Adjuvant radiotherapy following surgical resection for sinonasal leiomyosarcoma: a systematic review and case series
Hyuk Jin Kwun1
, Sandro de Paiva Leite1, Andy Chua2, Omid Ahmadi3,4
Contributions: (I) Conception and design: HJ Kwun, S de Paiva Leite; (II) Administrative support: HJ Kwun; (III) Provision of study materials or patients: HJ Kwun; (IV) Collection and assembly of data: HJ Kwun; (V) Data analysis and interpretation: HJ Kwun, S de Paiva Leite, O Ahmadi; (VI) Manuscript writing: All authors; (VII) Final approval of manuscript: All authors.
Background: Sinonasal leiomyosarcoma (SNL) is a rare malignancy with limited evidence guiding optimal post-operative management. This study aims to assess the efficacy of adjuvant radiotherapy following surgical resection for SNL, alongside a case series.
Methods: Two patients with SNL underwent surgical resection—one at Manukau Super Clinic, Auckland, New Zealand, and the other presented by otolaryngologists from Sengkang General Hospital, Singapore. A systematic review of studies published between January 2000 and August 2024 identified those involving surgical resection followed by adjuvant radiotherapy. Databases used include PubMed, Ovid Medline, and the Cochrane Library. Studies were screened by title and abstract, and relevant articles were reviewed for eligibility. A descriptive synthesis was performed to summarize the findings.
Results: The review identified 12 patients (age range, 28–84 years) treated with resection and adjuvant radiotherapy. Radiation doses ranged from 35 to 72 Gy, with follow-up from 9 months to 5 years. Nine patients showed no recurrence, one had local recurrence, one had distant metastasis, and two had unclear outcomes. Surgical methods included endoscopic resection, maxillectomy, and two unspecified approaches.
Conclusions: The review shows inconclusive outcomes for adjuvant radiotherapy after surgery. Our two cases align with the literature, supporting surgery as the primary treatment. However, further research is needed to assess the role of adjuvant therapy due to the rarity of this condition.
Keywords: Nasal cavity; paranasal sinuses; leiomyosarcoma; radiotherapy; adjuvant
Received: 24 February 2025; Accepted: 27 June 2025; Published online: 04 September 2025.
doi: 10.21037/ajo-25-22
Introduction
Sinonasal leiomyosarcomas (SNLs) are rare, aggressive malignant tumours arising from smooth muscle cells, generally believed to originate from the tunica media of blood vessels or pluripotent mesenchymal cells (1). They are typically associated with a poor prognosis due to their highly aggressive nature and local invasive potential (1). The primary treatment for SNLs is complete surgical resection, particularly for tumours of the nasal cavity, which offers favourable outcomes and lower recurrence rates (2). Additional treatments may include adjuvant radiotherapy and, in some cases, chemotherapy; however, the role of these adjuvant therapies remains controversial and is less well-defined (2).
Current literature on the efficacy of adjuvant radiotherapy in treating SNLs is limited, creating uncertainty regarding its role in the management of this rare tumour. In this study, we report clinical progress of two patients with SNL who underwent surgical resection but refused adjuvant therapy, followed by a systematic review with the aim of assessing the efficacy of adjuvant radiotherapy in management of SNL. This review aims to clarify the potential role of adjuvant radiotherapy in improving outcomes for patients with SNLs.
Methods
Case series data collection
The first patient presented to Rhinology Clinic in Manukau Super Clinic in Auckland, New Zealand. The second patient was treated at Sengkang General Hospital in Singapore. Electronic medical records of each patient were reviewed and data were collected.
Ethical considerations
The study was conducted in accordance with the Declaration of Helsinki and its subsequent amendments. This study involves two de-identified case reports and a systematic review of publicly available data, posing minimal risk and preserving patient confidentiality. As such, ethical review for the case reports was waived in accordance with institutional guidelines and the principles outlined in the Declaration of Helsinki. Written informed consent was obtained from the patients for the publication of this manuscript and any accompanying images. A copy of the written consent is available for review by the editorial office of this journal.
Literature search strategy
This review was conducted using the PRISMA reporting guidelines (available at https://www.theajo.com/article/view/10.21037/ajo-25-22/rc). Databases used include PubMed, Ovid Medline, and the Cochrane Library. Studies were not limited to study design to capture a wide range of results. Two independent investigators conducted literature search and data collection.
Search
Search terms included both medical subject headings (MeSH) and non-MeSH terms. MeSH terms used include: nasal cavity, paranasal sinuses, nose, leiomyosarcoma, radiotherapy, chemoradiotherapy, and adjuvant. Non-MeSH terms include: nose, sinus, leiomyosarcoma, radiation treatment, and radiation. A combination of these terms is used in the databases for the search.
Eligibility criteria
All studies that reported outcomes for SNL after treatment by surgical resection and adjuvant radiotherapy were included. Studies published in English between January 2000 and August 2024 were eligible for inclusion. Study types included case reports, case series, and retrospective studies.
Exclusion criteria included primary tumours outside of the sinonasal cavity, patients who did not receive radiotherapy, or receiving both radiotherapy and other systemic therapies.
Systematic review data collection process
Relevant data including origin of SNL, radiation dose (if available), follow-up period, and outcome, including recurrence rate, disease-free survival, and overall survival were collected and analysed.
Statistical analysis
Due to the diversity of the available studies and the inconsistency in how outcomes were reported, no meta-analysis was conducted. Consequently, a descriptive synthesis of the available data was conducted.
Results
Series of cases
Case 1
A 70-year-old Caucasian female presented with a 1-year history of chronic unilateral nasal obstruction and recurrent sinusitis associated with anosmia. She was a non-smoker with no other significant past medical history. Examination revealed a pale polypoid mass filling the left nasal cavity, extending from the middle meatus to obstruct bilateral choanae. A computed tomography (CT) scan of the sinuses demonstrated a large expansile mass in the left nasal cavity, extending into left maxillary sinus and ethmoids (Figure 1).
Endoscopic resection was performed, and post-operative histology confirmed a high grade, Fédération Nationale des Centres de Lutte Contre Le Cancer (FNCLCC) grade 3 leiomyosarcoma.
The post-operative PET-CT demonstrated small right level two nodes [maximum standardized uptake (SUVmax), 23.8], with no uptake in the sinonasal cavity and no distant metastases. An ultrasound-guided fine needle aspirate cytology of the right neck nodes showed no evidence of malignancy.
After discussion at a multi-disciplinary tumour board, the recommendation was to proceed with a wider resection of the margins of the primary lesion followed by adjuvant radiotherapy. She thus underwent surgery to completely strip the left maxillary sinus mucosa, and histology confirmed no evidence of residual tumour. The patient refused adjuvant radiotherapy treatment and has been follow-up in clinic for 4 years post-operatively with no signs of recurrence.
Case 2
A 52-year-old Chinese male first presented to Neurology Department with daily episodic left-sided headaches lasting 5 to 30 minutes, associated with left eye epiphora, redness, and clear rhinorrhoea. He was treated for cluster headache with no improvement, and thus underwent a CT scan of the brain which demonstrated partially imaged left-sided sinus mucosal thickening with no intracranial lesions seen (Figure 2). He was referred to the Rhinology Department for review.
Physical examination revealed a normal eye exam. Nasoendoscopy demonstrated a soft tissue mass arising from and replacing the entire left middle turbinate. A biopsy was performed in clinic, which returned as leiomyoma with atypical features and few mitoses (2 per 10 high-powered fields). A contrasted CT of the sinuses showed a 1 cm polypoid lesion arising from the middle turbinate, which enhanced similarly to sinonasal mucosa. Surgical resection was recommended, but patient initially refused any surgical intervention. Finally, 15 months after the initial diagnosis, the patient underwent surgical resection. By this time, the tumour had grown and taken on a vascular appearance. The lesion was resected along with the middle turbinate, with margins taken intraoperatively for frozen section—these were all uninvolved by tumour. The final histology returned as FNCLCC grade 1 leiomyosarcoma. The histology on the initial biopsy was then reviewed retrospectively and despite the knowledge of the definitive surgical histology, a definite diagnosis of leiomyosarcoma could not have been made based on the biopsy results.
The case was discussed at a multidisciplinary sarcoma board, with review of histology by an independent pathologist. CT of the thorax, abdomen, and pelvis showed no evidence of distal metastases. Adjuvant radiotherapy was recommended, but it was declined by the patient. He was last seen 3 years post-operatively with no evidence of recurrence.
Systematic review
Study selection
The literature search retrieved 56 articles, of which 15 were duplicates. After exclusion of duplicates, 41 studies were screened based on their titles and abstracts. From these, 18 were selected for full-text review, and 11 met the inclusion criteria (Figure 3). As summarized in Table 1, 10 of the included studies each presented one eligible patient, while one retrospective observational study presented two eligible patients.
Table 1
| Study | Number of patients, age (years), and gender | Treatment | Radiation dose (Gy) | Follow-up | Outcome | Grade |
|---|---|---|---|---|---|---|
| Rana et al. (3) | N=1, 28, M | Endoscopic tumour incomplete resection + radiation (sphenoid sinus) | 60 | Not mentioned | Not mentioned | Well-differentiated |
| Saadoun et al. (4) | N=1, 84, F | Endoscopic tumour resection followed by revision + radiotherapy | 52.5/64.5 in 2 fields | 9 months | No recurrence. No metastases | N/A |
| Chew et al. (5) | N=1, 36, M | Left maxillary sinus, subtotal maxillectomy | N/A | 3 years | No metastasis | N/A |
| Papoian et al. (6) | N=1, 83, F | Endoscopic tumour resection Followed by revision + radiotherapy and 3rd revision |
35 | 28 months | Recurrence after operation + radiotherapy (1 year later). Now 16 months free of tumour | Low-intermediate |
| d’Adesky et al. (7) | N=1, 72, F | Endoscopic resection followed by radiotherapy | 60 on the tumoural bed (five 2-Gy fractions/week) | 12 months | No recurrence. No metastases | 3 |
| Prasad et al. (8) | N=1, 42, F | Total left maxillectomy + radiotherapy | N/A | 18 months | No recurrence | N/A |
| Keck et al. (9) | N=1, 68, F | Endoscopic resection (ethmoids) + radiotherapy | 72 | 29 months | No recurrence or metastasis | N/A |
| Montgomery et al. (10) | N=2 | |||||
| (I) 37, M | (I) Maxillectomy + radiotherapy | (I) N/A | (I) 36 months | (I) No recurrence | (I) 2 | |
| (II) 32, F | (II) Maxillectomy + radiotherapy (maxillary/turbs) | (II) N/A | (II) 24 months | (II) Metastasis to calf 24 months | (II) 3 | |
| Huang et al. (11) | N=1, 37, M | Surgical excision + radiotherapy | N/A | 24 months | No recurrence | Low |
| Agaimy et al. (12) | N=1, 77, M | Right nasal cavity + maxillary sinus. Excision and radiotherapy | N/A | N/A | N/A | N/A |
| Sah et al. (13) | N=1, 56, M | Total left maxillectomy + radiotherapy | 66 | 5 years | No recurrence | N/A |
F, female; M, male; N/A, not available.
Synthesis of results
The 11 studies included a total of 12 patients with SNL who underwent surgical resection followed by adjuvant radiotherapy.
Patient ages ranged from 28 to 84 years, with a median age of 49 years [interquartile range (IQR), 38 years]. The dose of radiation ranged from 35 to 72 Gy, and the follow-up time ranged from 9 months to 5 years. The mean follow-up time was 29 months [standard deviation (SD), 9.5 months].
Five out of 12 patients had endoscopic resection, five had maxillectomy, and two were not specified.
Of the 12 patients, 9 did not experience local recurrence during their follow-up. Of the remaining three, one patient [reported by Papoian et al. (6)] had a local recurrence 1 year after the initial surgery, requiring revision surgery and radiotherapy. The other two patients [reported by Rana et al. (3) and Agaimy et al. (12)] had no clear information about local recurrence or distal metastasis. Additionally, one patient [reported by Montgomery et al. (10)] had distant metastasis to the calf but no local recurrence at 24 months post-surgical resection.
Results are summarised in Table 1.
Risk of bias and quality assessment
The quality and risk of bias of the included studies were assessed using the Joanna Briggs Institute (JBI) critical appraisal checklist (Appendix 1). All studies scored six out of 10 or higher, indicating a generally high level of methodological quality. However, four of the 11 studies provided inadequate information on radiation dosing, and three lacked sufficient detail on the surgical procedures performed, including specific complications and adverse events (Table 2).
Table 2
| Study | Risk of bias assessment | JBI score | Comments | |||||||||
|---|---|---|---|---|---|---|---|---|---|---|---|---|
| 1 | 2 | 3 | 4 | 5 | 6 | 7 | 8 | 9 | 10 | |||
| Rana et al. | Y | Y | Y | Y | Y | N | N | Y | – | – | 6/8 | Inadequate follow-up documentation |
| Saadoun et al. | Y | Y | Y | Y | Y | Y | Y | Y | – | – | 8/8 | – |
| Chew et al. | Y | Y | Y | Y | N | Y | Y | N | – | – | 6/8 | Inadequate information on radiation dosing |
| Papoian et al. | Y | Y | Y | Y | Y | Y | Y | N | – | – | 7/8 | – |
| d’Adesky et al. | Y | Y | Y | Y | Y | Y | Y | Y | – | – | 8/8 | – |
| Prasad et al. | Y | Y | Y | Y | N | Y | Y | N | – | – | 6/8 | Inadequate information on radiation dosing |
| Keck et al. | Y | Y | Y | Y | Y | Y | Y | N | – | – | 7/8 | – |
| Montgomery et al. | Y | Y | Y | Y | Y | Y | N | Y | Y | Y | 9/10 | Inadequate information on radiation dosing |
| Huang et al. | Y | Y | Y | Y | Y | Y | N | Y | Y | Y | 9/10 | – |
| Agaimy et al. | Y | Y | Y | Y | N | Y | Y | N | – | – | 6/8 | Inadequate information on radiation dosing |
| Sah et al. | Y | Y | Y | Y | Y | Y | Y | Y | – | – | 8/8 | – |
The JBI checklist for case reports and case series is consists of 8 and 10 questions respectively. This is available at https://jbi.global/critical-appraisal-tools. JBI, Joanna Briggs Institute; N, no; Y, yes.
Discussion
This systematic review identified 11 studies published between January 2000 and August 2024 that reported on SNL treated with surgical resection followed by adjuvant radiotherapy. Among 12 patients across these studies, 9 remained recurrence-free during the follow-up period. In addition, we presented two local cases of completely resected SNL, both managed endoscopically without recurrence over 3–4 years, despite declining adjuvant radiotherapy. These findings suggest that surgical resection remains the cornerstone of treatment for SNL, with adjuvant radiotherapy potentially offering additional benefit in selected cases.
Our findings are broadly aligned with the existing literature on leiomyosarcoma in the head and neck. Leiomyosarcoma accounts for 6.5% of all soft-tissue sarcomas, but only 3% arise in the head and neck region (14). These tumours are thought to originate from either the tunica media of blood vessels or from pluripotent mesenchymal cells. Complete surgical resection with clear margins is consistently associated with improved outcomes, particularly in low-grade, well-differentiated tumours (10,15,16). Montgomery et al. (10) reported that four of five patients with grade 1–2 tumours who underwent complete resection remained recurrence-free after 4 years. Wu et al. found an 88% 5-year survival in well-differentiated tumours vs. 53% in poorly differentiated ones (17). Tumour location also impacts prognosis: lesions arising in the nasal cavity have more favourable outcomes than those from the paranasal sinuses (18).
Adjuvant radiotherapy is commonly employed when complete surgical resection is not feasible, especially in tumours near vital structures such as the orbit or skull base (19-21). In such cases, radiotherapy targets residual disease at the tumour bed when margin status is unclear (4,22). Shellenberger also supports its use in high-grade tumours (23). However, recurrence remains high—up to 55% in some series—even after adjuvant treatment (7,19,20). Chemotherapy, in contrast, is generally considered the least effective modality (24). In our two cases, both patients declined radiotherapy, likely due to the lack of clear supporting evidence. This highlights the need for stronger data to guide shared decision-making and underscores how evidence gaps can influence patient choices.
This review has several limitations. SNL is an exceptionally rare malignancy, and only 11 eligible studies were identified over 24 years. The small number of cases introduces a high risk of publication bias, and several studies had limited follow-up durations. Key clinical data were often missing: only six of the 11 studies reported radiation dosing, with a wide range from 35 to 72 Gy. Among these, Saadoun et al. (4) reported 52.5 Gy/64.5 Gy in two fields, and d’Adesky et al. (7) administered 60 Gy to the tumour bed (five 2-Gy fractions per week); however, both studies had follow-up durations of only 9–12 months, potentially missing late recurrences. Additionally, tumour grade was known in only six of the 12 patients included, limiting interpretation of outcomes based on histological differentiation. Three studies lacked detail on the surgical technique used or the associated complications and adverse events.
Further research is necessary to define the role of adjuvant radiotherapy in the management of SNL. Prospective, multi-institutional studies with longer follow-up and comprehensive clinical reporting are essential. Until such data are available, surgical resection with clear margins remains the primary treatment modality. Adjuvant radiotherapy should be considered selectively—particularly for high-grade tumours, positive margins, or cases involving critical anatomical constraints—guided by multidisciplinary input and patient preferences.
Conclusions
In conclusion, our experience with the two cases presented corroborate the existing literature, reinforcing the assertion that surgical resection should be the primary treatment modality. Although adjuvant radiotherapy may possess a potential role, its efficacy remains ambiguous due to the paucity of robust evidence. Further studies are essential to elucidate its effectiveness and to formulate more definitive treatment guidelines.
Acknowledgments
The author H.J.K. would like to acknowledge Dr. Salil Nair, Dr. Andy Chua, Dr. Tze Choong Charn, and Dr. Joyce Tang for providing the cases included in the case series and extends gratitude to the patients for their consent to have these cases presented.
Footnote
Reporting Checklist: The authors have completed the PRISMA reporting checklist. Available at https://www.theajo.com/article/view/10.21037/ajo-25-22/rc
Data Sharing Statement: Available at https://www.theajo.com/article/view/10.21037/ajo-25-22/dss
Peer Review File: Available at https://www.theajo.com/article/view/10.21037/ajo-25-22/prf
Funding: None.
Conflicts of Interest: All authors have completed the ICMJE uniform disclosure form (available at https://www.theajo.com/article/view/10.21037/ajo-25-22/coif). The authors have no conflicts of interest to declare.
Ethical Statement: The authors are accountable for all aspects of the work in ensuring that questions related to the accuracy or integrity of any part of the work are appropriately investigated and resolved. The study was conducted in accordance with the Declaration of Helsinki and its subsequent amendments. Ethical review for the case reports was waived in accordance with institutional guidelines and the principles outlined in the Declaration of Helsinki. Written informed consent was obtained from the patients for the publication of this manuscript and any accompanying images. A copy of the written consent is available for review by the editorial office of this journal.
Open Access Statement: This is an Open Access article distributed in accordance with the Creative Commons Attribution-NonCommercial-NoDerivs 4.0 International License (CC BY-NC-ND 4.0), which permits the non-commercial replication and distribution of the article with the strict proviso that no changes or edits are made and the original work is properly cited (including links to both the formal publication through the relevant DOI and the license). See: https://creativecommons.org/licenses/by-nc-nd/4.0/.
References
- Schenberg ME, Slootweg PJ, Koole R. Leiomyosarcomas of the oral cavity. Report of four cases and review of the literature. J Craniomaxillofac Surg 1993;21:342-7. [Crossref] [PubMed]
- Eppsteiner RW, DeYoung BR, Milhem MM, et al. Leiomyosarcoma of the head and neck: a population-based analysis. Arch Otolaryngol Head Neck Surg 2011;137:921-4. [Crossref] [PubMed]
- Rana D, Gautam R, Mallya V, et al. Leiomyosarcoma of sphenoid sinus- A rare tumor at an unusual site. J Cancer Res Ther 2024;20:485-7. [Crossref] [PubMed]
- Saadoun R, Obermueller T, Franke M, et al. Leiomyosarcoma of the Nasal Cavity. Ear Nose Throat J 2022;101:NP218-21. [Crossref] [PubMed]
- Chew YK, Noorizan Y, Khir A, et al. Leiomyosarcoma of the maxillary sinus. Med J Malaysia 2009;64:174-5.
- Papoian V, Yarlagadda BB, Devaiah AK. Multifocal, recurrent sinonasal leiomyosarcoma: case report and review of literature. Am J Otolaryngol 2014;35:254-6. [Crossref] [PubMed]
- d'Adesky C, Duterme JP, Lejeune D, et al. Leiomyosarcoma of the inferior nasal concha: a case report and literature review. B-ENT 2012;8:213-7.
- Prasad KC, Alva TB, Khadilkar U, et al. Leiomyosarcoma of the maxillary sinuses: report of two cases. Ear Nose Throat J 2004;83:122-124, 126-7.
- Keck T, Mattfeldt T, Kühnemann S. Leiomyosarcoma of the ethmoidal cells. Rhinology 2001;39:115-7.
- Montgomery E, Goldblum JR, Fisher C. Leiomyosarcoma of the head and neck: a clinicopathological study. Histopathology 2002;40:518-25. [Crossref] [PubMed]
- Huang HY, Antonescu CR. Sinonasal smooth muscle cell tumors: a clinicopathologic and immunohistochemical analysis of 12 cases with emphasis on the low-grade end of the spectrum. Arch Pathol Lab Med 2003;127:297-304. [Crossref] [PubMed]
- Agaimy A, Semrau S, Koch M, et al. Sinonasal Leiomyosarcoma: Clinicopathological Analysis of Nine Cases with Emphasis on Common Association with Other Malignancies and Late Distant Metastasis. Head Neck Pathol 2018;12:463-70. [Crossref] [PubMed]
- Sah BP, Chettri ST, Karki S, et al. Leiomyosarcoma of Maxillary Sinus – A Rare Clinical Entity. Heighpubs Otolaryngology and Rhinology 2018. doi:
10.29328/journal.hor.1001015 . - Tanaka H, Westesson PL, Wilbur DC. Leiomyosarcoma of the maxillary sinus: CT and MRI findings. Br J Radiol 1998;71:221-4. [Crossref] [PubMed]
- Kwok MMK, Lee S, Hosking P. Leiomyosarcoma: a rare sinonasal malignancy. BMJ Case Rep 2018;2018:bcr2017224110. [Crossref] [PubMed]
- Nayak DR, Ghanpur AD, Reddy AN, et al. Smooth muscle tumour of uncertain malignant potential (SMTUMP) in the nasal cavity: an incidental finding. BMJ Case Rep 2016;2016:bcr2016217386. [Crossref] [PubMed]
- Wu AW, Suh JD, Metson R, et al. Prognostic factors in sinonasal sarcomas: analysis of the surveillance, epidemiology and end result database. Laryngoscope 2012;122:2137-42. [Crossref] [PubMed]
- Fusconi M, Magliulo G, Della Rocca C, et al. Leiomyosarcoma of the sinonasal tract: a case report and literature review. Am J Otolaryngol 2002;23:108-11. [Crossref] [PubMed]
- Sankary S, Sherwin RN, Malone PS, et al. Clonal chromosomal aberrations in a leiomyosarcoma of the sinonasal tract. Cancer Genet Cytogenet 1993;65:21-6. [Crossref] [PubMed]
- Ulrich CT, Feiz-Erfan I, Spetzler RF, et al. Sinonasal leiomyosarcoma: review of literature and case report. Laryngoscope 2005;115:2242-8. [Crossref] [PubMed]
- Saluja TS, Iyer J, Singh SK. Leiomyosarcoma: Prognostic outline of a rare head and neck malignancy. Oral Oncol 2019;95:100-5. [Crossref] [PubMed]
- Josephson RL, Blair RL, Bedard YC. Leiomyosarcoma of the nose and paranasal sinuses. Otolaryngol Head Neck Surg 1985;93:270-4. [Crossref] [PubMed]
- Shellenberger TD, Sturgis EM. Sarcomas of the head and neck region. Curr Oncol Rep 2009;11:135-42. [Crossref] [PubMed]
- Kuruvilla A, Wenig BM, Humphrey DM, et al. Leiomyosarcoma of the sinonasal tract. A clinicopathologic study of nine cases. Arch Otolaryngol Head Neck Surg 1990;116:1278-86. [Crossref] [PubMed]
Cite this article as: Kwun HJ, de Paiva Leite S, Chua A, Ahmadi O. Adjuvant radiotherapy following surgical resection for sinonasal leiomyosarcoma: a systematic review and case series. Aust J Otolaryngol 2025;8:38.
