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Histopathologic staging at initial diagnosis of mycosis fungoides and the Sézary syndrome. Definition of three distinctive prognostic groups.
Ann Intern Med 1988; 109(5):372-82AIM

Abstract

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 less than 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, greater than 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).

Authors+Show Affiliations

National Cancer Institute, Bethesda, Maryland.No affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info available

Pub Type(s)

Journal Article
Research Support, U.S. Gov't, P.H.S.

Language

eng

PubMed ID

3408055

Citation

Sausville, E A., et al. "Histopathologic Staging at Initial Diagnosis of Mycosis Fungoides and the Sézary Syndrome. Definition of Three Distinctive Prognostic Groups." Annals of Internal Medicine, vol. 109, no. 5, 1988, pp. 372-82.
Sausville EA, Eddy JL, Makuch RW, et al. Histopathologic staging at initial diagnosis of mycosis fungoides and the Sézary syndrome. Definition of three distinctive prognostic groups. Ann Intern Med. 1988;109(5):372-82.
Sausville, E. A., Eddy, J. L., Makuch, R. W., Fischmann, A. B., Schechter, G. P., Matthews, M., ... Veach, S. R. (1988). Histopathologic staging at initial diagnosis of mycosis fungoides and the Sézary syndrome. Definition of three distinctive prognostic groups. Annals of Internal Medicine, 109(5), pp. 372-82.
Sausville EA, et al. Histopathologic Staging at Initial Diagnosis of Mycosis Fungoides and the Sézary Syndrome. Definition of Three Distinctive Prognostic Groups. Ann Intern Med. 1988 Sep 1;109(5):372-82. PubMed PMID: 3408055.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - Histopathologic staging at initial diagnosis of mycosis fungoides and the Sézary syndrome. Definition of three distinctive prognostic groups. A1 - Sausville,E A, AU - Eddy,J L, AU - Makuch,R W, AU - Fischmann,A B, AU - Schechter,G P, AU - Matthews,M, AU - Glatstein,E, AU - Ihde,D C, AU - Kaye,F, AU - Veach,S R, PY - 1988/9/1/pubmed PY - 1988/9/1/medline PY - 1988/9/1/entrez SP - 372 EP - 82 JF - Annals of internal medicine JO - Ann. Intern. Med. VL - 109 IS - 5 N2 - 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 less than 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, greater than 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). SN - 0003-4819 UR - https://www.unboundmedicine.com/medline/citation/3408055/Histopathologic_staging_at_initial_diagnosis_of_mycosis_fungoides_and_the_Sézary_syndrome__Definition_of_three_distinctive_prognostic_groups_ L2 - https://www.annals.org/aim/fullarticle/doi/10.7326/0003-4819-109-5-372 DB - PRIME DP - Unbound Medicine ER -