FP1099 : Oncorrhoea – is it Real?

Dr.Sonal Chaugule, C14278, Dr. Kaustubh Mulay, Dr.Santosh G Honavar

Abstract:

Purpose: To describe clinical features, management and outcome in a case series with conjunctival and eyelid malignancies extending to the lacrimal drainage system

Methods: A retrospective, non-comparative, interventional case series with 8 cases presenting with primary or recurrent conjunctival and eyelid malignancies extending to the lacrimal drainage system. Clinical features, lacrimal evaluation and management was reviewed for each case.

Results: The mean age of the group was 51.5 (range, 43-75) years; with 5 males and 3 female patients. The series had either primary (n=4) or recurrent (n=4) tumors with a diagnosis of eyelid sebaceous gland carcinoma (n=4), ocular surface squamous neoplasia [OSSN] (n=2) and conjunctival melanoma (n=2). Nasolacrimal duct (NLD) obstruction or lacrimal sac involvement was found at the time of evaluation and was confirmed by imaging. Tumors were managed by complete excision (n=5) or orbital exenteration (n=3) with en-bloc excision of lacrimal sac and nasolacrimal duct, followed by histopathological confirmation. There was no contiguity with the main tumor in any of the cases. Adjuvant radiotherapy was provided in 4 cases. No evidence of local, regional recurrence or systemic metastasis was found at the mean follow up of 4.5 (range, 1-11) years.

Conclusion: Eyelid and conjunctival malignant tumors can extend to lacrimal sac and NLD by means of oncorrhoea – shedding and implantation of tumor cells and proliferation in the new location. The extension is possible without having contiguity with the main tumor. Complete en-bloc excision followed by radiotherapy, when indicated can ensure good local disease control.

Introduction:

Malignancies of eyelid and conjunctiva; specifically, sebaceous gland carcinoma of eyelid, squamous cell carcinoma of conjunctiva and conjunctival melanoma can involve structures of lacrimal drainage system (canaliculi, lacrimal sac and nasolacrimal duct), nasal cavity and paranasal sinuses. It has been suggested that the spread may occur by direct extension, by hematogenous spread or by shedding of exfoliated tumor cells in the tear film. ^(1-4)

The term‘Lacrimal oncorrhea’ means presence of floating cancerous cells in the tear film. This term has been used since a few decades as a probable route of local recurrence or regional spread to non-contiguous sites i.e. lacrimal drainage apparatus for eyelid as well as conjunctival malignancies.

We present a series of cases, both primary as well as recurrent malignancies of eyelid (sebaceous carcinoma of eyelid) and conjunctiva (conjunctival melanoma and squamous cell carcinoma of conjunctiva) which demonstrated local recurrence and/or regional spread to lacrimal draining apparatus (lacrimal sac and nasolacrimal duct) without involvement of ipsilateral puncta or canaliculi.

Methods:

A retrospective, non-comparative, interventional case series with 8 cases presenting with primary or recurrent conjunctival and eyelid malignancies extending to the lacrimal drainage system. Patient data was retrospectively reviewed to collect demographic information, clinical examination details, lacrimal system evaluation and management protocol involving surgery and /or radiotherapy. 

Results:

The mean age of the group was 51.5 (range, 43-75) years; with 5 males and 3 female patients. The series had either primary (n=4) or recurrent (n=4) tumors with a diagnosis of eyelid sebaceous gland carcinoma (n=4), ocular surface squamous neoplasia [OSSN] (n=2) and conjunctival melanoma (n=2). Nasolacrimal duct (NLD) obstruction or lacrimal sac involvement was found at the time of evaluation and was confirmed by imaging.

Tumors were managed by complete excision (n=5) or orbital exenteration (n=3) with en-bloc excision of lacrimal sac and nasolacrimal duct, followed by histopathological confirmation. There was no contiguity with the main tumor in any of the cases. Adjuvant radiotherapy was provided in 4 cases. No evidence of local, regional recurrence or systemic metastasis was found at the mean follow up of 4.5 (range, 1-11) years.(Table 1 & 2)

Discussion:

This case series demonstrates that extension of sebaceous gland carcinoma of eyelid; squamous cell carcinoma of conjunctiva and conjunctival melanoma can involve lacrimal sac and nasolacrimal duct without involving contiguous structures. Management of such condition requires caution to make sure the malignancy has been completely eradicated.

Eyelid and conjunctival malignant tumors can extend to lacrimal sac and NLD by means of oncorrhoea – shedding and implantation of tumor cells and proliferation in the new location. The extension is possible without having contiguity with the main tumor. Complete en-bloc excision followed by radiotherapy, when indicated can ensure good local disease control.

This series provides positive evidence towards the concept of ‘lacrimal oncorrhea’ as a method of tumor cells dissemination and extension.

Table 1: Clinical features and management in primary conjunctival and eyelid tumors extending to lacrimal drainage system

Case No	Age/ Sex	Diagnosis	Management		HPE findings
			Surgery	Radiotherapy
1	70/F	OD Sebaceous gland carcinoma of lower lid with orbital extension and diffuse conjunctival pagetoid invasion, with lacrimal sac involvement	Orbital exenteration with en-bloc excision of lacrimal sac and NLD	–	Sebaceous gland carcinoma involving lacrimal sac; no canalicular or NLD involvement found
2	43/M	OD Ocular surface squamous neoplasia with orbital extension, lacrimal sac enlargement on CT scan	Orbital exenteration with en-bloc excision of lacrimal sac and NLD	–	Squamous cell carcinoma found in lacrimal sac and sac- NLD junction; no evidence of canalicular involvement
3	45/F	OD Diffuse conjunctival melanoma with lentigo maligna of lower lid, NLD obstruction +	Orbital exenteration with en-bloc excision of lacrimal sac and NLD	–	Malignant melanoma found in nasolacrimal duct; no evidence of tumor in canaliculi or lacrimal sac
4	29/M	OS Sebaceous gland carcinoma involving lacrimal gland s/p recent DCR	Chemotherapy + En-bloc excision of lacrimal sac and NLD	EBRT	Few viable and non-viable areas of sebaceous gland carcinoma in lacrimal sac and NLD

NLD- Nasolacrimal duct, CT- Computer Tomography, DCR- Dacryocystorhinostomy, EBRT – External beam radiotherapy, HPE- Histopathology examination

Table 2: Clinical features and management in recurrent tumors of conjunctiva and eyelid extending to lacrimal drainage system

Case No	Age/ Sex	Clinical feature at presentation	Primary malignancy	Management		HPE findings
				Surgery	Radiotherapy
1	62/F	OS NLD obstruction with lacrimal sac mass	OS Sebaceous gland carcinoma of upper lid s/p complete excision with frozen section margin control (6 years back)	Extended dacryocystectomy with NLD excision	EBRT	Sebaceous gland carcinoma involving lacrimal sac and NLD; no canalicular involvement
2	75/M	OD NLD obstruction with lacrimal sac mass	OD Sebaceous gland carcinoma of lower lid s/p complete excision with frozen section margin control (3.5 years back)	Extended dacryocystectomy with NLD excision	EBRT	Sebaceous gland carcinoma involving lacrimal sac and NLD; no canalicular involvement
3	58/M	OS NLD obstruction with bloody regurgitation	OS Diffuse OSSN s/p Topical MMC x3, excision with clear margins (2.3 years back)	Extended dacryocystectomy with NLD excision	–	Squamous cell carcinoma in lacrimal sac and sac-NLD junction; no canalicular involvement
4	59/M	OD Recurrent conjunctival melanoma involving upper forniceal conjunctiva with NLD obstruction and oncorrhea	OD conjunctival melanoma s/p excision with frozen section margin control (5 years back)	Complete excision of conjunctival tumor with clear margins and Extended dacryocystectomy with NLD excision	EBRT	Malignant melanoma cells in lacrimal sac; no canalicular or NLD involvement

NLD- Nasolacrimal duct, EBRT – External beam radiotherapy, HPE- Histopathology examination

References:

1. Seregard S. Conjunctival melanoma. Survey of ophthalmology. 1998 Feb 28;42(4):321-50.
2. Khan JA, Grove AS, Joseph MP, GoodmanM. Sebaceous carcinoma: diuretic use, lacrimal system spread, and surgical margins. Ophthal Plast Reconstr Surg. 1989;5227- 234
3. Shields CL, Shields JA, Gündüz K, Cater J, Mercado GV, Gross N, Lally B. Conjunctival melanoma: risk factors for recurrence, exenteration, metastasis, and death in 150 consecutive patients. Archives of Ophthalmology. 2000 Nov 1;118(11):1497-507.
4. Esmaeli B, Wang X, Youssef A, Gershenwald JE. Patterns of regional and distant metastasis in patients with conjunctival melanoma: experience at a cancer center over four decades. Ophthalmology. 2001 Nov 30;108(11):2101-5.