Huntington’s disease (HD) is caused by an abnormal expansion of CAG trinucleotide repeats encoding polyglutamine (polyQ) in the first exon of the huntingtin (htt) gene. Despite considerable efforts, the pathogenesis of Huntington’s disease (HD) remains largely unclear due to a paucity of models that can reliably reproduce the pathological characteristics of HD. Here, we report a neuronal cell model of HD using the previously established tetracycline regulated rat neuroprogenitor cell line, HC2S2. Stable expression of enhanced green fluorescence protein (EGFP) tagged-htt exon 1 (referred to as 28Q and 74Q, respectively) in the HC2S2 cells did not affect rapid neuronal differentiation. However, compared to the cells expressing wild type htt, the cell line expressing mutant htt showed an increase in time-dependent cell death and neuritic degeneration, and displayed increased vulnerability to oxidative stress. Increased protein aggregation during the process of neuronal aging or when the cells were exposed to oxidative stress reagents was detected in the cell line expressing 74Q but not in its counterpart. These results suggest that the neuroprogenitor cell lines mimic the major neuropathological characteristics of HD and may provide a useful tool for studying the neuropathogenesis of HD and for high-throughput screening of therapeutic compounds.

Background: Myoepithelial cell carcinoma is a rare malignant neoplasm of salivary gland origin that typically presents in the parotid gland and minor salivary glands. It has been described previously in head and neck sites such as buccal mucosa, alveolar ridge, and base of tongue. Methods: A 55-year-old man presented with 30 years of right-sided tongue pain and 10 years of gradually worsening ulceration. Physical examination demonstrated a 2.5 cm ulcerative lesion of the anterior right oral tongue. An initial biopsy was consistent with moderately to poorly differentiated squamous cell carcinoma. Imaging included a positron emission tomography (PET)/computed tomography (CT) scan that demonstrated the right tongue lesion as well as hypermetabolic right level II adenopathy. The patient underwent surgical excision of the right tongue, upper aerodigestive tract endoscopy, and a bilateral supraomohyoid neck dissection. The tongue defect was closed primarily. Results: Final pathology of the surgical specimen demonstrated myoepithelial cell carcinoma. All of the margins were free of tumor and no cervical lymph nodes showed metastasis. Immunohistochemistry demonstrated myoepithelial differentiation. The tumor did not show EWSR1 gene rearrangement on genetic testing, suggesting salivary gland origin. Multidisciplinary tumor board evaluation recommended no adjuvant therapy. The patient recovered well after surgery and nearly a year later is without evidence of recurrent or residual disease. Conclusions: We present the first reported case of myoepithelial cell carcinoma with primary origin in the oral tongue and review the available literature on this unusual tumor. We discuss the clinical, pathological, and immunohistochemical features and treatment of myoepithelial cell carcinoma.

Self-inflicted gunshot wounds (GSW) to the palate result in complex bony and soft tissue trauma to the mid and upper face. Patients who survive these injuries are faced with significant speech and feeding difficulties. Upper and midface fractures open reduction and internal fixation (ORIF) is required for many of these patients, and consideration to incision planning is critical in order to preserve a primary option for oroantral fistula repair. The temporoparietal fascia (TPF) flap is an excellent option for primary palate repair as it is often exposed in the operative field during facial fracture ORIF and can be readily used for this purpose if its blood supply and width is not inadvertently compromised while making a temporal incision. This flap is easy to elevate, does not require any microvascular expertise, and using the TPF to reconstruct the palate injury primarily may save the patient years of wearing an obturator and/or subsequent trips to the OR for operative fistula management. In contrast to the temporalis muscle flap, this flap does not create temporal hollowing after elevation, which is a significant aesthetic complaint among patients. Proper incision planning is critical to preserve this flap as an option for palate fistula repair as the fascial layer is often incised when making coronal incisions. Primary repair of palate injuries using the TPF flap at the same time as upper facial ORIF has very little morbidity in this setting, and greatly augments patients’ quality of life.

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Objective This investigation seeks to evaluate the effect of gross pathologic analysis on our management of patients undergoing routine tonsillectomy and to evaluate charges and reimbursement. Study Design Retrospective chart review from 2005 through 2016. Setting Academic medical center. Subjects and Methods Participants were pediatric patients aged 14 years and younger undergoing tonsillectomy for either sleep-disordered breathing or tonsillitis, with tonsillectomy specimens evaluated by pathology, and without any risk factors for pediatric malignancy. Records were reviewed for demographics, surgical indications, and pathology. Abnormal reports prompted an in-depth review of the chart. Charges and reimbursement related to both hospital and professional fees for gross tonsil analysis were evaluated. Results From 2005 to 2016, 3183 routine pediatric tonsillectomy cases were performed with corresponding specimens that were sent for gross analysis revealing no significant pathologic findings; 1841 were males and 1342 were females. Ten cases underwent microscopy by pathologist order, revealing normal tonsillar tissue. The mean charge per patient for gross analysis was $60.67 if tonsils were together as 1 specimen and $77.67 if tonsils were sent as 2 separate specimens; respective reimbursement amounts were $28.74 and $35.90. Conclusions Gross pathologic analysis did not change our management of routine pediatric tonsillectomy patients. Foregoing the practice at our institution would eliminate $19,171.72 to $24,543.72 in charges and $9081.40 to $11,344.40 in reimbursement per year. Eliminating this test would improve the value of patient care by saving health care resources without compromising clinical outcomes.