Table 1 Nordic consensus for treating sebaceous carcinoma of the eyelid

 Biopsy

A full thickness or in minimum incisional biopsy, request histological analysis for sebaceous cancer.

[13]

 Biopsy in chalazion surgery

Request histological analysis, when the lesion is clinically suspicious or recurrent.

[13]

 Preoperative conjunctival mapping biopsies

Consider, if there is suspected conjunctival involvement.

[1, 13, 19,20,21]

 Regional lymph node scanning

Offer for category T2b (AJCC 7-th edition) or T2c (AJCC 8th- edition) and worse.

[8, 13, 18]

 Colonoscopy

Should preferably be offered for all patients with sebaceous cancer. 1)

[18, 22]

 A genetic counseling for Muir-Torre syndrome

Should preferably be offered if:

• two or more primary sebaceous tumours in one patient and/or

• under 60 years old and history of another MTS or Lynch cancer (colon, rectum, endometrial, ovarian, small bowel, gastric, urinary tract and brain) and/or

• under 60 years old and at least one first degree relative with a tumour above. 2)

[24]

 Primary treatment method

Surgery with posterior lamellar resection.

[25, 26]

 Clinical margin

At least 4–5 mm. 3)

[6, 10, 13, 38,39,40,41,42,43]

 Method of surgery

Multi-stage resection with delayed closure, frozen sections or Mohs surgery are recommended to verify tumour-free margins. Conjunctival mapping biopsies can be performed together with the final surgery if performed as multi-stage resection with delayed closure.

[6,7,8, 12, 13, 20, 27,28,29,30,31,32,33,34,35,36,37]

 Sentinel lymph node biopsies

SLNB could be considered for tumours larger than 10 mm.

[15, 18, 30, 44,45,46,47,48,49,50]

 PET/CT

PET/CT could be considered in the initial staging.

[56]

 Cryo

In cases with pagetoid spread, additional cryotherapy to the remaining conjunctiva is optional. The primary treatment is local resection if possible without extensive conjunctival resection.

[13, 14, 19, 46, 53]

 Mitomycin-C

In cases with extensive conjunctival epithelial spread or residual conjunctival disease, topical Mitomycin- C could be considered as an alternative to extensive surgery or exenteration. If there is growth deep to the epithelium, Mitomycin-C is not an option.

[6, 8, 46, 49, 51]

 Postoperative adjuvant radiation

Offer radiation for tumors staged T3 (AJCC 7-th edition) or more and in cases with perineural spread or insufficient margins. For patients who deny surgery, radiation at a sufficient dose could be considered.

[8, 13, 30, 46, 49, 52, 53]

 Preoperative chemoreduction

In selected cases preoperative chemoreduction can be considered.

[54, 55]

 The length of the follow-up

In minimum 5 years. 4)

[6,7,8, 11, 26, 41, 46, 57,58,59, 61]

Clinical follow-up interval

Follow-up interval is individual and depends on the post-diagnosis time-frame. In most cases four to 6 months follow-up interval can be considered.

–

 Follow-up examinations

The follow-up should in minimum comprise a clinical examination and palpation for lymph nodes. Patients should also be instructed to palpate the lymph nodes themselves in-between follow-ups. Annual scanning (ultrasound or MRI) for regional lymph node metastases is recommended. Scanning for distant metastases could be considered for category T2b (AJCC 7-th edition) or T2c (AJCC 8-th edition) or worse.

[8, 15, 46, 48, 58]