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Paul F. Engstrom, Juan Pablo Arnoletti, Al B. Benson III, Jordan D. Berlin, J. Michael Berry, Yi-Jen Chen, Michael A. Choti, Harry S. Cooper, Raza A. Dilawari, Dayna S. Early, Peter C. Enzinger, Marwan G. Fakih, James Fleshman Jr., Charles Fuchs, Jean L. Grem, James A. Knol, Lucille A. Leong, Edward Lin, Mary F. Mulcahy, Eric Rohren, David P. Ryan, Leonard Saltz, David Shibata, John M. Skibber, William Small Jr., Constantinos Sofocleous, James Thomas, Alan P. Venook and Christopher Willett

Overview An estimated 5290 new cases (2100 men and 3190 women) of anal cancer (involving the anus, anal canal, or anorectum) will occur in the United States in 2009, accounting for approximately 1.9% of digestive system cancers, and an estimated 710 deaths due to anal cancer. Although considered to be a rare type of cancer, the incidence rate of invasive anal carcinoma in the United States increased by approximately 1.6-fold for men and 1.5-fold for women from 1973-1979 to 1994-2000 (see Risk Factors, facing page). This manuscript summarizes the NCCN Clinical Practice Guidelines in Oncology for managing squamous cell anal carcinoma, which represents the most common histologic form of the disease. Other types of cancers occurring in the anal region are addressed in other NCCN guidelines (i.e., anal adenocarcinoma and anal melanoma are managed according to the NCCN Clinical Practice Guidelines in Oncology on Rectal Cancer and Melanoma, respectively). Except where noted, the recommendations in these guidelines are classified as category 2A, meaning that uniform NCCN consensus was present among the panel based on lower-level evidence that the recommendation is appropriate. The panel unanimously endorses patient participation in a clinical trial over standard or accepted therapy. Risk Factors Anal carcinoma has been associated with human papilloma virus (HPV) infection (anal-genital warts); history of receptive anal intercourse or sexually transmitted disease; history of cervical, vulvar, or vaginal cancer; immunosuppression after solid organ transplantation or HIV infection; and smoking. Currently, the association between anal carcinoma and persistent infection with a high-risk form...
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Paul F. Engstrom, Juan Pablo Arnoletti, Al B. Benson III, Yi-Jen Chen, Michael A. Choti, Harry S. Cooper, Anne Covey, Raza A. Dilawari, Dayna S. Early, Peter C. Enzinger, Marwan G. Fakih, James Fleshman Jr., Charles Fuchs, Jean L. Grem, Krystyna Kiel, James A. Knol, Lucille A. Leong, Edward Lin, Mary F. Mulcahy, Sujata Rao, David P. Ryan, Leonard Saltz, David Shibata, John M. Skibber, Constantinos Sofocleous, James Thomas, Alan P. Venook and Christopher Willett

Colon Cancer 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 Colorectal cancer is the fourth most frequently diagnosed cancer and the second leading cause of cancer death in the United States. In 2009, an estimated 106,100 new cases of colon and 40,870 cases of rectal cancer will occur. During the same year, it is estimated that 49,920 people will die from colon and rectal cancer.1 Despite these statistics, mortality from colon cancer has decreased slightly over the past 30 years, possibly due to earlier diagnosis through screening and better treatment modalities. This manuscript summarizes the NCCN Clinical Practice Guidelines in Oncology for managing colon cancer. The guidelines begin with clinical presentation to the primary care physician or gastroenterologist and address diagnosis, pathologic staging, surgical management, adjuvant treatment, management of recurrent and metastatic disease, and patient surveillance. When reviewing these guidelines, clinicians should be aware of...
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Paul F. Engstrom, Juan Pablo Arnoletti, Al B. Benson III, Yi-Jen Chen, Michael A. Choti, Harry S. Cooper, Anne Covey, Raza A. Dilawari, Dayna S. Early, Peter C. Enzinger, Marwan G. Fakih, James Fleshman Jr., Charles Fuchs, Jean L. Grem, Krystyna Kiel, James A. Knol, Lucille A. Leong, Edward Lin, Mary F. Mulcahy, Sujata Rao, David P. Ryan, Leonard Saltz, David Shibata, John M. Skibber, Constantinos Sofocleous, James Thomas, Alan P. Venook and Christopher Willett

Rectal Cancer 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 In 2009 an estimated 40,870 new cases of rectal cancer will occur in the United States (23,580 cases in men; 17,290 cases in women). During the same year, an estimated 49,920 people will die from rectal and colon cancers.1 Although colorectal cancer is ranked as the fourth most frequently diagnosed cancer and the second leading cause of cancer death in the United States, mortality from colorectal cancer has decreased during the past 30 years. This decrease may be due to earlier diagnosis through screening and better treatment modalities. The recommendations in these clinical practice guidelines are classified as category 2A except where noted, meaning that there is uniform NCCN consensus, based on lower-level evidence (including...
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R. Michael Tuttle, Douglas W. Ball, David Byrd, Gilbert H. Daniels, Raza A. Dilawari, Gerard M. Doherty, Quan-Yang Duh, Hormoz Ehya, William B. Farrar, Robert I. Haddad, Fouad Kandeel, Richard T. Kloos, Peter Kopp, Dominick M. Lamonica, Thom R. Loree, William M. Lydiatt, Judith McCaffrey, John A. Olson Jr., Lee Parks, John A. Ridge, Jatin P. Shah, Steven I. Sherman, Cord Sturgeon, Steven G. Waguespack, Thomas N. Wang and Lori J. Wirth

Overview There are 3 main histologic types of thyroid carcinoma: differentiated (including papillary, follicular, and Hürthle), medullary, and anaplastic (aggressive undifferentiated tumor). Of 53,856 patients treated for thyroid carcinoma between 1985 and 1995, 80% had papillary, 11% had follicular, 3% had Hürthle cell, 4% had medullary, and 2% had anaplastic thyroid carcinoma.1 These NCCN guidelines focus on medullary thyroid carcinoma (MTC). Another NCCN guideline addresses papillary, follicular, Hürthle cell, and anaplastic thyroid carcinomas (see NCCN Clinical Practice Guidelines in Oncology: Thyroid Carcinoma [to view the most recent version of these guidelines, visit the NCCN Web site at www.NCCN.org]). MTC derives from the neuroendocrine parafollicular calcitonin-producing (C) cells of the thyroid.2–4 Sporadic MTC accounts for approximately 80% of all cases of the disease. The remaining cases consist of inherited tumor syndromes, such as multiple endocrine neoplasia type 2A (MEN 2A), which is the most common type; MEN 2B; or familial MTC.5,6 Sporadic disease typically presents in the fifth or sixth decade. Familial forms of the disease tend to present at earlier ages.2 Because the C cells are predominantly located in the upper portion of each thyroid lobe, patients with sporadic disease typically present with upper pole thyroid nodules. Metastatic cervical adenopathy appears in approximately 50% of patients at initial presentation. Symptoms of upper aerodigestive tract compression or invasion are reported by up to 15% of patients with sporadic disease.7 Symptoms from distant metastases in the lungs or bones occur in 5% to 10% of patients. The ability of the tumor to...
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R. Michael Tuttle, Douglas W. Ball, David Byrd, Raza A. Dilawari, Gerard M. Doherty, Quan-Yang Duh, Hormoz Ehya, William B. Farrar, Robert I. Haddad, Fouad Kandeel, Richard T. Kloos, Peter Kopp, Dominick M. Lamonica, Thom R. Loree, William M. Lydiatt, Judith C. McCaffrey, John A. Olson Jr., Lee Parks, John A. Ridge, Jatin P. Shah, Steven I. Sherman, Cord Sturgeon, Steven G. Waguespack, Thomas N. Wang and Lori J. Wirth

OverviewEpidemiologyThyroid nodules are approximately 4 times more common in women than in men. Palpable nodules increase in frequency throughout life, reaching a prevalence of approximately 5% in the United States population aged 50 years and older.1–3 Nodules are even more prevalent when the thyroid gland is examined at autopsy or surgery, or when using ultrasonography, and 50% of these have nodules, which are almost always benign.2,4 New nodules develop at a rate of approximately 0.1% per year beginning in early life, but at a much higher rate (∼2% per year) after exposure to head and neck irradiation.5,6By contrast, thyroid carcinoma is uncommon. For the United States population, the lifetime risk of being diagnosed with thyroid carcinoma is less than 1% (0.83% for women and 0.33% for men).7 Approximately 37,200 new cases of thyroid carcinoma were diagnosed in the United States in 2009.8As with thyroid nodules, thyroid carcinoma occurs 2 to 3 times more often in women than in men. With the incidence increasing by 6.2% per year, thyroid carcinoma is currently the sixth most common malignancy diagnosed in women.8 Among persons age 15 to 24 years, thyroid carcinoma accounts for 7.5% to 10% of all diagnosed malignancies.9–11 The disease is also diagnosed more often in white North Americans than in African Americans. Although thyroid carcinoma can occur at any age, the peak incidence from 2004 to 2006 was near age 45 to 49 years in women and 65 to 69 years in men.7Thyroid carcinoma has...
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Daniel G. Coit, Robert Andtbacka, Christopher K. Bichakjian, Raza A. Dilawari, Dominick DiMaio, Valerie Guild, Allan C. Halpern, F. Stephen Hodi, Mohammed Kashani-Sabet, Julie R. Lange, Anne Lind, Lainie Martin, Mary C. Martini, Scott K. Pruitt, Merrick I. Ross, Stephen F. Sener, Susan M. Swetter, Kenneth K. Tanabe, John A. Thompson, Vijay Trisal, Marshall M. Urist, Jeffrey Weber and Michael K. Wong

Melanoma 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. In 2008, an estimated 62,480 new cases of melanoma will have been diagnosed and approximately 8420 patients will have died of the disease in the United States.1 However, these projections for new cases may represent a substantial underestimation, because many superficial and in situ melanomas treated in the outpatient setting are not reported. The incidence of melanoma continues to increase dramatically. Melanoma is increasing in men more rapidly than any other malignancy and more rapidly in women than any other malignancy except lung cancer. For someone born in the United States in 2005, the lifetime risk for developing melanoma may be as high as 1 in 55.2 Melanoma ranks second to adult leukemia in terms of loss of years of potential life, per death. The median age at diagnosis is 59 years. Risk factors for melanoma include...
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Daniel G. Coit, Robert Andtbacka, Christopher J. Anker, Christopher K. Bichakjian, William E. Carson III, Adil Daud, Raza A. Dilawari, Dominick DiMaio, Valerie Guild, Allan C. Halpern, F. Stephen Hodi Jr., Mark C. Kelley, Nikhil I. Khushalani, Ragini R. Kudchadkar, Julie R. Lange, Anne Lind, Mary C. Martini, Anthony J. Olszanski, Scott K. Pruitt, Merrick I. Ross, Susan M. Swetter, Kenneth K. Tanabe, John A. Thompson, Vijay Trisal and Marshall M. Urist

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Al B. Benson III, J. Pablo Arnoletti, Tanios Bekaii-Saab, Emily Chan, Yi-Jen Chen, Michael A. Choti, Harry S. Cooper, Raza A. Dilawari, Paul F. Engstrom, Peter C. Enzinger, James W. Fleshman Jr., Charles S. Fuchs, Jean L. Grem, James A. Knol, Lucille A. Leong, Edward Lin, Kilian Salerno May, Mary F. Mulcahy, Kate Murphy, Eric Rohren, David P. Ryan, Leonard Saltz, Sunil Sharma, David Shibata, John M. Skibber, William Small Jr., Constantinos T. Sofocleous, Alan P. Venook and Christopher Willett

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Anal Carcinoma, Version 2.2012

Featured Updates to the NCCN Guidelines

Al B. Benson III, J. Pablo Arnoletti, Tanios Bekaii-Saab, Emily Chan, Yi-Jen Chen, Michael A. Choti, Harry S. Cooper, Raza A. Dilawari, Paul F. Engstrom, Peter C. Enzinger, Marwan G. Fakih, James W. Fleshman Jr., Charles S. Fuchs, Jean L. Grem, Lucille A. Leong, Edward Lin, Kilian Salerno May, Mary F. Mulcahy, Kate Murphy, Eric Rohren, David P. Ryan, Leonard Saltz, Sunil Sharma, David Shibata, John M. Skibber, William Small Jr., Constantinos T. Sofocleous, Alan P. Venook, Christopher Willett and Deborah A. Freedman-Cass

The workup and management of squamous cell anal carcinoma, which represents the most common histologic form of the disease, are addressed in the NCCN Clinical Practice Guidelines in Oncology (NCCN Guidelines) for Anal Carcinoma. These NCCN Guidelines Insights provide a summary of major discussion points of the 2012 NCCN Anal Carcinoma Panel meeting. In summary, the panel made 4 significant changes to the 2012 NCCN Guidelines for Anal Carcinoma: 1) local radiation therapy was added as an option for the treatment of patients with metastatic disease; 2) multifield technique is now preferred over anteroposterior-posteroanterior (AP-PA) technique for radiation delivery and the AP-PA technique is no longer recommended as the standard of care; 3) PET/CT should now be considered for radiation therapy planning; and 4) a section on risk reduction was added to the discussion section. In addition, the panel discussed the use of PET/CT for the workup of anal canal cancer and decided to maintain the recommendation that it can be considered in this setting. They also discussed the use of PET/CT for the workup of anal margin cancer and for the assessment of treatment response. They reaffirmed their recommendation that PET/CT is not appropriate in these settings.