Low-grade Sinonasal Sarcoma with Neural and Myogenic Features

Hockstein, Neil; Dross, Peter; Farooqui, Shoheb; Wilhelm, Ian N

doi:10.1177/014556131809700609

Cited by 5 publications

(5 citation statements)

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“…A total of 100 cases were identified in 12 previously published reports in addition to 2 novel cases presented here. 1,[4][5][6][7][8][9][10][11][12] Of all 102 cases, the mean age of presentation was 52.9 years old (range 24-87). The ratio of female:male patients was 1.8:1.…”

Section: Resultsmentioning

confidence: 99%

“…Twenty-nine cases were treated with surgical excision, with 17 (59%) having follow-up. 5,[7][8][9][10][11]13 Ten cases were treated with surgical excision and adjuvant radiation therapy, 9 of which had follow-up. 10,11,14 Recurrence rates were equivalent between surgical excision alone and surgical excision with adjuvant radiation therapy (33.3%), suggesting little to no benefit for adjuvant radiation.…”

Section: Discussionmentioning

confidence: 99%

“…One case demonstrated erosion of the anterior and posterior frontal plate with significant intracranial extension. 9 Magnetic resonance imaging demonstrated a homogenously enhancing soft tissue mass with variable signal intensity (mild to strong). T1-weighted noncontrast images demonstrated low signal intensity that showed avid, homogenous enhancement in contrast sequences as well as heterogenous enhancement in postcontrast fat-saturated sequences.…”

Section: Radiographic Findingsmentioning

confidence: 99%

“…Of the reported cases, 9 included radiographic findings. 5,[7][8][9][10]13,14 Computed tomography scan demonstrated a homogeneous soft-tissue mass located in the paranasal sinuses and/or nasal canal. Additional findings included erosion through the lamina papyracea, hyperostotic bone formation, or thinning of the cribriform plate.…”

Section: Radiographic Findingsmentioning

confidence: 99%

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Biphenotypic Sinonasal Sarcoma: A Case Report and Review of Literature

et al. 2021

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Objectives: Biphenotypic sinonasal sarcoma (BSNS), previously low-grade sinonasal sarcoma with neural and myogenic features, is a rare tumor of the sinonasal tract first described in 2012. Due to its rarity, limited literature is available in providing clinicians with a standardized treatment regimen, particularly in cases of positive surgical margins. This article aims to provide a clinical review of the currently available reported cases of BSNS, as well as presenting clinical, radiologic, and pathologic details of 2 novel cases. Methods: Online electronic databases include PubMed and Embase where queried for reports of biphenotypic sinonasal sarcoma or low-grade sinonasal sarcoma with neural and myogenic features. Two previously unpublished cases were included in the results. Data including clinical presentation, epidemiologic data, radiologic evaluation, intraoperative details, histopathology, treatment modality, and postoperative follow-up information were included. Results: A total of 100 previously published cases were identified in 12 prior articles. Mean age at presentation was 52.9 years. Extrasinonasal extension was observed in 27.4% of cases with most common site of extension being cribriform plate. Forty-seven cases included treatment details with surgical excision being the most common modality. Recurrence rates were identical for both surgical excision alone and surgical excision with adjuvant radiotherapy (33.3%). Conclusions: Biphenotypic sinonasal sarcoma is a slow-growing tumor that is amenable to surgical resection. Recurrence rates are similar between surgical excision and surgical excision with adjuvant radiation therapy, but limited data in reported cases preclude a determination of treatment superiority.

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Section: Resultsmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

Section: Radiographic Findingsmentioning

confidence: 99%

Section: Radiographic Findingsmentioning

confidence: 99%

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Biphenotypic Sinonasal Sarcoma: A Case Report and Review of Literature

et al. 2021

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“…[ 3 ] However, in some rare instances, these tumours can also present with atypical symptoms of midline frontal mass. [ 4 ] Biphenotypic sinonasal sarcoma usually involves multiple sites in the sinonasal region. Nose and paranasal sinuses involvement are common with ethmoid sinus being the most common, followed by frontal sinus and sphenoid sinus is less frequently involved.…”

Section: Discussionmentioning

confidence: 99%

Biphenotypic Sinonasal Sarcoma with Intracranial Extension – A Case Report with Review of Literature

Kumar

Arora

Goel

2022

Annals of Maxillofacial Surgery

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Rationale: Biphenotypic sinonasal sarcoma is a rare malignant tumour exclusively involving the sinonasal cavity. These tumours have variable and atypical presentations. Early approach and correct treatment modalities are key factors in the management of such cases. Patient Concern: A 48-year-old male patient presented with left-sided nasal obstruction and intermittent nasal bleeding for one year. Diagnosis: Biphenotypic sinonasal sarcoma confirmed on histopathological examination and immunohistochemistry. Treatment: The patient underwent surgical excision with left lateral rhinotomy and bifrontal craniotomy with skull base repair. The patient also received postoperative radiotherapy. Outcome The patient is on regular follow-up with no similar complaints. Take-Away Lesson: Treating team should keep the diagnosis of biphenotypic sinonasal sarcoma in mind while investigating a patient with nasal mass. Surgical management is the treatment of choice, due to its local aggressive nature and proximity to the brain and eyes. Postoperative radiotherapy is vital to prevent tumour recurrence.

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Biphenotypic sinonasal sarcoma: European multicentre case-series and systematic literature review

Turri‐Zanoni

Dalfino

Lechner

et al. 2022

Acta Otorhinolaryngol Ital

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SUMMARY Objective Biphenotypic sinonasal sarcoma (BSNS) is a rare low-grade cancer that was included from the 4th edition of WHO classification of head and neck tumours. The purpose of this study is to analyse clinical behaviour, pattern of recurrences and survival outcomes of this neoplasm. Methods Retrospective review of patients affected by BSNS who were treated via an endoscopic-assisted approach in 6 European tertiary-care referral hospitals. Cases of BSNS described in literature since 2012 to date were fully reviewed, according to PRISMA guidelines. Results A total of 15 patients were included. Seven patients were treated via an endoscopic endonasal approach, 4 with endoscopic transnasal craniectomy, and 4 via a cranio-endoscopic approach. Adjuvant treatment was delivered in 2 cases. After a mean follow-up of 27.3 months, systemic metastasis was observed in 1 case; the 5-year overall survival and disease-free survival rates were 100% and 80 ± 17.9%, respectively. Conclusions BSNS is a locally aggressive tumour with a low recurrence rate and encouraging survival outcomes if properly treated with surgical resection and free margins followed by adjuvant radiotherapy for selected cases. Endoscopic-assisted surgery is safe and effective as an upfront treatment within a multidisciplinary care protocol.

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Low-grade Sinonasal Sarcoma with Neural and Myogenic Features

Cited by 5 publications

References 1 publication

Biphenotypic Sinonasal Sarcoma: A Case Report and Review of Literature

Biphenotypic Sinonasal Sarcoma: A Case Report and Review of Literature

Biphenotypic Sinonasal Sarcoma with Intracranial Extension – A Case Report with Review of Literature

Biphenotypic sinonasal sarcoma: European multicentre case-series and systematic literature review

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