Angioleiomyomas are smooth muscle masses originating from blood vessel tunica media. This is a rare tumor, comprising less than 1% of benign sinonasal cavity tumors. Failure to recognize and surgically excise nasal angioleiomyomas can result in lesion recurrence. We present 2 new cases of nasal septum angioleiomyoma at a single institution. Additionally, both cases underwent septal and nasal surgery. Due to the nonspecific clinical and imaging findings, a high index of suspicion is needed to diagnose nasal septum angioleiomyoma, often requiring histopathological verification. Preferred treatment is complete surgical excision. From our experience, concurrent corrective septal and sinus surgery can be completed with tumors less than 1.0 cm in size.

Background: PR domain containing 16 (PRDM16) is a key transcriptional regulator in the development of craniofacial, adipose, and neural tissues. Our lab identified PRDM16 expression in the epithelial cells of the Kölliker's organ (KO) that starts at ~E13.5 and is maintained until KO disappearance. A transgenic mouse model that carries a gene trap null allele of Prdm16 (Prdm16 cGT ) was used to characterize the impact of Prdm16 loss on cochlear development.Results: At P0 Prdm16 cGT null cochlea exhibited hypoplastic KO, shortened cochlear duct, increased density of hair cells (HCs) and supporting cells (SCs) in the apical turn as well as multiple isolated ectopic HCs within the KO domain. KO epithelial cells proliferation rate was reduced in the apical turn of the developing Prdm16 cGT null cochlea vs controls. Bulk RNA sequencing of cochlear duct cells at E14.5 followed by quantitative real time PCR and mRNA Fluorescence in-situ hybridization (FISH) validation identified differentially expressed genes in Prdm16 cGT null vs littermate control cochleae. Upregulated genes at E14.5 included Fgf20, as well as several Notch pathway genes (Lfng, Hes1, and Jag1).Conclusions: This study characterizes Prdm16 expression during cochlear development and establishes its requirement for KO development.

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BACKGROUND: Angioleiomyoma is an uncommon, benign tumour originating from vascular smooth muscle. Occurrence in the nasal cavity, especially the nasal septum, is rare with only 16 cases of nasal septal angioleiomyoma reported thus far. We present a new case of nasal septal angioleiomyoma. CASE PRESENTATION: 52-year-old female presented with a three-week history of persistent epistaxis and nasal obstruction. Physical examination revealed a right sided nasal mass originating from the septum. The tumour was endoscopically excised and sent for pathological evaluation which revealed the diagnosis of angioleiomyoma. The patient was followed for six months postoperati- vely and showed no evidence of lesion recurrence. CONCLUSIONS: Because nasal cavity angioleiomyoma recurrence has been reported due to incomplete tumour resection, wide marginal excision is necessary for successful treatment. There have been no cases of malignant transformation of nasal angiolei- omyomas; however, malignant transformation at other sites has been reported. For these reasons, it is important to adequately biopsy and remove angioleiomyoma lesions.