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2022, Volume 38, Number 2, Page(s) 153-157
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DOI: 10.5146/tjpath.2021.01543 |
Pleomorphic Adenoma of Breast: Report of Two Cases, One in A Male Patient |
Orwa ELAIWY, Khaled AL-SAWALMEH, Hayan ABO SAMRA, Abdulrazzaq HAIDER, Mohammed AKHTAR |
Department of Laboratory Medicine and Pathology, Hamad Medical Corporation, DOHA, QATAR |
Keywords: Pleomorphic adenoma, Breast, Male breast, Cartilage, Papilloma |
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Pleomorphic adenoma of breast (PAB) is a rare mammary tumor of a mixed epithelial-myoepithelial nature. We report two patients with PAB,
one of which is male. We believe our male patient is the sixth case of PAB in male breast in the literature. The two cases expressed heterogeneous
clinical and radiological characteristics while showing similar histology and immunohistochemical staining profile. The first case was managed
with surgical resection while the second underwent interventional radiology excision. PAB is usually a benign entity with occasional cases of
recurrence. Malignant transformation is rare but has been reported in a few cases. |
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Pleomorphic adenoma of breast (PAB) is a rare mammary
tumor of a mixed epithelial-myoepithelial nature and is
part of the so-called salivary gland-type mammary tumors,
initially recognized in 1906. The existence of such tumors
in the breast can be attributed to a shared embryonic origin
between salivary and mammary glands 1. Although most
patients are female with a wide age range, a few cases have
also been reported in males. PABs typically manifest as a
retro areolar, usually unifocal palpable mass 2. Imaging
studies may show a fibroadenoma-like appearance that
may harbor macrocalcifications 3. However, the rarity
of these tumors makes it difficult to obtain an accurate
radiological diagnosis prior to surgery; hence a lot of PABs
are misdiagnosed preoperatively as carcinomas 4. Local
excision is of both diagnostic and therapeutic importance
since accurate diagnosis in many cases cannot be reached
through needle core biopsy 5. PABs are grossly well
demarcated, measuring on average 2 cm in maximum
dimension and express a histological picture similar to
pleomorphic adenoma of salivary gland consisting of cords
and glandular formations of epithelial cells embedded in
a chondro-myxoid stromal background 3. Most of the
reported cases of PAB have behaved in a benign fashion;
however, a few cases of malignant PAB were reported 5.
Local recurrence has been reported after surgery; however,
PABs show an overall favorable outcome 1. We report
2 cases of PABs in a 38-year-old male and a 79-year-old female patient. To the best of our knowledge, our first
patient is the sixth case of PAB occurring in the male breast
in the English literature. |
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Abstract
Introduction
Methods
Case Presentation
Disscussion
Conclusion
References
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A 38-year-old man presented to the emergency department
with a history of painful left breast swelling of 9 months
duration with no nipple discharge. The swelling size had
increased significantly during the last two days.
The physical examination revealed a hard swelling
involving the left breast area, extending up to the nipple
but not adherent to the skin or underlying chest wall.
MRI of the breast showed a large, bulging, and lobulated
mass measuring 9.5 x 6.5 x 6 cm, located in the left breast
with no associated skin edema/thickening. The mass
abutted and partly compressed the underlying pectoral
muscle without signs of muscle infiltration, and showed
rim enhancement with washout and necrosis in the
lateral aspect. The mass was given a score of BI-RADS 4
with rapidly growing phyllodes tumor cited as the main
differential diagnosis. A core biopsy from the mass revealed
mixed benign myoepithelial and glandular epithelial cells
surrounded by chondromyxoid stroma with prominent
cartilaginous differentiation. The cartilaginous areas
showed no features of atypia, and neither did the epithelial
parts, which ruled out a possible differential diagnosis of metaplastic carcinoma. By immunohistochemistry the
myoepithelial cells expressed Smooth Muscle Myosin, p63
and CK5/6; glandular epithelial cells expressed CKAE1/
AE3; stromal cells expressed GFAP; and cartilaginous
cells in stroma expressed S100. Estrogen Receptor (ER)
and Smooth Muscle Actin (SMA) were negative. The
subsequent excision specimen contained a lobulated mass
measuring 9 x 8 x 3 cm with a partially red to yellow and
partially tan-white calcified cut surface. Several areas
revealed bluish-white coloration indicating cartilaginous
differentiation (Figure 1). Microscopically this mass showed identical histology to the biopsy (Figures 2, 3); no
immunohistochemistry was performed on the excision
specimen and the diagnosis remained the same in both
specimens as pleomorphic adenoma of the breast.
Click Here to Zoom |
Figure 1: Case 1. A gross picture of pleomorphic adenoma of
the breast (PAB) showing the yellow and tan-white calcified cut
surface with prominent bluish white areas indicating cartilaginous
differentiation. |
Click Here to Zoom |
Figure 2: Case 1. Photomicrograph depicting glandular epithelial
component in a background of myxoid stroma with prominent
cartilaginous differentiation (Hematoxylin and Eosin stain, 100x). |
Click Here to Zoom |
Figure 3: Case 1. Photomicrograph depicting glandular epithelial
component in a background of myxoid stroma with prominent
cartilaginous differentiation (Hematoxylin and Eosin stain, 200x). |
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Abstract
Introduction
Methods
Case Presentation
Disscussion
Conclusion
References
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A 79-year-old woman presented to the breast screening
clinic for the annual mammography which showed a
small well-defined sub areolar nodule in the right breast.
An ultrasonography revealed a complex cystic lesion at
7 o’clock and sub areolar region, measuring 1 x 0.6 cm.
The lesion was well-defined and had intra cystic nodular
component with mild internal vascularity.
A vacuum-assisted excisional biopsy yielded multiple
fibroadipose fragments measuring 2.7 x 2.3 x 0.5 cm in
aggregate. Microscopic examination showed a part of
a circumscribed nodule harboring benign glands and
tubules composed of glandular epithelium along with
myoepithelial cells in a background of variably fibrotic
and chondromyxoid stroma (Figures 4-6), as well as an
intraductal papilloma component in the vicinity (Figure 7).
Although the presence of chondromyxoid stroma might
have suggested the differential diagnosis of metaplastic
carcinoma, the lack of atypia or even hypercellularity
in both epithelial and stromal components negated that
differential. Immunohistochemistry showed expression
of Smooth Muscle Myosin, p63, S-100, GFAP and CK
5/6 in myoepithelial cells while glandular epithelial cells
expressed CK 7 (Figure 8). A diagnosis of pleomorphic
adenoma of breast with associated intraductal papilloma was established. The patient had no further interventions.
During a follow up hospital visit more than a year later, the
patient had no breast-related complaints.
Click Here to Zoom |
Figure 4: Case 2. Photomicrograph showing PAB’s glandular
component in a chondromyxoid stroma (Hematoxylin and Eosin
stain, 100x). |
Click Here to Zoom |
Figure 5: Case 2. Photomicrograph showing PAB’s glandular
component in a chondromyxoid stroma (Hematoxylin and Eosin
stain, 200x). |
Click Here to Zoom |
Figure 6: Case 2. Photomicrograph depicting prominent myxoid
differentiation of the stroma (Hematoxylin and Eosin stain, 200x). |
Click Here to Zoom |
Figure 7: Case 2. Photomicrograph depicting PAB in the right
side of the picture with adjacent intraductal papilloma in the left
side (Hematoxylin and Eosin stain, 40x). |
Click Here to Zoom |
Figure 8: Case 2. Photomicrograph depicting immunohistochemical
staining of stromal cells for GFAP (Immunohistochemical
stain, 100x). |
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Top
Abstract
Introduction
Methods
Case Presentation
Disscussion
Conclusion
References
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Although Pleomorphic Adenoma (PA) is a relatively
common neoplasm in the salivary glands, its mammary
counterpart is extremely rare with less than 80 cases
reported since their first description in 1906 2,6. PAB
patients show a wide age range from third to eighth decades,
with the majority in the post-menopausal age (1), a feature
expressed by our second patient. Only five cases have been reported in the male breast in the literature 6-10 with one
tumor arising in an accessory nipple 11. In the published
literature so far, our first patient is the sixth case of PAB in
a male patient. Table I summarizes all the reported cases of
PABs in male patients to date including one tumor arising
in an accessory nipple.
Click Here to Zoom |
Table I: Reported cases of breast and nipple pleomorphic adenomas in male patients. |
PABs are among the so-called salivary gland-type
mammary tumors 1, a heterogeneous group of tumors
that are usually subclassified into epithelial, myoepithelial
and epithelial-myoepithelial morphologic subtypes
(i.e., biphasic); the last of which includes PABs among others 3. The majority manifest clinically as a palpated
retroareolar solitary lump 2. Diagnostic imaging
findings varied significantly among authors, with some
reporting fibroadenoma-like appearance that may harbor
macrocalcifications 3, while others reported features
ranging from completely calcified fibroadenomas to
those suggesting carcinomas and mucinous carcinomas
4. Our patients also manifested this variety, with the
first patient having a palpable mass expressing radiologic
features of phyllodes tumor; while the second patient had
asymptomatic lesion that was only described by radiology
as a complex cyst. The rarity of PABs in the literature has
made it extremely difficult to establish a diagnosis even
on core biopsies, with a lot of cases being misdiagnosed as
carcinoma 4. The differential diagnosis for PABs is a wide
list, with metaplastic carcinomas at the top of the list as the
most worrisome possibility. The latter, along with matrixproducing
carcinomas must all be excluded, a challenging task in core biopsies. While the presence of unequivocal
features of malignancy (like severe nuclear atypia, absence
of myoepithelial cells and abundant mitotic activity among
others) favors the diagnosis of carcinoma, the absence of
those features in core biopsies does not negate malignancy
elsewhere in the lesion as many cases were found to be
malignant only after excision and examining the entire
lesion 1,3.
This has made surgery the mainstay of diagnosis and
management, with local excision and subsequent
histological examination of the specimen serving as the best
way to reach an accurate diagnosis 5. Gross examination of
PAB usually yields a well-demarcated lesion that measures
around 2 cm in maximum dimension 3. Microscopically
PABs resemble salivary gland pleomorphic adenomas with
a cellular component composed of cords and glandular
formations of epithelial and myoepithelial cells embedded
in a variable chondro-myxoid stromal component 3. An
intraductal papilloma is often seen in the vicinity of PAB,
which led many authors to consider them a variant of
papilloma, while other authors associated PABs with other
benign neoplasms like ductal adenomas 1. This feature
was expressed in the PAB of our second patient, which
featured a microscopic intraductal papilloma component.
Immunohistochemically, PABs express SMA and S100 and
GFAP in the myoepithelial cells and EMA and cytokeratin
in the epithelial cells 5 while being negative for ER, PR and
Her-2 (triple-negative) 1. Salivary pleomorphic adenomas
express translocations in chromosomes affecting genes
PLAG1, HMGI-C and HMGIY. Immunohistochemical
expression of HMGI-C and HMGIY have been reported,
but more research studying the genetics of PABs is needed
2.
PABs express mainly a benign clinical course, even
with reports of cases recurring locally 3. Carcinoma ex
pleomorphic adenoma is the term describing malignant transformation of PAB, an extremely exceptional
complication that has only been reported three times
before 3. Because of this slim malignant transformation
potential, some authors proposed annual follow up
extending to five years in PAB cases. |
Top
Abstract
Introduction
Methods
Case Presentation
Discussion
Conclusion
References
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We reported two cases of PAB, one of which occurred
in a male patient. The diagnostic work up for these cases
took a significant effort clinically, radiologically and
pathologically. Both cases were managed with excision.
Although the first case had a wide surgical excision, the
second was managed through interventional radiology. The
rarity of this entity, as well as the potential for misdiagnosis
makes it imperative to keep this possibility in mind while
working up a breast mass lesion.
CONFLICT of INTEREST
The authors declare no conflict of interest.
AUTHORSHIP CONTRIBUTIONS
Concept: All authors contributed, Design: OE, MA,
KAS, Data collection or processing: OE, HAS, Analysis or
Interpretation: All authors contributed, Literature search:
OE, KAS, Writing: OE, Akhtar, Approval: MA. |
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Abstract
Introduction
Methods
Case Presentation
Discussion
Conclusion
References
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7) Makek M, Von Hochstetter AR. Pleomorphic adenoma of the
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8) Simha MR, Doctor VM, Udwadia TE. Mixed tumour of salivary
gland type of the male breast. Indian J Cancer. 1992;29:14-7.
9) Butrón M, y Cajal SR, Moreno A, García-Andrade С, Rivas С,
Oliva H. Pleomorphic adenoma of male breast: Report of a case
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10) Molland JG, Morgan GJ, Walker DM, Lin BP. Pleomorphic
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11) Scaranelo AM. Pleomorphic adenoma of a supernumerary male
nipple. Journal of Breast Imaging. 2019;1(4):356-7. |
Top
Abstract
Introduction
Methods
Case Presentation
Discussion
Conclusion
References
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Copyright © 2022 The Author(s). This is an open-access article published by the Federation of Turkish Pathology Societies under the terms of the Creative Commons Attribution License that permits unrestricted use, distribution, and reproduction in any medium or format, provided the original work is properly cited. No use, distribution, or reproduction is permitted that does not comply with these terms. |
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