Impact of surgery and adjuvant treatment on the outcome of extraocular sebaceous carcinoma: a systematic review and individual patient's data analysis of 206 cases

Abstract

Background Extraocular sebaceous carcinoma (EOSC) is an aggressive malignancy of the sebaceous gland. Surgery is considered the cornerstone of treatment, but there is lack of clarity about extent and adjuvant treatment. Methods We conducted a systematic review and analysis of individual patient data of all published cases of EOSC to look into demography, pattern of care, importance of type of surgery, and other adjuvant treatment and survival outcome. A search of PubMed and Google Scholar was done with the key words sebaceous carcinoma, extraocular sebaceous carcinoma, and Muir‐Torre syndrome till December 2017. The data were compiled in an Excel chart and analyzed using SPSS IBM software. Results Data of 206 patients were retrieved. Median age at presentation was 65 years (range: 11–96 years). Surgery was performed in all except 13 patients. Of these 13, eight were deemed inoperable for extensive disease, and five had metastatic disease. Median PFS and OS for the entire cohort were 84 months (95% CI: 10–158 months) and 92 months (95% CI: 59–126 months). Univariate analysis revealed significantly poor survival for patients with a metastatic disease, regional nodal metastasis, and those with Mohs micrographic or incomplete surgery. Conclusion EOSC is a disease of elderly patients with good prognosis. Complete surgery with regional lymph node dissection is standard treatment. The role of adjuvant radiotherapy is debatable but can be considered in patients with incomplete surgery or high‐risk factors.

Clinical Features and Prognosis of Young and Middle-Aged Adults With Skin Sebaceous Adenocarcinoma

“…5,7,10,13 For extraocular SAC, wide excision was the surgery of choice. 18 For nonextraocular SAC, Mohs surgery could bring at least similar disease-specific and overall survival to wide excision. [19][20][21] The authors observed that the gross excision (Mohs surgery included) independently indicated better prognosis for young and middle-aged SAC, although higher proportions of small tumors (#20 mm) were removed by gross excision rather than by wide excision (84.4% vs 55.2%; p , .001).…”

A 20-Year Retrospective Analysis of Skin Cancers: Comparison of the First and Second Decades

“…Different figures have been reported regarding its lymphatic involvement and metastasis rates. Lymphatic metastasis rates of 1.3-16% and non-lymph node metastasis rates of 0.4-10% have been reported (55,56). Due to these different rates, there are different attitudes regarding the use of sentinel lymph nodes.…”

“…Although the role of adjuvant radiotherapy is controversial, radiotherapy can be given after complete tumor resection either alone or in combination with chemotherapy (55). Primary radiotherapy alone is not recommended due to high recurrence rates (56,58,60). It can be used in patients who refuse surgery or cannot undergo surgery due to comorbidities, and there are also publications that recommend its postoperative use after resection (55).…”

Sebaceous Carcinomas: A Clinicopathological Comparison of Ocular and Extraocular Variants

“…45 Our results mirror those clinical studies that have shown that extraocular SC are only locally aggressive tumors with low mortality rate; however, some studies have shown that some tumors may have demonstrated regional nodal and even visceral metastases. 29,44,[46][47][48] In our experience, cases of extraocular SC follow an indolent clinical course after complete excision. This study has some important limitations: none of our extraocular SC underwent sentinel lymph node biopsy; also, its retrospective nature limits the long-term clinical data available for analysis.…”