Abstract
Background. Primary malignant melanoma of the lacrimal sac is rare. A patient with lacrimal sac melanoma was presented, and 14 Japanese patients with lacrimal sac melanoma in the literature were reviewed. Case Presentation. A 78-year-old Japanese man was presented with painless swelling of the lacrimal sac on the left side. Dacryocystectomy revealed diffuse infiltration with large epithelioid cells, sometimes with pigments, which were positive for cocktail mix of antibodies to tyrosinase, melan A (MART-1), and HMB45, leading to pathological diagnosis of melanoma. One month later, positron emission tomography (PET) revealed 2 high-uptake sites (SUVmax = 10.29 and 15.38) at the levels of medial canthus and nasolacrimal duct, but no abnormal uptake in the other site of the body. The lesion had the BRAF V600E mutation. He began to take daily oral dabrafenib (BRAF inhibitor) and trametinib (MEK inhibitor), leading to no abnormal uptake on PET in half a year. He had stable disease in good physical status with small and weak uptake sites of lymph nodes on PET 1 year later. Results. In the review of 15 Japanese patients, including this patient, local recurrence was noted in 4 patients, regional lymph node metastasis only in 3, distant metastasis in 6, and no metastasis in 6. Five patients died within 2 years and the others were alive in short follow-up periods. Conclusions. Chemotherapy was the standard for local recurrence or metastasis. Emerging molecular target drugs, as shown in the present patient, would change the strategy for management of lacrimal sac melanoma.
Highlights
Primary malignant melanoma of the lacrimal sac is rare
We present a Japanese patient with lacrimal sac melanoma who underwent a modern combination therapy of molecular target drugs, dabrafenib (BRAF inhibitor) and trametinib (MEK inhibitor)
We reviewed 15 Japanese patients with lacrimal sac melanoma, including the present patient, who were described in the literature.[25,26,27,28,29,30,31,32,33,34,35,36,37]
Summary
The lacrimal sac is a component of the ocular adnexa in the orbit and serves as a drainage system of the lacrimal fluid from the ocular surface through the nasolacrimal duct to the nasal cavity. A 78-year-old man was presented with 10-month history of waxing and waning painless swelling of the lacrimal sac on the left side (Figure 1E). Computed tomographic scan showed a 1-cm-sized round homogeneous mass with no bony change on the left lacrimal fossa (Figure 1A). He underwent left lacrimal sac tumor extirpation under general anesthesia. A cluster of small lymphocytes were noted among the large neoplastic cells (Figure 2B) One month later, he developed subcutaneous pigmented bumpy lesions surrounding the medial canthus The subcutaneous and nasolacrimal ductal recurrent lesions resolved (Figure 1G), and PET/CT showed no abnormal uptake in the orbit on the left side (Figure 1D), with no abnormal uptake at other sites of the body. Except for occasional mild nausea, was noted throughout the course
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