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Successful Treatment of Mixed Yolk Sac Tumor and Mature Teratoma in the Spinal Cord: Case Report

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Successful Treatment of Mixed Yolk Sac Tumor and Mature Teratoma in the Spinal Cord: Case Report CASE REPORT J Neurosurg Spine 26:319–324, 2017 Successful treatment of mixed yolk sac tumor and mature teratoma in the spinal cord: case report *Akitake Mukasa, MD, PhD,1 Shunsuke Yanagisawa, MD,1 Kuniaki Saito, MD,1 Shota Tanaka, MD,1 Keisuke Takai, MD, PhD,1,5 Junji Shibahara, MD, PhD,2 Masachika Ikegami, MD,3 Yusuke Nakao, MD,3,6 Katsushi Takeshita, MD, PhD,3,7 Masao Matsutani, MD, PhD,4 and Nobuhito Saito, MD, PhD1 Departments of 1Neurosurgery, 2Pathology, and 3Orthopaedic Surgery and Spinal Surgery, The University of Tokyo Hospital, Tokyo; and 4Department of Neuro-Oncology/Neurosurgery, Saitama Medical University International Medical Center, Hidaka, Japan Primary spinal germ cell tumors are rare, and spinal nongerminomatous germ cell tumors represent an even rarer subset for which no standard therapy has been established. The authors report the case of a 24-year-old woman with multifo- cal primary spinal germ cell tumors scattered from T-12 to L-5 that consisted of yolk sac tumor and mature teratoma. After diagnostic partial resection, the patient was treated with 30 Gy of craniospinal irradiation and 30 Gy of local spinal irradiation, followed by 8 courses of chemotherapy based on ifosfamide, cisplatin, and etoposide (ICE). Salvage surgery was also performed for residual mature teratoma components after the third course of ICE chemotherapy. Chemo- therapy was continued after the operation, but ifosfamide was entirely eliminated from the ICE regimen because severe myelosuppression was observed after previous courses. The patient remains recurrence free as of more than 5 years after the completion of chemotherapy. This case suggests that this treatment strategy is an effective option for primary spinal yolk sac tumor. https://thejns.org/doi/abs/10.3171/2016.8.SPINE16465 KEY WORDS germ cell tumor; spinal cord; yolk sac tumor; teratoma; radiotherapy; chemotherapy; oncology ENTRAL nervous system (CNS) germ cell tumor is against spinal NGGCT thus need to be developed. Here, we a rare pathology that accounts for approximately present a case of spinal mixed germ cell tumor comprising 2%–3% of CNS tumors in Japan and 0.3%–0.6% in yolk sac tumor and mature teratoma. Successful treatment CWestern countries.10,15 Most CNS germ cell tumors occur was achieved using whole-craniospinal irradiation and lo- in the suprasellar or pineal region, followed by the basal cal spinal irradiation followed by ifosfamide, cisplatin, and ganglia, and germ cell tumors originating in the spinal cord etoposide (ICE) chemotherapy, based on a protocol widely are extremely rare. While a standard therapeutic strategy used in Japan for the subset of intracranial germ cell tu- for intracranial germ cell tumor has been evaluated to some mors categorized as having a poor prognosis.13,14 extent, recommended treatment regimens for primary spi- nal germ cell tumor are less developed due to the rarity of this entity. In particular, primary spinal nongermino- Case Report matous germ cell tumor (NGGCT) has been reported in History and Examination only a small number of cases,2,5,9,20,21 including 2 cases of A 24-year-old woman who had noticed back pain after spinal yolk sac tumor that had poor outcomes despite in- physical exercise a month earlier presented with progres- tensive chemoradiotherapy.5,9 Effective treatment protocols sive numbness and weakness of the lower extremities. She ABBREVIATIONS AFP = alpha-fetoprotein; BEP = bleomycin, etoposide, and cisplatin; CE = carboplatin and etoposide; CNS = central nervous system; ICE = ifosfamide, cisplatin, and etoposide; JPBTSG = Japanese Pediatric Brain Tumor Study Group; NGGCT = nongerminomatous germ cell tumor; OS = overall survival rate; PE = cisplatin and etoposide; PVB = cisplatin, vinblastine, and bleomycin; STGC = syncytiotrophoblastic giant cell. SUBMITTED April 26, 2016. ACCEPTED August 31, 2016. INCLUDE WHEN CITING Published online December 2, 2016; DOI: 10.3171/2016.8.SPINE16465. * Drs. A. Mukasa, S. Yanagisawa, and K. Saito contributed equally to this work. ©AANS, 2017 J Neurosurg Spine Volume 26 • March 2017 319 Unauthenticated | Downloaded 09/27/21 06:15 AM UTC A. Mukasa et al. FIG. 1. Gadolinium-enhanced T1-weighted (A–C, E, and F) and T2-weighted (D) MR images showing spinal germ cell tumors. Mass lesions (arrowheads) approximately 30 mm in diameter can be seen at T-10 (A, sagittal image; B, axial image) and L2–3 (A and C [axial image]). The sagittal T2-weighted image (D) shows peritumoral edema within the spinal cord. The sagittal images in panels E and F show several smaller lesions (arrows) between T-12 and L-5. could not walk but could still move her right leg. Neu- rological examination revealed severe paraparesis of the lower limbs (paralysis of the left lower leg and severe weakness of the right lower leg), sensory disturbance be- low T-10, and bladder-bowel dysfunction. Both knee-jerk and Achilles tendon reflexes were deteriorated. Magnetic resonance imaging (MRI) of the spinal cord demonstrated mass lesions 30 mm in diameter at T-10 and L2–3, as well as several smaller lesions between T-12 and L-5 (Fig. 1). Cranial MRI revealed no lesions in the brain. Imaging with 18F-fluorodeoxyglucose positron emission tomography (FDG-PET) showed elevated maximum stan- dardized uptake values (SUVmax) of 3.2 at the T-10 lesion and 4.2 at the L2–3 lesion. Operation and Pathological Findings Because the T-10 lesion was suspected of being re- sponsible for the progressive paraparesis, emergent de- compressive laminectomy of T9–10 and partial resection of the tumor located at T-10 were performed. Although decompressive laminectomy was performed, the patient’s symptoms progressed further, and her lower legs were completely paralyzed during the postoperative period. FIG. 2. Surgical specimens of yolk sac tumor obtained at the T-10 Histopathological examination indicated a yolk sac tumor level. A and B: H & E staining reveals primitive-appearing cells in a (Fig. 2), and blood tests showed elevated levels of alpha- reticular-microcystic pattern. C and D: Immunohistochemical staining fetoprotein (AFP) (2182 ng/ml) and normal levels of beta– reveals the presence of SALL4 (C) and AFP (D). Bar = 500 μm (A), 50 human chorionic gonadotropin (b-HCG). μm (B and D), and 100 μm (C). 320 J Neurosurg Spine Volume 26 • March 2017 Unauthenticated | Downloaded 09/27/21 06:15 AM UTC Treatment of spinal nongerminomatous germ cell tumor Postoperative Course totally removed (Fig. 3B). The histopathological diagnosis Because a standard chemoradiotherapy regimen is of this remnant was mature teratoma, with no evidence lacking for spinal germ cell tumors, the patient was treated of residual yolk sac tumor (Fig. 3C), suggesting that the with a slight modification of the protocol proposed by the chemoradiotherapy had successfully eradicated the yolk Japanese Pediatric Brain Tumor Study Group (JPBTSG) sac tumor component. for intracranial NGGCT with poor prognosis.13,14 Briefly, Five courses of adjuvant chemotherapy, consisting of the patient received 30 Gy of whole-craniospinal irradia- cisplatin and a 75%–100% dose of etoposide, were ad- tion delivered in 17 fractions, followed by local boost ir- ministered after the salvage surgery; the chemotherapy radiation at the spinal lesions consisting of 30 Gy deliv- was completed 18 months after the initial operation. ered in 17 fractions. Eight cycles of ICE chemotherapy MRI (Fig. 3D) and measurement of AFP levels (3 ng/ml) were then administered. All radiotherapy was conducted showed no evidence of recurrence at more than 5 years prior to chemotherapy, because the tumors were growing after completion of chemotherapy. The patient still has rapidly and radiotherapy was thought to be more reliable the severe paraplegia of the lower limbs, sensory dis- than chemotherapy for the immediate control of tumor turbance below T-10, and bladder-bowel dysfunction masses. The ICE chemotherapy regimen comprised 900 that were present before treatment, but she is otherwise mg/m2 of ifosfamide (Days 1–5), 20 mg/m2 of cisplatin healthy and did not incur any additional deficit due to the (Days 1–5), and 60 mg/m2 of etoposide (Days 1–5). Due nerve root resection. to severe myelosuppression appearing during the course of chemotherapy, dose reduction was conducted following Discussion the method described by Sawamura et al.;17 a reduced dose of etoposide and ifosfamide was administered for the sec- Here, we have described a case we encountered of ond and third courses, and ifosfamide was therefore not primary malignant spinal germ cell tumor, which is ex- used during the fourth to eighth courses. tremely rare. The patient was successfully treated with The patient’s serum AFP level rapidly decreased to less whole-craniospinal and local irradiation followed by ICE than 100 ng/ml toward the end of radiotherapy, and it fell chemotherapy and salvage surgery. Our experience and further to within the normal range after the first cycle of review of the literature suggest that this treatment strategy ICE chemotherapy, which was carried out right after the might be effective for malignant spinal germ cell tumor, radiotherapy. In accordance with the decrease in serum although further evaluation of the treatment protocol is AFP level, spinal MRI demonstrated marked shrinkage of warranted. the enhanced lesions, but remnant tumor was suspected at L-2 (Fig. 3A) after the third course of ICE chemotherapy. Primary Spinal Germ Cell Tumors Salvage surgery was therefore performed. One nerve root Primary spinal germ cell tumors are rare. To date, in- was found to be completely involved with the remnant tu- cluding our case of mixed germ cell tumor, 38 cases of mor, and both the residual tumor and the nerve root were primary spinal germ cell tumor have been reported—in- FIG. 3. A: Sagittal gadolinium-enhanced T1-weighted image of the spinal cord obtained after the third course of ICE chemo- therapy, revealing residual tumor at L-2 (arrowhead).
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