30 Malignant Tumors
MALIGNANT MELANOMA
Malignant melanoma (90–99) may arise from the cutaneous aspect or the conjunctival aspect of the eyelid. Since cutaneous malignant melanoma behaves differently from the conjunctival variety, the tumors will be discussed separately.
The principal types of cutaneous melanoma are lentigo maligna melanoma (Hutchinson’s melanotic freckle with malignant melanoma), superficial spreading melanoma (free malignant melanosis with malignant melanoma or pagetoid melanoma), acral lentiginous melanoma, and nodular melanoma. Acral lentiginous melanomas appear on the soles, palms, or sublingual area, and usually do not involve the eyelids. These skin melanomas are not usually classified according to the cell types, as are uveal melanomas. However, there is a report of a spindle-cell melanoma of the eyelid with orbital extension.52 Lentigo maligna melanoma develops in a preinvasive lesion: lentigo maligna (91–94). There is a radial growth phase which may exist for many years. but then a vertical growth phase (dermal invasion) may occur. At this point, the lesion becomes elevated. Superficial spreading malignant melanoma also has a prolonged radial growth phase before any vertical growth occurs. Clinically, this lesion appears as a nodule or plaque with variable pigmentation. Nodular malignant melanomas occur most frequently in the fifth decade and are twice as common in men. This melanoma has only a vertical growth phase with early involvement of the dermis and, therefore, has the worst prognosis among the cutaneous melanomas (95).53
The risk of metastasis is related to the level of invasion of the dermis and the tumor thickness. There are five Clark levels of invasion. Level 1 involves only the epidermis. As the tumor extends deeper into the dermis, it is classified as levels 2, 3, and 4. Level 5 invades the subcutaneous tissue. Breslow thickness is also used for determining prognosis. The tumor is measured in histologic sections from the epi-
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dermis into the dermis. Tumors classified as Clark level 1 or <1 mm thick have a very good prognosis. As the tumor extends below the epidermis, the prognosis worsens as each new Clark level or increased thickness is reached.54, 55
TREATMENT
Surgical treatment is the most efficacious method of eliminating malignant melanoma. Since lentigo melanoma rarely metastasizes, treatment by wide local excision is usually sufficient. However, since the eyelid is small compared to the rest of the body, the term ‘wide’ needs definition. A margin 5 mm (0.2 in) should be taken for flat tumors contained with the epidermis. This might include lid margin and other important structures such the canaliculi. Frozen section control is not appropriate for evaluating margins for melanoma. Therefore, a wide margin is necessary since re-excision will have to be done if margins are not free of tumor when permanent sections are examined.
Superficial spreading melanoma and nodular melanoma should be widely excised, and local excision of lymph nodes is often recommended. Systemic evaluation for the possibility of metastases should always be performed, since the presence of metastasis elsewhere in the body may affect the decision regarding surgical treatment of the eyelid lesions.
COMMENT
As with many malignancies, the host’s immune system affects the rapidity and extensiveness of malignant spread. A patient presented to the author with nodular melanoma of the eyelids with extension onto the conjunctiva in which the tumor remained localized in these areas. Periodically, the tumor nodules were removed. There has been no evidence of spread of this melanoma to the orbit or deeper ocular tissues, or of any metastasis (96). The patient was alive and well with no evidence of metastasis at the time the first edition of this book was written, 14 years after the recognition of the eyelid tumors.
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90, 91 Early stages of malignant melanoma. 90: Melanocytic hyperplasia. Small pigmented lesion in a 37-year-old female. Clinical diagnosis was lentigo, but histological examination showed melanosis with a focus of severe atypical melanocytic hyperplasia.This type of lesion might progress to a malignant melanoma. 91: Lentigo maligna in the left lower lid of a 52-year-old male. He had gone to his primary care physician for five years and was told that this flat, pigmented area was benign.The patient was suspicious of cancer because the lesion was getting larger. Eventually he went to an ophthalmologist who did a biopsy and found malignant melanoma in situ.The lesion was flat and very superficial in the skin.The entire tumor was removed and 80% of the lower lid was reconstructed.
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92–94 Lentigo maligna. 92: A 60-year-old female with lentigo maligna of the lower lid. 93:The entire lower eyelid from canthus to canthus was replaced in order to get free margins. 94: Same patient three months following surgery using a modified Hughes procedure with a skin graft (see Chapter 8: Lower Eyelid Reconstruction).
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Malignant Melanoma 31
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95 Malignant melanoma in the left upper lid of an 89-year-old female. This lesion progressed from a 5-mm (0.2 in) mass in a period of nine months. Systemic evaluation revealed evidence of multiple metastases.
96 Malignant melanoma. Eighty-year-old male with a 14-year history of malignant melanoma of the eyelids treated symptomatically by local excisions.The patient refused exenteration.There was no evidence of metastatic spread during a 14-year follow-up.
32 Malignant Tumors
MALIGNANT MELANOMA OF THE CONJUNCTIVA
Malignant melanoma of the conjunctiva is an extremely rare disease. The lesion usually involves the bulbar conjunctiva, but it may be localized to the tarsal conjunctiva (97–99). These melanomas may arise de novo from malignant degeneration within a pre-existing benign nevus, or as a nodule arising as widespread or diffuse precancerous melanosis. Since melanotic lesions of the conjunctiva are usually benign, a biopsy is necessary to make the diagnosis. Fine needle aspiration has been shown to be effective in diagnosing conjunctival melanoma of the tarsal conjunctiva.56 If a true melanoma does exist and is localized, a full-thickness resection of the eyelid to remove the tumor and surrounding tissues is indicated. If the lesion is widespread along the conjunctiva and surgical resection does not seem appropriate, cryotherapy can be used to treat these lesions.57, 58 In a study of 255 conjunctival melanomas in 151 patients, 20 required eventual exenteration. Of these 20 cases, only one tumor arose in the tarsal conjunctiva. The others came from the bulbar of forniceal conjunctiva.59
SWEAT GLAND CARCINOMA
Carcinomas arising in sweat glands are rare skin tumors derived from eccrine or apocrine glands. The more common type is the mucinous variety which usually occurs in the sixth and seventh decades of life and may follow an indolent course for many years.60 The tumor usually presents as a firm, cystic nodule which may appear as a red tumefaction,61 or as a nontender nodule with normalappearing overlying skin (100).62 The tumors may occur in the upper lid, lower lid, or medial canthus and can invade the orbit and surrounding bone.63
Primary mucinous sweat gland carcinoma of the eyelid can also be histologically confused with other tumors, such as histiocytoid, histiocytoid carcinoma of the eyelids secondary to carcinoma of the breast, and granular cell myoblastoma.64 A very unusual type of sweat gland carcinoma is a malignant syringoma which usually appears on the upper lip. Histologically, malignant syringoma can be confused with the benign variety. The clinical appearance of the malignant syringoma does not distinguish it from many other subcutaneous lesions.65, 66 In two cases reported in young adults, the tumors appeared as subcutaneous nodules or indurations in the lower lid with essentially normal overlying skin.60
The most common sweat gland carcinoma of the eyelid is the mucin-secreting adenocarcinoma.60 Mucin secretion is considered evidence of cellular differentiation. Therefore, these tumors have a better prognosis with less potential for metastasis; however, sweat gland carcinomas can metastasize.63
Sweat gland carcinomas have a low metastatic potential but can spread to regional lymph nodes.62 One patient developed liver metastases more than 15 years after the initial lesion was removed from the eyelid.67 Since these tumors vary in appearance and clinical presentation, it is essential that a biopsy be performed.
Most of these carcinomas are resistant to radiation therapy;60 therefore, proper treatment is complete surgical excision. Evaluation for spread to the orbit or adjacent structures, as well as evaluation for metastases is indicated.
Mucoepidermoid carcinoma is a malignant epithelial neoplasm which rarely occurs on the eyelid. In the eye and ocular adnexa, it usually arises from conjunctiva, lacrimal sac, and lacrimal gland. These tumors are usually aggressive. However, an unusual case which seemed to arise from the sweat glands in the eyelid skin was reported to be less aggressive. It was clinically mistaken for a basal cell carcinoma.68
MERKEL CELL TUMOR
The Merkel cell tumor is a rare neoplasm composed of cells thought to be derived from the neural crest and normally found in the skin. The tumor is considered a low grade neoplasm with the ability to metastasize. Histologically, it can mimic undifferentiated small cell carcinoma from lung or other primary sites (101).
Although there have been very few reported cases, clinical appearance apparently differs among the tumors. One author described the tumors as hard, smooth-surfaced, and violaceous with overlying telangiectasis.69 Another report described a tumor measuring 35 × 35 × 30 mm (1.4 × 1.4 × 1.2 in). The lesion was nontender, and the skin was intact and smooth. This pedunculated tumor caused a mechanical ptosis.70 Based on a series of six patients, ophthalmologists in Germany concluded that the clinical characteristics of Merkel cell carcinoma should be diagnostic. They found them to be painless, reddish to violet, nodular, often protuberant lesions arising near the eyelid margin. The surfaces often had dilated telangiectatic blood vessels on their surfaces (11, 101).71
Treatment should consist of complete excision and evaluation for metastatic spread.
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97–99 Malignant melanoma of the eyelid conjunctiva. 97: Note the raised, brown lesion on the tarsal conjunctiva.This woman already had preauricular node involvement. 98:This 27-year-old male presented with a conjunctival lesion involving the lateral half of the left upper lid at the lid margin.The entire tumor was removed and 75% of the lid was reconstructed using the Cutler–Beard technique (see Chapter 9: Upper Eyelid Reconstruction). 99: Same patient nine years postoperative. At the time of this writing he is 11 years postoperative, without any evidence of recurrence or metastasis.
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100 Sweat gland carcinoma showing induration of the upper and lower lids and cheek. (Courtesy of Mark R. Levine, MD.)
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101 A 70-year-old male with a Merkel cell tumor of the left lower lid.