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罕见复合型纵隔肿瘤的多层面肿瘤学管理:一例报告

Multi-Level Oncological Management of a Rare, Combined Mediastinal Tumor: A Case Report.

作者信息

Theocharidis Vasileios, Rallis Thomas, Gogakos Apostolos, Paliouras Dimitrios, Lazopoulos Achilleas, Koutourini Meropi, Tzinevi Myrto, Vildiridi Aikaterini, Dimopoulos Prokopios, Kasarakis Dimitrios, Kousidis Panagiotis, Nikolaidou Anastasia, Vrochidis Paraskevas, Mironidou-Tzouveleki Maria, Barbetakis Nikolaos

机构信息

Thoracic Surgery Department, "Theageneio" Cancer Hospital, 54639 Thessaloniki, Greece.

Second Department of Medical Oncology, "Theageneio" Cancer Hospital, 54639 Thessaloniki, Greece.

出版信息

Curr Oncol. 2025 Jul 28;32(8):423. doi: 10.3390/curroncol32080423.

Abstract

Malignant mediastinal tumors are a group representing some of the most demanding oncological challenges for early, multi-level, and successful management. The timely identification of any suspicious clinical symptomatology is urgent in achieving an accurate, staged histological diagnosis, in order to follow up with an equally detailed medical therapeutic plan (interventional or not) and determine the principal goals regarding efficient overall treatment in these patients. We report a case of a 24-year-old male patient with an incident-free prior medical history. An initial chest X-ray was performed after the patient reported short-term, consistent moderate chest pain symptomatology, early work fatigue, and shortness of breath. The following imaging procedures (chest CT, PET-CT) indicated the presence of an anterior mediastinal mass (meas. ~11 cm × 10 cm × 13 cm, SUV: 8.7), applying additional pressure upon both right heart chambers. The Alpha-Fetoprotein (aFP) blood levels had exceeded at least 50 times their normal range. Two consecutive diagnostic attempts with non-specific histological results, a negative-for-malignancy fine-needle aspiration biopsy (FNA-biopsy), and an additional tumor biopsy, performed via mini anterior (R) thoracotomy with "suspicious" cellular gatherings, were performed elsewhere. After admission to our department, an (R) Video-Assisted Thoracic Surgery (VATS) was performed, along with multiple tumor biopsies and moderate pleural effusion drainage. The tumor's measurements had increased to DMax: 16 cm × 9 cm × 13 cm, with a severe degree of atelectasis of the Right Lower Lobe parenchyma (RLL) and a pressure-displacement effect upon the Superior Vena Cava (SVC) and the (R) heart sinus, based on data from the preoperative chest MRA. The histological report indicated elements of a combined, non-seminomatous germ-cell mediastinal tumor, posthuberal-type teratoma, and embryonal carcinoma. The imminent chemotherapeutic plan included a "BEP" (Bleomycin/Cisplatin/Etoposide) scheme, which needed to be modified to a "VIP" (Cisplatin/Etoposide/Ifosfamide) scheme, due to an acute pulmonary embolism incident. While the aFP blood levels declined, even reaching normal measurements, the tumor's size continued to increase significantly (DMax: 28 cm × 25 cm × 13 cm), with severe localized pressure effects, rapid weight loss, and a progressively worsening clinical status. Thus, an emergency surgical intervention took place via median sternotomy, extended with a complementary "T-Shaped" mini anterior (R) thoracotomy. A large, approx. 4 Kg mediastinal tumor was extracted, with additional RML and RUL "en-bloc" segmentectomy and partial mediastinal pleura decortication. The following histological results, apart from verifying the already-known posthuberal-type teratoma, indicated additional scattered small lesions of combined high-grade rabdomyosarcoma, chondrosarcoma, and osteosarcoma, as well as numerous high-grade glioblastoma cellular gatherings. No visible findings of the previously discovered non-seminomatous germ-cell and embryonal carcinoma elements were found. The patient's postoperative status progressively improved, allowing therapeutic management to continue with six "TIP" (Cisplatin/Paclitaxel/Ifosfamide) sessions, currently under his regular "follow-up" from the oncological team. This report underlines the importance of early, accurate histological identification, combined with any necessary surgical intervention, diagnostic or therapeutic, as well as the appliance of any subsequent multimodality management plan. The diversity of mediastinal tumors, especially for young patients, leaves no place for complacency. Such rare examples may manifest, with equivalent, unpredictable evolution, obliging clinical physicians to stay constantly alert and not take anything for granted.

摘要

恶性纵隔肿瘤是一组对早期、多层面及成功治疗提出极高肿瘤学挑战的疾病。及时识别任何可疑的临床症状对于实现准确的分期组织学诊断至关重要,以便后续制定同样详细的医学治疗方案(无论是否进行干预),并确定这些患者有效整体治疗的主要目标。我们报告一例24岁男性患者,既往无病史。患者自述有短期、持续的中度胸痛症状、早期工作疲劳和呼吸急促后,进行了首次胸部X线检查。随后的影像学检查(胸部CT、PET-CT)显示前纵隔有一肿块(大小约为11 cm×10 cm×13 cm,SUV:8.7),对右心腔均有额外压迫。甲胎蛋白(aFP)血液水平至少超过正常范围50倍。在其他地方进行了两次连续的诊断尝试,组织学结果均不明确,细针穿刺活检(FNA活检)结果为恶性阴性,另一次肿瘤活检通过迷你前外侧(右侧)开胸手术进行,发现有“可疑”细胞聚集。患者入院后,进行了右侧电视辅助胸腔镜手术(VATS),同时进行了多次肿瘤活检和中度胸腔积液引流。根据术前胸部MRA数据,肿瘤大小增加至最大径:16 cm×9 cm×13 cm,右下叶实质(RLL)有严重肺不张,对上腔静脉(SVC)和右心窦有压迫移位效应。组织学报告显示为混合型非精原性生殖细胞纵隔肿瘤、后结节型畸胎瘤和胚胎癌。即将实施的化疗方案为“BEP”(博来霉素/顺铂/依托泊苷)方案,但由于发生急性肺栓塞事件,需改为“VIP”(顺铂/依托泊苷/异环磷酰胺)方案。虽然aFP血液水平下降,甚至恢复到正常水平,但肿瘤大小继续显著增加(最大径:28 cm×25 cm×13 cm),有严重的局部压迫效应、体重快速下降和临床状况逐渐恶化。因此,通过正中胸骨切开术并辅以“T形”迷你前外侧(右侧)开胸手术进行了紧急手术干预。切除了一个约4千克的巨大纵隔肿瘤,同时进行了右中叶和右上叶“整块”段切除术及部分纵隔胸膜剥脱术。后续组织学结果除了证实已知的后结节型畸胎瘤外,还显示有散在的小病灶,为混合型高级别横纹肌肉瘤、软骨肉瘤和骨肉瘤,以及大量高级别胶质母细胞瘤细胞聚集。未发现先前发现的非精原性生殖细胞和胚胎癌成分的明显迹象。患者术后状况逐渐改善,后续继续进行六个疗程的“TIP”(顺铂/紫杉醇/异环磷酰胺)治疗,目前由肿瘤学团队进行定期“随访”。本报告强调了早期准确组织学识别的重要性,以及结合任何必要的手术干预(诊断性或治疗性)和后续多模式管理计划的应用。纵隔肿瘤的多样性,尤其是对于年轻患者,容不得半点自满。此类罕见病例可能以同样不可预测的方式演变,迫使临床医生时刻保持警惕,不能想当然。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/6903/12384428/11ef9b803d37/curroncol-32-00423-g001.jpg

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