A Case of Alveolar Rhabdomyosarcoma of the Ethmoid Sinus Invading the Orbit in an Adult

PURPOSE: A case study and literature review of alveolar rhabdomyosarcoma (RMS) in an adult. METHODS: A 48-year-old male patient presented at our clinic complaining of proptosis that had persisted for 2 weeks in his left eye. A computed tomography (CT) scan revealed a destructive soft-tissue mass in the left ethmoid sinus with invasion of the left orbit and compression of the medial rectus muscle. Endoscopic intranasal biopsy revealed alveolar RMS. Conservative debulking and orbital wall decompression were performed. RESULTS: Immunohistochemical testing was positive for desmin, S-100, and smooth muscle actin (SMA), supporting the diagnosis of RMS. Since ipsilateral cervical and spinal metastasis was detected, systemic treatment was administered simultaneously. CONCLUSIONS: Although rarely found in adults, RMS should be considered in the differential diagnosis of orbital tumors. Immunohistochemical analysis plays an important role in the definitive diagnosis of RMS.

Visual Improvement in High Myopic Amblyopic Adult Eyes following Phakic Anterior Chamber Intraocular Lens Implantation

PURPOSE: To evaluate the efficacy and safety of angle-supported phakic anterior chamber intraocular lenses in amblyopic adult eyes with very high myopia. METHODS: We evaluated 12 eyes in nine patients with very high myopic amblyopia who received angle-supported phakic intraocular lenses (Phakic 6H(R)) and followed them for more than six months. Uncorrected visual acuity (UCVA), best-corrected visual acuity (BCVA), and complications were evaluated. A satisfaction score was rated by patients using a 5-point (1~5) numeric scale. RESULTS: The mean age of patients was 37.3+/-9.4 years, ranging from 29 to 59 years old. The preoperative mean refraction (spherical equivalent, SE) was -20.10+/-5.41 diopters (D). The postoperative mean refraction (SE) was -1.75+/-0.76 D at six months. The postoperative BCVA improved an average 3.92+/-1.24 lines over preoperative values, and mean endothelial cell loss was 8.9% at six months. Development of cataracts, glaucoma, and pupil abnormalities were not demonstrated in any case during the study. The patients were all very satisfied, as the average satisfaction score was 4.3. CONCLUSIONS: This study indicates that angle-supported phakic anterior chamber intraocular lens implantation may be an effective surgical alternative for the correction of amblyopic adult eyes with very high myopia. However, long-term evaluation is necessary to assess possible complications and long-term safety.

Recurrent Sebaceous Carcinoma Presenting the Huge Mass of Face

PURPOSE: To report two cases of sebaceous carcinoma which presented as a huge mass on the face and extended deep into the around tissue. METHODS: A 71-year-old female who had a history of eyelid mass excision for three times visited our department for about 10cm sized mass on and around her right eye. We performed surgical excision and biopsy of the mass, followed by radiotherapy. An 86-year old female with recurrent right upper lid mass for 2 years had visited our department and her histopathologic diagnosis had been a sebaceous carcinoma. But she had refused all the management and been lost for follow up. Finally she revisited for about 9cm sized mass on her right eye. RESULTS: We performed the exenteration, ethmoidectomy, middle and inferior turbinectomy on the 71-year-old female patient as a palliative therapy and refer to radiologist for radiotherapy. She showed cosmetic improvement and no evidence of mass recurrence until postoperative 5 months. We couldn't provide any surgical management to the 86-year-old patient because of her refusal. CONCLUSIONS: Sebaceous carcinoma needs early histopathologic diagnosis and surgical management because of its potency to extend around the tissue and progress to huge mass when proper management delayed. In this study, we experienced two rare cases of far advanced sebaceous carcinoma which showed huge mass on and around the eye.