ABSTRACT
BACKGROUND: Systemic treatments for angiosarcoma remains an area of unmet clinical need. The authors conducted this retrospective study to assess the clinical activity of checkpoint inhibitors in patients with angiosarcoma. The primary objective was to assess the objective response rate, and the secondary objective was to assess the progression-free and overall survival durations and disease control rate. METHODS: Patient data were obtained using The University of Texas MD Anderson Cancer Center Tumor Registry database. The final study population was refined to only include patients who had undergone pembrolizumab monotherapy. The objective response rate was evaluated using RECIST/irRECIST version 1.1. Progression-free survival and overall survival were defined as the time from the initiation of immunotherapy to disease progression or recurrence, death, or last follow-up and to death or last follow-up, respectively. RESULTS: The final cohort comprised 25 patients. Most patients had metastatic disease (72%) and had undergone at least two lines of systemic therapy (80%) before starting pembrolizumab. The objective response rate was 18%, whereas the disease control rate was 59%. The median progression-free survival duration was 6.2 months and was not significantly different between the cutaneous (4.7 months) and visceral angiosarcoma (6.2 months) groups (p = .42). The median overall survival duration was 72.6 months. Toxicities were recorded for eight patients, with fatigue, anemia, constipation, and rash being the most common. CONCLUSIONS: Pembrolizumab shows durable clinical activity in angiosarcoma. These findings suggest that checkpoint inhibition as monotherapy or combination therapy is likely to have a high probability of success.© 2022 American Cancer Society. LAY SUMMARY: This is the largest retrospective study to assess the clinical activity of checkpoint inhibitor monotherapy in angiosarcomas. The study includes an adequate number of patients with visceral angiosarcoma that enabled to obtain meaningful clinical insights that were previously unavailable. Our findings indicate an improvement in progression-free survival with pembrolizumab that is comparable to other active agents in angiosarcoma. Pembrolizumab monotherapy in angiosarcomas also has a favorable tolerability profile. Our findings emphasize the need for prospective studies to evaluate the activity of pembrolizumab monotherapy and combination therapy.
Subject(s)
Hemangiosarcoma , Humans , Immunotherapy , Progression-Free Survival , Prospective Studies , Retrospective StudiesABSTRACT
BACKGROUND: Primary cardiac angiosarcomas are rare, but they are the most aggressive type of primary cardiac neoplasms. When patients do present, it is with advanced pulmonary and/or cardiac symptoms. Therefore, many times the correct diagnosis is not made at the time of initial presentation. These patients have metastatic disease and the vast majority of these patients die within a few months after diagnosis. Currently the treatment choices are limited and there are no targeted therapies available. CASE PRESENTATION: A 56-year-old male presented with shortness of breath, night sweats, and productive cough for a month. Workup revealed pericardial effusion and multiple bilateral pulmonary nodules suspicious for metastatic disease. Transthoracic echocardiogram showed a large pericardial effusion and a large mass in the base of the right atrium. Results of biopsy of bilateral lung nodules established a diagnosis of primary cardiac angiosarcoma. Aggressive pulmonary disease caused rapid deterioration; the patient went on hospice and subsequently died. Whole exome sequencing of the patient's postmortem tumor revealed a novel KDR (G681R) mutation, and focal high-level amplification at chromosome 1q encompassing MDM4, a negative regulator of TP53. CONCLUSION: Mutations in KDR have been reported previously in angiosarcomas. Previous studies also demonstrated that KDR mutants with constitutive KDR activation could be inhibited with specific KDR inhibitors in vitro. Thus, patients harboring activating KDR mutations could be candidates for treatment with KDR-specific inhibitors.
Subject(s)
DNA Mutational Analysis , Heart Neoplasms/genetics , Hemangiosarcoma/genetics , Cell Cycle Proteins , Exome/genetics , Fatal Outcome , Gene Amplification , Humans , Male , Middle Aged , Mutation , Nuclear Proteins/genetics , Proto-Oncogene Proteins/genetics , Vascular Endothelial Growth Factor Receptor-2/geneticsABSTRACT
Ewing sarcoma is a highly resistant disease with a <10% chance of survival at 5 years after failure of frontline chemotherapy. This is a case report of an Ewing sarcoma patient with metastatic disease recurrence <2 years after standard chemotherapy/radiation who achieved a durable and sustained complete response after 2 series of treatments with Vigil (GMCSF/bi-shRNA furin DNA autologous tumor immunotherapy) serially manufactured from first and second recurrences with ELISPOT assay correlation. Results support justification of further testing of Vigil with ELISPOT assay as a biomarker to assess level of immune response and correlation with disease control.
Subject(s)
Benzhydryl Compounds/administration & dosage , Cancer Vaccines/administration & dosage , Immunotherapy/methods , Sarcoma, Ewing/therapy , Adolescent , Benzhydryl Compounds/therapeutic use , Cancer Vaccines/therapeutic use , Enzyme-Linked Immunospot Assay , Female , Granulocyte-Macrophage Colony-Stimulating Factor/therapeutic use , Humans , Modafinil , RNA, Small Interfering/therapeutic use , Salvage Therapy/methods , Sarcoma, Ewing/diagnosis , Treatment OutcomeABSTRACT
Ewing's sarcoma is a devastating rare pediatric cancer of the bone. Intense chemotherapy temporarily controls disease in most patients at presentation but has limited effect in patients with progressive or recurrent disease. We previously described preliminary results of a novel immunotherapy, FANG (Vigil) vaccine, in which 12 advanced stage Ewing's patients were safely treated and went on to achieve a predicted immune response (IFNγ ELISPOT). We describe follow-up through year 3 of a prospective, nonrandomized study comparing an expanded group of Vigil-treated advanced disease Ewing's sarcoma patients (n = 16) with a contemporaneous group of Ewing's sarcoma patients (n = 14) not treated with Vigil. Long-term follow-up results show a survival benefit without evidence of significant toxicity (no ≥ grade 3) to Vigil when administered once monthly by intradermal injection (1 × 10e(6) cells/injection to 1 × 10e(7) cells/injection). Specifically, we report a 1-year actual survival of 73% for Vigil-treated patients compared to 23% in those not treated with Vigil. In addition, there was a 17.2-month difference in overall survival (OS; Kaplan-Meier) between the Vigil (median OS 731 days) and no Vigil patient groups (median OS 207 days). In conclusion, these results supply the rational for further testing of Vigil in advanced stage Ewing's sarcoma.
ABSTRACT
Stathmin1 (STMN1) is a microtubule modulator that is expressed in multiple cancers and correlates with poor survival. We previously demonstrated in vivo safety of bifunctional (bi) shRNA STMN1 bilamellar invaginated vesicle (BIV) and that systemic delivery correlated with antitumor activity. Patients with superficial advanced refractory cancer with no other standard options were entered into trial. Study design involved dose escalation (four patients/cohort) using a modified Fibonacci schema starting at 0.7 mg DNA administered via single intratumoral injection. Biopsy at baseline, 24/48 hours and resection 8 days after injection provided tissue for determination of cleavage product using next-generation sequencing (NGS) and reverse transcription quantitative polymerase chain reaction (RT-qPCR), 5' RLM rapid amplification of cDNA ends (RACE) assay. Serum pharmacokinetics of circulating plasmid was done. Twelve patients were entered into three dose levels (0.7, 1.4, 7.0 mg DNA). No ≥ grade 3 toxic effects to drug were observed. Maximum circulating plasmid was detected at 30 seconds with less than 10% detectable in all subjects at 24 hours. No toxic effects were observed. Predicted cleavage product was detected by both NGS (n = 7/7 patients analyzed, cohorts 1, 2) and RLM RACE (n = 1/1 patients analyzed cohort 3). In conclusion, bi-shRNA STMN1 BIV is well tolerated and detection of mRNA target sequence-specific cleavage product confirmed bi-shRNA BIV mechanism of action.
Subject(s)
Neoplasms/therapy , RNA, Small Interfering/metabolism , Stathmin/genetics , Tubulin Modulators/pharmacology , Aged , Aged, 80 and over , Dose-Response Relationship, Drug , Drug Delivery Systems , Female , High-Throughput Nucleotide Sequencing , Humans , Injections, Intralesional , Male , Middle Aged , Neoplasms/genetics , Plasmids/blood , RNA, Messenger/genetics , RNA, Messenger/metabolism , RNA, Small Interfering/genetics , Stathmin/pharmacokinetics , Toxicity TestsABSTRACT
We report on 12 consecutive patients with advanced/metastatic Ewing's sarcoma who were treated as a separate cohort of a phase 1 trial of FANG autologous immunotherapy (1 × 10(6)-2.5 × 10(7) cells/intradermal injection each month for minimum 4 months). Safety and clinical response were monitored. Patient immune response to unmodified autologous tumor cells was assessed by gamma interferon-enzyme-linked immunospot (γIFN-ELISPOT) assay using peripheral blood mononuclear cells from baseline (pretreatment) and multiple postvaccination time points. None of the 12 patients (47 vaccinations) developed grade 2/3/4 drug-related toxicity. Median product release granulocyte-macrophage colony-stimulating factor expression was 1,941 pg/10(6) cells, and TGFß1and TGFß2 knockdown were 99 and 100%, respectively. Eight patients were assessed for ELISPOT response to autologous tumor cells at baseline and all (100%) were negative. In contrast, follow-up ELISPOT response at month 1 or month 4 (one patient) after FANG was positive in all eight patients. One patient achieved a partial tumor response (38% tumor reduction, RECIST 1.1). The Kaplan-Meier estimated survival of these 12 patients at 1 year was 75%. In this phase 1 study in patients with Ewing's sarcoma, FANG immunotherapy was well tolerated, elicited a tumor-specific systemic immune response in all patients, and was associated with favorable 1-year survival. Further clinical testing is indicated.
Subject(s)
Immunotherapy/methods , Sarcoma, Ewing/therapy , Adolescent , Adult , Female , Gene Expression Regulation , Granulocyte-Macrophage Colony-Stimulating Factor/genetics , Granulocyte-Macrophage Colony-Stimulating Factor/metabolism , Humans , Interferon-gamma/metabolism , Leukocytes, Mononuclear/metabolism , Male , Middle Aged , Neoplasm Metastasis/therapy , Pilot Projects , Transforming Growth Factor beta1/genetics , Transforming Growth Factor beta1/metabolism , Transforming Growth Factor beta2/genetics , Transforming Growth Factor beta2/metabolism , Treatment Outcome , Young AdultABSTRACT
Treatment of advanced melanoma has significantly improved with the advent of checkpoint inhibitor therapy. With the widespread use of these agents, side effects are being increasingly recognized, including immune-related adverse events. We report the onset of adrenal insufficiency in a patient with advanced melanoma who was exposed to two checkpoint inhibitors: ipilimumab and nivolumab. His symptoms initially resolved with steroid replacement but he was unable to be weaned off hormone replacement and required long-term oral hydrocortisone treatment.
ABSTRACT
Purpose This focused update addresses the use of MammaPrint (Agendia, Irvine, CA) to guide decisions on the use of adjuvant systemic therapy. Methods ASCO uses a signals approach to facilitate guideline updates. For this focused update, the publication of the phase III randomized MINDACT (Microarray in Node-Negative and 1 to 3 Positive Lymph Node Disease May Avoid Chemotherapy) study to evaluate the MammaPrint assay in 6,693 women with early-stage breast cancer provided a signal. An expert panel reviewed the results of the MINDACT study along with other published literature on the MammaPrint assay to assess for evidence of clinical utility. Recommendations If a patient has hormone receptor-positive, human epidermal growth factor receptor 2 (HER2)-negative, node-negative breast cancer, the MammaPrint assay may be used in those with high clinical risk to inform decisions on withholding adjuvant systemic chemotherapy due to its ability to identify a good-prognosis population with potentially limited chemotherapy benefit. Women in the low clinical risk category did not benefit from chemotherapy regardless of genomic MammaPrint risk group. Therefore, the MammaPrint assay does not have clinical utility in such patients. If a patient has hormone receptor-positive, HER2-negative, node-positive breast cancer, the MammaPrint assay may be used in patients with one to three positive nodes and a high clinical risk to inform decisions on withholding adjuvant systemic chemotherapy. However, such patients should be informed that a benefit from chemotherapy cannot be excluded, particularly in patients with greater than one involved lymph node. The clinician should not use the MammaPrint assay to guide decisions on adjuvant systemic therapy in patients with hormone receptor-positive, HER2-negative, node-positive breast cancer at low clinical risk, nor any patient with HER2-positive or triple-negative breast cancer, because of the lack of definitive data in these populations. Additional information can be found at www.asco.org/breast-cancer-guidelines and www.asco.org/guidelineswiki .
Subject(s)
Biomarkers, Tumor/metabolism , Breast Neoplasms/drug therapy , Breast Neoplasms/metabolism , Chemotherapy, Adjuvant , Decision Making , Decision Support Techniques , Female , HumansABSTRACT
We describe a 23-year-old white man who presented with anasarca and a new periumbilical mass. He had preserved kidney function and laboratory findings consistent with nephrotic syndrome, including 9.7 g/day albuminuria. Serum serologies were positive for anti-SSa and anti-SSb and low complements but were negative for antinuclear antibody. Pathologic findings of the abdominal mass showed a mammary-type myofibroblastoma. A kidney biopsy revealed a diffuse proliferative and membranous immune-mediated glomerulonephritis with 10% interstitial fibrosis. This is a novel case of mammary-type myofibroblastoma associated with nephrotic syndrome mimicking a proliferative lupus pattern.
ABSTRACT
PURPOSE: To provide recommendations on appropriate use of breast tumor biomarker assay results to guide decisions on adjuvant systemic therapy for women with early-stage invasive breast cancer. METHODS: A literature search and prospectively defined study selection sought systematic reviews, meta-analyses, randomized controlled trials, prospective-retrospective studies, and prospective comparative observational studies published from 2006 through 2014. Outcomes of interest included overall survival and disease-free or recurrence-free survival. Expert panel members used informal consensus to develop evidence-based guideline recommendations. RESULTS: The literature search identified 50 relevant studies. One randomized clinical trial and 18 prospective-retrospective studies were found to have evaluated the clinical utility, as defined by the guideline, of specific biomarkers for guiding decisions on the need for adjuvant systemic therapy. No studies that met guideline criteria for clinical utility were found to guide choice of specific treatments or regimens. RECOMMENDATIONS: In addition to estrogen and progesterone receptors and human epidermal growth factor receptor 2, the panel found sufficient evidence of clinical utility for the biomarker assays Oncotype DX, EndoPredict, PAM50, Breast Cancer Index, and urokinase plasminogen activator and plasminogen activator inhibitor type 1 in specific subgroups of breast cancer. No biomarker except for estrogen receptor, progesterone receptor, and human epidermal growth factor receptor 2 was found to guide choices of specific treatment regimens. Treatment decisions should also consider disease stage, comorbidities, and patient preferences.
Subject(s)
Antineoplastic Agents/therapeutic use , Biomarkers, Tumor/analysis , Breast Neoplasms/drug therapy , Carcinoma, Ductal, Breast/drug therapy , Clinical Decision-Making/methods , Receptor, ErbB-2/analysis , Receptors, Estrogen/analysis , Receptors, Progesterone/analysis , Antineoplastic Agents, Hormonal/therapeutic use , Breast Neoplasms/chemistry , Breast Neoplasms/mortality , Breast Neoplasms/pathology , Carcinoma, Ductal, Breast/chemistry , Carcinoma, Ductal, Breast/mortality , Carcinoma, Ductal, Breast/pathology , Chemotherapy, Adjuvant , Comorbidity , Disease-Free Survival , Evidence-Based Medicine , Female , Humans , Neoplasm Staging , Plasminogen Activator Inhibitor 1/analysis , Predictive Value of Tests , Randomized Controlled Trials as Topic , Reproducibility of Results , Survival Analysis , Urokinase-Type Plasminogen Activator/analysisABSTRACT
PURPOSE: To compare the efficacy of pegylated liposomal doxorubicin (PLD) with that of a common salvage regimen (comparator) in patients with taxane-refractory advanced breast cancer. PATIENTS AND METHODS: Following failure of a first- or second-line taxane-containing regimen for metastatic disease, 301 women were randomly assigned to receive PLD (50 mg/m(2) every 28 days); or comparator-vinorelbine (30 mg/m(2) weekly) or mitomycin C (10 mg/m(2) day 1 and every 28 days) plus vinblastine (5 mg/m(2) day 1, day 14, day 28, and day 42) every 6 to 8 weeks. Patients were stratified before random assignment based on number of previous chemotherapy regimens for metastatic disease and presence of bone metastases only. RESULTS: Progression-free survival (PFS) and overall survival (OS) were similar for PLD and comparator (PFS: hazard ratio [HR], 1.26; 95% CI, 0.98 to 1.62; P =.11; median, 2.9 months [PLD] and 2.5 months [comparator]; OS: HR, 1.05; 95% CI, 0.82 to 1.33; P =.71; median, 11.0 months [PLD] and 9.0 months [comparator]). In anthracycline-naïve patients, PFS was somewhat longer with PLD, relative to the comparator (n = 44; median PFS, 5.8 v 2.1 months; HR, 2.40; 95% CI, 1.16 to 4.95; P =.01). Most frequently reported adverse events were nausea (23% to 31%), vomiting (17% to 20%), and fatigue (9% to 20%) and were similar among treatment groups. PLD-treated patients experienced more palmar-plantar erythrodysesthesia (37%; 18% grade 3, 1 patient grade 4) and stomatitis (22%; 5% grades 3/4). Neuropathy (11%), constipation (16%), and neutropenia (14%) were more common with vinorelbine. Alopecia was low in both the PLD and vinorelbine groups (3% and 5%). CONCLUSION: PLD has efficacy comparable to that of common salvage regimens in patients with taxane-refractory metastatic breast cancer, thereby representing a useful therapeutic option.
Subject(s)
Breast Neoplasms/drug therapy , Bridged-Ring Compounds/therapeutic use , Doxorubicin/therapeutic use , Mitomycin/therapeutic use , Taxoids/therapeutic use , Vinblastine/analogs & derivatives , Vinblastine/therapeutic use , Adult , Aged , Antineoplastic Combined Chemotherapy Protocols , Breast Neoplasms/pathology , Doxorubicin/adverse effects , Drug Resistance, Neoplasm , Female , Humans , Liposomes , Middle Aged , Mitomycin/adverse effects , Neoplasm Metastasis , Salvage Therapy , Vinblastine/adverse effects , VinorelbineABSTRACT
BACKGROUND: This phase II study evaluated the efficacy, safety, and health outcomes of pemetrexed treatment in heavily pretreated patients with advanced breast cancer. PATIENTS AND METHODS: Women with metastatic breast cancer, Karnofsky performance status > or = 70, and previous treatment with > or = 3 regimens containing anthracyclines, taxanes, and capecitabine were eligible. Pemetrexed 500 mg/m2 intravenous infusion was administered on day 1 of a 21-day treatment cycle. RESULTS: Eighty patients were enrolled, and 60 received concurrent folic acid and vitamin B12 supplements per protocol amendment to minimize possible pemetrexed-related toxicity. The median numbers of cycles delivered were 3 for vitamin-supplemented patients and 2 for non-vitamin-supplemented patients. Regardless of vitamin supplementation, the overall response rate was 8% (95% CI, 3%-16.6%), stable disease was exhibited in 36% of patients, median time to disease progression was 2.9 months, and median survival was 8.2 months. Improvements in patient-reported symptoms ranged from 16.2% for pain intensity to 32.1% for nausea. Major grade 3/4 toxicities were hematologic, with grade 4 neutropenia in 10% of patients and grade 3 toxicities consisting primarily of neutropenia (29%) and leukopenia (21%). There were no clear trends of the effect of supplementation on toxicity. CONCLUSION: Pemetrexed has modest antitumor activity and is well tolerated in heavily pretreated patients with breast cancer. Further evaluation of this multitargeted antifolate in advanced breast cancer is warranted.
Subject(s)
Antineoplastic Agents/therapeutic use , Breast Neoplasms/drug therapy , Glutamates/therapeutic use , Guanine/analogs & derivatives , Adult , Aged , Anthracyclines/therapeutic use , Antimetabolites, Antineoplastic/therapeutic use , Antineoplastic Agents/administration & dosage , Antineoplastic Agents/adverse effects , Breast Neoplasms/pathology , Capecitabine , Deoxycytidine/analogs & derivatives , Deoxycytidine/therapeutic use , Disease Progression , Drug Resistance, Neoplasm , Female , Fluorouracil/analogs & derivatives , Glutamates/administration & dosage , Glutamates/adverse effects , Guanine/administration & dosage , Guanine/adverse effects , Guanine/therapeutic use , Humans , Infusions, Intravenous , Middle Aged , Pemetrexed , Taxoids/therapeutic use , Treatment OutcomeABSTRACT
PURPOSE: To provide recommendations on the appropriate use of breast tumor biomarker assay results to guide decisions on systemic therapy for metastatic breast cancer. METHODS: A literature search and prospectively defined study selection identified systematic reviews, meta-analyses, randomized controlled trials (RCTs), prospective-retrospective studies, and prospective comparative observational studies published from 2006 through September 2014. RESULTS: The literature search revealed 17 articles that met criteria for further review: 11 studies reporting discordances between primary tumors and metastases in expression of hormone receptors or human epidermal growth factor receptor 2 (HER2), one RCT that addressed the use of a biomarker to decide whether to change or continue a treatment regimen, and five prospective-retrospective studies that evaluated the clinical utility of biomarkers. RECOMMENDATIONS: In patients with accessible metastases, biopsy for confirmation of disease process and retesting of estrogen receptor, progesterone receptor, and HER2 status should be offered, but evidence is lacking to determine whether changing anticancer treatment on the basis of change in receptor status affects clinical outcomes. With discordance of results between primary and metastatic tissues, the Panel consensus is to use preferentially the estrogen receptor, progesterone receptor, and HER2 status of the metastasis to direct therapy if supported by the clinical scenario and patient's goals for care. Carcinoembryonic antigen, cancer antigen 15-3, and cancer antigen 27-29 may be used as adjunctive assessments, but not alone, to contribute to decisions regarding therapy. Recommendations for tumor rebiopsy and use of circulating tumor markers are based on clinical experience and Panel informal consensus in the absence of studies designed to evaluate the clinical utility of the markers. As such, it is also reasonable for clinicians to not use these markers as adjunctive assessments.
Subject(s)
Biomarkers, Tumor/analysis , Breast Neoplasms/diagnosis , Breast Neoplasms/therapy , Biomarkers, Tumor/metabolism , Breast Neoplasms/metabolism , Breast Neoplasms/pathology , Female , Humans , Neoplasm Metastasis , Prospective Studies , United StatesABSTRACT
This prospective clinical trial was designed to assess the impact of adjuvant chemotherapy in women with rapidly proliferating node-negative breast cancer. This group has been predicted to have a 5-year disease-free survival (DFS) of 70% without adjuvant chemotherapy. In this study, 449 women with rapidly proliferating breast cancer (91% measured by S-phase fraction and 9% by histochemistry) received adjuvant chemotherapy with doxorubicin/cyclophosphamide (AC) plus tamoxifen for estrogen receptor-positive or progesterone receptor-positive cancer. The 5-year DFS was 90% (+/- 2%) and the 5-year overall survival was 94% (+/- 1%). At a median follow-up of 62 months, the strategy of administering 6 cycles of AC to women with T2 N0 cancer and 3 cycles in those with smaller T1 N0 cancers appeared to eliminate tumor size as a potential prognostic factor. Adjuvant chemotherapy with AC appears effective in reducing recurrence rates for women with rapidly proliferating node-negative breast cancer.
Subject(s)
Anticarcinogenic Agents/therapeutic use , Antineoplastic Agents/therapeutic use , Antineoplastic Combined Chemotherapy Protocols/therapeutic use , Breast Neoplasms/drug therapy , Breast Neoplasms/mortality , Cyclophosphamide/therapeutic use , Doxorubicin/therapeutic use , Lymph Nodes/pathology , Tamoxifen/therapeutic use , Adult , Aged , Anticarcinogenic Agents/administration & dosage , Antineoplastic Agents/administration & dosage , Antineoplastic Combined Chemotherapy Protocols/administration & dosage , Breast Neoplasms/pathology , Chemotherapy, Adjuvant , Cyclophosphamide/administration & dosage , Disease-Free Survival , Doxorubicin/administration & dosage , Female , Humans , Middle Aged , Neoplasm Invasiveness/pathology , Neoplasm Invasiveness/prevention & control , Prospective Studies , Survival Rate , Tamoxifen/administration & dosageABSTRACT
A 70-year-old woman being treated with folinic acid, fluorouracil, and oxaliplatin (FOLFOX) therapy for relapsed colon cancer metastatic to the lung presented to the hospital with a 1-week history of abdominal pain, anorexia, a 1-day history of diarrhea, and a fever of 101°F. Neutropenia and a peripheral eosinophilia were present, and computed tomogram of the abdomen showed thickening of the wall of a segment of small bowel with luminal stenosis. Colonoscopy and double-balloon small bowel enteroscopy found a stenosis in the ileum that upon biopsy revealed small bowel eosinophilic enteritis. She improved rapidly with the administration of dexamethasone. A Medline search for reports of small bowel eosinophilic enteritis in response to any of the components of FOLFOX was unrevealing.
ABSTRACT
Primary angiosarcoma of the breast is a rare malignant tumor. We report a case of breast primary cutaneous angiosarcoma in a patient with a strong family history of malignancy. For definitive diagnosis, a tissue biopsy is needed, with immunostaining for the presence of blood vessel endothelial markers CD31 and CD34. Total mastectomy is the preferred method of surgical treatment. Chemotherapy has not been shown to increase overall survival, but in some instances it may improve local control and disease-free survival. Surgery combined with radiation may increase local control, but patients at high risk of recurrence may benefit from adjuvant treatment as well. We discuss the potential benefits from various treatments for primary cutaneous breast angiosarcoma.
ABSTRACT
Primary cutaneous large B-cell lymphoma, leg type, is a rare and aggressive neoplasm as defined by the recently updated World Health Organization-European Organization for Research and Treatment of Cancer classification of cutaneous lymphomas. We present a case of a 74-year-old woman who presented with a cutaneous lesion on her forearm. Skin biopsy revealed pathology consistent with this entity. The patient was treated with systemic chemotherapy with rituximab combined with doxorubicin, cyclophosphamide, vincristine, and prednisone. Here, we review the available literature and summarize clinical features and management of this uncommon subtype of non-Hodgkin lymphoma.
ABSTRACT
Primary effusion lymphoma (PEL), formerly known as body cavity-based lymphoma, is a high-grade B-cell non-Hodgkin's lymphoma associated with Kaposi's sarcoma and human herpesvirus 8 infection. It usually affects serous body cavities and results in recurrent lymphomatous effusions. PEL is often diagnosed in patients with HIV infection and carries a poor prognosis, with median survival near 6 months. We describe a patient who presented with symptomatic pericardial effusion, secondary to newly diagnosed PEL, and no prior history of HIV infection.