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Year : 2018  |  Volume : 14  |  Issue : 9  |  Page : 542-543

Recurrent fibrosarcoma of maxillary sinus involving the cranial base: A rare case

1 Department of Oral and Maxillofacial Surgery, Peking University Shenzhen Hospital, Shenzhen, Guangdong 518001; Department of Stomatology, Anhui Medical University, Hefei, Anhui, 230000, P.R. China
2 Department of Oral and Maxillofacial Surgery, Peking University Shenzhen Hospital, Shenzhen, Guangdong 518001, China

Date of Web Publication29-Jun-2018

Correspondence Address:
Hong-Yu Yang
Department of Oral and Maxillofacial Surgery, Peking University Shenzhen Hospital, 1120 Lianhua Road, Shenzhen, Guangdong 518001
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/0973-1482.176180

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 > Abstract 

Adult fibrosarcoma (FS) of the maxillary sinus and the cranial base is a rare soft tissue sarcoma which is clinically characterized by a high frequency of local repeated recurrence if not excised widely. At present, the standard treatment option for FS is surgical resection. Here, we report a case of a 46-year-old male with a 5 months history recurrent FS of the maxillary sinus. The patient possessed an enormous mass in the right maxillofacial region extending to the cranial base, as observed by computed tomography. Histologically, the lessions were composed of hypercellular cells with heterogenous groups. The recurrence and pathologically heterogenous groups of FS lead to therapeutic complexities. Due to the challenging anatomy in head and neck region, it is crucial to define the lesion areas and weigh the balance between life quality and functional reconstruction.

Keywords: Cranial base, fibrosarcoma, maxillary sinus, recurrence

How to cite this article:
Jin L, Yang HY, Wang YF, Yang HJ. Recurrent fibrosarcoma of maxillary sinus involving the cranial base: A rare case. J Can Res Ther 2018;14, Suppl S2:542-3

How to cite this URL:
Jin L, Yang HY, Wang YF, Yang HJ. Recurrent fibrosarcoma of maxillary sinus involving the cranial base: A rare case. J Can Res Ther [serial online] 2018 [cited 2019 Oct 16];14:542-3. Available from: http://www.cancerjournal.net/text.asp?2018/14/9/542/176180

 > Introduction Top

Fibrosarcoma (FS), defined by 2013 World Health Organization (WHO) as a malignancy composed of “herringbone” architecture fibroblasts, is a rare soft tissue sarcoma, with extremities being the most common affected region. Only 7% to 10% of FSs arise in head and neck region. FS of maxillary sinus can be found a few in the literature.[1] The generally accepted criteria of diagnosis are immunohistochemistry and histopathology. Unfortunately, The case of locoregional recurrence has frequently been observed, while the etiology is unknown.[2] The current study provides the case of a 46-year-old male with the right maxillofacial recurrence of pathologically diagnosed FS and discusses some relevant literature.

 > Case Report Top

In January 2015, a 45-year-old male who had presented with right cheek swelling underwent right maxillary sinus resection by induction radiation with PT32Gy/16 fractions in other institution. 5 months later, he referred to our institution hospital for the finding of tumor recurrence observed by magnetic resonance imaging (MRI). Multimodality imagings including MRI and computed tomography (CT) demonstrated enormous mass without right maxillary sinus extending posteriorly to the frontotemporal apophysis, superior to the orbital floor and inferomedial to the wing of the sphenoid and zygomatic arch. Thus, neither narrow lumen nor ectasia of anteries was observed, as indicated from CT angiography. There was no radiographic evidence of lyphadenopathy and distant metastasis of abdomen and chest.

A weber-Fergusson incision was performed with preauricular stretching on the right side to allow exposure of the lesions. According to the consulted suggestions from ophthalmologists and neurosurgeon, extended tumor resection was performed including local excision of right mandibular and parotid, total excision of the right maxillary. The eyeball overhanging by muscles was finally preserved. The tumor was excised along with the infiltrated right masseter, medial and lateral pterygoids. With all obvious lesions being resected, we reconstructed the defect of the durawith a random and temporial flap.

The excised gray-white specimen with multinodules was measured 110 mm × 100 mm × 40 mm. Macroscopically, the section was characterized with fusiform spindle cells exhibiting distinct heteromorphosis. A variable amount of mitotic activities and vascular proliferation were observed. The immunohistochemical assay demonstrated that tumor tissues expressed strong immunoreactivity for clusters of differentiation 31 (CD31) and vimentin. The labeling index of ki-67 was 60%. Negative results were found for other markers including smooth muscle actin, S-100, CD34, and cytokeration7. Based on the above findings, the tumor was suggestive of a malignant mesenchymal FS.

An incomplete excision near the dura and several approximately 10 mm × 26 mm sized cervical lymph nodes from metastasis were suggested in postoperative CT and MRI, the patient underwent chemotherapy with epirubicin 70 mg d1, 2 and cytoxan 5.0 d1, 2/q21d.

 > Discussion Top

In head and neck region, soft tissue sarcoma comprised a rare form of malignancies, with extremities occupying two-thirds of all. Only a limited number of cases concerning FS of maxillary sinus were published.[3] As a result, management has not been standard, a prospective program of treatment has not been developed, and clinical outcomes frequently relied on diverse states of patient cohort. According to the American Joint Committee on cancer staging system, soft tissue sarcoma is classified by its pathology, size, presence of regional and distant metastasis. However, the factor which is being ignored is the unique of head and neck region. For example, the resectability of lesions involving the important vessels and cranial base are not included in this system.[4]

The exact etiology of the lesion is still under exploration. Radiation exposure has been thought to be a dispreposing factor for FS, followed by genetic alterations. Wadhwan et al.[5] has also indicated that tissue damages, such as scarring, may play a role. FS was over diagnosed in earlier studies, and the final diagnosis was frequently applied to hypercellular lesions composed of collagen-forming spindle cells by WHO in 2002.[5] Myofibrosarcoma has been suggested for this entity because of positive immunoreactivity for CD31 and Vimentin. Sometimes it might be diagnosed benign because of “bland” histological features like inconspicuous nuclear pleomorphism. Actually, similar to some aggressive sarcomas, multiple local recurrences were presented. Some studies had suggested that tumor size <5 cm was a positive risk factor for local recurrence. Besides, other recurrence factors were the margin status of resected tumor and whether FS was just a de novo or recurrent lesion.[2],[6] The range between 3 cm and 5 cm required for resection margin was extremely difficult to achieve because of close proximity to critical anatomic structure, such as lessions involving great vessels and cranial base. As a matter of fact, failure of local control and intracranial extension were responsible for its high mortality. The overall survival of <70% at 2 years, <40 at 5 years.[2],[7]

The current cornerstone of treatment remains surgery, recommended as extensive radical resection with tumor-free margin. Adjuvant chemotherapy and radiotherapy were used for positive microscopic margin or incomplete surgical excision. FSs of the maxillary sinus and cranial base significantly were worse than other anatomic sub-sites, implying a more aggressive therapies for these lesions.[1],[6]

In our patient, a large recurrent FS of maxillary sinus involving cranial base had invaded the cervical lymph nodes, which was extremely rare. Kuhn et al.[7] has reported 4% cervical lymphonodes metastasis in this region. The current report may increase the cognition of FS in head and neck region, especially involving the cranial base. In this region, multimodality imagings should be used to define tumor size, margin and relationship with arteries because of critical anatomic structure and important vessel, such as eyes, dura, and vertebral artery. Thus, in this region, FS usually develops aggressive with repeated local recurrence and low risk of metastasis, suggesting that extensive radical resection and postoperative radiation may be beneficial for our patient. Furthermore, more studies should be focused on the etiology and mechanism. Finally, the standard stage system and prospective program of treatment in this special region is required.

Financial support and sponsorship

This work was supported by the National Natural Science Foundation of China (Grant no. 81572654), International cooperation Foundation of Shenzhen (Grant no. ZYA201106100080A) and Shenzhen Science and Research Innovation Foundation (Grant no. JCYJ20150403091443303).

Conflicts of interest

There are no conflicts of interest.

 > References Top

Bercin S, Muderris T, Kiris M, Kanmaz A, Kandemir O. A rare sinonasal neoplasm: Fibrosarcoma. Ear Nose Throat J 2011;90:E6-8.  Back to cited text no. 1
Mansouri H, Rzin M, Marjani M, Sifat H, Hadadi K, Hassouni K, et al. Fibrosarcoma of the maxillary sinus. Indian J Otolaryngol Head Neck Surg 2006;58:104-5.  Back to cited text no. 2
Kotilingam D, Lev DC, Lazar AJ, Pollock RE. Staging soft tissue sarcoma: Evolution and change. CA Cancer J Clin 2006;56:282-91.  Back to cited text no. 3
Shuman AG, Brennan MF, Palmer FL, Kuk D, Moraco N, Singer S, et al. Soft tissue sarcoma of the head and neck: Nomogram validation and analysis of staging systems. J Surg Oncol 2015;111:690-5.  Back to cited text no. 4
Wadhwan V, Chaudhary MS, Gawande M. Fibrosarcoma of the oral cavity. Indian J Dent Res 2010;21:295-8.  Back to cited text no. 5
[PUBMED]  [Full text]  
Maliki O, Aleksandrov O, Carles P, Planquart X, Vaz E, Bertrand C, et al. Fibrosarcoma of the nasal cavity: A case report. Egypt J Ear Nose Throat Allied Sci 2014;15:275-7.  Back to cited text no. 6
Kuhn FA, Javer AR. Low-grade fibrosarcoma of the anterior skull base: Endoscopic resection and repair. Am J Rhinol 2003;17:347-50.  Back to cited text no. 7


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