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. 2019 Aug;5(5):333-339.
doi: 10.1159/000494497. Epub 2019 Jan 24.

Conjunctival Leiomyosarcoma, a Rare Neoplasm Always Originating at the Limbus? Report of a New Case and Review of 11 Published Cases

V De Groot  1   2   3 , E Verhelst  2 , P C W Hogendoorn  3 , R J W de Keizer  1   2
Affiliations

Conjunctival Leiomyosarcoma, a Rare Neoplasm Always Originating at the Limbus? Report of a New Case and Review of 11 Published Cases

V De Groot et al. Ocul Oncol Pathol. 2019 Aug.

Abstract

Purpose of the study: To describe the differential diagnosis and management of a rare conjunctival malignancy.

Procedures: A 79-year-old man presented with a conjunctival mass at the limbus. Excisional biopsy revealed a malignant mesenchymal tumor with myogenic differentiation. Six months later, three suspect lesions developed at the border of the previous excision. Pathological diagnosis pointed to a leiomyosarcoma. Adjuvant radiotherapy with strontium-90 brachytherapy was applied. After 3 years, there was neither recurrence nor distant metastases. A literature review revealed 11 cases of conjunctival leiomyosarcoma.

Results: All 12 cases seemed to originate at the limbal conjunctiva. Scleral invasion was found only in one patient with multiple previous resections. Corneal invasion was reported in two patients. Four patients had a globe-sparing resection. In two of them, margins were not tumor free and additional brachytherapy gave a tumor-free follow-up of 1 and 3 years. Four cases underwent an exenteration.

Conclusion and message: Primary conjunctival leiomyosarcoma is a rare tumor with a favorable prognosis due to early detection and consequently limited size. Diagnosis involves histopathological investigation including immunohistochemistry. If possible, complete resection has the best prognosis. Adjunctive radiotherapy can be effective when the margins are not free and should be considered.

Keywords: Conjunctival tumor; Eye; Leiomyosarcoma; Limbus; Orbit; Soft tissue tumor.

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Conflict of interest statement

None of the authors has any conflict of interest to disclose. None of the authors received sponsorship or funding for this study.

Figures

Fig. 1
Fig. 1
a Nasal subconjunctival mass on initial presentation. b Local recurrences temporal and inferior to previous resection area, 6 months after excisional biopsy.
Fig. 2
Fig. 2
a Overview of the mesenchymal tumor (H&E staining). b Spindle-shaped cells, with cell and nuclear pleomorphism (H&E staining, magnification 200×).
Fig. 3
Fig. 3
Staining for alpha-smooth muscle actin (magnification 200×).
See this image and copyright information in PMC

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