Low-Grade Fibromyxoid Sarcoma of the Back.

A 29-year-old male presented with a seven-year history of a slow-growing, painless, firm, mobile mass in the right upper back that was bothersome when supine or with direct pressure. On initial presentation, a clinical diagnosis of lipoma was given. The mass progressively increased in size over several years but remained painless. The mass measured 15 x 10 cm on examination. Excision of the lesion was performed, which revealed a white cut surface with cystic degenerative changes. Histologically, the lesion revealed spindle cell morphology with occasional mitosis. Diffuse immunohistochemical staining with MUC4 supports a diagnosis of low-grade fibromyxoid sarcoma (LGFMS). Tumor was present with focal extension into the deep margin. However, serial magnetic resonance imaging studies performed suggest no residual disease and negative regional lymph node involvement. This case demonstrates the growth pattern of LGFMS, but also denotes the importance of correlating radiological and pathological features to accurately diagnose and treat these tumors in a timely fashion.

Optimizing Patient Selection for Irreversible Electroporation of Locally Advanced Pancreatic Cancer: Analyses of Survival.

BACKGROUND: Irreversible electroporation (IRE) has emerged as a viable consolidative therapy after induction chemotherapy, in which this combination has improved overall survival of locally advanced pancreatic cancer (LAPC). Optimal timing and patient selection for irreversible electroporation remains a clinically unmet need. The aim of this study was to investigate preoperative factors that may assist in predicting progression-free and overall survival following IRE. METHODS: A multi-institutional, prospectively maintained database was reviewed for patients with LAPC treated with induction chemotherapy followed by open-technique irreversible electroporation from 7/2015-5/2019. RECIST 1.1 criteria were used to assess tumor response and radiological progression. Overall survival (OS) and progression-free survival (PFS) were recorded. Survival analyses were performed using Kaplan Meier and Cox multivariable regression analyses. RESULTS: 187 LAPC patients (median age 62 years range, 21 - 91, 65% men, 35% women) were treated with IRE. Median PFS was 21.7 months and median OS from diagnosis was 25.5 months. On multivariable analysis, age ≤ 61 (HR 0.41, 95%CI 0.21-0.78, p<0.008) and no prior radiation (HR 0.49, 95%CI 0.26-0.94, p=0.03) were positive predictors of OS after IRE. Age ≤ 61(HR 0.53, 95%CI, 0.28-.99, p=0.046) and FOLFIRINOX followed by gemcitabine/abraxane induction chemotherapy (HR 0.37,95%CI 0.15-0.89, p=0.027) predicted prolonged PFS after IRE. Abnormal CA19-9 values at the time of surgery negatively impacted both OS (HR 2.46, 95%CI 1.28-4.72, p<0.007) and PFS (HR 2.192, 95%CI 1.143-4.201, p=0.018) following IRE. CONCLUSIONS: Age, CA 19-9 response, avoidance of pre-IRE radiation, and FOLFIRINOX plus gemcitabine/abraxane induction chemotherapy are prominent factors to consider when referring or selecting LAPC patients to undergo IRE.

Paraneoplastic Limbic Encephalitis in a Patient with Primary Well-differentiated Teratoma and Metastatic Poorly Differentiated Embryonal Carcinoma.

Testicular tumors account for 1-2% of all tumors in men, with 95% of these being germ cell tumors. Paraneoplastic limbic encephalitis is a rare sequela of testicular tumors associated with anti-Ma2 and KLH11 antibodies. The most effective treatment for paraneoplastic limbic encephalitis is treatment of the primary malignancy. We report a 41-year-old male that presented to the emergency department with episodic alteration of consciousness and memory disturbances. Negative neurologic evaluation and imaging led to concern for a paraneoplastic process from a distant malignancy. CT imaging revealed an enlarged, necrotic para-aortic lymph node and subsequent ultrasound demonstrated a right-sided testicular mass. Right radical orchiectomy was performed. Microscopically, the mass consisted of mixed respiratory epithelium, gastrointestinal glands, and squamous epithelium with keratinization consistent with a post-pubertal testicular teratoma with associated in situ germ cell neoplasia. Resection of the para-aortic mass revealed large anaplastic cells with epithelioid features, nuclear pleomorphism and frequent mitoses. Immunostaining was positive for Pan-Keratin and OCT4, consistent with poorly differentiated embryonal carcinoma. Resection of the primary and metastatic disease, as well as treatment with corticosteroids, resulted in resolution of the encephalitis. This presentation of severe neurological disturbances in the setting of a metastatic mixed non-seminomatous germ cell tumor represents a rare presentation of paraneoplastic limbic encephalitis.

Atypical Spindle Cell Lipomatous Lesion Resected From Patient With History of CLL.

Atypical spindle cell lipomatous neoplasm, also known as well-differentiated spindle cell liposarcoma, represents a newly discovered entity of adipocytic tumors. Recent research has shown this tumor variant to be more related to spindle cell lipoma, rather than the originally hypothesized atypical lipomatous tumor spectrum. Here we present a case of a 58-year-old man with a history of chronic lymphocytic leukemia with an enlarging mass on the posterior left shoulder, initially hypothesized to be a benign lipoma. However, magnetic resonance imaging showed a large, multiseptated, heterogeneous mass concerning for soft tissue sarcoma. After resection, pathologic analysis showed cells closely resembling spindle cell lipoma, with additional cellular and fascicular zones containing lipoblasts and mitotic figures. Molecular analysis showed no MDM2 amplification. This lack of amplification indicates this tumor is distinctly different from an atypical lipomatous tumor, which characteristically displays MDM2 amplification. However, tumor expression of RB1 was normal. The majority of atypical spindle cell lipomatous neoplasms are associated with RB1 deletions. We conclude that we have a unique example of an atypical spindle cell lipomatous tumor.

A prospective, multi-institution assessment of irreversible electroporation for treatment of locally advanced pancreatic adenocarcinoma: initial outcomes from the AHPBA pancreatic registry.

BACKGROUND: The optimal treatment and management of locally advanced pancreatic cancer (LAPC) remains unclear and controversial. This study aimed to report the initial outcomes of the AHPBA Registry and evaluate the reproducibility of existing evidence that the addition of Irreversible Electroporation (IRE), a nonthermal ablative treatment, confers survival benefits beyond standard therapeutic options for patients with LAPC. METHODS: From December 2015 to October 2017, patients with LAPC were treated with open-technique IRE following the AHPBA Registry Protocols. Patient demographics, long-term outcomes, and adverse events were recorded. Survival analyses were performed using Kaplan-Meier (KM) curves for overall survival (OS), progression free survival (PFS) and time to progression (TTP). RESULTS: A total of 152 patients underwent successful IRE. Morbidity and mortality were 18% and 2% respectively, with 19 (13%) patients experiencing severe adverse events. Nine (6%) patients presented with local recurrence. Median TTP, PFS, and OS from diagnosis were 27.3 months, 22.8 months, and 30.7 months respectively. CONCLUSION: The combination of IRE with established multiagent therapy is safe and demonstrates encouraging survival among patients with LAPC. IRE is associated with a low rate of serious adverse events and has been optimized for more widespread adoption through the standardized protocols available through the AHPBA registry.

Clinical and epidemiological factors associated with suicide in colorectal cancer.

PURPOSE: While increased suicidal tendencies among cancer patients have been well documented, this study aims to examine suicide rates and factors associated with suicide specifically in patients with colorectal cancer (CRC). METHODS: Patients diagnosed with CRC between the years of 1988-2010 were selected from the Surveillance, Epidemiology, and End Result (SEER) database. Comparisons with the general population were done using the National Center for Disease Control registry. RESULTS: One thousand three hundred eighty-one suicides among 884,529 patients were identified, with a standardized mortality ratio (SMR) of 1.53 (95% CI, 1.13-1.33) compared to the general population. No statistically significant difference in suicide rate was found with respect to age, marital status, socio-economic status, surgical intervention, histologic subtype, or stage at diagnosis. Within the CRC population, Whites were significantly more likely to commit suicide than non-Whites (OR, 2.28; 95% CI, 1.89-2.75; P < 0.001), and males were significantly more likely than females (OR, 5.635; 95% CI, 4.85-6.54; P < 0.001). Most suicides occurred in patients with distal lesions in the sigmoid/rectosigmoid junction (P < 0.001). SMRs for CRC patients were 4.24 for females (95% CI, 3.69-4.86), 1.35 for males (95% CI, 1.28-1.43), 0.38 for African-Americans (95% CI, 0.28-0.52), 1.77 for Whites (95% CI, 1.68-1.87), and 0.90 for other races (95% CI, 0.72-1.12). CONCLUSION: Identification of risk factors associated with suicide among patients with CRC is an important step in developing screening strategies and management of psychosocial stressors. These results could be helpful in formulating a comprehensive suicide risk scoring system for screening all cancer patients.

Demographic and clinical factors associated with suicide in gastric cancer in the United States.

While increased suicidal tendencies among cancer patients have been well documented, there has been no specific examination of suicide and gastric cancer. The purpose of this study is to characterize suicide incidence among patients diagnosed with gastric cancer from 1973 to 2013 and identify variables associated with higher suicide rates. Patients with gastric cancer were identified in the Surveillance, Epidemiology, and End Results (SEER) database of the National Cancer Institute. The study included clinical and demographic data from 1973 to 2013. Standardized mortality ratios (SMRs) and 95% confidence intervals (95% CIs) were calculated. Comparisons with the general US population were based on mortality data collected by the Centers for Disease Control and Prevention's National Center for Injury Prevention and Control using the Web-based Injury Statistics Query and Reporting System. Multivariable logistic regression models generated odds ratios (ORs) to assess factors associated with increased suicide in gastric malignancy. There were 210 suicides for patients with gastric cancer (SMR, 3.21; 95% CI: 2.80-3.67). Female gender (SMR 8.54), White race (SMR 4.08), age ≤39 years (SMR 3.06), and age 70-79 years (SMR 2.90), were found to be significant for an increased incidence of suicide compared with the general population. There was not a statistically significant relationship between suicide and marital status, income, mode of radiation therapy, and the role of surgical intervention. Approximately 77% of deaths by suicide occurred within the first year following diagnosis. Female gender, White race, age ≤39 years, and age 70-79 years are factors associated with increased risk of suicide in patients with gastric cancer. These results, coupled with further studies and analyses, will be used to formulate a comprehensive suicide risk factor scoring system for screening all cancer patients.

Pancreatic neoplasms in pregnancy: diagnosis, complications, and management.

BACKGROUND: Neoplasms of the pancreas during pregnancy are rare, with less than 25 cases of benign and malignant tumors reported in the literature. METHODS: We present three unique cases of pancreatic tumors occurring during pregnancy--one mucinous cystic neoplasm and two adenocarcinomas. We review the literature regarding pancreatic neoplasms during pregnancy and discuss the diagnosis, complications, and management of these tumors. RESULTS: Magnetic resonance imaging and ultrasound are the imaging modalities of choice in pregnancy. In patients with benign or premalignant tumors, surgical resection may be postponed until the second trimester. In symptomatic patients, or if there is a concern for intrauterine growth restriction, urgent surgical intervention should be performed. With malignant tumors, the benefit of delaying surgery must be balanced with the risk of maternal disease progression. Termination of the pregnancy should be discussed when a malignant tumor is diagnosed during the first trimester. Pancreatic tumors diagnosed during the third trimester may be resected after delivery. If malignant, early delivery of the fetus and subsequent maternal operation can be considered at appropriate fetal maturity. CONCLUSION: When these tumors occur during pregnancy, they present a diagnostic and treatment dilemma, with variation in treatment based on gestational age and patient preference.