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Editor,—The signet ring cell carcinoma of the eccrine sweat gland is a very rare tumour of the eyelid. Only six cases have been published up to now. There is a preponderance of males; only one woman was affected. The patients' ages ranged from 47 to 78 years. This tumour shares some histological features with breast carcinoma, the metastasis of which represents the most important differential diagnosis—Indian file formations, signet ring cells, and expression of oestrogen, as well as progesterone, receptors. One significant difference is that the positive staining for those hormone receptors is found mainly in the cytoplasm in signet ring cell carcinoma, in contrast with the nuclear staining of breast carcinomas cells.
Different treatment modalities have been applied in cases of eccrine sweat gland carcinoma. Our case demonstrates that tumour control can be achieved with radiotherapy alone despite extensive orbital involvement.
An 87 year old male patient was seen initially in our department in July 1998. He had noticed swelling of his right lower eyelid. We saw a diffuse thickening and induration of the right eyelids at initial presentation in our hospital. An magnetic resonance image (MRI) showed a tumour that had infiltrated the upper (Fig 1) as well as the lower eyelid, the eyebrow, and the perioral muscles. The optic nerve was also surrounded by tumour mass. Two biopsies revealed an infiltrating tumour with Indian file formations; some of the tumour cells had a signet ring appearance with nuclei located peripherally as a result of intracytoplasmic vacuoles. The latter possessed microvilli, as could be demonstrated by electron microscopy. The cytoplasm stained positive with periodic acid Schiff (Fig 2) and with antibodies against oestrogen and progesterone receptors as well as human milk fat globulin. No hormone receptor expression was found in the nuclei. The growth fraction was 5%, determined with MIB1.
Systemic examination did not reveal any other tumour, especially no breast carcinoma and no adenocarcinoma of the gastrointestinal tract. Two treatment modalities were discussed—orbital exenteration and radiotherapy. Since the patient refused orbital exenteration radiotherapy was started in September and October 1998, and performed over 6 weeks, with a total dosage of 56 Gy. Two months later the cornea showed erosions which were treated with lubricants; the ocular motility was heavily impaired. Fourteen months after radiotherapy the lid skin was soft again without any evidence of tumour recurrence, the cornea only showed irregular epithelium, and the eye motility had returned to almost normal. Unfortunately, because of optic nerve damage by glaucoma and radiotherapy, the right eye went blind.
In most cases of signet ring cell carcinoma described in the literature, upper and lower lids of one eye were involved. Three patients were initially treated by excision alone; all of them had a period of survival of at least 6 years. One of these patients developed a recurrence after 10 years, with preauricular lymph node metastasis, as a result of which he was treated with radiotherapy (50 Gy). He had at least 18 months of remission thereafter, but showed subclinical liver metastasis at necropsy; his death was not tumour related.Another patient was found to have pulmonary metastases from which he finally died.
Radiotherapy alone (35 Gy), as in our case, was applied in another patient. He showed infiltration of the lids and the anterior orbit, and had at least 6 years of remission.The only patient treated by orbital exenteration, radiotherapy, and tamoxifen died as a result of liver and bone metastases within a period of less than 6 years after initial diagnosis. His pretreatment status, showing extensive orbital infiltration, was quite similar to that of the patient we are presenting here.
From the cases reported in the literature, we conclude that this tumour possesses a low to intermediate grade of malignancy. To date, 14 months after radiotherapy, our patient still shows remission. Thus, for elderly patients with extensive infiltration of orbital and adnexal tissue by an eccrine sweat gland carcinoma, we consider this conservative treatment sufficient to achieve local control.
We thank Dr T Rudolphi for providing clinical data, Dr T Wesendahl for contributing histological sections, and Professor Dr H Witschel and Dr J P Alexander for reading the manuscript.