MEASURES: Overall survival (OS) and disease-specific survival (DSS). RESULTS: Ten-year OS and DSS rates were 54% and 97%, respectively. Unfavorable factors for OS were increasing age (hazard ratio [HR] 1.08; 95% confidence interval [CI], 1.07-1.09; P < .001), distant metastases (3.26; 2.34-4.53; P < .001), no surgical procedure (1.27; 1.01-1.59; P = .04), grade IV tumors (1.97; 1.18-3.28; P = .009), nodal metastases (2.19; 1.40-3.43; P = .001), and T3 tumors (1.37; 1.00-1.87; P = .04). Favorable factors for OS were a wide surgical excision (HR, 0.78; 95% CI, 0.68-0.89; P < .001), female sex (0.73; 0.65-0.82; P < .001), malignant eccrine spiradenoma (0.72; 0.53-0.99; P = .04), and histologic findings of sweat duct carcinoma (0.63; 0.44-0.90; P = .01). Unfavorable factors for DSS included N1 status (HR, 6.77; 95% CI, 2.11-21.68; P < .001), distant metastases (12.24; 6.03-24.85; P < .001), histologic findings of malignant eccrine spiradenoma (5.62; 1.25-25.34; P = .02), and no surgical procedure (2.81; 1.09-7.23; P = .03). Favorable factors for DSS included female sex (HR, 0.52; 95% CI, 0.30-0.91; P = .02). CONCLUSIONS: Five-year survival among patients with malignant cutaneous adnexal tumors is good in the absence of distant metastases. Wide resection may be preferable to less aggressive excision. The prognostic importance of lymph node metastases warrants consideration of lymph node basin staging.

Cutaneous carcinosarcoma: adnexal vs. epidermal types define high- and low-risk tumors. Results of a meta-analysis.

OBJECTIVE: We report four cases of cutaneous carcinosarcoma (CS) and perform a meta-analysis of the cutaneous CS literature. RESULTS: CS occurred in elderly patients (mean of 80 years) on sun-damaged skin, and were keratotic papules of short duration. They did not recur after excision. CS exhibited basal cell carcinoma mixed with atypical fibroxanthoma cell populations. Immunophenotyping revealed vimentin+/keratin- spindle cells and vimentin-/keratin+ epithelial cells. Three cases exhibited p53 protein expression of both carcinomatous and sarcomatous components. Literature review identified 38 cases of cutaneous CS that could be broadly classified into two distinct groups. Epidermal-derived (basal or squamous cell carcinoma epithelial component) CS arose on the sun-damaged skin of the head and neck of elderly males (mean age 72 years) and had a 70% 5-year disease-free survival. In contrast, adnexal CS (spiradenocarcinoma, porocarcinoma, proliferating tricholemmal cystic carcinoma, or matrical carcinoma) occurred in younger patients (mean age 58 years), showed recent growth in a long-standing nodule and had a 25% 5-year disease-free survival. Age less than 65 years, recent growth, long-standing skin tumor, and tumor size greater than 2 cm significantly correlated with poor outcome. CONCLUSIONS: Cutaneous CS is an aggressive skin cancer with high risk for advanced disease. Significant risk factors exist whose identification will allow for better management of CS patients.

Histology according to the Revised European-American Lymphoma Classification significantly predicts the prognosis of ocular adnexal lymphoma.

Lymphoid infiltrates in the ocular adnexa are mostly low-grade B-cell lymphoma, but their clinicopathologic characteristics and prognostic factors have not been extensively analyzed according to the Revised European-American Lymphoma (REAL) Classification. We reviewed histopathologic sections from 77 patients with primary ocular adnexal lymphoid infiltrates, and conducted univariate and multivariate analyses of possible prognostic factors. Fifty-seven of the 77 patients were confirmed to have malignant lymphoma. Histopathologic sections from 44 of the 57 patients were reclassified into the following categories; marginal zone lymphoma (MZL) in 35, mantle cell lymphoma (MCL) in two, diffuse large cell lymphoma (DLCL) in six, and lymphoplasmacytoid lymphoma (LPL) in one. In the remaining 13 patients, biopsied specimens were inadequate for further subclassification. The cause-specific survival rates of the 57 patients with primary ocular adnexal lymphoma at 5, 10, and 15 years were 90.1%, 84.8% and 84.8%, respectively. The univariate analysis showed that the clinical stage, serum lactate dehydrogenase (LDH) value and histopathologic subtype were significant. The 5-year cause-specific survival rate of the 35 patients with MZL was 100%, whereas that of the eight patients with non-MZL (DLCL and MCL) was 25% (p<0.0001). The multivariate analysis revealed that the histologic subtype (p=0.010) and serum LDH value (p=0.015) were independent significant predictors of survival. We conclude that malignant lymphomas occurring in the ocular adnexa histologically consist mostly of MZL. The histologic subtype according to the REAL Classification significantly predicts the prognosis of ocular adnexal lymphoma.