Unusual Course of a Retinoblastoma's Alteration into a Leiomyosarcoma

In this report the rare case of a 34-year-old woman is presented. The medical history starts at about 2 years of age. Retinoblastomas in both eyes had been removed, at age 2, followed by radiotherapy. Seventeen years later a leiomyosarcoma developed in the periorbital region. A major craniofacial resection was performed in another craniofacial center. Seven years later recurrence developed, which was treated in an oncologic department by an invasive radiochemotherapy. The young woman, now 27 years old, was presented to us with a further recurrence combined with osteoradioneurosis of the upper face. After difficult discussion with the neurosurgeons and craniomaxillofacial and reconstructive surgeons, the family, and a priest, another craniofacial resection was performed including a great part of the skull, the periorbital region, and an extensive dural resection with dural reconstruction using an artificial dura. The external defect was covered with a free latissimus dorsi flap anastomosed to the external carotid artery and the retromandibular vein, respectively, after a conservative total parotidectomy. The appearance of the young woman was improved using an epithesis. Some minor recurrences were treated using sterotactic irradiation. Six years later a larger recurrence was noted in the skull region. The above-described operation was performed again on the other side. One year later the next recurrence was removed from the dura. During the next 4 months no recurrence was observed. Family history showed that the father and the child of the patient also suffered from retinoblastoma. The aims of this case report are twofold: (1) Extreme situations make extreme therapies necessary. However, only time can show whether or not this decision was a benefit for the patient. In our case the patient is absolutely thankful for the treatment, even if she fears dying from other recurrences or metastases; (2) We ask the audience for help and whether anyone has experience with a comparable case so we can prevent the expected death of our patient.