Adenoid cystic carcinoma in the nasolacrimal duct: a case report

Abstract

Adenoid cystic carcinoma (ACC) rarely occurs in the nasolacrimal duct (NLD), and when it does, it has nonspecific manifestations. To the best of our knowledge, one case of ACC in the NLD has been reported in the literature. Herein, we report a second case of ACC in the left NLD, concerning a man in his late 60s. He presented with epiphora, and swelling and pain below the left medial canthus. Nasal endoscopy revealed bulging of the lateral wall in the left nasal cavity, with an intact nasal mucosa. Paranasal sinus computed tomography showed an enhancing mass at the epicenter of the left NLD, involving the maxillary sinus, inferior turbinate, and nasal cavity, with bony destruction. The patient underwent superomedial maxillectomy. The pathology was high-grade ACC. Despite undergoing five rounds of postoperative radiotherapy, the ACC recurred 11 months after the operation. Early identification is critical for reducing the morbidity and mortality of patients with ACC in the NLD.

Introduction

Tumors that affect the lacrimal drainage system (including the canaliculi, lacrimal sac, and nasolacrimal duct [NLD]) are extremely rare and have a high mortality rate. Most patients with neoplasms in these organs present with signs and symptoms of punctual discharge, a palpable medial canthal mass, and acquired signs of NLD obstruction, such as epiphora, which can lead to an incorrect diagnosis of dacryostenosis or chronic dacryocystitis. ¹ Consequently, the delayed tumor diagnosis leads to a worse prognosis. ² Among the various parts of the lacrimal drainage system, the lacrimal sac is the site of most tumors. Approximately 70% of neoplasms in the lacrimal drainage system are epithelial, and the most common malignant pathological forms are squamous cell, transitional cell, and mucoepidermoid carcinomas. ³ Adenoid cystic carcinoma (ACC) is a rare form of cancer, especially in the NLD where it has nonspecific manifestations. To the best of our knowledge, only one previous case of ACC in the NLD has been reported in the literature. ⁴ Herein, a second case of a patient with ACC of the NLD is described.

Case presentation

A man in his late 60s presented with swelling and pain below the medial canthus of the left eye that had manifested 1 month previously, and he reported experiencing epiphora for the previous 3 months. The patient had a medication for hypertension and benign prostatic hyperplasia, and reported no past medical histories or any family history of malignant tumors. On physical examination, a bony protrusion of the left cheek was observed. There was no ophthalmologic history of abnormal vision, light sensitivity, pain with eye movement, or eyelid problems. There were no further medical histories, symptoms, or signs of systemic disease, and laboratory tests were unremarkable. No abnormalities were found in routine blood and urine analyses. Nasal endoscopy revealed bulging of the lateral wall in the left nasal cavity, with an intact nasal and paranasal mucosa. Paranasal sinus computed tomography (CT) showed an enhancing mass in the epicenter of the left NLD, involving the anterior wall of the left maxillary sinus, left inferior turbinate, and lateral wall of the left nasal cavity, with bony destruction (Figure 1). Given a presumptive diagnosis of malignancy, wide excision (superomedial maxillectomy) was performed, and the eye and lacrimal sac were conserved. Grossly, the tumor was an ill-defined irregular mass, and the cut surface was grayish tan in color, firm, and granular. Histological examination of hematoxylin and eosin-stained sections revealed a biphasic salivary gland tumor composed of ductal and myoepithelial cells. The tumor showed combined solid, cribriform, and tubular patterns. The predominant solid pattern was formed from sheets composed of basaloid cells, with bony destruction (Figure 2(a)). The minor cribriform pattern was characterized by nests of tumor cells with microcystic-like spaces filled with hyaline or eosinophilic mucoid material (Figure 2(b)). Remarkable tumor cell invasion of the perineural spaces in a tubular pattern (Figure 2(c)), and of the lymphovascular lumens (Figure 2(d)), were evident. High-grade transformation, characterized by comedo-type necrosis, nuclear pleomorphism, and high mitotic activity, was evident in a small portion of the examined tissue (Figure 2(d)). Immunohistochemistry (IHC) for c-Kit protooncogene (CD117) showed positivity in tumor cells of solid pattern (Figure 2(e)). IHC for cytokeratin-5 (CK5) highlighted a myoepithelial component in the tumor of tubular pattern (Figure 2(f)). The pathologic diagnosis was high-grade ACC. The border of the tumor was too infiltrative to be resected completely, as the resection margins included deep and medial radial margin involvement. The patient received five rounds of postoperative radiotherapy, comprising an overall dose of 66 Gy in 33 fractions (2 Gy per fraction for 8 weeks, including a total of 3 weeks of rest periods). ACC recurrence was discovered 11 months after the operation. The tumor was found to have recurred farther below its original location and involved the left nasal cavity, maxillary sinus, hard palate, and buccal space. At the latest assessment, the patient was scheduled for surgery and postoperative radiotherapy for the recurrent tumor. This study was approved by the Institutional Review Board of Chungbuk National University Hospital (2024-09-012), and written informed consent was obtained from the patient for publication of this report and any accompanying images. The reporting of this study conforms to CARE guidelines. ⁵

Figure 1.

Paranasal sinus computed tomography images from a man in his late 60s who presented with epiphora, and swelling and pain below the left medial canthus, showing an enhancing mass (arrow) in the epicenter of the left nasolacrimal duct, involving the anterior wall of the left maxillary sinus, left inferior turbinate, and lateral wall of the left nasal cavity, with bony destruction.

Figure 2.

Histopathological analysis of the excised tumor from a man in his late 60s who presented with epiphora, and swelling and pain below the left medial canthus, showing (a) a predominant solid pattern, with sheets composed of basaloid cells with bony destruction; (b) minor cribriform pattern, characterized by nests of tumor cells with microcystic-like spaces filled with hyaline or eosinophilic mucoid material. Comedo-type necrosis is also identified (arrow); (c) nerve sheath invaded by tumor cells in a tubular pattern (perineural invasion, arrow); (d) tumor cells invading the lymphatics (arrow), with high-grade transformation, characterized by nuclear pleomorphism and high mitotic activity; (e) positive immunohistochemical signal for c-Kit protooncogene (CD117) in tumor cells of solid pattern (left upper); and (f) positive immunohistochemical signal for cytokeratin-5 (CK5) highlighting myoepithelial component in the tumor of tubular pattern (right lower).

Discussion

The case of a male patient in his late 60s with ACC in the NLD is described herein, and to the best of our knowledge, this represents the second reported case. A wide variety of etiologies exist for such masses in the lacrimal drainage system, including infections, inflammation, and neoplasms. ⁶ Neoplastic lesions can be either benign or malignant, the distinction of which may be difficult when based on clinical evaluation alone. The presence of pain may help to predict the nature of the lesion, as ACC usually exhibits perineural invasion. The first published report described the case of a 72-year-old woman who had pain with bloody and purulent lacrimation, and a mass that was identified in the inferior meatus. She was treated with proton beam therapy and showed a favorable response. Most malignant lesions grow more quickly than benign lesions, with their progression averaging less than 6 months from onset to diagnosis. ⁷ Malignant tumors in the lacrimal drainage system generally carry a poor prognosis. ⁸ Because malignant tumors in this site tend to spread up and down intraepithelially, extensive surgical excision with pre- and/or postoperative radiotherapy is required. A previous study found that treatment through wide excision had a recurrence rate of 12.5% versus 43.7% in patients treated with localized excision. ⁹ The overall mortality rate of patients managed with a combination of wide excision and radiotherapy was 37.5%. ⁹

In the present case, the location of the tumor could not be determined until the completion of nasal endoscopy, paranasal sinus CT, and surgery. Considering incidence, the lacrimal gland and the paranasal sinus was initially considered. However, the observation of intact paranasal mucosa during endoscopy, the epicenter of the NLD during paranasal sinus CT, and the lacrimal gland preserved during surgery indicated the NLD to be the primary site. The NLD is lined by a ciliated pseudostratified columnar epithelium and contains goblet cells. ¹⁰ ACC, a malignant epithelial tumor composed of epithelial and myoepithelial cells, usually occurs in areas that contain serous and/or mucinous glands, such as the salivary and lacrimal glands. ¹¹ However, the NLD contains few glandular components, and the most commonly occurring type of cancer in the lacrimal drainage system is squamous cell carcinoma, followed by transitional cell carcinoma. ¹² Therefore, ACC rarely occurs in the NLD, and the patient described here is only the second reported case to be diagnosed with this unique cancer scenario. Embryologically, the NLD originates from the epithelial cord of the lacrimal fossa between the lateral nasal and maxillary processes. ¹³ An ectodermal cord in the lacrimal fossa proliferates laterally toward the eyelid and inferiorly toward the inferior turbinate. During this development, the lacrimal glandular components may be mixed with the NLD. ¹³

Features that should raise suspicion of malignancy include a palpable, firm, incompressible, or immobile mass; telangiectasia or ulceration of the skin overlying the mass; bloody discharge; and continuous epiphora. ¹⁰ Even though ACC of the lacrimal drainage system is uncommon, its early identification is critical for reducing patient morbidity and mortality, given that it is a life-threatening disease. When a malignancy is suspected, imaging study and excisional biopsy should be performed.