Ординатура / Офтальмология / Английские материалы / Ocular Pathology_6th edition_Yanoff, Sassani_2009

A

C

9.The net result of the changes associated with mela- noma-associated spongiform scleropathy may be to facilitate tumor extension

MELANOTIC TUMORS OF THE OPTIC DISC AND OPTIC NERVE

Melanocytoma (Magnocellular Nevus of the

Nerve Head)

I.A melanocytoma (Fig. 17.51A) is a nevus composed entirely of maximally pigmented, plump, polyhedral nevus cells that contain giant melanosomes (macromelanosomes).

Rarely, melanocytomas may be associated with bone formation.

A.They may be found in any part of the uvea (Fig. 17.52; see also Fig. 17.51B and C) in addition to the optic disc, and even in the sclera.

B

Fig. 17.50 Metastatic uveal tumors. A, Clinical appearance of metastatic carcinoma from breast. Usually, metastatic tumors are easily recognized clinically. B, Occasionally metastatic carcinoma is mistaken for melanoma, resulting in enucleation of the eye. The presence of significant stroma (pink areas between collections of tumor cells) is a tip-off that a tumor is metastatic. C, Increased magnification shows sheets of malignant epithelial cells.

B.Because a melanocytoma is a type of nevus, a more appropriate term for the tumor is magnocellular nevus.

C.When in the anterior uvea, a melanocytoma can present with glaucoma.

II.Usually, the tumor is located at the inferior temporal aspect of the optic disc in patients who have “dark” fundi.

A.The tumor usually fills less than half the optic disc, but rarely, it may fill the whole disc and may even “spill out” into the adjacent choroid and retina.

Rarely, a melanocytoma of the optic disc may cause a central retinal vascular obstruction. A bilateral case in a 10-month-old infant has been reported.

B.It may extend into the vitreous to an alarming degree, either directly or as a dispersion of pigment.

III.Whereas the ratio of white patients to black patients a ected with uveal malignant melanoma is 15 :1, the white-to-black ratio for melanocytoma of the optic disc is 0.1 :1.

Even when a melanocytoma is present in white patients, it is usually seen in patients who have dark fundi. The melanocytoma

A

C

frequently involves the adjacent neural retina for a short distance and clinically is seen to end in the neural retina with feathered edges.

IV. It is a benign lesion.

A.In about 11% of cases it may enlarge.

B.It probably has the same very low malignant potential of any nevus, and when it occurs in a white person, it has a higher potential for malignant change than in a black person.

V.Melanocytoma may undergo necrosis resulting in such atypical features as opaque media, pain, and inflammation, thereby mimicking a necrotic malignant melanoma.

Malignant Melanoma

I.Primary malignant melanomas of the optic disc are exceedingly rare.

II.Most malignant melanomas thought to be primary in the nerve head are actually primary in the juxtapapillary choroid with secondary invasion of the optic disc.

B

Fig. 17.51 Melanocytoma of optic disc. A, Characteristic black color and inferior temporal location of tumor in 37-year-old black woman. B, A histologic section of a ciliary body melanocytoma shows that the tumor is composed of maximally pigmented nevus cells. C, Bleached section demonstrates benign, plump, polyhedral nevus cells. A melanocytoma is simply a nevus that is entirely composed of maximally pigmented, plump, polyhedral nevus cells. (Modified with permission from Scheie HG, Yanoff M: Arch Ophthalmol 77:781, 1967. © American Medical Association. All rights reserved.)

III.Approximately 5% of choroidal malignant melanomas invade the optic disc and nerve (see Fig. 17.45C and

D).

MELANOTIC TUMORS OF THE ORBIT

I.Primary melanoma of the orbit is quite rare.

Most primary orbital melanomas occur in white patients and are associated with blue nevi.

II.Malignant melanoma of the orbit may be associated with congenital oculodermal melanocytosis (the nevus of Ota) or with a primary melanoma of the lacrimal sac.

Primary melanoma of the orbit should be differentiated from clearcell sarcoma (melanoma of soft parts), which produces melanin, is S-100-positive, and is probably a member of the family of malignant peripheral nerve cell tumors.

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