Autologous stem cell transplantation experience in an adult recurrent medulloblastoma patient: Case report

IF 1.8 Q3 HEMATOLOGY
Kemal Fıdan , Ali Ünal , Neslihan Mandacı Şanlı , Ebubekir Sıddık İpekçi
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Abstract

Case report: Introduction

Medulloblastoma is the most common malignant primary embryonal brain tumor in children and occurs in the cerebellum. Approximately 70% of patients are diagnosed before the age of 20. The disease is rare after the 4th decade of life. It originates from the brainstem and metastasizes to other brain tissue, ventricles and medulla spinalis via CSF. Metastasis to bone, bone marrow, lung or lymph nodes outside the CNS is a very rare condition.

Surgery, chemotherapy and radiotherapy are used in the treatment of medulloblastoma. In some patients (patients in the high-risk group, relapsed/refractory patients), autologous stem cell transplantation(ASCT) is performed following high-dose chemotherapy to increase survival rates.

Here, we will present a case of medulloblastoma in which we performed autologous stem cell transplantation in our center.

Key words: Medulloblastoma, autologous stem cell transplantation

Case report

A 30-year-old male patient applied to the neurology clinic in May 2020 with complaints of headache, dizziness, nausea, vomiting and fainting. In the brain imaging, a 6 × 4 cm mass lesion was observed in the posterior fossa, located in the ventricle and causing compression symptoms (Cystic Astrocytoma? Medulloblastoma?). The patient underwent ventriculoperitoneal shunt and subtotal mass excision at the neurosurgery clinic. The biopsy pathology result was reported as medulloblastoma (classical type, p53 mutation positive). Chemotherapy was recommended by the oncology clinic, but the patient did not accept the treatment. In August 2020, the patient was given cranial RT and was subsequently followed without medication.

in June 2023 due to complaints of pain and weakness in both lower extremities, there was an intradural mass lesion (25 × 19 mm) obliterating the spinal cord at the T11-T12 level and extending to the extraspinal area, and a diffuse mass lesion within the spinal cord at the T10 level with a craniocaudal length of 17 mm. Mass excision as a result of pathology; It was reported as classical medulloblastoma (non-WNT/non-SHH group (grade 4)).

After the patient was given 2 courses of mini-ICE chemotherapy, a nearly complete response in the imaging. The patient was mobilized with G-CSF. In our center, the patient was performed autologous stem cell transplantation (6.55 × 106 /kg cells) with temozolamide (2 × 200mg/m2 on days -6,-5,-4), etoposide (100 mg/m2 on days -7,-6,-5,-4,-3,-2), thiotepa (300 mg/m2, on days -4,-3,-2) protocol in November 2023.The patient, who had neutrophil and platelet engraftment on the 10th day after transplantation, was discharged with outpatient clinic control.

Discussion and conclusion

Although the prognosis has improved in children with medulloblastoma, an estimated 20-30% will relapse following initial treatment (1). Recurrences may be local or widespread (brain and vertebra) (2,3,4). In case of recurrent disease after initial treatment, the likelihood of long-term survival is significantly reduced. Autologous hematopoietic cell transplantation after high-dose chemotherapy has been evaluated in small series and resulted in prolonged disease-free survival in approximately 20-25% of patients (7,8). In the study conducted by Eduvian et al., they showed that autologous stem cell transplantation after chemotherapy has a definite, albeit limited, role for selected pediatric brain tumors with poor prognosis and complete/partial remission before transplantation(9).

成人复发性髓母细胞瘤患者的自体干细胞移植经验:病例报告
病例报告导言成髓母细胞瘤是儿童最常见的恶性原发性胚胎性脑肿瘤,好发于小脑。约 70% 的患者在 20 岁前确诊。该病在出生后第 4 个 10 年罕见。它起源于脑干,通过 CSF 转移到其他脑组织、脑室和脊髓。转移到中枢神经系统以外的骨、骨髓、肺或淋巴结的情况非常罕见。有些患者(高危组患者、复发/难治性患者)在接受大剂量化疗后会进行自体干细胞移植(ASCT),以提高存活率:髓母细胞瘤、自体干细胞移植病例报告一名 30 岁的男性患者于 2020 年 5 月因主诉头痛、头晕、恶心、呕吐和昏厥来神经内科就诊。在脑部影像学检查中,发现后窝有一个 6 × 4 厘米的肿块病变,位于脑室,引起压迫症状(囊性星形细胞瘤? 髓母细胞瘤?)患者在神经外科诊所接受了脑室腹腔分流术和肿块次全切除术。活检病理结果报告为髓母细胞瘤(经典类型,p53 突变阳性)。肿瘤诊所建议进行化疗,但患者不接受治疗。2023 年 6 月,患者因主诉双下肢疼痛和无力,出现硬膜内肿块病变(25 × 19 毫米),阻塞 T11-T12 水平的脊髓并延伸至脊髓外区域,T10 水平脊髓内出现弥漫性肿块病变,颅尾长 17 毫米。患者接受了两个疗程的迷你ICE 化疗后,影像学检查显示患者的反应接近完全。患者接受了两个疗程的迷你ICE化疗后,影像学检查出现了近乎完全的反应。患者接受了G-CSF动员治疗。2023年11月,患者在本中心接受了自体干细胞移植(6.55×106个/千克细胞),并接受了替莫唑胺(2×200毫克/平方米,第-6、-5、-4天)、依托泊苷(100毫克/平方米,第-7、-6、-5、-4、-3、-2天)、噻替帕(300毫克/平方米,第-4、-3、-2天)方案。该患者在移植后第10天出现中性粒细胞和血小板移植,在门诊控制下出院。讨论与结论尽管髓母细胞瘤患儿的预后有所改善,但估计仍有20%-30%的患儿会在初次治疗后复发(1)。复发可能是局部的,也可能是广泛的(脑部和脊椎)(2,3,4)。如果初次治疗后疾病复发,长期存活的可能性就会大大降低。在小范围内对大剂量化疗后进行自体造血细胞移植进行了评估,结果显示约 20-25% 的患者延长了无病生存期(7,8)。在Eduvian等人的研究中,他们发现化疗后自体干细胞移植对某些预后不良、移植前完全/部分缓解的小儿脑肿瘤有明确的作用,尽管作用有限(9)。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
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CiteScore
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