Vol.2, No.5, 302-305 (2013) Case Reports in Clinical Medicine
Unusually aggressive primary cloacogenic
carcinoma of the vulva: A case report and
literature review
Rajni Chibbar1, Kimberly A. Wood2, Christopher K. Giede3, Anit a Agrawal4*
1Department of Pathology, University of Saskatchewan, Saskatoon, Canada
2College of Medicine, University of Saskatchewan, Saskatoon, Canada
3Department of Obstetrics and Gynecology, Royal University Hospital, Saskatoon, Canada
4Department of Obstetrics and Gynecology, University of Saskatchewan, Saskatoon, Canada;
*Corresponding Author: anita.agrawal@usask.ca
Received 11 November 2012; revised 8 December 2012; accepted 12 January 2013
Copyright © 2013 Rajni Chibbar et al. This is an open access article distributed under the Creative Commons Attribution License,
which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.
Vulvar cancer is an uncommon tumor and re-
presents 3% - 5% of all female genital tract ma-
lignancies. The overall incidence is 1.5/100,000
women. Hi stop a thologi cally the v ast m ajori ty (90% )
are squamous cell carcinomas. Primary cloaco-
genic carcinoma of the vulva is extremely rare
with less than 20 cases reported in English lit-
erature [1]. The se tumors are though t to arise from
embryonic or ectopic rest s of cloacog enic tissue.
The majority of these reported ca ses is relativ ely
indolent cancer , only one case of cloacogenic car-
cinoma of the vulva reported metastatic spread
to the inguinal lymph nodes and none that de-
scribe distant metastases. Here we present an
aggressive and diagnostically challenging case
of cloacogenic carcinoma of the vulva and a re-
view of current literature to date.
Keywords: Cloacogenic Vulvar Cancer; Me tas tasis
Primary cancer of the vulva is rare accounting for only
3% - 5% of all lower female genital tract cancers. Pri-
mary cloacogenic carcinoma of the vulva is extremely
rare with less than 20 cases reported in English literature
[1]. Only one case reported metastatic spread to inguinal
lymph nodes and none that describe distant metastases.
These tumors are thought to arise from embryonic or ec-
topic rests of cloacogenic tissue. Here we present a case
of primary cloacogenic carcinoma of the vulva which
metastasized to the lungs and bilaterally to inguinal and
pelvic lymph nodes at initial presentation.
A 49-year-old nulligravida postmenopausal woman
presented in March of 2010, with multiple small vulvar
lesions an accompanying three weeks history of pain,
pressure on vulva, bleeding, pruritus and swelling. Phy-
sical examination revealed severe swelling and multiple
erythematous tender small nodules less than 1 cm in size
on the vulva and lower vaginal canal and palpable lymph
nodes in the right groin (Figure 1(A)).
Magnetic resonance imaging (MRI) of the pelvis showed
a 3.8 × 3.4 cm lobular heterogeneously enhancing mass
located in the lower region of the vaginal canal and vulva.
CT scan reported spread to the lung and inguinal and
pelvic lymph nodes with bilateral heterogeneously en-
hancing multi-lobulated masses in the groin measuring
6.1 × 5.9 cm in the right, and 4.8 × 5.0 cm in the left.
Punch biopsies of the left vulva reported moderately dif-
ferentiated adenocarcinoma with a depth of invasion of
3.74 mm (Figure 1(B)). Lymphovascular space invasion
was noted. A non-neoplastic, slightly dilated gland lined
by non-neoplastic columnar epithelium and goblet cells
was present within the overlying squamous epithelium.
On immunohistochemistry neoplastic cells were high-
lighted with cytokeratin (CK) 20 (Figure 1(C)), CDX-2
(Figure 1(D)), carcinoembryonic antigen (CEA) and p53.
Mucicarmine stain was positive. Neoplastic cells were
negative with CK7, estrogen receptor (ER), progesterone
receptor (PR), Wilm’s tumor-1 (WT-1), p16, vimentin
and S-100 antibodies. Based on histology and immune
profiling a metastatic from the gastrointestinal tract was
suggested. Clinical evalu ation of the gastrointestinal tract
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R. Chibbar et al. / Case Reports in Clinical Medicine 2 (2013) 302-305 303
Figure 1. (A) Gross appearance of lesions on
vulva; (B) Low power (H & E ×4) of lesion
present on excisional biopsy of vulva and lym-
phovascular invasion (arrow); (C) CDX-2 nu-
clear staining of neoplastic cells; (D) CK-7 cy-
toplasmic staining for neoplastic cells and non-
neoplastic gland (*).
by colonscopy and computer topography (CT) showed
no pathology.
The vulvar cancer was treated urgently with radical
concurrent chemo-radiation over 43 days. After comple-
tion she underwent bilateral inguinal lymph node de-
bulking. Residual vulvar tumor, persistent enlarged pel-
vic lymph nodes, and lung lesions were noted in a sub-
sequent CT follow up and she was treated with car-
boplatin-paclitaxel as a second line chemotherapy for 6
cycles. After that bilateral external iliac lymph node de-
bulking was carried out for persistent pelvic lymph nodes
and histopathology reported viable tumor cells. In all
three histopathology specimens from various procedures,
neoplastic cells had a striking resemblance to colonic
adenocarcinoma and a diagnosis of cloacogenic carcino-
ma was made. The patient was treated with FOLFIRI
chemotherapy for three cycles with no response and was
then advised for no further chemotherapy. She was re-
ferred to palliative services for pain control and she died
after 27 months of initial diagnosis.
Only a few cases have reported adenocarcinoma aris-
ing from cloacal remnants. The average age of women
reported with cloacogenic tumors of the vulva or lower
genital tract was 51 years old and ranged from 35 to 67
years of age. Majority of the cases (Table 1) described
solitary tumors that ranged in size from 1.0 to 2.0 cm
largest diameter, presenting on the external urethral mea-
tus [2], labia majora [3], posterior vulva [4], posterior
fourchette [4-6], Bartholin’s gland [1], vagina [7] and
cervix [7]. One case describes multiple tumors ranging in
size from 0.1 to 1.4 cm on posterior to the fourchette [8].
Cell origin of these tumours is still unclear but it has
been proposed that they arise from remnant cells of the
cloacal membrane [2,6-8]. During the fourth to sixth
week of gestation the urorectal septum partitions the
cloaca into the primitive urogenital sinus ventrally and
the anal canal dorsally [9]. Distally, the urorectal septum
comes into contact with the anal membrane in this area
and forms the perineum. The anal pit is formed from pro-
liferating mesenchymal cells around the anal membrane.
The anal canal becomes patent around the seventh to
eighth week of gestation when the anal membrane rup-
tures creating a zone of separation that is demonstrated
by an irregular folding of the adult mucosa, the pectinate
The primitive urogenital sinus consists of three areas.
The superior portion expands to derive the bladder, the
narrow middle portion forms the pelvic urethra and the
inferior portion expands to form the definitive urogenital
sinus [9]. The definitive urogenital sinus is enclosed by
the urogenital membrane and goes on to form the endo-
derm-lined urethral groove, and later on the vestibule.
Externally, a pair of swellings adjacent to the urogenital
membrane forms the cloacal folds (the urogenital folds)
and the labioscrotal folds, which go on to form the labia
minoria and labia majoria, respectively. In the seventh
week of gestation the urogenital membrane ruptures cre-
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R. Chibbar et al. / Case Reports in Clinical Medicine 2 (2013) 302-305
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Table 1. Summary of clinical presentations and management of 12 patients.
Author Age Lesion type
Lymph node
metastasis Distant
metastasis Treatment Prognosis (months)
Lui et al., 2002, [1] 49 Left labia minora No No WLE/UL IFLND AWH at 24 months
Tiltman et al., 1978, [2] 50 External urinary meatus Yes No Modified radical
Vulvectomy and B/L IFLND NA
Dube et al., 2004, [3] 58 Right labia majora No No Right hemivulvectomy and
UL IFLND AWH at 16 months
Kennedy et al., 1993, [4] 54
Left Posterior vulva
Posterior fourchette No No
Radical vulvectomy and B/L
10 years (died of
AWH at 4 years
Ghamande et al ., 1995, [5] 67 Posterior fourchette No No Radical vulvectomy and
Willen et al., 1998, [6] 57 Left posterior vestibulumNo No WLE AWH at 26 months
Fox et al., 1988, [7] 35, 43, 59Vaginal vault
Zaidi et al., 2001, [8] 43 Posterior fourchette No No Partial posterior vulvectomy AWH at 18 months
Lee et al., 1990, [11] 27 Multifocal No
ating a patent urogen ital sinus. Therefore in the vestibule
and the labia minora, remnants of cloacal membrane
could persist and give rise to cloacogenic carcinoma of
the vulva. Remnants of cloacal membrane persist proxi-
mal to the pectinate line from where transitional cell
cloacogenic carcinoma can arise [10].
Treatment for the majority of the cases presented was
radical vulvectomy and bilateral local lymph node exci-
sion [2,4,5,8], with the more recent cases pursuing local
excision with bilateral local lymph node excision [1,3,6].
In the majority of the cases the tumor did not metasta-
sized and was referred to as indolent in nature and exci-
sion of the lesion(s) appeared to be curative[1,3-5,7,8]
however Willen et al. [6] reported recurrence of the tu-
mor one year later, and performed a wider resection of
the tumor. Our case, the tumor was aggressive in nature
with metastatic spread to inguinal and pelvic lymph
nodes and the lung. Complete excision of the tumor was
not possible in this case due the extent of tumor in-
volvement and metastasis and therefore was initially
treated with concurrent chemo-radiation therapy. Patient
had partial response to the treatment with some tumor
regression and pain relief, but the neoplastic cells re-
mained viable in the vulvar lesions after therapy was
completed. Patient then received second line carboplatin-
paclitaxel chemotherapy for persistent metastatic disease
after initial treatment. The other reported case of metas-
tatic spread to local lymph node, excision only was re-
quired for treatment and patient was reported to be doing
well one year after [2].
The majority of the cases reported a single tumor aris-
ing in direct continuity with the surface epithelium [1-6].
The tumor in our case was multifocal and it was difficult
to assess if the tumors arose in continuity with or beneath
the surface epithelium as only two punch and one ex-
isional biopsy of the vulva where evaluated, where other
cases examine tissue from complete or partial vulvec-
tomy. Fox et al. [7] describe a tumor originating in the
vagina vault that had similar microscopic appearance as
the tumor described here, with the lesion presenting be-
neath an ulcerated overlying epithelium. Zaidi and Con-
ner [8] reported a case of multiple surface nodules in
direct continuity with the surface epithelium that was
focally ulcerated.
Metastatic adenocarcinoma of the vulva was consid-
ered initially as the lesions had many characteristics of a
tumor derived from enteric origin however this was ex-
cluded as there was no evidence of a previous or concur-
rent primary tumor of similar histological appearance.
Also metastatic spread to the pelvic and inguinal lymph
nodes did not consist with a primary colonic adenocar-
cinoma but was in keeping with a primary carcinoma of
the vulva. Therefore, primary cloacogenic carcinoma was
determined in this case due to morphological appearance
and immune-histochemistry staining of the neoplastic
cells along with pattern of metastatic spread. Neoplastic
cells had a striking resemblance to colonic adenocarci-
noma with the presence of one non-neoplastic gland. The
presence of non-neoplastic glands was remarked by oth-
ers [2,3,6,8].
Primary cloacogenic carcinoma of the vulva is very
rare but it is important for pathologists and clinicians to
be aware of. Any lesion seen on the vulva and perineum
should be biopsied at its earliest and have a pathology
review for diagnosis confirmation. Detailed clinical work-
up and careful histopathological examination using histo-
pathology and immune-histochemistry is necessary to
make the early diagnosis and less radical management. It
is considered to be of indolent nature but in the case of
R. Chibbar et al. / Case Reports in Clinical Medicine 2 (2013) 302-305 305
our case it acted aggressively despite radical treatment.
Therefore, early recognition and wide local excision of
the tumor(s) prior to metastatic spread is important for
favourable prognosis and decreased reoccurrence.
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