Sir,

The primary signet ring cell carcinoma of the eyelid (PSCE) is an extremely rare tumor. Only a few cases have been reported in the peer-reviewed literature.1, 2, 3, 4 Nearly all of the 23 patients reported so far were healthy middle-aged or elderly males.2 In our clinic, however, we observed a PSCE in a young woman.

Case report

The 33-year old patient presented had an indolent swelling of the left eyelid (Figure 1a), which she had been noticing for 4 months. The skin surface and conjunctiva over the lesion were normal. The tumor was removed in the operating theater assuming that the patient was suffering from a chalazion. The removed tissue was routinely sent to the pathology department. Microscopic examination detected numerous tumor cells with accumulation of PAS-positive intracellular mucoid material and marginalized hyperchromatic nuclei (Figure 1b). These signet ring cells showed immunohistochemical positivity for cytokeratins 7 (Figure 1c) and 5/6, whereas the reaction with antibodies against cytokeratin 20 was negative. The immunoreactions with antibodies directed against the estrogen receptor α (ER) and progesterone receptor (PR) were both negative (data not shown). This reaction pattern was compatible with the differential diagnosis of a metastatic gastric signet ring cell carcinoma or a primary signet ring cell carcinoma of the periorbital region. The negativity for ER and PR made the diagnosis of a metastatic lobular breast carcinoma unlikely.

Figure 1
figure 1

(a) Indolent tumor of the left upper eyelid. (b) Signet ring cell carcinoma with PAS-positive intracellular mucoid material ( × 200). (c) Signet ring cell carcinoma with strong cytokeratin 7 immunoreaction ( × 200).

Full size image

Periorbital metastases from primary infiltrating carcinomas elsewhere have been frequently reported.5 Because PSCE is a diagnosis of exclusion, a thorough work-up is crucial to exclude extracutaneous metastasizing signet ring cell carcinoma, especially from the gastrointestinal tract, breast (lobular subtype), and urinary bladder.2 Clinical and radiological examinations (computer tomography and magnetic resonance imaging, gynecological examination, mammography, colonoscopy, and gastroduodenal endoscopy) did neither reveal a metastatic malignant breast carcinoma nor malignancies of the abdomen. Furthermore, metastases of the PSCE could be excluded.

Because a radical excision with wide margins is essential in case of PSCE, a second surgery was performed to remove tissue of the tumor, which might have remained. The deep excision included conjunctival, tarsal, subtarsal, and muscular tissue. The defect was covered with amniotic membrane. In the histological work-up, the margins of the specimen were tumor-free and no residual malignant cells were detectable. In more advanced stages, orbital exenteration, radiation therapy, and adjuvant chemotherapy should be considered.2

We have followed the patient in an interdisciplinary follow-up program for carcinoma patients for 1 year so far. There have been neither signs of a local recurrence nor of metastases until now.

Comment

Our case demonstrates the importance of a meticulous histological work-up, even when a lesion does not appear to be suspicious.