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Mixed Epithelial and Mesenchymal Tumors, Pathology of the cervix 299
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show high-grade transformati on with large hyperchromatic and pleomorphic cells (Hodgson et al.
2017). Stromal overgrowth is defined as at least
25% of pure sarcomatous growth.
Immunophenotype
ER, PR, and CD10 positive, but might be negative
with sarcomatous overgrowth (Gallardo and Prat
2009). p53 can show abnormal staining in high-
grade adenosarcoma (Hodgson et al. 2017).
Molecular Features
Amplification of MDM2 and CDK4 is seen in
16–28% of cases (Bean et al. 2019; Howitt et al.
2015). MYBL1 amplification can rarely occur.
There can be alterations in PIK3CA/AKT/PTEN
pathway members. Mutations in ATRX and
DICER1 have been described (Bean et al. 2019).
Differential Diagnosis
The major differential diagnosis is with a benign
endocervical polyp. This lacks leaf-like architecture and periglandular stromal condensation.
Embryonal rhabdomyosarcoma usually occurs
in younger age demographic and lacks the epithelial component. A negative DICER1 mutation test
can help rule out embryonal rhabdomyosarcoma,
but the presence of the DICER1 mutation does not
help in distinguishing the two entities.
Endometrial stromal sarcoma lacks the epithelial component. Adenosarcoma may stain positive
with BCOR which can be a pitfall when highgrade endometrial stromal sarcoma is in the differential (Muthukumarana et al. 2020) (Figs. 2, 3,
and 4).
Mixed Epithelial and Mesenchymal Tumors, Pathology of the cervix, Fig. 2 Adenosarcoma of the cervix
forming a polypoid mass harboring glands with leaf-like
architecture
M
Mixed Epithelial and Mesenchymal Tumors, Pathology of the cervix, Fig. 3 Adenosarcoma of the cervix
showing periglandular stromal condensation
Carcinosarcoma of the Uterine Cervix
Synonyms
Malignant mesodermal mixed tumor; Malignant
mixed Müllerian tumor; Metaplastic carcinoma.
Definition
Biphasic malignant neoplasm composed of
mixed malignant epithelial and mesenchymal
components.
Mixed Epithelial and Mesenchymal Tumors, Pathology of the cervix, Fig. 4 Adenosarcoma of the cervix.
The stroma is hypercellular with moderate atypia and
scattered mitoses
300 Mixed Epithelial and Mesenchymal Tumors, Pathology of the cervix
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Clinical Features
• Incidence
Rare. Carcinosarcomas account for less than
5% of uterine malignancies. The majority
arise within the uterine corpus and a minority
from the cervix (Arend et al. 2011). Some
tumors are associated with high-risk human
papillomavirus (HPV) infection (Grayson
et al. 2001).
• Age
Mostly postmenopausal women. Mean age:
61 years (Grayson et al. 2001).
• Sex
Female.
• Site
Mixed Epithelial and Mesenchymal Tumors, Pathology of the cervix, Fig. 5 Carcinosarcoma of the uterine
cervix. The epithelial origin is a cervical mesonephric
carcinoma which formed spindled cells and heterologous
chondrosarcomatous differentiation
Cervix.
• Treatment
Radical hysterectomy, chemotherapy, and
radiotherapy (Comert et al. 2017).
differentiation such as rhabdomyosarcoma, osteosarcoma, or chondrosarcoma (Munakata et al.
2013; Kadota et al. 2009) (Fig. 5).
• Outcome
The 2-year disease-free survival and overall
survival are 49% and 60%, respectively
(Arend et al. 2011; Comert et al. 2017). Stage
alone is an independent prognostic factor for
recurrence and death.
Macroscopy
These are usually pedunculated tumors. The cut
Immunophenotype
Pankeratin and PAX8 are usually positive in carcinomatous component, but depend on the carcinoma origin. CD10, SMA, or desmin can be
positive in the sarcomatous component. Mesonephric carcinosarcomas can be GATA3 positive
(Roma 2014).
surface is soft yellow/white with hemorrhage and
necrosis.
Molecular Features
None.
Microscopy
Carcinosarcoma is generally considered a form of
transdifferentiation (epithelial to mesenchymal
transition) in a varie ty of tumors types as they
progress to high-grade tumor morphology. As
such, a variety of epithelial components have
been described. The epithelial (carcinomatous)
component can be composed of adenocarcinoma,
basaloid, adenoid basal, adenoid cystic, neuroendocrine (Munakata et al. 2013), and mesonephric
carcinoma (Clement et al. 1995). Carcinosar-
Differential Diagnosis
Spindle cell squamous cell carcinoma shows
gradual transition from conventional squamous
to spindle morphology. The spindle cell component is keratin positive and lacks heterologous
differentiation.
Adenosarcoma also has a malignant mesenchymal component, but it has a benign glandular
component.
comas of the cervix can rarely have endocervical
or endometrioid adenocarcinoma as an epithelial
component (Clement et al. 1998). The spindle
(sarcomatous) component is mostly homologous
“fibrosarcoma- or endometrial stromal sarcoma-
like.” It can also show heterologou s
References and Further Reading
Arend, R., Doneza, J. A., & Wright, J. D. (2011). Uterine
carcinosarcoma. Current Opinion in Oncology, 23(5),
531–536. PMID: 21743326.
Mixed Epithelial and Mesenchymal Tumors, Pathology of the cervix 301
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Bean, G. R., Anderson, J., Sangoi, A. R., Krings, G., &
Garg, K. (2019). DICER1 mutations are frequent in
Müllerian Adenosarcomas and are independent of
rhabdomyosarcomatous differentiation. Modern
Pathology: An Official Journal of the United States
and Canadian Academy of Pathology, Inc, 32(2),
280–289. PMID: 30266945.
Casey, S., & McCluggage,W. G. (2015). Adenomyomas of
the uterine cervix: Report of a cohort including endocervical and novel variants [corrected]. Histopathology,
66(3), 420–429. PMID: 25219586.
Clement,P.B.,Young,R.H.,Keh,P.,Ostör,A.G.,
& Scully, R. E. (1995). Malignant mesonephric
neoplasms of the uterine cervix. A report of
eight cases, including four with a malignant spindle cell component. The American Journal of
Surgical Pathology, 19(10), 1158– 1171. PMID:
7573674.
Clement, P. B., Zubovits, J. T., Young, R. H., & Scully,
R. E. (1998). Malignant mullerian mixed tumors of the
uterine cervix: A report of nine cases of a neoplasm
with morphology often different from its counterpart in
the corpus. International Journal of Gynecological
Pathology: Official Journal of the International Society
of Gynecological Pathologists, 17(3), 211–222. PMID:
9656116.
Gallardo, A., & Prat, J. (2009). Mullerian adenosarcoma:
A clinicopathologic and immunohistochemical study
of 55 cases challenging the existence of adenofibroma.
The American Journal of Surgical Pathology, 33(2),
278–288. PMID: 18941402.
Gilks, C. B., Young, R. H., Clement, P. B., Hart, W. R., &
Scully, R. E. (1996). Adenomyomas of the uterine
cervix of endocervical type: A report of ten cases of a
benign cervical tumor that may be confused with adenoma malignum [corrected]. Modern Pathology: An
Official Journal of the United States and Canadian
Academy of Pathology, Inc, 9(3), 220–224. PMID:
8685218.
Grayson, W., Taylor, L. F., & Cooper, K. (2001). Carci-
nosarcoma of the uterine cervix: A report of eight
cases with immunohistochemical analysis and evaluation of human papillomavirus status. The American
Journal of Surgical Pathology, 25(3), 338–347.
PMID: 11224604.
Hodgson, A., Amemiya, Y., Seth, A., Djordjevic, B., &
Parra-Herran, C. (2017). High-grade Müllerian
Adenosarcoma: Genomic and Clinicopathologic characterization of a distinct neoplasm with prevalent TP53
pathway alterations and aggressive behavior. The
American Journal of Surgical Pathology, 41(11),
1513–1522. PMID: 28834809.
Howitt, B. E., Sholl, L. M., Dal Cin, P., Jia, Y., Yuan, L.,
MacConaill, L., Lindeman, N., Kuo, F., Garcia, E.,
Nucci, M. R., & Quade, B. J. (2015). Targeted genomic
analysis of Müllerian adenosarcoma. The Journal of
Pathology, 235(1), 37–49. PMID: 25231023.
Jones, M. W., & Lefkowitz, M. (1995). Adenosarcoma of
the uterine cervix: A clinicopathological study of
12 cases. International Journal of Gynecological
Pathology: Official Journal of the International Society
of Gynecological Pathologists, 14(3), 223–229. PMID:
8600073.
Kadota, K., Haba, R., Ishikawa, M., Kushida, Y.,
Katsuki, N., Hayashi, T., Miyai, Y., Bando, K.,
Shiota, A., & Hata, T. (2009). Uterine cervical carci-
nosarcoma with heterologous mesenchymal compo-
nent: A case report and review of the literature.
Archives of Gynecology and Obstetrics, 280(5),
839–843. PMID: 19263060.
Kimyon Comert, G., Turkmen, O., Karalok, A., Basaran,
D., Bulbul, D., & Turan, T. (2017). Therapy modalities,
prognostic factors, and outcome of the primary cervical
carcinosarcoma: Meta-analysis of extremely rare tumor
of cervix. International Journal of Gynecological Can-
cer: Official Journal of the International Gynecologi-
cal Cancer Society, 27(9), 1957–1969. PMID:
28708788.
Mikami, Y., Maehata, K., Fujiwara, K., & Manabe,
T. (2001). Endocervical adenomyoma. A case report
with histochemical and immunohistochemical studies.
APMIS: Acta pathologica, microbiologica, et
immunologica Scandinavica, 109(7–8), 546–550.
PMID: 11552953.
Mohammadizadeh, F., Rajabi, P., Behnamfar, F., Hani,
M., & Bagheri, M. (2016). Extensive overgrowth of
sex cord-like differentiation in uterine Mullerian
Adenosarcoma: A rare and challenging entity.
International Journal of Gynecological Pathology:
Official Journal of the International Society of Gyne-
cological Pathologists, 35(2), 153–161. PMID:
26840033.
Munakata, S., Iwai, E., Tanaka, T., Nakamura, M., &
Kanda, T. (2013). Malignant müllerian mixed tumor
of the uterine cervix with a small cell neuroendocrine
carcinoma component. Case Reports in Pathology,
2013, 630859. PMID: 23533892.
Muthukumarana, V., Fix, D. J., Stolnicu, S., Park, K. J.,
Soslow, R. A., Benayed, R., Ladanyi, M., Antonescu,
C. R., & Chiang, S. (2020). BCOR expression in
Mullerian adenosarcoma: A potential diagnostic pitfall.
The American Journal of Surgical Pathology, 44(6),
765–770. PMID: 32011345.
Roma, A. A. (2014). Mesonephric carcinosarcoma involv-
ing uterine cervix and vagina: Report of 2 cases with
immunohistochemical positivity for PAX2, PAX8, and
GATA-3. International Journal of Gynecological
Pathology: Official Journal of the International Society
of Gynecological Pathologists, 33(6), 624–629. PMID:
25272303.
Seagle, B. L., Kanis, M., Strohl, A. E., & Shahabi, S. (2016).
Survival of women with Mullerian adenosarcoma:
ANationalCancerDataBasestudy.Gynecologic Oncol-
ogy, 143(3), 636–641. PMID: 27771166.
Yuan, Z., Shen, K., Yang, J., Cao, D., Zhang, Y., Zhou, H.,
Wu, H., & Yu, M. (2019). Uterine adenosarcoma:
A retrospective 12-year single-center study. Frontiers
in Oncology, 9, 237. PMID: 31139558.
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302 Mixed Epithelial and Mesenchymal Tumors, Pathology of the Ovary
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survival is comparable but likely somewhat
Mixed Epithelial and
Mesenchymal Tumors,
Pathology of the Ovary
Ghassan Allo
Department of Pathology and Laboratory
Medicine, Henry Ford Health, Detroit, MI,
USA
Ovarian Adenosarcoma
Synonyms
Müllerian adenosarcoma of ovary.
Definition
Ovarian adenosarcomas are biphasic tumors composed of benign to atypical epithelium and a lowgrade malignant stromal component.
Clinical Features
• Incidence
These tumors are rare, estimate to comprise
0.04% of ovarian cancers (Seagle et al. 2016).
• Age
Ovarian adenosarcomas have been diagnosed
in premenopausal and postmenopausal women
of a wide age range of 30–84 years, with a
reported mean age of diagnosis of these tumors
is 65.5 +/ 13 years, comparable to the more
common uterine counterpart (Eichhorn et al.
2002; Seagle et al. 2016).
• Clinical presentation
Patients with ovarian adenosarcomas usually
present with nonspecific symptoms similar to
those of other ovarian tumors. These include
abdominal swelling, abdominal pain, and
bowel obstruction, or as torsion.
• Treatment
Early and complete surgical resection is the
mainstay in treatment. There is no definitive
role for adjuvant therapy.
• Outcome
Patients with ovarian adenosarcomas have
shown to have a long survival after complete
surgical resection of nonmetastatic tumor with
a median survival of 130 months. This lengthy
lower than that of the more common uterine
counterparts (Eichhorn et al. 2002; Seagle et al.
2016). Higher patient age and positive resec-
tion margins are associated with worse overall
survival (Seagle et al. 2016).
Macroscopy
Adenosarcomas are usually unilateral solid to
focally cystic large tumors, ranging from 6 to
16 cm (average, 10 cm). Solid flesh-like areas
may denote areas of stromal overgrowth. Gross
hemorrhage and necrosis may be present.
Microscopy
Müllerian adenosarcomas of the ovary resemble
their uterine counterparts; they are composed of
polypoid and papillary proliferations of epithelial
and stromal cells. The epithelial cells show serous,
endometrioid, mucinous, or squamous differentiation, lining the surface of the polypoid proliferations and forming glandular structures dispersed
regularly within the tumor. They are generally
benign but can show focal atypia (Fig. 1a). The
stromal component is composed of low-grade sarcoma cells that protrude into the polypoid structures and show subepithelial condensation
(“cambium” layer) forming cleft-like spaces and
phyllodes-like growth pattern (Fig. 1a–b). The sarcoma exhibits low-grade endometrial stromal sarcoma phenotype but can show fibromatous,
smooth muscle, or less likely myxoid differentiation. Sex cord differentiation may be seen but has
no apparent significance (Stolnicu et al. 2016).
Sarcomatous overgrowth, defined as sarcoma com-
prising 25% or more of the tumor, usually resembles low-grade endometrial sarcomas, but can
show features of high-grade endometrial stromal
sarcoma with high-grade cytologic atypia,
increased mitotic activity, and necrosis, or contain
heterologous elements in the form of rhabdomyosarcoma or osteosarcoma (Clement 1989). At time
of initial resection and diagnosis, distant and lymph
node metastases are rare.
Immunophenotype
Immunohistochemistry is not specific and reflect
the differentiation of various components; the
Mixed Epithelial and Mesenchymal Tumors, Pathology of the Ovary 303
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Mixed Epithelial and Mesenchymal Tumors, Pathology of the Ovary, Fig. 1 (a) Ovarian adenosarcoma
showing stromal and epithelial proliferation showing
sarcomatous component may express CD10,
estrogen receptor, and progesterone receptor
when there is endometrial stromal differentiation,
and smooth muscle markers, such as desmin and
caldesmon, when it shows smooth muscle differentiation. High-grade sarcoma may lose markers
of differentiation. Rhabdomyosarcomatous areas
can express myogenin and MYOD1, and sex cord
stromal areas may express inhibin and calretinin.
Molecular Features
There are no known genetic changes in ovarian
adenosarcomas.
Differential Diagnosis
Ovarian adenosarcomas must be differentiated
from the more common adenofibromas, ovarian
fibromas, carcinosarcomas, and other sarcomas.
Both adenofibromas and adenosarcomas
display biphasic epithelial and stromal components, and may arise from endometriosis. However, adenofibromas are less cellular than
adenosarcomas, displaying more stromal fibrosis
but lacking subepithelial stromal condensation,
stroma atypia, and significant mitotic activity.
Ovarian fibromas form a circumscribed mass
that is composed of bland spindle cells with
admixed collagen bundles in variably fibrotic
stroma, lacking the diffusely dispersed epithelial
elements.
Adenosarcomas with a prominent sarcomatous
component or heterologous elements, especi ally
those with high-grade atypia, should be examined
carefully for malignant epithelial component to
focal atypia (b) intraluminal polypoid projections with
subepithelial stromal condensation
rule out the possibility of a carcinosarcoma,
whether primary ovarian or metastatic. When the
epithelial component is minimal to absent, other
sarcomatous neoplasms should be ruled out, such
as endometrioid stromal sarcoma, metastatic
endometrial stromal sarcoma, sex cord stromal
tumors with sarcomatous differentiation, and
other metastatic sarcomas.
Both endometrioid stromal sarcoma and metastatic endometrial stromal sarcoma display minimal if any glandular component, but show the
typical morphology of tumor that resembles proliferative type endometrium with arteriolar proliferation. These tumors, especially the metastatic
sarcoma, may show tongue-like pattern of invasion which is not a feature of ovarian
adenosarcomas. In challenging cases, the tumors
may be tested for gene rearrangements seen in
endometrial stromal sarcomas, commonly involving JAZF1-JJAZ1, JAZF1-PHF1, EPC1-PHF1,
or YWHAE-NUTM2B gene fusion.
Adenosarcomas with sarcomatous overgrowth
should also be differentiated from sex cord stromal tumors with sarcomatous features, e.g., adult
granulosa cell tumors with sarcomatous differentiation or poorly differentiated Sertoli-Leydig cell
tumors. These tumors lack at least focal typical
polypoid growth pattern of adenosarcoma with
epithelial lining, but contain at least focal classical
sex-cord component. Care should be taken not to
confuse the latter with focal sex cord differentiation in adenosarcoma or other sarcomas. Ancillary
testing in the form of immunohistochemistry for
sex cord stromal markers of differentiation
M
304 Mixed Epithelial and Mesenchymal Tumors, Pathology of the Uterine Corpus
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(steroid factor 1, inhibin, and calretinin) may be of
benefit to highlight focal well-differentiated areas.
Testing for specific mutations in FOXL2 or
DICER1 genes may be performed to investigate
the possibility of adult granulosa cell tumor or
Sertoli-Leydig cell tumor, respectively.
References and Further Reading
Clement, P. B. (1989). Mullerian adenosarcomas of the uterus
with sarcomatous overgrowth. A clinicopathological
analysis of 10 cases. The American Journal of Surgical
Pathology, 13(1), 28–38. https://doi.org/10.1097/
00000478-198901000-00004.
Eichhorn, J. H., Young, R. H., Clement, P. B., & Scully,
R. E. (2002). Mesodermal (mullerian) adenosarcoma of
the ovary: A clinicopathologic analysis of 40 cases and a
review of the literature. The American Journal of Surgi-
cal Pathology, 26(10), 1243–1258. https://doi.org/10.
1097/00000478-200210000-00001.
Seagle,B.L.,Kanis,M.,Strohl, A. E., & Shahabi, S. (2016).
Survival of women with Mullerian adenosarcoma:
A national cancer data base study. Gynecologic Oncology,
143(3), 636–641. https://doi.org/10.1016/j.ygyno.2016.
10.013.
Stolnicu, S., Molnar , C., Barsan, I., Boros, M., Nogales, F. F.,
& Soslow, R. A. (2016). The impact on survival of an
extensive sex cord-like component in mullerian adenosarcomas: A study comprising 6 cases. International
Journal of Gynecological Pathology, 35(2), 147–152.
https://doi.org/10.1097/PGP.0000000000000231.
Mixed Epithelial and
Mesenchymal Tumors,
Pathology of the Uterine
Corpus
Eman Abdulfatah
University of Michigan, Ann Arbor, MI, USA
Adenomyoma
Synonyms
Endometrioid-type adenomyoma.
Definition
Benign mixed mullerian tumor composed of
endometrial glands and endometrial-type stroma
surrounded by smooth muscle.
Clinical Features
• Incidence
Accounts for approximately 2% of endometrial
polyps.
• Age and epidemiology
Predominantly affects premenopausal women.
• Site
Uterine corpus more than cervix.
Predominantly intramyometrial (can be sub-
mucosal, intramural, or subserosal).
• Treatment
Excision or hysterectomy (depending on the
location and fertility status).
• Outcome
Excellent.
Macroscopy
• Well-circumscribed.
• Polypoid masses if endometrial or cervical.
• Firm, whorled, gray-white cut surface.
• Variable number of cysts may be seen.
Microscopy
• Endometrial glands that may be cystically
dilated, lined by endometrioid- type epithe-
lium, surrounded by endometrial stroma and
fascicles of smooth muscle (which is the pre-
dominant component) (Fig. 1).
• Squamous, tubal, and mucinous metaplasia
may be seen within the glands.
• Smooth muscle component may show a range
of changes seen in leiomyomas, including var-
iable cellularity, thick blood vessels, and cleft-
like spaces, bizarre nuclei.
Immunophenotype
• Glandular component: AE1/AE3, ER, and PR
positive.
• Stromal component: CD10 positive.
• Smooth muscle component: positive for
smooth muscle markers (smooth muscle
actin, desmin, and caldesmon).
Differential Diagnosis
• Leiomyoma with entrapped glands
– Glands typically entrapped at the periphery.
• Atypical polypoid adenomyoma
– Glands with squamous metaplasia.
– No endometrial stroma.
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• Age and epidemiology
Mean age: 40 years.
May occur in women with Turner syndrome.
• Site
Centered in the lower uterine segment.
• Treatment
Excision or hysterectomy (depending on the
fertility status).
• Outcome
Excellent.
Recurrence rate ranges from 10% to 45%
depending on the treatment modality;
seen if treated with curettage or local
excision.
Progression to or association with atypical endo-
metrial hyperplasia or endometrioid adenocarci-
noma within the lesion and in the adjacent
endometrium has been described.
Macroscopy
• Solitary, well circumscribed, polypoid mass.
Mixed Epithelial and Mesenchymal Tumors, Pathology of the Uterine Corpus, Fig. 1 (a and b)
Adenomyoma: endometrioid-type glands that are
cystically dilated, surrounded by fascicles of smooth muscle. In (b), endometrial stroma is seen surrounding the
glands in addition to the smooth muscle layer which
shows hydropic changes and hyalinization
• Rubbery consistency.
Microscopy
• Glandular component shows architectural
complexity and cytological atypia (Fig. 2).
• Prominent squamous metaplasia in the form of
squamous morules that may show central
necrosis is often present.
• Mullerian adenosarcoma
– Malignant stromal component.
– Cystically dilated glands with periglandular
• Glands are surrounded by fascicles of bland
smooth muscle and fibrous stroma.
• No endometrial type stroma.
stromal condensation.
Immunophenotype
• Glandular component: AE1/AE3, ER, and PR
Atypical Polypoid Adenomyoma
positive.
• Stromal component: positive for smooth mus-
Synonym
Atypical polypoid adenomyofibroma.
cle markers (smooth muscle actin, desmin, and
caldesmon).
M
Definition
Benign mixed mullerian tumor composed of
endometrial glands with architectural complexity,
set in fibromuscular stroma, typically with squamous metaplasia.
Clinical Features
• Incidence
Uncommon.
Molecular Features
• MLH1 hypermethylation (40% of tumors) and
microsatellite instability.
Differential Diagnosis
• Adenomyoma
– Less abundant glandular component, less
architectural complexity.
– Variable amounts of endometrial stroma.
306 Mixed Epithelial and Mesenchymal Tumors, Pathology of the Uterine Corpus
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Mixed Epithelial and Mesenchymal Tumors, Pathology of the Uterine Corpus, Fig. 2 (a and b) Atypical
polypoid adenomyoma. Benign endometrial glands
surrounded by fascicles of smooth muscle and fibrous
stroma. (c) Glandular component showing architectural
complexity. (d and e) Prominent squamous metaplasia in
the form of squamous morules; central necrosis is seen
in (d)
Mixed Epithelial and Mesenchymal Tumors, Pathology of the Uterine Corpus 307
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• Mullerian adenosarcoma
– Malignant stromal component.
– Cystically dilated glands with periglandular
stromal condensation.
Adenofibroma
Synonyms
Mullerian adenofibroma; Papillary adenofibroma.
Definition
Benign biphasic tumor composed of benign
mullerian epithelium and endometrial stroma.
Clinical Features
• Incidence
– Rare.
• Age and epidemiology
– Most commonly occurs in perimenopausal
or postmenopausal women.
• Site
– Mostly endometrial.
– Rarely endocervical, ovarian, and pelvic
ligaments.
• Treatment
– Hysterectomy.
• Outcome
– Excellent.
Macroscopy
• Polypoid masses within the endometrial cavity.
• Usually solid but cystic component may be
present.
Mixed Epithelial and Mesenchymal Tumors, Pathology of the Uterine Corpus, Fig. 3 Adenofibroma. (a)
Benign endometrial glands surrounded by benign endometrial/fibroblastic stroma. (b) Benign endometrial-type epithelium covering broad papillary fronds
Differential Diagnosis
• Mullerian adenosarcoma
– Malignant stromal component.
– Cystically dilated glands with periglandular
stromal condensation.
Mullerian Adenosarcoma
M
Microscopy
• Benign endometrial-type epithelium covering
broad papillary fronds of stroma (Fig. 3).
• Mesenchymal component composed of benign
endometrial stroma or fibroblastic stroma.
• Cleft-like formation that is typically seen in
adenosarcoma, may be seen in adenofibroma.
• No periglandular stromal condensation, atypia,
or increased mitotic activity.
Synonyms
Adenosarcoma.
Definition
• Biphasic mullerian neoplasm composed of
benign mullerian glands and low-grade malignant stroma.
• When at least 25% of the tumor contains a
high-grade sarcomatous component, it is
308 Mixed Epithelial and Mesenchymal Tumors, Pathology of the Uterine Corpus
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classified as an “adenosarcoma with sarcomatous overgrowth.”
Clinical Features
• Incidence
Uncommon.
• Age and epidemiology
The majority occur in postmenopausal women;
however, 30% occur in premenopausal women.
• Site
Uterine corpus.
• Treatment
Total abdominal hysterectomy and bilateral
salpingo-oophorectomy.
Adjuvant chemotherapy or radiotherapy if sarcomatous overgrowth is present.
• Outcome
Good prognosis if confined to the endometrium (stage 1A).
Poor prognostic factors: myometrial invasion
and sarcomatous overgrowth.
Risk of recurrence increases from 15% in
non-myoinvasive to 45% in myoinvasive
tumors.
Mixed Epithelial and Mesenchymal Tumors, Pathology of the Uterine Corpus, Fig. 4 Mullerian adeno-
sarcoma. (a) Benign endometrial glands with irregular
architecture. (b) Cellular stroma with atypia and increased
mitotic activity
Macroscopy
• Polypoid mass filling the endometrial cavity
and may protrude through the cervical os.
• Solid/cystic gray-w hite cut surfaces.
• Fleshy with areas of hemorrhage and necrosis
if sarcomatous overgrowth present.
Microscopy
• Irregular glandular architecture and intraglandular polypoid projections (Figs. 4 and 5).
• Glandular component epithelium may vary
from endometrioid to mucinous, squamous,
or tubal metaplasia. Occasionally, glandular
atypia is present.
• Stroma is typically cellular, with periglandular
stromal cuffing.
• The stromal c omponent may exhibit a wide
range of differentiation, including homologous
and heterologous differentiation, and some
cases show sex cord-like components.
Immunophenotype
• Glandular component: AE1/AE3, ER, and PR
positive.
• Stromal component: ER, PR, CD10, WT1 positive
with variable expression of smooth muscle markers.
Molecular Features
• TP53 mutations are prevalent in high-grade
adenosarcoma.
Differential Diagnosis
• Adenofibroma
– No periglandular cuffing, stromal atypia, or
mitotic activity.
• Adenomyoma
– Well-circumscribed.
– Benign endometrioid glands and smooth
muscle stroma.
– Variable amounts of endometrial stroma
around the glands without cytologic atypia
or periglandular condensation.
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