Next Article in Journal
Exploring Extracellular Vesicle Surface Protein Markers Produced by Glioblastoma Tumors: A Characterization Study Using In Vitro 3D Patient-Derived Cultures
Previous Article in Journal
Beta-2-Microglobulin Maintains Overall Survival Prediction in Binet A Stage Chronic Lymphocytic Leukemia Patients with Compromised Kidney Function in Both Treatment Eras of Chemoimmunotherapy and Targeted Agents
 
 
Font Type:
Arial Georgia Verdana
Font Size:
Aa Aa Aa
Line Spacing:
Column Width:
Background:
Systematic Review

Biphenotypic Sinonasal Sarcoma: Literature Review of a Peculiar Pathological Entity—The Neurosurgical Point of View

1
Department of Neurosciences and Reproductive and Odontostomatological Sciences, Division of Neurosurgery, University of Naples “Federico II”, 80131 Naples, Italy
2
Department of Neurosciences and Reproductive and Odontostomatological Sciences, Division of Ophthalmology, University of Naples “Federico II”, 80131 Naples, Italy
3
Multidisciplinary Department of Medical-Surgical and Dental Specialties, University of Campania “Luigi Vanvitelli”, 81100 Caserta, Italy
4
Department of Advanced Biomedical Sciences, University of Naples “Federico II”, 80131 Naples, Italy
*
Author to whom correspondence should be addressed.
Cancers 2024, 16(22), 3747; https://doi.org/10.3390/cancers16223747
Submission received: 13 October 2024 / Revised: 24 October 2024 / Accepted: 5 November 2024 / Published: 6 November 2024
(This article belongs to the Section Cancer Pathophysiology)

Simple Summary

Biphenotypic sinonasal sarcoma is a rare low-grade tumor arising from the mucosa of the upper respiratory tract characterized by aggressive biological behavior and a high tendency to invade the skull base and the orbit. The main presenting symptoms and signs include nasal obstruction, facial discomfort, epistaxis and ocular impairment. Surgical resection represents the gold standard of treatment, allowing for the resolution of clinical manifestations and gross total tumor resection in most cases; the role of adjuvant treatments is unclear. The local recurrence rate after treatment is 26%. Tumor-related mortality is very rare.

Abstract

Background: Biphenotypic sinonasal sarcoma (BSNS) is a low-grade tumor of the sinonasal tract with frequent extension to the orbit and skull base. Due to its rare incidence and recent histopathological and molecular characterization, little data are available in regard to its natural history, treatment and surveillance protocol. Methods: A comprehensive literature review in Embase online electronic databases on BSNS was made. The analyzed factors included the patients’ sex and age, presenting symptoms and signs, anatomical origin and pattern of growth of the tumor, immunohistochemical and molecular features, time to treatment, type of treatment, surgical approach, extent of resection, peri- and post-operative complications, adjuvant therapies, clinical outcome, recurrence and overall survival rates. Results: This literature review involved 34 studies for an overall series of 149 cases of BSNS. The female (66.9%) and middle-aged populations (median 54.88 years old) were mainly affected. The most frequent clinical onset was nasal obstruction (81%), followed by facial discomfort (44%), epistaxis (15.5%) and ocular impairment (14.3%). Ethmoid sinus (67.8%) and nasal cavity (45.4%) were the most common anatomical site of tumor origin, while an extension to the orbit and skull base was registered in 28.7% and 24.5% of cases. Surgery was the main treatment, especially in the form of endoscopic endonasal approach (56.9%), and allowed for gross total resection in 79% of cases. The recurrence rate was 26.2%; three cases of tumor-related death were reported. Median follow-up was 4.6 years. Conclusions: Biphenotypic sinonasal sarcoma is a rare and unique tumoral entity in terms of biological and clinical behavior. Based on the current knowledge, surgery plays the leading role in treatment, accounting for gross total tumor resection in most cases, allowing for clinical symptom and sign resolution and presenting a low rate of perioperative complications. The type of approach and the aim of surgery should be assessed case by case according to patient and pathology features and the surgeon’s experience, as well as the aim of the treatment. Further studies including large surgical series and with long follow-up are required to define prognostic factors and guidelines of treatment for this peculiar pathological entity.

1. Introduction

Biphenotypic sinonasal sarcoma (BSNS), first described in 2012 by Lewis et al. [1] as a “low-grade sinonasal sarcoma with neural and myogenic differentiation” and introduced in the fourth WHO classification of head and neck tumors in 2017 [2], is a rare low-grade tumor of the sinonasal tract [3], with its origin in the paranasal sinuses or nasal cavity, with a tendency to invade the orbit and/or skull base.
The “biphenotypic” aspect of the tumor is due to the coexistence of both neural and myogenic markers in immunophenotypic studies. Indeed, the tumor exhibits immunoreactivity for both S100 (neural marker) and smooth muscle markers (smooth muscle actin (SMA), muscle specific actin (MSA) or calponin); nevertheless, the distribution of the intensity and the extent of staining are variable. Several sinonasal diseases, such as schwannoma, fibrosarcoma, leiomyosarcoma, malignant peripheral nerve sheath tumor, solitary fibrosus tumor, and fibromatosis, can mimic the BSNS from a histopathological point of view. Thus, a proper diagnosis requires immunophenotyping and immunofluorescence studies [4].
Due to the rarity of this pathological entity and its recent histopathological and molecular characterization, studies including a long follow-up and large surgical series are lacking; thus, nowadays, it is difficult to identify the main prognostic factors, as well as to provide well-defined guidelines of treatment and surveillance protocol. Therefore, the aim of the present study was to retrospectively analyze, from a detailed and comprehensive literature review on BSNS, the demographic, clinical, radiological and pathological features, as well as treatment and outcome, of this rare tumor entity.

2. Methods

A Medline search up from January 2012 to May 2024 in the Embase online electronic database was conducted in accordance with the Preferred Reporting Items for Systematic Reviews and Meta-Analysis (PRISMA) guidelines [5], by using the following key phrases: “biphenotypic sinonasal sarcoma” OR “low-grade sinonasal sarcoma”, “biphenotypic sarcoma”, “frontal sinus”, “ethmoid sinus”, “maxillary sinus”, “orbit”. They were combined as follows: (“biphenotypic sinonasal sarcoma” AND “orbit”), (“frontal sinus” AND “biphenotypic sarcoma”), (“ethmoid sinus” AND “biphenotypic sarcoma”), (“maxillary sinus” AND “biphenotypic sarcoma”) (“frontal sinus” AND “ethmoid sinus” AND “maxillary sinus” AND “biphenotypic sarcoma”), (“frontal sinus” AND “ethmoid sinus” AND “orbit” AND “biphenotypic sarcoma”). After duplicate removal, all abstracts were evaluated, and each article of interest was marked for further review. The full text of the marked studies was independently screened by two authors (S.C. and G.C.) and included in this systematic review following the inclusion and exclusion criteria, as summarized in Figure 1.
The inclusion criteria were surgical series, reviews, and case reports in the English language concerning BSNS cases with immunohistochemical diagnosis confirmed or not by molecular exams, and studies reporting clinical and surgical data. Demographic (sex and age), clinical (presenting symptoms), radiological, (anatomical origin, skull base and orbital involvement), pathological (immunohistochemical and molecular diagnosis), treatment (time to treatment, type of treatment, surgical approach, extent of resection, complications) and outcome (clinical, recurrence, overall survival) data were analyzed.

Statistical Analysis

The normality Shapiro–Wilk test was adopted for categorical and qualitative analyses, p values lower than 0.05 were considered statistically significant.

3. Results

A comprehensive systematic literature review disclosed 114 studies. After duplicate removal, and screening of the full texts of the marked studies included according to the inclusion criteria, 34 studies were eligible for this literature review [1,6,7,8,9,10,11,12,13,14,15,16,17,18,19,20,21,22,23,24,25,26,27,28,29,30,31,32,33,34,35,36,37], most of which (n = 22/34, 64.7%) included only one case or a series of fewer than five cases. The final entire sample included 149 patients, whose data are separately reported in Table 1 and Table 2 and summarized in Table 3 and Table 4.

3.1. Demographic, Clinical, Neuroradiological and Pathological Data (Table 1 and Table 3, Figure 2)

The overall sample included 99 (66.9%) females and 49 males (33.1%), with a median age of 54.88 years (range 22–79 y.o.). Presenting symptoms were reported in 56.3% of cases and were mainly represented by nasal obstruction (n = 68/84, 81%), followed by facial discomfort (n = 37/84, 44%)—including facial pain and/or pressure—epistaxis (n = 13/84, 15.5%) and ocular impairment (n = 12/84, 14.3%)—including diplopia, epiphora, and gaze restriction.
Data on the site of origin of the lesion and its pattern of growth were reported in 96% of cases (n = 143/149). The most frequent site of origin was the ethmoid sinus (n = 97/143, 67.8%), followed by the nasal cavity (n = 65/143, 45.4%), frontal sinus (n = 34/143, 23.7%), maxillary sinus (n = 18/143, 12.6%) and sphenoid sinus (n = 5/143, 3.5%). From the site of origin, the lesion extended to the skull base (mainly anterior cranial fossa) in 24.5% of the cases (n = 35/143), whereas the orbital invasion was reported in 28.7% of the cases (n = 41/143), and mainly occurred through the lamina papyracea.
The diagnosis of biphenotypic sinonasal sarcoma was achieved through an immunohistochemical study in 37.6% of cases (n = 56/149), and with the integration of biomolecular examination in the remaining 62.4% (n = 93/149).

3.2. Treatment and Outcome Data (Table 2 and Table 4, Figure 2)

The time lap from clinical symptoms and/or signs onset to treatment is reported just in 11 out of 149 patients (7.3%) and it is 12 months (mean time).
In 104 cases (69.8%), the type of treatment adopted was described. Surgery was performed in all but five cases, where only a biopsy (4.8%) was carried out, and two cases (1.9%) where only the combination of radio- and chemotherapy was administered. In detail, surgical procedure was adopted as a unique treatment in 69 patients (66.3%), while it was followed by adjuvant radiotherapy in 20 cases (19.2%), by chemotherapy in 3 (2.9%), and associated with both radio- and chemotherapy in 5 (4.8%).
The description of the type of surgical approach selected was reported in 58 out of 149 cases (39%). The most adopted surgical option was the endoscopic endonasal route (n = 33/58, 56.9%), followed by the combined microsurgical transcranial–endoscopic endonasal approach (n = 17/58, 29.3%) and isolated microsurgical transcranial approach (n = 7/58, 12%). A transorbital approach was chosen in only one case (1.7%).
The extent of tumor resection was reported in 62 cases of the overall series (41.6%). It was gross total (GTR) in 49 (79%) and sub-total (STR) in the remaining 13 (21%).
Data concerning peri-operative complications were reported in only twelve cases (8%): they occurred in four patients (33.3%) and mainly consisted of transient CSF leak.
In regard to the outcome data, post-treatment clinical conditions were reported just in five patients, registering an improvement in all but one of them, where they remained stable.
Data on the recurrence rate were reported in 84 out of 149 patients (56.3%) during a follow-up from 1 to 9 years. Among them, local recurrence was observed in 22 cases (26.2%), including five patients who experienced more than one recurrence over their lifetime.
The status of 85 out of 149 patients of the overall series (57%) was reported at last follow-up (mean 4.6 years): seventy-seven of them (90.6%) were alive and eight died. Three died due to tumor persistence/progression, one due to surgical complications and four from other causes.

4. Discussion

BSNS exhibits unique characteristics, differing histologically from malignant sarcomas or other sinonasal cancers, harboring biphenotypic markers’ expression and a peculiar identity combining clinical, morphological, histological and genetic features [30].
Several neoplastic diseases and with a different grade of malignancy can affect the sinonasal region [38]; among them, biphenotypic sarcoma, albeit exceptional for incidence (1–5% of head and neck malignancies [13]), with only 149 cases identified in the present literature review, should be considered in the differential diagnosis. This lesion mainly affects female (ratio F:M = 2:1) and middle-aged populations; nevertheless, no reactivity was reported for sexual tumor markers such as estrogen and progesterone receptors.
As the tumor arises from the mucosa of the upper airway (nasal cavity, 45.4%) and/or air-filled cavities like paranasal sinuses (ethmoid, frontal, maxillary or sphenoid sinus, in 67.8%, 23.7%, 12.6% and 3.5%, respectively), the clinical onset is mainly represented by nasal obstruction (81%), followed by facial discomfort (44%), which are not specific clinical symptoms and very commonly shared with several different diseases, thus requiring a wide differential diagnosis often being underestimated.
BSNS presents some peculiar intrinsic features with intermediate biological behavior between malignant and benign tumors:
  • Slow growth: mean time from clinical onset to treatment is 12 months (even if cases up to 3 years are described (Table 2)); nevertheless, considering the small sizes and the function of the common sites of origin of the tumor—such as nasal cavity and paranasal sinuses—as well as of the adjacent structures usually involved—such as the orbit—it is easy to understand that a tumor becomes symptomatic quite early;
  • Local aggressiveness: the tumor invades and destructs adjacent structures, both bony and soft tissues, including the medial wall, floor and roof of the orbit, cribriform plate, and orbital fat; therefore, a prompt and proper diagnosis and treatment are mandatory to prevent neuro-ophthalmological complications, such as CSF leak, meningitis, meningocele, seizures, pneumocephalus, anosmia, proptosis, and diplopia;
  • Infiltrative pattern of growth: this makes it hard to achieve clear margins after surgical excision despite the high rates of gross total resection and low rate of peri- and post-operative complications;
  • Long time to and very low frequency of malignant transformation;
  • Tendency to locally recur: Recurrence was observed in patients regardless of the extent of tumor resection and the administration of adjuvant radiotherapy; there is no significant evidence to support the need for concomitant radiotherapy or surgical excision alone. Post-operative RT is mainly adopted when the examination of the surgical margins is found to be positive or inconclusive [6]. Therefore, it is important to collect further studies with large case series and long follow-up to analyze the main risk factors for recurrence.
  • No distant metastasis.
BSNS is a primarily local aggressive disease, with the involvement of highly functional anatomical structures such as the upper respiratory tract, the orbit and the skull base; therefore, surgical resection represents the gold standard of treatment.
Nevertheless, due to the rarity of the pathology, well-defined guidelines of treatment, as well as a surveillance protocol, are missing, and management varies among different institutions, with most centers proposing the surgical procedure as the best option, both as a unique treatment and combined with radio- or chemotherapy, or radio- and chemotherapy.
In regard to the surgical strategy, including the goal of surgery and the approach selection, several factors, both related to the patient and pathology, must be considered.
In the presence of young patients with good clinical conditions and long expectancy of life, we consider that the maximal safe tumor resection should be attempted. Conversely, in the presence of elderly patients, with not a long expectancy of life, unnecessary overtreatment should be avoided and other primary goals should be pursued, including (1) subtotal resection through tumor debulking to ensure the patency of the airways and drainage of the affected paranasal sinuses; (2) resolution of the mass effect on the adjacent structures to stop and/or prevent a further worsening of neuro-ophthalmological deficits; and (3) preventing associated intracranial complications, like mucocele, CSF leak, pneumocephalus, meningitis, seizure, brain abscess, and subdural empyema. The extended endoscopic endonasal approach (EEEA) plays the leading role among surgical procedures for addressing pathologies of the ventral midline skull base [39,40,41,42,43,44]; in fact, it represents the most adopted surgical option for BSNS, isolated or in a combined manner with the transcranial approach. As an alternative, a microsurgical transcranial approach (TCA) is reserved for cases with large intracranial extension due to skull base invasion and it not being suitable for the EEEA. Finally, the transorbital approach (TOA) was selected just once. This option can be adopted in a combined bi-portal approach with the EEEA for BSNS with lateral extension to the paramedian and lateral aspects of anterior cranial fossa and orbital cavity [37]. Particularly, the endoscopic transorbital approach, initially mainly adopted by ophthalmologists for the management of intraorbital pathologies, owing to its peculiar advantages, over the last fifteen years, has become very popular among neurosurgeons for addressing lesions involving the paramedian regions of the anterior and middle skull base [45,46,47,48,49,50,51,52,53,54,55,56,57], and with spheno-orbital meningiomas, representing the optimal indication in carefully selected cases [58,59,60,61]. The combined endoscopic endonasal and transorbital approach for frontal sinus lesions is widely demonstrated as safe and effective [62,63,64]. In a recent paper, our group proposed a modular system of approach selection, considering the endoscopic endonasal route as the master approach and the endoscopic transorbital and open transcranial as complementary routes, which can be variously combined based on the tumor origin and pattern of growth [37]. In detail, the tumor component involving the midline structures, like the nasal cavity, ethmoid and frontal sinuses, can be addressed through the endoscopic endonasal approach; the tumor extension into the superolateral compartment of the orbit and/or the far lateral end of the frontal sinus can be approached through the endoscopic transorbital route; finally, for large intracranial tumor extension or involvement of the lateral end of frontal sinus bilaterally, a transcranial approach can be considered.
Despite the multicompartmental and locally invasive pattern of growth of BSNS, gross total tumor resection is achieved in 79% of cases, while a sub-total resection, including biopsies, is achieved in the remaining 21%. This accounts for the resolution of clinical symptoms and signs related to the mass-effect, which occurs in 80% of the cases.
Peri-operative complications are reported in four cases and mainly consist of transient cerebrospinal fluid leakage. This finding is understandable considering the aggressive and destructive nature of the lesion on the anterior skull base, especially on the cribriform plate, and the prevalent selection of the endoscopic endonasal route as a surgical approach, whose main complication is CSF leak [65,66,67,68]. Nevertheless, it should be considered that the continued refinements in skull base reconstruction techniques after endoscopic endonasal surgery [65,69,70] accounted for a decrease in the CSF leak rate under 5%.
At last follow-up, most patients were alive with no evidence of disease; among the eight cases of death reported, four (50%) were due to other causes, one was related to the surgical procedure and three we due to to tumor persistence/progression. Three cases of histologically tumor progression from low-grade to high-grade sarcoma were reported [20,21,25], of whom one died due to tumor progression, one died due to heart failure and one is alive.

Limitations and Advantages of This Study

Its retrospective nature represents the first limitation of this study. In addition, the small size of the sample of patients included and the heterogeneity of the data represent other limitations of this study. Much of the data are incomplete, as such as those related to time to treatment, peri- and post-operative complications, clinical outcome, overall survival rate, and follow-up. Nevertheless, the present review is comprehensive and analyzes the main factors affecting the course of this rare disease, providing a significant contribution to better understand the natural history of BSNS and the impact of the different strategies of treatment on the outcome.

5. Conclusions

Biphenotypic sinonasal sarcoma is a rare and unique tumor entity in terms of biological and clinical behavior. Based on the current knowledge, surgery plays the leading role in treatment, accounting for gross total tumor resection in most cases, with clinical symptom and sign resolution and a low rate of peri-operative complications. The type of approach and the aim of surgery should be assessed case by case according to patient and pathology features. The role of adjuvant therapies is still unclear.
Further studies including large surgical series and those with long follow-up are required to define prognostic factors and guidelines of treatment for this peculiar pathological entity.

Author Contributions

S.C.: Conceptualization, Methodology, Investigation, Data Curation, Writing—Original Draft; Validation; G.C., R.A. and G.P.: Data Curation; G.M., A.I., D.S., M.B., A.E. and O.d.D.: Study Supervision, Validation. All authors have read and agreed to the published version of the manuscript.

Funding

The authors declare that no funding was received for this work.

Institutional Review Board Statement

Not applicable.

Informed Consent Statement

Not applicable.

Data Availability Statement

Data of the current original research are available from the corresponding author on reasonable request.

Conflicts of Interest

The authors declare no conflicts of interest.

References

  1. Lewis, J.T.; Oliveira, A.M.; Nascimento, A.G.; Schembri-Wismayer, D.; Moore, E.A.; Olsen, K.D.; Garcia, J.G.; Lonzo, M.L.; Lewis, J.E. Low-grade sinonasal sarcoma with neural and myogenic features: A clinicopathologic analysis of 28 cases. Am. J. Surg. Pathol. 2012, 36, 517–525. [Google Scholar] [CrossRef]
  2. Stelow, E.B.; Bishop, J.A. Update from the 4th Edition of the World Health Organization Classification of Head and Neck Tumours: Tumors of the Nasal Cavity, Paranasal Sinuses and Skull Base. Head. Neck Pathol. 2017, 11, 3–15. [Google Scholar] [CrossRef] [PubMed]
  3. Carter, C.S.; East, E.G.; McHugh, J.B. Biphenotypic Sinonasal Sarcoma: A Review and Update. Arch. Pathol. Lab. Med. 2018, 142, 1196–1201. [Google Scholar] [CrossRef] [PubMed]
  4. Triki, M.; Ayadi, L. Low-Grade Sinonasal Sarcoma with Neural and Myogenic Features: A Recently Discovered Entity with Unique Features and Diagnostic Challenge. Arch. Pathol. Lab. Med. 2017, 141, 718–721. [Google Scholar] [CrossRef] [PubMed]
  5. Page, M.J.; McKenzie, J.E.; Bossuyt, P.M.; Boutron, I.; Hoffmann, T.C.; Mulrow, C.D.; Shamseer, L.; Tetzlaff, J.M.; Akl, E.A.; Brennan, S.E.; et al. The PRISMA 2020 statement: An updated guideline for reporting systematic reviews. BMJ 2021, 372, n71. [Google Scholar] [CrossRef] [PubMed]
  6. Chitguppi, C.; Koszewski, I.; Collura, K.; Curtis, M.; Nyquist, G.; Rabinowitz, M.; Rosen, M. Biphenotypic Sinonasal Sarcoma-Case Report and Review of Clinicopathological Features and Diagnostic Modalities. J. Neurol. Surg. B Skull Base 2019, 80, 51–58. [Google Scholar] [CrossRef]
  7. Powers, K.A.; Han, L.M.; Chiu, A.G.; Aly, F.Z. Low-grade sinonasal sarcoma with neural and myogenic features—Diagnostic challenge and pathogenic insight. Oral. Surg. Oral. Med. Oral. Pathol. Oral. Radiol. 2015, 119, e265–e269. [Google Scholar] [CrossRef] [PubMed]
  8. Rooper, L.M.; Huang, S.C.; Antonescu, C.R.; Westra, W.H.; Bishop, J.A. Biphenotypic sinonasal sarcoma: An expanded immunoprofile including consistent nuclear β-catenin positivity and absence of SOX10 expression. Hum. Pathol. 2016, 55, 44–50. [Google Scholar] [CrossRef]
  9. Wong, W.J.; Lauria, A.; Hornick, J.L.; Xiao, S.; Fletcher, J.A.; Marino-Enriquez, A. Alternate PAX3-FOXO1 oncogenic fusion in biphenotypic sinonasal sarcoma. Genes. Chromosomes Cancer 2016, 55, 25–29. [Google Scholar] [CrossRef]
  10. Huang, S.C.; Ghossein, R.A.; Bishop, J.A.; Zhang, L.; Chen, T.C.; Huang, H.Y.; Antonescu, C.R. Novel PAX3-NCOA1 Fusions in Biphenotypic Sinonasal Sarcoma With Focal Rhabdomyoblastic Differentiation. Am. J. Surg. Pathol. 2016, 40, 51–59. [Google Scholar] [CrossRef]
  11. Cannon, R.B.; Wiggins, R.H.; Witt, B.L.; Dundar, Y.; Johnston, T.M.; Hunt, J.P. Imaging and Outcomes for a New Entity: Low-Grade Sinonasal Sarcoma with Neural and Myogenic Features. J. Neurol. Surg. Rep. 2017, 78, e15–e19. [Google Scholar] [CrossRef]
  12. Lin, Y.; Liao, B.; Han, A. Biphenotypic sinonasal sarcoma with diffuse infiltration and intracranial extension: A case report. Int. J. Clin. Exp. Pathol. 2017, 10, 11743–11746. [Google Scholar] [PubMed]
  13. Andreasen, S.; Bishop, J.A.; Hellquist, H.; Hunt, J.; Kiss, K.; Rinaldo, A.; Skálová, A.; Willems, S.M.; Williams, M.; Ferlito, A. Biphenotypic sinonasal sarcoma: Demographics, clinicopathological characteristics, molecular features, and prognosis of a recently described entity. Virchows Arch. 2018, 473, 615–626. [Google Scholar] [CrossRef] [PubMed]
  14. Kakkar, A.; Rajeshwari, M.; Sakthivel, P.; Sharma, M.C.; Sharma, S.C. Biphenotypic sinonasal sarcoma: A series of six cases with evaluation of role of β-catenin immunohistochemistry in differential diagnosis. Ann. Diagn. Pathol. 2018, 33, 6–10. [Google Scholar] [CrossRef]
  15. Fudaba, H.; Momii, Y.; Hirano, T.; Yamamoto, H.; Fujiki, M. Recurrence of Biphenotypic Sinonasal Sarcoma With Cerebral Hemorrhaging. J. Craniofac Surg. 2019, 30, e1–e2. [Google Scholar] [CrossRef] [PubMed]
  16. Alkhudher, S.M.; Al Zamel, H.; Bhat, I.N. A rare case of nasal biphenotypic sino-nasal sarcoma in a young female. Ann. Med. Surg. 2019, 37, 4–6. [Google Scholar] [CrossRef]
  17. Le Loarer, F.; Laffont, S.; Lesluyes, T.; Tirode, F.; Antonescu, C.; Baglin, A.C.; Delespaul, L.; Soubeyran, I.; Hostein, I.; Pérot, G.; et al. Clinicopathologic and Molecular Features of a Series of 41 Biphenotypic Sinonasal Sarcomas Expanding Their Molecular Spectrum. Am. J. Surg. Pathol. 2019, 43, 747–754. [Google Scholar] [CrossRef]
  18. Sethi, S.; Cody, B.; Farhat, N.A.; Pool, M.D.; Katabi, N. Biphenotypic sinonasal sarcoma: Report of 3 cases with a review of literature. Hum. Pathol. 2021, 24, 200491. [Google Scholar] [CrossRef]
  19. Hanbazazh, M.; Jakobiec, F.A.; Curtin, H.D.; Lefebvre, D.R. Orbital Involvement by Biphenotypic Sinonasal Sarcoma with a Literature Review. Ophthalmic Plast. Reconstr. Surg. 2021, 37, 305–311. [Google Scholar] [CrossRef] [PubMed]
  20. Bell, D.; Phan, J.; DeMonte, F.; Hanna, E.Y. High-grade transformation of low-grade biphenotypic sinonasal sarcoma: Radiological, morphophenotypic variation and confirmatory molecular analysis. Ann. Diagn. Pathol. 2022, 57, 151889. [Google Scholar] [CrossRef] [PubMed]
  21. Hasnie, S.; Glenn, C.; Peterson, J.E.G.; El Rassi, E.T.; McKinney, K.A. High-Grade Biphenotypic Sinonasal Sarcoma: A Case Report. J. Neurol. Surg. Rep. 2022, 83, e105–e109. [Google Scholar] [CrossRef] [PubMed]
  22. Turri-Zanoni, M.; Dalfino, G.; Lechner, M.; Dallan, I.; Battaglia, P.; Facco, C.; Franzi, F.; Gravante, G.; Ferrari, M.; Terzakis, D.; et al. Biphenotypic sinonasal sarcoma: European multicentre case-series and systematic literature review. Acta Otorhinolaryngol. Ital. 2022, 42, 545–553. [Google Scholar] [CrossRef] [PubMed]
  23. Nichols, M.M.; Alruwaii, F.; Chaaban, M.; Cheng, Y.W.; Griffith, C.C. Biphenotypic Sinonasal Sarcoma with a Novel PAX3::FOXO6 Fusion: A Case Report and Review of the Literature. Head. Neck Pathol. 2023, 17, 259–264. [Google Scholar] [CrossRef]
  24. Ingle, A.; Mahendra, N.; Gopal Reddy, G.V. Biphenotypic sinonasal sarcoma-A recently described entity with many mimics: A case report. Indian. J. Pathol. Microbiol. 2023, 66, 396–399. [Google Scholar] [CrossRef] [PubMed]
  25. Meyer, A.; Klubíčková, N.; Mosaieby, E.; Grossmann, P.; Kalmykova, A.; Koshyk, O.; Michal, M. Biphenotypic sinonasal sarcoma with PAX3::MAML3 fusion transforming into high-grade rhabdomyosarcoma: Report of an emerging rare phenomenon. Virchows Arch. 2023, 482, 777–782. [Google Scholar] [CrossRef] [PubMed]
  26. Kominsky, E.; Boyke, A.E.; Madani, D.; Kamat, A.; Schiff, B.A.; Agarwal, V. Biphenotypic Sinonasal Sarcoma: A Case Report and Review of Literature. Ear Nose Throat J. 2023, 102, 385–390. [Google Scholar] [CrossRef] [PubMed]
  27. Bhele, S.; Chrisinger, J.S.A.; Farrell, N.F.; Van Tine, B.A.; Raptis, C.A.; Chernock, R.D. Biphenotypic Sinonasal Sarcoma with a Novel PAX7::PPARGC1 Fusion: Expanding the Spectrum of Gene Fusions Beyond the PAX3 Gene. Head. Neck Pathol. 2023, 17, 826–831. [Google Scholar] [CrossRef] [PubMed]
  28. Viramontes, A.; Mueller, N.; Gocke, C.D.; Deklotz, T.R.; Ozdemirli, M. Novel PAX3::INO80D Fusion in Biphenotypic Sinonasal Sarcoma in an Adult. JAMA Otolaryngol. Head. Neck Surg. 2023, 149, 849–850. [Google Scholar] [CrossRef]
  29. Muraoka, E.; Kato, I.; Matsumura, M.; Arai, Y.; Suenaga, J.; Yamanaka, S.; Fujii, S. Biphenotypic Sinonasal Sarcoma: A Genetically Confirmed Case Showing Bone Invasion Accompanying a Non-neoplastic Respiratory Epithelium. Int. J. Surg. Pathol. 2023, 31, 1414–1419. [Google Scholar] [CrossRef]
  30. Anastasiadou, S.; Karkos, P.; Constantinidis, J. Biphenotypic Sinonasal Sarcoma with Orbital and Skull Base Involvement Report of 3 Cases and Systematic Review of the Literature. Indian. J. Otolaryngol. Head. Neck Surg. 2023, 75, 3353–3363. [Google Scholar] [CrossRef]
  31. Okuda, H.; Kuze, B.; Shibata, H.; Hayashi, H.; Nishihori, T.; Mizuta, K.; Kohyama, K.; Yasue, Y.; Kato, H.; Aoki, M. Biphenotypic siononasal sarcoma with acute exacerbation: A case report. Otolaryngol. Case Rep. 2020, 16, 100190. [Google Scholar]
  32. Okafor, S.; Halderman, A.; Bishop, J.; Ryan, M.; Marple, B. Biphenotypic Sinonasal Sarcoma a Newly Recognized Sinonasal Neoplasm: Case Report and Review of the Literature. J. Neurol. Surg. Part B Skull Base 2020, 81, S1–S272. [Google Scholar]
  33. Kühn, A.; Jalisi, S.; Nishino, M.; Ivanovic, V. Biphenotypic sinonasal sarcoma—Description of radiologic, intraoperative and pathologic findings. Otolaryngol. Case Rep. 2019, 11, 100113. [Google Scholar] [CrossRef]
  34. Miglani, A.; Lal, D.; Weindling, S.M.; Wood, C.P.; Hoxworth, J.M. Imaging characteristics and clinical outcomes of biphenotypic sinonasal sarcoma. Laryngoscope Investig. Otolaryngol. 2019, 4, 484–488. [Google Scholar] [CrossRef]
  35. Koszewski, I.J.; Garcia, H.G.; Rabinowitz, M.R.; Nyquist, G.G.; Evans, J.J.; Rosen, M.R. Biphenotypic Sinonasal Sarcoma with Focal Rhabdomyoblastic Differentiation: Case Report of a Newly Described Malignancy with a Review of the Literature. J. Neurol. Surg. Part B Skull Base 2018, 79, S1–S188. [Google Scholar] [CrossRef]
  36. Hockstein, N.G.; Dross, P.E.; Farooqui, S.; Wilhelm, I.N. Low-grade sinonasal sarcoma with neural and myogenic features. Ear Nose Throat J. 2018, 97, 149–150. [Google Scholar] [CrossRef] [PubMed]
  37. Corvino, S.; de Divitiis, O.; Iuliano, A.; Russo, F.; Corazzelli, G.; Cohen, D.; Di Crescenzo, R.M.; Palmiero, C.; Pontillo, G.; Staibano, S.; et al. Biphenotypic Sinonasal Sarcoma with Orbital Invasion: A Literature Review and Modular System of Surgical Approaches. Cancers 2024, 16, 3316. [Google Scholar] [CrossRef] [PubMed]
  38. Agarwal, M.; Policeni, B. Sinonasal Neoplasms. Semin. Roentgenol. 2019, 54, 244–257. [Google Scholar] [CrossRef]
  39. Kassam, A.; Snyderman, C.H.; Mintz, A.; Gardner, P.; Carrau, R.L. Expanded endonasal approach: The rostrocaudal axis. Part II. Posterior clinoids to the foramen magnum. Neurosurg. Focus. 2005, 19, E4. [Google Scholar] [CrossRef]
  40. Kassam, A.; Snyderman, C.H.; Mintz, A.; Gardner, P.; Carrau, R.L. Expanded endonasal approach: The rostrocaudal axis. Part I. Crista galli to the sella turcica. Neurosurg. Focus 2005, 19, E3. [Google Scholar] [CrossRef]
  41. Özer, M.; Kutlay, A.M.; Durmaz, M.O.; Kirik, A.; Yaşar, S.; Tehli, Ö.; Kural, C.; Temiz, N.; Durmaz, A.; Ezgu, M.C.; et al. Extended endonasal endoscopic approach for anterior midline skull base lesions. Clin. Neurol. Neurosurg. 2020, 196, 106024. [Google Scholar] [CrossRef]
  42. Gellner, V.; Tomazic, P.V. Limits of the endoscopic transnasal transtubercular approach. J. Neurosurg. Sci. 2018, 62, 297–300. [Google Scholar] [CrossRef]
  43. Ceylan, S.; Koc, K.; Anik, I. Extended endoscopic approaches for midline skull-base lesions. Neurosurg. Rev. 2009, 32, 309–319, discussion 318–309. [Google Scholar] [CrossRef]
  44. Ceylan, S.; Anik, I.; Koc, K.; Cabuk, B. Extended endoscopic transsphenoidal approach infrachiasmatic corridor. Neurosurg. Rev. 2015, 38, 137–147, discussion 147. [Google Scholar] [CrossRef] [PubMed]
  45. Moe, K.S.; Bergeron, C.M.; Ellenbogen, R.G. Transorbital neuroendoscopic surgery. Neurosurgery 2010, 67, ons16–ons28. [Google Scholar] [CrossRef] [PubMed]
  46. Di Somma, A.; Kong, D.S.; de Notaris, M.; Moe, K.S.; Sánchez España, J.C.; Schwartz, T.H.; Enseñat, J. Endoscopic transorbital surgery levels of difficulty. J. Neurosurg. 2022, 137, 1187–1190. [Google Scholar] [CrossRef]
  47. de Notaris, M.; Kong, D.S.; Di Somma, A.; Enseñat, J.; Hong, C.K.; Moe, K.; Schwartz, T.H. Superior eyelid transorbital approaches: A modular classification system. J. Neurosurg. 2024, 1, 1–6. [Google Scholar] [CrossRef]
  48. Corvino, S.; Sacco, M.; Somma, T.; Berardinelli, J.; Ugga, L.; Colamaria, A.; Corrivetti, F.; Iaconetta, G.; Kong, D.-S.; de Notaris, M. Functional and clinical outcomes after superior eyelid transorbital endoscopic approach for spheno-orbital meningiomas: Illustrative case and literature review. Neurosurg. Rev. 2023, 46, 1–12. [Google Scholar] [CrossRef] [PubMed]
  49. Corvino, S.; Armocida, D.; Offi, M.; Pennisi, G.; Burattini, B.; Mondragon, A.V.; Esposito, F.; Cavallo, L.M.; de Notaris, M. The anterolateral triangle as window on the foramen lacerum from transorbital corridor: Anatomical study and technical nuances. Acta Neurochir. 2023, 165, 2407–2419. [Google Scholar] [CrossRef]
  50. Corvino, S.; Guizzardi, G.; Sacco, M.; Corrivetti, F.; Bove, I.; Enseñat, J.; Colamaria, A.; Prats-Galino, A.; Solari, D.; Cavallo, L.M.; et al. The feasibility of three port endonasal, transorbital, and sublabial approach to the petroclival region: Neurosurgical audit and multiportal anatomic quantitative investigation. Acta Neurochir. 2023, 165, 1–11. [Google Scholar] [CrossRef]
  51. Corvino, S.; Villanueva-Solórzano, P.; Offi, M.; Armocida, D.; Nonaka, M.; Iaconetta, G.; Esposito, F.; Cavallo, L.; de Notaris, M. A New Perspective on the Cavernous Sinus as Seen through Multiple Surgical Corridors: Anatomical Study Comparing the Transorbital, Endonasal, and Transcranial Routes and the Relative Coterminous Spatial Regions. Brain Sci. 2023, 13, 1215. [Google Scholar] [CrossRef]
  52. de Notaris, M.; Sacco, M.; Corrivetti, F.; Grasso, M.; Corvino, S.; Piazza, A.; Kong, D.S.; Iaconetta, G. The Transorbital Approach, A Game-Changer in Neurosurgery: A Guide to Safe and Reliable Surgery Based on Anatomical Principles. J. Clin. Med. 2023, 12, 6484. [Google Scholar] [CrossRef] [PubMed]
  53. Corvino, S.; Kassam, A.; Piazza, A.; Corrivetti, F.; Spiriev, T.; Colamaria, A.; Cirrottola, G.; Cavaliere, C.; Esposito, F.; Cavallo, L.M.; et al. Open-door extended endoscopic transorbital technique to the paramedian anterior and middle cranial fossae: Technical notes, anatomomorphometric quantitative analysis, and illustrative case. Neurosurg. Focus 2024, 56, E7. [Google Scholar] [CrossRef] [PubMed]
  54. Vural, A.; Carobbio, A.L.C.; Ferrari, M.; Rampinelli, V.; Schreiber, A.; Mattavelli, D.; Doglietto, F.; Buffoli, B.; Rodella, L.F.; Taboni, S.; et al. Transorbital endoscopic approaches to the skull base: A systematic literature review and anatomical description. Neurosurg. Rev. 2021, 44, 2857–2878. [Google Scholar] [CrossRef] [PubMed]
  55. Paluzzi, A.; Gardner, P.A.; Fernandez-Miranda, J.C.; Tormenti, M.J.; Stefko, S.T.; Snyderman, C.H.; Maroon, J.C. "Round-the-Clock" Surgical Access to the Orbit. J. Neurol. Surg. B Skull Base 2015, 76, 12–24. [Google Scholar] [CrossRef] [PubMed]
  56. Corvino, S.; de Notaris, M.; Sommer, D.; Kassam, A.; Kong, D.S.; Piazza, A.; Corrivetti, F.; Cavallo, L.M.; Iaconetta, G.; Reddy, K. Assessing the Feasibility of Selective Piezoelectric Osteotomy in Transorbital Approach to the Middle Cranial Fossa: Anatomical and Quantitative Study and Surgical Implications. World Neurosurg. 2024, in press. [Google Scholar] [CrossRef] [PubMed]
  57. Corvino, S.; Kassam, A.; Piazza, A.; Corrivetti, F.; Esposito, F.; Iaconetta, G.; de Notaris, M. Navigating the Intersection Between the Orbit and the Skull Base: The “Mirror” McCarty Keyhole During Transorbital Approach: An Anatomic Study with Surgical Implications. Oper. Neurosurg. 2024. [Google Scholar] [CrossRef]
  58. Mariniello, G.; Corvino, S.; Iuliano, A.; Maiuri, F. Spheno-orbital Meningiomas. In Cranio-Orbital Mass Lesions; Bonavolontà, G., Maiuri, F., Mariniello, G., Eds.; Springer: Cham, Switzerland, 2023. [Google Scholar]
  59. Kong, D.S.; Kim, Y.H.; Hong, C.K. Optimal indications and limitations of endoscopic transorbital superior eyelid surgery for spheno-orbital meningiomas. J. Neurosurg. 2020, 134, 1472–1479. [Google Scholar] [CrossRef]
  60. Mariniello, G.; Corvino, S.; Corazzelli, G.; de Divitiis, O.; Fusco, G.; Iuliano, A.; Strianese, D.; Briganti, F.; Elefante, A. Spheno-Orbital Meningiomas: The Rationale behind the Decision-Making Process of Treatment Strategy. Cancers 2024, 16, 2148. [Google Scholar] [CrossRef]
  61. Mariniello, G.; de Divitiis, O.; Corvino, S.; Strianese, D.; Iuliano, A.; Bonavolontà, G.; Maiuri, F. Recurrences of spheno-orbital meningiomas: Risk factors and management. World Neurosurg. 2022, 161, e514–e522. [Google Scholar] [CrossRef]
  62. Makary, C.A.; Limjuco, A.; Nguyen, J.; Ramadan, H.H. Combined Lid Crease and Endoscopic Approach to Lateral Frontal Sinus Disease With Orbital Extension. Ann. Otol. Rhinol. Laryngol. 2018, 127, 637–642. [Google Scholar] [CrossRef]
  63. Miller, C.; Berens, A.; Patel, S.A.; Humphreys, I.M.; Moe, K.S. Transorbital Approach for Improved Access in the Management of Paranasal Sinus Mucoceles. J. Neurol. Surg. B Skull Base 2019, 80, 593–598. [Google Scholar] [CrossRef]
  64. Arosio, A.D.; Coden, E.; Valentini, M.; Czaczkes, C.; Battaglia, P.; Bignami, M.; Castelnuovo, P.; Karligkiotis, A. Combined Endonasal-Transorbital Approach to Manage the Far Lateral Frontal Sinus: Surgical Technique. World Neurosurg. 2021, 151, 5. [Google Scholar] [CrossRef] [PubMed]
  65. Hardesty, D.A.; Montaser, A.; Kreatsoulas, D.; Shah, V.S.; VanKoevering, K.K.; Otto, B.A.; Carrau, R.L.; Prevedello, D.M. Complications after 1002 endoscopic endonasal approach procedures at a single center: Lessons learned, 2010–2018. J. Neurosurg. 2022, 136, 393–404. [Google Scholar] [CrossRef]
  66. Kasemsiri, P.; Carrau, R.L.; Ditzel Filho, L.F.; Prevedello, D.M.; Otto, B.A.; Old, M.; de Lara, D.; Kassam, A.B. Advantages and limitations of endoscopic endonasal approaches to the skull base. World Neurosurg. 2014, 82, S12–S21. [Google Scholar] [CrossRef]
  67. Consortium, C. CSF Rhinorrhoea After Endonasal Intervention to the Skull Base (CRANIAL)—Part 1: Multicenter Pilot Study. World Neurosurg. 2021, 149, e1077–e1089. [Google Scholar] [CrossRef]
  68. Porras, J.L.; Rowan, N.R.; Mukherjee, D. Endoscopic Endonasal Skull Base Surgery Complication Avoidance: A Contemporary Review. Brain Sci. 2022, 12, 1685. [Google Scholar] [CrossRef] [PubMed]
  69. Werner, M.T.; Yeoh, D.; Fastenberg, J.H.; Chaskes, M.B.; Pollack, A.Z.; Boockvar, J.A.; Langer, D.J.; D’Amico, R.S.; Ellis, J.A.; Miles, B.A.; et al. Reconstruction of the Anterior Skull Base Using the Nasoseptal Flap: A Review. Cancers 2023, 16, 169. [Google Scholar] [CrossRef] [PubMed]
  70. Snyderman, C.H.; Wang, E.W.; Zenonos, G.A.; Gardner, P.A. Reconstruction after endoscopic surgery for skull base malignancies. J. Neurooncol. 2020, 150, 463–468. [Google Scholar] [CrossRef]
Figure 1. Flowchart showing the methods for the selection of the studies included in this review. From [5].
Figure 1. Flowchart showing the methods for the selection of the studies included in this review. From [5].
Cancers 16 03747 g001
Figure 2. Histograms showing patient distributions for sex and age, main presenting symptoms and signs, anatomical origin of the lesion. Kaplan–Meier curve of the follow-up.
Figure 2. Histograms showing patient distributions for sex and age, main presenting symptoms and signs, anatomical origin of the lesion. Kaplan–Meier curve of the follow-up.
Cancers 16 03747 g002
Table 1. Demographic, clinical, radiological and pathological data of 149 cases of BSNS.
Table 1. Demographic, clinical, radiological and pathological data of 149 cases of BSNS.
StudiesDemographic and Clinical DataRadiological DataDiagnosis
Authors/YearNum of CasesSex,
Mean Age (Years)
Presenting SymptomsAnatomical OriginSkull Base InvolvementOrbit Involvement
1Lewis et al. [1],
2012
2821 F
7 M
(52 years)
Breath difficulty, congestion, facial pressure19 ES,
8 NC,
1 SS.
3 YES (ACF)7 YESImmunohistochemical
2Powers et al. [7]
2015
1M, 59Sinusitis, congestion, facial pressure, anosmia, dysgeusiaES-NCYES (ACF)NoneImmunohistochemical
3Rooper et al. [8]
2016
118 F
3 M
(44 years)
n.a.4 ES
3 FS
3 NC
1 ES-NC
None2 YESImmunohistochemical molecular
4Wong et al. [9]
2016
1M, 33Recurrent brisk epistaxisNC-SSNoneNoneImmunohistochemical molecular
5Huang et al. [10]
2016
74 M
3 F
(52 years)
n.a.2 FS
2 ES-NC
2 NC
1 ES
NoneNoneImmunohistochemical molecular
6Cannon et al. [11]
2017
33 F
(67.6 years)
Diplopia, facial discomfort,
supraorbital swelling
nasal obstruction, facial pressure
3 FS-ES3 YES3 Lamina papiraceaImmunohistochemical molecular
7Lin et al. [12]
2017
1F, 67Nasal obstructionES-FS-SS-MSYESNoneImmunohistochemical
8Hockstein et al. [36]
2018
1F, 79AsymptomaticFSYESRoofImmunohistochemical
9Andreasen et al. [13]
2018
32 F, 1 M
(59.6 years)
Nasal obstruction and midfacial pressure2 ES
1 ES-NC
NoneNoneImmunohistochemical
10Koszewski et al. [35]
2018
1M, 53Unilateral nasal obstruction and epiphoraNCYES (ACF)Lamina papiraceaImmunohistochemical
11Kakkar et al. [14]
2018
65 F, 1 M
(51 years)
Nasal obstruction1 NC
1 NC, MS
1 NC, MS, ES
1 NC, MS, ES
1 NC, MS, ES, FS
1 NC, ES
1 YESNoneImmunohistochemical
12Quadros et al.
2019
1F, 55Obstruction of the left nasal cavityNCNoneNoneImmunohistochemical
13Chitguppi et al. [6]
2019
1M, 53n.a.ES-NCYESYESImmunohistochemical molecular
14Alkhudher et al. [16]
2019
1F, 35Nasal obstruction, epistaxisNC, MS, ESNoneLamina papiraceaImmunohistochemical
15Miglani et al. [34]
2019
54 F, 1 M
(56 years)
n.a.5 NC-ES5 YES (ACF)5 Lamina PapiraceaImmunohistochemical
16Fudaba et al. [15]
2019
1M, 70Loss of consciousness and vomitingESYESNoneImmunohistochemical molecular
17Le Loarer et al. [17]
2019
4116 M, 25 F
(51 years)
n.a.14 NC
11 ES
10 ES-FS
6 n.a.
4 YES4 YESImmunohistochemical molecular
18Kuhn et al. [33]
2019
1n.a.Worsening nasal obstruction, rhinorrhea, left orbital pain, proptosis and blurry visionNC-ESYES (ACF)Lamina papiraceaImmunohistochemical molecular
19Okafor et al. [32]
2020
1M, 54Left-sided nasal airway obstruction and anosmiaNC-MS-ES-FSYES (ACF)Lamina papiraceaImmunohistochemical
20Okuda et al. [31]
2020
1F, 64Nasal obstructionNC-MS-ES pterygopalatine fossaYES (MCF)YESImmunohistochemical
21Sethi et al. [18]
2021
33 F
(56 years)
Left-sided nasal congestion and headaches/right nasal obstruction/rhinorrhea and left-sided nasal congestion3 ES-MS-FS-NC1 YES (ACF)2 YESImmunohistochemical
22Hanbazazh et al. [19]
2021
1M, 50Orbital pain and pressure, diplopia, blurred vision, lateral gaze restrictionESYESLamina papiraceaImmunohistochemical molecular
23Bell et al. [20]
2022
1M, 66Swelling of left eyelid, vertical
diplopia and purulent nasal discharge
NCYES (ACF)YESImmunohistochemical molecular
24Hasnie et al. [21]
2022
1F, 72Nasal obstruction, episodic epistaxis and facial pressure/headaches, decreased sense of smellMS-ES-Bilateral FS-NCYES (ACF)Lamina papiraceaImmunohistochemical molecular
25Turri-Zanoni et al. [22]
2022
153 M, 12 F
(54 years)
14 nasal airway obstruction
9 epistaxis,
6 olfactory disfunction3 facial pain
13 ES
2 FS
NoneNoneImmunohistochemical molecular
26Nichols et al. [23]
2023
1M, 54Persistent headaches, postnasal
drip, thickened nasal secretions, and epistaxis after sneezing
ES-SSNoneNoneImmunohistochemical molecular
27Ingle et al. [24]
2023
1F, 47Swelling eyelid, proptosisNC, FS, ES, MSNoneLamina papiraceaImmunohistochemical
28Meyer et al. [25]
2023
1M, 67Nasal congestion and epiphora, right-sided ocular proptosisES-MS-FSNoneYESImmunohistochemical molecular
29Kominsky et al. [26]
2023
22 M
(65 years)
Bilateral nasal congestion and blurry visionES-NC-FS2 YES2 Lamina papiraceaImmunohistochemical molecular
30Bhele et al. [27]
2023
1F,22Vision loss, headache, hyposmia, facial pressureNC-ES-SS-MSYES (ACF)Lamina papiraceaImmunohistochemical
31Viramontes et al. [28]
2023
1F, 40Progressive obstruction of the right nasal cavity,NCNoneNoneImmunohistochemical molecular
32Muraoka et al. [29]
2023
1F, 73Purulent nasal discharge and dull pain in the left cheek areaNC-ES-FSYES (ACF)NoneImmunohistochemical molecular
33Anastasiadou et al. [30]
2023
33 F
(43 years)
Exophthalmos, headachesNC-MS1 YES2 YESImmunohistochemical molecular
34Corvino et al. [37]
2024
1M, 46l. proptosis, upward gaze restrictionFS-ESYES (ACF)RoofImmunohistochemical
M: male, F: female, n.a.: not available; l: left; ACF: anterior cranial fossa; mo.: months; ES: ethmoid sinus: FS: frontal sinus; SS: sphenoid sinus; MS: maxillary sinus; NC: nasal cavity.
Table 2. Treatment and outcome data of 149 cases of BSNS.
Table 2. Treatment and outcome data of 149 cases of BSNS.
StudiesTreatment DataOutcome Data at Last Follow Up
Authors/YearNum of CasesTime to TreatmentType of TreatmentType of Surgical ApproachEORPeri-Post
Operative Complications
ClinicsRecurrenceStatus
1Lewis et al. [1]
2012
28n.a.n.a.n.a.n.a.n.a.n.a.7/16
(range 12–118 mo.)
(mean 8.3 years)
14 alive
2 dead due to other causes
2Powers et al. [7]
2015
1n.a.SEEAGTRCSF leakn.a.NoneAlive 10 mo.
3Rooper et al. [8]
2016
11n.a.n.a.n.a.n.a.n.a.n.a.2/7
(range 1–26 mo.)
(mean 4 years)
1/7 dead due to tumor
4Wong et al. [9]
2016
1n.a.S
Ad-CHT-RT
EEAGTRn.a.n.a.NoneAlive 5 mo.
5Huang et al. [10]
2016
7n.a.6 S
1 S + Ad-CHT-RT
n.a.4 GTRn.a.n.a.1/4
(36 mo)
(mean 8 years)
4 alive
6Cannon et al. [11]
2017
3n.a2 S
1 Biopsy
1 EEA −
1 EEA + TCA1 EEA Biopsy
2 GTR
1 STR
n.a.n.a.1/3
(17 mo.)
(mean 25 mo.)
3 alive
7Lin et al. [12]
2017
1n.aSEEAGTRSubarachnoid hemorrhage;
brain herniation
n.a.n.a.Dead due to surgery
8Hockstein et al. [36]
2018
112 mo.SEEA + TCAGTRn.a.n.a.NoneAlive
9Andreasen et al. [13]
2018
3n.a1S
2 S + Ad.RT
n.a.3 GTRNonen.a.1/3
(11, 21 and 24 mo)
(mean 67.3 mo.)
3 alive
10Koszewski et al. [35]
2018
14 mo.S + Ad.RTn.a.STRn.a.n.a.NoneAlive
11Kakkar et al. [14]
2018
6n.a.3 S
3 Biopsy
3 EEA4 STRn.a.n.a.None1/6 dead due to other causes
12Quadros et al.
2019
1n.aSEEAn.a.n.a.n.a.n.a.n.a.
13Chitguppi et al. [6]
2019
1n.a.S + Ad-RTTCA + ETOASTRn.a.n.a.NoneAlive
14Alkhudher et al. [16]
2019
12 mo.SEEAGTRn.a.ImprovedNoneAlive 2 years
15Miglani et al. [34]
2019
5n.a4 S
1 S + Ad-RT
3 TCA
2 EEA
4 GTR
1 STR
n.a.n.a.2/5
(mean 31.4 mo.)
(mean 31.4 mo.)
5 alive
16Fudaba et al. [15]
2019
1REC after 11 yearsSEEA + TCAGTRn.a.n.a.No furtherAlive
17Le Loarer et al. [17]
2019
41n.a.20 S
8 S + RT
2 S + RT + CHT
1 RT+ CHT
2 S + CHT
n.a.n.a.n.a.n.a.8/25
(range 9–95 mo.)
(mean 45 mo.)
18Kuhn et al. [33]
2019
1n.aSTCAGTRNonen.a.n.a.n.a.
19Okafor et al. [32]
2020
15 mo.2 SEEA1 STR
1 GTR
Nonen.a.n.a.n.a.
20Okuda et al. [31]
2020
1REC after 2 mo.S + Ad.CHTTCAGTRNonen.a.YES
(after 2 mo)
Dead 8 mo., death due to tumor progression
21Sethi et al. [18]
2021
3n.a1 S + Ad.RT
2 S
3 EEA3 GTRNonen.a.None2 alive
(mean 22 mo)
22Hanbazazh et al. [19]
2021
136 mo1 Biopsy
1 S
1 S + Ad.RT
Biopsy EEA
TO
TCA
STRNoneImprovedNoneAlive
23Bell et al. [20]
2022
1REC after 15 years1 S + Ad.RTTCAGTRNoneStableNo furtherAlive 10 mo.
24Hasnie et al. [21]
2022
124 mo.SEEA + TCAGTRInfection, pneumocephaln.a.NoneDeath due to other causes
25Turri-Zanoni et al. [22]
2022
15n.a13 S
2S + RT
7 EEA
8 EEA + TCA
13 GTR2 STRn.a.n.a.1/15
(after 35 and 47 mo)
(27.3 months)
15 alive
26Nichols et al. [23]
2023
1n.aSEEAn.a.n.a.ImprovedNoneAlive 3 mo.
27Ingle et al. [24]
2023
12 mo.SEEA + TCAGTRn.a.n.a.NoneAlive 3 mo.
28Meyer et al. [25]
2023
136 mo.Biopsy, RT, CHTEEABiopsyn.a.n.a.ProgressionDead 15 mo., death due to tumor progression
29Kominsky et al. [26],
2023
23 weeks (1)2 S2 EEA2 GTRn.a.n.a.None2 alive
(mean 13 mo.)
30Bhele et al. [27]
2023
18 mo.Biopsy, Neo-CHT,
S,
Ad-PB
TCA + EEASTRn.a.n.a.NoneAlive, 10 mo.
31Viramontes et al. [28],
2023
1n.aSEEAGTRn.a.n.a.NoneAlive, 16 mo.
32Muraoka et al. [29]
2023
1n.aSTCA + EEAGTRn.a.n.a.NoneAlive
33Anastasiadou et al. [30],
2023
3n.a1 S,
2 S + Ad.RT
3 EEA3 GTR1 CSF leakn.a.NoneAlive 7 years
34Corvino et al. [37]
2024
12 mo.STCA + EEAGTRNoneImprovedNoneAlive, 10 mo.
available data; n.a.: not available; GTR: gross total resection; STR: sub-total resection; S: surgery; RT: radiotherapy; CHT: chemotherapy; Ad: adjuvant; TCA: transcranial approach; EEA: endoscopic endonasal approach.
Table 3. Summarized available demographic, clinical, neuroradiological and pathological data of 149 cases of biphenotypic sinonasal sarcoma.
Table 3. Summarized available demographic, clinical, neuroradiological and pathological data of 149 cases of biphenotypic sinonasal sarcoma.
CovariatesOverall Sample
149 (%)
Statistical Analysis
(p Value)
Demographic and clinical data
Sex
-F
-M
148/149 * (99.3%)
99/148 (66.9%)
49/148 (33.1%)
p = 0.6
Age range
(median)
22–79 years
(54.88 y.o.)
p = 0.04
Main presenting symptoms
-Nasal obstruction
-Facial pressure/pain/discomfort
-Epistaxis
-Ocular impairment
84/149 * (56.3%)
68/84 (81%)
37/84 (44%)
13/84 (15.5%)
12/84 (14.3%)
p = 0.46
Radiological data
Anatomical Origin
-NC
-ES
-FS
-MS
-SS
143/149 * (96%)
65/143 (45.4%)
97/143 (67.8%)
34/143 (23.7%)
18/143 (12.6%)
5/143 (3.5%)
p = 0.32
Skull Base involvement
-Yes
-Not
143/149 * (96%)
35/143 (24.5%)
108/143 (75.5%)
p = 0.22
Orbit involvement
-Yes
-Not
143/149 * (96%)
41/143 (28.7%)
102/143 (71.3%)
p = 0.26
Pathological Diagnosis
Diagnostic method
-immunohistochemical alone
-immunohistochemical and molecular
149/149 * (100%)
56/149 (37.6%)
93/149 (62.4%)
p = 0.55
* available data.
Table 4. Summarized available treatment and outcome data of 149 cases of biphenotypic sinonasal sarcoma.
Table 4. Summarized available treatment and outcome data of 149 cases of biphenotypic sinonasal sarcoma.
CovariatesOverall Sample
149 (%)
Statistical Analysis
(p Value)
Treatment Data
Time to treatment
(mean in months)
11/149 (7.3%)
12 months
p = 0.11
Type of treatment
-S
-S + RT
-Biopsy alone
-S + CHT
-S + RT + CHT
-RT + CHT
104/149 * (69.8%)
69/104 (66.3%)
20/104 (19.2%)
5/104 (4.8%)
3/104 (2.9%)
5/104 (4.8%)
2/104 (1.9%)
p = 0.43
Type of surgical approach
-EEA
-TCA
-TOA
-Combined
58/149 * (39%)
33/58 (56.9%)
7/58 (12%)
1/58 (1.7%)
17/58 (29.3%)
p = 0.1
EOR
-GTR
-STR
62/149 * (41.6%)
49/62 (79%)
13/62 (21%)
p = 0.45
Peri- and post-operative complications
-Yes
-None
12/149 * (8%)
4/12 (33.3%)
8/12 (66.7%)
Outcome
Clinical
-Improved
-Stable
-Worsened
5/149 * (3.3%)
4/5 (80%)
1/5 (20%)
---
Recurrence
-Yes
-Not
84/149 * (56.3%)
22/84 (26.2%)
62/84 (73.8%)
p = 0.6
Status
-Alive
-Dead
85/149 * (57%)
77/85 (91.8%)
8/85 (8.2%)
p = 0.87
Follow-upMean 4.6 yearsSt. Dev = 3.05
* available data. S: surgery; RT: radiotherapy; CHT: chemotherapy; TCA: transcranial approach; EEA: endoscopic endonasal approach; TOA: transorbital approach; GTR: gross total resection; STR: sub-total resection.
Disclaimer/Publisher’s Note: The statements, opinions and data contained in all publications are solely those of the individual author(s) and contributor(s) and not of MDPI and/or the editor(s). MDPI and/or the editor(s) disclaim responsibility for any injury to people or property resulting from any ideas, methods, instructions or products referred to in the content.

Share and Cite

MDPI and ACS Style

Corvino, S.; Corazzelli, G.; Mariniello, G.; Iuliano, A.; Altieri, R.; Pontillo, G.; Strianese, D.; Barbarisi, M.; Elefante, A.; de Divitiis, O. Biphenotypic Sinonasal Sarcoma: Literature Review of a Peculiar Pathological Entity—The Neurosurgical Point of View. Cancers 2024, 16, 3747. https://doi.org/10.3390/cancers16223747

AMA Style

Corvino S, Corazzelli G, Mariniello G, Iuliano A, Altieri R, Pontillo G, Strianese D, Barbarisi M, Elefante A, de Divitiis O. Biphenotypic Sinonasal Sarcoma: Literature Review of a Peculiar Pathological Entity—The Neurosurgical Point of View. Cancers. 2024; 16(22):3747. https://doi.org/10.3390/cancers16223747

Chicago/Turabian Style

Corvino, Sergio, Giuseppe Corazzelli, Giuseppe Mariniello, Adriana Iuliano, Roberto Altieri, Giuseppe Pontillo, Diego Strianese, Manlio Barbarisi, Andrea Elefante, and Oreste de Divitiis. 2024. "Biphenotypic Sinonasal Sarcoma: Literature Review of a Peculiar Pathological Entity—The Neurosurgical Point of View" Cancers 16, no. 22: 3747. https://doi.org/10.3390/cancers16223747

APA Style

Corvino, S., Corazzelli, G., Mariniello, G., Iuliano, A., Altieri, R., Pontillo, G., Strianese, D., Barbarisi, M., Elefante, A., & de Divitiis, O. (2024). Biphenotypic Sinonasal Sarcoma: Literature Review of a Peculiar Pathological Entity—The Neurosurgical Point of View. Cancers, 16(22), 3747. https://doi.org/10.3390/cancers16223747

Note that from the first issue of 2016, this journal uses article numbers instead of page numbers. See further details here.

Article Metrics

Back to TopTop