Diffuse Cystic Angiomatosis of the Breast

MONICA MORROW, MD,* DAVID BERGER, MD,* AND WILLIAM THELMO, MDt

Vascular tumors of the breast are extremely rare, and the majority are malignant. In this report, the presentation of a diffuse, benign, cystic, vascular tumor that histologically was angiomatosis of the breast is described. The clinical presentation, magnetic resonance imaging (MRI) appearance, and histologic features of this rare lesion are reviewed. This case is of particular interest because the tumor recurred during pregnancy and underwent massive enlargement despite negative hormone receptor values. Angi- omatosis is prone to local recurrence, so complete excision with histologically negative margins is the treatment of choice.

Cuncer 62:2392-2396, 1988.

ASCULAR TUMORS OF THE BREAST are extremely V rare. The literature on this subject focuses on the distinguishing pathologic and clinical features of angio- sarcoma, the most common vascular lesion of the

Scanty information exists to guide the clinician in the management of benign vascular In this report, the presentation and management of a case of dif- fuse cystic angiomatosis of the breast is described and the literature on this subject is reviewed.

Case Report

The patient, a 19-year-old Haitian woman, was seen initially in July 1985 with the complaint of an increase in the size of her right breast. She was nulliparous, with no prior history of breast disease. She had a negative family history for breast carcinoma. The breast enlargement was painless and was not associated with skin changes, nipple discharge, or fever. There was no history of breast trauma. Physical examination showed the right breast to be approximately twice the size of the left. The upper outer quadrant was occupied by a poorly defined, nontender, cystic mass that transilluminated. There was no adenopathy. Needle aspiration of the mass yielded 50 ml of straw-colored fluid. This fluid was cytologically negative. Aspiration of the cyst resulted in some decrease in breast size, but the right breast remained larger than the left. At reexamination 1 week later, the cystic cavity had refilled. Repeat aspiration yielded 125 ml of serosan- guinous fluid. Surgical excision of the mass was recommended. At surgery, an 11 X 5 X 4-cm poorly encapsulated cystic mass was excised. The mass bled profusely, and the estimated blood loss from the excisional biopsy was 700 ml. The patient recovered uneventfully, and the pathologic diagnosis was “blood filled cyst

From the Departments of *Surgery and tPathology, SUNY-Health Science Center at Brooklyn, Brooklyn, New York.

Address for reprints: Monica Morrow, MD, The University of Chicago, Department of Surgery (Box 402). 5841 South Maryland Avenue, Chi- cago, IL 60637.

Accepted for publication June 9, 1988.

with granulation tissue consistent with cavernous hemangioma.” The patient was lost to follow-up 3 months postoperatively at which time there was no evidence of recurrence. She was seen again in April 1987 at 36 weeks gestation with the complaint of progressive enlargement of her right breast throughout the course of her pregnancy. The right breast was now four times the size of the left (Fig. 1). A diffuse, cystic, poorly defined lesion was present throughout the breast with hyperpigmentation of the overlying skin. There was no associated bruit, nipple inversion, or axillary adenopathy. After the delivery of a healthy baby, the patient underwent a magnetic resonance imaging (MRI) scan to define the extent of the breast lesion. The MRI scan showed a 15 X 10-cm mass with fatty septa that occupied the bulk of the right breast. The mass was surrounded by intact fat and did not involve the chest wall. Tz-weighted images showed a striking increase in signal intensity believed to be consistent with hem- angioma (Fig. 2). Complete excision of the mass with less than a total mastectomy was believed to be unfeasible due to the size and vascular nature of the mass. On the operating table, 1200 ml of straw-colored fluid was aspirated from the breast. The patient then underwent an uncomplicated simple mastectomy with a 100-ml blood loss. Her postoperative course was un- eventful and she is doing well in the early posthospital period.

Results

The mastectomy specimen weighed 1300 g and mea- sured 22 X 14 X 8 cm. The majority of the breast was occupied by a large unilocular cyst measuring 15 cm in diameter. This cyst was partially filled with serosanguinous fluid. The cyst was 2.5 to 3.0 cm thick and had a spongy appearance. It was not encapsulated and was surrounded by a distinct rim of normal adipose tissue (Fig. 3).

Histopathologic examination of the cyst wall showed a diffuse network of small and large, empty, anastomosing, vascular channels. The vascular channels were seen to proliferate around the breast lobules. The endothelial lin- ing of the vascular channels was flat and normal in ap-

2392

No. I 1 ANGIOMATOSIS OF THE BREAST * Morrow et a/.

FIG. 1. Appearance of the right breast at the time of surgery. Massive enlargement and hyperpigmentation of the skin are apparent.

2393

FIG. 2. The T,-weighted images show a striking increase in signal intensity consistent with a vascular lesion.

2394 CANCER December 1 1988 Vol. 62

pearance, with no mitosis or tufting of the endothelial cells (Fig. 4). Comparison with the original biopsy spec- imen of 2 years ago failed to show any of the atypical plump endothelial cells seen at that time. Extensive sam- pling of the lesion did not demonstrate any of the features of low-grade angiosarcoma. The final pathologic diagnosis was angiomatosis (diffuse hemangioma) of the breast. The estrogen receptor content of the tumor was 8 femtomoles (fmol) per milligram and the progesterone receptor con- tent was less than 5 fmol/mg.

Discussion

Vascular tumors of the breast are extremely rare lesions, and the majority are Currently, approxi- mately 150 cases of angiosarcoma of the breast have been reported in the literature. Only recently have benign vascular tumors of the breast been recognized as clinical entities capable of producing symptom^.^-^ In a recent review of 100 clinically evident vascular tumors of the breast, 62 were angiosarcomas and only 20 were charac- terized as benign, symptomatic, mammary parenchymal tumors. The benign lesions included venous and capillary hemangiomas and angiomatosis, with angiomatosis being the least common of these lesions.s-8 Three previous cases of angiomatosis of the breast have been described by Ro- sen,6 and an additional case was described here.

FIG. 3. Cross section through the cyst. The large unilocular cyst, thick spongy wall, and surrounding rim of fat are seen.

The lesions of angiomatosis are relatively large, with all the specimens in Rosen's series measuring at least 9 X 5 cm.6 The specimen in this report was significantly larger, weighing 1300 g and measuring 22 X 14 X 8 cm. The histology in this case was similar to that reported previously for angiomatosis, a lesion characterized by dif- fuse, empty, anastornosing, vascular channels with flat endothelial cells. All cases of angiomatosis lack two of the most important features of angiosarcoma, tufting and hy- perchromasia of the endothelial cells.6 The current case is unique in that the central portion of this tumor was a large, thick-walled cyst, with the wall of the cyst com- prising the angiomatous component of the lesion.

Angiomatosis, like angiosarcoma, appears to be a dis- ease found mostly in young women. The four patients reported as having angiomatosis currently have a mean age of 33 years. The mean age of the patients with angio- sarcoma in the review by Donne11 et al. was 34 years, with most patients presenting in their third and fourth decades.

The diagnosis of angiomatosis of the breast is a difficult one to make. Differentiation of angiomatosis from angio- sarcoma is crucial. Patients with both malignant and be- nign vascular breast tumors usually have a painless breast mass.'-' Imaging studies may be helpful in identifying the tumor as being vascular in nature, as the MRI scan did in this case. However, imaging studies are not helpful in differentiating benign vascular tumors from malignant

No. 11 ANGIOMATOSIS OF THE BREAST * Morrow et al. 2395

FIGS. 4A AND 4B. (A) Photomicrograph demonstrating anastomosing, empty vascular channels of varying sizes dissecting around the breast lobules (H & E, X60). (B) Photomicro- graph demonstrating the flat, normal endothe- lial cells of the vascular channels seen in (A) (H & E, X 100).

vascular tumors. Histologically, angiosarcoma is difficult to diagnose because it may exhibit areas with a benign appearance. Dunegan et al., in a review of angiosarcoma of the breast, reported 19 of 48 patients diagnosed initially

as having benign lesion^.^ Chen et al., in a review of 82 cases, reported 32 as not being identified as malignant initially.*

Angiomata of other bodily soft tissue sites are prone to

2396 CANCER December 1 1988 Vol. 62

recur if not excised ~ompletely.~ Our patient had recur- rence of her lesion 19 months after the initial excisional biopsy. Also, Rosen reported one patient who appeared to have two recurrences of her lesion.6 This suggests that angiomatosis of the breast behaves similarly to angiomata at other sites. Due to the propensity for local recurrence and the difficulty of distinguishing angiomatosis from an- giosarcoma on small biopsy specimens, we concur with other authors in recommending complete excision of all vascular lesions of the breast. Simple mastectomy is in- dicated for benign lesions that cannot be removed com- pletely by excisional biopsy. In addition, reexcision of the biopsy site is indicated if there are any questions as to the margin of resection.'-8 Angiomatosis has not been found to metastasize in any case,6 however, close follow-up is indicated because experience with this lesion is limited.

There is some indication that vascular tumors of the breast may have hormonal dependence. In the review by Chen ez al., 1 1 of 87 cases of angiosarcoma occurred dur- ing pregnan~y.~ Hormone receptor data for vascular le- sions of the breast are extremely scanty. Brentani et al. reported the presence of high affinity receptors for estro- gen, progesterone, and glucocorticoids" in two cases of mammary angiosarcoma, whereas Bracaglia et al. were unable to detect receptors for estradiol, progesterone, or testosterone in one case." In the current report, our pa- tient had dramatic growth of her tumor during pregnancy. However, both the estrogen receptor level (8 fmol/mg) of this tumor and the progesterone receptor level (4 fmol/ mg) were negative. Further data are needed before con- clusions regarding the hormonal sensitivity of this lesion are made.

In summary, benign symptomatic vascular tumors of the breast are extremely rare lesions, with angiomatosis being the least common of these tumors. Angiomatosis usually occurs at a young age and presents as a painless mass. Before any vascular tumor of the breast is considered benign, a thorough histologic evaluation of the entire le- sion is necessary. Definitive treatment of angiomatosis involves wide local excision or simple mastectomy.

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