Heighpubs Otolaryngology and Rhinology

ISSN:2573-7716

Case Report

上颌窦的平滑肌肉瘤 - 一种罕见的临床实体

SAH BP.^1*，Chettri St.², Karki S², Shah SP¹，Manandhar S.¹and Jaiswal RK³

¹Associate Professor, Department of Otorhinolaryngology and Head & Neck Surgery, B.P. Koirala Institute of Health Sciences, Dharan, Nepal
²Professor, Department of Pathology, B.P. Koirala Institute of Health Sciences, Dharan, Nepal
³高级居民，Otorhinolaryngology和Head＆Neck手术部，B.P.Koirala尼泊尔达兰省卫生科学研究所

*Address for Correspondence:Dr. Bajarang Prasad Sah, MS, Associate Professor, Department of Otorhinolaryngology and Head and neck Surgery, B.P. Koirala Institute of Health Sciences, Dharan, Nepal, Tel: +9779842030356; Email: drbpsah.headneck@gmail.com

Dates:Submitted:25 June 2018;得到正式认可的：2018年7月16日;发布：17 July 2018

如何引用本文：SAH BP.，Chettri St., Karki S, Shah SP, Manandhar S, et al. Leiomyosarcoma of Maxillary Sinus – A Rare Clinical Entity. Heighpubs Otolaryngol and Rhinol. 2018; 2: 001-005. DOI:10.29328 / journal.hor.1001015.

Copyright License:© 2018 Sah BP, et al. This is an open access article distributed under the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.

Leiomyosarcoma accounts for 6.5 % of all soft-tissue sarcomas, and only 3% of them arise in the head and neck [1]. Leiomyosarcoma of the head and neck are believed to be originated in the tunica media of the blood vessels or pluripotent mesenchymal cells. Leiomyosarcoma with regional lymph node involvement are also rare and cervical metastasis has been reported in 15% of leiomyosarcomas arising in the oral cavity [2]. Clinically, leiomyosarcoma is very aggressive and carries a poor prognosis. The 5-year survival rate with disease-free status is 23 % [3].

Leiomyosarcoma of nose and paranasal sinuses is very rare. When they occur in the sinonasal tract, the most common sites are the nasal cavity, the maxillary sinus, and the ethmoid sinus, in decreasing order. We report a rare case of leiomyosarcoma of the maxillary sinus.

我们通过职业举行了56年的男性，左侧脸颊地区的痛苦肿胀6个月。自从过去1个月以来，他在与鼻梗阻相关的肿胀上产生了疼痛。没有遗产，馅蹄和松散的牙齿。临床检查显示出5×4平方米的单个球状肿胀，面部4厘米的左侧侧向中线，下方2cm以下，横向延伸至颧骨过程。边缘定义很好，坚定坚定的稠度，非柔软，不动，不粘附在皮肤上，而是贴近潜在的结构，对咳嗽和过敏试验没有脉冲是阴性的。有足够的嘴巴开口开口，牙齿多个尼古丁染色。在颈部，双侧没有可触及的淋巴结可触及（图1,2）。

在前鼻镜检查中，存在连接左下鼻甲与隔膜的纤维带。没有任何出血标志，没有任何明显的鼻塞或凸出。所有的颅神经都是完整的。制造了左颌骨恶性肿瘤的临床诊断。鼻子和剖腹产术的计算机断层扫描揭示了左颧骨和左脸颊区域5×4厘米的软组织质量。横向，它导致左侧脸颊中的局灶性骨骼的一部分颧骨破坏，维持用皮肤和皮下组织的中间脂肪平面。内侧后，左上颌窦的一部分后末端和前壁的部分破坏，延伸到上颌窦和左上颌窦内侧的薄膜，左上颌窦，封闭左上骨架堵塞并凸出到左侧鼻腔中。犹豫不染，在左侧的颌面膜的硬腭变薄和侵蚀，左侧翼形板，左侧内侧和横向翼形工艺。优越的是，左轨道地板的薄层和侵蚀，质量膨胀到左侧轨道。较低，延伸到缺失的infratemporal窝，丢失止脂平面，左侧有颞骨和肌肉肌肉。 The lesion was mildly heterogenously enhancing with few nonenchancing hypodense areas within. Soft tissue opacifications were seen in left maxillary sinus suggestive of retained secreations. The neck showed submental, bilateral submandibular and upper jugular lymph nodes, largest measuring 15mm (Figures 3,4).

On Fine Needle Aspiration Cytology from the left maxillary region yielded blood mixed aspirate and the cells were arranged in large tissue fragments, sheets, cluster and singly.The individual spindle cells showed moderate pleomorphism with dense bipolar to fibrillary cytoplasam,bizare looking cells with irregular nuclear membrane and prominent nucleoli. The overall cytomorphological features were those of Malignant Mesenchymal Tumor (Figure 5). The patient underwent total maxillectomy by lateral rhinotomy approach. The surgical wound was closed primarily without any flap. An obturator plate was placed at the defect of the hard palate (Figure 6).

Analysis of the biopsy specimen revealed that the tumor tissue comprised of fascicles of spindle-shaped cells with eosinophilic cytoplasm and oval to elongated and blunt ended vesicular nuclei. Mitosis was conspicuous. Areas of necrosis, fibrosis, hyalinization, and inflammatory infiltrates were seen. The reticulin stain showed abundant reticulin fibers around individual tumor cells. The van Gieson’s and Masson’s trichrome stains showed smooth-muscle differentiation in the spindle cells. The tumour cells showed strong vimentin and smooth muscle actin (SMA) stains. These features were suggestive of leiomyosarcoma.

The patient received 33#/ 66Gy of radiotherapy. The patient is currently well and under regular follow up for the past 5 years. Clinical evaluation and nasal endoscopy on follow up revealed normal mucosa with no sign of locoregional recurrence as well as distant metastasis (Figures 7-10).

Leiomyosarcoma are uncommon malignant neoplasams of smooth muscle origin that tend to occur in the alimentary tract, uterus and retroperitonuem. Leiomyosarcomas are very unusual soft tissue sarcomas of the head and neck region. The first case of leiomyosarcoma of nasal cavity and paranasal sinuses was reported in 1958 [4]. Since 1950, only 38 cases of leiomyosarcoma of the head and neck structures have been reported [5].

Leiomyosarcomas of the sinonasal tract are more common in men than in women. The average age of diagnosis is 50 years. Initial symptoms in order of decreasing frequency include nasal obstruction, epistaxis, facial pain, and facial swelling. Sinonasal tract leiomyosarcoma is characterized as locally aggressive with a low metastatic potential [1]. Our patient presented with painless facial swelling with nasal obstruction in the absence of epistaxis and locally invasive features. Sinonasal tract leiomyosarcoma is characterised as a locally aggressive lesion rather than having high metastatic potential [6]. The standard treatment is complete resection of the tumour with at least one cm margin. Radical resection alone having a high rate of local control but achieving a complete resection is usually not feasible due to anatomical constraints. Local recurrences are therefore common and a recurrence rate of 35-50% has been reported [7]. Metastasis occurs via hematogenous route most commonly to the lungs. Cervical metastasis, mostly due to contiguous spread has been reported in 15% cases [8].

In our case experience, the total maxillextomy with post operative radiotherapy is adequate and patient doing well without any local recurrences or distant metastatis in a follow up period of 5 years.

Leomyosarcoma of maxillary sinus is extremely rare. Accurate diagnosis, adequate surgical resection with adjuvent radiotherapy is the standard of care for such patient.