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Angiosarcoma of the breast after quadrantectomy and postoperative radiotherapy for carcinoma
R. J. I. Colville, A. Ramsden, A. Malcolm and N. R. Mclean

Paper accepted by the British Journal of Plastic Surgery on the 5th January 2000

Royal Victoria Infirmary, Newcastle upon Tyne, UK


SUMMARY:   Angiosarcoma of the breast following conservation surgery and radiotherapy for carcinoma is rare. We report a case occurring after a latent period of 8 years, which required excision with a 5 cm margin and latissimus dorsi flap repair.

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Angiosarcoma of the breast after quadrantectomy and postoperative radiotherapy for carcinoma

R. J. I. Colville, A. Ramsden, A. Malcolm and N. R. Mclean

Paper accepted by the British Journal of Plastic Surgery on the 5th January 2000

Royal Victoria Infirmary, Newcastle upon Tyne, UK

SUMMARY Angiosarcoma of the breast following conservation surgery and radiotherapy for carcinoma is rare. We report a case occurring after a latent period of 8 years, which required excision with a 5 cm margin and latissimus dorsi flap repair.

Keywords: breast. angiosarcoma, conservation surgery, radiotherapy.

Angiosarcoma of the breast after conservation surgery for carcinoma and adjuvant radiotherapy is a fairly rare occurrence, with only 70 cases reported.1'2 To date, none have been published in the Plastic Surgery literature. Increasing numbers of women are receiving this form of treatment for breast cancer and therefore more cases of radiation-induced angiosarcoma may present in the
future and be referred to plastic surgeons as multifocal skin lesions for excision and reconstruction.


Case report

In December 1990, a 46-year-old woman had a left lower quadrantectomy and axillary sampling for a 1cm malignant lump in the left breast. Histology showed a grade 1 ductal carcinoma and unexpectedly two out of eight nodes were positive (T1 N1 Mx ). The patient received tamoxifen and 50 Gy in 25 fractions over 33 days, two fields to the left breast and one to the left supraclavicular fossa. In 1992 she underwent a total abdominal hysterectomy and bilateral salpingo-oophorectomy as part of hormonal control of the disease after tamoxifen had caused unacceptable fluid retention. She had no upper limb lymphoedema.

In July 1998, a cutaneous angiosarcoma was completely excised from the left breast with a 10mm clearance at histology, but had clinically recurred by October (Fig. 1) and was associated with diffuse oedema of the breast. A left mastectomy was performed with a 5 cm margin, taking pectoralis major, and reconstructed with a latissimus dorsi flap. Histology showed a tumour in the skin, invading connective and muscular tissue. The appearances were of a low grade intradermal angiosarcoma following radiation and clearance margins of 20mm were reported (Fig. 2).

image of figure 1 showing the left breast with multiple cutaneous nodules and the scar from the previously excised angiosarcoma

Figure 1The left breast shows multiple cutaneous nodules and the scar from the previously excised angiosarcoma.

A further recurrence inferior to the flap was excised in March 1999 and closure achieved with upper abdominal skin advancement. Histology confirmed this to be fully excised multifocal recurrent angiosarcoma, and postoperative adjuvant electron treatment of 40 Gy in 16 fractions was given. She recently presented with a further recurrence lateral to the flap, which has been completely excised.

image of figure 2 showing an histology image

Figure 2Histology shows vascular spaces lined by pleomorphic endothelial cells invading subdermal fat (haematoxylin and eosin x 220).

Discussion

Angiosarcoma of the breast is rare, being responsible for 0.04% of malignant primary breast neoplasms. (3) Angiosarcoma may occur secondarily after treatment for a previously unrelated breast tumour and is equally rare. This case should not be considered as metastatic or recurrent angiosarcoma but as angiosarcoma secondary to irradiation, or secondary angiosarcoma. This disease, first described by Stewart and Treves after radical mastectomy and adjuvant radiotherapy, was thought to be largely due to the resultant chronic upper limb lymphoedema. (4) Over 200 cases have now been reported. More recently, radiation-induced secondary angiosarcomas have also been recorded following segmentectomy and irradiation of the breast, and these are not associated with lymphoedema. There are only 70 cases to date, but these numbers may increase with the growing popularity of conservative surgical treatment. The appearance of an angiosarcoma after segmentectomy and radiotherapy has an average latency of 74 months occurring in good-prognosis breast carcinomas (1) and manifests clinically as bluish or purple nodules and may be associated with diffuse breast oedema or erythema. Mammography and FNAC are very poor at detecting this tumour, while open biopsy appears more specific.' Histology cannot distinguish primary from secondary angiosarcomas, which must be deduced from the history and time intervals. The tumour is likely to be locally aggressive, with recurrence at a median of 7.5 months.' Recurrence may be due to incomplete excision of any one lesion, residual microscopic disease or residual discontinuous lesions beyond the surgical specimen (multifocal tumour).

The literature advises surgery as primary treatment, with total mastectomy either alone (2,6) or with axillary dissection. (1,7) Sarcomas rarely metastasise to lymph nodes and there is no evidence that secondary angiosarcomas metastasise to lymph nodes more frequently than other sarcomas. It appears that there is no scientific rationale for performing nodal clearance. (7) There is no consensus on the role of adjuvant therapy. (5,6) After diagnosis the median survival is 15.5 months' and the majority of deaths are from progressive local disease.

For patients diagnosed with radiation-induced angiosarcoma of the breast (secondary angiosarcoma) we advise completion mastectomy with margins of at least 5 cm around the lesion and appropriate soft tissue reconstruction.

References

  • Marchal C, Weber B, De Lafontan B, et al. Nine breast angiosarcomas after conservative treatment for breast carcinoma: a survey from French comprehensive cancer centers. Int J Radiat Oncol Biol Phys 1999; 44: 11319.
  • Wijnmaalen A, Van Ooijen B, Van Geel BN, Henzen-Logmans SC, Treurniet-Donker AD. Angiosarcoma of the breast following lumpectomy, axillary lymph node dissection, and radiotherapy for primary breast cancer: three case reports and a review of the literature. lnt J Radiat Oncol Biol Phys 1993; 26: 1359.
  • Hunter TB, Martin PC, Dietzen CD, Tyler LT. Angiosarcoma of the breast: two case reports and a review of the literature, cancer 1985; 56: 2099106.
  • Stewart FW, Treves N. Lymphangiosarcoma in postmastectomy lymphedema: a report of six cases in elephantiasis chirurgica. cancer 1948; 1: 6481.
  • Kuten A, Sapir D, Cohen Y, Haim N, Borovik R, Robinson E. Postirradiation soft tissue sarcoma occurring in breast cancer patients: report of seven cases and results of combination chemotherapy. J Surg Oncol 1985; 28: 16871.
  • Givens SS, Ellerbroek NA, Butler JJ, Libshitz HI, Hortobagyi GN, McNeese MD. Angiosarcoma arising in an irradiated breast: a case report and review of the literature. Cancer 1989; 64: 221416.
  • Turner WH, Greenall MJ. Sarcoma induced by radiotherapy after breast conservation surgery. Br J Surg 1991; 78: 13 1718.

The Authors

R. James I. Colville MSc, FRCS, Specialist Registrar in Plastic Surgery
Alex Ramsden FRCS, SHO in Plastic Surgery
Archie Malcolm MB, ChB, FRCPath, Professor of Pathology
Neil R. McLean MD, FRCS, Consultant Head and Neck/Plastic and Reconstructive Surgeon

Royal Victoria Infirmary, Queen Victoria Road, Newcastle upon Tyne, NE1 4LE, UK.

Angiosarcoma of the breast after quadrantectomy and postoperative radiotherapy for carcinoma : Accepted 5 June 2000, after revision by the British Journal of Plastic Surgery

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