LACRIMAL SAC TUMORS JOSEPH C. FLANAGAN, MD PHILADELPHIA, PENNSYLVANIA and

D. PARKER STOKES, MD BY INVITATION

FLORENCE, SOUTH CAROLINA Tumors of the lacrimal sac are a rare entity in ophthalmology, but represent a potentially life-threatening situation that can easily be overlooked. This paper will review the literature and describe clinically and pathologically the more common types of lacrimal sac tumors, and give a description of the less common types of primary and secondary tumors of the lacrimal sac and a case presentation.

LACRIMAL SAC TUMORS

HISTORICALLY, tumors of the lacrimal sac have been described as early as 1772 by Janin. 1 More recent attempts to collect and classify case reports of tumors of the lacrimal sac have been made by Radnot and Gall 2 in 1966, and by Scheneck and co-workers3 in 1973. Radnot and Gall 2 reviewed a total of 184 cases of which 46 were pseudotumors or inflammatory granulomas. Of the remaining 138 tumors, there were 86 epithelial tumors (22 benign, 64 malignant),

Submitted for publication Oct 4, 1977. From the Department of Ophthalmology Thomas Jefferson University School of Medicin'e' Oculoplastic Service, Wills Eye Hospital' L~nkenau Hospital, Philadelphia (Dr Flanaga~)' McCleod Memorial Hospital, Florence, se (Dr Stokes). Presented at the Eighty-second Annual Meeting of the American Academy of Ophthalmology and Otolaryngology, Dallas, Oct 2-6, 1977. Reprint requests to Lankenau Hospital Suite 135 Philadelphia, PA 19151 (Dr Flanagan). '

31 mesenchymal tumors (5 benign 26 malignant), 6 pigmented tumors' and 15 reticuloses. Scheneck et al3 added 21 cases of which one was a pseudotumor and 20 were neoplasms (15 of which were malignant). In these reviews, transitional cell epidermoid carcinoma was the most common type of malignant tumor. Less common primary tumors of the lacrimal sac are those of lymphoreticular tissue and melanomatous tumors. Lymphomas of the lacrimal sac are commonly associated with leukemia. Lymphosarcomas usually occur in the younger age group. Melanomatous tumors of the lacrimal sac are not common. Nine cases of melanoma have been reported, eight of which were malignant; the only benign melanoma was reported in 1953 by Eitrem.4 Rare primary tumors of the lacrimal sac include fibromas hemangiomas, and neurofibr(jm~s. Metastatic carcinoma to the lacrimal sac is rare. Rollet5 reported a malignant melanoma of the lacrimal sac in a patient who had a primary choroidal neoplasm. Secondary tumors of the lacrimal sac can also occur by means of direct spread from tumors of the lower lid palpebral conjunctiva,6 and th~ paranasal sinuses, especially the ethmoids. It is evident from these statistics (Table) that approximately

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LACRIMAL SAC TUMORS LACRIMAL SAC TUMORS

TYPE

RADNOT AND GALL2

SCHENECK ET AL3

1975*

4

1 2

27 76

TOTAL

Epithelial benign malignant

22 64

10

Mesenchymal benign malignant

5 26

1 2

0

1

6 29

Melanomas benign malignant

1 5

0 3

0 0

1 8

Reticuloses

15

0

1

16

Pseudotumors

46

1

2

...1fl

Total

184

21

7

212

Total malignant

110

15

3

128

I

·Wills Eye Hospital, Philadelphia.

25% of lacrimal sac tumors are pseudotumors, the rest being benign and malignant tumors. The malignant tumors strongly outweigh the benign forms. CLINICAL PRESENTATION

The clinical presentation of tumors of the lacrimal sac differs little from polyps or malignant neoplasms. A biopsy specimen is the only way to determine the cause of a lacrimal sac mass with any degree of certainty. Simple tumors, typified by the lacrimal polyp, go through two, and occasionally three, clinical phases. The first is that of pseudodacryocystitis, and the second is tumor formation. Visible extension is a third, but rare, clinical phase.

Polyps of the lacrimal sac usually first appear as a chronic inflammation of the sac that may lead to suppuration. In this stage, pus or mucopurulent material can be expressed from the puncta. In all cases, a constant and usually long history of epiphora is noted, suggesting an inflammatory stenosis. Frequently the tumor is discovered during a surgical attempt to relieve the inflammatory condition. Pain and tenderness is minimal. The growth rate of these tumors is extremely variable. Polyps of the lacrimal sac, like papillomas elsewhere, have a tendency to relapse into malignancy; consequently, polyps of the lacrimal sac should be surgically excised. Ryan and Font,7 in a review of 27 primary epithelial neoplasms of the lacrimal sac, found that seven of the 18 papillomas

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were malignant or became so. Over 50% of the malignant cases had recurrences after local excision. Two of the 27 died as a result of local invasion of the tumors. Ashton et al,8 in a comprehensive review of the subject in 1952, made the statement that papillomas arising from the lacrimal sac epithelium are probably malignant from the beginning. Malignant tumors have the same initial clinical history as benign tumors and evolve through three stages. The first stage is apparent simple dacryocystitis with epiphora, followed by tumor formation, and finally extension of the tumor. Malignant tumors, especially epitheliomas and lymphosarcomas, may first appear as simple dacryocystitis for prolonged periods of time (up to several years). Epiphora is a constant finding, as in the benign tumors. Pain is often a symptom in malignant tumors but certainly not a consistent finding. Blood-stained discharge from the puncta should always be regarded as a significant finding whether spontaneous, secondary to pressure on the sac, or after irrigation; eventually a swelling above the medial canthal ligament is suggestive of a lacrimal sac tumor, whereas a swelling below the ligament is common in obstructive dacryocystitis. It is at this stage that surgical treatment is usually recommended. The clinical stage of extension, seen commonly with malignant tumors, is characterized by a local fluctuant and edematous swelling followed by skin involvement. Ulceration of the skin about the lacrimal sac may ensue. At this stage the diagnosis of a malignancy is certain. Regional adenopathy may develop, with involvement of the preauricular, submaxillary, and cervical

OPHTH

AAOO

nodes being the most common. Distant metastases do occur, but local spread is more common with ulcerative destruction of the face , nose, ethmoidal and maxillary sinuses, orbit, and finally intracranial extension. Recurrences are common with both epitheliomas and sarcomas. Despite wide excision and repeated radiation therapy, these tumors may recur and subsequently lead to the death of the patient. In addition to the clinical features of lacrimal sac tumors, there are several methods of investigation that might aid the physician in diagnosing such a tumor. Examination of the nose revealing papillomas (polyps) should alert the ophthalmologist to the likelihood of lacrimal sac polyp formation. Roentgenogram studies including polytomography of the lacrimal fossa and surrounding bony structures may be helpful. Dacryocystography is an extremely useful study and should be considered an essential preoperative procedure in cases where the diagnosis of a lacrimal sac tumor is being considered. A characteristic dacryocystogram, as described by Veirs,9 shows a distended sac shadow, uneven or mottled density of the contrast media, and patency with residual media present 30 minutes after injection. The diagnostic triad for primary tumors of the lacrimal sac consists of a dacryocystitis in a patent system, an irreducible mass (especially if it develops above the medial canthal tendon), and a characteristic dacryocystogram. PATHOLOGY

Epithelial tumors of the lacrimal sac have been classified by Harry

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LACRIMAL SAC TUMORS

and Ashton8 as (1) transitional cell papilloma, (2) intermediate type, and (3) transitional cell carcinoma. Histopa thologically, papillomas show true stratification with uniformity of cell types. Their propensity toward malignant change must be considered in their treatment. The intermediate transitional cell epithelial tumors apparently represent a stage of tumor development between the benign and malignant forms. Histopathologically they show a more irregular epithelium with

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squamous metaplasia and prominent mitotic figures. There is no infiltration of the subepithelial tissues and the basement membrane remains intact. Transitional cell carcinoma represents the malignant end of the continuum of epithelial tumors. Histopathologically these tumors show marked cellular pleomorphism with conspicuous mitotic figures and invasion through the basement membrane into the underlying stroma (Fig 1 and 2).

Fig I-Marked cellular pleomorphism with conspicuous mitotic figures (hematoxylin-eosin, moderately reduced from x250).

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OPHTH AAOO

Fig 2-Cords of epithelial cells invading underlying stroma (hematoxylin·eosin, moderately reduced from X50).

CASE REPORT A 47-year-old woman's chief complaint was a tearing of the left eye for a period of three months. Ocular examination was normal except for epiphora and a nontender mass over the left lacrimal sac. Irrigation of the left nasolacrimal system was unsuccessful, with reflux of blood-stained mucoid materiaL A dacryocystogram did not reveal retained contrast media but did show evidence of a soft tissue mass arising from the inner aspect of the orbit with questionable involvement of the ethmoid air cells. Tomograms revealed similar findings. A clinical diagnosis of tumor of the lacrimal sac with possible extracystic extension into the ethmoid air cells was made. Surgical exploration revealed a firm grayish mass involving the entire lacrimal sac

and the ethmoid air cells. Frozen sections were reported as squamous cell carcinoma. A dacryocystectomy and exenteration of the ethmoid air cells was performed. Permanent sections were reported as a poorly differentiated malignant epithelial tumor consistent with transitional cell carcinoma (Fig 1 and 2). After an uneventful postoperative course, the patient received a total of 6,000 rads over a 45-day period beginning 12 days after surgical treatment. The patient has been followed for four years with no evidence of recurrence. Ocular examinations have remained normal except for decreased tear production on the involved side, measured by Shirmer testing. This keratitis sicca is secondary to radiation; however, the patient has been comfortable using artificial tears four times a day and ointment at bedtime.

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Despite wide excision and postoperative irradiation, the recurrence rate within a five-year period approaches 50%. This figure was reported by Spaeth1(l in 1969 and by Ryan and Font7 in 1973. Although the five-year survival rate differs from study to study, the most favorable results show only 85% alive and well five years postoperatively. Spaeth 10 believed that more than 50% of the patients with recurrent primary epithelial carcinoma of the lacrimal sac were dead within five years. SUMMARY

A high index of suspicion IS important in the early diagnosis and treatment of potentially lethal tumors of the lacrimal sac. The triad of a mass above the medial canthal tendon, chronic dacryocystitis that irrigates freely, and bloody reflux on irrigation should alert the ophthalmologist of this potentially fatal situation. Complete surgical excision and postoperative irradiation are important in the treatment of malignant tumors. Epithelial tumors are the most common lacrimal sac tumors and are classified as transitional cell papilloma, intermediate type, and transitional cell carcinoma.

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Key Words: Lacrimal sac tumor; transitional cell carcinoma; dacryocystectomy; dacryocystogram; polyp; inflammatory pseudotumor.

REFERENCES 1. Janin de Combe·Blance J, cited by Duke·Elder S (ed): System of Ophthal· mology, vol 13, The Ocular Adnexa: Part II, Lacrimal, orbital and para·orbital diseases. St Louis, CV Mosby Co, 1974, p 733.

2. Radnot VM, Gall J: Tumoren des tranensacks. Ophthalmologica 151:2·22, 1966. 3. Scheneck NL, Ogura JR, Pratt LL: Cancer of the lacrimal sac: Presentation of five cases and review of the literature. Ann Otol Rhinol Laryngol 82:153·161, 1973. 4. Eitrem E: Innocent, pigmented nae· vus·cell tumor (melanoma) of the lacrimal sac. Acta Ophthalmol 31:283·285,1953. 5. Rollet E: Episcleral lymphoma and double·sided lymphoma of the lachrymal gland. Arch Ophthalmol 34:194, 1905. 6. Spaeth EB: Carcinomas in the region of the lacrimal sac. Arch Ophthalmol 57:689·693, 1957. 7. Ryan SJ, Font RL: Primary epithelial neoplasms of the lacrimal sac. Am J Ophthalmol 76:73·88, 1973. 8. Ashton N, Choyce DP, Fison LG: Carcinoma of the lacrimal sac. Br J Oph· thalmol 35:366·376, 1951. 9. Veirs ER: The Lacrimal System. St Louis, CV Mosby Co, 1971, p 81. 10. Spaeth EB: A surgical technique for lacrimal sac malignancy. Trans Ophthalmol Soc UK 89:351·354, 1969.