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Shunsuke Terasaka, Max B. Medary, Donald M. Whiting, Takanori Fukushima, Evalynne J. Espejo and Girija Nathan

✓ Sinonasal teratocarcinosarcoma is a rare malignant neoplasm characterized by the combined histological features of carcinosarcoma and teratoma. The primary symptoms of this tumor are usually nasal obstruction and epistaxis, and a nasal cavity mass is the most common clinical finding. The authors describe an exceptionally rare case in which the patient presented with massive intracranial extension and exhibited confusion as an initial symptom. He subsequently underwent combined radical surgery and radiation therapy and has remained free of disease for 31 months. The surgical approach to the lesion, histological features, and clinical course are detailed.

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Shigeru Yamaguchi, Shunsuke Terasaka, Hiroyuki Kobayashi, Tohru Shiga, Reiko Usui, Kenji Hirata, Kanako Kubota, Junichi Murata and Yoshinobu Iwasaki

Object

Intrinsic tumors arising in the dorsal midbrain cause obstructive hydrocephalus and have an indolent clinical course. Positron emission tomography (PET) with fluorine-18–labeled fluorodeoxyglucose (FDG) and l- [methyl-11C]methionine (MET) was used to evaluate the biological behaviors of dorsal midbrain tumors.

Methods

The authors report on 4 patients (3 males and 1 female) with dorsal midbrain tumors who presented with obstructive hydrocephalus. A diagnosis was made with MR imaging in each patient. To manage the hydrocephalus, endoscopic third ventriculostomy was performed in all cases. The patients did not undergo any other surgical procedures except endoscopic biopsy procedure, chemotherapy, or radiation therapy. The patients in 3 cases underwent FDG- and MET-PET within 6 months of CSF-diverting procedures, and the patient in 1 case underwent PET 10 years after the procedure.

Results

After the CSF-diverting procedure, clinical symptoms resolved or improved in all patients. Gliosis or glial proliferation was diagnosed in 1 patient, and possible low-grade glioma in 2 patients. Although all tumors appeared hyperintense on T2-weighted MR images, their appearance on T1-weighted images was variable (iso- and/or hypointense), and partial lesion enhancement was observed on images from 2 patients. On the other hand, the PET features of these lesions were almost identical, and the scans did not show a high uptake of FDG and MET compared with the cortical uptake in a normal brain. The mean tumor tissue/normal tissue ratio of FDG uptake was 0.65, and that of MET was 0.99.

Conclusions

Positron emission tomography findings suggested that the indolent dorsal midbrain lesion had nontumorous characteristics, thus supporting a good prognosis. Positron emission tomography studies may be more informative and predictive of the biological behavior of dorsal midbrain tumors than a biopsy procedure.

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Katsuyuki Asaoka and Shunsuke Terasaka

The authors demonstrate a step-by-step surgical technique of the combined petrosal approach for resection of petroclival meningioma. The basic concept of this approach is the combination of the anterior– and posterior–petrosal approaches uniting the infra- and supratentorial surgical fields, thereby providing wide surgical exposure. Our techniques are featured by 1) mastoidectomy preceding craniotomy for minimal bone loss; 2) removal of the tentorium over the tumor for achieving devascularization and wide exposure; 3) water-tight dural closure by using autologous fascia graft, non-penetrating titanium clips, and multi-layered technique for avoiding postoperative cerebrospinal fluid leakage.

The video can be found here: http://youtu.be/zMlNE8kMcHA.

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Yukitomo Ishi, Shigeru Yamaguchi, Akihiro Iguchi, Yuko Cho, Junjiro Ohshima, Kanako C. Hatanaka, Emi Takakuwa, Hiroyuki Kobayashi, Shunsuke Terasaka and Kiyohiro Houkin

Primary intracranial rhabdomyosarcoma is quite rare, and its prognosis is poor compared with that for rhabdomyosarcoma in other organs. The authors present a case of pineal rhabdomyosarcoma successfully managed with multimodal therapy including surgery, chemotherapy, radiation, and high-dose chemotherapy (HDC) followed by autologous peripheral blood stem cell transplantation (HDC/APBSCT). An 8-year-old girl presenting with headache and nausea was referred to the authors' institution. Computed tomography and MRI revealed a pineal tumor associated with obstructive hydrocephalus. Subsequently, an emergent endoscopic tumor biopsy and third ventriculostomy were performed. The patient's symptoms immediately improved. The most likely pathological diagnosis was embryonal rhabdomyosarcoma. Chemotherapy with etoposide, cyclophosphamide, cisplatin, pirarubicin, ifosfamide, actinomycin D, and vincristine was followed by a second-look operation and whole-brain and craniospinal radiation. Because the intraoperative findings and pathological examination of the second operation suggested a definitive diagnosis of rhabdomyosarcoma and the presence of viable residual tumor cells, HDC with etoposide and melphalan was followed by APBSCT. The patient was discharged from the hospital without residual tumor or any neurological deficit. No recurrence was observed at 30 months. This is the first case of primary pineal rhabdomyosarcoma treated with HDC/APBSCT. Although the efficacy of HDC/APBSCT for rhabdomyosarcoma has not been established, the prognosis of primary intracranial rhabdomyosarcoma treated with conventional treatment is quite poor. High-dose chemotherapy followed by APBSCT may contribute to a better prognosis for primary intracranial rhabdomyosarcoma.