Primary Intracranial Leiomyosarcoma - Case Report and Principles for Treatment

Abstract

Introduction: Primary intracranial leiomysarcoma (LMS) is a rare clinical entity. We present a case of a patient with primary intracranial LMS ten years after surgical resection of a pilocytic astrocytoma in the posterior fossa and highlight different diagnostic and therapeutical features. Methods: Case reportResults: A 43 years old male presented with an indolent swelling at occiput without neurological symptoms. Ten years earlier he had undergone surgery elsewhere for an astrocytoma WHO grade 1 of the right cerebellar hemisphere, without following radiotherapy. MRI showed a contrast enhancing lesion of the right posterior fossa infiltrating the occipital bone. Proton magnetic resonance spectroscopy (MRS) indicated malignancy. The tumor could be removed totally with retention of the transverse sinus with negative surgical margins. Histological work-up revealed LMS grade 2. Staging examinations with CT of the chest and abdomen revealed no other tumor. Three months later the patient presented again with a right sided swelling of the neck. MRI of the neck showed contrast enhancing space occupying tissue bordering the former operations field. Surgery was done in view of a recurrent tumor, however, histological work up showed scar tissue without tumor. Nevertheless the patient received GD 192iridium -HDR -brachytherapy up to 20 Gy and radiotherapy of the corresponding muscle tissue with 50Gy. Three and six months follow up MRI of the brain and spine showed no recurrence. Conclusion:Primary LMS of the CNS is very infrequent. Reported cases of intracranial LMS are often metastasic. An elevated incidence in association with Ebstein-Barr virus, AIDS and after radiotherapy is described. Neuro-imaging should contain MRI, whereby MRS is helpful to identify malignancy. In view of longest survival time, a complete tumor resection with negative margins is to be aspired. After diagnosis of LMS, adjuvant radiotherapy is recommended, because 40-50% of soft tissue sarcomas show remission. Success of adjuvant chemotherapy is currently uncertain. Despite multimodal aggressive therapy, prognosis of LMS remains poor, because of early haematogenous spread.

Primary intracranial leiomyosarcoma presenting with frontal bone mass: a case report

“…The prevalence of bone and soft tissue sarcomas is four in 100,000 people per year [ 17 ]. LMS is found more frequent in uterus, stomach, intestines or walls of blood vessels.…”

“…There is a report about late brain tumor recurrence by Maslehaty et al [ 17 ] in 2016. They reported a 43-year-old man with the history of resected astrocytoma grade 1 in right cerebellar hemisphere 10 years before.…”

Primary intracranial leiomyosarcoma presenting with massive peritumoral edema and mass effect: Case report and literature review

“…[ 1 2 3 4 5 10 11 12 13 17 18 20 22 23 26 28 ] Out of 15 patients described, 3 patients had developed LMS after prior surgery (without any adjuvant chemotherapy or radiotherapy). [ 10 18 28 ] Out of these three, one developed LMS nine years after surgery for a neurofibroma located in the CPA (here the authors hypothesized that heat/mechanical stimulation of the previous surgery may be an etiological factor for tumor development). [ 10 ] Another case of intra-ventricular LMS, reported by Louis et al .,[ 17 ] also underwent surgery for basal cell carcinoma and intra-ductal breast carcinoma before the development of LMS; however, the role of preceding surgery in the pathogenesis of a later occurring LMS remains unclear.…”

“…However, involvement of CNS is seen in few cases only. [ 1 2 3 4 5 10 11 12 13 16 17 18 20 22 23 25 26 28 ] Most reported cases of intracranial LMS represent metastases from other primary sites such as the gut, uterus, retro-peritoneum, or from subcutaneous tissue. Primary intracranial LMS is known to occasionally occur in immunocompromised patients,[ 10 20 28 ] whereas occurrence in adult immuno-competent patients is exceptionally rare.…”

“…A thorough literature review revealed only 15 cases of primary intracranial LMSs in immuno-competent adult patients [ Flowchart 1 ]. [ 1 2 3 4 5 10 11 12 13 17 18 20 22 23 26 28 ] Most of the cases described thus far showed dural-based lesions, mimicking meningiomas. Purely intraparenchymal involvement has very rarely been seen.…”