Paget's & Radiotherapy

The conservative management of Pagetís disease of the breast with radiotherapy: 10- and 15-year results

J.K. Marshall 1 , K.A. Griffith 2 , B.G. Haffty 3 , L.J. Solin 4 , F.A. Vicini 5 , B. McCormick 6 , D.E. Wazer 7 , A. Recht 8 and L.J. Pierce 1

Purpose/Objective:

Previous reports have demonstrated the success at 5 years of breast-conserving surgery (BCS) and radiotherapy (RT) in patients treated for Pagetís disease of the breast.

The purpose of the current report was to provide 10- and 15-year estimates of tumor control in the breast, disease-free and overall survival following BCS and RT in a cohort of patients with Pagetís disease presenting without a palpable mass or mammographic density.

Materials/Methods:

Through a collaborative review from seven institutions of patients treated with BCS and RT, 38 cases of Pagetís disease of the breast presenting without a palpable mass or mammographic density were identified. All patients had pathologic confirmation of typical Pagetís cells at time of diagnosis.

Thirty-six of 38 patients had a minimum follow-up of greater than 12 months and constitute the present study cohort. Ninety-four percent of patients underwent complete or partial excision of the nipple-areolar complex and all patients received external beam irradiation to the whole breast, to a median dose of 50 Gray (Gy), range, 45-54 Gy.

Ninety-seven percent of patients also received a boost to the remaining nipple or tumor bed, for a median total dose of 61.5 Gy, range, 50.4-70 Gy.

Results:

With a median follow-up of 113 months (range, 18-257 months), 4 of 36 patients (11%) developed a recurrence in the treated breast as the only site of first failure. Two of the four recurrences were DCIS only and 2 were invasive with DCIS. Two additional patients had a recurrence in the breast as a component of first failure.

Actuarial local control rates for a breast failure as the only site of first failure were 91% at 5 years (95% confidence interval (CI), 80-100%) and 87% (95% CI, 75-99%) at both 10 and 15 years. Actuarial local control rates for breast failure, as a component of first failure, were 91% (95% CI, 80-100%), 83% (95% CI, 69-97%), and 76% (95% CI, 58-94%) at 5, 10, and 15 years, respectively.

No clinical factors were identified as significant predictors for recurrence in the breast. Five of the six patients developing a breast recurrence underwent salvage surgery, of which all were successfully salvaged. The sixth patient developed a simultaneous local and distant recurrence and expired with disease without undergoing surgical salvage.

Four of the five salvaged patients remain free of disease with a median follow-up of 12.3 years (range, 6.9-17.7 years), with the fifth patient expiring with disease following a distant recurrence. In total, of the 36 patients studied, 2 patients expired with disease and 2 patients expired free of disease, yielding 5-, 10-, and 15-year actuarial rates for survival without disease of 97% (95% CI, 90-100%) and 5-, 10-, and 15-year actuarial rates for overall survival of 93% (95% CI, 84-100%) at 5 years and 90% (95% CI, 78-100%) at 10 and 15 years.

Conclusions:

To our knowledge, these results represent the longest follow-up of a patient cohort treated with BCS and RT for Pagetís disease of the breast. These data confirm excellent rates of local control, disease-free and overall survival at 10 and 15 years, and success of surgical salvage following a conservative approach.

This study continues to support the recommendation of local excision and definitive breast irradiation as an alternative to mastectomy in the treatment of Pagetís disease presenting without a palpable mass or mammographic density.

Affiliations:

1 Dept. of Radiation Oncology, University of Michigan School of Medicine, Ann Arbor, MI, USA. 2 Biostatistics Unit, University of Michigan School of Medicine, Ann Arbor, MI, USA. 3 Dept. of Radiation Oncology, Yale University School of Medicine, New Haven, CT, USA. 4 Dept. of Radiation Oncology, Hospital of the University of Pennsylvania, Philadelphia, PA, USA. 5 Dept. of Radiation Oncology, William Beaumont Hospital, Royal Oak, MI, USA. 6 Dept. of Radiation Oncology, Memorial Sloan-Kettering Cancer Center, New York, NY, USA. 7 Dept. of Radiation Oncology, Tufts-New England Medical Center, Boston, MA, USA. 8 Dept. of Radiation Oncology, Beth Israel Deaconess Medical Center, Boston, MA, USA.

Int J Radiat Oncol Biol Phys, 11/02

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