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Hindawi Publishing Corporation Case Reports in Surgery Volume 2013, Article ID 139276, 2 pages http://dx.doi.org/10.1155/2013/139276 Case Report Bilateral Primary Angiosarcoma of the Breast P. Keshav 1 and Shruti S. Hegde 2 1 Associate Professor, Department of Surgery, KMC, Mangalore, Manipal University, Karnataka 575003, India 2 KMC, Mangalore, Manipal University, Karnataka 575003, India Correspondence should be addressed to Shruti S. Hegde; [email protected] Received 11 August 2013; Accepted 5 September 2013 Academic Editors: K. Honma and M. L. Quek Copyright © 2013 P. Keshav and S. S. Hegde. is is an open access article distributed under the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited. Primary breast sarcomas are very rare entities, accounting for 0.04% of all malignant neoplasms. Angiosarcoma of breast is infrequent and is an endothelial malignant tumor with bad prognosis because of the frequency of metastasis and recurrence. We present a case of a 30-year-old female who presented with an ulcerated leſt breast lesion which on further workup revealed to be a primary angiosarcoma of breast with metastasis to right breast. 1. Introduction Angiosarcomas are uncommon malignant neoplasms char- acterized by rapidly proliferating and extensively infiltrating anaplastic cells derived from blood vessels and lining irreg- ular, blood-filled spaces. e term angiosarcoma is applied to a wide range of malignant endothelial vascular neoplasm’s that affect a variety of sites. Angiosarcomas are aggressive and tend to recur locally and spread widely and have a high rate of lymph node and systemic metastases. 2. Case Report A 30-year-old unmarried lady presented with an ulcerated lump in the leſt breast. Initially, patient had noticed a peanut sized painless swelling in her leſt breast six months back which rapidly increased in size and the skin over the swelling spontaneously ulcerated with seropurulent discharge with occasional bleeding from the ulcer. ere was no history of breast surgery or breast irradiation. On examination the lump was 8 cms ×8 cms in diameter and was fixed to skin but free from underlying muscle and chest wall. ere were multiple enlarged axillary lymph nodes with the largest measuring 3 cms ×3 cms. In the opposite breast, a lump measuring 5 cms in diameter was detected which the patient had failed to notice. Axillary nodes were palpable on the right side as well. A provisional diagnosis of stromal sarcoma of the breast was considered aſter a Fine Needle Aspiration Cytology (FNAC) from both lumps. e patient underwent bilateral modified radical mastectomy with axillary clearance. e histology revealed multiple irregular vascular spaces of different sizes lined by single layer of endothelial cells which were surrounded by groups, bundles and masses of oval, and spindle shaped and pleomorphic cells having ovoid and pleomorphic hyperchromatic nuclei (Figures 1 and 2). e histological feature was consistent with the bilateral Angiosarcomas of breast. Lymph nodes, however, showed reactive hyperplasia. 3. Discussion Angiosarcoma of the breast is a rare and highly lethal neoplasm accounting for less than 0.1% of malignant breast tumors [1]. All Angiosarcomas tend to be aggressive and oſten are multicentric. Malignant vascular tumors are clinically aggressive and difficult to treat and have a reported 5-year survival rate of less than 20% and a median survival of just 22 months [2]. Advanced stage at presentation and lack of extensive excision are associated with higher recurrence, dis- tant metastasis rates, and worsened survival. is malignant tumor occurs primarily in young women, with 6% to 12% of the cases found during pregnancy, implying a hormonal effect [2]. Preoperative diagnosis of angiosarcoma of the breast by
Transcript of CaseReport Bilateral Primary Angiosarcoma of the Breast€¦ · 2 CaseReportsinSurgery...
Hindawi Publishing CorporationCase Reports in SurgeryVolume 2013, Article ID 139276, 2 pageshttp://dx.doi.org/10.1155/2013/139276
Case ReportBilateral Primary Angiosarcoma of the Breast
P. Keshav1 and Shruti S. Hegde2
1 Associate Professor, Department of Surgery, KMC, Mangalore, Manipal University, Karnataka 575003, India2 KMC, Mangalore, Manipal University, Karnataka 575003, India
Correspondence should be addressed to Shruti S. Hegde; [email protected]
Received 11 August 2013; Accepted 5 September 2013
Academic Editors: K. Honma and M. L. Quek
Copyright © 2013 P. Keshav and S. S. Hegde. This is an open access article distributed under the Creative Commons AttributionLicense, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properlycited.
Primary breast sarcomas are very rare entities, accounting for 0.04% of all malignant neoplasms. Angiosarcoma of breast isinfrequent and is an endothelial malignant tumor with bad prognosis because of the frequency of metastasis and recurrence. Wepresent a case of a 30-year-old female who presented with an ulcerated left breast lesion which on further workup revealed to be aprimary angiosarcoma of breast with metastasis to right breast.
1. Introduction
Angiosarcomas are uncommon malignant neoplasms char-acterized by rapidly proliferating and extensively infiltratinganaplastic cells derived from blood vessels and lining irreg-ular, blood-filled spaces. The term angiosarcoma is appliedto a wide range of malignant endothelial vascular neoplasm’sthat affect a variety of sites. Angiosarcomas are aggressive andtend to recur locally and spread widely and have a high rateof lymph node and systemic metastases.
2. Case Report
A 30-year-old unmarried lady presented with an ulceratedlump in the left breast. Initially, patient had noticed a peanutsized painless swelling in her left breast six months backwhich rapidly increased in size and the skin over the swellingspontaneously ulcerated with seropurulent discharge withoccasional bleeding from the ulcer. There was no historyof breast surgery or breast irradiation. On examination thelump was 8 cms × 8 cms in diameter and was fixed toskin but free from underlying muscle and chest wall. Therewere multiple enlarged axillary lymph nodes with the largestmeasuring 3 cms × 3 cms. In the opposite breast, a lumpmeasuring 5 cms in diameter was detected which the patienthad failed to notice. Axillary nodes were palpable on theright side as well. A provisional diagnosis of stromal sarcoma
of the breast was considered after a Fine Needle AspirationCytology (FNAC) from both lumps. The patient underwentbilateralmodified radicalmastectomywith axillary clearance.The histology revealed multiple irregular vascular spacesof different sizes lined by single layer of endothelial cellswhich were surrounded by groups, bundles and masses ofoval, and spindle shaped and pleomorphic cells having ovoidand pleomorphic hyperchromatic nuclei (Figures 1 and 2).The histological feature was consistent with the bilateralAngiosarcomas of breast. Lymph nodes, however, showedreactive hyperplasia.
3. Discussion
Angiosarcoma of the breast is a rare and highly lethalneoplasm accounting for less than 0.1% of malignant breasttumors [1]. All Angiosarcomas tend to be aggressive and oftenare multicentric. Malignant vascular tumors are clinicallyaggressive and difficult to treat and have a reported 5-yearsurvival rate of less than 20% and a median survival of just22 months [2]. Advanced stage at presentation and lack ofextensive excision are associated with higher recurrence, dis-tant metastasis rates, and worsened survival. This malignanttumor occurs primarily in young women, with 6% to 12% ofthe cases found during pregnancy, implying a hormonal effect[2]. Preoperative diagnosis of angiosarcoma of the breast by
2 Case Reports in Surgery
Figure 1: Histological appearance under low magnification.
Figure 2: Histological appearance under high magnification.
aspiration cytology and biopsy is often difficult, with a false-negative biopsy rate of 37% in one large review [3].
In most cases, the tumor is larger than 4 cm in diam-eter. In a series at Mayo Clinic tumor size was a morevaluable prognostic factor than tumor grade [4]. Theserapidly growing lesions often arise deep within breast tissue,causing diffuse breast enlargement with associated bluishskin discoloration. They usually spread locally as ill-defined,hemorrhagic, spongy masses. Unlike breast carcinomas, skinretraction, nipple discharge, and axillary lymphnode involve-ment are absent. In our case, however, we noticed that the skinwas involved, but the lymph nodes were negative for malig-nancy. An exceptional case old male breast angiosarcoma hasalso been described [5].
Low grade angiosarcomas are readilymistaken for benignhemangioma. Angiolipoma features intermingling vesselsand fat lobules which may be mistaken for invasion offat by an angiosarcoma. Papillary endothelial hyperplasiaas described by Branton et al. is the “great imposter forangiosarcoma” [6]. The treatment of angiosarcoma of thebreast is early and complete surgical excision of the masswith tumor-free margins because of the neoplasm oftenextends microscopically beyond its gross limits. Adjuvantchemotherapy that includes doxorubicin for patients withpoorly differentiated angiosarcoma of the breast results ina higher proportion of patients who are relapse-free com-pared to patients not receiving adjuvant chemotherapy [7].Radiotherapy is reserved after lumpectomy, and followingtotal mastectomy if the tumor is larger than 5 cm, themarginsare positive, or if the skin or regional nodes are affected.
References
[1] S. W. Weiss and J. R. Goldblum, Soft Tissue Tumors, Mosby,St.Louis, Mo, USA, 4th edition, 2001.
[2] K. T. K. Chen, D. D. Kirkegaard, and J. J. Bocian, “Angiosarcomaof the breast,” Cancer, vol. 46, no. 2, pp. 368–371, 1980.
[3] K. T. K. Chen, D. D. Kirkegaard, and J. J. Bocian, “Angiosarcomaof the breast,” Cancer, vol. 46, pp. 268–271, 1980.
[4] C. Adem, C. Reynolds, J. N. Ingle, and A. G. Nascimento,“Primary breast sarcoma: clinicopathologic series from theMayo Clinic and review of the literature,” British Journal ofCancer, vol. 91, no. 2, pp. 237–241, 2004.
[5] H. Mansouri, A. Jalil, L. Chouhou, N. Benjaafar, A. Souadka,and B. El Gueddari, “A rare case of angiosarcoma of the breastin a man: case report,” European Journal of GynaecologicalOncology, vol. 21, no. 6, pp. 603–604, 2000.
[6] P. A. Branton, R. Lininger, and F. A. Tavassoli, “Papillaryendothelial hyperplasia of the breast: the great impostor forangiosarcoma: a clinicopathologic review of 17 cases,” Interna-tional Journal of Surgical Pathology, vol. 11, no. 2, pp. 83–87, 2003.
[7] K. H. Antman, J. Corson, J. Greenberger, and R. Wilson,“Multimodality therapy in the management of angiosarcoma ofthe breast,” Cancer, vol. 50, no. 10, pp. 2000–2003, 1982.
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