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    View The Christ Hospital 2009 Cancer Program Annual View The Christ Hospital 2009 Cancer Program Annual Document Transcript

    • The Christ Hospital 2009 Cancer Program Annual Report www.TheChristHospital.com
    • The Christ Hospital 2009 Cancer Program Annual Report (reporting on 2008 data) Table of Contents: Introduction from the Cancer Collaborative Clinical Committee Chairman and Medical Director, Including Program Activities………………..................................................................................1 Membership Roster: Cancer Collaborative Clinical Committee……...…………………………..5 Oncology Registry Summary, Including Special Site Reports .…………………………………..6 • Thyroid……….…………………………………………………………..…..10 • Renal Cell…………………………………………………………………….16 2
    • From the Cancer Committee Chairman and Medical Director: As the chairman of the Cancer Collaborative Clinical Committee (CCCC) and as Medical Director of the Cancer Center, we would like to introduce our annual report of cancer activities. Another year has brought some significant contributions and major accomplishments in the quality of services for our cancer patients. As we prepare for another successful ACoS Survey in August 2010, the program is growing and improving continuously. In delivering the most up-to-date and comprehensive cancer treatment, the research program continued its focus on improving services. An average of 27 protocols were opened. There were 129 patients who received a full workup, but 4,281 were screened for inclusion in research studies. Out of these, 35 were placed in a study (27 percent). In addition, 69 accruals from other sites were able to be counted to meet and exceed the minimum requirement of four percent, for a total of 105 accruals (7.2%). This exceeds the six percent required to achieve Commendation status from ACoS. Implementation of the chart screening process to facilitate physician/staff awareness of potential opportunities relevant to each patient is continuing. For community education, melanoma became a focus, with local news coverage and distribution of materials at a Cincinnati Reds game, as well as free skin cancer screenings with a dermatologist. A genetic testing referral and counseling program is continuing. Twice a month on Fridays, a genetic counselor from Cincinnati Children’s Hospital’s human genetics department sees patients upon referral from a physician. More than 65 patients have been seen this year. Technology was enhanced this year in the field of radiation. A new image guided radiotherapy (IGRT) program called BrainLab was introduced and offered a plan for delivering a prescribed dose of radiation, while minimizing dose to surrounding healthy tissues. It has been used on multiple sites: brain, head & neck, and pelvis. A continuation in the improvement of the oncology information system (IMPAC) as well as diagnostic and planning upgrades were accomplished; and integration of MOSAIQ and Epic was established. On the medical oncology side, the new Epic (EMR) was implemented in May 2008, with the implementation of Beacon for treatment and supportive plans to be started in April 2009. A change to CADD pumps, for delivery of three-day continuous chemo, was implemented. This technology provides a safer administration for toxic drugs. Last year, our 30-bed inpatient unit (4 West) accomplished many goals. Clinically, the fall rate and medication error rates have decreased. A focus on education of nursing staff continues to be a priority. Between the annual “Intro to Oncology Nursing Course” in October, a chemotherapy and biotherapy course, oncology certification review course, skills day, the critical care internship program and the “Updates in Oncology” seminar, new and experienced nurses are given excellent educational forums to learn. Evaluations of the courses maintain an excellent rating. With the rapidly changing environment of drugs to treat cancer, chemo certification of the staff is an evolving process. ONS PEP (putting evidence into practice) cards were purchased as a resource to facilitate the nurses’ use of evidence-based practice in symptom management. Both the inpatient and outpatient nursing staff experienced minimal turnover this year. In the Cancer Center, 14 out of 18 nurses have maintained oncology certification. On 4 West, there are seven nurses who have maintained this certification, with one being an advanced oncology certified nurse (AOCN). Patient satisfaction is extremely important to the dedicated professional 3
    • staff. For this year, the two outpatient clinical departments average a 93.65 rating for five focused areas. The hospital goal is 90 percent. The inpatient satisfaction had an overall average of 83.4 for five focused areas. It trended lower than the hospital’s average for the same five areas. Community outreach projects continue to provide prevention, education and support for patients and families. The American Cancer Society’s quarterly program, “Look Good, Feel Better,” was hosted by the Cancer Center and was well attended. Housing referrals are made on a regular basis to the American Cancer Society’s Hope Lodge, which is just a ten minute drive from the hospital. The Cancer Corner continues utilizing the American Cancer Society’s Cancer Awareness Calendar, highlighting topics related to cancer treatment–for example, nutrition, care giving, and palliative care as a monthly educational tool. In addition, an agreement with the hospital and the Cincinnati Sports Club provided an opportunity for more community educational programs for skin, breast and colorectal cancers. The weekly cancer conferences (tumor boards) presented 136 cases with the appropriate CCCC- directed disciplines in attendance. There were 88 breast cancer cases with the appropriate disciplines at the weekly Multidisciplinary Breast Conferences with an average of 6.7 physicians attending. Overall the evaluations were positive. Ninety percent strongly agreed that the facility and set-up were conducive to learning; in addition, 90 percent strongly agreed that the presentation was relevant. The third Tuesday of every month is dedicated to colorectal cancer cases, which are led by Dr. Janice Rafferty. The staging rate of the hospital decreased to 93 percent by the end of the year (this was down three percent from the previous year). Education of the professional staff is a focus to maintain the highest quality of care for our patients. Nurses, tumor registrars, research nurses and research associates, physicists, and radiation therapist attended national meetings, and tumor registrars, managers and nurses attended local meetings. Three out of the four CTRs have attained certification. We have chosen two interesting site studies--thyroid cancer and renal cell carcinoma. Both studies had 395 and 184 cases respectfully. In closing, we would like to thank the many dedicated staff who work with the cancer program and continue to improve the programs and services offered to our patients, families and the broader community each year. Philip Leming, M.D. Robert Cody, M.D. Chairman, Cancer Clinical Collaborative Committee Medical Director, Cancer Center 4
    • 2008 Cancer Collaborative Clinical Committee Roster The Cancer Committee is a multi-disciplinary team of physicians and health care professionals who meet five times per year to develop and improve The Christ Hospital Cancer Program services. Name Specialty Philip Leming, M.D.-chairman Hematology Oncology Helmut Schellhas, M.D. Gynecology Oncology Leeann Budde, M.D. Hematology/Oncology James Cornwell, M.D. Radiology James Essell, M.D. Hematology/Medical Oncology Robert Cody, M.D. Hematology/Oncology Rodney Geier, M.D. Radiation Oncology Blake Nestok, M.D. Pathology Martin Popp, M.D. General Surgery Kathryn Weichert, M.D. Radiation Oncology Janet Milne, R.N. Nurse Practitioner, Palliative Care William Baltes Staff Pharmacist Connie Cook Director, Cancer Center Victor DiPilla V.P., Administration Hyla Goddard Medical Support Staff Cathy Greene Clinical Manager, 4 West Joan Burkhart Certified Tumor Registrar Brandon Fletcher Director, Research Mike Shook Manager, Radiation Oncology Mary Tyle Social Worker, Cancer Center Lou Ann Swan Quality & Utilization Review Megan Rader Clinical Dietician Anne Henry American Cancer Society Stephanie Meade Divisional Director, Nursing 5
    • Oncology Registry According to the National Cancer Registrars Association, a Tumor Registry (Cancer Registry) is defined as an information system designed for the collection, management and analysis of data on persons with the diagnosis of a malignant or neoplastic disease. Tumor registries can be classified into three general types: o Healthcare institution registries, which maintain data on all patients diagnosed and/or treated for cancer at their facility. Healthcare facilities report cancer cases to the central or state registry as required by law. o Central registries are population based registries that maintain data on all cancer patients within certain geographical areas o Special purpose registries maintain data on a particular type of cancer, such as brain tumors. The Tumor Registry staff at The Christ Hospital maintains a summary of each cancer patient diagnosed and/or treated at its facility. This includes demographics, cancer identification, treatment, and follow-up data, which contribute to treatment planning, staging and continuity of care. The registry is a vital resource for outcomes research and evaluation of the quality of care provided to cancer patients. This information is collected by registrars who strive to ensure that timely, accurate and complete data is incorporated and maintained on all types of cancer. For 2008, the Tumor Registry accessioned 1,496 cases. Of these, 1,401 are analytic cases, meaning they were originally diagnosed or treated at The Christ Hospital. There were 95 non- analytic cases reported and they had a subsequent diagnosis or treatment at this facility. These cases are reported to the Ohio Cancer Incidence and Surveillance System of the Ohio Department of Health and to the National Cancer Data Base. The top five sites reported by this registry for 2008 include breast, colon/rectum, lung, prostate and thyroid. The Tumor Registry participates in studies when requested by the National Cancer Data Base. Under the direction of the Cancer Clinical Collaborative Committee, two site studies (kidney and thyroid) were completed. In addition to reporting data, the registry staff co-ordinates Tumor Board. The Tumor Board is held each Tuesday at 7:30 a.m. in Classroom 3 at the hospital. A site specific Breast Conference is held every Friday at 7 a.m. in the Cancer Center Conference Room. The registry is staffed by three certified tumor registrars, one additional registrar, a registry assistant, and a follow-up clerk. Since continuing education is key in staying abreast of new developments in the oncology field and maintaining certification, the registrars attend webinars, and national, state, and local education meetings. They are also actively involved with their national, state, and local organizations. They have taken part in making meals for the American Cancer Society’s HOPE Lodge in Cincinnati through the Greater Cincinnati Cancer Registrars Association and assisted with an educational workshop held at Mercy Anderson Hospital in April 2009. Jennifer Karaus served as Treasurer of the Greater Cincinnati Cancer Registrars Association in 2009, and Joan Burkhart served as Vice President of the Ohio Cancer Registrars Association in 2009. 6
    • The Tumor Registry Staff at The Christ Hospital are: Joan Burkhart, CTR Jennifer Karaus, CTR Uraiwan (Toy) Lukes, CTR Jaimee Woods, Tumor Registrar Shirley Spears, Registry Assistant Megan Quinlan-Waters, Follow-up Data Clerk ANALYTIC CASES THE CHRIST HOSPITAL PRIMARY SITE TOTAL SEX STAGE GROUP M F 0 I II III IV UNK N/A 59 80 7 40 32 20 18 ALL SITES 1401 2 9 9 5 8 4 3 90 112 ORAL CAVITY 22 16 6 0 3 4 1 7 7 0 LIP 0 0 0 0 0 0 0 0 0 0 TONGUE 7 5 2 0 0 1 0 3 3 0 OROPHARYNX 2 2 0 0 0 1 0 1 0 0 HYPOPHARYNX 1 1 0 0 0 0 0 0 1 0 OTHER 12 8 4 0 3 2 1 3 3 0 14 1 DIGESTIVE SYSTEM 265 4 121 0 48 62 67 45 19 14 ESOPHAGUS 16 14 2 0 1 2 6 4 3 0 STOMACH 13 8 5 0 3 1 1 5 3 0 COLON 94 42 52 6 22 26 21 16 2 1 RECTUM 77 47 30 4 17 21 24 5 4 2 ANUS/ANAL CANAL 6 5 1 0 0 3 3 0 0 0 LIVER 3 2 1 0 1 0 0 1 1 0 PANCREAS 31 13 18 0 2 5 9 11 3 1 OTHER 25 13 12 0 2 4 3 3 3 10 RESPIRATORY SYSTEM 183 88 95 0 53 15 35 62 14 4 NASAL/SINUS 1 1 0 0 1 0 0 0 0 0 LARYNX 14 11 3 0 7 2 2 1 2 0 LUNG/BRONCHUS 164 73 91 0 44 13 33 58 12 4 OTHER 4 3 1 0 1 0 0 3 0 0 BLOOD & BONE MARROW 20 10 10 0 0 0 0 0 0 20 LEUKEMIA 6 2 4 0 0 0 0 0 0 6 MULTIPLE MYELOMA 13 8 5 0 0 0 0 0 0 13 OTHER 1 0 1 0 0 0 0 0 0 1 BONE 3 3 0 0 1 0 0 1 1 0 7
    • CONNECT/SOFT TISSUE 6 1 5 0 1 1 1 1 2 0 SKIN 76 47 29 11 38 6 11 4 4 2 MELANOMA 73 45 28 11 38 6 11 3 4 0 OTHER 3 2 1 0 0 0 0 1 0 2 BREAST 261 4 257 46 92 74 25 9 14 1 13 FEMALE GENITAL 132 0 2 4 65 4 32 8 13 6 CERVIX UTERI 8 0 8 0 5 0 0 2 1 0 CORPUS UTERI 74 0 74 0 46 1 12 1 8 6 OVARY 37 0 37 0 11 2 17 5 2 0 VULVA 9 0 9 4 2 1 2 0 0 0 OTHER 4 0 4 0 1 0 1 0 2 0 16 14 MALE GENITAL 162 2 0 0 6 2 3 7 4 0 PROSTATE 156 156 0 0 2 142 1 7 4 0 TESTIS 6 6 0 0 4 0 2 0 0 0 OTHER 0 0 0 0 0 0 0 0 0 0 URINARY SYSTEM 65 44 21 8 29 6 9 8 5 0 BLADDER 32 26 6 8 14 4 3 1 2 0 KIDNEY/RENAL 33 18 15 0 15 2 6 7 3 0 OTHER 0 0 0 0 0 0 0 0 0 0 BRAIN & CNS 21 9 12 0 0 0 0 0 0 21 BRAIN (BENIGN) 1 1 0 0 0 0 0 0 0 1 BRAIN (MALIGNANT) 10 4 6 0 0 0 0 0 0 10 OTHER 10 4 6 0 0 0 0 0 0 10 ENDOCRINE 94 25 69 0 62 9 10 8 2 3 THYROID 91 23 68 0 62 9 10 8 2 0 OTHER 3 2 1 0 0 0 0 0 0 3 LYMPHATIC SYSTEM 50 25 25 0 7 5 10 23 5 0 HODGKIN'S DISEASE 7 5 2 0 1 1 2 2 1 0 NON-HODGKIN'S 43 20 23 0 6 4 8 21 4 0 UNKNOWN PRIMARY 29 13 16 0 0 0 0 0 0 29 OTHER/ILL-DEFINED 12 1 11 0 0 0 0 0 0 12 8
    • Thyroid Study Approximately, one in 20 thyroid nodules are cancerous. The two most common types of thyroid cancer are papillary carcinoma and follicular carcinoma. Hurthle cell carcinoma is a subtype of follicular carcinoma. Medullary thyroid carcinoma, anaplastic carcinoma, and thyroid lymphoma all occur less often. Papillary carcinoma accounts for about eight out of 10 thyroid cancers. These typically are slow growing and usually develop in only one lobe of the thyroid gland. However, they can occur in both lobes and spread to lymph nodes in the neck. Most of the time, these can be successfully treated and are rarely fatal. One variant of papillary carcinoma is follicular variant, which is the most common variant. Papillary and follicular variant thyroid carcinoma have the same outlook for survival, and treatment is the same for both. Other variants including columnar, tall cell and diffuse sclerosis are not as common and tend to grow and spread more quickly. Follicular carcinoma is the next most common type and is more common in countries where people do not get enough iodine in their diet. These cancers usually remain in the thyroid gland, usually don’t spread to lymph nodes, but can spread to the lungs or bones. The prognosis for follicular carcinoma is probably not as good as papillary carcinoma; however, it is still very good in most cases. Hurthle cell carcinoma is a type of follicular carcinoma. This subtype is harder to find and treat as it is less likely to absorb radioactive iodine. Medullary thyroid carcinoma develops from the C cells of the thyroid gland. This cancer can metastasize before a thyroid nodule is discovered. Medullary cancer does not take up radioactive iodine, so prognosis is not as good as it is for other thyroid cancers. There are two types of Medullary thyroid carcinoma. The first type is sporadic Medullary thyroid carcinoma and is not inherited. The other type is familial medullary carcinoma; this type is inherited and can occur in each generation of a family. These often develop during childhood or early adulthood and can spread early. Patients usually have cancer in both thyroid lobes and in several areas of each lobe. Anaplastic carcinoma is a rare form of thyroid cancer. It is thought to sometimes develop from an existing papillary or follicular cancer. This is an aggressive cancer that rapidly invades the neck, often spreads to other parts of the body, and is very hard to treat. Risk factors for thyroid cancer include a diet low in iodine, exposure to radiation, hereditary conditions, diagnosis of another thyroid cancer, gender, and age. Thyroid cancer occurs about three times more often in women than men. Most cases are diagnosed in people between the ages of 20 and 60. Researchers have identified two genetic variations that account for 57 percent of cases of thyroid cancer. This finding could lead to earlier detection among people at high risk for the disease. The report states that two variants each lie at a site on the human genome near genes that control development of the thyroid gland. The variants are changes in a single chemical unit of the genome, which is three billion units in length. Compared with people who have neither variant, the risk associated with these variants is almost six fold. 9
    • The study was performed by an Icelandic company. They replicated the findings in two other populations of European descent; one study took place in Columbus, Ohio and one in Spain. About four percent of people of European descent carry both variants. There was no information on other ethnic groups. It was found that the subjects with two variants had lower levels of thyroid-stimulating hormone in their bloodstream. That hormone, produced by the pituitary gland, directs the thyroid gland to generate its own hormones. The thyroid stimulating hormone also makes cells of the thyroid gland mature. It is conceivable that in people with the double variants, the thyroid cells were not properly differentiated, and that the immature cells might be the cause of cancer. If so, supplying extra thyroid-stimulating hormone could make the cells mature and reduce the cancer risk. However, this is still just speculation. About 300,000 patients in the United States and 200,000 people in Europe now live with thyroid cancer. All of these patients require lifelong surveillance, so any change in follow-up paradigms would likely affect many people. Studies show that the role of diagnostic whole body scans has been seriously challenged, mainly based on low sensitivity and high cost. However, retrospective studies demonstrate that up to 95 percent of patients with recurrent/persistent thyroid cancer will have either a positive diagnostic whole body scan or TSH-stimulated thyroglobulin of more than 2 ng/mL with either hypothyroid withdrawal or recombinant human thyrotropin-alpha preparation. Staging for thyroid cancer differs from other head and neck sites because it is not based entirely on the anatomic extent of disease. The histological diagnosis and the age of the patient are such important factors that they are both included in the staging of this disease. The age factor determination for staging of this disease is 45 years of age. A patient under 45 years of age is considered Stage 2, even with an M1 stage. At The Christ Hospital, between the years of 2004 and 2008, there were 395 thyroid cancers diagnosed and/or treated; there are 282 Stage 1, 32 are Stage 2, 38 are Stage 3 and 25 are Stage 4. Eighteen cases had an unknown stage. Histology varied within this group. However, among the 395 patients with papillary carcinoma, follicular variant was the diagnosis for 162 or 41 percent of patients and papillary carcinoma of the thyroid was the histology for 137 or 35 percent of patients. The remaining histologies ranged from papillary carcinoma, nos to insular carcinoma. 10
    • Thyroid Cancer Study Histology 180 162 160 137 140 120 Cases 100 80 60 26 40 14 11 10 10 6 20 6 4 2 2 1 1 1 1 1 0 pa pa no pa pa fo m fo ox pa pa ca in ca sq pa m l li ed ca, l li su ed pi pi ne yp ,n ,a ua pi pi pi pi pi cu cu ll a ll a la ll a la ll a ul ll a ll a u a , oy hi nc os na m la la ry rc ar ry ry ry li c ry ry ry ou ll a ap pl r ra ry a ca ca ca ca ad ca m ad as s ry su de ca ,f ic ce ,n ,c ti c en ,e ca en la m ro ol no rc ol ll c os ,n te nc oc in oc ca li c w in ph ca um ted d im a os a ap ith o m va ar ul rc sc rc il ic ,n na ar al ci su in am in le a os ly no om rc ce om va la ro in yl m ll ria el si a oi a, a l ng d nt si no st ve ca s ro m a In this population, there is an overwhelming number of women versus men. There are 303 (77%) females and 92 (23%) males. When race is compared, there are 326 white patients, which is 82 percent of the population; 35 black patients or 9 percent; 33 unknown; and one Asian, which is the remaining 9 percent. Thyroid Cancer Study Cases & Genders 303 350 300 250 200 Cases 92 150 100 50 0 MALE FEMALE 11
    • ASIAN INDIAN PAKISTANI, 1 UNKNOWN, 11 OTHER, 22 WHITE, 326 BLACK, 35 Surgery is the first step in treating thyroid cancer. Some patients are treated with radioactive iodine after surgery. The radioactive iodine is absorbed by the thyroid and kills remaining cancer cells in the thyroid tissue. Other options can include external radiation and hormone therapy. Thyroid cancers tend not to respond well to chemotherapy although this may be used in some cases. This population shows that 98 percent or 389 patients were treated with surgery. Radioactive iodine was given additionally to 39 percent, or 150 of these patients. Survival is compared to the National Cancer Data Base. The national overall five-year survival is 92.6 percent, while The Christ Hospital shows overall survival at 94 percent. When compared by stage, it is noteworthy that at The Christ Hospital five-year survival for Stage III is at 100 percent. There are 18 patients in this timeframe who have Stage III thyroid cancer and all are alive after five years. This compares with NCDB Stage III, which is 85.4 percent. 12
    • O B S E R VE D S UR VIVA L T C H & N C D B 100.0 98.0 96.0 94.0 TCH NCDB 92.0 90.0 88.0 1 2 3 4 5 6 THE CHRIST HOSPITAL OBSERVED SURVIVAL CHART 100.0 STAGE I & III STAGE II 90.0 80.0 70.0 60.0 50.0 40.0 STAGE VI 30.0 20.0 10.0 0.0 BEGIN % YEAR 1 YEAR 2 YEAR 3 YEAR 4 YEAR 5 13
    • Kidney Cancer According to the American Cancer Society, there will be about 57,760 new cases of kidney cancer diagnosed in the United States in 2009 and approximately 12,980 people will die from this disease. These statistics include both renal cell cancers and transitional cell carcinomas of the renal pelvis. Overall, the lifetime risk of developing kidney cancer is about one in 75; the risk is higher in men than women. The rate of people developing kidney cancer has been rising slowly since the 1970s. This rate, at least in part, is probably due to the development of CT scans, which have picked up some cancers that may never have been found otherwise. The death rates have remained fairly stable since the mid 1980s. Researchers are studying several genes that may play a part in changing normal kidney cells into renal cell carcinoma. Renal cell carcinoma accounts for approximately three percent of adult malignancies and 90-95 percent of neoplasms arising from the kidney. It is characterized by a lack of early warning signs, diverse clinical manifestations, resistance to radiation and chemotherapy and infrequent, but reproducible, responses to immunotherapy agents, such as interferon alpha and interleukin (IL)-2. New agents such as sorafenib and sunitinib, having anti- angiogenic effects through targeting multiple receptor kinases, have activity in patients failing immunotherapy. The tissue of origin for renal cell carcinoma is proximal renal tubular epithelium. Renal cancer occurs in both sporadic (nonhereditary) and hereditary form, and both forms are associated with structural alterations of the short arm of chromosome 3 (30). Genetic studies of the families at high risk for developing renal cancer led to the cloning of genes, whose alteration results in tumor formation. These genes are either tumor suppressors (VHL, TSC) or oncogenes (MET). At least four hereditary syndromes associated with renal cell carcinoma are recognized: (1) von Hippel-Lindau (VHL) syndrome; (2) hereditary papillary renal carcinoma (HPRC); (3) familial renal oncocytoma (FRO) associated with Birt-Hogg-Dube syndrome (BHDS); and (4) hereditary renal carcinoma (HRC). Von Hippel-Lindau disease is transmitted in an autosomal dominant familial multiple-cancer syndrome, which confers predisposition to a variety of neoplasms, including the following: o Renal cell carcinoma with clear cell histological features o Pheochromocytoma o Pancreatic cysts and islet cell tumors o Retinal angiomas o Central nervous system hemangioblastomas o Endolymphatic sac tumors o Epididymal cystadenomas Renal cell carcinoma develops in nearly 40 percent of patients with von Hippel-Lindau disease and is a major cause of death among these patients. Deletions of 3p occur commonly in renal cell carcinoma associated with VHL disease. The VHL gene is mutated in a high percentage of tumors and cell lines from patients with sporadic (nonhereditary) clear cell renal carcinoma. 14
    • Several kindred, with familial clear cell carcinoma, have a constitutional balanced translocation between 30 and either chromosome 6 or chromosome 8. Mutations of the VHL gene result in the accumulation of hypoxia inducible factors (HIFs) that stimulate angiogenesis through vascular endothelial growth factor and its receptor (VEGF and VEGFR respectively). VEGF and VEGFR are important new therapeutic targets. Hereditary papillary renal carcinoma is an inherited disorder with an autosomal dominant inheritance pattern. Affected individuals develop bilateral, multifocal papillary renal carcinoma. Germline mutations in the tyrosine kinase domain of the MET gene have been identified. Individuals affected with familial renal oncocytoma can develop bilateral, multifocal oncocytoma of oncocytic neoplasms in the kidney. Birt-Hogg-Dube syndrome is a hereditary cutaneous syndrome. Patients with Birt-Hogg-Dube syndrome have a dominantly inherited predisposition to develop benign tumors of the hair follicle (fibrofolliculomas), predominantly on the face, neck and upper trunk, and are at risk for developing renal tumors, colonic polyps or tumors, and pulmonary cysts. Renal cell carcinomas are more common in people of Northern European ancestry and North Americans than in those of Asian or African descent. In the United States, the incidence among African Americans is increasing rapidly. Renal cell carcinoma is more common in males and occurs mostly in people aged 40 through 60. However, the disease has been reported in younger people, especially younger people with family clusters. Renal cell carcinoma can remain clinically occult for most of its course. Twenty-five to 30 percent of patients are asymptomatic and their renal cell carcinomas are found incidentally on radiologic studies. AJCC Staging for renal cell carcinoma is as follows 1. Stage 1 – Tumor 7 cm or smaller in greatest dimension limited to the kidney, no lymph nodes, no distant metastasis 2. Stage 2 – Tumor larger than 7 cm, limited to the kidney, no lymph nodes, no distant metastasis 3. Stage 3 – Tumor any size, limited to the kidney, metastasis to a single regional lymph node and no distant metastasis or tumor extending into major veins or invades adrenal gland or perinephric tissues but not beyond Gerota fascia, either no nodes or 1 single node involved and no distant metastasis 4. Stage 4 – Tumor invading beyond Gerota fascia, or tumor any size with metastasis in more than one regional lymph node and no distant metastasis, or tumor of any size or extension, with or without lymph node involvement and distant metastasis. At the Christ Hospital, 184 patients were diagnosed and/or treated for kidney cancer from 2004 through 2008. Of these, 118 are Stage I, 11 are Stage II, 20 are Stage III and 32 are Stage IV. One case was not stageable and two had stages that were unknown. 15
    • 118 120 100 80 60 Cases 32 40 20 11 1 2 20 0 1 2 3 4 88 99 Stage The histologies vary from carcinoma, nos to synovial sarcoma. The predominant histologies include renal cell carcinoma and clear cell carcinoma. There are a total of 128 patients with these histologies. 80 72 70 56 60 50 35 40 Ca se s 30 20 7 4 2 10 2 2 1 1 1 1 0 There are 103 men and 81 women. One hundred thirty-two are Caucasian and 43 African American. There are nine where the race was not recorded. 16
    • Male Female Number of Cases by Sex The overall survival compares favorably to the National Cancer Data Base (NCDB). At the Christ Hospital (TCH) for the years 1998 – 2001, the overall survival is 54.1 percent compared to the NCDB overall survival for the same time period being 56.2 percent. T CH Survival R ate by Stage Overall Observed Survival N CDB & T CH 120 120 100 100 80 STAGE 1 80 TCH 60 STAGE 2 NCDB 60 40 STAGE 3 40 20 STAGE 4 20 0 0 1 2 3 4 5 1 2 3 4 5 Years from Diagnosis Years from D iagnosis Survival by stage shows NCDB survival is higher on Stage 1 at 80 percent versus TCH at 69.2 percent. Stage 2 shows NCDB to be at 73.5 percent versus TCH 64.8 percent. Stage 3 survival per NCDB is 52.5 percent versus TCH at 64.8 percent and Stage 4 shows NCDB at 7.3 percent versus TCH at 4.5 percent. Survival for Stage 4 renal cell cancers for 2004 at The Christ Hospital was studied. This five- year survival data is at 37.5 percent. There are eight patients with Stage 4 renal cell cancers with three still alive in 2009. The high survival rate could be due in part to small sample size or possibly to the discovery of new therapies. The Stage 4 patients from 2004 through 2008 were checked to see if NCCN guidelines were followed. All 30 patients received nephrectomy, where applicable. They additionally received or were recommended to receive radiation and/or chemotherapy and/or immunotherapy. When nephrectomy was not possible, patients received radiation or chemotherapy or immunotherapy or were referred to hospice programs. The cancer research department at The Christ Hospital is currently participating in three renal cell carcinoma trials. These include a dose-escalation Study of MDX-1106, ECOG 2804 Phase II Study, and ECOG 2805 ASSURE (Adjuvant Sorafenib or Sunitinib for unfavorable renal carcinomas). 17
    • www.TheChristHospital.com 513-585-2000 18