Endocrine mucin-producing sweat gland carcinoma: a systematic review and meta-analysis.

BACKGROUND: Endocrine mucin-producing sweat gland carcinoma is a rare, under-reported cutaneous adnexal tumor that is often misdiagnosed and has an unknown incidence of metastasis. OBJECTIVE: To determine the incidence of metastasis and tumor recurrence, as well as diagnostic accuracy and current trends in treatment modality. METHODS: A search was performed following the Preferred Reporting Items for Systematic Reviews and Meta-Analyses guidelines. Tumor pathology and clinical data concerning demographics, presentation, diagnosis, treatment and follow-up were assessed. RESULTS: A total of 36 publications with 110 cases were identified. Initial pathological diagnosis was incorrect in 45.5% of cases. One case of metastatic disease was reported. The incidence of locoregional recurrence was 10.6% over a mean follow-up period of 21.3 months. Of cases with known methods of resection, 34.6% were resected by excisional biopsy, 42.8% were resected by wide surgical excision, and 31.3% were cleared by Mohs micrographic surgery. LIMITATIONS: The low reported incidence and level of evidence was suboptimal with only case reports and retrospective case studies being reported. CONCLUSION: Reported cases of this pathology demonstrate poor diagnostic accuracy. High rates of misdiagnosis and inadequate definitive treatment suggest the need for more comprehensive work-up and management of lesions suspicious for this pathology.

Endoscopic repair of spontaneous skull base defects decreases the incidence rate of intracranial complications.

BACKGROUND: Spontaneous skull base defects can result in life-threatening intracranial complications (ICCs), including meningitis and pneumocephalus. Endoscopic skull base reconstruction (ESBR) has traditionally been the treatment of choice, but its impact upon ICCs is not known. In this study, we aimed to describe the incidence rate of preoperative ICCs in patients with spontaneous skull base defects, risk factors associated with ICC development, and the impact of surgical repair on the incidence rate of ICCs. METHODS: A retrospective review was performed of all spontaneous skull base defects undergoing ESBR from 2005 to 2019 at 2 academic tertiary care medical centers. The incidence rate of ICCs and the demographics information and risk factors were collected. RESULTS: In 222 spontaneous skull base defects, preoperative ICCs occurred in 46 subjects (20.7%) with an incidence rate of 22.7 per 100 person-years. Factors significantly associated with preoperative ICCs included symptom duration, reduced body mass index (BMI), resolved cerebrospinal fluid rhinorrhea, and location in the frontal or lateral sphenoid sinuses. Endoscopic repair was successful in 97.2% of subjects and the postoperative ICC incidence rate was significantly reduced at 0.8 per 100 person-years (p < 0.001). CONCLUSION: Spontaneous skull base defects pose significant risk for life-threatening ICCs. Our findings reveal significantly elevated odds of ICC development associated with resolved CSF rhinorrhea, lower BMI, longer duration of symptoms, and defect location. Endoscopic repair is highly successful with low morbidity and significantly reduces the incidence rate of intracranial complications.

Treatment Outcomes of Bilateral Medialization Thyroplasty for Presbylaryngis.

BACKGROUND: Presbylaryngis is a common cause of dysphonia in elderly patients. Type I thyroplasty serves to improve glottic closure and vocal quality by correcting bowing. Although unilateral and injection-based procedures are well-characterized in the treatment of broadly defined glottic insufficiency, there are insufficient outcomes data for bilateral medialization thyroplasty in the treatment of presbylaryngis. The aim of this study was to review the change in measures of vocal quality before and after bilateral medialization thyroplasty for presbylaryngis. STUDY DESIGN: This is a retrospective case series. METHODS: The records of 21 patients with presbylaryngis undergoing bilateral medialization thyroplasty between 2007 and 2014 were reviewed. Implant materials included silastic (n = 17) and hydroxyapatite (n = 4). Preoperative and postoperative comparison of vocal function was conducted using Voice Handicap Index, maximum phonation time, auditory-perceptual severity ratings, and blinded paired-comparison of Consensus Auditory-Perceptual Evaluation of Voice and Visual-perceptual stroboscopic ratings. Paired sample t tests were used to assess all outcome measures. RESULTS: Significant improvements were found in Voice Handicap Index scores (P < 0.007), maximum phonation time (P < 0.03), Consensus Auditory-Perceptual Evaluation of Voice (P < 0.04), and clinician rating of vocal quality (P < 0.0001). Blinded raters noted a significant improvement in audio (P < 0.05) and videostroboscopic (P < 0.003) samples after surgery. There were no operative complications observed, and median hospital stay was one night. CONCLUSIONS: Patients with presbylaryngis demonstrated significant improvement in both objective and subjective measures of vocal quality following bilateral medialization thyroplasty. These data suggest that medialization thyroplasty is a safe option that warrants consideration in the treatment of presbylaryngis.

Secondary free-flap reconstruction following ablation for acute invasive fungal sinusitis.

OBJECTIVE: Acute invasive fungal sinusitis (AIFS) is a frequently fatal infection for which extensive and debilitating surgical debridement is a mainstay of therapy. Resulting defects are often composite in nature, mandating free tissue-transfer reconstruction. Outcomes data for free flap reconstruction are limited. The purpose of this study was to examine surgical outcomes and survival in patients undergoing free flap transfer following invasive fungal sinusitis. STUDY DESIGN: Retrospective case series. METHODS: Between 1995 and 2015, patients undergoing operative debridement for AIFS were identified. Surgical records were used to identify survivors of acute infection who subsequently underwent free flap reconstructive surgery. Patient demographics, cause of immune compromise, defect description, flap type, perioperative complications, indications for revision surgery, functional outcomes, and long-term survival were reviewed. RESULTS: Forty-four patients were treated for AIFS, of those, 30 (68%) survived acute infection. Ten patients underwent maxillectomy, six with orbital exenteration, and were designated candidates for reconstruction. Eight patients underwent reconstruction. Median time from debridement to reconstruction was 67.5 days. Flap types included latissimus dorsi, scapula, anterolateral thigh, rectus, radial forearm, and fibula. Median follow-up was 7.7 months. No perioperative complications were encountered, and all subjects remained disease-free, able to speak and eat normally without prosthetic supplementation. Seven patients (87%) are currently alive. CONCLUSION: Reconstruction of defects left by invasive fungal sinusitis using free-tissue transfer resulted in successful flap survival, with no disease recurrence for all defects and flap types reviewed. Survivors of AIFS are able to tolerate midface reconstruction, with favorable functional outcomes and survival rates. LEVEL OF EVIDENCE: 4. Laryngoscope, 127:815-819, 2017.

High-grade malignant transformation of a radiation-naïve nasopharyngeal angiofibroma.

BACKGROUND: Nasopharyngeal angiofibromas are typically considered benign vascular neoplasms, with descriptions of high-grade sarcomatous change found only in lesions with prior radiotherapy. METHODS AND RESULTS: We describe the first reported case of high-grade malignant change in a nasopharyngeal angiofibroma naive to radiation. A 45-year-old man presented with left-sided nasal congestion and fullness and was found to have a left-sided nasopharyngeal mass with intracranial extension on CT scan. A biopsy of the mass revealed nasopharyngeal angiofibroma. The patient opted for MRI surveillance, which revealed interval growth 3 years later. Decompression surgery revealed only angiofibroma, but resection 9 months later demonstrated high-grade sarcoma and concomitant angiofibroma. The patient had residual disease which progressed through chemoradiation, and is now pursuing clinical trial enrollment. CONCLUSION: Malignant transformation of nasopharyngeal angiofibroma is extremely rare. As highlighted by this report, high-grade undifferentiated lesions may arise in tumors without previous radiation. © 2016 Wiley Periodicals, Inc. Head Neck 38: E2425-E2427, 2016.