20 Malignant Tumors

BASAL CELL NEVUS SYNDROME

Basal cell nevus syndrome is a rare autosomal dominant dermatosis. It is associated with multiple organ system abnormalities including jaw cysts, congenital skeletal anomalies, and pits of the hands and feet. The cutaneous tumors may be few or hundreds, have an early onset, and on microscopic examination be indistinguishable from basal cell carcinomas. They are frequently invasive, destructive, and aggressive with resultant disfigurement.26 Although surgical excision with microscopic evaluation of the margins is the best technique for removing these tumors, the surgery itself may cause disfigurement and become difficult because of lack of tissue available for multiple reconstructions. Nonsurgical techniques, such as cryotherapy, can be useful for these lesions.

Hematoporphyran derivative (HpD) has been reported to be efficacious in controlling basal cell carcinomas within the basal cell nevus syndrome. HpD is a photodynamically active dye that is preferentially retained by malignant tissues and initiates a cytotoxic reaction when exposed to red light. Normal tissues adjacent to a tumor retain HpD to a lesser degree and undergo no significant damage from the lightinduced reaction.27 HpD use is considered experimental, but early reports show some promise for its use in patients where surgical treatment of the tumors is inappropriate.

SQUAMOUS CELL CARCINOMA

Squamous cell carcinoma is the second most common eyelid malignancy, and yet it occurs with a frequency 2.5–10% that of basal cell carcinoma,28 although the incidence varies in different reports. From a practical point of view, squamous cell carcinoma and sebaceous cell carcinoma occur with a frequency <10% that of basal cell carcinoma in the western hemisphere (Chapter 4 Histopathology). The tumor presents with various degrees of malignancy which, in fact, may be a continuum from the premalignant situation of actinic keratosis to invasive squamous cell carcinoma.

The less invasive presentation is intraepidermal squamous cell carcinoma (squamous cell carcinoma in situ). This often presents as a telangiectatic flat area. It may arise from a precancerous keratosis, such as actinic keratosis, or it may arise de novo (54–61). Intradermal squamous cell carcinoma may progress to invasive carcinoma.

Bowen’s disease is a clinicopathological entity consisting of squamous cell carcinoma in situ in which an erythematous, sharply demarcated, scaly patch grows slowly and spreads in a superficial centrifugal manner forming irregular serpiginous borders. The lesion may remain stationary for many years, or it may progress. Bowen’s disease is associated with other skin tumors, both malignant and premalignant, in up to 50% of patients and with internal cancer in up to 80% of patients.29

54

55

54, 55 Appearance of squamous cell carcinoma on the eyelid skin. 54: Squamous cell carcinoma in situ in the medial aspect of the right upper lid in a 74-year-old male. 55: Squamous cell carcinoma in situ involving the entire upper eyelid of a 78-year-old male.

56–58 Appearance of squamous cell carcinoma on the eyelid skin. 56: Squamous cell carcinoma in situ involving the skin of the entire right upper lid of a 60-year-old male. 57:The extent of the defect necessitated frozen section evaluation of the margins to insure the entire tumor was removed. 58:The appearance of the upper lid two and one-half months following repair with a retroauricular skin graft.

60. 61Appearance of squamous cell carcinoma on the eyelid skin. 60: An 80-year-old male with keratotic lesion in the left medial canthus. Diagnosis was squamous cell carcinoma – keratoacanthoma type. 61: Defect remaining after

the entire tumor was removed based on frozen section analysis.

59 Appearance of squamous cell carcinoma on the eyelid skin. Keratoacanthoma in the lower eyelid. A well circumscribed lesion with keratin center. (Photo taken after fluorescein instilled in the eye.)

22 Malignant Tumors

When present on the tarsal conjunctiva, squamous cell carcinoma in situ may be papillomatous or look like a velvety layer over the tarsal conjunctiva (62–65). Even if the diagnosis of squamous cell carcinoma in situ is made histologically, it is usually prudent to take a biopsy including tarsus and surrounding structures, since invasive squamous cell carcinoma may be present in the surrounding structures when in situ carcinoma is diagnosed on the tarsal conjunctiva (62). Unlike the basal cell carcinoma which rarely arises on the conjunctival surface, squamous cell carcinoma is the most common epithelial malignancy of the conjunctiva.

Squamous cell carcinoma, like basal cell carcinoma, presents in various ways (66–68). When it presents on the skin surface, it may be raised with an eroded center or it may simply present as a flat, ulcerated lesion. It may be confused with a keratoacanthoma30 but the keratoacanthoma usually has a more rapid onset. Usually, it appears in areas which have been exposed to sunlight or X- rays. The metastatic rate is low, but when metastasis occurs, it is usually by way of the lymphatic system. However, metastases such as intracranial dissemination along the trigeminal nerve have been reported.31

62

62 Squamous cell carcinoma involving the conjunctiva. A 79-year-old female with a papillomatous lesion on the conjunctival surface of the lateral canthal angle.There was also a velvety appearance to the adjacent tarsal conjunctiva.The entire suspicious area was removed and was read as squamous cell carcinoma in situ. Six months later, there appeared to be further involvement of the conjunctiva showing this same velvety appearance. A full-thickness section of eyelid was removed for pathological evaluation. It showed carcinoma in situ associated with deeply invasive squamous cell carcinoma.

63

63 Slightly raised, smooth spreading lesion involving the conjunctiva of the upper lid.This is squamous cell carcinoma spreading across the conjunctiva.This type usually begins as the in situ variety but, if not treated, can become invasive.

24 Malignant Tumors

Squamous cell carcinoma may occur in association with basal cell carcinoma, and in some cases the tumor may have histological elements of both squamous and basal cell

carcinoma. There is some evidence that both basal and squamous cell carcinomas are derived from the same pluripotential epidermal or adnexal epithelial cells (69–74).32