Ciliated Hepatic Foregut Cyst: Definitive Diagnosis Is Critical to the Optimal Treatment Pathway.

Background. Ciliated hepatic foregut cyst (CHFC) is a rare, benign cyst of the liver, derived from the embryonic foregut epithelium. Although CHFCs are typically asymptomatic, some present with nonspecific abdominal symptoms. Imaging modalities alone are insufficient for diagnosis, with intrahepatic cholangiocarcinoma included in the differential due to nonspecific imaging features; definitive diagnosis relies on histologic confirmation. These lesions are often benign; however, larger lesions can have malignant transformation into squamous cell carcinoma (SCC), which carries a poor prognosis, thus making a definitive diagnosis, no matter what size, essential. Here, we present a case of CHFC as well as a comprehensive literature review. Given these data, we propose an algorithm for definitive diagnosis.

Obstructive sleep apnea and white matter hyperintensities: correlation or causation?

Obstructive sleep apnea (apnea) is thought to cause small vessel ischemic episodes in the brain from hypoxic events, postulated as white matter hyperintensities (hyperintensities) identified on MRI which are implicated in cognitive decline. This study sought to evaluate these correlations. A retrospective evaluation of adults who underwent polysomnography (4/1/2016 to 4/30/2017) and a brain MRI prior to apnea diagnosis or within a year post-diagnosis was completed. MRI visual evaluation of hyperintensities using Fazekas scores were collected blind to clinical data. Collated clinical/MRI data were stratified and analyzed using chi-square, fishers t-tests, ANOVA/ANCOVA and linear regression. Stratification by apnea category revealed no significant differences in any variables including hyperintensity measures (Fazekas p=0.1584; periventricular p=0.3238; deep p=0.4618; deep total p=0.1770). Stratification by Fazekas category, periventricular and deep hyperintensities revealed increasing prevalence with age (p=0.0001); however, apnea categories were not significantly associated (Fazekas p=0.1479; periventricular p=0.3188; deep p=0.4503), nor were any individual apnea indicators. Continuous apnea measurements werre not associated with any hyperintensity factor; total deep hyperintensities were not associated with any apnea factors. Continuous BMI was not found to be associated with any apnea or hyperintensity factors. Only hypertension was noted to be associated with Fazekas (p=0.0045), deep (p=0.0010) and total deep (p=0.0021) hyperintensities; however, hypertension was not associated with apnea category (p=0.3038) or any associated factors. These data suggest apneas alone from OSA are insufficient to cause WMH, but other factors appear to contribute to the complex development of small vessel ischemic injury associated with age and cognitive decline.

Community-Acquired, Extended-Spectrum ß-Lactamase-Producing and Extensively Drug-Resistant Escherichia coli in a 28-Year-Old Pyelonephritis Patient Lacking Risk Factors.

While Escherichia coli is a common cause of urinary tract infections and pyelonephritis, there are few documented cases of extended-spectrum ß-lactamase (ESBL)-producing and extensively drug-resistant (XDR) isolates from the community resulting in infection requiring hospitalization, especially in individuals lacking risk factors. In the United States, exposure to ESBL-producing E. coli is typically nosocomial, whereas patients from developing countries often encounter ESBL-producing E. coli in the community through the consumption of contaminated food or water. Considering the rarity at which XDR E. coli isolates are encountered, there is also a scarcity of literature describing the successful treatment of ESBL-producing XDR E. coli. Here we present a case of an otherwise healthy 28-year-old female delicatessen worker infected with ESBL-producing and XDR E. coli without recent travel, antibiotic use, or healthcare contact, who required admission to the intensive care unit (ICU) with pyelonephritis and septic shock. Treatment with intravenous meropenem through a peripherally inserted central catheter (PICC) line at home was curative and follow up thereafter unremarkable. Given the patient's lack of obvious exposure to and risk factors for an ESBL-producing XDR E. coli infection and the specific lack of risk factors for severe pyelonephritis requiring hospitalization, this case represents a unique addition to the literature and is of value to clinicians by describing successful treatment.

Glyphosate and Polyoxyethyleneamine Ingestion Leading to Renal, Hepatic, and Pulmonary Failure.

ABSTRACT: Glyphosate is an organophosphorus compound and the active ingredient in commonly used herbicides, whereas polyoxyethyleneamine (POEA) is a nonionic surfactant often coupled with glyphosate in these herbicides to increase their efficacy. Cases of glyphosate-POEA ingestion have shown a variety of outcomes, ranging from skin and mucosal surface irritation to death. Here, we report mortality after ingestion of at least 237 mL of an herbicide confirmed to contain both glyphosate and POEA. The decedent's electronic medical record indicates presentation to the emergency department shortly after ingestion and rapid decompensation, with death occurring on the fourth day of admission. The autopsy report showed extensive pulmonary edema and congestion with no alimentary tract abnormalities. Microscopically, airway inflammation, edema, and hemorrhage were shown as well as pericentral necrosis and macrovascular hepatic steatosis. This case is unusual for several reasons including the fatal outcome in a young 30-year-old patient, the large volume of the herbicide consumed, the associated large volume aspirated, and the lung pathology associated with exposure to glyphosate-POEA since inhalation, and in this case, aspiration is an uncommon route of glyphosate-POEA exposure. This report therefore offers rare respiratory tract pathological findings and the clinical course after aspiration of a large volume of glyphosate-POEA.

GIST identified during bariatric surgery: to treat or not to treat?

BACKGROUND: Gastrointestinal stromal tumors (GIST) are rare GI tumors that compose 1% of GI tumors. With the rise in obesity, bariatric surgery is becoming an increasingly common procedure and the incidental GISTs in this population have been noted more often than in the general population. OBJECTIVE: We evaluated and characterized the incidental GISTs in our bariatric surgical population. SETTING: The study was completed at a Metabolic and Bariatric Surgery Accreditation and Quality Improvement Program-accredited academic hospital system. METHODS: All GISTs identified during Roux-en-Y gastric bypass and laparoscopic sleeve gastrectomy between January 1, 2005 and December 31, 2016 were evaluated. Typical demographic, clinicopathologic, treatment, follow-up, and outcome data were recorded. RESULTS: Within the 2655 bariatric surgeries at our institution, 17 GISTs were identified (.64%). Mean age was 54 years; 94% of lesions were identified intraoperatively. Lesions were identified in the fundus (29.4%) or body (70.6%), were unifocal, and <1 cm; 94.1% of resections had clear margins. Histology revealed 88.2% spindle cell and 11.8% mixed histology with <5 mitoses/50 fields, portending a low malignancy potential. Follow-up included the bariatric surgeon and oncology consult; 17.6% were recommended by oncology for computed tomography surveillance. No recurrences were recorded. CONCLUSION: We present the largest cohort to date of incidental GISTs in a bariatric population. A diligent intraoperative examination of the serosa in the left-behind portion of the remnant in bypass and the discarded remnant in sleeves allows the bariatric surgeon the opportunity to leave the patient cancer-free after removal of incidental tumor.

Head and neck cancer PEG site metastases: Association with PEG placement method.

BACKGROUND: Many patients with head and neck cancer (HNC) will require feeding tube placement for nutritional support using percutaneous endoscopic gastrostomy (PEG) tube. Rarely, HNC metastases have been reported at the PEG site, a morbidity associated with a poor outcome. METHODS: Along with a case report, an evaluation of PEG placement methods with metastases from the literature was completed along with a statistical analysis of the literature to determine PEG site metastases and method of placement correlations. RESULTS: The incidence of PEG metastases in patients with HNC with the "pull" method is statistically identical to that of patients receiving any other method for PEG placement. CONCLUSIONS: When considering options for the placement of PEG tubes in patients with HNC, the "pull" method should not be considered as a technique which will put patients at risk for PEG site metastases more than any other method of placement.

Predictive genetic profiles for regional lymph node metastasis in primary cutaneous melanoma: a case-matched pilot study.

Melanoma confers an estimated lifetime risk of one in 50 for 2016. Clinicopathologic staging and sentinel lymph node biopsy (SLNB) have been the standard of care for T2 and T3 lesions. Molecular biomarkers identified in the primary lesion suggestive of metastatic potential may offer a more conclusive prognosis of these lesions. Our purpose was to investigate molecular mutations in primary melanoma that were predictive for micrometastasis as defined by a positive sentinel lymph node (SLN) in a case-controlled manner: nine patients with negative SLN and nine with positive SLN. The two cohorts were statistically identical as shown by a t-test for age (P=0.17), race (P=0.18), Breslow depth (P=0.14), Clark level (P=0.33), host response (P=0.17), ulceration (P=0.50), satellite nodules (P=0.17), lymphovascular invasion (P=0.50), and mitotic activity (P=0.09). While no single gene was significantly associated with SLN status, multivariate analysis using classification and regression tree assessment revealed two unique gene profiles that completely represented regional metastases in our cohort as defined by a positive SLN: PIK3CA (+) NRAS (-) and PIK3CA (-) ERBB4 (-) TP53 (+) SMAD4 (-). These profiles were identified in 89% of the patients with positive SLN; none of these profiles were identified in the SLN-negative cohort. We identified two unique gene profiles associated with positive SLN that do not overlap other studies and highlight the genetic complexity that portends the metastatic phenotype in cutaneous melanoma.

Primary undifferentiated sarcoma of the meninges: A case report and comprehensive review of the literature.

BACKGROUND AND IMPORTANCE: Sarcomas make up 1% of all cases of adult cancer, with 5-10% of those classified as undifferentiated pleomorphic sarcomas (UPS/PUS) and 0.1-4.3% primary intracranial sarcomas. Intracranial undifferentiated sarcoma is characterized by an earlier age of onset and generally poorer prognosis compared to extracranial undifferentiated sarcomas. Current therapies involve surgical excision with wide margins and radiotherapy, with minimal data available regarding the efficacy of chemotherapy. CASE DESCRIPTION: A 79-year-old man with a history of remote superficial bladder cancer presented with a large frontal scalp lesion. A biopsy was initially attempted by a dermatologist in the outpatient setting, but a follow-up CT scan revealed a skull-eroding, enhancing soft tissue lesion. Neurosurgical treatment revealed an undifferentiated sarcoma. The patient underwent adjuvant radiation therapy of 59.4 Gy fractionated over 45 days following surgery. Follow-up brain MRIs at 1-, 6-, 9-, 12-, 15-, 21-, and 27 months after surgery have not shown any indications of local recurrence or tumor metastasis. Despite the high propensity that undifferentiated sarcomas have for recurrence and metastasis and the patient's advanced age, this patient remains uniquely disease-free. CONCLUSION: We provide a description of an unusual case and comprehensive literature review of UPS to clarify the hallmarks of the disease, identify the difficulties in diagnosis, and provide a summary of therapies employed in the literature with their corresponding patient outcomes.

Mutational analysis of selected high-grade malignancies in a premenopausal gynecologic cancer population: a potential for targeted therapies?

Background: In 2017, there will be 107,000 cases of gynecologic cancer diagnosed in the US with an overall survival of around 70%-most occurring in post-menopausal individuals. In this study, we have examined a younger (≤ 40 years of age) subpopulation of these women with high grade/ high stage gynecologic malignancies, attempting to identify unique genetic abnormalities or combinations thereof through tissue block specimens. This information was then analyzed in light of known target therapies to see if genetic analysis in this setting would yield significant therapeutic advantage. Methods: We retrospectively evaluated patients with high grade/high stage gynecologic cancers (≤ 40 years of age), examined the presence and status of 400 oncogenes and tumors suppressor genes from Formalin-fixed, Paraffin-embedded (FFPE) tissue and functionally classified mutations by SIFT and Polyphen. Results: Twenty women were identified and stratified into positive and negative outcomes. No demographic, clinicopathologic or treatment factors were significant between these groups. Of the 400 genes evaluated, twelve mutations were significant between the groups, six with targeted therapies. Mutations associated with negative outcomes within histologies/locations were evaluated: ERBB3 in epithelial (ovarian), ALK/GPR124/KMT2D in neuroendocrine (ovarian/endometrial), ROS1/EGFR, ROS1/ERBB3/KMT2D/NIRK1 and GPR124 in sarcoma. All negative outcomes were void of mutations in APC/ABL2. A predictive model for negative outcomes in our cohort was developed from these data: AKAP9-/MBD1-/APC-/ABL2- with a mutation load of > 20.5. Conclusions: Unique multi-gene and mutational outcome correlations were identified in our cohort. Resulting complex mutational profiles in distinctly aggressive gynecologic cancers suggested potential for novel therapeutic treatment. Future larger scale studies will be needed to correlate the genotypic and phenotypic features identified here (AU)

The impact of the American College of Surgeons Oncology Group (ACOSOG) Z0011 trial: An institutional review.

BACKGROUND: Axillary dissection (AD) was historically recommended for all patients with breast tumor involvement discovered by sentinel lymph node biopsy (+SLNB). However, after the ACOSOG Z0011 trial, omission of AD became the recommendation for selected patients with a +SLNB. We report the impact of ACOSOG Z0011 on the completion AD rate in patients with +SLNB at our institution. METHODS: We retrospectively reviewed all patients diagnosed with breast cancer between March 2009 and February 2013 (n = 1781). This cohort was divided into two groups: 1) those diagnosed BEFORE Z0011 and 2) those diagnosed AFTER Z0011. We calculated both the percentage of patients with a +SNLB who underwent AD and, from those patients, the percentage who did and did not meet the Z0011 criteria. RESULTS: The BEFORE group contained 849 patients; 144 had +SLNB and from those 113 underwent AD. The AFTER group contained 932 patients: 139 had +SLNB and from those 73 underwent AD. The completion AD rate in the BEFORE group was 78.5%, compared to 52.5% in the AFTER group (p < 0.001). From the patients who met the Z0011 criteria, 75.6% of the BEFORE patients underwent AD, compared to only 2.2%% in the AFTER group (p < 0.001). Among those who did not meet the Z0011 criteria, a similar percentage of patients underwent AD in each group (BEFORE 79.8%, AFTER 74.4%, p = 0.384). CONCLUSION: Following the publication of the ACOSOG Z0011 trial, we experienced a significant decrease in the completion AD rate among patients with a +SLNB who met the Z0011 inclusion criteria.