Clinical History
A 50 year old women with a known history of uterine leiomysarcoma and intractable headache.
Imaging Findings
A 50 year old woman presented at the hospital for an elective resection of a soft tissue mass over the right shoulder. She had a known history of a uterine leiomyosarcoma, first diagnosed 7 years before. She was initially treated with hysterectomy and pelvic lymphadenectomy. Because of a R0-resection and no nodal metastasis no adjuvant therapy was given. About 6 years after the first diagnosis she noticed gluteal soft tissue swelling. In the preoperative work up lung nodules were noted. The gluteal soft tissue mass and three lung nodules were resected and pathologically confirmed as metastasis of the known uterine leiomysarcoma. She also received chemotherapy. One year later she noticed another soft tissue mass over her right shoulder. She presented for surgery in our hospital. At the initial examination she reported about progressive and intractable headache. Because of the known malignancy a head CT scan was ordered. Already the native examination showed a large left-sided frontotemporal mass. After the admission of contrast media the mass showed a strong rim enhancement and another small enhancing mass on the contralateral side.
Discussion
Uterine leiomyosarcomas are rare malignancies, accounting for only about 1% of all uterine neoplasms. The most common symptom is abnormal vaginal bleeding, so a substantial portion of leiomyosarcomas is discovered in early tumour stages. But also in this cases prognosis is not favourable, because in most cases micrometastasis are already present at the time of diagnosis. In fact the overall 5-year survival rate is about 60% [1]. The most common sites for metastasis are the lungs and in the peritoneal cave. Cerebral metastases are very rare, with less than 40 cases reported in the literature [2]. Treatment depends on tumour stage. To date total hysterectomy and salpingo-oophorectomy is the only known potential curable approach in early stage leiomysarcomas. The use of chemotherapeutic agents and radiation is controversial, with most studies reporting only modest benefits [1,3]. Brain metastases are usually only found in disseminated disease, like in our case with known pulmonary and soft tissue metastasis. On the other hand a case with neurological signs because of brain metastasis as the first manifestation of a uterine leiomysarcoma is reported [4]. Imaging findings are unspecific with most case reports reporting rim-enhancing supratentorially located masses [1,5,6], but infratentorial mass is also possible [4]. In our case no pathological confirmation was performed, because the patient refused further therapy. But the known disseminated metastasis of a known uterine leiomyosarcoma and the presence of two supratentorial masses, make the diagnosis of cerebral metastasis almost certain. Treatment of cerebral metastasis is only palliative and consists of craniotomy and excision of the tumour followed by irritation, but prognosis is poor with reported mean survival of only 18 months [6].
In conclusion, cerebral metastasis of uterine leiomysarcoma is a rare occurrence. Radiologists should be aware of this possibility to establish proper differential diagnosis in patients with known uterine leiomysarcoma and brain masses.
Differential Diagnosis List
Cerebral metastasis of an uterine leiomysarcoma.
Final Diagnosis
Cerebral metastasis of an uterine leiomysarcoma.
