A corneal diffuse neurofibroma as a manifestation of von recklinghausen disease.
OBJECTIVE
To report a case of a primary corneal diffuse neurofibroma in a patient with von Recklinghausen disease (NF-1).


METHODS
Case report. A physical examination and histopathology were performed. The immunohistochemical studies were performed using an avidin-biotin-peroxidase complex technique on formalin-fixed and paraffin-embedded tissue. Histologic sections from corneal tissue were incubated with primary antibodies against vimentin and S-100 protein. A complementary ultrastructural study of the same formalin-fixed and paraffin-embedded tissue was made.


RESULTS
The ophthalmologic examination revealed a yellowish-white elevated mass that involved the supratemporal cornea but not the limbus. Histologic study showed a tumor of the peripheral nerve sheath, a diffuse neurofibroma in the corneal stroma, and proliferation of spindle cells with markedly elongated nuclei. Cells comprising the tumor reacted with vimentin and S-100 protein, and the ultrastructural studies revealed myelinated nerve fibers confirming the diagnosis.


CONCLUSIONS
The development of a primary diffuse neurofibroma in the cornea of patients with von Recklinghausen disease is possible. The present case supports the statement that neurofibromas arising from the peripheral nerve sheath may involve any part of the body.
Sánchez-Huerta
Valeria
V
Asociación Para Evitar la Ceguera en México, I.A.P., Hospital Dr. Luis Sánchez Bulnes, San Lucas Coyoacán 04030, México City, México.
Rodríguez-Reyes
Abelardo A
AA
Hernández-Quintela
Everardo
E
Ramírez
Manuel
M
Rodríguez-Martínez
Héctor A
HA
Naranjo-Tackman
Ramón
R
D003315:Cornea / Q000473:pathology; D003316:Corneal Diseases / Q000209:etiology*; D005134:Eye Neoplasms / Q000150:complications* / Q000175:diagnosis; D006801:Humans; D008297:Male; D008875:Middle Aged; D009455:Neurofibroma / Q000209:etiology*; D009456:Neurofibromatosis 1 / Q000150:complications* / Q000175:diagnosis; D016896:Treatment Outcome; D014792:Visual Acuity