Lymphoma is the most common primary tumor of the orbit, accounting for 55% of all orbital malignancies. When divided into histopathological subtypes, extranodal marginal zone lymphoma of mucosa-associated lymphoid tissue (MALT lymphoma) comprises the largest proportion. Clinical manifestations are unspecific, but in patients with slow-growing painless orbital mass, or red conjunctival lesion suggestive of ‘salmon patch’, ocular adnexa lymphoma (OAL) should be suspected. Although the pathogenetic mechanism of ocular adnexal MALT lymphoma (OAML) is not yet fully understood, the relationship between OAML and Chlamydia psittaci has been hypothesized recently, similar to that between gastric MALT lymphoma and Helicobacter pylori. This suggests a new treatment option for OAML; bacterial eradication therapy with systemic antibiotics. Several other treatment methods for OAML have been introduced, but no treatment guidelines have been established yet. In this article, we summarize the current knowledge on the clinical features, pathogenesis, diagnostic methods, therapeutic strategies, and prognosis of OAML.
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Mesenchymal chondrosarcoma is a rare malignant tumor of skeletal and extraskeletal origin, and which shows aggressive local behavior as well as a high metastatic potential. We report 3 cases of mesenchymal chondrosarcoma. Two cases were male and one was female. The ages ranged from 25 to 32 years(mean: 28 years). Tissue was obtained by wide excision in two patients, and incisional biopsy in one. The mass locaterd in the rib(case 1), orbital floor(case 2), and abdominal wall(case 3). Roentgenographically, the tumor resembles ordinary chondrosarcoma, showing osteolytic and obstructive appearance with stippled calcification. Grossly, the tumor was lobulating, solid fish-fleshy like mass with calcification and ossification. Histologically, the tumor shows characteristic bimorphic pattern composed of islands of well differentiated hyaline cartilage admixed with a cellular area of undifferentiated small cells. The small cells usually displayed a hemangiopericytoid or an alveolar pattern.
The authors reviewed 95 cases (46 men and 49 women) of the orbital tumors diagnosed histopathologically at the Department of Ophtalmology, Yeungnam University Hospital from March 1984 through August 1994. Seventy-five cases of benign tumors were found evenly in all decades, but twenty cases of malignant tumors were noticed more frequently in 1st, 6th, and 7th decades. The frequency of benign orbital tumors was in this order; dermolipoma (21%, 20 cases), dermoid cyst (11.6%, 11 cases), mucocele (8.4%, 8 cases), lipoma (7.4%, 7 cases) and pleomorphic adenoma (5.3%, 5 cases). Of malignant orbital tumors, the frequency was in this order; retinoblastoma (3 cases), malignant melanoma (2 cases), sebaceous carcinoma (2 cases), and maxillary sinus carcinoma (2 cases). Malignant orbital tumors of 8 expired patients were revealed as secondary or metastatic tumors.