Dermatologic Manifestations of Sebaceous Carcinoma Treatment & Management

Updated: Mar 22, 2019
  • Author: Wesley Wu, MD; Chief Editor: Dirk M Elston, MD  more...
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Treatment

Medical Care

Sebaceous gland carcinoma is an aggressive tumor with a tendency for both local recurrence and metastasis. Delay in diagnosis may contribute to the poor outcome in this tumor; therefore, a high degree of suspicion when eyelid lesions occur and a willingness to perform a biopsy will most likely contribute to a better prognosis for patients with this tumor. Radiation therapy has traditionally been considered palliative but not curative. Chemotherapy has also been used in aggressive cases with mixed results and requires further study.

In a small series of 6 patients, all 6 experienced a relapse 2 months to 2 years following radiation therapy, but they remained tumor-free following subsequent surgery. [91]

Rao et al reported a mortality rate of 78% in patients treated primarily with radiation therapy, in contrast to a rate of 7% for those treated with wide excision [18] ; however, one case report describes two patients who refused surgery who were successfully treated with radiation therapy [92] and another case series describes two patients who underwent radiation therapy as well, with no recurrence after 27 and 36 months, respectively. Radiotherapy is useful in patients who are not candidates for surgery. One study of 13 patients with stage T3 sebaceous carcinoma reported reduced recurrence rates in those who received adjuvant radiotherapy (28%) compared with those who did not (83.3%). [93]

While the significance of pagetoid spread is debated, most authors agree treatment of this epithelioid spread is warranted. It has been suggested, but not studied, that topical chemotherapy to the involved conjunctivae following surgical excision of the invasive tumor may be beneficial. [45, 47] Mitomycin 0.04% four times daily for 1 week repeated every 2 weeks for 6 weeks has been reported to be effective for pagetoid lesions in case studies. [94]

Systemic chemotherapy may be an option for sebaceous carcinoma refractory to surgical excision and radiotherapy. Case studies have reported variable results with use of 5-fluorouracil, cisplatin, docetaxel, capecitabine, paclitaxel, and doxorubicin (Adriamycin) for aggressive tumors. These reports have suggested its potential use as a preoperative neoadjuvant therapy. [95, 96, 97, 98, 99]

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Surgical Care

Sebaceous gland carcinoma remains a dangerous tumor and produces significant morbidity and mortality. Heightened awareness by the clinician and early biopsy may impact management of this rare tumor. Therapeutically, cryosurgery and surgical excision have been used for sebaceous carcinoma. [47]

Cryotherapy has been useful for a variety of cutaneous tumors and holds the theoretic advantage of treating large areas of conjunctivae if pagetoid spread is present. Targeting margins and the scleral bed may reduce recurrence rates. [100] Long-term studies evaluating the use of cryosurgery for sebaceous gland carcinoma with pagetoid spread need to be performed.

Surgery has been and remains the primary treatment modality for sebaceous gland carcinoma. When orbital involvement is documented, therapy has traditionally been orbital exenteration. [79] Without orbital involvement, surgical therapy typically involves excision of the visible tumor plus 5-6 mm of healthy-appearing tissue in all directions, followed by either frozen section or permanent section for histologic analysis. [101] This approach has not been completely satisfying, because local recurrence occurs in approximately one third of patients, [6] with a 5-year mortality rate reported to range from 18-30%. [101]

The use of the fresh-tissue Mohs technique has been successful in a number of case reports. Spencer et al treated 18 patients with periocular sebaceous gland carcinoma using the Mohs technique, with an average follow-up period of 37 months. The recurrence rate was 11.1%, one of which also developed metastatic disease to the parotid lymph nodes. [101] Similarly, a retrospective review of 49 cases of sebaceous carcinoma treated by the Mohs technique revealed a local recurrence rate of 12% (6 of 49) and a metastatic rate of 8%. [102] In a retrospective study at the Mayo clinic comparing wide local excision with the Mohs technique in 52 patients with 70 sebaceous carcinomas, the Mohs group had a recurrence rate of 1 case in 35 at 6 years postoperatively and 1 case in 24 in the wide local excision group (median margin 1 cm) at 4 months after surgery. [103] Washington University published a retrospective review of 37 patients with 45 sebaceous carcinomas with an average follow up of 3.6 years, reporting zero instances of local recurrences, metastases, or disease-specific deaths. [68] Most recently, a Chinese study published a 360-patient multicenter cohort study with 5-year follow-up, showing improved recurrence, metastatic, and disease-specfic death rates for Mohs micrographic surgery (15.7%, 7.8%, and 5.2%) compared with wide local excision (39.6%, 15.5%, and 8.6%, respectively). However, in patients with pagetoid intraepithelial neoplasia, the recurrence, metastasis, and tumor-related mortality rates were significantly higher. [104] Although recurrence rates may be attributed to discontiguous growth, Mohs micrographic surgery seems to offer a reduced recurrence rate compared with standard excision, with some proponents arguing it is the treatment of choice for nonpagetoid sebaceous carcinomas not involving the orbit.

The treatment of tumors with pagetoid spread remains controversial. Some authorities have suggested that complete excision of involved epithelia is necessary, while others have suggested only frank invasive tumor needs to be treated, after which only careful clinical observation of the involved epithelia is warranted. It is recommended that patients with signs of pagetoid spread such as diffuse eyelid thickening and conjunctival inflammation should consider multiple conjunctival map biopsies and adjuvant therapy such as cryotherapy and mitomycin C. Given the aggressive nature of this tumor, treating pagetoid spread as direct tumor extension and continuing surgical excision until all margins are clear, including clear of pagetoid spread, is wise. [105]

One retrospective study from England using “slow Mohs,” or delayed reconstruction after complete excision with paraffin section control for 17 patients, reported improved evaluation of pagetoid spread with paraffin-embedded sections and local recurrence of 18% and mortality of 12% after an average follow-up of 5 years. [106]

Future larger series are needed to better delineate the true significance of pagetoid spread.

Given that lymph node involvement may be seen in 15-21% of cases, prophylactic lymph node excision or sentinel lymph node biopsy may be considered in conjunction with surgical excision of sebaceous carcinoma. However, neither of these options has been recommended by a sufficient body of evidence. [107, 108]

Based on the Surveillance, Epidemiology, and End Results Program (SEER) reported rates of regional and distant metastasis for ocular sebaceous carcinoma (4.4%) and extraocular head and neck sebaceous carcinoma (0.9%), [108] Chang et al suggest sentinel lymph node biopsy for ocular sites and routine reginal lymph node surveillance for extraocular head and neck sites. [72] In addition, the authors in a retrospective study of 50 patients with ocular sebaceous carcinoma concluded that a sentinel lymph node biopsy or strict regional lymph node surveillance may be appropriate for ocular sebaceous carcinomas greater than 10 mm. [55]

Parotidectomy has also been reported in cases of sebaceous carcinoma with regional lymphadenopathy or metastasis. [10] Given the potential for metastasis to the parotid gland, further evaluation is warranted, particularly with an upper eyelid sebaceous carcinoma. [7, 45, 59]

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Consultations

Referral to an internist, urologist, and gastroenterologist is warranted in patients diagnosed with sebaceous carcinoma in order to evaluate for the presence of internal (internist) and bowel (gastroenterologist) lesions associated with Muir-Torre syndrome. A geneticist is also recommended for further workup of syndromic associations.

An ophthalmologist or oculoplastic surgeon may also be considered if there is orbital involvement and possible need for exenteration. Of note, if corneal involvement is suspected, the patient should avoid cataract surgery to avoid seeding tumor into the globe.

For systemic disease, an oncologist is necessary for chemotherapy. A radiation oncologist referral may be helpful for tumors in patients who are poor surgical candidates, and this can be used as adjuvant radiotherapy.

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Complications

Radiotherapy can result in chronic dry eyes, trichiasis, exposure keratopathy, conjunctival keratinization, cataract, optic neuropathy, retinopathy, and permanent loss of visual acuity.

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