Transitional Cell Carcinoma of the Lacrimal Sac Presenting with Bloody Tears

Introduction

Transitional cell carcinomas (TCC) of the lacrimal sac are uncommon tumors that can have significant morbidity and mortality if not diagnosed and treated in a timely fashion. These tumors have variable clinical and histologic features. Bloody tears, also known as dacryohemorrhea, has been reported only once previously as a presenting sign of the tumor [1] .We describe a case of lacrimal sac TCC presenting with epiphora, dacryohemorrhea, and medial canthal mass.

Case report

A 54-year-old African-American man presented with right lower eyelid swelling and bloody tears for six months. On examination, his visual acuity was 20/25 in the right eye and 20/20 in the left, with no relative afferent pupillary defect. Intraocular pressures were 12 mmHg in both eyes. On external examination, there was a palpable subcutaneous mass in medial canthal area; upon palpating the lacrimal sac, a flesh-colored mass with serosangouinous discharge protruded from the lower lid punctum. Nasolacrimal duct irrigation was deferred. Computed tomography demonstrated a heterogeneous soft tissue mass, measuring 1.8 × 2.2 × 1.8 cm, centered on the right lacrimal sac fossa and extending into the proximal aspect of the nasolacrimal duct (Figure 1A).

Figure 1.

A, Orbital CT scan demonstrates a heterogeneous, relatively well circumscribed soft tissue mass, measuring 1.8 × 2.2 × 1.8 cm, centered on the right lacrimal sac fossa. Note bone remodeling of the fossa. B, Intraoperative photo demonstrates a large mass within the lacrimal sac.

The patient underwent an incisional biopsy of the lacrimal sac. It was noted intraoperatively that the lesion was friable and discohesive upon opening the sac (Figure 1B). On histologic examination, the specimen exhibited papillary proliferation of atypical transitional epithelial cells with conspicuous mitotic figures and severe nuclear pleomorphism (Figures 2 A&B). The tumor did not invade the basement membrane. Diagnosis of TCC in situ was made. The specimen was subsequently evaluated for P16 expression, a marker for human papillomavirus (HPV)-16, which was strongly positive (Figure 2 C), suggesting HPV as an underlying etiology. The patient was referred to otolaryngology for multidisciplinary resection and reconstruction; however, he has to date refused any further surgical intervention.

Figure 2. Transitional cell carcinoma of the lacrimal sac.

A, Papillary proliferation of transitional cells. H&E, ×40. B, Nuclear pleomorphism and mitotic figures are noted. H&E, ×400. C, Immunohistochemistry for HPV-16 demonstrates positive reactivity, suggesting HPV involvement. IHC for HPV-16, ×400.

Comments

Epithelial tumors of the lacrimal sac arise from the transitional epithelium. Histologically, these tumors are divided into squamous cell carcinoma, transitional cell carcinoma, and adenocarcinoma. TCCs of the lacrimal sac carry the worst prognosis [2]. Like the more common TCC of the urinary bladder, cells show marked pleomorphism, prominent nucleoli, and conspicuous mitotic figures.

We found a total of 37 cases of TCC of the lacrimal sac in the literature [1-6]. The mean age was 50 years with no gender predilection [1-6]. The most common presenting symptoms were epiphora and medial canthal mass [4]. Bloody tear is a rare presenting symptom for TCC of the lacrimal sac and it has only been reported once in the literature [1].

While it is possible to suspect a diagnosis of TCC of the lacrimal sac based on clinical history, physical examination, and imaging studies, cases were frequently misdiagnosed as dacryocystitis, nasolacrimal duct obstruction, and mucocele [4]. Accurate diagnosis in majority of published cases was made at the time of surgery and after histologic examination.

Treatment for lacrimal sac TCC consists of surgical excision alone or in combination with radiation therapy [5]. The average mortality rate for patients with TCC of the lacrimal sac is 44%, ranging from 0-100% depending on the case series [6]. This large variability in mortality rate is likely due to the higher mortality associated with delayed diagnosis [2].

Our patient presented with dacryohemorrhea in addition to other common presenting signs. Dacryohemorrhea has only been described once previously in association with TCC of the lacrimal sac [1]. Given the malignant nature of this tumor and high mortality rate, clinicians must have a high index of suspicion when encountering a patient with dacryohemorrhea and a lacrimal sac mass.