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Histopathologic Staging at Initial Diagnosis of Mycosis Fungoides and the Sézary Syndrome: Definition of Three Distinctive Prognostic Groups

Edward A. Sausville, MD, PhD; Joyce L. Eddy, RN; Robert W. Makuch, PhD; A. B. Fischmann, MD; Geraldine P. Schechter, MD; Mary Matthews, MD; Eli Glatstein, MD; Daniel C. Ihde, MD; Frederic Kaye, MD; Stephen R. Veach, MD; Ruby Phelps; Terry O'Connor, MPH; Jane B. Trepel; James D. Cotelingam, MD; Adi F. Gazdar, MD; John D. Minna, MD; and Paul A. Bunn Jr., MD
[+] Article, Author, and Disclosure Information

The opinions or assertions in this article are the private views of the authors and are not to be construed as official or as reflecting the views or policies of the Department of the Navy or the Department of Defense.

Grant Support: Partial support by grant 5P30-CA-16359 from the National Cancer Institute.

Requests for Reprints: Edward A. Sausville, MD, PhD, Vincent T. Lombardi Cancer Center, Georgetown University Medical Center, 3800 Reservoir Road, N.W., Washington, DC 20007.

Current Author Addresses: Dr. Sausville: Georgetown University Medical Center, Vincent T. Lombardi Cancer Center, Washington, DC 20007.

Ms. Eddy, Phelps, and Trepel; Drs. Ihde, Kaye, Gazdar, and Minna: Navy Medical Oncology Branch; Dr. Glatstein: Radiation Oncology Branch; Dr. Veach: Department of Medicine/Hematology and Oncology; and Dr. Cotelingam: Box 213; National Cancer Institute, Naval Hospital, Bethesda, MD 20814.

Drs. Fischmann and Schechter: Veterans Administration Medical Center, Washington, DC 20422.

Drs. Makuch and Ms. O'Connor: Division of Biostatistics, Yale University School of Medicine, New Haven, CT 06510.

Dr. Bunn: Division of Medical Oncology, University of Colorado Health Sciences Center, Denver, CO 80262.

From the National Cancer Institute, National Institutes of Health, U.S. Naval Hospital, and Uniformed Services University of the Health Sciences, Bethesda, Maryland; Georgetown University, Veterans Administration Medical Center, and George Washington University, Washington, D.C.; Yale University School of Medicine, New Haven, Connecticut; and University of Colorado Health Sciences Center, Denver, Colorado. For current author addresses, see end of text.†Deceased. To whose memory this paper is dedicated.

© 1988 American College of PhysiciansAmerican College of Physicians

Ann Intern Med. 1988;109(5):372-382. doi:10.7326/0003-4819-109-5-372
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Study Objective: To determine the optimal staging evaluation at the time of initial diagnosis of mycosis fungoides or the Sézary syndrome.

Design: Retrospective review of a uniformly staged inception cohort.

Setting: Single-institution tertiary care center.

Patients: 152 consecutive patients who had mycosis fungoides with or without the Sézary syndrome within 6 months of the initial definitive diagnosis.

Intervention: A detailed staging evaluation including physical examination, routine laboratory studies, chest roentgenogram, lymphangiogram, peripheral blood smear, lymph node biopsy, bone marrow aspirate or biopsy, and liver biopsy in selected patients.

Measurements and Main Results: Univariate adverse prognostic features at initial diagnosis in patients with mycosis fungoides included (P < 0.01) one or more cutaneous tumors or generalized erythroderma, adenopathy, blood smear involvement with Sézary cells, lymph node effacement, eosinophilia, and visceral involvement. Important, independent prognostic factors in a multivariate analysis are the presence of visceral disease and type of skin involvement.

Conclusions: A staging system based on histopathologic evaluation of skin, lymph nodes, blood, and visceral sites provides more comprehensive prognostic information than clinical evaluation of skin disease and adenopathy. Patients may be divided at initial presentation into three prognostic groups: good-risk patients, who have plaque-only skin disease without lymph node, blood, or visceral involvement (median survival, > 12 years); intermediate-risk patients, who have cutaneous tumors, erythroderma, or plaque disease with node or blood involvement but no visceral disease or node effacement (median survival, 5 years); and poor-risk patients, who have visceral involvement or node effacement (median survival, 2.5 years).





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