Spotlight on SMARCB1-deficient
Sinonasal Carcinoma

An Underrecognized
SMARCB1 Entity

We are particularly focused on raising awareness of SMARCB1-deficient sinonasal carcinoma (SDSC) among pathologists, medical teams, sinonasal cancer patients, and the general public. We provide support to SDSC patients and strive to connect them with specialized multidisciplinary teams and SMARCB1 research scientists for guidance and optimal treatment.

SDSC is one of the lesser-known members of the SMARCB1 cancer family, yet it touches a broader age group (from adolescence to old age) than most of the others. First described in 2014, and with fewer than 200 cases reported worldwide, SDSC remains an underrecognized distinct sinonasal tumor entity. Many pathologists and oncology teams, including those with experience in head and neck pathology, have little or no experience with it. 

SDSC is a challenging sinonasal cancer to diagnose, especially on morphology alone, and several extra biopsy stains than would ordinarily be done are required. SDSC is often incorrectly diagnosed and getting a correct diagnosis early on is very important to establish the best treatment plan possible. SMARCA4-deficient sinonasal carcinoma, a very similar tumor entity considered to be part of the same family, is even less known than SDSC.

Differential diagnoses for SDSC include: sinonasal undifferentiated carcinoma (SNUC), poorly differentiated sinonasal carcinoma, NUT midline carcinoma, myoepithelial carcinoma, high grade neuroendocrine carcinoma, HPV-related multiphenotypic sinonasal carcinoma, adamantinoma-like Ewing sarcoma, and others.

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