Metastatic Prostate Cancer Involving the Sphenoid Sinus and Mandible.

Prostate cancer is the third most leading cause of cancer in men in the United States. Although expected metastatic spread to bone, liver, and lymph nodes are often monitored, there are other rare presentations that can occur. This case report demonstrates a rare presentation of prostate cancer spreading to the paranasal sinuses and orbit. Not only did this case have an atypical presentation mimicking infection, the diagnosis was also only achieved through pathological evaluation after an endoscopic examination and biopsy. This case demonstrates the importance of a low threshold for endoscopic examinations in uncertain sinonasal presentations, and consistent biopsies when performing endoscopic examinations.

The Incidence of Velopharyngeal Insufficiency in Stickler Syndrome.

OBJECTIVE: Stickler Syndrome (SS) is an inherited collagenopathy characterized by heterogenous orofacial, ocular, auditory, and skeletal abnormalities. The orofacial manifestations are variable and some patients present with cleft palate and velopharyngeal insufficiency (VPI). The incidence of VPI in SS is poorly studied and no studies have compared the incidence of VPI between Type I (COL2A1) and Type II (COL11A1) SS. The objective of this study is to compare the incidence of VPI between SS subtypes and discuss the surgical techniques used to treat them. DESIGN: Single-institution, retrospective chart review. SETTING: Tertiary pediatric hospital. PATIENTS/PARTICIPANTS: Forty-three children were diagnosed with SS between January 2003 and December 2018. Genetic testing results, genetics notes, craniofacial clinic notes, and operative reports were reviewed. Patients without genetic testing or craniofacial/otolaryngologic evaluation were excluded. Thirty-one patients met criteria and were included. MAIN OUTCOME MEASURE: Primary outcome was VPI incidence. RESULTS: There were 18 patients with Type I SS and 13 with Type II SS. Five (16%) patients had VPI, 2 (11%) with Type I SS compared to 3 (23%) with Type II SS (P > .05). All patients with VPI underwent surgery with either sphincter pharyngoplasty (3) or pharyngeal flap (2). Two patients with Type II SS underwent revision sphincter pharyngoplasty, with one conversion to pharyngeal flap. CONCLUSION: VPI is common for patients with SS. In this study, there was no significant difference in the incidence of VPI between SS subtypes. Future studies are needed to confirm these findings, which could be important for patient counseling and treatment planning.

Preclinical evidence for pioglitazone and bexarotene combination in oral cancer chemoprevention.

BACKGROUND: Head and neck squamous cell carcinoma (HNSCC) requires new treatments and targeted approaches to improve survival. The peroxisome proliferator-activated receptor γ (PPARγ) and retinoic X receptor alpha (RXRα) nuclear receptor pathways may be targetable with repurposed Food and Drug Administration (FDA)-approved agents for prevention and treatment. METHODS: Oral cancer and leukoplakia cell lines were treated with the PPARγ agonist (pioglitazone) and RXRα activator (bexarotene). PPARγ activation, cellular proliferation, apoptosis activity and phenotype, including the pharmacodynamic marker, involucrin (IVL), were subsequently analyzed using a reporter gene assay, genomic data, MTT assay and western blot. RESULTS: Microarray analysis of HNSCC tumor versus normal tissue shows IVL expression is significantly increased in normal tissue compared to HNSCC tumors (p < 0.0001). In MSK Leuk1 and CA 9-22 cell lines, pioglitazone increases PPARγ DNA binding activity and IVL promoter activity in a dose dependent manner (p < 0.01 and p < 0.0001). Combination treatment with pioglitazone and bexarotene increases PPARγ DNA binding activity and IVL promoter activity (p < 0.01 and p < 0.0001). MTT analysis shows decreases in cell proliferation when cells are treated with pioglitazone and bexarotene. Decreases in cell proliferation are significant to at least p < 0.05 for all combination versus single agent treatments. Western blot on whole-cell lysate from cells treated with pioglitazone and bexarotene alone or in combination for IVL showed increased protein levels with combination treatment. CONCLUSIONS: Targeting the PPARγ/RXRα heterodimer with pioglitazone and bexarotene was effective in this preclinical project. This was functional in both preneoplastic and oral cancer cell lines. A better understanding of the molecular mechanism on downstream effects on cellular proliferation could potentially have implications clinically, both in oral preneoplasia and possibly head and neck cancer; however, more research needs to be done to explore the potential these medications have in chemoprevention.

Incidence of mandibular distraction osteogenesis in Stickler Syndrome: Variation due to COL2A1 and COL11A1.

OBJECTIVE: To determine whether the two most common genetic mutations seen in Stickler Syndrome (SS) (COL2A1 and COL11A1) affect the incidence of mandibular distraction osteogenesis (MDO) and what impact Robin sequence (RS) has on diagnosis. SS is an autosomal dominant connective tissue disorder characterized by almost complete penetrance. COL2A1 and COL11A1 are the two most common mutations seen in SS patients. SS often presents at birth with RS, which is characterized by the triad of micrognathia, glossoptosis, and tongue-based airway obstruction. MDO is one surgical intervention that has been shown to be successful in relieving tongue base obstruction and is the surgical intervention of choice for this condition. METHODS: A retrospective chart review was performed on all patients with a diagnosis of SS at a tertiary pediatric hospital between January 1, 2003 and December 31, 2018. The included patient charts were reviewed for demographic information, SS mutation, and history of MDO. Forty-six patients had a clinical diagnosis of SS. Of those, 31 met inclusion criteria which involved having a molecular diagnosis of SS and sufficient follow up information to determine if MDO was indicated or performed. Twenty-two of the 31 included patients had a diagnosis of RS (70.96%). Thirteen of the 31 patients (41.94%) included in this study required MDO as a neonate. RESULTS: Fifty-percent of patients with type I (COL2A1) required MDO as a neonate compared to only 31% of patients with type II (COL11A1), though the difference between the two groups was not statistically significant. CONCLUSION: The findings of this study suggest that patients with type I mutation may have a higher incidence of MDO than patients with a type II mutation, though further research with larger sample sizes is needed. This information is helpful in counseling those with SS or family history of SS about what they can expect related to RS and need for MDO based on genetic findings. LEVEL OF EVIDENCE: 3.