Conjunctival tumours - types and causes

Tumori conjunctivale reprezintă formațiuni anormale care apar pe membrana conjunctivală a ochilor. Simptomele tumorilor conjunctivale pot include senzația de corp străin, iritație oculară persistentă sau umflături vizibile la nivelul ochilor. Cauzele exacte ale acestor tumori sunt încă necunoscute, dar expunerea la radiații solare, infecțiile virale și anumite afecțiuni oculare pot fi factori predispozanți. Tratamentul tumori conjunctivale poate implica intervenții chirurgicale, terapie cu laser sau chimioterapie, în funcție de dimensiunea și agresivitatea tumorii.

Amelanotic lesions

1. Limbic Dermoid

It is a benign congenital tumor, usually located in the inferotemporal region of the sclera, but may also involve the central cornea. The tumor is white, solid, circumscribed and may contain hair follicles. It may grow, especially at puberty. With time, intracorneal lipid deposition may occur at the anterior border of the dermoid. May be associated with eyelid coloboma, preauricular papillomas and vertebral abnormalities (Goldenhar syndrome). Surgical removal may be performed for cosmetic purposes or if the formation affects the visual axis, although a white corneal scar may persist postoperatively and cause astigmatism. A lamellar or penetrating keratoplasty may be required later.

2. Conjunctival dermolipomus

It is a congenital benign, solid yellowish-white tumor that usually occurs under the temporal bulbar conjunctiva, often superotemporally . It consists almost entirely of adipose tissue and may have hair follicles arising from the surface. It has a high association with Goldenhar syndrome. Surgical removal is avoided because of the frequent posterior extension of this tumour into the orbit. If necessary, partial resection of the anterior portion can be performed.

3. Pyogenic Granuloma

This tumor is a non-neoplastic mass, consisting of aggregates of immature blood vessels and fibroblastic stroma, lymphocytes, plasma cells and neutrophils. It is considered to be an aberrant healing form of a focal conjunctival lesion (previous surgical incision, trauma, chalazion).

4. Conjunctival lymphangioma

Probabil congenital, dar adesea nu este detectat decât in copilaria tarzie, adesea inainte de varsta de 6 ani. O leziune benignă lent progresivă care apare ca difuză, multiloculată și cu aspect de masa chistica. Leziunea se poate mări, uneori din cauza unei infecţii ale căilor respiratorii superioare si uneori umple cu sange (aspect de „chist de ciocolata”). Concomitent limfangioamele pleoapelor, orbitale, faciale, nazale sau orofaringiene pot fi prezente. Excizia chirurgicală poate fi efectuată pentru scopuri cosmetice sau funcționale, dar de multe ori trebuie repetata pentru că este dificil de realizat îndepărtarea întreaga tumoră cu o singură procedură chirurgicală. Leziunile se stabilizează adesea la vârsta adultă timpurie. Aceste leziuni nu regresează ca hemangioamele capilare.

5. Inflammatory granuloma

It can occur at any age, predominantly on the tarsal conjunctiva. It does not have a distinct clinical appearance, but patients may have associated embedded foreign body, sarcoidosis, tuberculosis or other granulomatous diseases. For systemic granulomatous diseases (e.g. sarcoidosis), conjunctival granulomas may be an excellent source of diagnostic tissue. It can closely resemble malignant conjunctival lymphoma, so biopsy is necessary to establish diagnosis and therapeutic management.

6. Conjunctival viral papilloma

Frequent multiple pedunculated or sessile lesions in children and young adults. May occur at the palpebral or conjunctival bulbar level. They are benign and are usually left untreated because of their high recurrence rate (which is often multiple) and their tendency for spontaneous resolution.

7. Squamous conjunctival papilloma

Usually a single sessile or pedunculated lesion with conjunctival location and may represent precancerous lesions with malignant potential. Wide excisional biopsy supplemented with cryotherapy at the conjunctival margin is the preferred treatment because it may be difficult to differentiate from squamous cell carcinoma.

8. Kaposi's sarcoma

It is a malignant disease with conjunctival localization. The tumour appears as a dark red conjunctival mass, frequently haemorrhagic, and may simulate conjunctival haemorrhage. Occurs predominantly in individuals with AIDS or other forms of systemic immunosuppression. This type of lesion may remit when patients are on active antiretroviral therapy. Other treatments include vinblastine, vincristine, excision, cryotherapy, interferon alpha 2b or irradiation.

9. Conjunctival Intraepithelial Neoplasia (Epithelial Dysplasia and Carcinoma in Situ)

It represents the diffuse or localized replacement of normal conjunctival epithelium with malignant cells derived from the stratified squamous epithelium. When malignant epithelial cells invade only a portion of the thickness of the epithelium, the lesion is called conjunctival squamous epithelial dysplasia. When neoplastic cells replace the entire thickness of the conjunctival epithelium, but do not invade the substance propria, the lesion is called squamous carcinoma in situ. It usually appears in individuals after middle age as a greyish-white gelatinous lesion that usually starts at the tongue. Occasionally, a papillomatous, fern-like vascular appearance develops. Usually unilateral and unifocal. May progress to squamous cell carcinoma in situ and metastasis if not treated early and successfully. Preferred treatment is excisional biopsy, followed by additional cryotherapy to the adjacent conjunctiva remaining after excision. Regular consultations are necessary to detect possible recurrences.

10. Lymphoid tumours (Benign reactive lymphoid hyperplasia)

It can occur in young and middle-aged adults, but the average age of diagnosis is 61. It usually appears as a salmon-colored, light pink lesion. It can appear in the bulbar conjunctiva, where it is usually oval, or in the fornix, where it is usually horizontal, conforming to the outline of the fornix. An excisional or incisional biopsy is performed for immunohistochemical studies (requires fresh unfixed tissue). Symptomatic benign reactive lymphoid hyperplasia can be treated by excisional biopsy or topical steroid drops. Lymphomas should be completely excised when possible without damage to extraocular muscles or excessive sacrifice of the conjunctiva. If this is not possible, an incisional biopsy is warranted and further treatment with rituximab, chemotherapy or low-dose radiotherapy is recommended. For a systemic evaluation a consultation by the internist and oncologist is required. Systemic lymphoma may develop if not already present.

11. Conjunctival osteoma

It is a rare benign choristoma, composed mainly of bone tissue, more commonly located in the superotemporal region of the fornix. It is generally detected in adolescence, when the affected individual identifies a hard epibulbar lesion following rubbing of the eye. Surgical removal may be performed for cosmetic purposes and to confirm to the patient its benign nature.

12. Conjunctival amyloidosis

Smooth, waxy, pinkish-yellow masses are seen especially in the inferior fornix when the conjunctiva is involved. There is often small associated haemorrhages. A definitive diagnosis is established after biopsy. Consideration should be given to examination for systemic amyloidosis, although most cases with conjunctival involvement are localized and solitary.

13. Conjunctival Melanoma

A cancerous tumor that arises from melanocytes normally present in the basement membrane of the conjunctival epithelium. Most conjunctival melanomas are dark brown in colour, but in some cases the lesion has an amelanotic appearance, partially or completely. The pigmentation of conjunctival melanoma is variable, but the presence of a solid epibulbar tumour, irrigated by prominent conjunctival blood vessels are key signs for diagnosis. The most common location of conjunctival melanoma is in the limbus or adjacent medial or temporal. These tumours are less likely to metastasise than tumours originating from the caruncle, lunate fold, palpebral conjunctiva or fornix, but if lymph node metastases are suspected, lymph node marking and excision is recommended when treatment against the tumour is initiated.

14. Sebaceous Carcinoma

Although usually involving the tarsal conjunctiva, this tumor may involve the bulbar conjunctiva (when there is a pagetoid invasion of the conjunctiva). This diagnosis should always be considered in elderly patients with refractory unilateral blepharoconjunctivitis.

Melanotic lesions

1. Conjunctival nevus

It frequently develops during puberty, with typical localization at the medial limbic or temporal level. Usually well demarcated with variable pigmentation. Degree of pigmentation may change with time. A key diagnostic sign is the presence of intraepithelial microcysts. Benign nevi may enlarge; however, a melanoma may occasionally develop from a nevus, and enlargement may be an early sign of malignant transformation. Palpebral conjunctival nevi are rare, but when they occur acquired primary melanosis and malignant melanoma should be considered in such lesions. A baseline photograph of the nevus should be taken; and the patient should be followed every 6 to 12 months. Surgical excision is elective. Nevi may be amelanotic.

2. Ocular and Oculodermal Melanocytosis

A congenital episcleral (not conjunctival) lesion, as demonstrated (after topical anaesthesia by moving the conjunctiva back and forth over the area of pigmentation with a cotton swab the conjunctival pigmentation will move with the conjunctiva). Usually the lesion is unilateral, blue-gray and often accompanied with a darker iris and a darker ipsilateral choroid. In oculodermal melanocytosis , also called Ota nevus, the periocular skin is also pigmented. These lesions are slightly pigmented at birth, but may become even more pigmented at puberty. Both conditions predispose to melanoma of the uveal tract, orbit and brain (most common in whites) and glaucoma. It is estimated that 1/400 affected patients develop uveal melanoma.

3. Primary Melanosis Acquired

It is an intraepithelial lesion caused by melanocytic hyperplasia to neoplasia, mainly found in adults, arising from a previously unaffected portion of the conjunctiva. It appears as flat, brown, cyst-free pigmentation ects in the conjunctiva during or after middle age and almost always occurs in whites. Classically, about 10% to 30% of these lesions progress to melanoma. Malignant transformation should be suspected when an increase or enlargement of the vasculature in one of these areas develops. Management options depend on the size of the lesion. If it is 1 to 2 hours old watch, careful observation and photographic comparisons are recommended. If it is 2 to 4 clock hours, then excisional biopsy followed by cryotherapy is recommended. If size is >4 clock hours, then incisional biopsy plus cryotherapy is performed.

4. Malignant Melanoma

It usually appears from middle age onwards. The lesion has the appearance of a brown, well-vascularised nodular mass. Often a large conjunctival vessel for the tumor is present. It may develop againfrom a nevus or acquired primary melanosis. Check for underlying ciliary body melanoma (examination of dilated fundus, transillumination and ultrasonographic biomicroscopy). Intraocular and orbital extension may appear. Excisional biopsy using a "no-touch technique" (with additional cryotherapy) is performed unless there is intraocular or orbital invasion. In advanced cases, orbital exenteration is required. Sentinel lymph node biopsy is recommended for early detection of metastases.

Less Common Causes of Conjunctival Pigmentation

Ocronosis with alkaptonuria: Autosomal recessive enzyme deficiency. Occurs in young adults with arthritis and dark urine. Pigment is in the sclera, which can cause a " pigmented pinguecula. " Bluish-black discoloration of the skin of the ear.

Argyrosis: Silver deposition causes black discoloration. Patients may have a history of long-term use of silver nitrate drops.

Haemochromatosis: Can cause hyperpigmentation of the skin. It can be complicated by diabetes, also called bronchial diabetes.

Ciliary staphyloma: Scleral thinning with uveal highlighting.

Adrenochrome deposits: Occurs after long-term use of epinephrine or dipivefrine.

Mascara application: Usually occurs in the lower fornix and becomes trapped in the epithelium or swollen.

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Oftalmologie Timisoara - Doctor oftalmolog Timisoara - Dr. Yasar Servet, Medic primar oftalmolog

Primary Ophthalmologist Dr. Yasar Servet

Dr. Servet Yașar is an expert with more than 15 specialist credentials in the field of ophthalmology, attending courses in countries such as Turkey, Cyprus, Czech Republic and the United States. With over 11 years of experience in ophthalmology, including 8 years spent in Turkey and over-specialization in Vitroretinal Surgery, Dr. Yașar has been with the team of Prof. Dr. Munteanu Ophthalmology Center since 2020. Yașar is experienced in cataract surgery, glaucoma surgery, pterygium surgery and other anterior segment surgery, as well as eyelid aesthetic surgery, botox applications, strabismus surgery and vitreoretinal surgery.

- 20th September 2022

Conjunctival tumours