[Neoplastic pathology in maxillofacial region including the eye cavity].
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Ten cases of the basal cell naevus syndrome (BCNS) are reported. The study is based on clinical findings with no investigations beyond two skull radiographs. The diagnosis is made on some or all of the following findings: family history, jaw cysts, progressive development of basal cell carcinomas (BCCs), pitting of the palms and soles, frontal bossing, abnormality of the skeletal system and ectopic calcification. Eight of the cases are in one family covering three generations. With the exception of a one-year-old child in the third generation (not included) all of this group have the syndrome. The dominant trait is demonstrated, as is the high degree of expressivity and penetrance. Genetic counselling is essential where applicable. Sympathetic regular clinical examination over a lifetime is recommended to avoid disastrous complications.
Two patients had distinctive adnexal skin tumors that showed cellular differentiation toward the formation of more than one adnexal structure. In one patient, the tumor showed differentiation toward pilar and sweat ductal structures. In the second patient, tumor differentiation toward the formation of sebaceous glands, pilar, and sweat ductal structures was found. We propose the term "combined adnexal tumor of the skin" for this neoplasm.
A case of verrucous carcinoma occurred on the face of a patient and prompted a review of the literature on this disorder. It is difficult to make a histopathologic diagnosis in such cases.
Curettage and electrodesiccation, the customary treatment for primary basal and squamous cell carcinomas, lacks histologic control and standardization. A review of the literature cites these factors as potential causes for undesirable recurrence rates, especially in cosmetically sensitive areas. Curettage and shave excision offers histologic control, yet with no more sacrifice of normal tissue than curettage and electrodesiccation. An experimental study with 100 patients is described in which curettage and shave excision was used, instead of curettage and electrodesiccation, to treat primary carcinomas 1.0 cm or less. It is suggested that curettage and shave excision, while simple in concept, requires formal training and warrants consideration for inclusion into graduate training programs as an alternative effective treatment for primary carcinomas.
The epithelial component of a syringoma is usually composed of cuboidal cells that have a moderate amount of eosinophilic cytoplasm in tissue sections stained with hematoxylin and eosin. Some syringomas have variable numbers of larger epithelial cells that have pale or clear cystoplasm. Such lesions have been called clear-cell syringomas. Examination of 18 cases by conventional microscopy revealed that their general organization is the same as ordinary syringomas but that the epithelial component constitutes a greater proportion of neoplastic volume. Histochemical studies confirmed that the clear cells contain abundant glycogen. The cells near lumina also contained a mucous substance that had a staining profile consistent with sialomucin. Examination by electron microscopy of two lesions composed entirely of clear cells revealed numerous multivesicular bodies, some in large aggregates, in periluminal cells. All cells contained numerous glycogen granules. Lesions in which there were incomplete changes in clear cells tended to have cells predominantly around the lumina. These observations suggest that the majority of clear cells result from accumulation of glycogen. The periluminal cells may be clear owing to the large number of multivesicular bodies and storage of sialomucin in them.
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The diagnosis of basal cell epitheliomas of the face is usually made at an early stage (lesions of less than one centimetre in diameter) in France. Because of their good prognosis, without metastatic risk (95% recovery rate), therapy can be chosen as a function of its cost and the esthetic result obtained. The author considers that surgery, endocurietherapy with iridium, and plesioradiotherapy are equally effective but should be chosen according to the extent and location of the lesion. The ear, and to a lesser degree the nose, are the major indications for curietherapy. For the cyclids and flat surfaces (temporal or frontal regions etc...) surgery, and plesioradiotherapy are the treatment of choice.
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The early detection and referral of a small basal cell carcinoma of the face will prevent extensive surgical excision and repair necessary for a neglected lesion. Since these tumors occur in the older population, careful examination of the skin should be performed on patients 50 years of age and older. This cancer has been estimated to constitute 90% of all malignant lid tumors and a suspicious lesion on the lid should be considered a basal cell carcinoma until proven otherwise. The lesion has a very characteristic appearance as described in the text; however, they can present with an atypical picture. An important sign in the evaluation of a possible malignant lid tumor is the loss of eyelashes within or adjacent to the lesion, which indicates the destructive nature of the tumor to the hair follicles. Basal cell carcinomas do not metastasize to distant locations in the body but rather extend locally and can produce deep tissue invasion. Differential diagnosis can present problems to the clinician but the definitive diagnosis is dependent upon the pathologist. The treatment usually requires surgical removal with wide excisional margins; however, other forms of treatment have been attempted. Recurrences of basal cell carcinomas are not uncommon, especially following an initial recurrence and it is mandatory that these patients be re-examined at least every six months for a two year follow-up period.