Adrenocortical Carcinoma

Welcome to our web site dedicated to patients, caregivers, physicians, and researchers whose interest is the rare cancer adrenocortical carcinoma. We hope you find this site helpful and informative.

DISCLAIMER - Data and web links found on this site are intended for information and educational purposes only. This information is not intended to take the place of or to provide medical treatment decisions. Those decisions are the sole responsibility of patients and their physicians.

What is Adrenocortical Carcinoma?

Cancer of the adrenal cortex, (a rare cancer-approximately 1 in 2,000,000 cancer occurrence) is a disease in which cancer (malignant) cells are found in the adrenal cortex, which is the outside layer of the adrenal gland. Cancer of the adrenal cortex is also called adrenocortical carcinoma. There are two adrenal glands, one above each kidney in the back of the upper abdomen. The adrenal glands are part of the endocrine system.

Wait and See after Surgery for Adrenocortical Carcinoma?

In my opinion, the rare cancer Adrenocortical Carcinoma is a cancer that does not lend itself to a "wait and see" approach after surgical removal of an Adrenocortical Carcinoma tumor. I believe that patients with this cancer should consider: consultation at a National Cancer Institute Center of Excellence that specializes in Adrenocortical Carcinoma treatment; follow-on treatment therapy; frequent scans (every 2-3 months); and frequent clinical follow up by an oncologist familiar with treatment of Adrenocortical Carcinoma. Re-surgery should be considered where that is an option if there is a re-occurrence.

Cytotoxic Agents for Consideration for Adrenocortical Carcinoma

There are a number of cytotoxic agents that have shown anti-proliferative effects with this cancer. Some of the more commonly used are: Mitotane; Cisplatin; Cisplatin/VP16 (Etoposide); Doxorubicin (Adriamycin); Cisplatin/Doxorubicin; Gemzar(Gemcitabine); Doxorubicin/Vincristine/VP16; 5-Fluorouracil; and Cyclophosphamide. Taxol(Paclitaxel)has shown activity with this cancer and therefore the related drug Taxotere(Docetaxel) may be of benefit also. Mitotane is also used in combination with some of the above cytotoxic agents.

In the following presentation abstract at the American Society of Clinical Oncology Meeting 2003 which is the final report of this Multi-Center Trial - Multicenter phase II study of mitotane associated with etoposide, doxorubicin and cisplatin in the treatment of advanced adrenocortical carcinoma. Year: 2003 Printable Version Abstract No: 1573 Category: Other Genitourinary Cancer Author(s): L. Dogliotti, P. Sperone, A. Berruti, M. Terzolo, A. Pia, G. Gorzegno, A. Angeli, for the Adrenal Cancer International Study Group; Oncologia Medica Azienda Ospedaliera San Luigi, Orbassano, Italy; Medicina Interna Azienda Ospedaliera San Luigi, Orbassano, Italy; - the authors report that - Treatment consisted of Etoposide 100 mg/mq on days 5-7, Doxorubicin 20 mg/mq, on days 1 and 8, Cisplatin 40 mg/mq on days 1 and 9, administered intravenously every 4 weeks, in association with 4 g/day of oral mitotane
(or the maximum tolerated dose) without any interruption between chemotherapy cycles. They further report that - According to WHO criteria, a complete response was achieved in 3/66 (5%) patients and partial response in 29/66 (44%), for an overall response rate of 49%. Stable disease was observed in 15(23%) patients and progressive disease in 19 (29%). It is recommended that oncologists consider the significance of the reported results of this trial.

The combination of Streptozocin (Zanosar) and Mitotane (o,p'DDD) has shown activity with this cancer. That protocol is call SO therapy. Suramin and Gossypol show activity with this cancer but these substances need further study.

Overexpressions for possible targeted biological agents in adrenocortical carcinoma.

* In my opinion, it is significant for oncologists treating adrenocortical carcinoma to note that medical abstracts from PubMed indicate that at least 80% of adrenocortical carcinoma tumors are moderate to strongly positive for Epidermal Growth Factor Receptor (EGFR). One study indicated that 94% of the adrenocortical carcinoma tumors were positive for EGFR. One Ref. PubMed Abstract: Hum Pathol 1990 Mar;21(3):277-82 Immunohistochemical expression of epidermal growth factor receptors in human adrenocortical carcinoma.
Kamio T, Shigematsu K, Sou H, Kawai K, Tsuchiyama H. Department of Pathology 2, Nagasaki University School of Medicine, Japan.

**Another prominent overexpression in adrenocortical carcinoma is IGF2 associated with gene mutations at location 11p15.5. Ref. Adrenocortical Carcinoma Genetics link below for this and other known overexpressions and genetics mutations associated with adrenocortical carcinoma.

See the NCI Site below and other relevant links for more information about adrenocortical carcinoma.

Support the Adrenocortical Carcinoma Research Fund at the University of Texas M.D. Anderson Cancer Center.

This fund has been established for adrenocortical carcinoma site specific research at M.D. Anderson Cancer Center a recognized center of excellence for this rare cancer. This fund supports the research of Associate Professor Dr. Andrzej P. Kudelka, M.D. and his associates at the M.D. Anderson Cancer Center who are involved in adrenocortical carcinoma clinical treatment and research.

To contribute to this fund make check payable to M.D. Anderson Cancer Center **Important - mark for Adrenocortical Carcinoma Research Fund and send contributions to:

Adrenocortical Carcinoma Research Fund
Attention: Nan Duhon
The University of Texas
M. D. Anderson Cancer Center
Development Office
P. O. Box 297153
Houston, TX 77297-0153

To contact Ms. Duhon (only for contributions not medical information)

Tel: 713/563-4033 email: nduhon@mdanderson.org

To contact Dr. Andrzej P. Kudelka at M.D. Anderson email: akudelka@mdanderson.org

Information about The University of Texas M.D. Anderson Cancer Center.

Links to other centers of excellence that treat and research adrenocortical carcinoma.

To read an inspirational book by an adrenocortical carcinoma survivor please visit Amazon.com or Barnes & Noble on-line and order: Finding Hope When Doctors Say There Is None: Surviving Cancer in the Harsh World of HMO Medicine -- F.H. Scribner

Review of Mr. Scribner's book

Inspirational and Spoken from the Heart, May 7, 2001 Reviewer: Richard Beverlin, Jr. from Kemah, Texas

Fred Scribner's book "Finding Hope" is a truly inspirational work which is spoken from the heart and from his life experiences battling a rare cancer and HMO medicine simultaneously! This book is must reading for patients, caregivers, physicians, health insurance executives, and elected officials who have an interest in rare cancer treatment funding and health care issues. I provide this review from the viewpoint of a spouse of a rare cancer patient and a scientist/researcher who has an interest in novel cancer treatment and funding for rare cancer research. My wife and I have faced many of the author's struggles and trials-including hearing that there is-"no cure or little hope for curing this cancer." My wife is battling the same rare cancer-adrenocortical carcinoma that Mr. Scribner has battled these many years! Most importantly, this book is a reminder that God not man controls our time on this earth. It reminds us of the importance of reaching out to help others in any way we can with our knowledge and caring. I recommend you put down the novel, and take time to read this inspirational book.