Case 2 – Anomalous retinal detachment: a case report of infraorbital desmoid fibromatosis

Abstract

Desmoid tumors, also known as aggressive fibromatosis, are benign, fibrous neoplasms arising from aponeurotic muscle structures of the body. Although they are described as having benign features, these tumors have high tendency to be locally invasive. In some cases where the tumor is attached to an unusual anatomical site such as the head or neck, complete resection is not possible. Therefore, additional treatment such as radiotherapy, hormonal therapy, chemotherapy or noncytotoxic agents may be necessary. We report the case of a 55-year-old man with a desmoid tumor of the left orbital region. The patient presented with retinal detachment, that was initially treated surgical silicone cerclage. A new laser correction was made because of retinal detachment relapse. The appearance of eyelid edema and episodic diplopia resulted in the patient being referred for a computed tomography scan. This revealed the presence of intra-orbital non-homogeneous tissue in the left eye. Local relapse occurred after initial surgical removal and the patient underwent a second surgery. Final pathological examination confirmed a diagnosis of aggressive fibromatosis. Taking into account the site of the neoplasm and the risks related to additional surgery, a chemotherapeutic regimen with vinorelbine and methotrexate was prescribed. Treatment was well tolerated and the orbital mass remained still stable at the last follow-up after 12 months of chemotherapy.