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Mesenchymal Tumors, Pathology of the Ovary 229
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tumors that push native ovarian elements but rather
involves the ovarian stroma and incorporates ovarian follicles. In addition, massive ovarian edema
encompasses eosinophilic proteinaceous material
rather than basophilic myxoid material. Myxoid
degeneration of an ovarian sex cord-stromal
tumor, such as a fibroma or sclerosing stromal
tumor may be seen. This may be challenging to
distinguish from a myxoma, especially as the latte
may develop within the same ovary and in association with ovarian sex cord-stromal tumors. This
association may be related to a shared tissue of
origin for these two types of tumors. In the case
of myxoid degeneration of a tumor, the myxoid
areas do not form a distinct well-circumscribed
mass but rather involve cellular tumors in an illdefined intermingled fashion that cannot be delineated from the remainder tumor. One of the most
significant differential consideration for a
circumscribed myxoid tumor is a low-grade sarcoma with myxoid degeneration, e.g., myxoid
liposarcoma. The presence of any significant atypia
and pleomorphism, or mitotic activity should
worry extensive sampling and careful examination
of the tumor to rule out a sarcoma. Myxoid
liposarcoma may share a number of morphologic
features with myxomas, such as the cellularity and
the thin-walled vasculature, but also contains vacuolated lipoblasts and harbors specificgene
rearrangement, the most common of which being
t(12;16)(q13;p11.2) FUS-DDIT3, which are absent
in ovarian myxomas.
References and Further Reading
Amador-Ortiz, C., Roma, A. A., Huettner, P. C., Becker,
N., & Pfeifer, J. D. (2011). JAZF1 and JJAZ1 gene
fusion in primary extrauterine endometrial stromal sar-
coma. Human Pathology, 42(7), 939–946. https://doi.
org/10.1016/j.humpath.2010.11.001.
Chang, K. L., Crabtree, G. S., Lim-Tan, S. K., Kempson,
R. L., & Hendrickson, M. R. (1993). Primary extrauter-
ine endometrial stromal neoplasms: A clinicopatho-
logic study of 20 cases and a review of the literature.
International Journal of Gynecological Pathology,
12(4), 282–296.
Chiang, S., Ali, R., Melnyk, N., McAlpine, J. N., Hunts-
man, D. G., Gilks, C. B., ... Oliva, E. (2011). Fre-
quency of known gene rearrangements in endometrial
stromal tumors. The American Journal of Surgical
Pathology, 35(9), 1364–1372. https://doi.org/10.1097/
PAS.0b013e3182262743.
Cojocaru, E., Palahepitiva Gamage, G., Butler, J., Barton,
D. P., Thway, K., Fisher, C., ... Jones, R. L. (2021).
Clinical management and outcomes of primary ovarian
leiomyosarcoma – Experience from a sarcoma specialist unit. Gynecologic Oncology Reports, 36, 100737.
https://doi.org/10.1016/j.gore.2021.100737.
Eichhorn, J. H., & Scully, R. E. (1991). Ovarian myxoma:
Clinicopathologic and immunocytologic analysis of
five cases and a review of the literature. International
Journal of Gynecological Pathology, 10(2), 156–169.
Furutake, Y., Fukagawa, T., Suga, Y., Nagasawa, T., Sato,
S., Omi, H., ... Sugiyama, T. (2018). Gemcitabine and
docetaxel in a patient with primary ovarian
leiomyosarcoma: A case report and review of literature.
International Cancer Conference Journal, 7(1), 11–15.
https://doi.org/10.1007/s13691-017-0309-7.
Kandalaft, P. L., & Esteban, J. M. (1992). Bilateral massive
ovarian leiomyomata in a young woman: A case report
with review of the literature. Modern Pathology, 5(5),
586–589.
Kikuchi, N., Sugita, S., Nakanishi, K., Sugawara, T., Segawa,
K.,Ito,Y.,... Hasegawa, T. (2017). Ovarian high-grade
endometrioid stromal sarcoma with YWHAE and
NUTM2B rearrangements. Pathology International,
67(6), 327–329. https://doi.org/10.11 11/pin.12542.
Lerwill, M. F., Sung, R., Oliva, E., Prat, J., & Young, R. H.
(2004). Smooth muscle tumors of the ovary:
A clinicopathologic study of 54 cases emphasizing
prognostic criteria, histologic variants, and differential
diagnosis. The American Journal of Surgical Pathol-
ogy, 28(11), 1436–1451. https://doi.org/10.1097/01.
pas.0000141393.99300.d0.
Li, Z., Maeda, D., Kudo-Asabe, Y., Tamura, D., Nanjo, H.,
Hayashi, A., ...Goto, A. (2018). MED12 is frequently
mutated in ovarian and other adnexal leiomyomas.
Human Pathology, 81,89
j.humpath.2018.06.013.
Masand, R. P., Euscher, E. D., Deavers, M. T., & Malpica,
A. (2013). Endometrioid stromal sarcoma:
A clinicopathologic study of 63 cases. The American
Journal of Surgical Pathology, 37(11), 1635–1647.
https://doi.org/10.1097/PAS.0000000000000083.
Oliva, E., Egger, J. F., & Young, R. H. (2014). Primary
endometrioid stromal sarcoma of the ovary:
A clinicopathologic study of 27 cases with morphologic and behavioral features similar to those of uterine
low-grade endometrial stromal sarcoma. The American
Journal of Surgical Pathology, 38(3), 305–315. https://
doi.org/10.1097/PAS.0000000000000145.
Roth, L. M., Gaba, A. R., & Cheng, L. (2013). The patho-
genesis of ovarian myxoma: A neoplasm sometimes
arising from other ovarian stromal tumors. International
Journal of Gynecological Pathology, 32(4), 368–378.
https://doi.org/10.1097/PGP.0b013e3182630d4e.
Stewart, C. J., Leung, Y. C., Murch, A., & Peverall,
J. (2014). Evaluation of fluorescence in-situ hybridization in monomorphic endometrial stromal neoplasms
–95. https://doi.org/10.1016/
M
230 Mesenchymal Tumors, Pathology of the Peritoneum
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and their histological mimics: A review of 49 cases.
Histopathology, 65(4), 473–482. https://doi.org/10.
1111/his.12406.
Xie, W., Bi, X., Cao, D., Yang, J., Shen, K., &You, Y.(2017).
Primary endometrioid stromal sarcomas of the ovary:
A clinicopathological study of 14 cases with a review of
the literature. Oncotarget, 8(38), 63345–63352. https://
doi.org/10.18632/oncotarget.18805.
Mesenchymal Tumors,
Pathology of the Peritoneum
Ben Davidson
Department of Pathology, Norwegian Radium
Hospital, Oslo University Hospital, Oslo, Norway
University of Oslo, Faculty of Medicine, Institute
of Clinical Medicine, Oslo, Norway
Leiomyomatosis Peritonealis
Disseminata
Synonyms
Disseminated peritoneal leiomyomatosis.
• Age
Predominantly premenopausal women, often
associated with pregnancy or use of oral
contraceptives.
• Sex
Female.
• Site
Peritoneum, omentum, outer surface of the
uterus, adnexae, and intestine.
• Treatment
Surgery, antihormonal (GnRH analogs and
aromatase inhibitors).
• Outcome
Benign condition. Rare transformation to
leiomyosarcoma described.
Macroscopy
Multiple tan-white nodules or variable size.
Microscopy
Smooth muscle cells with no evidence of malignancy, as in uterine leiomyoma (Fig. 1).
Immunophenotype
Tumors are positive for smooth muscle markers
(SMA, actin, desmin, and caldesmon).
Definition
Presence of multiple benign smooth muscle nod-
Molecular Features
MED12 mutations.
ules in the abdominal cavity.
Differential Diagnosis
Clinical Features
• Incidence
Uncommon condition.
Mesenchymal Tumors, Pathology of the Peritoneum, Fig. 1 (a, b) Leiomyomatosis peritonealis disseminata:
Nodules of smooth muscle lacking atypia, brisk mitotic activity, or necrosis in an omental resection
1. Gastrointestinal stromal tumor (GIST) metastases. Negative or focal IHC for c-Kit and
DOG-1 is helpful.
Mesenchymal Tumors, Pathology of the Peritoneum 231
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Desmoplastic Small Cell Round Tumor
Definition
Malignant small cell tumor with desmoplastic
response and EWSR1-WT1 gene fusion.
Clinical Features
• Incidence
Rare.
• Age
Adolescents and young adults.
• Sex
Male predominance.
• Site
The majority are abdominal (pelvis, retroperitoneum, omentum, and mesentery).
• Treatment
Surgery and chemotherapy.
• Outcome
Poor, the majority of patients die within 2–3 years.
Macroscopy
Multiple nodules manifesting clinically as
sarcomatosis.
Microscopy
Sharply demarcated nests of small, usually round
cellssurroundedbymarkeddesmoplasticresponse.
Tumor cells have hyperchromatic or vesicular
nuclei, inconspicuous nucleoli, scanty cytoplasm,
and indistinct cell borders. Necrosis and high
mitotic count are commonly seen (Fig. 2).
Immunophenotype
Polyphenotypic, expressing epithelial (AE1/AE3
and EMA), muscle (desmin; often dot-like), and
neural (NSE, CD57, and WT1) markers. WT1
positivity is only seen with antibodies directed at
the carboxy-terminus. S100 and actin are variably expressed, and CD99 staining is usually
negative.
Mesenchymal Tumors, Pathology of the Peritoneum,
Fig. 2 (a, b, c) Desmoplastic small cell round tumor:
Peritoneal resection showing a small cell tumor with
M
extensive desmoplasia. Tumor cells are small, with low
n/c ratio and brisk mitotic activity
232 Mesenchymal Tumors, Pathology of the Peritoneum
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Molecular Features
EWSR1-WT1 gene fusion.
Solitary Fibrous Tumor
Definition
A fibroblastic tumor carrying NAB2-STAT6 gene
rearrangement.
Clinical Features
• Incidence
Rare (<2% of all soft tissue masses).
• Age
Wide age range, most often diagnosed in the
fifth or sixth decade.
• Sex
Both; equal distribution.
• Site
Multiple anatomic locations, most commonly
intrathoracic, followed by intra-abdominal.
• Treatment
Surgery.
• Outcome
Variable. CNS location, hypoglycemia, and dedifferentiation associated with a worse outcome.
Macroscopy
Solid, well-circumscribed, variable size.
Microscopy
Haphazard growth of fibroblastic cells with thinwalled branching vessels. Usually low-grade
atypia and low mitotic count. Multinucleated
giant cells or dedifferentiation (transition to sarcoma) may be seen (Fig. 3).
Immunophenotype
Tumors are positive for CD34 and STAT6 (Fig. 4).
Molecular Features
NAB2-STAT6 gene rearrangement.
Aggressive Fibromatosis
Synonyms
Desmoid-type fibromatosis.
Definition
A myofibroblastic tumor characterized by locally
invasive growth harboring CTNNB1 or APC
mutation.
Clinical Features
• Incidence
Rare.
• Age
Wide age range (15–60), most often diagnosed
in young adults.
• Sex
Female predominance (two to three-fold).
• Site
Multiple anatomic locations, most commonly
abdominal wall, limbs, girdles, and
mesenterium.
Mesenchymal Tumors,
Pathology of the
Peritoneum,
Fig. 3 Solitary fibrous
tumor: Spindle cells with
low-grade atypia, separated
by thick collagen bundles
Mesenchymal Tumors, Pathology of the Peritoneum 233
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Mesenchymal Tumors,
Pathology of the
Peritoneum,
Fig. 4 Solitary fibrous
tumor: STAT6
immunostaining showing
typical nuclear expression
• Treatment
Follow-up in nonprogressive cases; Surgery
when mandated; chemotherapy, radiotherapy,
or antihormonal therapy in selected cases.
• Outcome
Variable, recurrence usually local. CTNNB1
S45F mutation associated with incre ased risk
for recurrence.
Macroscopy
Solid, relatively well-circumscribed, tumor with
firm white cut surface.
Microscopy
Monotonous growth of fibroblastic or
myofibroblastic cells with few mitotic figures.
Variable collagen deposition. Often involved margin in surgical specimens.
Immunophenotype
Many tumors have nuclear expression of β-catenin.
Molecular Features
Activating CTNNB1 mutations or inactivating
APC mutations, the latter associated with familial
adenomatous polyposis (FAP).
Clinical Features
• Incidence
Rare; association with pregnancy and trauma.
• Age
Wide age range, most often diagnosed in children or young adults.
• Sex
Slightly more often in females (female: male
ratio 1.27).
• Site
Multiple anatomic locations, most commonly
soft tissue. May be found in the pleural or
peritoneal surfaces or associated with the gastrointestinal tract.
• Treatment
Surgery. Follow-up in selected patients with
small tumors.
• Outcome
Good. Local recurrence may occur with
incomplete resection.
Macroscopy
Well-circumscribed unencapsulated tumor
(average size 2.6. cm, range 1–10 cm) with
homogenous gritty gray-white cut surface.
M
Calcifying Fibrous Tumor
Definition
Benign mesenchymal tumor characterized by
hyalinized stroma, scattered calcifications, and
lymphoplasmacytic inflammation.
Microscopy
Hypocellular tumor with monomorphic spindle
cells lacking atypia and abundant hyalinized collagen. Few mitotic figures and no necrosis. Scattered
calcifications which may be psammomatous or dystrophic. A lymphoplasmacytic infiltrate, occasionally with the formation of lymphoid follicles.
234 Mesenchymal Tumors, Pathology of the Uterine Corpus
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Immunophenotype
Positive staining for CD34, vimentin, and factor
XIIIa; negative staining for smooth muscle actin,
muscle-specific actin, ALK, desmin, S100,
cytokeratin, DOG1, and c-kit.
Molecular Features
No diagnostic or clinically relevant findings.
References and Further Reading
Davanzo, B., Emerson, R. E., Lisy, M., Koniaris, L. G., &
Kays, J. K. (2018). Solitary fibrous tumor. Transla-
tional Gastroenterology and Hepatology, 3, 94.
Fredericks, S., Russell, P., Cooper, M., & Varol, N. (2005).
Smooth muscle in the female pelvic peritoneum:
A clinicopathological analysis of 31 women. Pathol-
ogy, 37,14–21.
Kasper, B., Baumgarten, C., Garcia, J., Bonvalot, S., Haas,
R., Haller, F., Hohenberger, P., et al. (2017). An update
on the management of sporadic desmoid-type
fibromatosis: A European Consensus Initiative
between Sarcoma PAtients EuroNet (SPAEN) and
European Organization for Research and Treatment of
Cancer (EORTC)/Soft Tissue and Bone Sarcoma
Group (STBSG). Annals of Oncology, 28, 2399–2408.
Kilpatrick, S. E., Reith, J. D., & Rubin, B. (2018). Ewing
sarcoma and the history of similar and possibly related
small round cell tumors: From whence have we come
and where are we going? Advances in Anatomic
Pathology, 25, 314–326.
Mehrad, M., LaFramboise, W. A., Lyons, M. A., Trejo
Bittar, H. E., & Yousem, S. A. (2018). Whole-exome
sequencing identifies unique mutations and copy number losses in calcifying fibrous tumor of the pleura:
Report of 3 cases and review of the literature. Human
Pathology, 78,36–43.
Penel, N., Chibon, F., & Salas, S. (2017). Adult desmoid
tumors: Biology, management and ongoing trials. Cur-
rent Opinion in Oncology, 29, 268–274.
Rieker, R. J., Agaimy, A., Moskalev, E. A., Hebele, S.,
Hein, A., Mehlhorn, G., Beckmann, M. W., et al.
(2013). Mutation status of the mediator complex subunit 12 (MED12) in uterine leiomyomas and concurrent/metachronous multifocal peritoneal smooth
muscle nodules (leiomyomatosis peritonealis
disseminata). Pathology, 45, 388–392.
Skubitz, K. M. (2017). Biologyand treatment of aggressive
fibromatosis or desmoid tumor. Mayo Clinic Proceed-
ings, 92, 947–964.
Stiles, Z. E., Murphy, A. J., Anghelescu, D. L., Brown,
C. L., Davidoff, A. M., Dickson, P. V., Glazer, E. S.,
et al. (2020). Desmoplastic small round cell tumor:
Long-term complications after cytoreduction and
hyperthermic intraperitoneal chemotherapy. Annals of
Surgical Oncology, 27, 171–178.
Tavassoli, F. A., & Norris, H. J. (1982). Peritoneal
leiomyomatosis (leiomyomatosis peritonealis
disseminata): A clinicopathologic study of 20 cases
with ultrastructural observations. International Journal
of Gynecological Pathology, 1,59–74.
Thway, K., & Fisher, C. (2019). Mesenchymal tumors with
EWSR1 gene rearrangements. Surgical Pathology
Clinics, 12, 165–190.
Turbiville, D., & Zhang, X. (2020). Calcifying fibrous
tumor of the gastrointestinal tract: A clinicopathologic
review and update. World Journal of Gastroenterology,
26, 5597–5605.
WHO Classification of Tumours Editorial Board (Female
Genital Tumours) (Vol. 4, 5th ed.). (2020). Lyon: International Agency for Research on Cancer.
Yamada, Y., Kohashi, K., Kinoshita, I., Yamamoto, H.,
Iwasaki, T., Yoshimoto, M., Ishihara, S., et al. (2019).
Clinicopathological review of solitary fibrous tumors:
Dedifferentiation is a major cause of patient death.
Virchows Archiv, 475, 467–477.
Mesenchymal Tumors,
Pathology of the Uterine
Corpus
Sarah Chiang
Memorial Sloan Kettering Cancer Center,
New York, NY, USA
Leiomyoma
Definition
Uterine leiomyoma is a benign tumor arising from
myometrial smooth muscle cells.
Clinical Features
• Incidence
Uterine leiomyomas are the most common pelvic tumor in women (Baird et al. 2003). Their
incidence is difficult to determine due to few
longitudinal studies (Stewart et al. 2017).
Some uterine leiomyomas may occur in the
setting of hereditary leiomyomatosis and
renal cell cancer (HLRCC), an autosomal dominantly inherited disease, in which the incidence is also unknown.
• Age
Leiomyomas typically affect women of reproductive age (Styer and Rueda 2016) with only
Mesenchymal Tumors, Pathology of the Uterine Corpus 235
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rare cases reported in adolescents (Moroni
et al. 2015). In the HLRCC setting, uterine
leiomyomas develop at an earlier age than in
the general population with a median age of
28–30 years (Alam et al. 2005).
• Site
Leiomyomas may be submucosal, intramural,
or subserosal.
• Treatment
Treatment is aimed at reduction of symptoms
(heavy menstrual bleeding, bulk symptoms,
pain, and impaired fertility) and depends on
whether fertility preservation is desired. Myomectomy is typically the first option in patients
desiring pregnancy (Stewart 2015). Surgical or
medical management of fibroid-related symptoms may be recommended in patients who do
not desire future fertility (Laughlin-Tommaso
et al. 2020; Yao et al. 2017). Initial treatment
options include myomectomy or estrogenprogestin contraceptives (Yao et al. 2017).
Gonadotropin-releasing hormone (GnRH) agonists and antagonists, endometrial ablation, and
hysterectomy may be considered in patients
with persistent symptoms (Lee et al. 2016;
Lethaby et al. 2017; Wright et al. 2013). Multidisciplinary managementis required for patients
with HLRCC and including symptomatic control of uterine and cutaneous leiomyomas, renal
cancer surveillance, and genetic counseling
(Schmidt and Linehan 2014).
• Outcome
Uterine leiomyomas are benign. Symptoms
attributable to leiomyomas often resolve at
menopause due to tumor shrinkage (Cramer
and Patel 1990). Patients with HLRCC may
develop cutaneous leiomyomas and/or renal
cell carcinoma with a lifetime renal cancer
risk of approximately 15% (Menk o et al.
2014; Toro et al. 2003); transformation of
leiomyoma to leiomyosarcoma in the setting
of HLRCC is rare (Ylisaukko-oja et al. 2006).
Macroscopy
Uterine leiomyomas are often multiple and variably sized (Cramer and Patel 1990). Most tumors
are intramural. Submucosal leiomyomas may
bulge into the endometrial cavity and appear sessile
or pedunculated. Some pedunculated tumors may
prolapsethrough the cervical os. Subserosal tumors
may also be pedunculated. Lesions are round, well
circumscribed, and unencapsulated with firm,
bulging, whorled, solid, and white to tan cut surfaces. Degenerative changes such as hemorrhage
and necrosis can occur particularly in large
leiomyomas, pedunculated tumors that undergo
torsion, or in the setting of pregnancy or progestin
therapy (Bennett et al. 2016). Cystic degeneration
and calcification can also occur. Some leiomyoma
variants exhibit gross features distinct from usual
type leiomyomas. Fish flesh, rubbery, and soft cut
surfaces may beseen in cellular leiomyomas (Oliva
et al. 1995). Myxoid leiomyomas may demonstrate
soft and translucent cut surfaces. Lipoleiomyomas
often show soft yellow cut surfaces.
Microscopy
Usual Leiomyoma
Usual leiomyomas consist of intersecting fascicles of uniform elongated spindle cells with indistinct borders, abundant eosinophilic cytoplasm,
centrally located cigar-shaped nuclei, even chromatin, and small nucleoli (Fig. 1a). Nuclear
palisading can be seen and is sometimes a prominent featu re. Mitotic figures are usually infrequent. Hyalinization and edema are common
(Clement et al. 1992; Coad et al. 1997) (Fig. 1b,
c). Sharply demarcated zones of hemorrhage, cys-
tic degeneration, and dystrophic calcifications can
be seen. Infarct necrosis (characterized by mummified neoplastic smooth muscle and blood vessels often accompanied by hemorrhage and/or a
peripheral rim of granulation, fibrous, or
hyalinized tissue) (Fig. 1d), inflammation, and
increased mitotic activity without nuclear atypia
may be seen particularly in submucosal
leiomyomas. In general, uterine leiomyomas are
circumscribed, but some tumors may demonstrate
irregular borders with interdigitation with the surrounding myometrium. Peripheral cleft spaces
and thick-walled blood vessels are common.
Cellular and Highly Cellular Leiomyoma
Rare leiomyomas may exhibit cellularity greater
than the surrounding myometrium (Fig. 2)
M
236 Mesenchymal Tumors, Pathology of the Uterine Corpus
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Mesenchymal Tumors, Pathology of the Uterine Corpus, Fig. 1 Leiomyoma. (a) Histologically bland spindle
cells are arranged in fascicles. (b) Hyaline plaques are
common. Degenerative changes can be present, including
Mesenchymal Tumors, Pathology of the Uterine Corpus, Fig. 2 Cellular leiomyoma. The tumor is densely
cellular but lacks cytologic atypia
(Oliva et al. 1995). Cells may appear round and/or
spindle with scant cytoplasm and form fascicular
patterns. Interdigitation with the surrounding
myometrium may be seen (Oliva 2014). Cellular
leiomyomas lack tumor necrosis and cytologic
atypia, and mitotic a ctivity is 4 mitotic figures
(c) edema and (d) infarct necrosis, characterized by hemorrhage and necrosis surrounded by a rim of granulation
tissue
(MF)/10 high power fields (HPF). While arterioles may be present, blood vessels are often large
and thick-walled. Peripheral cleft spaces are
common.
Mitotically Active Leiomyoma
Leiomyomas with typical or cellular morphology
and 5 MF/10 HPF are considered mitotically
active (Fig. 3). The mitotic index is usually 5–9
MF/10 HPF, but rare tumors may exhibit 10–20
MF/10 HPF. Atypical mitotic figures, cytologic
atypia, and tumor necrosis are not seen. Mitotically
active leiomyomas may be associated with reproductive age, pregnancy, and treatment with progestin or tamoxifen (Bell et al. 1994; Dgani et al. 1998;
Liu et al. 2006;O’Connor and Norris 1990;
Perrone and Dehner 1988; Prayson and Hart 1992).
Apoplectic Leiomyoma
Leiomyomas with apoplectic change typically
exhibit multiple stellate zones of hemorrhage
Mesenchymal Tumors, Pathology of the Uterine Corpus 237
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2014). The bizarre cells are characterized by
multilobated or multinucleated, hyperchromatic
nuclei, clumped and/or smudged chromatin,
large cytoplasmic pseudoinclusions, and abundant eosinophilic cytoplasm (Fig. 5b) (Bennett
et al. 2017b; Kefeli et al. 2018; Ubago et al.
2016). Staghorn vessels, globoid cytoplasmic
inclusions, prominent nucleoli with perinuclear
halos, and alveolar edema are common (Fig. 5c)
(Bennett et al. 2017b; Kefeli et al. 2018).
Karyorrhectic and pyknotic nuclei are frequent
Mesenchymal Tumors, Pathology of the Uterine Corpus, Fig. 3 Mitotically active leiomyoma. Numerous
mitotic figures are seen in an otherwise typical leiomyoma
and may mimic atypical mitotic figures, but
mitotic activity is <5 MF/10 HPF (Fig. 5d).
Tumor necrosis is absent.
Epithelioid Leiomyoma
Epithelioid leiomyomas consist of round or
polygonal cells with centrally positioned round
nuclei and eosinophilic or clear cytoplasm
arranged in sheets, clusters, cords, or trabeculae
(Fig. 6a, b). Some tumors may also demonstrate a
spindle component. Mitotic activity is <5 MF/10
HPF. No tumor necrosis or cytologic atypia is
present.
M
Mesenchymal Tumors, Pathology of the Uterine Corpus, Fig. 4 Apoplectic leiomyoma. Stellate zones of
recent hemorrhage are surrounded by histologically bland
neoplastic smooth muscle
surrounded by a hypercellular rim of round and/or
spindle cells with eosinophilic cytop lasm, increased
mitotic activity, pyknotic nuclei, edema, and
myxoid change (Bennett et al. 2016; Boyd and
McCluggage 2011; Myles and Hart 1985;Norris
et al. 1988)(Fig.4). No cytologic atypia or tumor
necrosis is present. Away from the hemorrhage, the
features are of a typical leiomyoma. Apoplectic
change may be associated with progestin therapy
or pregnancy.
Leiomyoma with Bizarre Nuclei (Bizarre
Leiomyoma, Symplastic Leiomyoma, and Atypical
Leiomyoma)
Otherwise typical leiomyomas may contain
bizarre cells that may be unifocal or more commonly multifocal or diffuse (Fig. 5a) (Croce et al.
Myxoid Leiomyoma
Myxoid leiomyomas are rare and considered a
diagnosis of exclusion. Tumors are characteri zed
by abundant a morphous extracellular myxoid
material (Fig. 7a). Lesions are well circumscribed
and exhibit no cytologic atypia, tumor necros is, or
mitotic activity (<2 MF/10 HPF) (Fig. 7b) (Burch
and Tavassoli 2011; Busca and Parra-Her ran
2017).
Leiomyoma with Other Elements
Other cellular elements may occasionally be present in otherwise typical leiomyomas.
Adipocytic differentiation is the most common
(Fig. 8a) (Wang et al. 2006). Other tissue types
such as brown fat, skeletal muscle, and cartilage
may also be seen. Hematopoietic cells may infil-
trate leiomyomas, and include extramedullary
hematopoiesis, prominent histiocytes, mast
cells, eosinophils, or lymphocytes (Fig. 8b)
(Fornelli et al. 1999;Gilbert1976; Martin-Reay
et al. 1991;Parkeretal.2005; Yamadori et al.
1993).
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Mesenchymal Tumors, Pathology of the Uterine Corpus, Fig. 5 Leiomyoma with bizarre nuclei. (a) Diffuse,
marked nuclear atypia is evident on low magnification. (b)
Large cells exhibit bizarre, often multilobated or multiple
hyperchromatic nuclei with numerous pseudoinclusions.
Mesenchymal Tumors, Pathology of the Uterine Corpus, Fig. 6 Epithelioid leiomyoma. Tumor cells may have (a)
eosinophilic or (b) clear cytoplasm
Immunophenotype
Leiomyomas express desmin, smooth muscle
actin (SMA), muscle-specific actin, h-caldesmon,
calponin, smooth muscle myosin heavy chain
(SMMHC), and HDAC8 (Agoff et al. 2001;de
(c) Prominent nucleoli, perinucleolar halos, and eosinophilic globoid cytoplasmic inclusions are common. (d)
Bizarre and karyorrhectic nuclei resemble atypical mitotic
figures
Leval et al. 2006; Eyden et al. 1992; Nucci et al.
2001; Oliva et al. 1995). They are also frequently
positive for WT1 and transgelin (Agoff et al.
2001; Delgado et al. 2021; Hwang et al. 2015).
A subset of tumors may express CD10 and
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