Sabato 27 ottobre 2012 - Pacini Editore

COmuNiCaziONi ORali
GEnITALE MASCHILE E FEMMInILE
Choriocarcinomatous differentiation in a low-grade
endometrioid adenocarcinoma:
a rare histopathological finding in an otherwise
healthy perimenopausal woman
G. Crisman1 , L. Sollima1 , M. Di Nicola2 , V. Accurti2 , F. Patacchiola3
, G. Coletti4 , P. Leocata1 , G. Carta2 1 Anatomia Patologica, Dipartimento di Scienze della Salute, Università
dell’Aquila, L’Aquila, Italia; 2 U.O.C. Ginecologia ed Ostetricia D.U.,
Università degli Studi dell’Aquila, L’Aquila, Italia; 3 U.O.C. Ginecologia
ed Ostetricia, Ospedale “Val Vibrata”, Sant’Omero (TE), Italia; 4 U.O.C.
Anatomia Patologica, Ospedale Civile “San Salvatore”, L’Aquila, Italia
Background. A choriocarcinomatous differentiation has been
reported in association with some cancer types, especially with
those arising in the female genital tract and rarely with carcinomas
of other sites (i.e., urinary tract, gastrointestinal tract, lung
and breast). We report on a case of a 50-year-old woman with
a low-grade endometrioid adenocarcinoma (EAC) with choriocarcinomatous
differentiation (CD). Up to now, less than twenty
cases have been reported so far 1-16 .
Material and methods. We report on a case of a 50- year-old
nulliparous, peri-menopausal woman presented to our Department
with an enlarged uterus, high serum CA125 levels, and persistent
metrorrhagia. Anamnestic data revealed a eighteen-month
history of multiple intramural and subserous uterine myomas and
dysfunctional uterine bleeding, in the absence of the conventional
risk factor for endometrial adenocarcinoma, such as hypertension,
obesity or diabetes. A previous diagnostic hysteroscopy
with biopsy, performed after the first clinical examination in
March 2010, ruled out a diagnosis of glandular hyperplasia of
the endometrium. In September 2011, the patient presented to
our Department with an enlarged uterus reaching the transumbilical
plane. Serum CA125 levels significantly increased up to 241
mU /ml eighteen months later. An abdominal hysterectomy with
salpingo-oophorectomy was performed.
Grossly, the uterus appeared enlarged and deformed by the presence
of many intramural and subserous miomas.
Results. Histopathological features revealed tumor mainly composed
of a well-differentiated endometrioid adenocarcinoma with
an admixed component of cytotrophoblast-like pleomorphic cells
with large, rounded nuclei and clear cytoplasm, and syncityallike
cells with multiple, irregular and hyperchromatic nuclei and
eosinophilic cytoplasm. Immunohistochemical stainings showed
a strong and diffuse positivity for Cytokeratin 7 and Cytokeratin
AE1/AE2 of the EAC tumor cells whereas a negativity for
p53 was detected. A single submillimetric focus of myometrial
invasion was observed and highlighted by the Cytokeratin AE1/
AE3 stain. Stains for Cytokeratin AE1/AE2, β-hCG and HER-2
showed a positive reaction in syncytiotrophoblast-like cells.
Thus, a diagnosis of low-grade (G1) endometrioid adenocarcinoma
(EAC) with choriocarcinomatous differentiation (CD) was
posed.
Up to now, the patient’s health conditions are good, with any
evidence of local or distant metastases.
Conclusions. First described by Civantos and Rywlin in 1972,
endometrial adenocarcinoma with choriocarcinomatous differentiation
is still considered a very rare entity with an aggressive
biological course. It usually occurs in middle age women, with a
mean age of 69.7 years (range 48-88). The aetiology and patho-
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Fig. 1A and B. a well-differentiated endometrioid adenocarcinoma
with an admixed component of cytotrophoblast-like pleomorphic
cells with large, rounded nuclei and clear cytoplasm, and syncityal-like
cells with multiple, irregular and hyperchromatic nuclei and eosinophilic
cytoplasm. (H&E, 4x and 20x magnification).
genesis of a trophoblastic differentiation within adenocarcinoma
of the endometrium is still poorly understood. Abnormal uterine
bleeding, bloodstained vaginal discharge and/or abdominal pain
represent the most common clinical presentations. Surgical treatment
represents the gold standard therapy of this kind of tumors,
even though chemotherapy has been sometimes suggested in addition.
A close follow-up is recommended.
Up to now, less than twenty cases have been reported so far 1-16 .
A CD has been reported in association with some kind of tumors,
generally sited in the female genital tract and rarely with
carcinomas of other sites (i.e., urinary tract, gastrointestinal tract,
lung and breast). Thus, a differential diagnosis with giant cell
carcinoma, carcinosarcoma, undifferentiated carcinoma with
giant cells should be considered and an accurate anamnestical,
clinical, histopathological and immunohistochemical study
should be performed in the aim to achieve the correct diagnosis.
The presence of at least a focus of a conventional EAC should
support the diagnosis of a CD, as well as an advanced age of the
patient, the presence of syncityal-like giant β-hCG positive cells,
as in the present case.
Since a CD is reported to have an aggressive biological course,
with early extensive metastases and poor prognosis if compared
to other gynaecological cancers without this component, the goal
of every pathologist is to achieve the correct diagnosis in the aim
to improve the patient’s outcome, by ensuring that the most appropriate
therapy approach is chosen.
Fig. 2. Strong expression for Cytokeratin 7 in both component (CK7,
10x magnification).