Comparing Multivisceral Resection with Tumor-only Resection of Liposarcoma Using the Win Ratio.

BACKGROUND: Multivisceral resection of retroperitoneal liposarcoma (LPS) is associated with increased morbidity and may not confer a survival benefit compared with tumor-only (TO) resection. We compared both approaches using a novel statistical method called the "win ratio" (WR). METHODS: Patients who underwent resection of LPS from 2004 to 2015 were identified from the National Cancer Database. Multivisceral resection was defined as removal of the primary site in addition to other organs. The WR was calculated based on a hierarchy of postoperative outcomes: 30-day and 90-day mortality, long-term survival, and severe complication. RESULTS: Among 958 patients (multivisceral 634, TO 324) who underwent resection, the median age was 63 years (interquartile range [IQR] 54-71) with a median follow-up of 51 months (IQR 30-86). There was no difference in the WR among patients who underwent TO versus multivisceral resection in the matched cohort (WR 0.82, 95% confidence interval [CI] 0.61-1.10). In patients aged 72-90 years, those who underwent multivisceral resection had 36% lower odds of winning compared with patients undergoing TO resection (WR 0.64, 95% CI 0.40-0.98). A subgroup analysis of patients classified as not having adjacent tumor involvement at the time of surgery revealed that those patients who underwent multivisceral resection had 33% lower odds of winning compared to TO resection (WR 0.67, 95% CI 0.45-0.99). CONCLUSIONS: Based on win-ratio assessments of a hierarchical composite endpoint, multivisceral resection in patients without adjacent tumor involvement may not confer improved outcomes. This method supports the rationale for less invasive resection of LPS in select patients, especially older patients.

Liver Transplant as a Treatment of Primary and Secondary Liver Neoplasms.

Importance: Liver malignancies are an increasing global health concern with a high mortality. We review outcomes following liver transplant for primary and secondary hepatic malignancies. Observations: Transplant may be a suitable treatment option for primary and secondary hepatic malignancies in well-selected patient populations. Conclusions and Relevance: Many patients with primary or secondary liver tumors are not eligible for liver resection because of advanced underlying liver disease or high tumor burden, precluding complete tumor clearance. Although liver transplant has been a long-standing treatment modality for patients with hepatocellular carcinoma, recently transplant has been considered for patients with other malignant diagnoses. In particular, while well-established for hepatocellular carcinoma and select patients with perihilar cholangiocarcinoma, transplant has been increasingly used to treat patients with intrahepatic cholangiocarcinoma, as well as metastatic disease from colorectal liver and neuroendocrine primary tumors. Because of the limited availability of grafts and the number of patients on the waiting list, optimal selection criteria must be further defined. The ethics of organ allocation to individuals who may benefit from prolonged survival after transplant yet have a high incidence of recurrence, as well as the role of living donation, need to be further discerned in the setting of transplant oncology.

Advances and considerations in the use of immunotherapies for primary hepato-biliary malignancies.

Hepatocellular carcinoma (HCC) and cholangiocarcinoma (CCA) comprise the two most common primary liver malignancies. Curative treatment options often include hepatectomy or liver transplantation; however, many patients present with advanced disease that is not amenable to surgical management. In turn, many patients are treated with systemic or targeted therapy. The tumor microenvironment (TME) is a complex network of immune cells and somatic cells, which can foster an environment for disease development and progression, as well as susceptibility and resistance to systemic therapeutic agents. In particular, the TME is comprised of both immune and non-immune cells. Immune cells such as T lymphocytes, natural killer (NK) cells, macrophages, and neutrophils reside in the TME and can affect tumorigenesis, disease progression, as well as response to therapy. Given the importance of the immune system, there are many emerging approaches for cancer immunotherapy. We herein provide a review the latest data on immunotherapy for primary HCC and BTC relative to the TME.

Dermatofibrosarcoma protuberans: a case report of an abdominal wall mass and review of the literature.

Dermatofibrosarcoma protuberans (DFSP) is a rare, slow-growing malignancy that often presents with an ambiguous clinical presentation due to its nonspecific findings. We present the case of a 41-year-old male who presented with a slow-growing mass on his abdomen that became protuberant and firm, prompting his desire to pursue excision. Upon return of histologic examination, the specimen was noted to be DFSP. DFSP is a rare cutaneous neoplasm that originates from the dermis and invades the underlying tissue creating a classic protuberant appearance. In some cases, chemotherapy and radiation may be indicated based on margins and locations. The sarcoma is notoriously complicated with early recurrence, making the disease process difficult to control. Surgeons should be familiar with this malignancy due to the indication of additional nonsurgical treatments and the necessity for long-term follow-up for surveillance of recurrence.

Locoregional Therapy for Intrahepatic Cholangiocarcinoma: The Role of Intra-Arterial Therapies.

Intrahepatic cholangiocarcinoma (ICC) is a rare disease with a rising incidence. While surgical resection is the only curative option, the disease process is often identified in advanced stages, as this malignancy often remains clinically silent in early development. Only one-third of patients are eligible for resection at the time of diagnosis. For patients who cannot undergo resection, intra-arterial therapies are reasonable palliative treatment options; in rare occasions, these may be bridging therapies, as well. The premise of bland embolization and most chemoembolization intra-arterial therapies is that the arterial supply of the tumor is occluded to induce tumor necrosis, while radioembolization utilizes the arterial flow of the tumor to deliver radiation therapy. In this review, we discuss the use of transarterial embolization, transarterial chemoembolization, and selective internal radiation therapy for the treatment of ICC. Phase III randomized controlled clinical trials are difficult to tailor to this extremely rare and aggressive disease, but ultimately, further investigation should be pursued to define the patient population that will derive the greatest benefit from each modality.

Mutational Landscape and Precision Medicine in Hepatocellular Carcinoma.

Hepatocellular carcinoma (HCC) is the fourth most common malignancy worldwide and exhibits a universal burden as the incidence of the disease continues to rise. In addition to curative-intent therapies such as liver resection and transplantation, locoregional and systemic therapy options also exist. However, existing treatments carry a dismal prognosis, often plagued with high recurrence and mortality. For this reason, understanding the tumor microenvironment and mutational pathophysiology has become the center of investigation for disease control. The use of precision medicine and genetic analysis can supplement current treatment modalities to promote individualized management of HCC. In the search for personalized medicine, tools such as next-generation sequencing have been used to identify unique tumor mutations and improve targeted therapies. Furthermore, investigations are underway for specific HCC biomarkers to augment the diagnosis of malignancy, the prediction of whether the tumor environment is amenable to available therapies, the surveillance of treatment response, the monitoring for disease recurrence, and even the identification of novel therapeutic opportunities. Understanding the mutational landscape and biomarkers of the disease is imperative for tailored management of the malignancy. In this review, we summarize the molecular targets of HCC and discuss the current role of precision medicine in the treatment of HCC.

Syringocystadenoma papilliferum: case series and review of the literature.

Syringocystadenoma papilliferum (SCAP) is a rare, hamartomatous tumor of the apocrine glands, which typically manifests in the head and neck region. We present a case of 60-year-old male with a several-year history of the lesion located on the abdominal wall and a second case of a 58-year-old male with a history of a slow-growing lesion located on the tragus. Despite varying presentations and locations, both patients were identified to have SCAP on pathological evaluation. Appropriate treatments of SCAP range from CO2 laser treatment to surgical excision; we recommend surgical excision due to the risk of malignant transformation.

Hamartomatous polyps: Diagnosis, surveillance, and management.

Hereditary polyposis syndrome can be divided into three categories: Ade-nomatous, serrated, and hamartomatous polyps. Hamartomatous polyps, malformations of normal tissue presenting in a disorganized manner, are characterized by an autosomal dominant inheritance pattern. These syndromes exhibit hamartomatous gastrointestinal polyps in conjunction to extra-intestinal manifestations, which require conscientious and diligent monitoring. Peutz-Jeghers syndrome, Cowden syndrome, and juvenile polyposis syndrome are the most common displays of hamartomatous polyposis syndrome (HPS). Diagnosis can be pursued with molecular testing and endoscopic sampling. Early identification of these autosomal dominant pathologies allows to optimize malignancy sur-veillance, which helps reduce morbidity and mortality in both the affected patient population as well as at-risk family members. Endoscopic surveillance is an important pillar of prognosis and monitoring, with many patients eventually requiring surgical intervention. In this review, we discuss the diagnosis, surveillance, and management of HPS.

Surgical Treatment of Distal Cholangiocarcinoma.

Distal cholangiocarcinoma (dCCA) is a rare malignancy arising from the epithelial cells of the distal biliary tract and has a poor prognosis. dCCA is often clinically silent and patients commonly present with locally advanced and/or distant disease. For patients identified with early stage, resectable disease, surgical resection with negative margins remains the only curative treatment strategy available. However, despite appropriate treatment and diligent surveillance, risk of recurrence remains high with nearly 50% of patients experiencing recurrence at 5 years subsequent to surgical resection; therefore, it is prudent to continue to optimize neoadjuvant and adjuvant therapies in order to reduce the risk of recurrence and improve overall survival. In this review, we discuss the clinical presentation, workup and surgical treatment of dCCA.

Adenoid Cystic Carcinoma of the Breast: A Case Report.

Adenoid cystic carcinoma (ACC), characterized by proliferating glands and stromal and basement membrane elements, is a tumor most commonly occurring in the salivary glands; very rarely does this tumor present as a primary tumor in the breast. We present the case of a 53-year-old female who presented with a breast mass noted to be concerning on imaging and confirmed to be ACC on biopsy. The mass was amenable to surgical excision with referrals to medical and radiation oncology. Due to the rarity of the disease, there is no consensus regarding the optimal treatment strategy for the pathology, including the use of chemotherapy and radiation. Long-term follow-up is, however, recommended in order to monitor for recurrence.

Flood syndrome: a rare case of spontaneous rupture of umbilical hernia in a cirrhotic patient.

Flood syndrome is a rare and potentially fatal complication of long-standing ascites resulting from a spontaneous rupture of an umbilical hernia. We present the case of a 67-year-old male with a medical history of diastolic heart failure, cocaine abuse, hepatitis C and cirrhosis secondary to daily alcohol consumption who presented to the emergency department with spontaneous rupture of an umbilical hernia with continuous, perfuse drainage of ascites from a 3 cm hernia defect. In this case report, we highlight the successful temporizing and definitive management of flood syndrome, as the patient presented to and was temporized a community hospital and was subsequently transferred to a tertiary care center for definitive management.

Blunt cerebrovascular injury of cervical and petrous ICA with stroke: A case study.

This case report documents treatment of a 32-year-old male patient after being extricated from an extensively damaged motor vehicle after a collision. On first presentation, the patient had a Glascow Coma Score of 14 and a pan-scan revealed a severely comminuted fracture of the proximal left femoral shaft, a fracture of the left patella, and multiple rib fractures. A neurological exam on a later evaluation showed global aphasia and sharp decline in movements of the right upper and lower extremities even with painful stimuli. A CT Angiography of the head and neck was obtained and revealed evidence of occlusion in the upper cervical segment and proximal petrous segment of the left internal carotid artery (ICA). Two 6x40mm precise stents were placed restoring normal intracranial flow to the patient's left ICA. The patient was discharged having sensation and motor function in all extremities and with resolved aphasia and no neurologic deficits.

A case of sporadic Peutz-Jeghers syndrome presenting as multiple intussusceptions.

Peutz-Jeghers syndrome (PJS) is an autosomal dominant mutation of the STK11/LKB1 gene on chromosome 19 often characterized by mucocutaneous pigmentation, hamartomatous polyps, anemia, gastrointestinal bleeding and intussusception. We present the case of a 21-year-old female with no pertinent family history who received the diagnosis of PJS after presenting to the hospital with two episodes intussusception. Patients with PJS have an increased lifetime risk of developing stomach, small bowel, colon, pancreatic, breast, cervical, uterus and testicular cancer requiring religious surveillance at an early age.

Case report-successful management of acute buried bumper syndrome.

Percutaneous gastrostomy (PEG) tube placement is often the preferred approach to addressing nutritional deficits in patients requiring long-term feeding access. Numerous major and minor complications may occur with PEG tube insertion; buried bumper syndrome is a rare, long-term outcome of PEG tube placement, comprising <2.4% of complications. We present the case of a 60-year-old female with laryngeal cancer whom developed acute buried bumper syndrome after PEG tube insertion which was managed successfully with surgical intervention.