Poorly differentiated sebaceous carcinoma of the lacrimal sac in a young adult: A case report

Abstract

Lacrimal sac and nasolacrimal duct tumors are extremely rare, and most of them are malignant tumors, which are often misdiagnosed as chronic dacryocystitis. We herein report a rare case of a 29-year-old female, presented with a history of watering in the right eye associated with a rapidly progressive mass for 4 months near the medial canthus. Further clinical examination revealed firm, non-tender mass occupying the lacrimal sac fossa extending above the medial canthus. The systemic examination was unremarkable, with a palpable right submandibular lymph node palpable. Contrast-enhanced computerized tomography (CECT) orbit revealed a well-defined mass at the medial canthus extending into the osseous nasolacrimal canal. An excision biopsy was performed, and histopathology revealed a poorly differentiated sebaceous carcinoma of the lacrimal sac. The oncologist advised CECT chest, face, and neck post-surgery, which revealed malignant neoplastic changes at the right lacrimal sac region and lacrimal duct with metastasis at right nodes I b, II, V, and left nodes I b and II. Five-month follow-up showed no signs of recurrence.

Introduction

Lacrimal sac and nasolacrimal duct tumors are extremely rare, and most of them are malignant tumors, that too are often misdiagnosed as chronic dacryocystitis. ¹ Lacrimal sac/nasolacrimal duct tumors can be divided into epithelial and non-epithelial categories. The majority of tumors are epithelial in origin, and the majority, of these are malignant. ¹ Sebaceous carcinoma is an aggressive and potentially lethal tumor of the skin, most commonly occurring in the eyelid. Involvement of the lacrimal apparatus (gland, sac, and canaliculi) and even inferior nasal turbinate by sebaceous carcinoma is rare.^2,3 Herein, we report a rare case of right lacrimal sac sebaceous carcinoma with nasolacrimal osseous extension and regional node metastasis in a young Asian Indian adult.

Permission from the patient to print identifiable photographs was obtained and archived. Written Informed consent was obtained from the patient in question to publish their medical case and accompanying images. The case described in this report is compliant with the Declaration of Helsinki and health insurance Portability and Accountability Act regulations.

Case description

History and examination

A 29-year-old female presented with right eye watering associated with a mass at medial canthus area for 4 months. It was rapidly progressive and not associated with any other ocular complaint. The best corrected visual acuity was 6/6 in both eyes. Ocular movements were full of all gazes. On evaluation, a well-defined, firm, non-tender mass (2.5 × 2 cm) was present at the lacrimal sac fossa extending above the medial canthus. Diagnostic probing of the lacrimal apparatus revealed a soft stop. Conjunctival and other aspects of the ocular examination were within normal limits, thus ruling out the possibility of any spread from ocular adnexa. Since there was no suspicious lesion thus conjunctival map biopsy was also not performed on this patient. The systemic examination was clinically unremarkable, with the right submandibular lymph node palpable. Based on the clinical findings, a diagnosis of the lacrimal sac tumor was made.

Investigations and treatment

Contrast-enhanced computerized tomography (CECT) orbit revealed a well-defined soft tissue mass with a focal necrotic component at the medial canthus region extending into the osseous nasolacrimal canal with osseous widening with no deep intra-orbital extension. A small calcific lesion is seen in the proximal osseous nasolacrimal canal (Figure 1a and b). The patient underwent an elective excision biopsy under general anesthesia through an incision over the anterior lacrimal crest, mass removed in toto (Figure 1c and d). Histopathology revealed a nodular tumor, composed of lobules and nests of malignant cells with central luminal necrosis. The tumor cells show moderate amount of vacuolated cytoplasm, large bizarre, markedly pleomorphic, hyperchromatic nuclei, and frequent mitoses. Resection margin was free of tumor (R0 resection). These morphological features were suggestive of poorly differentiated sebaceous carcinoma of the lacrimal sac (Figure 2a and b).

Figure 1.

(a, b) CECT showing right soft tissue mass at medial canthus region and calcific foci in proximal nasolacrimal canal. (c) Medial view of orbit showing intra-operative lacrimal sac mass. (d) Post-operative lacrimal sac mass (2.5 × 2 cm).

Figure 2.

(a, b) High and low-power images of H&E stained sections show lobules and nests of malignant cells with central luminal necrosis. The tumor cells show moderate amount of vacuolated cytoplasm, large bizarre, markedly pleomorphic, hyperchromatic nuclei, and frequent mitoses, suggestive of poorly differentiated sebaceous carcinoma.

The post-operative period was uneventful, the oncologist advised CECT chest, face, and neck post-operatively, which revealed heterogeneously enhanced soft tissue thickening involving right lacrimal sac region, tract of lacrimal duct with mild to moderate fat stranding. Partially calcified mildly enlarged metastatic nodal mass with central necrosis at right node level I B (8.6 × 16.7 mm) was present. Right node level II/V and left node levels I B and II were suspicious for metastasis. Wide local excision with modified radical neck dissection with free flap reconstruction was planned, followed by radiotherapy, but the patient denied further treatment. A 5-month follow-up showed no signs of mass recurrence in the lacrimal sac area (Figure 3). Despite being counseled about a grave prognosis, the patient refused further investigation, and treatment and lost to follow-up later.

Figure 3.

Five-month follow-up with no recurrence.

Discussion

Sebaceous carcinoma is a highly malignant tumor arising from the sebaceous glands, such as those of Meibomian glands in the tarsus, the Zeiss glands of the eyelashes, the caruncle, and the skin of the eyebrow. ⁴ Primary lacrimal sac sebaceous carcinoma with nasolacrimal canal extension or involvement of lacrimal apparatus (glands, sac, and canaliculi) by contiguous or non-contiguous spread is rare. A major review of 331 patients with primary malignant epithelial tumors of the lacrimal drainage system showed squamous cell carcinoma being the most common tumor (61%) in the lacrimal sac, and they found only one case report of an exceptionally rare primary Sebaceous gland carcinoma (SGC) of the lacrimal sac. ¹ In a retrospective analysis from December 2001 to June 2012 by El-Sawy et al., 14 patients with primary lacrimal sac/nasolacrimal duct carcinoma were found with no single case of sebaceous carcinoma. ⁵

Bi et al., in a past 50-year retrospective analysis, reported 96 cases of primary lacrimal sac tumors with no single case of sebaceous carcinoma. Among these lacrimal sac tumor, 94.8% were malignant tumors. All epithelial lacrimal sac tumors were malignant and squamous cell carcinoma was the most common tumor. ⁶ Ocular and periocular tumors analysis from September 2013 to August 2018 by Kaliki and Das reported only 11 cases (<1%) of lacrimal sac tumors, with 5 cases being malignant. ²

Rath et al. described a case of the sebaceous carcinoma of eyelid metastasizing to the ipsilateral lacrimal sac 5 years after a complete primary excision. ⁷ Jakobiec described a case of necrotic sebaceous carcinoma in the lumen of the lacrimal canaliculus. ³

Vahdani et al. reported two cases of lacrimal sebaceous carcinoma, where case one was a 74-year-old woman with primary lacrimal sac sebaceous carcinoma without any history of eyelid or adnexal malignancy, and case two had a previous history of local tumor resection. ⁸

The literature review indicates that primary lacrimal sac sebaceous carcinoma and contiguous or non-contiguous spread of sebaceous carcinoma to lacrimal apparatus are rare entities, and all cases have been reported in older individuals.^7–10 This is the first case of primary lacrimal sac sebaceous carcinoma with regional nodal metastasis in a young Asian Indian adult without concurrent or previous ocular adnexal sebaceous neoplasia.

A hypothetical explanation could be the presence of an ectopic (choristomatous) sebaceous gland in the lacrimal sac, ¹¹ leading to malignant transformation or sebaceous gland metaplasia in lacrimal sac with malignant transformation. Extra-orbital sebaceous cell carcinoma involving the parotid gland may arise from pluripotent or ectopic cells retained during embryologic development. ⁴ It is extremely difficult as of now to prove any of these theories.

Conclusion

Rapidly progressive mass at the lacrimal sac area extending above the medial canthus should be kept under suspicion for malignancy and urgent imaging and biopsy are paramount, conjunctival map biopsy to be considered in suspicious cases to rule out pagetoid spread.

Long-term follow-up is vital as recurrence or metastasis can occur many years after primary surgery for sebaceous carcinoma.