Practical Radiation Oncology (2012) 2, e53–e57
www.practicalradonc.org
Teaching Case
Definitive treatment of supernumerary lower chest wall primary breast cancer Brandi R. Page MDa,⁎, Faisal Ahmed BSa , John W. Thomson MDb a
Department of Radiation Oncology, University of Utah School of Medicine, Salt Lake City, Utah Department of Radiation Oncology, Intermountain Health Care, Murray, Utah
b
Received 14 December 2011; revised 22 January 2012; accepted 25 January 2012
Introduction Ectopic breast tissue is estimated to occur in 1% of the population. 1 These include accessory mammary glands (polymastia), supernumerary nipples (hyperthelia) along “milk lines” from the axilla to the groin. Embryologic mammary ridges regress during development, excluding the 2 pectoral areas, later forming the breasts. Failure of regression may yield supernumerary breast tissue. 2 Some cases have been associated with congenital urologic anomalies 3 and familial inheritance. 4 Though difficult to accurately characterize, between 0.28% and 0.6% of breast cancers have been reported to occur in ectopic breast tissue away from the milk line, 1,5 or “mammae erraticae.” Reported sites include the axillae, 6-8 vulva, 9 sub-inframammary fold, 10 thigh in a male, 11 perineum 12, buttock, and face. 13 Few publications discuss treatment implications. We report our approach to staging, diagnosis, and treatment and review of the literature for accessory breast cancer.
Case presentation A 47-year-old previously healthy female presented with self-discovered masses, a 2-cm mass located 3-cm below her right inframammary fold, and a 3-cm mass in her right Conflicts of interest: None. ⁎ Corresponding author. Department of Radiation Oncology, University of Utah School of Medicine, 1950 Circle of Hope, Rm 1570, Salt Lake City, UT 84112. E-mail address: [email protected] (B.R. Page).
axilla. No skin or subcutaneous abnormalities were discovered along the milk lines. Past medical history was significant for hypothyroidism; she had no risk factors for breast cancer. Mammography discovered a 3.6-cm right axillary lymph node but no breast abnormality. An ultrasoundguided core biopsy of the lower anterior chest wall nodule revealed grade 3 breast carcinoma and viable breast tissue with no carcinoma present, suggesting against a metastasis. Biopsy of the enlarged right level II axillary lymph node revealed grade 3 infiltrating ductal carcinoma (Fig 1). A positron emission tomographycomputed tomography (PET-CT) revealed 5 enlarged hypermetabolic axillary nodes (Fig 2). A smaller hypermetabolic lesion was seen deep in the right breast consistent with an intramammary node. A tattoo was placed on the skin overlying the chest wall nodule for an accurate planned resection in the case of a complete pathologic response after neoadjuvant chemotherapy. She was treated with neoadjuvant carboplatin and Taxotere (Sanofi US, Bridgewater, NJ) for 6 cycles, according to the National Cancer Care Network (NCCN) guidelines for node-positive breast cancer. She had a complete clinical response. Modified radical mastectomy and wide local excision of the accessory breast tissue revealed a complete pathologic response. Comprehensive radiotherapy to 5000 cGy was performed utilizing 3 fields. Tangent fields (Fig 3) were extended inferiorly to cover the lower chest wall with minimal dose to the liver (Fig 4) and a third supraclavicular field was placed. Electrons were considered, but because of her topography photons afforded better penumbra and normal tissue sparing. She did well through radiotherapy treatment with
1879-8500/$ – see front matter © 2012 American Society for Radiation Oncology. Published by Elsevier Inc. All rights reserved. http://dx.doi.org/10.1016/j.prro.2012.01.009
e54
B.R. Page et al
Practical Radiation Oncology: October-December 2012
Figure 1 Topography. The volume at the anterolateral right lower chest wall represents the excision site of the supernumerary breast tissue. Arrow denotes the location of the primary tumor site. The left intact breast is at the level of the removed right intact breast. Nodal volumes are outlined in the axilla.
expected sequelae, including Common Terminology Criteria for Adverse Events v.3 grade 2 radiation-induced dermatitis (moderate to brisk erythema) and grade 1 fatigue (mild fatigue over baseline).
Discussion Ectopic breast tissue may present with varying symptoms and is at risk for developing breast cancer. A complete physical examination of skin and tissue along the milk line should be performed. 5 In our patient, primary presentation was axillary lymphadenopathy and a nodule at the lower anterior chest wall. A mammogram did not identify a primary breast tumor, but confirmed axillary lymphadenopathy. She had no sign of polythelia, polymastia, or any other suspicious subcutaneous nodules prior to diagnosis. Thus, even with traditional screening methods, this cancer likely would not have been discovered prior to palpable axillary nodes. A PET-CT was performed as suggested in the NCCN guidelines in situations where standard staging studies are equivocal or to define unsuspected nodal disease. PET-CT confirmed abnormal nodes in the axilla, a node in the intramammary
region, and the primary site at the lower anterior chest wall 3-cm below her right inframammary ridge. PET-CT confirmed the most likely primary breast cancer source. Magnetic resonance imaging is potentially useful, as NCCN guidelines suggest, for discovering multifocal unilateral tumors or bilateral breast cancer. In our case, magnetic resonance imaging was unnecessary because PET-CT identified the location of the primary and ruled out distant metastatic disease. Treatment of accessory breast cancer depends largely on location. As in carcinoma of an unknown primary, ultrasound-guided fine-needle aspiration for cytologic diagnosis 14 is frequently utilized in ectopic breast cancers. This can be an appropriate option for sampling suspicious areas. Sentinel lymph node biopsy has also been suggested in the diagnosis and treatment of accessory breast cancers. 15 We considered utilizing sentinel lymph node biopsy; however, we concluded enough evidence existed by PET-CT to identify involved lymph nodes. Previous reports of patients having breast cancer in accessory breast tissue have advised clinicians to consider prophylactic excision of accessory breast tissue in patients with a higher family history of breast cancer if those factors are present.
Practical Radiation Oncology: October-December 2012
Supernumerary breast cancer treatment
e55
Figure 2 Positron emission tomography–computed tomography depicting the primary tumor at the anterolateral lower chest wall with locally advanced disease in the axilla.
Conclusions For patients who may be diagnosed as having an unknown primary breast cancer, searching for a primary tumor beyond the breast and axilla is very important. Keeping in mind possible accessory breast cancers is important for diagnosis, treatment, and prognosis as it could change the approach to include local, rather than solely systemic, therapy. Radiation therapy should be considered to the site of malignant tissue and draining lymphatics in supernumerary breast cancers. PET-CT is useful both to help identify involved lymphatics as well as rule out distant disease. In conclusion, ectopic breast cancers present a problem not seen with traditional
screening or treatment techniques. Ectopic breast cancers have unique needs for diagnosis, treatment, and prognosis and individual patient characteristics need to be considered for a specialized treatment plan.
References 1. Marshall MB, Moynihan JJ, Frost A, Evans SR. Ectopic breast cancer: case report and literature review. Surg Oncol. 1994;3: 295-304. 2. Virgili A, Trincone S, Durante E, Corazza M. Breast cancer of the axillary extension. Acta Derm Venereol. 2005;85:81-82. 3. Varsano IB, Jaber L, Garty BZ, Mukamel MM, Grünebaum M. Urinary tract abnormalities in children with supernumerary nipples. Pediatrics. 1984;73:103-105.
e56
B.R. Page et al
Practical Radiation Oncology: October-December 2012
Figure 3
Extended tangential field with supraclavicular field coronal view.
Figure 4
Liver dose-volume histogram.
Practical Radiation Oncology: October-December 2012 4. Toumbis-Ioannou E, Cohen PR. Familial polythelia. J Am Acad Dermatol. 1994;30:667-668. 5. Cheong JH, Lee BC, Lee KS. Carcinoma of the axillary breast. Yonsei Med J. 1999;40:290-293. 6. Teke Z, Kabay B, Akbulut M, Erdem E. Primary infiltrating ductal carcinoma arising in aberrant breast tissue of the axilla: a rare entity. Report of a case. Tumori. 2008;94:577-583. 7. Shin SJ, Sheikh FS, Allenby PA, Rosen PP. Invasive secretory (juvenile) carcinoma arising in ectopic breast tissue of the axilla. Arch Pathol Lab Med. 2001;125:1372-1374. 8. Goyal S, Puri T, Gupta R, Julka PK, Rath GK. Accessory breast tissue in axilla masquerading as breast cancer recurrence. J Cancer Res Ther. 2008;4:95-96. 9. Godoy-Gijón E, Yuste-Chaves M, Santos-Briz A, EstebanVelasco C, de Unamuno-Pérez P. Accessory breast on the vulva [e-pub ahead of print July 26, 2011]. Actas Dermosifiliogr. doi:10.1016/j.ad.2011.02.015.
Supernumerary breast cancer treatment
e57
10. Madej B, Balak B, Winkler I, Burdan F. Cancer of the accessory breast–a case report. Adv Med Sci. 2009;54:308-310. 11. Boivin S, Segard M, Delaporte E, Cotten H, Piette F, Thomas P. Complete supernumerary breast on the thigh in a male patient. [Article in French] Ann Dermatol Venereol. 2001;128:144-146. 12. Basu S, Bag T, Saha KS, Biswas PC. Accessory breast in the perineum. Trop Doct. 2003;33:245. 13. Deaver JB, McFarland J, Herman JL. The breast: its anomalies, its diseases and their treatment. Philadelphia. Pennsylvania: P. Blakiston's Son & Co. 1917. 14. Das DK, Gupta SK, Mathew SV, Sheikh ZA, al-Rabah NA. Fine needle aspiration cytologic diagnosis of axillary accessory breast tissue, including its physiologic changes and pathologic lesions. Acta Cytol. 1994;38:130-135. 15. Thorne AL, Jackson A, Yiangou C. The use of sentinel node biopsy in the treatment of cancer of an accessory breast. Breast. 2003;12: 153-155.
www.practicalradonc.org
Teaching Case
Definitive treatment of supernumerary lower chest wall primary breast cancer Brandi R. Page MDa,⁎, Faisal Ahmed BSa , John W. Thomson MDb a
Department of Radiation Oncology, University of Utah School of Medicine, Salt Lake City, Utah Department of Radiation Oncology, Intermountain Health Care, Murray, Utah
b
Received 14 December 2011; revised 22 January 2012; accepted 25 January 2012
Introduction Ectopic breast tissue is estimated to occur in 1% of the population. 1 These include accessory mammary glands (polymastia), supernumerary nipples (hyperthelia) along “milk lines” from the axilla to the groin. Embryologic mammary ridges regress during development, excluding the 2 pectoral areas, later forming the breasts. Failure of regression may yield supernumerary breast tissue. 2 Some cases have been associated with congenital urologic anomalies 3 and familial inheritance. 4 Though difficult to accurately characterize, between 0.28% and 0.6% of breast cancers have been reported to occur in ectopic breast tissue away from the milk line, 1,5 or “mammae erraticae.” Reported sites include the axillae, 6-8 vulva, 9 sub-inframammary fold, 10 thigh in a male, 11 perineum 12, buttock, and face. 13 Few publications discuss treatment implications. We report our approach to staging, diagnosis, and treatment and review of the literature for accessory breast cancer.
Case presentation A 47-year-old previously healthy female presented with self-discovered masses, a 2-cm mass located 3-cm below her right inframammary fold, and a 3-cm mass in her right Conflicts of interest: None. ⁎ Corresponding author. Department of Radiation Oncology, University of Utah School of Medicine, 1950 Circle of Hope, Rm 1570, Salt Lake City, UT 84112. E-mail address: [email protected] (B.R. Page).
axilla. No skin or subcutaneous abnormalities were discovered along the milk lines. Past medical history was significant for hypothyroidism; she had no risk factors for breast cancer. Mammography discovered a 3.6-cm right axillary lymph node but no breast abnormality. An ultrasoundguided core biopsy of the lower anterior chest wall nodule revealed grade 3 breast carcinoma and viable breast tissue with no carcinoma present, suggesting against a metastasis. Biopsy of the enlarged right level II axillary lymph node revealed grade 3 infiltrating ductal carcinoma (Fig 1). A positron emission tomographycomputed tomography (PET-CT) revealed 5 enlarged hypermetabolic axillary nodes (Fig 2). A smaller hypermetabolic lesion was seen deep in the right breast consistent with an intramammary node. A tattoo was placed on the skin overlying the chest wall nodule for an accurate planned resection in the case of a complete pathologic response after neoadjuvant chemotherapy. She was treated with neoadjuvant carboplatin and Taxotere (Sanofi US, Bridgewater, NJ) for 6 cycles, according to the National Cancer Care Network (NCCN) guidelines for node-positive breast cancer. She had a complete clinical response. Modified radical mastectomy and wide local excision of the accessory breast tissue revealed a complete pathologic response. Comprehensive radiotherapy to 5000 cGy was performed utilizing 3 fields. Tangent fields (Fig 3) were extended inferiorly to cover the lower chest wall with minimal dose to the liver (Fig 4) and a third supraclavicular field was placed. Electrons were considered, but because of her topography photons afforded better penumbra and normal tissue sparing. She did well through radiotherapy treatment with
1879-8500/$ – see front matter © 2012 American Society for Radiation Oncology. Published by Elsevier Inc. All rights reserved. http://dx.doi.org/10.1016/j.prro.2012.01.009
e54
B.R. Page et al
Practical Radiation Oncology: October-December 2012
Figure 1 Topography. The volume at the anterolateral right lower chest wall represents the excision site of the supernumerary breast tissue. Arrow denotes the location of the primary tumor site. The left intact breast is at the level of the removed right intact breast. Nodal volumes are outlined in the axilla.
expected sequelae, including Common Terminology Criteria for Adverse Events v.3 grade 2 radiation-induced dermatitis (moderate to brisk erythema) and grade 1 fatigue (mild fatigue over baseline).
Discussion Ectopic breast tissue may present with varying symptoms and is at risk for developing breast cancer. A complete physical examination of skin and tissue along the milk line should be performed. 5 In our patient, primary presentation was axillary lymphadenopathy and a nodule at the lower anterior chest wall. A mammogram did not identify a primary breast tumor, but confirmed axillary lymphadenopathy. She had no sign of polythelia, polymastia, or any other suspicious subcutaneous nodules prior to diagnosis. Thus, even with traditional screening methods, this cancer likely would not have been discovered prior to palpable axillary nodes. A PET-CT was performed as suggested in the NCCN guidelines in situations where standard staging studies are equivocal or to define unsuspected nodal disease. PET-CT confirmed abnormal nodes in the axilla, a node in the intramammary
region, and the primary site at the lower anterior chest wall 3-cm below her right inframammary ridge. PET-CT confirmed the most likely primary breast cancer source. Magnetic resonance imaging is potentially useful, as NCCN guidelines suggest, for discovering multifocal unilateral tumors or bilateral breast cancer. In our case, magnetic resonance imaging was unnecessary because PET-CT identified the location of the primary and ruled out distant metastatic disease. Treatment of accessory breast cancer depends largely on location. As in carcinoma of an unknown primary, ultrasound-guided fine-needle aspiration for cytologic diagnosis 14 is frequently utilized in ectopic breast cancers. This can be an appropriate option for sampling suspicious areas. Sentinel lymph node biopsy has also been suggested in the diagnosis and treatment of accessory breast cancers. 15 We considered utilizing sentinel lymph node biopsy; however, we concluded enough evidence existed by PET-CT to identify involved lymph nodes. Previous reports of patients having breast cancer in accessory breast tissue have advised clinicians to consider prophylactic excision of accessory breast tissue in patients with a higher family history of breast cancer if those factors are present.
Practical Radiation Oncology: October-December 2012
Supernumerary breast cancer treatment
e55
Figure 2 Positron emission tomography–computed tomography depicting the primary tumor at the anterolateral lower chest wall with locally advanced disease in the axilla.
Conclusions For patients who may be diagnosed as having an unknown primary breast cancer, searching for a primary tumor beyond the breast and axilla is very important. Keeping in mind possible accessory breast cancers is important for diagnosis, treatment, and prognosis as it could change the approach to include local, rather than solely systemic, therapy. Radiation therapy should be considered to the site of malignant tissue and draining lymphatics in supernumerary breast cancers. PET-CT is useful both to help identify involved lymphatics as well as rule out distant disease. In conclusion, ectopic breast cancers present a problem not seen with traditional
screening or treatment techniques. Ectopic breast cancers have unique needs for diagnosis, treatment, and prognosis and individual patient characteristics need to be considered for a specialized treatment plan.
References 1. Marshall MB, Moynihan JJ, Frost A, Evans SR. Ectopic breast cancer: case report and literature review. Surg Oncol. 1994;3: 295-304. 2. Virgili A, Trincone S, Durante E, Corazza M. Breast cancer of the axillary extension. Acta Derm Venereol. 2005;85:81-82. 3. Varsano IB, Jaber L, Garty BZ, Mukamel MM, Grünebaum M. Urinary tract abnormalities in children with supernumerary nipples. Pediatrics. 1984;73:103-105.
e56
B.R. Page et al
Practical Radiation Oncology: October-December 2012
Figure 3
Extended tangential field with supraclavicular field coronal view.
Figure 4
Liver dose-volume histogram.
Practical Radiation Oncology: October-December 2012 4. Toumbis-Ioannou E, Cohen PR. Familial polythelia. J Am Acad Dermatol. 1994;30:667-668. 5. Cheong JH, Lee BC, Lee KS. Carcinoma of the axillary breast. Yonsei Med J. 1999;40:290-293. 6. Teke Z, Kabay B, Akbulut M, Erdem E. Primary infiltrating ductal carcinoma arising in aberrant breast tissue of the axilla: a rare entity. Report of a case. Tumori. 2008;94:577-583. 7. Shin SJ, Sheikh FS, Allenby PA, Rosen PP. Invasive secretory (juvenile) carcinoma arising in ectopic breast tissue of the axilla. Arch Pathol Lab Med. 2001;125:1372-1374. 8. Goyal S, Puri T, Gupta R, Julka PK, Rath GK. Accessory breast tissue in axilla masquerading as breast cancer recurrence. J Cancer Res Ther. 2008;4:95-96. 9. Godoy-Gijón E, Yuste-Chaves M, Santos-Briz A, EstebanVelasco C, de Unamuno-Pérez P. Accessory breast on the vulva [e-pub ahead of print July 26, 2011]. Actas Dermosifiliogr. doi:10.1016/j.ad.2011.02.015.
Supernumerary breast cancer treatment
e57
10. Madej B, Balak B, Winkler I, Burdan F. Cancer of the accessory breast–a case report. Adv Med Sci. 2009;54:308-310. 11. Boivin S, Segard M, Delaporte E, Cotten H, Piette F, Thomas P. Complete supernumerary breast on the thigh in a male patient. [Article in French] Ann Dermatol Venereol. 2001;128:144-146. 12. Basu S, Bag T, Saha KS, Biswas PC. Accessory breast in the perineum. Trop Doct. 2003;33:245. 13. Deaver JB, McFarland J, Herman JL. The breast: its anomalies, its diseases and their treatment. Philadelphia. Pennsylvania: P. Blakiston's Son & Co. 1917. 14. Das DK, Gupta SK, Mathew SV, Sheikh ZA, al-Rabah NA. Fine needle aspiration cytologic diagnosis of axillary accessory breast tissue, including its physiologic changes and pathologic lesions. Acta Cytol. 1994;38:130-135. 15. Thorne AL, Jackson A, Yiangou C. The use of sentinel node biopsy in the treatment of cancer of an accessory breast. Breast. 2003;12: 153-155.
