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鼻窦畸胎癌肉瘤:一种罕见且侵袭性极强的肿瘤。

Sinonasal Teratocarcinosarcoma: A Rare and Highly Aggressive Neoplasm.

作者信息

Hanif Huma, Misbah Dawood, Maqsood Saad, Iftikhar Sadia, Inam Yasir

机构信息

Clinical and Radiation Oncology, Shifa International Hospital Islamabad, Islamabad, PAK.

Clinical and Radiation Oncology, Shaukat Khanum Memorial Cancer Hospital and Research Centre, Peshawar, PAK.

出版信息

Cureus. 2023 Jul 5;15(7):e41396. doi: 10.7759/cureus.41396. eCollection 2023 Jul.

Abstract

Sinonasal teratocarcinosarcomas (SNTCSs) are malignant and highly aggressive neoplasms arising in the nasal cavity and paranasal sinuses with extension into surrounding structures and intracranial extension in few instances. They are commonly found in males (7-8 times more than in females). They pose difficulty in diagnosis due to their diverse histology. Ideal treatment modalities have not been devised yet due to the rare and highly aggressive nature of SNTCSs as shown in this case report. A 29-year-old Asian male was initially misdiagnosed on biopsy. Surgical debulking was performed initially which showed SNTCSs with radiological evidence of residual disease for which gross tumor clearance was done. He presented within a short span of a month, post re-resection with gross local recurrence. Urgent palliative radiotherapy was planned and started but unfortunately, he developed pulmonary and hepatic metastasis during radiation therapy and was commenced on palliative care only due to significant deterioration of performance status. Treatment of SNTCSs is often delayed due to their difficulty in diagnosis. Its highly aggressive nature prompts an urgent and aggressive treatment approach with adjuvant chemoradiation. Any type of adjuvant therapy is better than surgical resection only given its timely administration and close surveillance.

摘要

鼻窦畸胎癌肉瘤(SNTCSs)是发生于鼻腔和鼻窦的恶性且侵袭性很强的肿瘤,少数情况下可侵犯周围结构并向颅内蔓延。该病常见于男性(男性发病率是女性的7 - 8倍)。因其组织学表现多样,故诊断困难。如本病例报告所示,由于SNTCSs罕见且侵袭性强,目前尚未制定出理想的治疗方式。一名29岁的亚洲男性最初活检时被误诊。最初进行了手术减瘤,结果显示为SNTCSs,并有残留病灶的影像学证据,随后进行了肿瘤全切。他在再次切除术后短短一个月内就出现了局部广泛复发。于是计划并开始了紧急姑息性放疗,但不幸的是,他在放疗期间出现了肺和肝转移,由于身体状况显著恶化,仅接受了姑息治疗。由于SNTCSs诊断困难,其治疗往往会延迟。因其高度侵袭性,需要采取紧急且积极的治疗方法,辅以放化疗。鉴于辅助治疗的及时应用和密切监测,任何类型的辅助治疗都优于单纯手术切除。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/bfcd/10401896/22afc3fcb4b6/cureus-0015-00000041396-i01.jpg

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