REPAIR AND RECONSTRUCTION
Malignant Lesions of the Eyelids KENNETH L. PIEST, MD _
_
_
_
_
~
BACKGROUND. The ocular adnexal area contains virtually every tissue type; nearly any type of malignant tumor may develop in this area. However, the vast majority of malignant eyelid tumors are carcinomas. Three types of carcinomas produce most of the malignancies in thisgroup. They are: basal cell carcinomas, squamous cell carcinomas, and sebaceous cell carcinomas. OBJJXTWE. The management of these three tumors is discussed. METHODS.A review of current treatment options for eyelid
A
pproximately 50,000 to 60,000 malignant eyelid tumors occur in the United States each year. Three types of carcinomas account for ,the vast majority of the malignant eyelid tumors: basal cell carcinomas (BCCs), squamous cell carcinomas (SCCs), and sebaceouscell carcinomas. Basal cell carcinomas are by far the most common malignancy of the eyelid. Squamous cell carcinomas and sebaceous cell carcinomas are much less common than BCCs. However, because the ocular adnexal area contains virtually every type of tissue, a wide array of malignant tumors may form, but they are extremely rare.
Basal Cell Carcinoma Basal cell carcinomas arise from the basal cells of the epidermis. They generally occur in areas of sun-exposed skin. In the ocular adnexal region it is most common in the lower eyelid and medial canthal area. The growth pattern of BCC is characterized by slow local growth, but it is capable of extensive local invasion and even metastasis.'S2 The most common clinical presentation for a BCC is that of a translucent nodule with superimposed telangiectatic vessels. Stromal tissue and a good blood supply are essential to the tumor's continued growth.3,' As the
From the Department of Ophthalmology, Uniuersity of Texus Health Science Center, San Antonio, Texas. Address correspondence and reprint requests to: Kenneth L Piest, MD, Department of Ophthalmology, Uniu. of Texus Health Science Center, 7703 Floyd Curl Dr., San Antonio, Tx 78284-6230.
1056
malignancies was performed. summary of the current treatment of the three most common eyelid carcinomas is discussed. CONCLUSION. With early recognition and appropriate management of eyelid malignancies, an extremely favorable prognosis can be obtained. The management is twofold and consists of curing the patient of the malignancy and then restoring the form andfunction of the eyelid in order to save the eye if possible. J Dermatol Surg Oncol1992;18:10561059. RESULTS. A
tumor enlarges, central tumor necrosis may occur as growth surpasses the nutrient supply, producing a central ulcer in the nodule (Figure 1).Other clinical variations are possible. All lesions do not necessarily have elevated margins. The aggressive morpheaform variant may present as an indurated sclerotic plaque with ill-defined borders. Melanin may be present, producing varying degrees of tumor pigmentation. Numerous subtypes of BCC have been described. These can be simplified into four basic types: nodular, ulcerative, morpheaform (sclerosing), and multicentric? The nodular form is the "classic" BCC. Growth of the tumor produces a localized mass. Production of an angiogenic factor produces the characteristic superficial telangiectaticvessels? A pseudocapsule is formed as the tumor grows and compresses the adjacent dermal tissues. Histologically, the tumor nodule is composed of a uniform population of basaloid cells with the peripheral cells demonstrating a palisading pattern. Small to large cysts may form within the tumor nodule, which may make the diagnosis more dif&mlt. If surface ulceration o c m , the pseudocapsule usually is not present. Instead, a chronic dermal inflammatory reaction is seen. In the morpheaform or sclerosing type of BCC, elongated strands of basoloid cells are surrounded by a dense fibrous stroma. This appearance resembles scirrhous carcinoma of the breast. Morpheaform BCCs are aggressive and deeply infiltrate adjacent tissue. The multicentric form has a more irregular surface than the nodule type. As the name implies, this type of BCC exhibits a diffuse multicentric involvement of the epidermis and superficial dermis. 0 1992 by Elsevier Science Publishing Co., Inc. 0148-0812/92/$5.00
J Denatol Surg Oncol 1992;18:1056- 1059
PEST 1057 MALIGNANT LESIONS O F THE EYELID
In cases where orbital extension has occurred, exenteration is the recommended treatment. If the disease involves the regional lymph nodes, surgery with radiation should be considered. Distant spread is obviously associated with a poor prognosis, but chemotherapeutic treatments are available in these s i t u a t i ~ n s . ~ ~ J ~ After treating a patient for BCC, regular follow-up exams are imperative. Most recurrences occur within 5 years, but late recurrences are A new BCC may also arise and 20 to 30% of these appear within 1 year of the original lesion. All patients at risk of developing skin carcinomasshould avoid excessive sun exposure and use sun screens with a sun protective factor greater than a rating of 15 whenever possible. Figure 1. Basal cell carcinoma of the right lower eyelid. Note the large, elevated nodular appearance with central ulceration.
Squamous Cell Carcinoma
The goal in management of a BCC is the complete elimination of the lesion. In the nodular form, surgical excision of a small lesion with histologic confirmation that the margins are free of tumor is appropriate. However, the ulcerative, morpheaform, and the multicentric types often extend beyond the area of apparent clinical involvement. Tumor excision with frozen section examination of all margins including the tumor base or Mohs micrographic surgery are the best available methods for complete tumor excision when these tumor subtypes are or a tumor recurrence is encountered.7-l0 However, even with the best techniques, evaluation of the margins may be quite difficult, especially in the morpheaform type or in a recurrence within scar tissue. Cryotherapy may be appropriate in situations where multiple tumors are present, excision is not possible, or both. With cryotherapy a double freeze-thaw method with tissue temperatures to -50' C is needed to destroy the tumor cells." Metastasis from BCC is infrequent with incident estimates ranging from 0.028 to l.0%.'213 When it does occur, lymphatic spread to the regional lymph nodes and hematogenous spread to bone, lungs, and liver are the most common sites. Despite their low metastatic rate, BCCs are locally invasive. Orbital involvement with subsequent intracranial extension is the most common cause of death due to tumor spread." Disseminated disease from BCC is rare, but if suspected a systemic workup is required. This includes a detailed history, physical examination with particular attention to the regional lymph nodes, and testing consisting of a complete blood count, liver profile, chest x-ray examination, bone scan, liver scan, and computed tomography scan if orbital or intracranial extension is suspected.
Squamous cell carcinoma is a malignant tumor of the c e k of the epidermis. Less than 5% of the epithelial neoplasms of the eyelids are S C C S . ~ Squamous ~ cell carcinoma most commonly involves the lower eyelid, but may occur in any location. When the tumor involves the upper lid and lateral canthal location, the SCC is more common than the BCC. It is thought to arise from the same factors that promote the development of BCCs (actinic damage, fair complexioned individuals, etc). Genetic conditions such as xeroderma pigmentosa and albinism are well known conditions with an increased risk of developing SCC. Immune suppression may also increase the risk of developing SCC; the lesions primarily occur in sun exposed areas.- Previous radiation treatment is also another risk factor. Squamous cell carcinomas usually arise in actinically damaged skin. They usually present as an elevated, indurated plaque or nodule with irregular borders. As the tumor enlarges, ulceration may occur, which is often covered by crusting debris (Figure 2). The same treatment considerations for BCC apply to the treatment of SCC. These lesions should be completely excised. Usually excision under frozen section examination of the margins or Mohs surgery is recommended, especialIy if the lesion is a recurrence. Metastasis from eyelid lesions detected early is rare (1.0%). This is in contrast to SCCs located in other anatomic sites, which may metastasizeearly and can be fatal. Advanced or neglected eyelid lesions do have the potential to spread both by local invasion and by metastasis. Dissemination to regional lymph nodes is more common than spread to distant organs." Furthermore, direct perineural invasion into the central nervous system can occur, resulting in death. If local extension or orbital involvement is suspected, imaging studies should be obtained. Invasive SCC is potentially fatal, but early eyelid le-
1058 PEST
Figure 2. Recurrent squamous cell carcinoma. Squamous cell carcinoma of the left eyelid and lateral canthal area. Keratin is seen on the surface of the lesion. (Courtesy of Charles R Leone, MD.)
sions treated appropriately have an excellent prognosis. The same follow-up and preventative measures as outlined previously for BCC apply even more strongly for SCC.
Sebaceous Cell Carcinomas Sebaceouscell carcinoma of the eyelid accounts for 2% to 5% of the epithelial eyelid malignancies. They arise from the meibomian glands and Zeis glands of the eyelid or from sebaceous glands in the caruncle or brow (see the section on anatomy in the article by Drs. Dailey and Wobig-). The upper lid contains approximatelytwice the number of meibomian glands as does the lower lid, which likely accounts for the fact that the upper lid is involved twice as frequently as the lower lid. Outside of the ocular area, sebaceouscell carcinoma is an extremely rare tumor. Sebaceous cell carcinomas are perhaps the most dangerous eyelid tumors because they can present with such a broad sDectrum of clinical Dresentations. Thev frequently mimic inflammatory eyelid conditions, which often can confuse and delay the true diagnosis. The term "masquerade syndrome" has been used to describe this situation. Common diagnoses masqueraded by sebaceous cell carcinomas include atypical or recurring chalazia, unilateral blepharoconjunctivit, or papillomas. The true diagnosis can be obscured until systemic metastasis occurs, resulting in discovery of the primary tumor. Most commonly a sebaceous cell carcinoma presents as a firm nodule resembling a chalazion (Figure 3). However, others may have a more diffuse thickening of the tarsus or a papillomatous growth pattern. As the lesion involves the lid margin, loss of eyelashes occurs as the 1
J D e n a t o l Surg Oncol 2 992;2 8:Z 056- 2059 tumor invades the lash follicles. Ulceration may also develop. Sebaceous cell carcinomas tend to invade the overlying forming scattered nests of malignant cells (pagetoid invasion) or completely replacing the epithelium (intraepithelial carcinoma). This may give the clinical appearance of a unilateral blepharoconjunctivitk that should immediately alert the clinician because a true blephraroconjunctivit is most commonly a bilateral condition. Another extremely important feature of this type of carcinoma is its tendency to have multicentricsites of origin. Independent foci of tumor cells, which can not only involve multiple sites on the same lid, but can also involve the upper and lower lid simultaneously, are reported 6 to 10% of the time.27This makes the task of complete excision of the lesion extremely difficult. Treatment of sebaceous cell carcinomasof the eyelid is by full-thickness excision with wide margins and with frozen section pathologic control. However, the recurrence rate after resection is approximately 30'3".28If the bulbar conjunctivais involved, cryotherapymay be helpful in attempts to avoid loss of the globe. If the tumor has extended into the orbit, exenteration is the recommended treatment. In addition to local invasion, sebaceous cell carcinoma can spread to regional lymph nodes and produce distant metastases. Regional metastases occur in up to 25% of patients. Vascular invasion can produce distant metastasis with the primary distant metastatic sites being lung, liver, brain, and skull. However, if distant metastases occur, they are almost always associated with a history of involvement of regional lymph nodes. Thus,one should always examine the patient with a suspected malignant eyelid lesion for palpable regional nodes. The overall mortality from this tumor is estimated to be Figure 3. Sebaceous cell carcinoma of the left lower eyelid. A small to medium sized lesion is noted at the lid margin in the medial 3 of the lower lid. (Courtesy of Charles R Leone, MD.)
J Dermatol Surg Oncol 1992;18:1056- 1059
20%. However, with the current higher index of suspicion and earlier diagnosis, the mortality rate is decreas-
ing.= Any suspicious lesion should be biopsied. Atypical chalazia should be treated appropriately with expressed debris or a biopsy submitted for pathologic evaluation. Any unilateral blepharoconjunctivit, especially if chronic, should be suspected and scraped with cytologic examination performed. If suspiciouscells are found, a lid biopsy should be performed to obtain the diagnosis. Carcinomas of the eyelid are by far the most common malignant entities involving the ocular adnexal area, but virtually any other soft tissue or bony neoplasm can occur in or around the eyelid, including malignant melanomas and metastatic lesions. Any treatment should be primarily based on early recognition, early diagnosis (biopsy), and complete excision of the lesion so as to give the patient the best prognosis. Reconstructive efforts are then undertaken to restore both the form and function of the eyelid in order to protect the globe.
Acknowledgment. This work was supported in part by an unrestricted grant from Research to Prevent Blindness, lnc.
References 1. Miller SJ.Biology of basal cell carcinoma (part I). J Am Acad Dermatol 1991;24:1-13. 2. Miller SJ. Biology of basal cell carcinoma (part 11). J Am Acad Dermatol 1991;24:161-75. 3. Lyles TW,Freeman RG, Knox JM. Transplantation of basal cell epitheliomas. J Invest Dennatol1960;34:353. 4. Van Scott EJ, Reinertson RP. Modulating influence of stromal environment of epithelial cells studied in human autotransplants. J Invest D m t O l 1961;36:109-31. 5. Sexton M, Jones DB, Maloney ME.Histologic pattern analysis of basal cell carcinoma. Study of a series of 1039 consecutive neoplasms. J Am Acad Dermatol 1990;23:111826. 6. Wolf JE,Hubler WR.Tumor angiogenic factor and human skin tumors. Arch Dermatol1975;111:321-27. 7. Domes RN, Walker NP,Collin JR. Micrograph (MOW) surgery in the management of periocular basal cell epitheliomas. Eye 1990;4:160-8. 8. Monheit GD, Callahan MA, Callahan A. Mohs micrographic surgery for periorbital skin cancer. Dermatol Clin 1989;7677-97. 9. Frank HJ. Frozen section control of excision of eyelid basal cell carcinomas: 8f years experience. Br J Ophthalmol 1989;73:328- 32. 10. Doxanas MT, Green WR,Iliff CE. Factors in the successful surgical management of basal cell carcinoma of the eyelid. Am J Ophthalmol 1981;91:726-36.
PIEST 1059 MALIGNANT LESIONS OF THE EYELID
11. G u n n m n G, Lark0 0,Hersle K. Cryosurgeryof the eyelid for basal cell carcinoma. Ada Ophthalmol (Copenh) 1990;68:241-5. 12. Farmer ER, Helwig EB. Metastatic basal cell carcinoma: a clinicopathological study of seventeen cases. Cancer 1980;46:748-57. 13. Lo JS, Snow SN, Reizner CT,Mohs FE, Larson PO, Hruza GJ. Metastaticbasal cell carcinoma: report of 12caseswith a review of the literature. J Am Acad Dermatol 1991;24: 715-9. 14. Glover AT, Grove AS. Orbital invasion by malignant eyelid tumors. Ophthalmic P l a t R ~ o n ~Surg t r 1989;S:l-12. 15. Morley M, Finger PT, Perlin M, Weiselberg LR, DeBlasio DS. Cis-platinum chemotherapy for ocular basal cell carcinoma. Br J Ophthalmol1991;75:407-10. 16. Baxter DL, JoyceAP,Feldman BD, Lynch JW.Cis-platinum chemotherapy for basal cell carcinoma: the need for posttreatment biopsy-report of a case. J Am Acad Derm 1990;6:1 167- 8. 17. Robinson JK. What are adequate treatment and following care for nonmelanotic cutaneous cancer. Arch Dermatol 1987;123:331-3. 18. Robinson JK. Risk of developing another basal cell caranoma: a five year prospective study. Cancer 1987;60:11820. 19. Epstein E. Value of follow-up after treatment of basal cell carcinoma. Arch Dermatol1987;108:798-802. 20. Schrieber MM,Moon TE,Fox SH, Davidson J. The risk of developing subsequent nonmelanotic skin cancer. J Am Acad Dermatol 1990;23:1114-8. 21. Kwitko ML, Boniuk M, Zimmerman LE. Eyelid tumors with reference to lesions confused with squamous cell carcinomas. Arch Ophthalmol 1963;69:693-7. 22. Lutzner MA. skin cancer in immunosuppressed organ transplant recipients. J Am AcadDermatol1984;11:891-3. 23. Rao NA, Dunn SA, Romero JL, Stout W. Bilateral carcinomas of the eyelid. Am J Ophthalmol1986;101:480-2. 24. Lund HZ.How often does squamous cell carcinoma of the skin metastasize. Arch Dermatol 1965;92:635- 7. 25. Dailey RA, Wobig JL. Eyelid anatomy. J Dennatol Surg On~011992;18:1023-7. 26. Font RL. Eyelids and lacrimal drainage system. In. Spencer WH,ed. Ophthalmic Pathology: An Atlas and Textbook, vol3. Philadelphia. WB Saunders, 1986. 27. Roa NA, Hidayat AA, Mckan IW,Z i m m m LE. Sebaceous gland carcinoma of the ocular adnexa: a clinicopathological study of 104 cases with five year follow-up data. Hum Pathol 1982;13:113-22. 28. Epstein GA, Puttennan AM. Sebaceousadenocarcinomaof the eyelid. Ophthalmic Surg 1983;14:935-40. 29. Char DH. Adult and pediatric lid tumor diagnosis. In:Char DH, ed. Clinical Ocular Oncology. New York Churchill Livingstone, 1989.
Malignant Lesions of the Eyelids KENNETH L. PIEST, MD _
_
_
_
_
~
BACKGROUND. The ocular adnexal area contains virtually every tissue type; nearly any type of malignant tumor may develop in this area. However, the vast majority of malignant eyelid tumors are carcinomas. Three types of carcinomas produce most of the malignancies in thisgroup. They are: basal cell carcinomas, squamous cell carcinomas, and sebaceous cell carcinomas. OBJJXTWE. The management of these three tumors is discussed. METHODS.A review of current treatment options for eyelid
A
pproximately 50,000 to 60,000 malignant eyelid tumors occur in the United States each year. Three types of carcinomas account for ,the vast majority of the malignant eyelid tumors: basal cell carcinomas (BCCs), squamous cell carcinomas (SCCs), and sebaceouscell carcinomas. Basal cell carcinomas are by far the most common malignancy of the eyelid. Squamous cell carcinomas and sebaceous cell carcinomas are much less common than BCCs. However, because the ocular adnexal area contains virtually every type of tissue, a wide array of malignant tumors may form, but they are extremely rare.
Basal Cell Carcinoma Basal cell carcinomas arise from the basal cells of the epidermis. They generally occur in areas of sun-exposed skin. In the ocular adnexal region it is most common in the lower eyelid and medial canthal area. The growth pattern of BCC is characterized by slow local growth, but it is capable of extensive local invasion and even metastasis.'S2 The most common clinical presentation for a BCC is that of a translucent nodule with superimposed telangiectatic vessels. Stromal tissue and a good blood supply are essential to the tumor's continued growth.3,' As the
From the Department of Ophthalmology, Uniuersity of Texus Health Science Center, San Antonio, Texas. Address correspondence and reprint requests to: Kenneth L Piest, MD, Department of Ophthalmology, Uniu. of Texus Health Science Center, 7703 Floyd Curl Dr., San Antonio, Tx 78284-6230.
1056
malignancies was performed. summary of the current treatment of the three most common eyelid carcinomas is discussed. CONCLUSION. With early recognition and appropriate management of eyelid malignancies, an extremely favorable prognosis can be obtained. The management is twofold and consists of curing the patient of the malignancy and then restoring the form andfunction of the eyelid in order to save the eye if possible. J Dermatol Surg Oncol1992;18:10561059. RESULTS. A
tumor enlarges, central tumor necrosis may occur as growth surpasses the nutrient supply, producing a central ulcer in the nodule (Figure 1).Other clinical variations are possible. All lesions do not necessarily have elevated margins. The aggressive morpheaform variant may present as an indurated sclerotic plaque with ill-defined borders. Melanin may be present, producing varying degrees of tumor pigmentation. Numerous subtypes of BCC have been described. These can be simplified into four basic types: nodular, ulcerative, morpheaform (sclerosing), and multicentric? The nodular form is the "classic" BCC. Growth of the tumor produces a localized mass. Production of an angiogenic factor produces the characteristic superficial telangiectaticvessels? A pseudocapsule is formed as the tumor grows and compresses the adjacent dermal tissues. Histologically, the tumor nodule is composed of a uniform population of basaloid cells with the peripheral cells demonstrating a palisading pattern. Small to large cysts may form within the tumor nodule, which may make the diagnosis more dif&mlt. If surface ulceration o c m , the pseudocapsule usually is not present. Instead, a chronic dermal inflammatory reaction is seen. In the morpheaform or sclerosing type of BCC, elongated strands of basoloid cells are surrounded by a dense fibrous stroma. This appearance resembles scirrhous carcinoma of the breast. Morpheaform BCCs are aggressive and deeply infiltrate adjacent tissue. The multicentric form has a more irregular surface than the nodule type. As the name implies, this type of BCC exhibits a diffuse multicentric involvement of the epidermis and superficial dermis. 0 1992 by Elsevier Science Publishing Co., Inc. 0148-0812/92/$5.00
J Denatol Surg Oncol 1992;18:1056- 1059
PEST 1057 MALIGNANT LESIONS O F THE EYELID
In cases where orbital extension has occurred, exenteration is the recommended treatment. If the disease involves the regional lymph nodes, surgery with radiation should be considered. Distant spread is obviously associated with a poor prognosis, but chemotherapeutic treatments are available in these s i t u a t i ~ n s . ~ ~ J ~ After treating a patient for BCC, regular follow-up exams are imperative. Most recurrences occur within 5 years, but late recurrences are A new BCC may also arise and 20 to 30% of these appear within 1 year of the original lesion. All patients at risk of developing skin carcinomasshould avoid excessive sun exposure and use sun screens with a sun protective factor greater than a rating of 15 whenever possible. Figure 1. Basal cell carcinoma of the right lower eyelid. Note the large, elevated nodular appearance with central ulceration.
Squamous Cell Carcinoma
The goal in management of a BCC is the complete elimination of the lesion. In the nodular form, surgical excision of a small lesion with histologic confirmation that the margins are free of tumor is appropriate. However, the ulcerative, morpheaform, and the multicentric types often extend beyond the area of apparent clinical involvement. Tumor excision with frozen section examination of all margins including the tumor base or Mohs micrographic surgery are the best available methods for complete tumor excision when these tumor subtypes are or a tumor recurrence is encountered.7-l0 However, even with the best techniques, evaluation of the margins may be quite difficult, especially in the morpheaform type or in a recurrence within scar tissue. Cryotherapy may be appropriate in situations where multiple tumors are present, excision is not possible, or both. With cryotherapy a double freeze-thaw method with tissue temperatures to -50' C is needed to destroy the tumor cells." Metastasis from BCC is infrequent with incident estimates ranging from 0.028 to l.0%.'213 When it does occur, lymphatic spread to the regional lymph nodes and hematogenous spread to bone, lungs, and liver are the most common sites. Despite their low metastatic rate, BCCs are locally invasive. Orbital involvement with subsequent intracranial extension is the most common cause of death due to tumor spread." Disseminated disease from BCC is rare, but if suspected a systemic workup is required. This includes a detailed history, physical examination with particular attention to the regional lymph nodes, and testing consisting of a complete blood count, liver profile, chest x-ray examination, bone scan, liver scan, and computed tomography scan if orbital or intracranial extension is suspected.
Squamous cell carcinoma is a malignant tumor of the c e k of the epidermis. Less than 5% of the epithelial neoplasms of the eyelids are S C C S . ~ Squamous ~ cell carcinoma most commonly involves the lower eyelid, but may occur in any location. When the tumor involves the upper lid and lateral canthal location, the SCC is more common than the BCC. It is thought to arise from the same factors that promote the development of BCCs (actinic damage, fair complexioned individuals, etc). Genetic conditions such as xeroderma pigmentosa and albinism are well known conditions with an increased risk of developing SCC. Immune suppression may also increase the risk of developing SCC; the lesions primarily occur in sun exposed areas.- Previous radiation treatment is also another risk factor. Squamous cell carcinomas usually arise in actinically damaged skin. They usually present as an elevated, indurated plaque or nodule with irregular borders. As the tumor enlarges, ulceration may occur, which is often covered by crusting debris (Figure 2). The same treatment considerations for BCC apply to the treatment of SCC. These lesions should be completely excised. Usually excision under frozen section examination of the margins or Mohs surgery is recommended, especialIy if the lesion is a recurrence. Metastasis from eyelid lesions detected early is rare (1.0%). This is in contrast to SCCs located in other anatomic sites, which may metastasizeearly and can be fatal. Advanced or neglected eyelid lesions do have the potential to spread both by local invasion and by metastasis. Dissemination to regional lymph nodes is more common than spread to distant organs." Furthermore, direct perineural invasion into the central nervous system can occur, resulting in death. If local extension or orbital involvement is suspected, imaging studies should be obtained. Invasive SCC is potentially fatal, but early eyelid le-
1058 PEST
Figure 2. Recurrent squamous cell carcinoma. Squamous cell carcinoma of the left eyelid and lateral canthal area. Keratin is seen on the surface of the lesion. (Courtesy of Charles R Leone, MD.)
sions treated appropriately have an excellent prognosis. The same follow-up and preventative measures as outlined previously for BCC apply even more strongly for SCC.
Sebaceous Cell Carcinomas Sebaceouscell carcinoma of the eyelid accounts for 2% to 5% of the epithelial eyelid malignancies. They arise from the meibomian glands and Zeis glands of the eyelid or from sebaceous glands in the caruncle or brow (see the section on anatomy in the article by Drs. Dailey and Wobig-). The upper lid contains approximatelytwice the number of meibomian glands as does the lower lid, which likely accounts for the fact that the upper lid is involved twice as frequently as the lower lid. Outside of the ocular area, sebaceouscell carcinoma is an extremely rare tumor. Sebaceous cell carcinomas are perhaps the most dangerous eyelid tumors because they can present with such a broad sDectrum of clinical Dresentations. Thev frequently mimic inflammatory eyelid conditions, which often can confuse and delay the true diagnosis. The term "masquerade syndrome" has been used to describe this situation. Common diagnoses masqueraded by sebaceous cell carcinomas include atypical or recurring chalazia, unilateral blepharoconjunctivit, or papillomas. The true diagnosis can be obscured until systemic metastasis occurs, resulting in discovery of the primary tumor. Most commonly a sebaceous cell carcinoma presents as a firm nodule resembling a chalazion (Figure 3). However, others may have a more diffuse thickening of the tarsus or a papillomatous growth pattern. As the lesion involves the lid margin, loss of eyelashes occurs as the 1
J D e n a t o l Surg Oncol 2 992;2 8:Z 056- 2059 tumor invades the lash follicles. Ulceration may also develop. Sebaceous cell carcinomas tend to invade the overlying forming scattered nests of malignant cells (pagetoid invasion) or completely replacing the epithelium (intraepithelial carcinoma). This may give the clinical appearance of a unilateral blepharoconjunctivitk that should immediately alert the clinician because a true blephraroconjunctivit is most commonly a bilateral condition. Another extremely important feature of this type of carcinoma is its tendency to have multicentricsites of origin. Independent foci of tumor cells, which can not only involve multiple sites on the same lid, but can also involve the upper and lower lid simultaneously, are reported 6 to 10% of the time.27This makes the task of complete excision of the lesion extremely difficult. Treatment of sebaceous cell carcinomasof the eyelid is by full-thickness excision with wide margins and with frozen section pathologic control. However, the recurrence rate after resection is approximately 30'3".28If the bulbar conjunctivais involved, cryotherapymay be helpful in attempts to avoid loss of the globe. If the tumor has extended into the orbit, exenteration is the recommended treatment. In addition to local invasion, sebaceous cell carcinoma can spread to regional lymph nodes and produce distant metastases. Regional metastases occur in up to 25% of patients. Vascular invasion can produce distant metastasis with the primary distant metastatic sites being lung, liver, brain, and skull. However, if distant metastases occur, they are almost always associated with a history of involvement of regional lymph nodes. Thus,one should always examine the patient with a suspected malignant eyelid lesion for palpable regional nodes. The overall mortality from this tumor is estimated to be Figure 3. Sebaceous cell carcinoma of the left lower eyelid. A small to medium sized lesion is noted at the lid margin in the medial 3 of the lower lid. (Courtesy of Charles R Leone, MD.)
J Dermatol Surg Oncol 1992;18:1056- 1059
20%. However, with the current higher index of suspicion and earlier diagnosis, the mortality rate is decreas-
ing.= Any suspicious lesion should be biopsied. Atypical chalazia should be treated appropriately with expressed debris or a biopsy submitted for pathologic evaluation. Any unilateral blepharoconjunctivit, especially if chronic, should be suspected and scraped with cytologic examination performed. If suspiciouscells are found, a lid biopsy should be performed to obtain the diagnosis. Carcinomas of the eyelid are by far the most common malignant entities involving the ocular adnexal area, but virtually any other soft tissue or bony neoplasm can occur in or around the eyelid, including malignant melanomas and metastatic lesions. Any treatment should be primarily based on early recognition, early diagnosis (biopsy), and complete excision of the lesion so as to give the patient the best prognosis. Reconstructive efforts are then undertaken to restore both the form and function of the eyelid in order to protect the globe.
Acknowledgment. This work was supported in part by an unrestricted grant from Research to Prevent Blindness, lnc.
References 1. Miller SJ.Biology of basal cell carcinoma (part I). J Am Acad Dermatol 1991;24:1-13. 2. Miller SJ. Biology of basal cell carcinoma (part 11). J Am Acad Dermatol 1991;24:161-75. 3. Lyles TW,Freeman RG, Knox JM. Transplantation of basal cell epitheliomas. J Invest Dennatol1960;34:353. 4. Van Scott EJ, Reinertson RP. Modulating influence of stromal environment of epithelial cells studied in human autotransplants. J Invest D m t O l 1961;36:109-31. 5. Sexton M, Jones DB, Maloney ME.Histologic pattern analysis of basal cell carcinoma. Study of a series of 1039 consecutive neoplasms. J Am Acad Dermatol 1990;23:111826. 6. Wolf JE,Hubler WR.Tumor angiogenic factor and human skin tumors. Arch Dermatol1975;111:321-27. 7. Domes RN, Walker NP,Collin JR. Micrograph (MOW) surgery in the management of periocular basal cell epitheliomas. Eye 1990;4:160-8. 8. Monheit GD, Callahan MA, Callahan A. Mohs micrographic surgery for periorbital skin cancer. Dermatol Clin 1989;7677-97. 9. Frank HJ. Frozen section control of excision of eyelid basal cell carcinomas: 8f years experience. Br J Ophthalmol 1989;73:328- 32. 10. Doxanas MT, Green WR,Iliff CE. Factors in the successful surgical management of basal cell carcinoma of the eyelid. Am J Ophthalmol 1981;91:726-36.
PIEST 1059 MALIGNANT LESIONS OF THE EYELID
11. G u n n m n G, Lark0 0,Hersle K. Cryosurgeryof the eyelid for basal cell carcinoma. Ada Ophthalmol (Copenh) 1990;68:241-5. 12. Farmer ER, Helwig EB. Metastatic basal cell carcinoma: a clinicopathological study of seventeen cases. Cancer 1980;46:748-57. 13. Lo JS, Snow SN, Reizner CT,Mohs FE, Larson PO, Hruza GJ. Metastaticbasal cell carcinoma: report of 12caseswith a review of the literature. J Am Acad Dermatol 1991;24: 715-9. 14. Glover AT, Grove AS. Orbital invasion by malignant eyelid tumors. Ophthalmic P l a t R ~ o n ~Surg t r 1989;S:l-12. 15. Morley M, Finger PT, Perlin M, Weiselberg LR, DeBlasio DS. Cis-platinum chemotherapy for ocular basal cell carcinoma. Br J Ophthalmol1991;75:407-10. 16. Baxter DL, JoyceAP,Feldman BD, Lynch JW.Cis-platinum chemotherapy for basal cell carcinoma: the need for posttreatment biopsy-report of a case. J Am Acad Derm 1990;6:1 167- 8. 17. Robinson JK. What are adequate treatment and following care for nonmelanotic cutaneous cancer. Arch Dermatol 1987;123:331-3. 18. Robinson JK. Risk of developing another basal cell caranoma: a five year prospective study. Cancer 1987;60:11820. 19. Epstein E. Value of follow-up after treatment of basal cell carcinoma. Arch Dermatol1987;108:798-802. 20. Schrieber MM,Moon TE,Fox SH, Davidson J. The risk of developing subsequent nonmelanotic skin cancer. J Am Acad Dermatol 1990;23:1114-8. 21. Kwitko ML, Boniuk M, Zimmerman LE. Eyelid tumors with reference to lesions confused with squamous cell carcinomas. Arch Ophthalmol 1963;69:693-7. 22. Lutzner MA. skin cancer in immunosuppressed organ transplant recipients. J Am AcadDermatol1984;11:891-3. 23. Rao NA, Dunn SA, Romero JL, Stout W. Bilateral carcinomas of the eyelid. Am J Ophthalmol1986;101:480-2. 24. Lund HZ.How often does squamous cell carcinoma of the skin metastasize. Arch Dermatol 1965;92:635- 7. 25. Dailey RA, Wobig JL. Eyelid anatomy. J Dennatol Surg On~011992;18:1023-7. 26. Font RL. Eyelids and lacrimal drainage system. In. Spencer WH,ed. Ophthalmic Pathology: An Atlas and Textbook, vol3. Philadelphia. WB Saunders, 1986. 27. Roa NA, Hidayat AA, Mckan IW,Z i m m m LE. Sebaceous gland carcinoma of the ocular adnexa: a clinicopathological study of 104 cases with five year follow-up data. Hum Pathol 1982;13:113-22. 28. Epstein GA, Puttennan AM. Sebaceousadenocarcinomaof the eyelid. Ophthalmic Surg 1983;14:935-40. 29. Char DH. Adult and pediatric lid tumor diagnosis. In:Char DH, ed. Clinical Ocular Oncology. New York Churchill Livingstone, 1989.
![[Review of 114 cases of malignant tumors of the eyelids treated surgically].](/assets/img/hold.png)
