Case of primary neuroendocrine carcinoma in lacrimal gland

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Abstract

Purpose

Case of primary neuroendocrine carcinoma in lacrimal gland

Methods

A 86-year-old man noticed a swelling of the left upper eyelid three month earlier. Because the size of the nodule gradually increased, the patient was referred to the Chiba University Hospital. We performed excision biopsy and histopathological examination indicated that the tissue had low-grade atypical cells which were positive to CD56, CK7, and CKAE1/AE3. Magnetic resonance imaging and positron emission tomography excluded systemic metastasis. The patient was diagnosed with a primary NEC of the left lacrimal gland.

Results

He underwent chemotherapy (Carboplatin and etoposide) for four cycles, and the size of the tumor was significantly decreased. A month later, we performed tumor excision including the clinically visible margin, and a month later the patient had 50Gy radiotherapy at the surgical region. After the three-combination therapy, the tumor was undetectable in the MRI. A month later, however, a metastasis to the parotid lymph node was found, and the patient underwent total lymphadenectomy of the left parotid gland and 46Gy radiotherapy was applied to the left neck. At the last examination, the patient had neither recurrence nor metastasis.

Conclusion

Although primary NEC of the lacrimal gland is extremely rare, the tumor has high malignancy and readily metastasizes. Thus, combined surgery, radiotherapy, and chemotherapy are needed for the complete management of NEC.

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