Primary nasolacrimal duct malignacy is rare. Only a few tumours have been reported originating from this location including lymphoma,¹ Kaposi’s sarcoma,² adenocarcinoma,³ angiofibroma,⁴ and squamous cell carcinoma.⁵

Mucosal melanoma represents 1–2% of all melanomas,⁶ with 25–50% in the head and neck—that is, upper respiratory tract, oral cavity, and pharynx. Other sites include urinary, female genital, and ano-rectal tracts. These tumours are uncommon, present in the fifth to eighth decades, have slight male preponderance and are more common in darker skinned individuals. Cutaneous melanoma presents two decades earlier and is more common in white people and is associated with sun exposure.

Case report

A 41 year old Asian male presented to the oculoplastic service with a 3 month history of right sided intermittent bloody tears and epistaxis. He described right simple epiphora for at least 2 years treated with a three snip procedure. Probing the right nasolacrimal system confirmed normal canaliculi and easy entry of the probe into the lacrimal sac. Irrigation demonstrated complete nasolacrimal duct block and reflux of dark bloody tears. Nasal endoscopy (rigid Hopkins nasal endoscope) revealed a greyish mass emerging from the lower end of the nasolacrimal duct into the inferior meatus (fig 1).

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Figure 1

 Endonasal view of right nasal cavity. IT, inferior turbinate; SS, septal spur; IM, inferior meatus; T, tumour.

Magnetic resonance imaging (MRI) and computed tomography (CT) scans (fig 2) demonstrated dilation of the entire length of the nasolacrimal bony canal with a soft tissue mass occupying the lumen of the nasolacrimal duct. The mass was invading the osteomeatal complex, extending into the anterior ethmoidal sinus, causing obstruction to drainage of both maxillary and frontal sinuses.

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Figure 2

 Imaging. (Left) CT scan head, axial view. Lesion in region of lacrimal sac. (Middle) CT scan head, axial view. ND, dilated nasolacrimal duct; with fluid in maxillary sinus (MS). (Right) MRI scan head, coronal view. Soft tissue mass extending from lacrimal sac, via nasolacrimal duct, to inferior meatus.

A transnasal incisional biopsy of the inferior meatus lesion was performed under local anaesthetic. Histopathological analysis of the biopsy showed sheets of epithelioid cells with prominent nucleoli and vesicular cells on a routine haematoxylin and eosin stain (fig 3). Although the lesion was pale, pigment was visible on the haematoxylin and eosin stain, suggestive of melanoma. Immunohistochemical studies were positive for immunological markers HMB45 and S-100 protein, confirming a diagnosis of melanoma.

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Figure 3

 Histopathology slides. (A) High power 400×. Haematoxylin and eosin stain showing epithelioid cells and vesicular cells and pigment. (B) Medium power 200×. HMB-45 stain, positive for melanocytes. (C) High power 400×. Strongly positive S-100 protein immunohistochemical stain.

No regional lymphadenopathy was present and PET (positron emission tomography) scan excluded the presence of metastases.

Wide local cranio-facial-orbital resection of the tumour was performed, via a lateral rhinotomy approach (fig 4). The nasolacrimal duct, part of the lateral wall of the nose, the lacrimal sac, distal upper and lower canaliculi, and inferomedial orbital fat were excised. Tumour extended into the maxilla but not into the lacrimal sac. Peroperative frozen sections from the excision margins, including orbital fat, demonstrated clearance of tumour. This allowed preservation of the eye.

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Figure 4

 Wide local excision of malignant mucosal melanoma. T, tumour; EB, eyebrow.

Adjunctive therapy was given to limit local recurrence and distant spread. The patient received 20 sessions of radiotherapy (50 Gy) to the right maxillary antrum, and also immunological therapy (vaccination with autologous dendritic cells pulsed with allogenic tumour lysate, and whole body hyperthermia accompanied by low dose interferon).

Three years after presentation there was no local recurrence of tumour. Unfortunately, the patient developed bone pain and CT scan revealed metastatic spread to the right acetabulum and T8, T9, and T11 vertebrae with localised spinal cord compression. He underwent radiotherapy to the right acetabulum and spine. The patient died approximately 6 months later.

Comment

Nasolacrimal duct primary mucosal melanoma is previously unreported. The closest relations are lacrimal sac melanoma (5% of lacrimal sac tumours)^7,8 and sinonasal mucosal melanoma (5% of all sinonasal malignacy).^6,9

Lacrimal sac melanoma can present with epiphora, bloody tears, and epistaxis. Sinonasal melanoma can present with nasal obstruction and epistaxis. Orbital extension causes proptosis and diplopia. Both lacrimal sac melanoma and sinonasal melanoma are insidious in onset with poor visibility leading to late presentation.

Standard treatment of primary sinonasal melanoma is radical wide local surgical excision. Local recurrence can occur and the very vascular nature of mucosal tissue makes regional and distant metastases common, often accompanying late presentation. Post-surgical radiotherapy may prevent local recurrence, but is unlikely to prevent distant metastases.¹⁰ Other adjunctive treatments are not clinically proved to be beneficial. Chemotherapy has no positive effect, and newer immunological treatments are still under investigation.

Sinonasal mucosal melanoma has a high mortality rate, with 5 year survival between 10–50%.⁶ Lacrimal sac melanoma has a similar prognosis.^7,8

This case demonstrates the importance of thorough clinical examination (including nasal endoscopy) and imaging in patients presenting with epiphora, bloody tears, and/or epistaxis. Delay in detection and treatment may be fatal.