Case Presentation

Loong Shihleone
Singapore General Hospital
56F
Right persistent blocked nose x5 years
• a/w hyposmia
• no purulent nasal discharge
• no epistaxis
• no facial pain
Nasoendosopic findings
• Deviated nasal septum to right
• Right ANS polyp covering whole ANS, ?attachment
• Left ANS clear
• Postnasal space, bilateral fossa of Rosenmuller clear

• Neck no palpable masses

• Oral cavity and oropharynx clear
• Larynx bilateral VC intact and mobile
CT Paranasal Sinus
• There is a well-defined, polypoidal, soft
tissue mass-like lesion seen in the right
nasal cavity, extending into the right
middle meatus/osteomeatal complex
• Significant mass effect on the R middle
and inferior turbinates
• Attenuation of the medial
wall/scalloping of the medial wall of the
right maxillary sinus
Intraoperative findings
Functional sinus endoscopic surgery
• Irregular tumour filling olfactory cleft with broad based attachment
over cribriform, superior septum, and medial aspect of right superior
and middle turbinate
• Right middle turbinate attenuated and lateralised by tumour
Differentials
• Peripheral nerve sheath tumour
• Synovial sarcoma
• Solitary fibrous tumour
• Glomangiopericytoma
• Biphenotypic sinonasal sarcoma
S100 SOX10

SMA Calponin
Negative Ki67
• Desmin
• AE1/3
• EMA
• CD34
• β-catenin
• STAT6
• SS18-SSX
• Ki67 up to 5%
Summary
• Variably cellular fascicles of spindle cells
proliferation with focal herringbone pattern
• Entrapped respiratory epithelium
• No significant mitosis or necrosis

• Positive for S100, focal reactivity to

SMA/Calponin, but negative for SOX10 and
Desmin
• Low Ki67 proliferative fraction
Molecular
Targeted RNA sequencing via Archer FusionPlex assay
• PAX3 (exon 7) - MAML3 (exon 2) fusion identified

• Final diagnosis: Biphenotypic sinonasal sarcoma

Biphenotypic sinonasal sarcoma (BSNS)
• Biphenotypic sinonasal sarcoma (BSNS) is a rare low-grade sarcoma first
described by Lewis et al in 2012 as low-grade sinonasal sarcoma with
neural and myogenic differentiation.
• Primarily affect adults, F>>M
• Present with nonspecific symptoms of nasal passage obstruction, epistaxis,
and nasal/sinus pain and congestion.
• Typically involves multiple sinonasal subsites, with the superior nasal cavity
and ethmoid sinus most commonly involved, followed by the sphenoid
sinus.
• Can be locally destructive with invasion beyond the nasal cavity and
sinuses.
Biphenotypic sinonasal sarcoma (BSNS)
• Infiltrative growth composed of
spindled cells forming medium-
to-long fascicles, often with a
herringbone pattern
• Variations in cellular density,
can be densely cellular
• Tumour cells are uniform, ovoid
or wavy nuclei with fine
chromatin
• Often entrapped hyperplastic
respiratory epithelium Biphenotypic sinonasal sarcoma. Adapted from “Biphenotypic Sinonasal Sarcoma: A Review and
Update” by Carter CS, East EG, McHugh JB, Oct 2018
Biphenotypic sinonasal sarcoma (BSNS)
• Hemangiopericytoma-like
vessels can be seen
• Infiltration of bone is common
• Mitotic activity is consistently
low
• No necrosis

Biphenotypic sinonasal sarcoma with bone invasion. Adapted from “Biphenotypic Sinonasal Sarcoma:
A Review and Update” by Carter CS, East EG, McHugh JB, Oct 2018
 Differential diagnosis
Differential Morphology IHC
Respiratory epithelial adenomatoid Proliferation of branching glands, separated by edematous or
hamartoma fibrous stroma, usually with chronic inflammation, glands are
typically surrounded by thickened, brightly eosinophilic
basement membrane
Cellular schwannoma Antoni A and B areas, hyalinized blood vessels S100 &
SOX10+
MPNST High grade (mitosis, necrosis) Focal S100 &
SOX10+
Synovial sarcoma Densely cellular CD99 & SS18-
Epithelial proliferations in biphasic SS consist of polygonal SSX+
epithelioid cells rather than entrapped sinonasal epithelium

Solitary fibrous tumour Less cellular, short fascicles, collagenous stroma, more STAT6+
prominent hemangiopericytic vessels
Glomangiopericytoma Plump eosinophilic cytoplasm, β-catenin+
short fascicles, whorls, hemangiopericytic vessels
Molecular
Associated with recurrent driver gene fusions, most frequently
PAX3:MAML3 (60%)
• Alternative 3’ partners include NCOA1, NCOA2, FOXO1, and WWTR1
Prognosis and treatment
• Local recurrence up to 40 - 50%
• No distant metastases have yet been reported

• Local excision, with or without adjuvant radiation therapy