Abstract Listings 2024

Purpose

Primary mucinous adenocarcinomas (PMA) of the periorbital region represent a rare condition, often initially misdiagnosed as benign or metastatic due to their clinical presentation. Histopathological and immunohistochemical examinations, along with tumor staging, are thus essential for an accurate diagnosis. We present an attempt at neoadjuvant tamoxifen therapy for a PMA of the orbit with a positive hormone receptor status.

Methods

A 67-year-old male patient presented after external excision of a suspected hidrocystoma recurrence on the right lower eyelid. Intraoperatively, the lesion appeared to extend into the upper eyelid. The histopathological and immunohistochemical examination revealed ÖR 100%, PR 90%, Her2neu 1+. This finding is primarily compatible with metastatic spread of a mucinous adenocarcinoma of the breast. However, after tumor staging and DNA analysis, systemic seeding could be ruled out and the diagnosis of primary mucinous adenocarcinoma (PMA) of the periorbital region was made.

Results

Due to the limited accessibility for a surgical, eye-preserving excision caused by the tumor’s location, the tumor board decided to attempt antihormonal therapy. Regular MRI controls were conducted during this therapy. Under ongoing tamoxifen treatment, a stable condition without tumor size progression was observed over nine months. However, due to the lack of achievable regression and the existing risk of metastasis, a joint decision with the patient was made to proceed with exenteration. This procedure was performed without complications.

Conclusion

Due to the rarity of this condition, limited data are available regarding prognosis and optimal treatment. To date, surgical excision with clear margins is considered the gold standard. Close interdisciplinary collaboration and multimodality are essential for a definitive diagnosis and adequate treatment. In our case, antihormonal therapy was attempted to avoid exenteration in the absence of subjective symptoms, but unfortunately, it did not lead to the desired regression in tumor size.

Additional Authors

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