|Year : 2014 | Volume
| Issue : 1 | Page : 27-29
Transitional cell carcinoma of the lacrimal sac: A clinico-pathological case study
Bipasha Mukherjee1, Ravija Patel1, Jyotirmay Biswas2, S Krishnakumar2
1 Department of Orbit, Oculoplasty, Reconstructive and Aesthetic Services, Chennai, Tamil Nadu, India
2 Department of Ocular Pathology, Medical Research Foundation, Sankara Nethralaya, Chennai, Tamil Nadu, India
|Date of Submission||10-Jan-2013|
|Date of Acceptance||22-Jun-2013|
|Date of Web Publication||3-Dec-2013|
Department of Orbit, Oculoplasty, Reconstructive and Aesthetic Services, Medical Research Foundation, Sankara Nethralaya, 18, College Road, Chennai - 600 006, Tamil Nadu
Source of Support: None, Conflict of Interest: None
Tumors of the lacrimal sac are rarely encountered in clinical practice. The importance of recognizing this entity lies in the fact that the majority of the lacrimal sac tumors turn out to be malignant and thereby potentially life-threatening. Hence appropriate management of the lacrimal sac tumors is of utmost importance. We report a case of transitional cell carcinoma of the lacrimal sac, its presentation and management along with the review of the published literature.
Keywords: Carcinoma, dacryocystectomy, lacrimal sac
|How to cite this article:|
Mukherjee B, Patel R, Biswas J, Krishnakumar S. Transitional cell carcinoma of the lacrimal sac: A clinico-pathological case study. J Clin Ophthalmol Res 2014;2:27-9
|How to cite this URL:|
Mukherjee B, Patel R, Biswas J, Krishnakumar S. Transitional cell carcinoma of the lacrimal sac: A clinico-pathological case study. J Clin Ophthalmol Res [serial online] 2014 [cited 2022 Aug 9];2:27-9. Available from: https://www.jcor.in/text.asp?2014/2/1/27/122639
Carcinomas arising primarily from the lacrimal sac are rare with less than 30 cases of transitional cell carcinoma reported in the literature. It carries the poorest prognosis among all types of lacrimal malignancy. It is potentially lethal if it is not recognized and treated promptly and adequately. We report an unusual case of recurrent transitional cell carcinoma of the lacrimal sac. This case report highlights the need for accurate recognition, aggressive management and careful follow-up of these patients.
| Case Report|| |
A 63-year-old Asian-Indian male patient presented with the complaints of a gradually progressive swelling over the right lacrimal sac region over last 6 months. There was no history of watering, discharge, bloody tears, epistaxis or acute dacryocystitis before the onset of swelling. There was no history suggestive of associated sinonasal disease. Anterior and posterior segment ophthalmic examinations were unremarkable. There was a palpable, firm to hard, non-tender mass approximately 2 cm × 1.5 cm in size in the right lacrimal sac area. Skin overlying the mass was mobile and free from the underlying mass. The lesion was not extending above the medial canthal tendon [Figure 1]. Regurgitation on pressure over lacrimal sac was negative. Syringing was freely patent. There was no proptosis or restriction of ocular movements. Computerized tomography (CT) scan showed a soft-tissue lesion in the right medial canthal region measuring 2.1 cm × 1.6 cm in cross section with minimal remodeling of the lacrimal bone and extension into the nasolacrimal canal [Figure 2].
|Figure 1: External photograph of the patient showing right lacrimal sac mass displacing the lower lid medially|
Click here to view
|Figure 2: Computerized tomography of the orbits: (a) Coronal and (b) axial scan showing an oval soft-tissue lesion in the right medial canthal region. Soft-tissue extension into the nasolacrimal duct is seen (arrow)|
Click here to view
Patient was advised right dacryocystectomy after counseling about post-operative epiphora. Pre-operatively the lesion was firm, encapsulated and adherent to the surrounding tissue. The lacrimal sac mass was removed completely along with periosteum of the lacrimal sac fossa and the nasolacrimal duct as per standard procedure. 
The histopathological examination showed tumor composed of epithelial cells arranged in lobules and chord like pattern. The cells had squamoid morphology and were spindle shaped. There was nuclear atypia with mitotic activity in the epithelial cells [Figure 3]. Thus, a diagnosis of transitional cell carcinoma of the lacrimal sac was made.
|Figure 3: Microphotograph of the lacrimal sac specimen (H and E, ×100): Showing tumor composed of epithelial cells arranged in lobules and chord like pattern. The spindle shaped cells have squamoid morphology. Nuclear atypia with mitotic activity was seen in the epithelial cells (Inset: H and E, ×400)|
Click here to view
The post-operative period was uneventful. We referred the patient to oncologist who advised adjuvant radiotherapy. We explained about the need for regular and close follow-up to the patient clearly. Despite this, the patient defaulted and returned only after 10 months to the oncology center where adjuvant therapy was deemed ineffective due to the significant time lag. Three months after, the patient presented to us with history of bleeding from the right nostril. There were no external signs of recurrence. We referred him to otolaryngologist who did an endoscopic examination with histopathological examination of maxillary sinus lavage specimen, which revealed malignant cells. In view of recurrence of the disease, he was advised CT scans of orbits and paranasal sinuses followed by radical maxillectomy and radiotherapy. However, until date, he has refused to undergo any further intervention.
| Discussion|| |
Primary carcinomas of the lacrimal sac are rare with less than 30 transitional cell carcinomas described in the medical literature. Out of the 300 odd lacrimal sac tumors reported most are epithelial tumors (73%) of which 75% are malignant. 
Transitional cell carcinoma of the lacrimal sac is the second most common type of lacrimal malignancy; the first being squamous cell carcinoma.  It is potentially lethal if it is not recognized and treated promptly and adequately.
The most patients with lacrimal sac tumors present with a mass lesion and signs of obstruction of the lacrimal drainage system. In addition, a mass with extension above the medial canthal tendon and hemolacria (bloody tears) with pain is highly suggestive of a malignant neoplasm of the lacrimal sac. , Our patient, however, did not demonstrate any of the typical features apart from a lacrimal sac mass.
There are only a small number of documented cases of transitional cell carcinomas. Parmar and Rose described three cases of transitional cell carcinoma and one case of mixed transitional cell carcinoma and squamous cell carcinoma.  Ni et al. six, and Stefanyszyn et al. have reported five cases respectively. ,
Transitional cell carcinoma has a strong association with invasive papilloma. 
Harry and Ashton provided useful criteria for the diagnosis of benign and malignant transitional cell tumors and divided them into categories on the basis of morphology and clinical behavior. The Type III or Transitional cell carcinoma consisted of a neoplasm with irregular, stratified epithelium, invasive growth, marked cellular pleomorphism, conspicuous squamous metaplasia and conspicuous mitotic activity. 
According to Pang et al., good prognostic factors include well-differentiated cells with uniform columnar cells, rare mitotic figures, and an intact basement membrane. On the other hand, transitional cell carcinomas with marked pleomorphism, squamous metaplasia, numerous mitotic figures and areas of stromal invasion have high rates of recurrence in spite of radical treatment. 
Treatment recommendations have varied, and often multidisciplinary. The modalities include surgery, chemotherapy, and radiotherapy, depending of the histologic type and extent of the tumor. Where the tumors are localized to the lacrimal sac fossa, dacryocystectomy with amputation of the lacrimal sac from its connection with the common canaliculus laterally and the nasolacrimal canal inferiorly (with the periosteum of the fossa) is undertaken through a standard dacryocystorhinostomy incision.  Resection of the adjacent orbital and lateral nasal wall may be required with more extensive tumors or in advanced cases, orbital exenteration may be required. The use of the en bloc excision as a radical treatment to completely remove the lacrimal drainage apparatus and surrounding bony structures affords good tumor control against local recurrence and may provide the best opportunity for enhanced patient survival.  Adjuvant radiotherapy is recommended for unrecognized local extension, microscopic residual disease, involvement of margins, or aggressive histology. ,
Transitional cell carcinomas have the poorest prognosis among the epithelial tumors of the lacrimal sac. Ni et al. found 100% mortality from recurrence.  Preechawai et al. found one- third of patients having a recurrence with a 22% distant metastasis. The overall mortality rate quoted by Preechawai et al. is 44%.  According to Pang et al., 50% of carcinomas of the lacrimal sac recur and of these recurrences, the mortality rate is more than 50%. 
| Conclusion|| |
It is prudent to consider all lacrimal sac tumors as malignant unless proven otherwise by histology. These lesions merit aggressive treatment and lifelong follow-up in view of high chances of recurrence.
| Acknowledgment|| |
The authors would like to thank Dr. Olma Veena Noronha, M. D. (Radiology), VRR scans, Chennai, for expert opinion on computerized tomography scans.
| References|| |
|1.||Valenzuela AA, Selva D, McNab AA, Simon GB, Sullivan TJ. En bloc excision in malignant tumors of the lacrimal drainage apparatus. Ophthal Plast Reconstr Surg 2006;22:356-60. |
|2.||Parmar DN, Rose GE. Management of lacrimal sac tumours. Eye (Lond) 2003;17:599-606. |
|3.||Pang CS, Brown JD, Ganote CE, Youngberg GA. A mass of the right lacrimal sac in a 53-year-old man. Arch Pathol Lab Med 2005;129:1493-4. |
|4.||Azari AA, Kanavi MR, Saipe N, Lee V, Lucarelli M, Potter HD, et al. Transitional cell carcinoma of the lacrimal sac presenting with bloody tears. JAMA Ophthalmol 2013;131:689-90. |
|5.||Ni C, D'Amico DJ, Fan CQ, Kuo PK. Tumors of the lacrimal sac: A clinicopathological analysis of 82 cases. Int Ophthalmol Clin 1982;22:121-40. |
|6.||Stefanyszyn MA, Hidayat AA, Pe'er JJ, Flanagan JC. Lacrimal sac tumors. Ophthal Plast Reconstr Surg 1994;10:169-84. |
|7.||Heathcote JG. Transitional neoplasms of the naso-lacrimal system: A review of the histopathology and histogenesis. Saudi J Ophthalmol 2012;2:125-131. |
|8.||Harry J, Ashton N. The pathology of tumours of the lacrimal sac. Trans Ophthalmol Soc U K 1969;88:19-35. |
|9.||Karim R, Ghabrial R, Lin B. Transitional cell carcinoma of the nasolacrimal sac. Clin Ophthalmol 2009;3:587-91. |
|10.||Valenzuela AA, McNab AA, Selva D, O'Donnell BA, Whitehead KJ, Sullivan TJ. Clinical features and management of tumors affecting the lacrimal drainage apparatus. Ophthal Plast Reconstr Surg 2006;22:96-101. |
|11.||Preechawai P, Della Roccad RC, Della Rocca D, Schaefer S, McCormack S. Transitional cell carcinoma of the lacrimal sac. J Med Assoc Thai 2005;88 Suppl 9:S138-42. |
[Figure 1], [Figure 2], [Figure 3]