Vestibular schwannoma to malignant peripheral nerve sheath tumor transformation
Intracranial malignant peripheral nerve sheath tumor (MPNST) is a very rare entity and most often arise from the fifth cranial nerve, followed by the seventh and eighth nerves 1) 2).
Transition of a vestibular schwannoma to a malignant peripheral nerve sheath tumor with loss of H3K27 trimethylation after radiosurgery-a case report and review of the literature 3).
A 29-year-old woman who suffered right-sided hearing loss which remained untreated for 4.5 years. Magnetic resonance imaging revealed a right cerebellopontine tumor and the patient underwent tumor removal through retrosigmoid approach. Pathologically, the tumor was benign acoustic schwannoma with increased Ki-67 8%-10%. The tumor relapsed only 6 months later and was removed again-this time it was malignant peripheral nerve sheath tumor MPNST. The patient was treated with stereotactic radiotherapy, but despite that tumor growth was observed again and she underwent the third operation. Later on, tumor progression was noted with multiple intraaxial metastases and patient died 17 months after the diagnosis of MPNST had been confirmed. This is a seventh documented case of spontaneous transformation of VS into MPNST, which is clinically important for recording 4).
The first late malignant transformation of VS unrelated to neurofibromatosis or radiation exposure. After undergoing a near-total excision of a histologically benign VS, the patient developed malignant regrowth of the tumor remnant 42 months after the primary excision. This case challenges the dogmatic belief of absolute causality between radiation exposure and late malignant transformation of VS, and has important implications regarding future counseling and consent for the treatment of patients with VS 5).
A case presenting with malignant peripheral nerve sheath tumor (MPNST) at 8 years after GKRS after incomplete resections.
The tumor appeared to be a typical benign schwannoma at the surgery preceding GKRS, and rapidly enlarged after long-term control, causing progressive neurological deterioration. Operative findings showed that the tumor was composed of two different components, and histopathology distinctively demonstrated MPNST and benign schwannoma. The coexistence of benign and malignant components might indicate that the present MPNST had arisen from the benign schwannoma by transformation in association with GKRS 6).
A young adult who had combined operative/Gamma knife treatment for a benign vestibular schwannoma, followed by further surgery 2 years later. He represented 10 years after original diagnosis with facial numbness and ataxia, MRI showing gross tumor recurrence. After radical resection, histology showed malignant transformation to a malignant peripheral nerve sheath tumor. Within 3 months there was rapid, aggressive recurrence with brainstem compression, requiring further surgery for brainstem decompression. Histology confirmed further de-differentiation to an anaplastic sarcoma. While awaiting radiotherapy the tumor recurred again, the patient succumbing. The patient had no features of neurofibromatosis type 2. In the literature there are 13 other cases of malignant vestibular schwannomata. Only six had radiotherapy and of these only two had histological confirmation of a benign lesion preradiotherapy. Neither of these had neurofibromatosis. Three other cases had histological proof of malignancy postradiosurgery, but with no preradiotherapy histology; of these, two were positive for neurofibromatosis 7).
A 43-year-old woman with a malignant peripheral nerve sheath tumor arising from a benign schwannoma of the eighth cranial nerve are presented 8).
Han DH, Kim DG, Chi JG, Park SH, Jung HW, Kim YG. Malignant triton tumor of the acoustic nerve. Case report. J Neurosurg. 1992 May;76(5):874-7. doi: 10.3171/jns.1992.76.5.0874. PMID: 1564550.
A 54-year-old man whose right acoustic nerve signs had shown gradual worsening during the previous five years. No stigmata of von Recklinghausen's disease were noted. The tumor at autopsy extensively involved the brain stem, cerebellar hemisphere, tentorium of cerebellum, and cerebral occipitotemporal lobe on the right side. Histologically, the tumor was characterized by extreme hypercellularity, pleomorphism, and high mitotic activity. Ultrastructurally, there was an external lamina around the interdigitated cytoplasm of the tumor cells. Long-spacing collagens and junctional devices were also noted. This case is, to our knowledge, the first reported of a malignant nerve sheath tumor in the acoustic nerve 9).
A malignant nerve sheath tumor of the acoustic nerve was found in a 54-year-old man whose right acoustic nerve signs had shown gradual worsening during the previous five years. No stigmata of von Recklinghausen's disease were noted. The tumor at autopsy extensively involved the brain stem, cerebellar hemisphere, tentorium of cerebellum, and cerebral occipitotemporal lobe on the right side. Histologically, the tumor was characterized by extreme hypercellularity, pleomorphism, and high mitotic activity. Ultrastructurally, there was an external lamina around the interdigitated cytoplasm of the tumor cells. Long-spacing collagens and junctional devices were also noted. This case is, to our knowledge, the first reported of a malignant nerve sheath tumor in the acoustic nerve 10).