27.5Periorbital SCC

The second most common periorbital malignancy is squamous cell carcinoma [4]. SCC accounts for approximately 5–10% of all periorbital malignancies and has an incidence of 0.09–2.42 per 100,000 [5, 6, 27, 28]. The mean age of presentation is during the seventh decade of life [29]. Similar to BCC, SCC also presents more commonly on the lower eyelid but not to the same degree as BCC [30]. SCC has the following periorbital distribution: lower eyelid (48.6–68%), medial canthus (30–36%), upper eyelid (22.5%), and lateral canthus (16.2%) [31, 32]. SCC of the eyelid is much more likely to recur and metastasize when compared to other anatomical locations. Periorbital SCC metastatic rates have been reported to be as high as 21% [28]. Factors which are associated with a higher rate of periorbital SCC metastasis include: perineural invasion, recurrence following treatment, large tumor size, and poor differentiation [30].

Dr. Mohs used MMS for 213 cases of periorbital SCC and reported an overall 98.1% 5-year cure rate. Primary periorbital SCCs had a 98.5% cure rate versus 95.8% for recurrent SCCs [11].

Another large multicenter trial demonstrated that MMS had the lowest recurrence rate at 3.6% when compared to other modalities of treatment [32]. For periorbital SCC in situ, the overall recurrence rate was 8.3%, with a primary SCC in situ recurrence rate of 5% and a recurrent SCC in situ recurrence rate of 12% [32]. Using conventional surgical excision, the recurrence rate is approximately 18% for periorbital SCC [4, 33].

Moul and colleagues feel that “MMS should be a consideration especially for large or poorly delineated tumors, recurrent tumors, those with perineural involvement or moderately to poorly differentiated subtypes, and those close to important anatomic structures.” [4]

Another important clinical point to keep in mind when dealing with SCC is that demonstrated by a study conducted by Doxanas and colleagues who demonstrated the importance of accurate histopathologic diagnosis of SCC of the eyelid [34]. Forty-four tumors were originally diagnosed as squamous cell carcinoma, but in fact, only 31 of the tumors were squamous cell carcinomas of the eyelid, 75% occurring in patients over the age of 60 [34]. The incorrect initial pathologic diagnosis was most commonly sebaceous gland carcinoma, followed by basal cell carcinoma [34]. Others

were incorrectly diagnosed as seborrheic keratosis, inverted follicular keratosis, and papilloma [34]. This study also demonstrated the risk factors associated a likelihood of developing SCC, as three of the patients had a history of prolonged exposure to radiation therapy for acne, eczema, and SCC of the limbus 10–20 years prior to their diagnosis [34]. Squamous cell carcinoma presented in some patients as a lesion with rolled up margins and a central crater with or without intermittent bleeding, and in other patients as a chronic, scaly erythematous lesion. Three patients reported chalazion as their presenting symptom [34]. This study conveys the importance of performing biopsies on lesions that appear suspicious or as persistent benign lesions do not heal, especially because SCC has a higher metastatic potential.

Summary: Other Tumors

•It can be difficult to distinguish the surgical borders of melanoma on frozen sections, especially in the setting of chronic sun damage.

•Lentigo maligna is the most common type of melanoma to present in the periocular region.

•Frozen sections of melanoma are subject to artifact.

•Sebaceous carcinoma is most commonly located on the upper eyelid, and approximately 8% of these tumors metastasize.

•Spencer and colleagues have reported the largest series involving periorbital sebaceous carcinoma with 18 cases, of which 13 were primary and 5 recurrent. Two out of the 18 cases recurred over an average follow-up time of 37 months.

•Most cases of reported periorbital microcystic adnexal carcinoma have been located on the lower eyelid, and MMS or conventional frozen section excisions have become the standard of care.

•In general, Merkel cell carcinoma is associated with a very poor prognosis, and the current National Comprehensive Cancer Network guidelines recommend wide local excision with 2 cm or greater margins, MMS with permanent section technique, or MMS with frozen sections, along with appropriate lymph node biopsy and adjuvant radiation therapy.

27.6Other Tumors

Recently, there has been much investigation into the use of MMS for melanoma; however, very few of the studies specifically examined its use for periorbital melanoma [4]. Lentigo maligna is the most common type of melanoma to present in the periocular region, likely due to chronic sun exposure [4]. In general, melanoma presents some difficulties for MMS in that it can be problematic to accurately distinguish surgical borders when using frozen sections, especially in the setting of chronic sun damage. Frozen sections of melanoma are subject to artifact, making it nearly impossible in some instances to determine the surgical borders of the tumor. Modifications of MMS, such as rush permanent sections and immunostains attempt to minimize those artifacts or “highlight” the melanoma cells. Using a variation of MMS known as Mapped Serial Excision, Malhotra and colleagues demonstrated a recurrence rate of 7.4% with a mean follow-up time of 32 months for periorbital lentigo maligna and lentigo maligna melanoma [35]. For a deeper discussion of MMS for melanoma please refer to Chap. 17 and for a discussion of immunostaining melanoma cells in the setting of MMS, please refer to Chap. 13.

More studies should be carried out to examine the efficacy of MMS for melanomas located in the periorbital region. Lentigo maligna is a slowly growing lesion, and therefore the follow-up times for studies should be in excess of 5 years.

Sebaceous carcinoma is most commonly located on the upper eyelid [12]. It can resemble a chalazion or even blepharitis; however, care should be taken not mistake it for these benign conditions, as the metastatic rate is about 8% [4, 36]. Spencer and colleagues have reported the largest series involving periorbital sebaceous carcinoma with 18 cases, of which 13 were primary and five recurrent [37]. Two of the 18 cases recurred over an average follow-up time of 37 months. Snow and colleagues reviewed the literature and determined that out of 49 reported cases of sebaceous carcinoma, 12% recurred over a 3.1 mean follow-up period [36]. Sebaceous carcinoma is known to have “skip” areas and some experts have suggested using oil red O, CAM 5.2, or permanent sections to more clearly define the surgical tumor margins [38–40]. For an in depth discussion of Mohs micrographic surgery for sebaceous carcinoma, please refer to Chap. 2.

Another uncommon tumor encountered in the periorbital region is microcystic adnexal carcinoma

(MAC). Of the 15 cases found in the literature of periorbital MAC, ten were on the lower eyelid and five on the medial canthus [41–43]. For periorbital MAC, MMS or conventional frozen section excision has become the standard of care [4]. For a discussion of MAC in general, please refer to Chap. 19.

On rare occasions, Merkel cell carcinoma (MCC) can present in the periorbital region. The current National Comprehensive Cancer Network guidelines recommend wide local excision with two centimeter or greater margins or MMS with permanent or frozen sections [4]. The prognosis for MCC is very poor, with only a 30–64% 5-year survival rate [44, 45].

Summary: Conclusion

•Both BCC and SCC account for almost all of the periocular cutaneous tumors.

•It is important to consider Mohs surgery as a treatment option for periocular BCC and SCC to excise completely with conservation of the delicate tissue.

27.7Conclusion

In conclusion, both basal cell and squamous cell carcinoma account for almost all of the periocular cutaneous tumors. Periocular BCC and SCC have very high 5-year cure rates when using both MMS and the conventional frozen section technique. However, it appears that MMS tends to have a slightly higher 5-year cure rate, which is important to take into consideration because of the location of these tumors. Periocular tumors are located in an area such that a diagnosis needs to be make quickly and precisely so that the tumor can be excised quickly and completely to prevent recurrence which can cause significant damage to such a delicate tissue as the eye.

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