Multidisciplinary Management of Lacrimal Sac/Nasolacrimal Duct Carcinomas

Abstract

Purpose To determine rates of globe-sparing treatment and useful final visual function in patients with primary lacrimal sac/nasolacrimal duct carcinomas treated with multidisciplinary therapy. Methods The medical records of 14 patients with primary lacrimal sac/nasolacrimal duct carcinoma treated at 1 institution were retrospectively reviewed. Results The patients were 9 men and 5 women; the median age at diagnosis was 58.5 years (range, 45–73 years). Seven patients presented with epiphora, 7 with a palpable mass in the inferomedial orbit, and 2 with dacryocystitis. In 3 patients, the diagnosis of cancer was not considered until during or after dacryocystorhinostomy (DCR). Seven patients had squamous cell carcinoma, 2 transitional cell carcinoma, 2 adenoid cystic carcinoma, and 1 each adenocarcinoma, poorly differentiated carcinoma, and inverted papilloma with carcinoma in situ transformation. Nine patients underwent surgical resection of the lacrimal sac and nasolacrimal duct, resection of the medial upper and lower eyelids including canaliculi, partial ethmoidectomy, and medial maxillectomy. One patient underwent lacrimal sac biopsy only as another primary malignancy was discovered on the work-up for systemic disease. Four patients underwent orbital exenteration because of extensive involvement of the orbital soft tissue. Radiotherapy was recommended for 13 patients; in 1 patient, radiotherapy was not recommended because the patient had an inverted papilloma with carcinoma in situ transformation that was completely excised. The median radiation dose was 60 Gy. Eight patients received chemotherapy concurrent with radiation therapy (5 patients), as neoadjuvant treatment (1 patient), or for progressive or metastatic disease (3 patients). The median follow-up time was 27 months (range, 6–96 months). In 10 patients, the globe was spared. In 9 of these 10 patients, visual acuity was the same as at baseline or better than 20/40 at last follow-up. Conclusions With multidisciplinary therapy, the eye can be spared and reasonable visual function can be preserved in most patients with primary lacrimal sac/nasolacrimal duct carcinomas.

Primary adenoid cystic carcinoma of the nasolacrimal duct treated with proton beam therapy

“…Findings suspicious of malignancy include pain, bloody lacrimation and nasal discharge, and rapidly enlarging swelling. Malignant tumors developing in the lacrimal passage are very rare; there were only 14 patients in the 12-year study of El-Sawy et al, including 7 with squamous cell carcinomas, 2 with transitional cell carcinomas, 2 with adenoid cystic carcinomas, and 1 each with adenocarcinoma, poorly differentiated carcinoma, and inverted papilloma with carcinoma in situ transformation [4]. However, in their study, the lacrimal sac and nasolacrimal duct were not differentiated as the primary site.…”

Surgical resection of invasive adenoid cystic carcinoma of the lacrimal gland and wound closure using a vertical rectus abdominis myocutaneous free flap

“…Initially, the therapeutic approach was focused on a complete removal of the tumour with preservation of the orbital structures as described in some case reports 2 4. However, a T3 or T4 classification has been associated with worse prognosis5 and once a recurrence was noted with bony and skin invasion the orbital structures as well as the bone and surrounding tissues had to be excised in order to improve the prognosis and disease-free survival 6…”

Adenoid cystic carcinoma of the lacrimal sac: case report

“…The finding of a mass or gelatinous tissue during dacryocystorhinostomy may require termination of the procedure or performance of a biopsy and imaging studies to rule out the existence of neoplasia (11) . Ophthalmologists should be aware of the characteristic triad of primary lacrimal sac tumors, which includes the clinical findings of persistent dacryocystitis or epiphora, an irreducible mass, and abnormal dacrycystography.…”

“…The classical treatment of malignant lacrimal sac tumors has been complete excision of the tumor and lacrimal drainage system, including the canaliculi and nasolacrimal duct, followed by radiotherapy and/or adjuvant chemotherapy (1,9) . At present, a multidisciplinary approach to disease treatment is essential in obtaining the best clinical outcomes (11) . The need to perform orbital exenteration versus more conservative surgery should always be evaluated individually, as exenteration does not ensure a better prognosis (2) and causes substantial facial dis figurement that may lead to severe psychological and psychiatric disorders.…”

“…Following appropriate treatment, patients must undergo lifelong follow-up given the high rate of local recurrence of lacrimal sac tumors (11) . Because of the poor specificity of clinical symptoms, clinicians must have a high degree of suspicion to arrive at a prompt definitive diagnosis.…”