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平滑肌肉瘤:一种罕见的头颈部恶性肿瘤的预后概述。

Leiomyosarcoma: Prognostic outline of a rare head and neck malignancy.

机构信息

Stem Cell/Cell Culture Unit, Center For Advance Research, King George's Medical University, Chowk, Lucknow, Uttar Pradesh, India.

Department of Oral Pathology, MGM Dental College and Hospital, Navi Mumbai, Maharashtra, India.

出版信息

Oral Oncol. 2019 Aug;95:100-105. doi: 10.1016/j.oraloncology.2019.06.010. Epub 2019 Jun 13.

DOI:10.1016/j.oraloncology.2019.06.010
PMID:31345375
Abstract

Soft tissue sarcomas (STS) are mesenchymal malignant neoplasms with a broad spectrum of biologic behaviour. Most STS show predilection for extremities with rarity in head and neck. Leiomyosarcoma (LMS) is an extremely rare STS in head and neck due to the paucity of smooth muscles in this anatomical region. Owing to its rarity, diagnosis of LMS is often delayed or is often misdiagnosed. Our study aimed to evaluate clinico-demographic factors determining clinical course of primary head-neck LMS. Further, we also assessed cases of secondary head-neck LMS and LMS due to other causes to compare their clinical outcome with primary head-neck LMS. In primary LMS cases, intraoral LMS showed slightly better prognosis than extraoral LMS. Survival analysis revealed that prognosis of primary LMS was significantly better than secondary LMS. No significant difference in survival was seen between primary LMS and LMS due to other causes. These observations indicate that site of origin appears to determine the clinical behaviour of LMS. Results showed that size, recurrence and metastasis are important prognostic variables. Though large tumor size was associated with poor prognosis, tumor aggressiveness may not be directly proportional to its size. Surgical management with or without adjuvant therapy was associated with favourable outcome. As several factors are associated with prognostic outcome of head-neck LMS, multimodality therapy approach after careful analysis of various prognostic variables in each case on an individual basis is essential.

摘要

软组织肉瘤(STS)是一种具有广泛生物学行为的间叶恶性肿瘤。大多数 STS 倾向于发生在四肢,而在头颈部则很少见。由于头颈部平滑肌稀少,故平滑肌肉瘤(LMS)是一种极为罕见的头颈部 STS。由于其罕见性,LMS 的诊断常常被延误或误诊。本研究旨在评估决定原发性头颈部 LMS 临床病程的临床病理因素。此外,我们还评估了继发性头颈部 LMS 和其他原因引起的 LMS 的病例,以将其临床结果与原发性头颈部 LMS 进行比较。在原发性 LMS 病例中,口腔内 LMS 的预后略优于口腔外 LMS。生存分析显示,原发性 LMS 的预后明显优于继发性 LMS。原发性 LMS 和其他原因引起的 LMS 之间的生存率无显著差异。这些观察结果表明,起源部位似乎决定了 LMS 的临床行为。结果表明,大小、复发和转移是重要的预后变量。尽管大肿瘤大小与预后不良相关,但肿瘤侵袭性可能与其大小不成正比。手术治疗联合或不联合辅助治疗与良好的预后相关。由于头颈部 LMS 的预后与多个因素相关,因此在每个病例中,在仔细分析各种预后变量后,基于个体情况采用多模态治疗方法至关重要。

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