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Jilma Patrick and Seema Ahsan Khan

Management of the intact primary tumor in women presenting with stage IV breast cancer has classically been determined by the presence or absence of symptoms. However, multiple retrospective reviews completed over the past decade suggest a survival advantage with resection of the intact, asymptomatic primary tumor in these women. These reviews are not without bias, and recently completed randomized trials do not support a significant survival benefit, although local control benefits may exist. Completion of ongoing trials is needed to reach a definitive conclusion regarding the merit of primary tumor resection for local control and survival. Until unbiased data are available, local therapy for the asymptomatic primary cannot be routinely recommended, but may be considered in selected individuals, with multidisciplinary input, when systemic disease is well controlled.

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Nora M. Hansen, Sally Anne Scherer and Seema Khan

The management of patients with breast cancer has become very complex, and a multidisciplinary approach is paramount to optimal treatment. A multidisciplinary approach requires timely coordination among the varied disciplines involved in patient care, and timely intervention has been shown to lead to better outcomes. To evaluate some of the key processes in providing timely multidisciplinary care, NCCN awarded grants to institutions to evaluate opportunities for improvement in breast cancer care. This article reports on the opportunities for improvement project at Feinberg School of Medicine at Northwestern University.

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William G. Wierda, John C. Byrd, Jeremy S. Abramson, Seema Bhat, Greg Bociek, Danielle Brander, Jennifer Brown, Asher Chanan-Khan, Steve E. Coutre, Randall S. Davis, Christopher D. Fletcher, Brian Hill, Brad S. Kahl, Manali Kamdar, Lawrence D. Kaplan, Nadia Khan, Thomas J. Kipps, Jeffrey Lancet, Shuo Ma, Sami Malek, Claudio Mosse, Mazyar Shadman, Tanya Siddiqi, Deborah Stephens, Nina Wagner, Andrew D. Zelenetz, Mary A. Dwyer and Hema Sundar

Hairy cell leukemia (HCL) is a rare type of indolent B-cell leukemia, characterized by symptoms of fatigue and weakness, organomegaly, pancytopenia, and recurrent opportunistic infections. Classic HCL should be considered a distinct clinical entity separate from HCLvariant (HCLv), which is associated with a more aggressive disease course and may not respond to standard HCL therapies. Somatic hypermutation in the IGHV gene is present in most patients with HCL. The BRAF V600E mutation has been reported in most patients with classic HCL but not in those with other B-cell leukemias or lymphomas. Therefore, it is necessary to distinguish HCLv from classic HCL. This manuscript discusses the recommendations outlined in the NCCN Guidelines for the diagnosis and management of classic HCL.

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Kenneth C. Anderson, Melissa Alsina, William Bensinger, J. Sybil Biermann, Asher Chanan-Khan, Adam D. Cohen, Steven Devine, Benjamin Djulbegovic, Cristina Gasparetto, Carol Ann Huff, Madan Jagasia, Bruno C. Medeiros, Ruby Meredith, Noopur Raje, Jeffrey Schriber, Seema Singhal, George Somlo, Keith Stockerl-Goldstein, Guido Tricot, Julie M. Vose, Donna Weber, Joachim Yahalom and Furhan Yunus

Multiple Myeloma Clinical Practice Guidelines in Oncology NCCN Categories of Evidence and Consensus Category 1: The recommendation is based on high-level evidence (e.g., randomized controlled trials) and there is uniform NCCN consensus. Category 2A: The recommendation is based on lower-level evidence and there is uniform NCCN consensus. Category 2B: The recommendation is based on lower-level evidence and there is nonuniform NCCN consensus (but no major disagreement). Category 3: The recommendation is based on any level of evidence but reflects major disagreement. All recommendations are category 2A unless otherwise noted. Clinical trials: The NCCN believes that the best management for any cancer patient is in a clinical trial. Participation in clinical trials is especially encouraged. Overview Multiple myeloma (MM) is a malignant neoplasm of plasma cells that accumulate in bone marrow, leading to bone destruction and marrow failure. The American Cancer Society estimates that 20,580 new cases of MM will occur in the United States in 2009, including 11,680 in men and 8900 in women, with an estimated 10,580 deaths.1 The mean age of affected individuals is 62 years for men (75% > 70 years) and 61 years for women (79% > 70 years). The treatment of MM has dramatically improved over the past decade. The 5-year survival rate reported in the Surveillance Epidemiology and End Results database has increased from 25% in 1975 to 34% in 2003 because of the availability of newer and more effective treatment options.2,3 MM is typically sensitive to various cytotoxic drugs, both as initial treatment...
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Therese B. Bevers, Benjamin O. Anderson, Ermelinda Bonaccio, Sandra Buys, Mary B. Daly, Peter J. Dempsey, William B. Farrar, Irving Fleming, Judy E. Garber, Randall E. Harris, Alexandra S. Heerdt, Mark Helvie, John G. Huff, Nazanin Khakpour, Seema A. Khan, Helen Krontiras, Gary Lyman, Elizabeth Rafferty, Sara Shaw, Mary Lou Smith, Theodore N. Tsangaris, Cheryl Williams and Thomas Yankeelov

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Kenneth C. Anderson, Melissa Alsina, William Bensinger, J. Sybil Biermann, Asher Chanan-Khan, Adam D. Cohen, Steven Devine, Benjamin Djulbegovic, Edward A. Faber Jr., Cristina Gasparetto, Carol Ann Huff, Adetola Kassim, Bruno C. Medeiros, Ruby Meredith, Noopur Raje, Jeffrey Schriber, Seema Singhal, George Somlo, Keith Stockerl-Goldstein, Steven P. Treon, Guido Tricot, Donna M. Weber, Joachim Yahalom and Furhan Yunus

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Mary B. Daly, Robert Pilarski, Jennifer E. Axilbund, Michael Berry, Saundra S. Buys, Beth Crawford, Meagan Farmer, Susan Friedman, Judy E. Garber, Seema Khan, Catherine Klein, Wendy Kohlmann, Allison Kurian, Jennifer K. Litton, Lisa Madlensky, P. Kelly Marcom, Sofia D. Merajver, Kenneth Offit, Tuya Pal, Huma Rana, Gwen Reiser, Mark E. Robson, Kristen Mahoney Shannon, Elizabeth Swisher, Nicoleta C. Voian, Jeffrey N. Weitzel, Alison Whelan, Myra J. Wick, Georgia L. Wiesner, Mary Dwyer, Rashmi Kumar and Susan Darlow

The NCCN Guidelines for Genetic/Familial High-Risk Assessment: Breast and Ovarian provide recommendations for genetic testing and counseling and risk assessment and management for hereditary cancer syndromes. Guidelines focus on syndromes associated with an increased risk of breast and/or ovarian cancer and are intended to assist with clinical and shared decision-making. These NCCN Guidelines Insights summarize major discussion points of the 2015 NCCN Genetic/Familial High-Risk Assessment: Breast and Ovarian panel meeting. Major discussion topics this year included multigene testing, risk management recommendations for less common genetic mutations, and salpingectomy for ovarian cancer risk reduction. The panel also discussed revisions to genetic testing criteria that take into account ovarian cancer histology and personal history of pancreatic cancer.

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Mary B. Daly, Robert Pilarski, Michael Berry, Saundra S. Buys, Meagan Farmer, Susan Friedman, Judy E. Garber, Noah D. Kauff, Seema Khan, Catherine Klein, Wendy Kohlmann, Allison Kurian, Jennifer K. Litton, Lisa Madlensky, Sofia D. Merajver, Kenneth Offit, Tuya Pal, Gwen Reiser, Kristen Mahoney Shannon, Elizabeth Swisher, Shaveta Vinayak, Nicoleta C. Voian, Jeffrey N. Weitzel, Myra J. Wick, Georgia L. Wiesner, Mary Dwyer and Susan Darlow

The NCCN Clinical Practice Guidelines in Oncology for Genetic/Familial High-Risk Assessment: Breast and Ovarian provide recommendations for genetic testing and counseling for hereditary cancer syndromes and risk management recommendations for patients who are diagnosed with a syndrome. Guidelines focus on syndromes associated with an increased risk of breast and/or ovarian cancer. The NCCN Genetic/Familial High-Risk Assessment: Breast and Ovarian panel meets at least annually to review comments from reviewers within their institutions, examine relevant new data from publications and abstracts, and reevaluate and update their recommendations. The NCCN Guidelines Insights summarize the panel's discussion and most recent recommendations regarding risk management for carriers of moderately penetrant genetic mutations associated with breast and/or ovarian cancer.