Diffuse Atypical Papillomatosis of the Breast: Insights, Imaging, and Management
Diffuse atypical papillomatosis of the breast
is a rare and clinically significant papillary lesion involving multiple
intraductal papillomas with cytologic atypia. These lesions are typically
confined to a breast segment, but they may extend more diffusely or appear
bilaterally. Occupying a "borderline" space between benign papillomas
and ductal carcinoma in situ (DCIS), this condition requires meticulous imaging
evaluation and a multidisciplinary treatment strategy.
1.
Definition and Pathologic Spectrum
Papillary
breast lesions are defined histologically by frond-like fibrovascular cores
lined by both epithelial and myoepithelial cells. When five or more intraductal
papillomas occur within a single segment and exhibit atypical ductal
hyperplasia (ADH) or low-grade DCIS, they are categorized as diffuse atypical papillomatosis. This
entity has a well-documented risk of malignant transformation.
2.
Clinical Presentation and Epidemiology
This
condition most commonly affects women between the ages of 30 and 50, although
cases outside this range have been reported. Many patients remain asymptomatic.
When symptoms do occur, they may include:
A
palpable breast mass
Nipple
discharge
Mammographic
asymmetry
In
a highlighted case, the lesions were discovered incidentally during routine
imaging in the absence of clinical symptoms such as nipple discharge.
3.
Imaging Characteristics
A
multimodal imaging approach—combining mammography, ultrasound, and magnetic
resonance imaging (MRI)—is crucial for accurately evaluating the extent and
nature of the disease.
3.1
Mammography
Mammography
may demonstrate:
Clustered
nodular densities
Mass-like
asymmetries
Occasional
calcifications
However,
the actual disease extent is often underestimated radiographically.
Figure 1. Bilateral
digital mammogram reveals multiple small masses in the inferior medial quadrant
of the right breast, extending from anterior to posterior depth.
3.2
Ultrasound
Sonography
typically reveals:
Multiple
hypoechoic, irregular, or lobulated intraductal masses
Lesions are usually <1 cm in size
Vascular
stalks are evident on color Doppler
Figure 2. Targeted
ultrasound demonstrates multiple small, irregular, and loculated solid
hypoechoic lesions, correlating with mammographic findings.
3.3 MRI
MRI
is superior in delineating:
Segmental
or ductal enhancement
“String-of-pearls”
enhancement pattern
Multifocal
or bilateral disease involvement
Figure 3. MRI 3D
subtraction MIP image shows multiple segmental, tiny enhancing lesions in the
right breast (arrow).
4.
Histopathology and Underestimation Risk
Histologic
sampling via core needle biopsy
often underestimates the extent and grade of the lesion. Studies have shown that surgical excision results in a diagnostic upgrade in up to 67% of cases where atypia was initially
observed.
Figure 4 (C)
Low-power magnification shows solid hyperplastic architecture, with epithelial
proliferation filling ductal spaces and no glandular structures. (D)
High-power magnification demonstrates cytologic atypia, with uniform epithelial
proliferation, enlarged nuclei, and chromatin clumping near the nuclear
membrane.
These
histologic findings support the lesion’s potential for malignant progression
and justify surgical excision in most cases.
5.
Malignancy Risk
While
solitary papillomas have a
slightly elevated malignancy risk (1.5–2×), multiple papillomas with atypia increase the likelihood
of invasive carcinoma up to 7-fold.
This heightened risk demands aggressive and personalized treatment planning.
6.
Therapeutic Approach
6.1
Surgical Excision
Surgical
removal is recommended for all papillomas with atypia or discordant
imaging-pathology findings. Excision with ≥10 mm margins helps prevent local
recurrence.
6.2
Mastectomy
Mastectomy
is considered when:
The
disease is segmentally extensive
Multicentric
involvement is confirmed
Patient
preference dictates a more definitive approach
In
the reviewed case, bilateral mastectomy was performed—therapeutic on the right
and prophylactic on the left—largely influenced by patient anxiety. Final
pathology revealed diffuse atypical papillomatosis with no invasive carcinoma.
6.3
Reconstruction and Oncoplastic Techniques
For
patients undergoing mastectomy, nipple-sparing
mastectomy and reconstructive
surgery should be discussed to ensure oncologic safety and cosmetic
preservation.
7.
Surveillance and Multidisciplinary Management
7.1
Surveillance Strategy
Annual mammography is essential.
Breast MRI is advisable
for patients with dense breast tissue or high-risk histologic profiles.
Interval ultrasound may serve as a
supplementary tool, especially after breast-conserving surgery.
7.2
Multidisciplinary Collaboration
Care
should involve:
Breast
radiologists
Pathologists
Surgical
oncologists
Medical
oncologists
The
patient’s informed preferences
7.3
Psychosocial Support
Due
to the disease’s uncertain prognosis and possible disfiguring surgeries,
psychological counseling and peer support systems are invaluable.
8. Future
Directions
Emerging
technologies and innovations are reshaping management:
AI-enhanced imaging for more
accurate detection and classification
Molecular profiling for risk
stratification
Vacuum-assisted excision
as a less invasive therapeutic option
Prospective studies are needed to
define thresholds for mastectomy, optimal follow-up intervals, and the role of
newer diagnostic tools
Key
Clinical Takeaways
|
Point |
Clinical Implication |
|
Core needle biopsy
has high underestimation rates |
Surgical excision is
essential |
|
Atypia and
multifocality elevate malignancy risk |
Consider a mastectomy
for extensive disease |
|
MRI is the most
comprehensive imaging tool |
Crucial for surgical
planning |
|
Multidisciplinary
care improves outcomes |
Include patient
perspectives |
|
Lifelong surveillance
is mandatory |
Utilize mammogram ±
MRI/ultrasound |
References
Muttarak
M, et al. Spectrum of papillary lesions of
the breast: Clinical, imaging, and pathologic correlation. AJR.
2008;191:700–707. DOI: 10.2214/AJR.07.3483
Mercado
CL, et al. Papillary lesions of the breast at
percutaneous core needle biopsy. Radiology. 2006;238:801–808. DOI: 10.1148/radiol.2382041839
Ueng
S, et al. Papillary neoplasms of the breast:
A review. Arch Pathol Lab Med. 2009;133:893 907. DOI: 10.5858/133.6.893
Rella
R, et al. Multiple papillomas of the breast:
A review. J Imaging. 2022;8(7):198. DOI: 10.3390/jimaging8070198
Debnath
D, et al. Multiple papillomatosis of the breast
and the patient’s choice of treatment. Pathol Res Int. 2010;2010:540590. DOI: 10.4061/2010/540590
Al Sarakbi M, et al. Breast papillomas: Current management and new therapeutic modalities. Int Semin Surg Oncol. 2006;3:1. DOI: 10.1186/1477-7800-3-1
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