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Mixed Epithelial and Mesenchymal Tumors, Pathology of the Uterine Corpus 309
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Mixed Epithelial and Mesenchymal Tumors, Pathology of the Uterine Corpus, Fig. 5 Mullerian
adenosarcoma. (a) Intraglandular polypoid and finger-like
projections. Stroma demonstrating periglandular condensation or cuffing. (b) Adenosarcoma with areas of sarcomatous overgrowth
• Atypical polypoid adenomyoma
– Glands have squamous metaplasia, and
stroma is fibromyomatous.
• Carcinosarcoma (malignant mixed mullerian
tumor)
– High-grade malignant epithelial and stro-
mal components.
Uterine Carcinosarcoma
Synonyms
Malignant mixed mullerian tumor (MMMT).
Definition
• Biphasic mullerian neoplasm composed of
high-grade malignant glandular/epithelial and
stromal components.
Mixed Epithelial and Mesenchymal Tumors, Pathology of the Uterine Corpus, Fig. 6 Uterine carcinosar-
coma. (a) Carcinosarcoma comprised of high-grade
malignant epithelial and mesenchymal components.
(b) p53 immunostaining showing aberrant expression in
the stromal component
Clinical Features
• Incidence
Rare, comprises 2–5% of all uterine tumors.
• Age and epidemiology
Mostly in postmenopausal women, but age
range 30–90 years.
• Site
Uterine corpus.
• Treatment
Total abdominal hysterectomy and bilateral
salpingo-oophorectomy with staging.
Adjuvant chemotherapy and/or radiotherapy.
• Outcome
Poor outcomes, with a 5-year overall survival
rate of <35%.
Stage, tumor size, sarcoma predominance are
important prognostic factors.
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310 Mixed Epithelial and Mesenchymal Tumors, Pathology of the Uterine Corpus
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Macroscopy
• Polypoid mass filling the endometrial cavity.
• Fleshy with areas of hemorrhage and
necrosis.
Microscopy
• High-grade epithelial (carcinomatous) and
stromal (sarcomatous) components that are
either juxtaposed or admixed (Figs. 6, 7,
8 and 9).
• Carcinomatous component: endometrioid,
serous, clear cell carcinoma or mixed.
• Mostly high-grade but occasionally low-grade
endometrioid may be seen.
• Sarcomatous component may be homologous
(endometrial stromal sarcom a, leiomyosarcoma, and fibrosarcoma) or heterologous
(rhabdomyosarcoma – most common, liposarcoma, chondrosarcoma, and osteosarcoma).
• Carcinomatous component is the predominant histology seen within lymphovascular
spaces.
Immunophenotype
• Carcinomatous component: AE1/AE3, ER
and PR positive, vimentin positive if endometrioid, negative if serous which is positive
for p53.
• Sarcomatous component: vimentin, desmin,
CD10, CD34, and myogenic markers may be
positive.
• p16 and p53 are freque ntly expressed in both
components.
Molecular Features
• TP53 mutations.
Mixed Epithelial and Mesenchymal Tumors, Pathology of the Uterine Corpus, Fig. 7 Uterine carcinosar-
coma. Carcinomatous and sarcomatous components are
either juxtaposed (in a) or intermingled (admixed) in (b)
Mixed Epithelial and Mesenchymal Tumors, Pathology of the Uterine Corpus, Fig. 8 Uterine carcinosar-
coma. Carcinomatous component is high-grade serous
carcinoma (part a) or endometrioid carcinoma with squamous differentiation (part b). Heterologous sarcomatous
component
Mixed Epithelial and Mesenchymal Tumors, Pathology of the Uterine Corpus 311
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References and Further Reading
Abdulfatah, E., et al. (2019). Predictive histologic factors
in carcinosarcomas of the uterus: A multi-institutional
study. International Journal of Gynecological Pathol-
ogy, 38, 205–215.
Bettaieb, I., et al. (2007). Endometrial adenofibroma:
A rare entity. Archives of Gynecology and Obstetrics,
275, 191–193.
Brooks, S., et al. (2004). Surveillance, epidemiology, and
end results analysis of 2677 cases of uterine sarcoma
1989-1999. Gynecologic Oncology, 93, 204–208.
Cherniak, A., et al. (2017). Integrated molecular character-
ization of uterine Carcinosarcoma. Cancer Cell, 31,
411–423.
Gallardo, A., et al. (2009). Mullerian adenosarcoma:
A clinicopathologic and immunohistochemical study of
55 cases challenging the existence of adenofibroma.
American Journal of Surgical Pathology, 33(2), 278–288.
Gilks, C. B., et al. (2000). Uterine adenomyomas exclud-
ing atypical polypoid adenomyomas and
adenomyomas of endocervical type: A clinicopathologic study of 30 cases of an underemphasized lesion
that may cause diagnostic problems with brief consideration of adenomyomas of other female genital tract
sites. International Journal of Gynecological Pathol-
Mixed Epithelial and Mesenchymal Tumors, Pathology of the Uterine Corpus, Fig. 9 Uterine carcinosar-
coma. (a and b) Heterologous elements in the form of
rhabdomyosarcoma which is the most common sarcomatous element
• Similar molecular genetic alterations in carcinomatous and sarcomatous elements.
• Microsatellite inst ability (MSI-high).
Differential Diagnosis
• Mullerian adenosarcoma with sarcomatous
overgrowth
– Glandular component lacks the high-grade
features.
– Periglandular condensation or cuffing.
• Endometrioid carcinoma with heterologous
elements
– Low-grade endometrioid carcinoma with
benign heterologous elements, mostly fat
or cartilage and rarely bone.
• Dedifferentiated endometrioid carcinoma
– Well-differentiated endometrioid carci-
noma with juxtaposed sheets of
discohesive, round, and undifferentiated
carcinoma.
ogy, 19, 195–205.
Hogson, A., et al. (2017). High-grade Müllerian
Adenosarcoma: Genomic and clinicopathologic characterization of a distinct neoplasm with prevalent TP53
pathway alterations and aggressive behavior. The Amer-
ican Journal of Surgical Pathology, 41,1513–1522.
Horita, A., et al. (2010). Coexistent atypical polypoid
adenomyoma and complex atypical endometrial hyperplasia in the uterus. Diagnostic Cytopathology, 38,
527–532.
Kurnit, K., et al. (2019). Prognostic factors impacting
survival in early stage uterine carcinosarcoma. Gyne-
cologic Oncology, 152,31–37.
Longacre, T. A., et al. (1996). Atypical polypoid
adenomyofibromas (atypical polypoid adenomyomas)
of the uterus. A clinicopathologic study of 55 cases.
The American Journal of Surgical Pathology, 20,1–20.
McCluggage, W., et al. (2016). A practical approach to the
diagnosis of mixed epithelial and mesenchymal tumours
of the uterus. Modern Pathology, 29,S78–S91.
Nemejcova, K., et al. (2015). Atypical polypoid
adenomyoma of the uterus: An immunohistochemical
and molecular study of 21 cases. The American Journal
of Surgical Pathology, 39, 1148–1155.
Ohta, Y., et al. (2005). A case of uterine adenomyoma with
bizarre smooth muscle cells mimicking
leiomyosarcoma. Diagnostic Cytopathology, 32,
288–291.
Powell, M., et al. (2010). Phase II evaluation of paclitaxel
and carboplatin in the treatment of carcinosarcoma of
the uterus: A gynecologic oncology group study. Jour-
nal of Clinical Oncology, 28, 2727–2731.
M
312 Mixed Epithelial and Mesenchymal Tumors, Pathology of the Vagina
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Tahlan, A., et al. (2006). Uterine adenomyoma:
A clinicopathologic review of 26 cases and a review
of the literature. International Journal of Gynecologi-
cal Pathology, 25, 361–365.
Vellios, F., et al. (1973). Papillary adenofibroma of the
uterus: A benign mesodermal mixed tumor of
Müllerian orgin. American Journal of Clinical Pathol-
ogy, 60, 543–551.
Mixed Epithelial and
Mesenchymal Tumors,
Pathology of the Vagina
Raji Ganesan
Birmingham Women’s and Children’s NHS Trust,
Birmingham, UK
Adenosarcoma
Synonyms
Mullerian adenosarcoma.
Definition
This is a biphasic neoplasm composed of benign
epithelial elements and malignant stromal
components.
Clinical Features
Common symptoms at presentation are vaginal
mass, vaginal bleeding, and increased frequency
of urination.
• Outcome
This is a very rare tumor and most reports have
recorded recurrences.
Macroscopy
This is a polypoid growth and may have a cystic
cut surface.
Microscopy
Appearances are similar to uterine and cervical
adenosarcomas. The epithelial element is composed of glands that can be cystically dilated or
show a phyllodes morphology. The lining cells
can show any Mullerian phenotype. There is subepithelial and periglandular cuffing by cells that
are similar to endometrial stroma.
Immunophenotype
The stromal component is usually positive for
CD10, ER, and PR.
Differential Diagnosis
The main differential diagnoses are embryonal
rhabdomyosarcoma and carcinosarcoma. Carcinosarcomas have a malignant epithelial
component. In the differential with embryonal
rhabdomyosarcoma, adenosarcomas with rhabdomyoblastic differentiation show low-grade features in the sarcomatous element including the
rhabdomyoblasts. The latter are scattered singly
or in groups.
• Incidence
This is a very rare neoplasm when it presents as
a primary in the vagina (Mandato et al. 2018;
Wang et al. 2015).
• Age
It occurs over a wide age range (Wang et al.
2015). Vaginal adenosarcoma associated with
endometriosis has been recorded in perimenopausal women (Judson et al. 2000).
• Site
No particular site.
• Treatment
Surgery is the main treatment for primary vaginal adenosarcoma.
References and Further Reading
Judson, P. L., Temple, A. M., Fowler, W. C., Jr., Novotny,
D. B., & Funkhouser, W. K., Jr. (2000). Vaginal
adenosarcoma arising from endometriosis. Gyneco-
logic Oncology, 76(1), 123–125.
Mandato, V. D., Torricelli, F., Mastrofilippo, V., Valli, R.,
Aguzzoli, L., & La Sala, G. B. (2018). Primary extrauterine and extra-ovarian mullerian adenosarcoma:
Case report and literature review. BMC Cancer,
18(1), 134.
Wang, Y., Huang, Y. W., & Li, Y. F. (2015). Primary
vaginal sarcoma: Experience of a regional cancer center
in China. Journal of Obstetrics and Gynaecology
Research, 41(9), 1463–1468.
Mixed Germ Cell - Sex Cord-Stromal Tumors, Pathology of the Ovary 313
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these mitotic figures a re present in the germ
Mixed Germ Cell - Sex
Cord-Stromal Tumors,
Pathology of the Ovary
Oudai Hassan
Henry Ford Health System, Detroit,
MI, USA
cell component or in the sex cord stro mal
component (Zuntova et al. 199 2). Favorable
prognostic fea tures include freely mobile
tumors, encapsulation, completely excised
tumors with negative surgical margins and
the lack of metastasis (Talerman 1972a).
Macroscopy
Definition
This is a group of tumors that were described by
Tal e r man (1972a). The first case in the medical
literature with a description similar to the morphologic feature of a mixed g erm cell and sex
cord stromal tumor was reported back in 1923 by
Massonetal.andhecalledthattumor“epithelioma pflugerien” (Masson 1923). They have both
germ cell tumor component and sex cord stromal
tumor component intimately intermixed with
each other.
Clinical Features
• Incidence
Rare.
• Age
The majority of the cases reported in the
medical literature occurred in young girls in
the first decade of life. These tumors, in contrary to gonadoblastoma, tend to have normal
karyotype and usually are not associated with
sexual development abnorm alities (Arroyo
et al. 1998).
• Sex
Female.
• Site
Ovary.
• Treatment
Surgical treatment is indicated with or without
oncologic treatment.
• Outcome
One feature that has been associated with
malignancy in mixed germ cell and stromal
tumors is a mitotic count over 10 mitotic
figures per high power field (HPF) whether
Macroscopically, these tumors tend to be fleshy,
solid, gray-white, and in some cases, they might
show cystic change. In general, these tumors lack
necrosis or calcifications (Talerman 1972b, 1980;
Zuntova et al. 1992).
Microscopy
Microscopically, these tumors may show different
growth patterns, whether cord-like, tubular and
nested, sheet like, etc. The sex cord stromal cells
usually have scant cytoplasm, angulated and
grooved nuclei. In some cases, the sex cord stromal component may present as a spindle cell
component similar to what is seen in an ovarian
fibroma or fibroma thecoma. The germ cell component may show dysgerminoma like cells with
clear cytoplasm and squared off nuclei, any type
of germ cells may be present. These tumors are
composed of a mixture of germ cells and sex cord
stromal cells that are intermixed intimately with a
varying degree of cellular components. Some of
these tumors may show a predominant component
of sex cord stromal elements with minimal germ
cell component, some of the tumors may on the
other way be predominantly germ cells with minimal sex cord stromal cell component, while in
some cases, we might see a balanced distribution
between germ cells and sex cord stromal
components.
Arroyo et al. reported a case of mixed germ
cells and sex cord stromal tumors where the sex
cord stromal elements showed annular tubules
growth pattern. This tumor they reported showed
metastasis into the abdominal cavity (Arroyo
et al. 1998).
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314 Mixed Sex Cord-Stromal Tumors, Pathology of the Ovary
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In another case, Zuntova et al. reported a case
of mixed germ cell and stromal tumor with heterologous epithelium as their tumor showed glands
and cysts lined by mucinous columnar cells with
Mixed Sex Cord-Stromal
Tumors, Pathology of the
Ovary
goblet cells and argyrophilic cells (Zuntova et al.
1992). The question we would raise about this
case is that could these areas represent a component of Yolk sac tumor. This case was reported in
Neeraja Yerrapotu and Sudeshna Bandyopadhyay
Detroit Medical Center, Wayne State University,
Detroit, MI, USA
1992, and in the case report, the authors mentioned that they performed immunostains only
for vim entin and keratin as immunostains for
Synonyms
Alpha-fetoprotein, SALL4, and glypican were
not available back then.
Androblastoma; Arrhenoblastoma.
Although these tumors have similar components as seen in gonadoblastoma, they lack
round eosinophilic hyaline globules and calcific
Definition
concretions seen in gonadoblastoma. They appear
to be proliferative in contrary to gonadoblastoma,
and these patients also tend to have normal karyotype in contrary to patients with
gonadoblastoma.
This is a rare group of tumors including neoplastic
proliferation of mixed Sertoli (sex cord cell),
Leydig cells (stromal cell), primitive gonadal
stroma, and heterologous elements based on
degree of differentiation. Most of the patients
present with hormone-related symptoms; some
present with abdominal mass and fullness.
References and Further Reading
40–60% of cases present with virilization symptoms (Gui et al. 2012).
Arroyo, J. G., Harris, W., & Laden, S. A. (1998). Recurrent
mixed germ cell-sex cord-stromal tumor of the ovary in
an adult. International Journal of Gynecological
Pathology, 17(3), 281–283. https://doi.org/10.1097/
00004347-199807000-00015.
Masson, P. (1923). Epitheliomas pfl~geriens. In Diagnos-
tics de Laboratoire. Le tumenrs (pp. 477–478). Paris:
Maloine kdi-tions.
Talerman, A. (1972a). A distinctive gonadal neoplasm
related to gonadoblastoma. Cancer, 30(5), 1219–1224.
https://doi.org/10.1002/1097-014 2(197211)30:
5<1219::aid-cncr2820300512>3.0.co;2-u.
Talerman, A. (1972b). A mixed germ cell-sex cord stroma
tumor of the ovary in a normal female infant. Obstetrics
and Gynecology, 40(4), 473–478. https://www.ncbi.
nlm.nih.gov/pubmed/4342200.
Talerman, A. (1980). The pathology of gonadal neoplasms
composed of germ cells and sex cord stroma deriva-
tives. Pathology, Research and Practice, 170(1–3),
24–38. https://doi.org/10.1016/S0344-0338(80)
80153-1.
Zuntova, A., Motlik, K., Horejsi, J., & Eckschlager,
T. (1992). Mixed germ cell-sex cord stromal tumor
with heterologous structures. International Journal of
Gynecological Pathology, 11(3), 227–233. https://
www.ncbi.nlm.nih.gov/pubmed/1328081.
There are no characteristic radiological features to aid in the diagnosis of this entity. Furthermore, appropriate diagnosis and presence of any
high-grade morphology corresponding to malignant potential can be inferred exclusively by histological examination.
Clinical Features
• Incidence
Rare, <0.5% (Young and Scully 1985).
• Age
Young patients, average age – 25 years.
• Sex
Females, males (single case report with SLCT
in testis).
• Site
Ovary, testes (see above).
• Treatment
See “Outcome” below.
Mixed Sex Cord-Stromal Tumors, Pathology of the Ovary 315
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• Outcome
Overall, these tumors are rare, present at a low
stage and have low recurrence rates. Since
these tumors are mostly unilateral and occur
in young patients, fertility-sparing conservative surgeries are opted for.
Macroscopy
SLCTs typically occur as unilateral adnexal
tumors in young women. They are usually variably sized, solid or cystic masses with lobulated,
yellow-sectioned surfaces. The poorly differentiated forms are associated with hemorrhage and
necrosis. Retiform type is associated with soft
and spongy gross morphology (Young
et al. 2018).
Microscopy
The classic morphology of SLCTs is that of solid
tubular pattern in a background of fibrous stroma.
However, these tumors demonstrate diverse
degree of tubular differentiation and quantity of
primitive gonadal stroma and are therefore rightfully categorized into well differentiated, intermediate differentiated, poorly differentiated, and
retiform types. When heterologous elements are
present, these tumors are categorized either into
moderate/intermediate, poor, and retiform types.
1. Well differentiated: As mentioned above, these
tumors have solid tubular pattern in a fibrous
background arranged in lobules. The cells lining the tubules are Sertoli cells which appear
columnar and monomorphic. These cells are
identified as dark cells with scant cytoplasm.
The Leydig cells are eosinophilic or lipid rich/
vacuolated cells lacking cytological atypia
arranged in clusters, cords, or as single cells
at the periphery of the lobules.
2. Moderate/intermediate differentiated to poorly
differentiated forms: More cellular than welldifferentiated forms with sarcomatoid stroma/
primitive gonadal stroma and increased
Mixed Sex Cord-Stromal Tumors, Pathology of the
Ovary, Fig. 1 Sertoli-Leydig cell tumor, poorly differen-
tiated. The image shows a poorly differentiated tumor with
lobulated features, hypo- and hypercellular areas, with
atypical cells
mitoses. Mitotic figures range from about
5/10HPF to 20/10HPF in poorly differentiated
forms (Figs. 1, 2, 3, 4 and 5).
3. Retiform: Network-like architecture with
irregularly branching narrow, slit-like tubules
and cysts lined by cuboidal or columnar epithelium, resembling the rete testis and rete
ovarii. It may also include cystic structures
with papillary or polypoid projections or
microcystic structures with flattened
epithelium.
The tumors are graded based on quantitative
assessment of tubular forms of Sertoli cells and
primitive gonadal stroma. A higher grade is
provided to tumors with less tubular differentiation and high primitive stroma content.
Retiform pattern is known to commonly occur
in younger women and associated with increased
alpha-fetoprotein levels secondary to hepatocytic
differentiation (Mooney et al. 1999). Nevertheless, the levels are not as high as in yolk sac
tumors (Young et al. 2018).
The heterologous elements observed so far are
mucinous epithelium, hepatocytes, rhabdomyoblasts,
cartilage, neuroblastoma, and carcinoid. So far,
mucinous epithelium is demonstrated to be the
most common one. The mucinous epithelium can
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316 Mixed Sex Cord-Stromal Tumors, Pathology of the Ovary
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Mixed Sex Cord-Stromal Tumors, Pathology of the
Ovary, Fig. 2 Sertoli-Leydig cell tumor, poorly differen-
tiated. The atypical cells with scant to moderate amount of
cytoplasm, dark hyperchromatic nuclei, and numerous
mitotic figures
Mixed Sex Cord-Stromal Tumors, Pathology of the
Ovary, Fig. 3 SLCT, focally positive for calretinin
immunostain (nuclear)
range from benign to malignant (Lim and Oliva
2018).
Malignant SLCTs: The tumors which recur
and metastasize are considered clinically malignant, amount to 18% of the cases. The tumors
which are poorly differentiated, high grade, have
heterologous elements tend to be malignant
(Young and Scully 1985).
Mixed Sex Cord-Stromal Tumors, Pathology of the
Ovary, Fig. 4 SLCT, rhabdomyosarcomatous differenti-
ation. Other heterologous elements like chondrosarcomatous elements were also seen in this case
Mixed Sex Cord-Stromal Tumors, Pathology of the
Ovary, Fig. 5 SLCT, Positive for myogenin (nuclear).
Myogenin highlights the rhabdomyosarcomatous element
differentiate these tumors from other categories.
Keratins in particular highlight Sertoli and Leydig
cells. FOXL2 and DICER1 immunostains are the
novel stains used in these tumors. They are positive in Sertoli cells more than in Leydig cells
(Lim and Oliva 2018). Other stains used are
SF-1, CD99, CD56, vimentin, and WT-1.
Immunophenotype
Inhibin and calretinin highlight sex-cord cells and
stromal cells in variable degrees and can help
Molecular Features
Molecularly, some of the SLCTs have germline
DICER1 gene mutations and mosaicism. This
Molar Pregnancy, Pathology of the Placenta 317
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molecular abnormality is associated with multinodular goiter (MNG), thyroid cancer
(differentiated thyroid carcinomas are less likely
than MNG), pleuropulmonary blastoma, embryonal rhabdomyosarcoma of cervix, cystic
nephroma of kidney, and few other conditions.
Knowledge of the presence of this mut ation in
these women will allow their progeny to be tested
for the conditions of the syndrome, especially
pleuropulmonary blastoma.
Differential Diagnosis
SLCTs popularly resemble endometrioid type of
epithelial neoplasms category in their welldifferentiated tubular pattern. Absence of evidence of endometriosis (42% of ovarian endometrioid carcinomas are associated with
endometriosis), and inhibin positivity can aid in
the diagnosis. At times, retiform pattern of SLCTs
with papillary configuration resemble serous
tumors. Mucinous epithelium, the most common
heterologous element in SLCTs, when present
alone can resemble mucinous neopla sm of the
ovary. The caution should be maintained for adequate sampling of ovarian neoplasms in order to
avoid this error. The general guideline for tumor
sampling in ovary is one section/centimeter of
tumor (CAP guidelines for ovarian tumor sampling). The presence of gonadal stroma in close
apposition to heterologous elements is a great clue
towards SLCTs. As per Dr. Scully, some
Krukenberg tumors with tubular pattern can
resemble SLCTs and are termed by him as tubular
Krukenberg tumors. Both entities are associated
with stromal leutinization and edema. Nevertheless, the clinical history, bilaterality, intestinaltype glands with goblet cells associated with
Krukenberg tumors help with diagnosis (Young
and Scully 1985).
References and Further Reading
Gui, T., Cao, D., Shen, K., et al. (2012). A clinicopathological
analysis of 40 cases of ovarian Sertoli-Leydig cell tumors.
Gynecologic Oncology , 127(2), 384–389. https://doi.org/
10.1016/j.ygyno.2012.07.114 .
Lim, D., & Oliva, E. (2018). Ovarian sex cord-stromal
tumours: An update in recent molecular advances.
Pathology, 50(2), 178–189. https://doi.org/10.1016/j.
pathol.2017.10.008.
Mooney, E. E., Nogales, F. F., & Tavassoli, F. A. (1999).
Hepatocytic differentiation in retiform Sertoli-Leydig
cell tumors: Distinguishing a heterologous element
from Leydig cells. Human Pathology, 30(6), 611–617.
https://doi.org/10.1016/S0046-8177(99)90083-7.
Young, R. H., & Scully, R. E. (1985). Ovarian Sertoli-
Leydig cell tumors. A clinicopathological analysis of
207 cases. The American Journal of Surgical Pathol-
ogy, 9(8), 543–569. https://doi.org/10.1097/00000478-
198508000-00001.
Young, R. H., Morris, J. M., & Scully, R. E. (2018).
Ovarian sex cord-stromal tumors reflections on a
40-year experience with a fascinating group of tumors,
in, cluding comments on the seminal observations of
Robert E. Scully, MD. Archives of Pathology & Labo-
ratory Medicine, 142, 1459–1484. https://doi.org/10.
5858/arpa.2018-0291-RA.
Molar Pregnancy, Pathology
of the Placenta
Joseph T. Rabban
University of California – San Francisco,
San Francisco, CA, USA
Synonyms
Complete hydatidiform mole; Partial
hydatidiform mole.
Definition
Complete hydatidiform mole (CHM) is a genetically abnormal gestation defined by diandric diploid genotype (in most cases), chorionic villus
architectural abnormalities, and trophoblast proliferation without embryonic development. Partial
hydatidiform mole (PHM) is defined by diandric
triploid genotype, chorionic villous architectural
abnormalities, and patchy trophoblast proliferation (Fig. 1).
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318 Molar Pregnancy, Pathology of the Placenta
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Molar Pregnancy, Pathology of the Placenta,
Fig. 1 Complete mole hydatidiform mole exhibits uni-
form villous enlargement (a), Villous cisterns and exuberant villous trophoblast proliferation (b). Partial mole
Clinical Features
• Incidence
There are geographic differences in the incidence of molar pregnancy, the lowest being in
North American and western Europe
(approximately 0.8–1.5 cases/1000 pregnancies) and the highest being up to nearly tenfold
greater in southeastern Asia. The cause of the
variation remains to be fully defined.
• Age
Molar pregnancy can occur at any
reproductive age.
• Sex
Not applicable.
• Site
Molar pregnancies involve intrauterine gestations though rare cases have also been
exhibits a subset of enlarged villi with irregular contours
and a subset of smaller fibrotic villi (c), as well as villous
contour invaginations and inclusions and patchy villous
trophoblast proliferation (d)
reported in ectopic tubal and ovarian pregnancies. Extrauterine involvement by
lymphovascular spread from the uterus may
occur rarely.
• Treatment
Most p atients can be managed after uterine
evacuation of molar pregnancy without chemotherapy by conducting several months of
serial surveillance of quantitative serum
human chorionic gonadotropin (hCG),
which should norma lize shortly after the
evacuation procedure, in order to monitor
for development of persistent gestational
trophoblastic disease (GTD). Prophylactic
methotrexate at the time of diagnosis
may be appropriate for some patients for
whom serial hCG surveillance may not be
feasible.
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