Intraocular leiomyomas are uncommon and usually occur in the ciliary body. Primary leiomyoma of the iris is both rare and a difficult diagnosis to make, given melanocytic tumors are more common and may be amelanotic. The somewhat controversial diagnosis of iris leiomyoma requires further confirmation by immunohistochemistry and electron microscopy. Herein, we describe a 58-year-old man with a 2-mm round translucent pink lesion of the iris. The tumor was excised by sector iridectomy. Immunohistochemistry showed positivity for both smooth muscle actin and desmin and negativity for S-100, HMB45, SOX10, MelanA, CD31, CD34, and h-caldesmon. Epstein-Barr virus-associated smooth muscle tumor was excluded by chromogenic in situ hybridization–Epstein-Barr virus–encoded RNA. Ultrastructural analysis showed cytoplasmic myofilaments with focal fusiform densities and micropinocytotic vesicles. Our review of previous literature confirmed the unusual nature of this tumor. Primary iris leiomyoma should be considered in the differential of an amelanotic S-100-immunonegative iris tumor.
Lymphoma of the eyelid constitutes 5% of ocular adnexal lymphoma. In previously published cases, 56% of lymphomas of the eyelid are of B-cell origin and 44% are of T-cell origin. The most frequent B-cell lymphomas are extranodal marginal zone lymphoma (27 cases—14%) and diffuse large B-cell lymphoma (18 cases—9%). T-cell lymphomas are most frequently mycosis fungoides (25 cases—13%), extranodal natural killer/T-cell, nasal-type lymphoma (12 cases—6%), and primary cutaneous anaplastic large-cell lymphoma (12 cases—6%). This distribution differs from the distribution of ocular adnexal lymphoma and that of cutaneous lymphoma. The majority of subtypes occur in elderly patients, except for lymphoblastic lymphoma of B-cell and T-cell origin and Burkitt lymphoma, which occur in children and adolescents. Several subtypes have a male predominance, including peripheral T-cell lymphoma and Burkitt lymphoma. Only lymphomatoid papulosis has a female predominance. Signs of B-cell and T-cell lymphomas are tumor and swelling of the eyelid. Ulceration and erythema occur frequently among patients with T-cell lymphoma. Radiotherapy with or without surgery is the treatment of choice for low-grade, solitary lymphomas, whereas chemotherapy with or without adjuvant treatment is the treatment of choice for high-grade or disseminated lymphomas. The majority of subtypes, especially low-grade subtypes, have a good prognosis with few recurrences or progression. Some subtypes, including mycosis fungoides, have a poorer prognosis. Extranodal natural killer/T-cell lymphoma, nasal type has an exceedingly poor prognosis.
We compare outcomes following radiotherapeutic and surgical management of iris melanoma. End points included local tumor recurrence, metastases, and common complications. From an initial search that yielded 231 articles, we found 17 relevant studies with 761 eyes. The gender distribution was balanced with a mean age of 52 years. Most studies focused on either proton beam (49.4%) or plaque (31.4%) radiotherapy. Rates of local recurrence (range: 0%–8%) and metastatic development (0%–5%) were favorable following radiotherapy; however, common complications included cataract (36%–73%), glaucoma (3%–92%), and corneal conditions (0%–33%). A unique complication of proton beam radiotherapy was limbal stem cell deficiency (2%–33%). In contrast, iridectomy and comparable procedures showed similar rates of recurrence (0%–8%) and metastases (0% in one study), lower rates of postoperative cataract (0%–33%) and corneal conditions (2.3% in one study), and a greater incidence of photophobia (9%–25%). Following enucleation, one study of mostly advanced eyes demonstrated a higher rate of metastases (14%). Overall, despite a paucity of research in this setting, efficacy and safety outcomes remain favorable. Decision-making for management should be based on tumor size, location, angle seeding, secondary glaucoma, visual potential, and patient preference.