Purpose Among peripheral nerve sheet tumors, an orbital hybrid neurofibroma‐schwannoma has been described twice. We describe a third case of orbital tumor with features of both a neurofibroma and a schwannoma. Methods Case report. A 39 year old man presents with diplopia and decreased vision (0.6) in the left eye. He has a proptosis of 7 mm, with mechanical restriction of elevation and horizontal eye movements. MRI shows a well‐defined lesion (2,5 x 1,9 x 2,3 cm) in the superotemporal intraconal space, displacing the globe, optic nerve and superior and lateral rectus muscles. The tumor demonstrates heterogeneous contrast enhancement, and is diagnosed as a probable cavernous hemangioma. Through a transconjunctival approach a red‐blueish lesion was found with a thin capsule and without lateral adhesions. The tumor could be extracted in 2 parts and consisted of a yellow/white coherent soft mass. Results Patient had complete recovery of visual acuity, and only diplopia in extreme lateral gaze. The pathology revealed a tumor composed of small spindle cells arranged in fascicles. The cells showed no atypia and there was no mitotic activity. There were also less cellular areas with a myxoid component. At places we could recognize a fibrous capsule surrounding the tumor. The tumor cells were strongly and diffuse positive with S100. In a few enclosed axons there was focal reactivity with neurofilament. The image is that of a neural tumor with features of both a neurofibroma and a Schwannoma. Conclusions This is the third reported orbital hybrid neurofibroma‐schwannoma. Although this type of tumor elsewhere in the body is often associated with schwannomatosis and neurofibromatosis, our patient did not show signs of systemic involvement.
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.
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