Biphenotypic Sinonasal Sarcoma: A Case Report and Review of Literature.

OBJECTIVES: Biphenotypic sinonasal sarcoma (BSNS), previously low-grade sinonasal sarcoma with neural and myogenic features, is a rare tumor of the sinonasal tract first described in 2012. Due to its rarity, limited literature is available in providing clinicians with a standardized treatment regimen, particularly in cases of positive surgical margins. This article aims to provide a clinical review of the currently available reported cases of BSNS, as well as presenting clinical, radiologic, and pathologic details of 2 novel cases. METHODS: Online electronic databases include PubMed and Embase where queried for reports of biphenotypic sinonasal sarcoma or low-grade sinonasal sarcoma with neural and myogenic features. Two previously unpublished cases were included in the results. Data including clinical presentation, epidemiologic data, radiologic evaluation, intraoperative details, histopathology, treatment modality, and postoperative follow-up information were included. RESULTS: A total of 100 previously published cases were identified in 12 prior articles. Mean age at presentation was 52.9 years. Extrasinonasal extension was observed in 27.4% of cases with most common site of extension being cribriform plate. Forty-seven cases included treatment details with surgical excision being the most common modality. Recurrence rates were identical for both surgical excision alone and surgical excision with adjuvant radiotherapy (33.3%). CONCLUSIONS: Biphenotypic sinonasal sarcoma is a slow-growing tumor that is amenable to surgical resection. Recurrence rates are similar between surgical excision and surgical excision with adjuvant radiation therapy, but limited data in reported cases preclude a determination of treatment superiority.

Esophageal perforation as a complication of the Heimlich maneuver in a pediatric patient: A case report.

We report a case of cervical esophageal perforation caused by the Heimlich maneuver in a healthy 16-year-old boy. The patient reported a short coughing episode while eating rice, and his mother performed the Heimlich maneuver on him. Five days later, he presented to the emergency department with throat pain, odynophagia, secretion intolerance, muffled voice, and neck stiffness. He was admitted to the pediatric intensive care unit for conservative management. The next day he underwent transcervical incision and drainage of purulence, but the esophageal perforation could not be visualized at that time. The perforation was identified several days later and successfully repaired surgically. Esophageal perforation as a complication of the Heimlich maneuver is exceedingly rare, but the clinician should be aware of this entity in the differential diagnosis, as it is associated with a high mortality rate and warrants multidisciplinary care, including timely surgical intervention.

Systematic review and case report: Intracranial complications of pediatric sinusitis.

CONTEXT: Intracranial complications of rhinosinusitis are rare in the post-antibiotic era. However, due to potentially devastating outcomes, prompt recognition and management are essential. OBJECTIVE: This study aims to perform the first systematic review of the intracranial complications of rhinosinusitis in order to better characterize their clinical presentation, diagnosis, and treatment, and report a case of frontal lobe empyema secondary to pediatric frontoethmoid sinusitis. DATA SOURCES: Ovid MEDLINE, Cochrane Library, and Google Scholar. STUDY SELECTION: Full-text, peer-reviewed journal publications from 1947 to January 1, 2015 in English; focus on intracranial complications of sinusitis; pediatric patients (<18 years of age); studies including data on diagnostic workup and treatment. DATA EXTRACTION: Preferred Reporting Items for Systematic Reviews and Meta-Analyses guidelines. RESULTS: Sixteen studies involving 180 patients were included. An overwhelming majority of patients were young adolescent males (70%). The most common intracranial complications were subdural empyema (49%), epidural abscess (36%), cerebral abscess (21%), and meningitis (10%). Patients most often presented with nonspecific symptoms such as headache, fever, nausea and vomiting. Computed tomography with contrast or magnetic resonance imaging confirmed the diagnosis when intracranial complications were suspected. Typical treatment included surgical incision and drainage, often involving joint neurosurgical and otolaryngological procedures, combined with a long course of antibiotics. The morbidity rate was 27%, and the mortality rate was 3.3%. LIMITATIONS: All studies were retrospective chart reviews, case series or case reports. CONCLUSIONS: A review of the currently available literature shows that with a high degree of suspicion, multidisciplinary cooperation and aggressive treatment, favorable outcomes are attainable. The most effective surgical treatment for intracranial complications remains unclear and should be investigated further.

Suprasellar pediatric craniopharyngioma resection via endonasal endoscopic approach.

PURPOSE: Purely endoscopic endonasal approaches to surgical resection of pediatric suprasellar craniopharyngiomas are uncommonly performed. The aim of the study is to assess the feasibility and to describe the short-term outcomes of endonasal endoscopic approaches for the gross total resection of suprasellar craniopharyngiomas in the pediatric population. METHODS: A combined neurosurgical-otolaryngologic team performed gross total resection of craniopharyngiomas in seven pediatric patients (mean age 9.6 years) at The Children's Hospital of Philadelphia over 2011-2012. Short-term outcomes were analyzed over a mean follow-up period of 6.3 months. RESULTS: All tumors involved the sellar and/or suprasellar space and contained some cystic component. The mean maximal tumor diameter was 31.5 mm (range 18.5-62.0 mm). Using a binostril approach, gross total tumor resection was obtained in all patients (100 %). All patients with preoperative visual dysfunction demonstrated improvement in visual acuity. New or stable panhypopituitarism was observed in all cases. All patients developed postoperative diabetes insipidus, and cerebrospinal fluid leak occurred in one patient (15 %). CONCLUSIONS: Complete radiographic resection of pediatric craniopharyngioma can be achieved via a purely endoscopic endonasal approach. In particular, this approach can be performed safely using the "two-nostrils-four-hands" technique with intraoperative neuronavigation. This approach should be highly considered in patients with progressive visual dysfunction. Further studies are needed to characterize the long-term surgical and clinical outcome of pediatric patients treated with this surgical approach.

Complex reconstruction of the sella using absorbable mini-plate in revision endoscopic pituitary surgery: technical note.

BACKGROUND: Skull base reconstruction in the setting of revision endoscopic pituitary surgery with intraoperative cerebrospinal fluid (CSF) leak represents a unique challenge. MATERIALS AND METHODS: The demographics and outcomes of four patients undergoing revision endoscopic pituitary surgery with high-volume intraoperative CSF leak are described. The "gasket-seal" technique of sellar repair using bioabsorbable mini-plate is described. RESULTS: The indications for surgery were macroadenoma with suprasellar extension and optic nerve compression in three patients and growth hormone-secreting tumor in one patient with acromegaly. The multilayered reconstruction consisted of autologous fat placed in the tumor cavity followed by reconstruction of the sellar floor with an oversized sheet of either fascia lata or acellular dermal matrix placed over the skull base defect and sunken into the sella with a bioabsorbable mini-plate. One patient experienced postoperative CSF leak on postoperative day 2 that resolved with lumbar drainage alone. All patients were noted to have a well mucosalized sphenoid sinus and were free of CSF leak at last follow-up. CONCLUSION: Sellar reconstruction following endoscopic pituitary surgery represents a technical challenge, especially in revision cases with high-volume intraoperative CSF leak. The early experience with the "gasket-seal" closure using the bioabsorbable mini-plate appears favorable. Lumbar drain may be indicated in patients with postoperative CSF leak.

Molecular target based combinational therapeutic approaches in thyroid cancer.

BACKGROUND: Thyroid cancer, as with other types of cancer, is dependent on angiogenesis for its continued growth and development. Interestingly, estrogen has been shown to contribute to thyroid cancer aggressiveness in vitro, which is in full support of the observed increased incidence of thyroid cancer in women over men. Provided that estrogen has been observed to contribute to increased angiogenesis of estrogen responsive breast cancer, it is conceivable to speculate that estrogen also contributes to angiogenesis of estrogen responsive thyroid cancer. METHODS: In this study, three human thyroid cancer cells (B-CPAP, CGTH-W-1, ML-1) were treated with estrogen alone or estrogen and anti-estrogens (fulvestrant and 3,3'-diindolylmethane, a natural dietary compound) for 24 hours. The cell culture media was then added to human umbilical vein endothelial cell (HUVECs) and assayed for angiogenesis associated events. Vascular endothelial growth factor (VEGF) levels were also quantified in the conditioned media so as to evaluate if it is a key player involved in these observations. RESULTS: Conditioned medium from estrogen treated thyroid cancer cells enhanced phenotypical changes (proliferation, migration and tubulogenesis) of endothelial cells typically observed during angiogenesis. These phenotypic changes observed in HUVECs were determined to be modulated by estrogen induced secretion of VEGF by the cancer cells. Lastly, we show that VEGF secretion was inhibited by the anti-estrogens, fulvestrant and 3,3'-diindolylmethane, which resulted in diminished angiogenesis associated events in HUVECs. CONCLUSION: Our data establishes estrogen as being a key regulator of VEGF secretion/expression in thyroid cells which enhances the process of angiogenesis in thyroid cancer. These findings also suggest the clinical utility of anti-estrogens as anti-angiogenic compounds to be used as a therapeutic means to treat thyroid cancer. We also observed that 3,3'-diindolylmethane is a promising naturally occurring anti-estrogen which can be used as a part of therapeutic regimen to treat thyroid cancer.

Surgical management of pneumosinus dilatans frontalis in the setting of chronic rhinosinusitis and type III frontal Cell.

Pneumosinus dilatans (PD) represents a rare lesion of unknown etiology in which progressive enlargement of an air-filled paranasal sinus results in cosmetic and functional complications. The absence of pathologic mucosa has traditionally been considered a hallmark of this disorder. A 24-year-old man presented with 2 years of chronic rhinosinusitis and recurrent, acute episodes of severe forehead pain with worsening outward prominence of the frontal sinus during air travel. Computed tomography and findings at surgery were notable for diffuse nonpolypoid inflammatory changes of the paranasal sinuses, a type III frontal sinus cell, and PD of the frontal sinus with severe deformity and thinning of the anterior table. A bicoronal approach was used for frontal sinus obliteration and cranioplasty. Excellent cosmetic and functional results were noted at the last follow-up. To our knowledge, this is the first report of PD frontalis presenting in the setting of chronic rhinosinusitis. Surgical management of this disorder requires consideration of both the functional and cosmetic issues.

Chronic foreign body of the nasal cavity and sphenoid sinus: surgical implications.

Chronic sinonasal foreign bodies present unique surgical challenges including tissue integration and anatomic migration. A patient with a history of multiple surgeries for cleft lip, palate, and nasal deformity presented with radiographic findings of a 4.3-cm linear foreign body traversing the nasal cavity and sphenoid sinus. Mucosalization and integration into the sphenoid rostrum were noted at the time of surgery. Endoscopic surgery techniques facilitated removal of the overlying mucosa and encasing bone, allowing successful surgical extraction. On inspection, the foreign body was consistent with a retained Kirschner wire. Potential pathophysiologic and management implications of this case are discussed.

Estrogen-mediated angiogenesis in thyroid tumor microenvironment is mediated through VEGF signaling pathways.

OBJECTIVES: To explore the induction of a proangiogenic phenotype in endothelial cells in the thyroid tumor microenvironment by estrogen-treated thyroid cancer cells and to define the role of vascular endothelial growth factor (VEGF) in this interaction. DESIGN: Cell-based in vitro systems analysis. SUBJECTS: Thyroid tumor cell lines (BCPAP [papillary thyroid cancer] and ML-1 [follicular thyroid cancer]) were cultured with estradiol with and without an estrogen receptor (ER) inhibitor (fulvestrant or ICI) and used to treat human umbilical vein endothelial cells (HUVECs). INTERVENTIONS: Immunofluorescence was used to confirm the presence of ERα and ERß in BCPAP cells. Conditioned medium was then used to evaluate the induction of HUVEC tubulogenesis and migration. Secretion of VEGF in this medium was evaluated by Western blot analysis. The expression of phosphoinositide 3-kinase (PI3K), the initiator of a proangiogenic pathway, was evaluated with Western blot analysis of HUVEC lysates. The subsequent effects of an ER inhibitor (fulvestrant/ICI) and a neutralizing VEGF antibody were also observed. RESULTS: Estrogen receptor α and ERß are expressed in thyroid cancer cells. Estrogen-stimulated ML-1 cells secreted an increased amount of VEGF likely as a result of ER signaling. In contact with this environment, HUVECs demonstrate enhanced tubulogenesis and migration. Western blot analysis documented estrogen-mediated upregulation of PI3K in HUVECs. These effects were mitigated by an ER inhibitor (fulvestrant/ICI) and a neutralizing VEGF antibody. CONCLUSIONS: Our data provide evidence that estrogen can induce a proangiogenic endothelial cell phenotype in the thyroid tumor microenvironment through ER and VEGF signaling. Our findings suggest that the effect of antiestrogenic therapy targeting tumor angiogenesis can be enhanced through VEGF inhibition.

Unusual electroacoustic device failure and electroacoustic reimplantation with hearing preservation.

OBJECTIVE: To document the preservation of hearing after electroacoustic (EAS) cochlear reimplantation and to report on a method of managing recurrent air accumulation under the posterior skin flap. STUDY DESIGN: Retrospective case report. PATIENTS: A single patient report. INTERVENTION: After successful EAS cochlear implantation with hearing preservation, patient developed recurrent air accumulation under his posterior flap and a subsequent device failure. The patient was reimplanted using EAS protocol and a myringotomy tube used to prevent reaccumulation of air. MAIN OUTCOME MEASURE: Preservation of hearing. RESULTS: Hearing was preserved after reimplantation, and air accumulation was avoided. CONCLUSION: Hearing can be preserved in EAS reimplantation using EAS surgical techniques. Air accumulation can be avoided or treated with a myringotomy tube.

Impact of diabetes on burn injury: preliminary results from prospective study.

Reducing diabetes mellitus complications has been a major focus for Healthy People 2010. A prior retrospective cohort of our burn center's admissions revealed worse outcomes among diabetic patients, that is, increased infection rates, grafting and graft complications, and increased length of hospital stay. Therefore, a prospective study has been designed to carefully assess wound repair and recovery of diabetic and nondiabetic burn patients. Our long-term aim is to determine the characteristics of the wound milieu along with global responses to injury that may predict poor outcome among diabetic patients. This is an initial phase of a larger observational study of in-hospital diabetic (types 1 and 2) and nondiabetic patients, prospectively matched for age (18-70 and >70 years) and burn size (<5, 5-15, and 16-25%). Time (days) to complete index wound closure, documented through serial photography, is the main outcome measure. Secondary measures compare delays in presentation, prevalence of infections, graft rates, wound and graft complications, adverse events, and length of hospital stay. Detailed history, physical, and baseline hemoglobin A1C are elicited from all subjects who are assessed daily over the initial 72 hours poststudy entry, then weekly until complete index wound closure, and finally monthly through 3 months. Forty subjects are presented herein, 24 diabetic and 16 nondiabetic patients. Time to index wound closure was significantly prolonged in diabetic patients, despite increased grafting. These findings suggest that excision and grafting in diabetic patients may not alone be sufficient to ensure rapid closure, as graft complications may contribute to protracted closure. Evaluating graft need may be more complex among diabetic patients, suggesting the need for alternative management strategies. The current prospective study confirms our previous retrospective analysis, notably manifested by significant delays in index wound closure. Our efforts continue in identifying the most important predictors of outcome, especially modifiable factors that would create a basis of intervention to improve care.