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Primary Desmoid Tumor (Extraabdominal Fibromatosis) of the Breast

¹ Department of Diagnostic Radiology, Breast Imaging, The University of Texas M. D. Anderson Cancer Center, 1515 Holcombe Blvd., Unit 57, Houston, TX 77030.
² Department of Pathology, The University of Texas M. D. Anderson Cancer Center, Houston, TX 77030.

Received December 29, 2004; accepted after revision February 9, 2005.

Address correspondence to B. E. Dogan (basak.dogan{at}di.mdacc.tmc.edu).

A 25-year-old woman presented to our institution with the chief complaint of a palpable lump in her left breast for 5 months, which she noticed during her pregnancy. Mammography revealed a 2-cm spiculated mass in the upper inner quadrant of the left breast (Fig. 1A). Sonography showed a suspicious hypoechoic mass with irregular margins close to the chest wall (Fig. 1B). Sonographically guided core biopsy was performed, which revealed a desmoid tumor (Fig. 1C).

View larger version (95K): [in this window] [in a new window] [as a PowerPoint slide]   Fig. 1A —25-year-old lactating woman with desmoid tumor of left breast. Left mediolateral oblique mammogram shows 2-cm spiculated mass in upper left breast close to pectoral muscle. Skin marker indicates lesion is palpable.

View larger version (151K): [in this window] [in a new window] [as a PowerPoint slide]   Fig. 1B —25-year-old lactating woman with desmoid tumor of left breast. Sonography image shows irregular, hypoechoic mass with indistinct margins. Tumor is confined to breast parenchyma and does not involve retromammary structures.

View larger version (78K): [in this window] [in a new window] [as a PowerPoint slide]   Fig. 1C —25-year-old lactating woman with desmoid tumor of left breast. Power Doppler sonography image shows increased vascularity, especially in periphery of lesion.

View larger version (162K): [in this window] [in a new window] [as a PowerPoint slide]   Fig. 1D —25-year-old lactating woman with desmoid tumor of left breast. Photomicrograph shows lesion microscopically is composed of bland-appearing fibroblasts of moderate cellularity without appreciable mitotic activity. (H and E, x200)

Desmoid tumors constitute 0.2% of all breast tumors and 0.3% of all solid tumors [1]. Desmoid tumor confined to the breast and not involving the underlying fibroaponeurotic fascia is a rare lesion. The cause of desmoid tumors is not well understood, but an association with Gardner's syndrome, silicone breast implants, or surgical trauma has been reported. Although its occurrence is more common in young and fertile women, desmoid tumors also have been reported in men. In male patients, the growth rate has been reported as low. Estrogen dominance, as is the case during pregnancy, is considered to be a significant predisposition for developing desmoid tumor.

Clinically, desmoid tumors of the breast are movable, firm masses. Skin retraction or dimpling may be present. Lesions close to the nipple may present with nipple retraction. Mammographically, desmoid tumors are often irregularly shaped, uncalcified, high-density masses with spiculated margins that mimic breast cancer [2]. By sonography, desmoid tumors are frequently poorly marginated, hypoechoic masses with a thick echogenic rim and posterior attenuation [3]. More benign appearances have also been reported and are characterized by circumscribed borders and posterior acoustic enhancement.

By MRI, desmoid tumors appear as illdefined, hypo- to isointense masses on T1-weighted images and as heterogeneously hyperintense masses on T2-weighted images. They show suspicious, slow enhancement after contrast administration [4]. MRI is also useful to show chest wall involvement important for surgical planning.

Histologically, the lesion is composed of bundles of long sweeping and intersecting spindle cells with varying amounts of collagen deposition. Fingerlike extensions extend at the periphery of the lesion into adjacent breast parenchyma and adipose tissue. The histopathologic differential diagnosis includes benign reactive lesions such as a scar, fibrosarcoma, and the recently described fibromatosislike metaplastic spindle cell tumor.

The hallmark of a desmoid tumor is the presence of bland-looking spindle cells organized into long sweeping and intersecting fascicles. The overall cellularity is low to moderate, and there is no significant mitotic activity. The distinction between a desmoid tumor and fibrosarcoma is particularly important, as the latter has the capacity for distant metastasis [5].

Current management of desmoid tumors of the breast favors a wide excision with clear margins [6]. Since desmoid tumor is a benign process, the cosmetic result of the surgical procedure should be considered during surgical planning. However, recurrence is relatively common. It usually recurs within 3 years of excision and requires radical surgery. Adjuvant antihormonal therapy does not reduce the local recurrence rate, but postoperative radiation therapy can improve the 10-year recurrence-free survival rate.

References

1. Rosen PP, Ernsberger D. Mammary fibromatosis: a benign spindle-cell tumor with significant risk for local recurrence. Cancer 1989; 63:1363 –1369[CrossRef][Medline]
2. Cederlund CG, Gustavsson S, Linell F, Moquist-Olsson I, Andersson I. Fibromatosis of the breast mimicking carcinoma at mammography. Br J Radiol 1984;57 : 98–101[Medline]
3. Leibman AJ, Kossoff MB. Sonographic features of fibromatosis of the breast. J Ultrasound Med 1991;10 : 43–45[Medline]
4. Nakazono T, Satoh T, Hamamoto T, Kudo S. Dynamic MRI of fibromatosis of the breast. AJR 2003;181 :1718 –1719[Free Full Text]
5. Jones MW, Norris HJ, Wargotz ES, Weiss SW. Fibrosarcoma–malignant fibrous histiocytoma of the breast: a clinicopathological study of 32 cases. Am J Surg Pathol 1992; 16:667 –674[Medline]
6. Godwin Y, McCulloch TA, Sully L. Extra-abdominal desmoid tumour of the breast: review of the primary management and the implications for breast reconstruction. Br J Plast Surg2001; 54:268 –271[Medline]

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				H. B. Neuman, E. Brogi, A. Ebrahim, M. F. Brennan, and K. J. Van Zee Desmoid Tumors (Fibromatoses) of the Breast: A 25-Year Experience Ann. Surg. Oncol., January 1, 2008; 15(1): 274 - 280. [Abstract] [Full Text] [PDF]

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