Because pam is common, its transformation to conjunctival melanoma must be extremely rare, and biopsy of all pam lesions is unwarranted. If there is a larger quantity than normal of pigment in the tissues of the eye, the condition is referred to as melanosis bulbi melanosis oculi or primary acquired melanosis when on the conjunctival epithelium and limbus. It is recommended that intraepithelial melanocytic proliferation be adopted for histopathologic diagnosis. Limbal melanoma with adjacent corneal and conjunctival epithelium. It can be clinically confused with more common conditions, such as conjunctival nevus or primary acquired melanosis. Conjunctival melanoses are a common clinical finding of flat granular melanotic pigmentation of the conjunctival epithelium. An abnormal accumulation of melanin pigment in the skin or other tissues. Topical mitomycin chemotherapy for conjunctival malignant. Conjunctival metastasis as an initial sign of small cell. Conjunctival melano ma tends to locally recur, seed to distant parts of the conjunctiva, and systemically metastasize to regional lymph nodes. In some instances, a conjunctival nevus can assume an irregular or diffuse growth pattern. Dataset for the histopathological reporting of conjunctival. Squamous cell carcinomas arise from progressive stages of mild, moderate, and severe dysplasia. Although subconjunctival or orbital recurrences were noted, topical mitomycin chemotherapy warrants further investigation as an alternative treatment for primary acquired melanosis with atypia and conjunctival malignant melanoma.
Melanocytic lesions of the conjunctiva archives of. The classical signs are unilateral facial port wine stain. We audited our results with 76 invasive conjunctival melanomas. Conjunctival melanocytic intraepithelial neoplasia clinical features patients with cmin present in adulthood with a unilateral area of conjunctival melanosis synonymous pam figure 2 28,29. The pigmented areas of eye such as the choroid and iris are most commonly affected. Conjunctival primary acquired melanosis pam is an acquired flat, painless noncystic pigmented lesion of the conjunctiva 1,2. Dataset for the histopathological reporting of conjunctival melanoma and melanosis october 2007 coordinator. No complications that would preclude use of our dose regimen were noted.
Conjunctival malignant melanoma is a pigmented lesion of the ocular surface. Conjunctiva tumor an overview sciencedirect topics. Conjunctival melanoma is a rare but potentially deadly ocular malignancy. What is primary acquired melanosis of the conjunctiva. Conjunctival melanoma arising from primary acquired. Management of primary acquired melanosis, nevus, and conjunctival melanoma andrew kao, md, armin afshar, md, michele bloomer, md, and bertil damato, md, phd photo courtesy of lynn e. Clinical features of benign vs malignant conjunctival. Primary acquired melanosis of the conjunctiva trans am ophthalmol soc vol 105 2007 70 our philosophy for pam management is based on longterm clinical experience and the resu lts of this study. The management of conjunctival melanoma is difficult because of the rarity of the disease, confusing. In general, tumors measuring greater than 2mm in thickness are at significant risk for lymph node metastasis. Many pathologists would consider this melanoma in situ. Conjunctival nevi can account for up to 52% of the diagnoses from all.
The most common conjunctival malignancy in adults was squamous cell carcinoma, followed by melanoma, and pagetoid change associated with sebaceous gland carcinoma. Pigmentation is usually asymmetric and poorly circumscribed and often has a reticulated quality. In contrast, the concept of conjunctival melanosis and the restricted use of the term melanoma to. Conjunctival primary acquired melanosis pam accounts for 11% of all conjunctival tumors and 21% of melanocytic lesions. Reprinted from university of utah, peter debry, kwok li, and nick mamalis, md, ophthalmic pathology. Review of the literature jerman m alqahtani, md background.
Tm these tumors are primarily related to ultraviolet uv exposure. Benign conjunctival melanocytic intraepithelial neoplasia is also known as primary acquired melanosis pam without atypia or melanocytic intraepithelial neoplasia without atypia. Feline conjunctival melanoma 46 cases in the coplow database. Secondary acquired conjunctival melanosis resulting from. Progression of primary acquired melanosis with atypia during. Chronic condition combining erythema with telangiectasia and acnelike lesions. Malignant tumor of melanocytes originating in the conjunctiva. Although most of the melanocytic lesions are benign, some can carry a potential for malignancy, which makes it important to distinguish between these various conjunctival lesions14. Pigmentation of the basal layer of the epithelium without a proliferation of melanocytes. Conjunctival melanoma and melanocytic intraepithelial. Pdf premalignant melanosis of the conjunctiva and the. What is the cause and treatment for conjunctival malignant.
Conjunctival melanoma is a disease of middleaged and elderly persons, with very few cases reported in children. Conjunctival melanocytic nevi are similar to those occurring in the skin and are benign, acquired or congenital, circumscribed melanocytic proliferations. It is an uncommon but potentially devastating tumor that may invade the local tissues of the eye, spread systemically through lymphatic drainage and hematogenous spread, and recur in spite of treatment. Primary acquired melanosis of the conjunctiva pam is a potentially serious melanocytic lesion that can lead to the development of melanoma. Conjunctival pigmented lesions college of optometrists. In this condition, the melanocytes are increased in number but have a normal appearance and are in their normal location in the deepest layer of the epithelium.
The cmgs are guidelines on the diagnosis and management of a range of common and rare, but important, eye conditions that present with varying frequency in primary and first contact care. Com is the second most frequent malignant neoplasm of the conjunctiva after squamous cell carcinoma. Pigmented conjunctival cancers primary acquired melanosis. Primary acquired melanosis appears as flat, patchy, noncystic pigmentation in the conjunctival epithelium and can remain.
Fortyone cases of conjunctival primary acquired melanosis pam were studied to determine the frequency of progression to malignant melanoma and. Many terms have been applied to conjunctival atypical intraepithelial proliferations of melanocytes, such as precancerous melanosis, benign acquired melanosis, primary acquired melanosis pam with atypia a clinicopathologic term, conjunctival melanocytic intraepithelial neoplasia cmin and insitu melanoma. The term primary acquired melanosis pam was adopted by the world health organization in 1980 representing flat, brown, intraepithelial conjunctival lesions, in its international histological classification of tumours 6. A, initial biopsy specimen from the pigmented area in figure 1a showing atypical melanocytes within the lower half of the conjunctival epithelium arrows, considered to be primary acquired melanosis with moderate atypia hematoxylineosin, original magnification. Conjunctival pam is often precancerous and leads to lifethreatening malignant melanoma. A clinicopathologic study of 41 cases of primary acquired melanosis has provided information concerning the rate of progression to melanoma and has provided histologic parameters useful in predicting which lesions are likely to progress to malignant melanoma. It is not uncommon in clinical practice for an ophthalmologist to identify, during routine examination, unilateral or bilateral areas of flat stippled pigmentation in the bulbar or palpebral conjunctiva in middle aged or elderly patients.
Pam is a controversial term used to describe increased brown pigmentation of the conjunctiva, usually flat and speckled, associated histologically with an increased number of melanocytes in the epithelium. When the histiocytes merge, they form a foreign bodytype giant cell. The most commonly biopsied benign pigmented conjunctival lesion is the nevus2,5. When mitomycin chemotherapy was used as an adjuvant to excision and cryotherapy, 2 40% of 5 patients experienced tumor recurrence at a mean of 4. Conjunctival melanosis now differentiated histologically into hypermelanosis and conjunctival melanocytic intraepithelial neoplasia cmin cases formerly classified as pam are now classified as cmin. Melanocytic lesions clinically present as flat or nodular and it is in. To report a case of a conjunctival metastasis as the initial manifestation of small cell lung cancer. Impression cytology features of conjunctival nevi reported as. Dramatic regression of conjunctival and corneal acquired. Towards a more accurate assessment of the malignant potential.
The management of conjunctival melanoma is difficult because of the rarity of the disease. It is present at the limbus for up to 360 degrees and at the exposed interpalpebral bulbar conjunctiva. However, the classification scheme differs in the conjunctiva because of differences in anatomy, prognosis, and management. Conjunctival malignant melanoma is rare malignancy and most arise from a condition called primary acquired melanosis pigmentation. Ocular and periocular tumors in cats richard r dubielzig. Congenital melanosis definition of congenital melanosis by. Pdf conjunctival melanosis with retinal vessel tortuosity. Update on conjunctival pathology mudhar hs indian j ophthalmol. Conjunctival primary acquired melanosis pam is a painless flat brown spot on the eye that might superficially look like a freckle.
Anatomic distribution of feline primary ocular neoplasia n 2599 82 % 12. Staging according to the sixth edition of the tumour node metastasis tnm system did not correlate well with tumour extent and outcome. Several types of naevi can be identified in the conjunctiva, with some sitespecific peculiarities. Pam usually occurs unilaterally in middleaged individuals mostly caucasians and accounts for 11% of all conjunctival tumors and for 21% of all conjunctival melanocytic lesions 2. Other pediatric conjunctival tumors have been described in the literature. P ediatric conjunctival tumors are a diverse group of lesions.
He explained everything i needed to know about my issue with detail and attention, putting me at ease and giving me confidence to handle this problem for the rest of my life. Progression of conjunctival primary acquired melanosis pam. Typically racial melanosis is bilateral, asymmetric, occurs in the interpalpebral fissures, and does not contain cysts4. Unlike primary acquired melanosis, racial melanosis tends to involve both eyes and is typically present for the entire life of the patient. Blue nevi of the conjunctiva are lesions that have a low risk for malignant transformation but can appear clinically similar to primary acquired melanosis or melanoma. To evaluate clinical features and risks for transformation of conjunctival primary acquired melanosis pam into melanoma. The pigmentation can have been unrecognised for many years and be static but in a minority of cases the process may progress to malignant melanoma.
This paper aims to stimulate debate on the terminology, classification, grading and staging of conjunctival melanosis and melanoma. Conjunctival nevus, primary acquired melanosis pam, or melanoma sometimes has amelanotic component which should be noted. Pigmented lesions that arise from the conjunctiva include nevus, complexionassociated melanosis cam, primary acquired melanosis pam, and malignant melanoma. Conjunctival primary acquired melanosis wills eye hospital. An internet ocular pathology archive, with permission from university of utah ophthalmic pathology.
Conjunctival primary acquired melanosis is the most important precursor of conjunctival malignant melanoma. A 50yearold man without known systemic disease developed a conjunctival mass in his right eye. Precancerous and cancerous melanosis of the conjunctiva. These tumors have a low malignant potential but may behave more aggressively in the. Blue nevi of the conjunctiva are rare and represent 0. The melanosis can extend to the skin if the lesion involves the palpebral conjunctiva. Sotereanos, dmd, mst conjunctival melanosis is a term used to describe a variety of melanocytic lesions of the conjunctiva. This conjunctival melanoma likely developed from primary acquired melanosis pam with atypia and then grew onto the superficial surface of the cornea. Melanosis is an inadequate and misleading term because it does not distinguish between conjunctival intraepithelial melanin overproduction hyperpigmentation and intraepithelial melanocytic proliferation. Melanocytic lesions of the conjunctiva have a similar morphologic appearance to those of the skin. In addition to its rarity, there are several obstructive factors compounding our understanding of com. Jan 06, 2017 the same argument can be used for the term conjunctival melanoma in situ, which has been controversial for years, but really should be recognized and treated as such. Clinical characterization of primary acquired melanosis. Dr hardeep singh mudhar, royal hallamshire hospital unique document number g054 document name dataset for the histopathological reporting of conjunctival melanoma and melanosis version number 1.
Finger says, think of sunglasses as sun block for your eyes. As seen below, primary acquired melanosis typically affects one eye, in middleaged, fairskinned people. The incidence of conjunctival melanoma is based on 3 epidemiologic studies in germany, sweden and the netherlands with an incidence of. The ozone layer is thinning and the incidence of conjunctival melanoma is increasing. Several types of naevi can be identified in the conjunctiva, with some sitespecific peculiarities, familiarisation with which. Primary acquired melanosis with atypiaconjunctival melanocytic intraepithelial. It is an uncommon but potentially devastating tumor that may invade the local tissues of. This brown pigmentation is flat and irregular and can occur anywhere in the conjunctival or corneal epithelium. This definition includes the ephelis or freckle, so called complexionassociated conjunctival pigmentation, and benign acquired melanosis because the three entities may be indistinguishable clinically and under the microscope. A slitlamp examination may reveal subclinical melanosis around the clinically.
Clinical professor of ophthalmology, college of physicians and surgeons, columbia university, and the institute of ophthalmology of the presbyterian hospital, new york new york i have often commented on the high and rising standard of ophthalmology in the west. Management of primary acquired melanosis, nevus, and. However, a number of other lesions have a similar appearance but a different source, such as pigment deposits from silver and iron. Surgical techniques for conjunctival and corneal tumors. Conjunctival melanoma arising from primary acquired melanosis. Conjunctival melanosis and melanoma robert folberg, md ian w. Primary acquired melanosis pam is an acquired pigmentation of the conjunctival epithelium, a preinvasive. Common conjunctival lesions olivier continuing medical. Pdf melanocytic lesions of the conjunctiva researchgate. Sep 15, 2009 to evaluate the effect of topical interferon. However, a number of other lesions have a similar appearance but a different source, such as pigment deposits from. A large drawing of the conjunctival tumor replicating the extent of the lesion in the eye is used in all cases in the office and at the time of surgery. Melanic lesions range from nevus to acquired melanosis and melanomas.
Premalignant melanosis of the conjunctiva and the cornea in xeroderma pigmentosum article pdf available in british journal of ophthalmology 762. The signs of pam are irregular, unifocal or multifocal areas of. Dr jakobiec takes issue with our term hypermelanosis, maintaining that melanosis should suffice, since the conjunctiva is normally nonpigmented. He underwent incisional biopsy of the tumor and systemic evaluation. The pigmentation in pam may wax and wane and even disappear. Cmin score of 0 is melanosis only cmin score of 1 is pam with mild atypia cmin score of 2 3 is pam with moderate atypia. Conclusions topical mitomycin chemotherapy was found to induce regression of conjunctival melanoma and primary acquired melanosis with atypia. Nine patients with histologically proven acquired melanosis with atypia andor conjunctival melanoma were treated with recombinant ifn. Such an atypical nevus can occupy a large portion of the conjunctiva and lead to diagnostic confusion with pam, melanoma and, in the case of amelanotic lesions, with lymphoma or lymphangioma. Nevi do not involve the cornea, fornices, or palpebral conjunctiva and are well defined and mobile over the sclera. Page 3 tumors nevus a benign pigmented conjunctival tumor of the conjunctiva. A congenital increase in the number, size, and pigmentation of melanocytes of the uvea is associated with an increased number of pigmented melanocytes in the sclera and episclera.
Pdf sturgeweber syndrome sws is a rare neurooculocutaneous disorder. Conjunctival melanoma british journal of ophthalmology. Conjunctival melanoma com constitutes about 2% of all ocular malignancies and 5% of all ocular melanoma. In 1980, the who proposed the term pam to describe this disorder. Excised corneal tumor with thin epithelial covering merging with underlying. Differential diagnosis of these lesions is difficult and there is much confusion in the literature regarding their. Conjunctival tumors in children are typically benign and most often represent nevus, brlh, primary acquired melanosis, dermoid, and dermolipoma. A simple biopsy can determine whether a pigmented conjunctival tumor is a nevus, primary acquired melanosis, or conjunctival melanoma. Nevi and pam without atypia have a benign clinical course and are similar to nevi and lentigos in the skin. It can be found in children and adults and typically occurs on only one eye.
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