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Arsalan, Hussein, and Al-Saady: A case of high-grade primary breast sarcoma (NOS type) presenting with clinical and radiological features of benign mass

Introduction

Not otherwise specified (NOS)-type breast sarcomas are primary sarcomas of the breast with no specific differentiation, that is, sarcomas that cannot be subclassified based on morphology and immunohistochemistry. Primary NOS-type sarcomas of the breast are very rare.1 NOS-type mammary sarcoma is one subtype of the pure mammary sarcoma, or stromal sarcoma, which is an older and broader synonym.

In general, pure mammary sarcomas are extremely rare, accounting for about 0.5–1% of malignant lesions of the breast and < 5% of soft tissue sarcomas. They may occur in young women and also in males.26 These are a heterogeneous group of malignant neoplasms of the spindle cells, arising from the interlobular mesenchymal elements that constitute the supporting mammary stroma.68 Excluded from this presentation are malignant lymphomas and malignant phyllodes tumours.

Early diagnosis is essential; however, this is not easy as the features consistent with malignant lesions of the breast are often absent.3,4,9 Metastasis usually occurs via haematogenous spread. Most authors agree that the main treatment for breast sarcoma is surgical extirpation.2,3,10,11 The role of adjuvant radiotherapy and chemotherapy is unclear and controversial.12

Case report

We report on the case of a 49-year-old diabetic woman who underwent surgical excision of a mass in the right breast 1 year after first presenting with this mass. She had undergone previous operations for an ovarian cyst and cataract. The breast mass was first investigated in a polyclinic, using ultrasound, which reported a rounded mass measuring 3 × 2 cm in the right lower inner quadrant, with a mixed echo pattern. This appeared likely to be benign in nature (Figure 1).

FIGURE 1

Ultrasound scan taken around 1 year prior to surgery.

6-1-3-fig1.jpg

On examination, there was a 2 × 2 cm, soft to firm, mobile mass inferior to the areola of the right breast. There was no skin tethering, no nipple discharge and no palpable axillary lymph nodes.

A new preoperative ultrasound taken 1 year after the ultrasound in the polyclinic showed two masses in the right breast. One of these was a benign-looking 9.6 × 6.8 mm mass, described as a possible lipoma at the 10 o'clock position. The second was a solid and well-defined subareolar, hypoechoic mass measuring 18.3 × 9 mm. This was a possible intraductal papilloma found in the lower outer quadrant of the right breast, with no distal acoustic shadowing, no significant vascular flow detected using a colour Doppler technique and no axillary lymph nodes (Figures 2 and 3).

FIGURE 2

Preoperative ultrasound scan showing what appears to be a lipoma at the 10 o'clock position in the right breast.

6-1-3-fig2.jpg
FIGURE 3

Preoperative ultrasound scan showing the main lesion in the lower outer quadrant of the right breast.

6-1-3-fig3.jpg

Surgical excision of the breast mass under general anaesthesia was advised, with perioperative frozen-section study of the specimen. The operation was performed within 1 week of the patient's presentation. The gross appearance of the mass excised from the lower aspect around the 6 o'clock position was irregular, with a rounded, well-demarcated area of necrosis, and the perioperative frozen-section examination of the specimen showed a suspicious spindle cell tumour. The cavity after local excision was drained using a suction-type tube drain.

The finalized histopathological study of all sections reported a tumour composed of proliferating spindle cells, with marked atypia, necrosis and a large number of mitotic figures, some of which were atypical. No recognizable mesenchymal differentiation was found despite thorough sampling. The tumour was very close to, and even impinged on, excision margins. A panel of immunostaining, including pancytokeratin, desmin, vimentin, actin, CD10, CD34, CD117, CD31, p63 and S-100 protein, failed to define sarcomatous differentiation. Features were consistent with high-grade mammary sarcoma (NOS-type) (Figures 4 and 5).

FIGURE 4

Neoplastic spindle cells showing no definite mesenchymal differentiation (haematoxylin and eosin staining; magnification × 100).

6-1-3-fig4.jpg
FIGURE 5

Another area showing necrosis with frequent mitosis (haematoxylin and eosin staining; magnification × 400).

6-1-3-fig5.jpg

Unfortunately, the patient was unavailable for follow-up as she was travelling outside the country.

Discussion

Purely sarcomatous tumours of the breast (with the exception of cystosarcoma phyllodes – by definition a fibroepithelial lesion – and lymphomas) are extremely uncommon,8 constituting < 1% of malignant breast tumours.2 Sex hormones (plus many chemicals that act as endocrine disruptors) may be involved in the carcinogenesis of soft tissue (and also visceral) sarcomas.13

As breast sarcomas are rare, and imaging methods to establish an exact diagnosis are inadequate, they often present a diagnostic challenge. Radiologists and clinicians may misdiagnose and merely monitor such tumours.4,5,9 The lesion in this case was viewed radiologically and interpreted as benign-looking more than once. This situation can clearly affect the surgeon's judgement and, consequently, the surgical planning. A recent study showed that primary breast sarcomas have imaging features that are not typically seen in infiltrating ductal carcinomas, and that a large, oval, hypervascular mass with indistinct margins should raise suspicion that a primary breast sarcoma may be present, and prompt biopsy.9

Although surgical intervention was carried out together with study of a frozen section, the latter was not indicative of malignancy. A preoperative fine-needle aspiration cytology or Tru-cut biopsy may be diagnostic, as a review of previous studies has stated that these are reliable for diagnosis of sarcoma in the same way as for carcinoma.2 Appropriate immunohistochemistry is essential for differential diagnosis.5

Axillary dissection was not carried out in this case. Lymphatic spread is common in carcinoma, but rare in sarcoma. Lymph nodes were histologically involved in only 1 out of 39 axillary dissections performed in a French study, and in 2 of 22 dissections at Mayo Clinic, MN, USA.14,15 Recurrence rates and overall survival are not affected by the extent of surgery.15 Furthermore, the majority of stromal tumours of the breast are not multicentric.

Local recurrence of the lesion is common, and some studies advise mastectomy together with excision of the underlying pectoral muscle for better local control.2 Chemotherapy has been disappointing in the treatment of breast sarcoma,2 and, as such tumours do not appear to display hormone receptors, hormonal manipulations have no place in their treatment.

In conclusion, any breast lesion should be treated with a high degree of suspicion, even when the clinical findings and radiological features are reassuring. Such a presentation may, unfortunately, belie an aggressive tumour.

References

1. 

Moinfar F. Mesenchymal lesions/tumors. In: Moinfar F Essentials of Diagnostic Breast Pathology: A Practical Approach. Berlin, Heidelberg: Springer; 2007, pp. 389–90.

2. 

Pollard SG, Marks PV, Temple LN, et al. Breast sarcoma: a clinicopathological review of 25 cases. Cancer 1991; 66:941–4. http://dx.doi.org/10.1002/1097-0142(19900901)66:5〈941::AIDCNCR2820660522〉3.0.CO;2-B

3. 

Teo T, Wee SB. Clinically ‘benign’ breast lumps: sarcoma in hiding? Case reports and literature review. Ann Acad Med Singapore 2004; 33:270–4.

4. 

Tekbas G, Ince T, Kapan M, et al. Are breast masses in teenagers always benign? Undifferentiated mesenchymal sarcoma in a 14-year-old girl. Breast Care 2012; 7:144–6. http://dx.doi.org/10.1159/000337770

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Jeong YJ, Oh HK, Bong JG. Undifferentiated pleomorphic sarcoma of the male breast causing diagnostic challenges. J Breast Cancer 2011; 14:241–6. http://dx.doi.org/10.4048/jbc.2011.14.3.241

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Lee JY, Kim DB, Kwak BS, et al. Primary fibrosarcoma of the breast: a case report. J Breast Cancer 2011; 14:156–9. http://dx.doi.org/10.4048/jbc.2011.14.2.156

7. 

Rosen PP. Sarcoma. In: Rosen PP Rosen's Breast Pathology, 2nd edn. Philadelphia, PA: Lippincott Williams and Wilkins; 2001, p. 813.

8. 

Callery CD, Rosen PP, Kinne DW. Sarcoma of the breast. A study of 32 patients with reappraisal of classification and therapy. Ann Surg 1985; 201:527–32. http://dx.doi.org/10.1097/00000658-198504000-00020

9. 

Smith TB, Gilcrease MZ, Santiago L, et al. Imaging features of primary breast sarcoma. AJR Am J Roentgenol 2012; 198:W386–93. http://dx.doi.org/10.2214/AJR.11.7341

10. 

Moore MP, Kinne DW. Breast sarcoma. Surg Clin North Am 1996; 76:383–92. http://dx.doi.org/10.1016/S0039-6109(05)70445-X

11. 

Gutman H, Pollock RE, Ross MI, et al. Sarcoma of the breast: implications for extent of therapy. The MD Anderson experience. Surgery 1994; 116:505–9.

12. 

McGowan TS, Cummings BJ, O'Sullivan B, et al. An analysis of 78 breast sarcoma patients without distant metastases at presentation. Int J Radiat Oncol Biol Phys 2000; 46:383–90. http://dx.doi.org/10.1016/S0360-3016(99)00444-7

13. 

Mastrangelo G, Coindre JM, Ducimetiere F, et al. Incidence of soft tissue sarcoma and beyond: a population-based prospective study in 3 European regions. Cancer 2012; 118:5339–48. http://dx.doi.org/10.1002/cncr.27555

14. 

Zelak L, Llombart Cussac A, Terrier P, et al. Prognostic factors in primary breast sarcomas: a series of patients with long term follow up. J Clin Oncol 2003; 21:2538–88.

15. 

Blanchard DK, Reynolds CA, Grant CS, et al. Primary nonphylloides breast sarcomas. Am J Surg 2003; 186:359–69. http://dx.doi.org/10.1016/S0002-9610(03)00269-1





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