Multi-Phenotypic Breast Cancer Post-Radiotherapy for Hodgkin Lymphoma: A Case of Secondary Malignancy.

Morbidity and mortality associated with radiation-induced secondary malignancies (RISMs) have shifted treatment paradigms to minimize or eliminate radiation from treatment regimens. In this case, a 48-year-old woman was diagnosed with Hodgkin lymphoma (HL) and treated with radiotherapy in 2000. In 2018, she was diagnosed with ductal carcinoma in situ (DCIS) of the right breast and treated with a mastectomy. Soon after, she developed triple-negative invasive ductal carcinoma (IDC) in her reconstructed breast. The patient underwent a left lumpectomy, and pathology showed ER-/PR-/HER2+ IDC. This patient's multi-phenotypic DCIS and IDC presentation are suspected to be RISM due to her previous HL treatment regimen.

Blurry Vision as a Presentation of Waldenström's Macroglobulinemia: A Case Report With Review of Current Management.

A patient was diagnosed with Waldenström's macroglobulinemia (WM) after the initial findings of anemia and ophthalmological findings of retinal hemorrhage. Upon further workup, the patient was found to have an IgM predominant monoclonal gammopathy on serum protein electrophoresis (SPEP) and urine protein electrophoresis (UPEP). This highlights the need for open communication between different specialties, streamlining rapid and accurate diagnosis. Also highlighted are the unique pathophysiological changes involved in the development of WM. A patient's primary complaint was blurry vision. After the patient was noted to have a monoclonal gammopathy on SPEP, bone marrow biopsy was performed. The bone marrow biopsy findings were consistent with lymphoplasmacytic lymphoma (LPL). The patient received plasmapheresis and chemotherapy. The disease course is described. The patient saw rapid improvement in all lab abnormalities after the beginning of the appropriate therapy of plasmapheresis and chemotherapy. Remission is common with WM. Regular follow-up with this patient is important.

Metastatic Papillary Thyroid Cancer to the Liver: The Central Role of a Multidisciplinary Approach to Treatment.

Background: Differentiated thyroid cancer (DTC) is comprised of papillary and follicular subtypes, and both have an overall excellent long-term prognosis. Patients with localized DTC that is successfully treated, usually with surgery, exhibit long-term survival well above 90%. In contrast, patients who develop distant metastatic disease have a significantly worse overall prognosis and outcome, often with disease that is refractory to conventional therapy such as surgery, radioactive iodine, and hormone suppression. For patients who recur with distant metastatic disease, limited effective treatment options are available, and most die of their disease within 5 years of recurrence. Case Report: We report the case of a 26-year-old female who presented with recurrent papillary thyroid cancer and a metastatic lesion isolated to the liver. Because of the extremely large size of the metastatic liver mass upon initial presentation, we took a neoadjuvant, multifaceted approach to treatment that included selective internal radioembolization therapy, an oral multikinase inhibitor, and surgical resection of the tumor mass after maximal reduction in tumor size. However, the patient died of metastatic DTC after 39 months of treatment. Conclusion: A multimodal, comprehensive approach to managing such complex patients is essential to optimize both the sequence and therapeutic approach to treatment.

Three-year survival, correlates and salvage therapies in patients receiving first-line pembrolizumab for advanced Merkel cell carcinoma.

BACKGROUND: Merkel cell carcinoma (MCC) is an aggressive skin cancer associated with poor survival. Programmed cell death-1 (PD-1) pathway inhibitors have shown high rates of durable tumor regression compared with chemotherapy for MCC. The current study was undertaken to assess baseline and on-treatment factors associated with MCC regression and 3-year survival, and to explore the effects of salvage therapies in patients experiencing initial non-response or tumor progression after response or stable disease following first-line pembrolizumab therapy on Cancer Immunotherapy Trials Network-09/KEYNOTE-017. METHODS: In this multicenter phase II trial, 50 patients with advanced unresectable MCC received pembrolizumab 2 mg/kg every 3 weeks for ≤2 years. Patients were followed for a median of 31.8 months. RESULTS: Overall response rate to pembrolizumab was 58% (complete response 30%+partial response 28%; 95% CI 43.2 to 71.8). Among 29 responders, the median response duration was not reached (NR) at 3 years (range 1.0+ to 51.8+ months). Median progression-free survival (PFS) was 16.8 months (95% CI 4.6 to 43.4) and the 3-year PFS was 39.1%. Median OS was NR; the 3-year OS was 59.4% for all patients and 89.5% for responders. Baseline Eastern Cooperative Oncology Group performance status of 0, greater per cent tumor reduction, completion of 2 years of treatment and low neutrophil-to-lymphocyte ratio were associated with response and longer survival. Among patients with initial disease progression or those who developed progression after response or stable disease, some had extended survival with subsequent treatments including chemotherapies and immunotherapies. CONCLUSIONS: This study represents the longest available follow-up from any first-line anti-programmed death-(ligand) 1 (anti-PD-(L)1) therapy in MCC, confirming durable PFS and OS in a proportion of patients. After initial tumor progression or relapse following response, some patients receiving salvage therapies survived. Improving the management of anti-PD-(L)1-refractory MCC remains a challenge and a high priority. TRIAL REGISTRATION NUMBER: NCT02267603.

Corrigendum to "Pancreatic acinar cell carcinoma-literature review and case report of a 56-year-old man presenting with abdominal pain" [Radiol Case Rep 15 (2020) 39-43].

[This corrects the article DOI: 10.1016/j.radcr.2019.10.009.].

Pancreatic acinar cell carcinoma--literature review and case report of a 56-year-old man presenting with abdominal pain.

We present a case of acinar cell carcinoma of the pancreas (ACC) with metastasis to the liver in a patient who presented with complaints abdominal pain. The presentation, diagnosis, and management of a 56-year-old man with ACC are discussed here. Imaging with computerized tomography (CT) in particular is crucial in the diagnosis, which can identify the primary lesion as well as metastases. ACC should be considered in the differential as a source of abdominal, epigastric, or back pain with imaging that is suggestive of the diagnosis as prompt recognition and initiation of treatment is paramount in the overall prognosis.

Preparing Fellows for Graduation: Perspectives on Career Guidance.

At the completion of a hematology/oncology fellowship, trainees are likely to enter into a career differing from the one modeled by the faculty providing mentorship and teaching during training. Fellows benefit from open communication with regard to career goals and opportunities starting early in training. To ensure honesty in the process, program directors must be accepting and supportive of house staff selecting careers different from those of the training environment. Following identification of a long-term career goal, program directors may facilitate a smooth transition to the early career through thoughtful inclusion of alternative experiences and additional mentors. Barriers exist, including funding and limited time in training while completing educational requirements, which may make inclusion of the experiences a challenge.

Durable Tumor Regression and Overall Survival in Patients With Advanced Merkel Cell Carcinoma Receiving Pembrolizumab as First-Line Therapy.

PURPOSE: Merkel cell carcinoma (MCC) is an aggressive skin cancer often caused by the Merkel cell polyomavirus. Clinical trials of programmed cell death-1 pathway inhibitors for advanced MCC (aMCC) demonstrate increased progression-free survival (PFS) compared with historical chemotherapy data. However, response durability and overall survival (OS) data are limited. PATIENTS AND METHODS: In this multicenter phase II trial (Cancer Immunotherapy Trials Network-09/Keynote-017), 50 adults naïve to systemic therapy for aMCC received pembrolizumab (2 mg/kg every 3 weeks) for up to 2 years. Radiographic responses were assessed centrally per Response Evaluation Criteria in Solid Tumors (RECIST) v1.1. RESULTS: Among 50 patients, the median age was 70.5 years, and 64% had Merkel cell polyomavirus-positive tumors. The objective response rate (ORR) to pembrolizumab was 56% (complete response [24%] plus partial response [32%]; 95% CI, 41.3% to 70.0%), with ORRs of 59% in virus-positive and 53% in virus-negative tumors. Median follow-up time was 14.9 months (range, 0.4 to 36.4+ months). Among 28 responders, median response duration was not reached (range, 5.9 to 34.5+ months). The 24-month PFS rate was 48.3%, and median PFS time was 16.8 months (95% CI, 4.6 months to not estimable). The 24-month OS rate was 68.7%, and median OS time was not reached. Although tumor viral status did not correlate with ORR, PFS, or OS, there was a trend toward improved PFS and OS in patients with programmed death ligand-1-positive tumors. Grade 3 or greater treatment-related adverse events occurred in 14 (28%) of 50 patients and led to treatment discontinuation in seven (14%) of 50 patients, including one treatment-related death. CONCLUSION: Here, we present the longest observation to date of patients with aMCC receiving first-line anti-programmed cell death-1 therapy. Pembrolizumab demonstrated durable tumor control, a generally manageable safety profile, and favorable OS compared with historical data from patients treated with first-line chemotherapy.

No Decision Is Final: Career Planning and Career Transitions.

Several factors play a role in job selection after completion of a hematology/oncology training program, such as a fellows' overall career goals, expected income potential, and limitations imposed by visa status, among many others. Training programs play an integral role in mentoring trainees in career selection. For many, the first job is often not career-long. In addition to considerations for a fellow considering a first job out of fellowship, physicians will consider a change because of dissatisfaction at one's current position, desire for advancement opportunities, or a desire to work in a different sector. Other factors include non-occupational issues such as career opportunities for a spouse or desire for a different geographic location. Frequent employment changes are common with crossover between academia, clinical practice, industry, or government service. Possessing the skills needed to recognize one's strengths, weaknesses, and goal prioritization can allow for more optimal job selection should a career transition into a different discipline occur. Recognizing opportunities that present themselves and potentially taking advantage of them can lead to professional and personal growth.

Clinical Case of the Month: A 57-Year-Old Man with an Axillary Mass.

A 57-year-old man presented to the surgical oncology clinic with a mildly tender mass under his right arm. Four years prior, the patient had a melanoma removed from his right shoulder along with an ipsilateral right axillary sentinel lymph sampling. Computed tomography (CT) scan was negative for metastatic disease at that time. The patient did not undergo completion axillary node dissection and was lost to follow-up. The patient was originally from Australia, did not tan but reported multiple sunburns before age 18. He was of Irish ancestry. He denied weight gain, fever, fatigue, anorexia, or night sweats. The patient had a medical history of atrial fibrillation, hypertension, gout, melanoma, and benign prostatic hypertrophy. His surgical history included an appendectomy and a facial laceration repair. His brother died at 16 years old from leukemia and his mother died from colon cancer. He consumed 3 alcoholic beverages per day and denied tobacco or illicit drug use. On physical exam, the patient's temperature was 98.8° Fahrenheit, heart rate of 73 beats / minute, blood pressure of 121 / 59 mm Hg, respiratory rate of 18 / min. He appeared to be healthy and in no apparent distress. Cardiovascular, respiratory, breast, gastrointestinal, musculoskeletal, and neurological exam were unremarkable. His right axillary lymph node exam revealed a firm mass roughly 2.5 cm tall by 1.5 cm wide. This mass was biopsied and findings were consistent with metastatic melanoma. CT scan revealed small volume mediastinal adenopathy and a 4.5 cm right axillary mass. There was a 4.7 cm lesion within the anterior left lower lobe of the liver and periportal node conglomerate measuring 3.9 cm consistent with metastatic disease (Figure 1). He was negative for the BRAF V600E mutation. The patient was consented for treatment with combination immune checkpoint inhibition with ipilimumab and nivolumab. After two cycles the patient showed good response, but temporarily stopped treatment after complications related to a ST segment elevation myocardial infarction. He developed mild pneumonitis felt to be related to nivolumab, and recovered after a short course of glucocorticosteroids. Restaging CT scans were ordered after two cycles of therapy (Figure 2), which showed decrease in the size of the axillary and hepatic metastases. At six months, CT scans showed continued durable response (Figure 3).

PSA flare after initiation of abiraterone acetate.

Prostate cancer is the second-most common cause of male cancer-related death among US men. The American Cancer Society estimates that in 2014 there will be about 233,000 new cases of prostate cancer and about 29,500 deaths due to prostate cancer. Sixty percent of cases are diagnosed in men aged 65 years or older. Localized prostate cancers are managed with active surveillance or external beam radiation therapy or radical prostatectomy after risk stratification. Advanced prostate cancers are managed with hormonal therapy for castrate sensitive prostate cancer and with novel androgen blocking therapies, chemotherapy, or radio-immunotherapy for castrate resistant prostate cancers. Prostate-specific antigen (PSA) flare is characterized by a rise in the PSA level, followed by a decline to below baseline values after starting therapies such as androgen deprivation therapy, systemic chemotherapy, or local therapies such as brachytherapy or cryotherapy.

Hemoglobin SO-Arab and α-thalassemia diagnosed in an adult: A case-based review of the hemoglobinopathies.

Hemoglobin SO-Arab is a rare sickling disorder with a clinical course similar to that of hemoglobin SS. Hemoglobin C-Harlem is another rare condition that produces sickling disorders in affected individuals with a disease course and electrophoretic findings similar to that of hemoglobin SO-Arab. The authors report the case of a 38-year-old African American man with hemoglobin SO-Arab and the challenges that may arise in working up a rare hemoglobinopathy.

Small cell lung cancer: time to diagnosis and treatment.

OBJECTIVES: Small cell lung cancer (SCLC) is the most rapidly progressive form of lung cancer, and delays in treatment may increase the tumor burden. We determined the time from abnormal radiograph to diagnosis and treatment for patients with SCLC and investigated the effect of emergent presentation, stage, radiographic findings, and race on these measures and survival. METHODS: Retrospective analysis of clinical data for 45 consecutive evaluable patients at a single institution diagnosed as having SCLC. RESULTS: Median time from first abnormal radiograph to tissue diagnosis was 10 days and time from first abnormal radiograph to initiation of treatment was 35 days. Emergency department presentation led to earlier diagnosis compared with the clinic (3 vs 21 days), with a trend toward earlier treatment (15 vs 39 days; P = 0.057). No significant effect of radiographic findings, disease stage, or race was observed from time to diagnosis or treatment. Forty-three patients (96%) died, with a median survival time from initial abnormal radiograph to death of 375 days. Survival was longer for patients with limited stage disease (619 vs 230 days), but it was not significantly affected by emergent presentation, radiographic findings, or race. Consistent with the aggressive behavior of SCLC, 10 patients (22%) had a normal radiograph within 6 months prediagnosis, and 7 of these had extensive disease. CONCLUSIONS: The median time from abnormal radiograph to initiation of treatment for SCLC in our single-center study overlapped with the expected doubling time for this malignancy and likely allowed a preventable increase in tumor burden.

A paradigm shift in the treatment of advanced non-small cell lung cancer.

Lung cancer is the most common cause of cancer death for men and women worldwide. Nonsmall cell lung cancer (NSCLC) accounts for approximately 85% of all lung cancers. Systemic chemotherapy is a cornerstone of treatment in the management of stage IV NSCLC. First-line chemotherapy typically consists of a platinum-based treatment. The optimum approach to long-term treatment beyond 4 to 6 cycles of chemotherapy is still evolving. Second-line chemotherapy given at the time of disease progression has shown clear survival advantages when compared with best supportive care alone. With the recent increase in the number of active and more tolerable agents available to treat NSCLC, there is renewed interest in the role of continuation of antineoplastic agents (continuation maintenance) or switching to a different agent (switch maintenance) after first-line chemotherapy. This case-based review discusses the role of maintenance chemotherapy in the long-term management of advanced NSCLC.

Restoration of medical oncology services at LSU Interim Public Hospital in New Orleans after Hurricane Katrina: a two-year experience of LSUHSC.

BACKGROUND: Oncology services at Charity Hospital were discontinued following Hurricane Katrina in August 2005. Medical oncology and chemotherapy services resumed at the Louisiana State University Interim Public Hospital in 2007. Demographic, clinical, and displacement data of the re-established patient cohort were reviewed. METHODS: Patients evaluated in the Louisiana State University Health Sciences Center (LSUHSC) Oncology Clinics from September 1, 2007, to August 31, 2009, were identified and data collected included time from diagnosis of malignancy to initial oncology evaluation, insurance status, percentage displaced for six months or more due to Hurricane Katrina, ethnicity, referrals for radiation oncology, and the number of outpatient clinical encounters (OCE). RESULTS: 464 patients were evaluated in the study time period. Sixty-five percent of the patients had new cancer diagnoses and 35% re-established cancer care in the Charity System and a substantial proportion were either unfunded or had Medicaid coverage. Thirty-four percent were confirmed to be displaced from New Orleans for greater than six months and the majority of patients were black. The majority of new cancer diagnoses were breast, lung, and colon cancer. Human immunodeficiency virus (HIV) positive patients made up 7.5% of the patient cohort. There was a 70% decline in patient volumes following Hurricane Katrina. CONCLUSIONS: Oncology services for a minority-based, underinsured patient population were severely impacted by Hurricane Katrina. Following the storm, persistent systemwide resource limitations led to suboptimal timeliness of medical oncology evaluations. Health care systems serving underinsured patients require a disaster plan to minimize interruption of oncology care. Our experience illustrates the need for resources to ensure rapid re-establishment of care for economically disadvantaged patients following natural disasters.

Clinical case of the month. A rare case of Budd Chiari syndrome.

Budd Chiari syndrome is a rare disorder resulting from hepatic venous outflow tract obstruction anywhere from the small hepatic veins to the suprahepatic inferior vena cava. This patient has a hypercoagulable state secondary to heterozygous mutation of factor V and the JAK2 mutation and is being anticoagulated. We hypothesize that the low protein C and low antithrombin III levels seen in this patient resulted from decreased synthetic function of the liver and were not indicative of actual deficiencies. Indeed, reports of coexisting protein C and antithrombin III deficiencies are not existent in the literature and likely are not compatible with life. All patients with BCS warrant a hypercoagulable work up and JAK2 mutation is increasingly recognized as a contributing factor, even in those patients without obvious signs of polycythemia vera.

Adjuvant high-dose interferon-{alpha} for resected melanoma in a patient with HIV infection.

Adjuvant interferon (IFN)-alpha remains the standard adjuvant therapy for intermediate and high-risk melanoma after definitive surgical resection. Data addressing the role and safety of adjuvant immunotherapy in HIV-infected patients with melanoma are lacking. We report on an HIV(+) patient who received IFN-alpha as adjuvant treatment for high-risk melanoma. To our knowledge, this is the first reported case of such an approach.