(Radiology. 2001;219:781-784.)
© RSNA, 2001
Case 35: Intracystic Papillary Carcinoma with Invasion1
Laura Liberman, MD,
Timothy L. Feng, MD and
Barbara Susnik, MD
1 From the Departments of Radiology, Breast Imaging Section (L.L., T.L.F.), and Pathology (B.S.), Memorial Sloan–Kettering Cancer Center, 1275 York Ave, New York, NY 10021. Received March 11, 1999; revision requested May 3; final revision received September 9; accepted September 27. Address correspondence to L.L. (e-mail: libermal@mskcc.org).
Index terms: Breast neoplasms, diagnosis, 00.325 • Breast neoplasms, US, 00.1298, 00.12983 • Diagnosis Please
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HISTORY
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A 47-year-old woman with an unremarkable medical history presented with a nontender right breast mass of 6 months duration. She denied fever, nipple discharge, and other symptoms. Results of physical examination were remarkable for a large "cystlike" mass in the right lower outer quadrant at 8 o’clock and a mobile right axillary lymph node. There was no erythema, skin thickening, retraction, or ulceration.
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IMAGING FINDINGS
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A mediolateral oblique right mammogram revealed a dense, round, circumscribed mass 8.5 cm at the longest dimension in the lower outer quadrant, which corresponded to the palpable mass, and a dense right axillary lymph node 2.5 cm at the longest dimension (Fig 1). Ultrasonography (US) of the right breast mass showed it to be complex, with anechoic portions, septa, mural nodularity, and papillary projections (Fig 2). Doppler US interrogation demonstrated flow within the septa of the mass (Fig 3). US of the right axilla showed a mass consistent with an axillary node that was homogeneously hypoechoic, with loss of the normal echogenic hilum (Fig 4).
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Figure 1. Mediolateral oblique right mammogram shows a large, dense, round, circumscribed mass (solid arrow) in the lower outer quadrant, which corresponds to the palpable mass. A dense right axillary lymph node (open arrow) is seen in the right axilla. No skin thickening is identified.
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Figure 2a. Transverse US images of the palpable right breast mass in the lower outer quadrant in its (a) lateral, (b) central, and (c) medial portions demonstrate a complex mass with anechoic portions, as well as mural nodularity (open arrow in b and c), papillary projections (black arrow in c), and septa (white arrows in a and b). No overlying skin thickening is seen.
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Figure 2b. Transverse US images of the palpable right breast mass in the lower outer quadrant in its (a) lateral, (b) central, and (c) medial portions demonstrate a complex mass with anechoic portions, as well as mural nodularity (open arrow in b and c), papillary projections (black arrow in c), and septa (white arrows in a and b). No overlying skin thickening is seen.
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Figure 2c. Transverse US images of the palpable right breast mass in the lower outer quadrant in its (a) lateral, (b) central, and (c) medial portions demonstrate a complex mass with anechoic portions, as well as mural nodularity (open arrow in b and c), papillary projections (black arrow in c), and septa (white arrows in a and b). No overlying skin thickening is seen.
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DISCUSSION
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Although 98% of circumscribed breast masses are benign (1), the differential diagnosis of a large circumscribed breast mass includes benign entities (eg, cyst, fibroadenoma, papilloma, hematoma, or infection or abscess), as well as lesions that are potentially malignant (phyllodes tumor) or malignant (infiltrating ductal carcinoma not otherwise specified; specialized subtypes of infiltrating ductal carcinoma such as medullary carcinoma, mucinous or colloid carcinoma, or invasive papillary carcinoma; papillary ductal carcinoma in situ; hematopoietic tumors such as lymphoma; or metastatic disease).
In this case, a specific diagnosis can be suggested on the basis of the history and imaging findings. There is nothing in the clinical history to support a diagnosis of metastatic disease. The history and physical examination results, particularly the absence of erythema, skin thickening, and tenderness, argue against infection. The US findings argue against phyllodes tumor: Phyllodes tumors are usually solid at US, occasionally with anechoic regions within the solid mass (2). The presence of a suspicious-looking axillary lymph node favors a malignant lesion that spreads via the lymphatic vessels; this renders less likely the diagnosis of phyllodes tumor, which usually spreads hematogenously (2,3), or papillary ductal carcinoma in situ, which does not involve lymph nodes. The US features (intracystic mural nodules, papillary projections, and septa) in conjunction with the mammographic findings (a large circumscribed mass with a suspicious-looking axillary node) support the diagnosis of intracystic papillary carcinoma with invasion.
Papillary carcinoma, an unusual ductal malignancy, accounts for 1%–2% of breast carcinomas (4). The histologic hallmark of all papillary tumors, benign or malignant, is the arborization of fibrovascular stroma supporting the epithelial component. An inconspicuous or absent myoepithelial layer distinguishes papillary carcinomas from benign papillary lesions (4). Papillary carcinomas may be solitary or multiple. When the epithelium of a papillary carcinoma has features diagnostic of intraductal carcinoma, the lesion is classified as papillary ductal carcinoma in situ. If a cystic component is present, the tumor is described as an intracystic papillary carcinoma. In the absence of an appreciable cyst, a diagnosis of solid papillary carcinoma is appropriate. The relative frequency of cystic and solid papillary carcinoma is not known because of inconsistent reporting of these lesions, but solid papillary carcinomas have a higher tendency to invade (Rosen PP, oral communication, 1999).
Invasive elements arising in a papillary carcinoma are almost always detected at the periphery of the lesion; these invasive elements can have various growth patterns and rarely contain papillary structures. Grossly, papillary carcinomas are usually well circumscribed and often contain hemorrhagic and cystic areas (4). The fluid in intracystic papillary carcinomas is formed by the cyst and papillary growth. Hemorrhage may result from biopsy or may occur spontaneously.
The mean patient age at diagnosis of papillary carcinoma is 63–67 years. Approximately 50% of papillary carcinomas arise in the central (retroareolar) portion of the breast. Papillary carcinoma may manifest clinically as a palpable mass or nipple discharge, with the latter present in 22%–34% of patients; symptom duration of 1 year or more is not unusual. Although invasive papillary carcinomas tend to manifest as large masses owing to the frequent presence of a bulky cystic component, the size of the invasive element is often small, and axillary metastases are infrequent (4). Papillary carcinoma may also manifest as an asymptomatic lesion identified at screening mammography.
The most common mammographic pattern of invasive papillary carcinoma is a round, oval, or lobulated mass; multiple masses may occur, often within one quadrant (5–13). The mass margins are usually circumscribed but may be obscured or indistinct; spiculation is rare. US evaluation of invasive papillary carcinomas may reveal single or multiple masses (5,6,9,10,12–14). The masses may appear hypoechoic and solid, often with posterior acoustic enhancement; alternatively, complex cystic and solid masses may be evident, with mural nodularity and papillary projections (10,14). Anechoic regions are often present and may represent the US correlate of the hemorrhagic and cystic areas seen at pathologic evaluation.
There is overlap in the imaging patterns of invasive papillary carcinoma and papillary ductal carcinoma in situ (10). Papillary ductal carcinoma in situ may manifest as single or multiple clusters of calcifications at mammography, sometimes within dilated ducts. Alternatively, papillary ductal carcinoma in situ may be evident as single or multiple circumscribed masses at mammography and as solid or complex enhancing masses at US, indistinguishable from those described for invasive papillary carcinoma. Doppler US may demonstrate intratumoral blood flow or large feeding vessels (10).
For patients presenting with nipple discharge, galactography may be helpful in diagnosis. Like papillomas, papillary carcinomas may be evident at galactography as ductal obstruction, filling defects, or focal or diffuse ductal wall irregularity (15). Other galactographic findings described in papillary carcinomas include periductal extravasation of contrast material and displacement of otherwise normal ducts (15). Although useful in identifying and localizing intraductal lesions, galactography may not allow distinction between papilloma and papillary carcinoma or between papillary carcinoma with and that without invasion.
In this case, fine-needle aspiration of the right axillary lymph node demonstrated adenocarcinoma (Fig 5). Subsequent right mastectomy and axillary lymph node dissection demonstrated intracystic papillary carcinoma with invasion extending beyond the wall of the cyst (Fig 6). Axillary lymph node metastases were present.
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Figure 5. Photomicrograph of fine-needle aspiration cytologic specimen from the right axillary lymph node shows adenocarcinoma with high nuclear grade. Malignant cells (arrowhead) are identified singly and in clusters. (Hematoxylin-eosin stain; original magnification, x40.)
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Figure 6. High-power photomicrograph of a section obtained from the breast mass reveals a papillary frond (arrowheads) with a fibrovascular core (C) lined by cytologically malignant epithelium. The epithelium shows an increased number of pleomorphic cells (arrows) with enlarged hyperchromatic nuclei in a disorderly arrangement. Results of mastectomy and axillary lymph node dissection (not shown) demonstrated intracystic papillary carcinoma with invasion and axillary metastases. (Hematoxylin-eosin stain; original magnification, x20.)
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Our congratulations to the 42 individuals who submitted the most likely diagnosis (intracystic papillary carcinoma with invasion) for Diagnosis Please, Case 35. The names and locations of the individuals, as submitted, are as follows:
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- Gholamali Afshang, MD, Tinley Park, Ill
- Albert J. Alter, Madison, Wis
- A. R. Belknap, MD, Butte, Mont
- Amita Bhandari, MD, South San Francisco, Calif
- Dr. Rita J. Blom, Errington, British Columbia, Canada
- R. James Brenner, MD, FACR, Santa Monica, Calif
- Dr. Ioannis Chrissafis, Athens, Greece
- Wiluck Chu-Ongsakul, MD, Bangkok, Thailand
- John Coscia, MD, Fort Worth, Tex
- Kemal Demir, MD, Istanbul, Turkey
- Dra. Estela Di Nella, Buenos Aires, Argentina
- Dr. Chris I. Flowers, FRCR, Swansea, United Kingdom
- Milton R. Fuentealba, MD, General Roca, Rio Negro, Argentina
- José Garcia Revillo, MD, Córdoba, Spain
- Bhaskar Golla, MD, Rochester, NY
- Walter O. Grauer, MD, Zurich, Switzerland
- Ferris M. Hall, MD, Boston, Mass
- Helen T. Ho, MD, Chicago, Ill
- Marc J. Homer, MD, Boston, Mass
- Albert Kaminsky, Melbourne, Victoria, Australia
- Nancy Keroles, Rancho Palos Verdes, Calif
- Edward W. Kouri, MD, Charlotte, NC
- Eduardo Lassalle, MD, Quilmes, Argentina
- Peter Leyman, Aalst, Belgium
- Maria Angela Losano, MD, General Roca, Rio Negro, Argentina
- Stephen Manghisi, MD, Closter, NJ
- Katherine Martin, MD, Seattle, Wash
- Edward Menges, MD, Aptos, Calif
- Sergio J. Moguillansky, MD, Cipolletti, Rio Negro, Argentina
- Toshio Moritani, Rochester, NY
- Sanford M. Ornstein, MD, Phoenix, Ariz
- Harish Patel, MD, Little Rock, Ark
- Victor A. Pérez-Candela, MD, Las Palmas de Gran Canaria, Spain
- Shawn P. Quillin, MD, Charlotte, NC
- Enrique Remartinez Escobar, MD, Melilla, Spain
- Luiz Antonio Rossi, São Paulo, Brazil
- Steven M. Schultz, MD, Fort Worth, Tex
- Matt Shapiro, MD, Lowell, Mass
- S. Horatio Slawson, MD, Peoria, Ill
- D. Dean Thornton, MD, Birmingham, Ala
- Phillip Unger, MD, Fullerton, Calif
- Joe Yut, Olathe, Kan
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ACKNOWLEDGMENTS
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The authors thank Paul Peter Rosen, MD, for critical review of the manuscript and David C. Perlman, MD, for invaluable assistance.
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FOOTNOTES
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Part 1 of this case appeared 4 months previously and may contain larger images.
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REFERENCES
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Rosen PP. Papillary carcinoma In: Rosen’s breast pathology. Philadelphia, Pa: Lippincott-Raven, 1997; 335-354.
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TOP
HISTORY
IMAGING FINDINGS
