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Intracystic Papillary Carcinoma of the Breast with Invasion

¹ Division of Diagnostic Imaging, The University of Texas M. D. Anderson Cancer Center, 1515 Holcombe Blvd., Box 57, Houston, TX 77030.
² Division of Pathology, The University of Texas M. D. Anderson Cancer Center, Houston, TX 77030.

Received October 21, 2003; accepted after revision November 5, 2003.

Address correspondence to G. J. Whitman (gwhitman{at}di.mdacc.tmc.edu).

A 69-year-old woman presented to her local physician with a large right breast mass associated with hyperpigmentation. Mammography showed a large dense lobulated mass (Fig. 1A). Sonography showed a large complex mass with solid and cystic components (Fig. 1B). Sonographically guided core needle biopsy revealed high-grade papillary carcinoma in situ with apocrine features. The patient underwent a modified radical right mastectomy, and a pink friable intracystic tumor was removed (Fig. 1C). The tumor measured 9 cm in greatest dimension and had papillary architecture (Fig. 1D). The final histopathologic diagnosis was intracystic papillary carcinoma with apocrine features and associated invasive carcinoma. Axillary dissection revealed two of 16 axillary lymph nodes with metastatic carcinoma.

View larger version (67K): [in this window] [in a new window] [as a PowerPoint slide]   Fig. 1A. —69-year-old woman with intracystic papillary carcinoma of breast with invasion. Right mediolateral oblique mammogram shows large lobulated right breast mass.

View larger version (132K): [in this window] [in a new window] [as a PowerPoint slide]   Fig. 1B. —69-year-old woman with intracystic papillary carcinoma of breast with invasion. Extended-field-of-view sonogram shows complex cystic masses in upper outer right breast.

View larger version (95K): [in this window] [in a new window] [as a PowerPoint slide]   Fig. 1C. —69-year-old woman with intracystic papillary carcinoma of breast with invasion. Photograph of gross specimen from mastectomy shows lobulated tumor (arrow) within hemorrhagic cystic space.

View larger version (177K): [in this window] [in a new window] [as a PowerPoint slide]   Fig. 1D. —69-year-old woman with intracystic papillary carcinoma of breast with invasion. Photomicrograph of histopathologic specimen shows invasive carcinoma (arrow) extending into surrounding fibrous stroma. (H and E, x100)

Papillary carcinomas are rare malignant breast tumors, constituting 1–2% of breast carcinomas in women [1, 2]. Generally characterized by slow growth and a better prognosis than ductal carcinomas not otherwise specified, papillary carcinomas may have in situ (intracystic or intraductal) or invasive components [3]. Intracystic papillary carcinoma can occur in a pure form, or it may be associated with ductal carcinoma in situ or invasive carcinoma not otherwise specified [4].

On mammography, intracystic papillary carcinoma is usually seen as a well-circumscribed mass. Occasionally, satellite nodules or microcalcifications or both are present. Solid or complex cystic masses are often seen on sonography, and color Doppler sonography may show intratumoral blood flow or large feeding vessels. Contrast-enhanced MRI may show marked enhancement of cyst walls, septations, and mural nodules [3].

Pathologically, intracystic papillary carcinomas may show four cellular patterns: cribriform, compact columnar epithelial, stratified spindle cell, or a transitional cell form resembling urothelium, or a combination of two or more of these patterns may be seen [5]. The nuclear grade is assessed from 1 to 3 depending on the intensity of the nuclear hyperchromasia, pleomorphism, and the nuclear–cytoplasmic ratio [4]. Intracystic papillary carcinomas often have fibrous and vascular elements, and necrosis is often a prominent feature when an associated invasive component is present [2]. Most tumors are pink to tan, have a soft or friable consistency, and have a spherical circumscribed contour [5]. Hemorrhagic areas within the solid components of the tumor and blood and fluid within the cystic spaces are often identified [5].

Intracystic papillary carcinomas may be managed by mastectomy or segmental resection. Radiation therapy may also be administered. Sentinel lymph node biopsies or axillary dissections are often performed to assess the axillary lymph nodes [4]. The prognosis for intracystic papillary carcinoma is usually very good with disease-specific survival rates approaching 100% [6]. In cases with a large amount of nuclear atypia, the prognosis may be less optimistic [2].

References

1. Fisher ER, Palekar AS, Redmond C, Barton B, Fisher B. Pathologic findings from the National Surgical Adjuvant Breast Project (protocol no. 4). VI. Invasive papillary cancer. Am J Clin Pathol1980; 73:313 –322[Medline]
2. Carter D, Orr SL, Merino MJ. Intracystic papillary carcinoma of the breast. After mastectomy, radiotherapy or excisional biopsy alone. Cancer 1983;52:14 –19[Medline]
3. Soo MS, Williford ME, Walsh R, Bentley RC, Kornguth PJ. Papillary carcinoma of the breast: imaging findings. AJR1995; 164:321 –326[Abstract/Free Full Text]
4. Leal C, Costa I, Fonseca D, Lopes P, Bento MJ, Lopes C. Intracystic (encysted) papillary carcinoma of the breast: a clinical, pathological, and immunohistochemical study. Hum Pathol1998; 29:1097 –1104[Medline]
5. Lefkowitz M, Lefkowitz W, Wargotz ES. Intraductal (intracystic) papillary carcinoma of the breast and its variants: a clinicopathological study of 77 cases. Hum Pathol1994; 25:802 –809[Medline]
6. Solorzano CC, Middleton LP, Hunt KK, et al. Treatment and outcome of patients with intracystic papillary carcinoma of the breast. Am J Surg 2002;184:364 –368[Medline]

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