Eyelid disease spreads to the bulbar conjunctiva, dictating need for adjuvant cryotherapy and/or wide surgical excision.
Caruncle disease extends directly to the bulbar conjunctiva.
Tumor often extends to the adjoining lid at the canthus.
Small tumors are amenable to cure by surgery alone.
- Early diagnosis and recognition are critical.
For larger tumors, some advocate excision of the skin and eyelid disease by the Mohs technique.
- However, the primary focus with eyelid sebaceous carcinoma is on posterior extension to the bulbar conjunctiva, with slit lamp biomicroscopy and map biopsies if necessary, and serial evaluation as appropriate.
Deeper extension and larger tumors, with invasion of the orbit, recognized clinically and confirmed by biopsy, dictates the need for more aggressive surgery and/or adjuvant treatment.
- The risk of hematogenous spread and extension to regional lymph nodes is increased.
Regional lymphadenopathy recognized clinically and/or by sentinel lymph node biopsy dictates the need for lymph node dissection and possibly adjuvant regional radiotherapy.
Perineural invasion evident on histopathology is a risk factor for in transit disease and an indication for adjuvant radiotherapy (OPRS 2011; 27:356).
Distant metastases, most commonly to the lung, liver and brain, have no specific treatment or cure.
There is little data to suggest efficacy for chemotherapy or immunobiologic therapy with this tumor.
Neoadjuvant systemic chemotherapy has been described in ten patients (Kaliki, 2016)
- Cisplatin and 5-Fluorouracil for mean tumor basal diameter 36 mm (range, 20 to 65 mm), with orbital tumor extension in 9 cases.
- Three or four cycles of chemotherapy, mean percentage reduction of tumor basal diameter after neoadjuvant chemotherapy was 74% (range, 30% to 100%).
- Residual tumor was excised in 7 cases.
- Two cases with residual orbital component underwent eyelid-sparing orbital exenteration.
- No tumor recurrence in 7 cases at a mean follow-up period of 18 months (range, 3 to 63 months).
- One patient died due to systemic metastasis.
Topical mitomycin-C (Br J Ophthalmol 2004; 88:718) or 5-fluorouracil (Ophthalmology 2000; 107: 2190) may effectively reduce intraepithelial disease, but cannot be considered curative because of the depth of penetration by the drug and at best has an adjuvant role.
External beam radiation can be administered as primary or adjuvant therapy.
Thirteen patients were treated in Japan with primary radiotherapy, all responded with no local disease evident at five years (Int J Rad Oncol Biol Phys 2012; 82:605).
- A total dose of 50 to 66.6 Gy (median, 60 Gy) was delivered in 22 to 37 fractions.
In another series of 16 patients treated primarily with radiotherapy the median dose was 60 Gy, delivered in 18-37 fractions, with a median of 30 fractions (Strahlentherapie und Onkologie 2012: 188:1102)
Radiation fields are set with a 10‑mm tumor-free margin on either side — the eyelid typically measures 30 mm in width — therefore the entire eyelid and beyond are included in the typical treatment field.
A lens shield is mounted on the cornea, which protects the lens, but potentially limits treatment of bulbar conjunctival disease.
Irradiated tumors grossly disappear within 4 months.
Dry eye and cataracts are the primary long-term complications.
Complete wide surgical excision of the lid should include approximately 3-5 mm of histologically proven uninvolved tissue on either side of the tumor with margin control by either frozen section histopathology or Mohs' micrographic surgery.
- The decision to include a 3 or 5 mm margin – or even a larger margin of 7-8 mm - depends on the likely elapsed time that has allowed for tumor growth, and the estimate of tumor size.
- The tumor spreads radially with time, initially with thin fronts of extension - these may not be clinically evident, and are difficult to identify even on histopathologic examination of the surgical margin.
- Growth may be deep to the skin or conjunctival surface, such as along the tarsal surface, which may not be visible on biomicroscopy.
- The decision to remove additional full thickness lid, based on clinical judgment, balances vigilance in excising the tumor and a desire for tissue preservation.
Conjunctival disease, whether recognized by map biopsies or slit lamp biomicrsocopy, is treated by a combination of surgery and cryotherapy
- When extensive conjunctival treatment, excision and grafting are required, compromise of the ocular surface is expected.
When tumor has extended to the adjoining lid, at the canthus, a wide radial excision is needed for adequate tumor free margins.
When tumor growth invades deep into the fornices and/or canthi, with potential orbital invasion, a decision is made regarding exenteration.
- Untreated orbital invasion is initially more of a risk for local recurrence, but continued tumor growth, unrecognized in the orbit, can lead to regional and distant metastasis.
- It is not assumed that exenteration is necessary to minimize the risk of metastasis - potential deep invasion suggests that planes of tumor infiltration in the orbit can be difficult to identify.
- Globe sparing, more limited excision is an option for limited orbital extension.
- Study the orbit by random and contiguous sampling and assess the degree of orbital involvement – it is difficult to predict the direction of deep orbital invasion.
- Adjuvant radiotherapy is a reasonable option for potential tumor invasion; adequate data on the efficacy is unavailable.
Once tumor invasion to orbital fat or muscle is recognized, adjuvant radiotherapy and/or exenteration are two accepted management options, since no medical treatment has proven efficacy.
Common treatment responses, follow-up strategies
Careful, long term monitoring is needed.
A follow-up study of 34 sebaceous carinoma patients who had undergone excision with 5-mm surgical margins and paraffin section pathologic analysis, mean follow-up period was 43.7 ± 24.9 months (range 6–120 months) (Takahashi, 2016)
- Only four cases of recurrence or metastasis, all with tumor size greater than 20 mm. All patients without recurrence had tumor size ≤ 20 mm.
- Interval from excision to recurrence or metastasis ranged from 5-27 months.
Local tumor recurrence is treated by surgery and cryotherapy.
Regional recurrence is treated by surgery and adjuvant radiotherapy.
There is no effective treatment for distant metastasis.