|
Indian Journal of Plastic Surgery, Vol. 41, No. 2, July-December, 2008, pp. 229-234 Brief Communication Adnexal carcinomas of the head and neck Guerrissi JorgeO, Quiroga JuanPablo Argerich Hospital, Buenos Aires Code Number: pl08057 Abstract Adnexal carcinomas of the skin are rare and they derive from structures such as sweat glands, sebaceous glands, and hair follicles. Adnexal tumors represent 1-2% of skin cancers. Between 1998 and 2004, eight patients with malignant adnexal tumors of the head and neck were treated in the Plastic Surgery Service in Argerich Hospital in Buenos Aires, Argentina. Four (50%) of them had malignant cylindromas, two (25%) had sebaceous carcinoma, and the other two (25%) syringoid eccrine carcinoma. Tumor resection and local flaps were made in all cases. In one case, a radical neck dissection with superficial parotidectomy was performed to treat the metastatic cervical nodes. Local recurrence observed in two cases (25%) was associated with distant metastasis and death of the patients. In other six cases, the survival rate was 75% after five years.Keywords: Adnexal carcinomas, adnexal tumors, skin cancer Introduction Melanoma skin cancers account for 4-7% of all skin cancers, the non melanoma tumours for 93-96%. Both basal cell and squamous cell carcinomas represent > 80% of non melanoma skin cancers whereas benign and malign adnexal tumours represent only 1-2%, including mesenchymal, fatty, and vascular tumours. Adnexal carcinomas of the skin derive from structures that have a common origin such as the apocrine and eccrine sweat glands, sebaceous glands, and hair follicles [Table - 1]. Malignant adnexal tumours are frequently located in the head and neck region but may appear on the fingers and toes, the trunk as well as the extremities. [1],[2],[3],[4] All ages may be affected and there is no easily identifiable risk group. Most adnexal carcinomas of the skin are highly malignant and difficult to diagnose clinically or even histologically. The objective of this presentation is to attract the attention of plastic surgeons because a reliable cure rate is associated with prompt recognition and aggressive treatment. Material and Methods Between 1998 and 2004, eight patients with adnexal carcinomas of the skin in the head and neck region were admitted to the Service of Plastic Surgery in the Argerich Hospital. Four (50%) of them were diagnosed with malignant cylindromas; two (25%) with sebaceous carcinoma, and the remaining two (25%) with syringoid eccrine carcinoma. The patients´ ages ranged from 26 to 68 years and the follow-up period was for five years. Local recurrence was observed in two cases (25%) with malignant cylindroma and syringomoid eccrine carcinoma. The treatment of choice was a wide excision of the tumour (> 1.5 cm) and local flaps for reconstruction in seven cases and a split-thickness skin graft in the other case. A radical neck dissection including superficial parotidectomy was done in one of the two cases of sebaceous carcinoma when metastatic cervical nodes were detected. Six patients (75%) were followed up for at least five years; two others (25%) died of local recurrence and distant metastasis within a mean follow-up period of 36 months [Table - 2]. In these two patients, chemotherapy and radiotherapy were used with very limited success. Major complications were not observed and only two cases presented with infection of the incision. Malignant cylindroma Sebaceous carcinoma Syringoid eccrine carcinoma Discussion The adnexal carcinomas of the skin are not frequent and they derive from the foetal epidermis. [16] Some have a predilection for certain specific locations such as syringomas of the cheek and nose; cylindromas of the scalp and face; sebaceous carcinoma in the eyelids, etc. Clinical diagnosis of most adnexal skin carcinomas is difficult not only between them but also with adnexal benign tumours. Factors suggesting malignancy include poor demarcation, tumour necrosis, ulcers, border, tumour infiltration, etc. Malignant cylindroma is a rare tumour derived from the eccrine sweat glands. [5] Its origin was formerly considered to be the apocrine sweat gland but is now thought to be due to a mixed aetiology. Only 14 cases are known in literature and they have all presented with aggressive local expansion, perforation of the skull, and metastasis in the cervical nodes. [6],[7],[8],[10],[11],[12] They appear frequently in women and young adults as nodules 1.2-6 cm in diameter; pink in colour and of firm consistency. They may appear in two forms: Solitary (74%), which is the most frequent form seen usually in the face, and multiple (26%) which is of dominant autonomic inheritance, the gene of the familiar cylindromatosis being located in the chromosome 16q12-q13 [13] Histology is characterized by the presence of multiple compact epithelial lobes. Two types of cells are described: the central cells with clear nuclei; and the peripheral ones with small and dark nuclei. [14] Differential diagnoses are necessary with other pathologies such as trichoepitheliomas, epitheliomas to basal cell nodules as also metastatic tumours, and trichofolliculomas. Carcinoma of the sebaceous cells is a very rare tumour. It occurs in women between 60 and 70 years of age and is preferentially located on the eyelids. This carcinoma may appear as a solitary, yellowish, and painless nodule. Histology shows very cellular bands or basophilic masses extended from the dermis to the subcutaneous weave. [15] Metastasis takes place first in the regional lymphatic nodes and then, in the viscera, these carcinomas can also invade the facial bones. A syringomoid eccrine carcinoma is also know as a microcystic adnexal carcinoma, malignant syringoma, or a sclerosing carcinoma of the eccrine sweat glands. It is pronounced in aged patients and is usually located on the scalp, face, trunk, and extremities. It is a solitary tumour presenting as a slow-growing nodule or plaque. It can appear with alopecia and the lesion may secrete fluid. The following characteristics are observed in the histological analysis: cellular atypia, nuclear hyperchromatism, deep invasion, and many tubulocystic proliferations at the level of the dermis. [15] In all our cases of adnexal skin carcinomas of the head and neck region, surgical excision was the treatment of choice, the excision and reconstruction being conducted simultaneously by the same team. In previous reports, adnexal carcinomas have been considered to be radioresistant although new techniques of radiotherapy have been revaluated as part of primary and adjuvant treatments. [17],[18] Radical neck dissection must be done when metastatic cervical nodes are detected. Conclusion
References
Copyright 2008 - Indian Journal of Plastic Surgery The following images related to this document are available:Photo images[pl08057f7.jpg] [pl08057f8.jpg] [pl08057f3.jpg] [pl08057t2.jpg] [pl08057f11.jpg] [pl08057f10.jpg] [pl08057f5.jpg] [pl08057f1.jpg] [pl08057t1.jpg] [pl08057f2.jpg] [pl08057f4.jpg] [pl08057f6.jpg] [pl08057f9.jpg] |
|