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Sudip Thakar, Thomas Kandl, Oded Sagiv, Michael Tetzlaff, Bita Esmaeli; Desmoplastic Melanoma of the Periocular Region. Invest. Ophthalmol. Vis. Sci. 2017;58(8):3356.
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© ARVO (1962-2015); The Authors (2016-present)
Desmoplastic melanoma (DM) is a unique, rare variant of melanoma occurring in less than 4% of cutaneous melanomas. This subtype of melanoma is even more rare in the periocular region and often diagnosis is delayed or misdiagnosed. The goal of this report is to describe histologic features, clinical behavior, and treatment considerations for desmoplastic melanoma of the periocular region.
The medical records of two consecutive patients with locally advanced desmoplastic melanoma of the periocular region were retrospectively reviewed.
Both patients presented with greater than T2b melanomas (8th edition AJCC criteria). Patient 1 presented with DM of the left lateral canthus with upper and lower eyelid involvement. Patient 2 presented with DM of the left brow and supraorbital region and was also found to have nodal metastasis. In both cases, the lesions had been there for several years prior to the correct diagnosis. This was due to the amelanotic, flesh colored, inflammatory appearance of the lesions and also due to predominance of inflammatory cells on the biopsy specimens. In both patients, the presence of perineural invasion led to the decision to do adjuvant radiation therapy after wide excision and in both instances large surgical defects had to be repaired in the periocular region. Patient 2, with nodal metastasis, was also treated with an immune checkpoint inhibitor drug.
Our two cases add to only nine previously reported cases of periocular desmoplastic melanoma and highlight the importance of Ophthalmologists being aware of this rare variant of melanoma, its neurotrophic nature, and its potential to present with locally advanced lesions that lead to significant ocular morbidity and have the potential for metastasis.
This is an abstract that was submitted for the 2017 ARVO Annual Meeting, held in Baltimore, MD, May 7-11, 2017.
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