A case of symptomatic mass in the right iliac fossa: a Bermuda

A case of symptomatic mass in
the right iliac fossa
Ann. Ital. Chir.
Published online (EP) 24 February 2014
pii: S2239253X1402101X
www.annitalchir.com
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A Bermuda Triangle which often lies
the right diagnosis
Alessandra Panarese, Daniele Pironi, Stefano Pontone, Maurizio Vendettuoli,
Flaminia De Cristofaro*, Manila Antonelli**, Annamaria Romani, Angelo Filippini
“Sapienza” University of Rome, Italy
Department of Surgical Sciences
*Department of Radiological Sciences
**Department of Radiological, Pathological and Oncological Sciences
A case of symptomatic mass in the right iliac fossa: a Bermuda Triangle wich often lies the right diagnosis
Disease of the iliac fossa can often be accompanied by non-specific symptoms and some of these are exclusively caused by
the compression of bulky masses of other neighboring structures. In young women a differential diagnosis is a non trivial task as several possible causes have to be taken into account. Thus, intraligamentary tumors, which are extremely
rare finding, are frequently confused with uterus, ovary or intestinal tumors. Even if myomas are the most benign tumors
of the female genital tract, broad ligament leiomyomas are an unusual finding in women of reproductive age. These
tumors are often asymptomatic until they reach a volume likely to cause symptoms related to the mass pressure. An accurate patient’s anamnesis and examination serve as a guide to further examinations. Ultrasound is the first line imaging
as it can show ovarian or other pelvic mass and doesn’t involve exposure to radiations in young patients, who can be
pregnant. We describe the clinical presentation and imaging features of a broad ligament leiomyoma, which presented as
an inguinal mass in a patient with a right iliac fossa pain. We also report our diagnostic process performing the differential diagnosis with other potential pathologies of RIF. In these cases, a preoperative disease classification discriminating the benign or malignant tumor nature is closely linked to the proper patient management.
WORDS:
Leyomioma, Pelvic pain, Right iliac fossa, Road ligament tumors
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KEY
Introduction
Several pathologies, that often share non-specific symptoms, may occur against the right iliac fossa (RIF) 1,2
Moreover, in young women, this issue takes on greater
Pervenuto in redazione Dicembre 2012. Accettato per la pubblicazione
Gennaio 2013
Correspondence to: Dr. Stefano Pontone,Department of Surgical Sciences,
“Sapienza” University of Rome, V.le Regina Elena 324, 00161, Rome,
Italy (E-mail [email protected] )
significance considering the numerous structures that are
located in the area between the symphysis pubis, umbilicus and anterior superior spine iliac. For that reason too,
the intraligamentary tumors, which are extremely rare
finding 1, are frequently confused with uterus, ovary or
intestinal tumors. Among these tumors, arising from the
smooth muscle 2, myomas are the most benign tumors
of the female genital tract. While uterine cervix and
intraligamentary tumors are less frequent, the uterine
fibroids ones are the most common myomas 3. Even if
it is generally accepted that most leiomyosarcomas arise
de novo 3 leiomyosarcoma is diagnosed in 0.11-0.49%
of cases women undergoing total hysterectomy for presumed benign leiomyomas 4,5. The broad ligament supPublished online (EP) 24 February 2014 - Ann. Ital. Chir
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A. Panarese, et. al.
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tense signal intensity in 3T Coronal T2 weighted TSE
(Fig. 1). The formation was successfully surgically
removed and histologically examined. Microscopic
analysis revealed that tumor was composed of fascicles
of well-differentiated smooth muscle cells with abundant eosinophilic cytoplasm(Fig. 2). It was adherent to
the right broad ligament of the uterus(Fig. 1). Smoooth
muscle actin immunohistochemistry show positive stain
of neoplastic cells(Fig. 3) and Ki67 immunoistochemistry show low proliferative index (Fig. 4). These features were consistent with the diagnosis of leiomyoma
of the broad ligament. The patient was discharged on
the fifth post-operative day without complications. A
two-year follow-up was negative.
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port structures such as uterus, fallopian tubes and ovaries
anchoring them to the pelvic wall. The broad ligament
leiomyoma is considered as an extrauterine leiomyoma
and an unusual finding in women of reproductive age.
These tumors are often asymptomatic until they reach
a volume likely to cause symptoms related to the mass
pressure. We describe the clinical presentation and
imaging features of a broad ligament leiomyoma, which
presented as an inguinal mass in a patient with a RIF
pain. We also report our diagnostic process performing
the differential diagnosis with other potential pathologies of RIF. In these cases, a preoperative disease classification discriminating the benign or malignant tumor
nature is closely linked to the proper patient management.
Case report
Acute abdominal pain is the most common symptom,
which may lead to admission in an acute general surgical ward. Moreover, quite typically, (i.e. in about 50%
of the cases, cf. Ref.1), pain is localized in the right
iliac fossa. In young women a differential diagnosis is
a non trivial task as several possible causes have to be
taken into account. In fact, there are gastrointestinal
diseases, e.g. appendicitis, carcinoid of the appendix,
Crohn’s disease, diverticular disease, Meckel’s diverticulum, carcinoma of the cecum or ascending colon, right
inguinal or femoral hernia. There are also urinary diseases, e.g. infection or renal colic. Furthermore, the
onset of RIF pain can be due to gynecological diseases,
e.g. pelvic inflammatory disease, salpingitis, pelvic
abscess, ectopic pregnancy, ovarian torsion, ovarian and
pelvic tumors. Among the aforementioned tumors, uterine leiomyoma is the most common neoplasm, that
affects women. Leiomyomas affect 30% of all women
of reproductive age but the incidence of broad ligament leiomyoma is >1% 8.
Mc Cartan et al 2 report that in female patients, aged
between 15 and 50, complaining of RIF pain, the most
common final diagnosis is gynaecological. An accurate
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A 41-year-old female patient, presented with an acute
pain in the RIF, was admitted to our Emergency
Departement in April 2011. The pain, with onset dating to about a year earlier, had been more intense for
two months. She had no significant family history of
gynecological malignancy and no history of oral contraceptive use, she had had two normal pregnancies.
On examination, a round mass was palpated in the
right lower abdomen, causing a severe pain. The laboratory data revealed no abnormality in the tumor
markers carbohydrate antigen (CA) 125 (13.4 UI/ml),
CA19.9 (16.4 UI/ml), Ca15.3 (8.4 UI/ml), carcinoembryonic antigen (CEA)(1.2 ng/ml), alfa-fetoprotein (4.2 ng/ml) and blood tests (Hb 13.2 g/dL; RBC
4.68 x106/µL, WBC 7.05 x103/µL ). A transvaginal
ultrasonography demonstrate a diffuse fibromatosis with
polycystic ovary and a 80 mm diameter solid mass on
the bladder right side. Therefore, the formation was
studied by magnetic resonance imaging (MRI) and
computed tomography (CT). MRI demonstrated a solid formation (8.7x5.5 cm) in the right pelvic cavity,
with regular margins, with inhomogeneously hyperin-
Discussion
Fig. 1: A) 3T MRI Coronal T2-weighted TSE: solid formation in the right pelvic cavity, regular walls, the signal is heterogeneously hyperintense.
Observed relations of contiguity with the uterus, bladder and right colon are observed. B) 3T MRI Coronal T2-weighted TSE. The tumor is close
to the right adnex. C) Post-contrast Axial T1: tumor inhomogeneous contrast enhancement. D) Post-contrast Axial TC: tumor inhomogeneous contrast enhancement.
2
Ann. Ital. Chir - Published online (EP) 24 February 2014
A case of symptomatic mass in the right iliac fossa: a Bermuda Triangle which often lies the right diagnosis
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patient’s anamnesis and examination serve as a guide
to further examinations. Ultrasound (US) is the first
line imaging as it can show ovarian or other pelvic
mass and doesn’t involve exposure to radiations in
young patients, who can be pregnant. CT and MR feature a better imaging resolution and allow to distinguish between appendicitis and acute gynaecological
conditions. In case of smooth muscle tumors CT and
MR enable to also differentiate benign from non benign
tumors. Several studies suggested that malignancy
should be suspected when more than half of an apparent leiomyoma displays hyperintensity on T2-weighted
imaging at MR 6. Our case report sums up all the
characteristics described above. The patient was admitted to the Emergency Department with pain in RIF.
On examination a round mass was palpable.
Ultrasound, CT and MR defined the characteristics of
the mass and allowed an accurate differential diagnosis. Surgery solved the clinical case and enabled
histopathological diagnosis. RIF represents about 50%
of all cases of acute abdominal pain. In young women
diagnosis can be difficult, due to the numerous organs
located in the area between the symphysis pubis,
umbilicus and anterior superior spine iliac.
Gynaecological diseases are the most common ones in
childbearing women. The diagnosis should be supported by instrumental exams, which allow to properly distinguish between benign and malign tumors, as well as
guide the surgical procedure.
Fig. 2: The tumor is composed of fascicles of well-differentiated smooth
muscle cells with abundant eosinophilic cytoplasm.
Riassunto
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Fig. 3: Smoooth muscle actin immunohistochemistry show positive stain
of neoplastic cells.
Fig. 4: Ki67 immunoistochemistry show low proliferative index.
Le patologie occupanti le aree addominali inferiori sono
frequentemente accompagnate da una sintomatologia
aspecifica causata, il più delle volte, da compresione «ab
estrinseco» a carico degli organi contigui. Nelle donne
in età fertile la diagnosi differenziale gioca un ruolo di
primo piano per una corretta gestione del paziente.
Avviene così che alcune tipologie di tumori rari, come
quelli intralegamentari, vengano confusi con tumori di
altra proveninenza (utero, annessi o intestino). Alcuni
tra questi, come il leiomioma del legamento largo,
inusuali nelle donne giovani, insorgano e persistano senza alcuna manifestazione clinica sino al momento in
cui il volume di massa non porti a compressioni nei
confronti di strutture contigue. In questi casi una accurata anamnesi è fondamentale e deve essere sempre
accompagnata da una indagine ecografica che accerti in
maniera mininvasiva i l’origine ed i rapporti delle masse sospette. Vengono di seguito descritti: il percorso diagnostico e gestionale in un raro caso di leiomioma del
legamento largo in una paziente con sintomatologia
dolorosa in fossa iliaca destra. Nel nostro caso la diagnosi differenziale preoperatoria ha consentito un corretto approccio chirurgico discriminando sulla la natura benigna della lesione individuata.
Published online (EP) 24 February 2014 - Ann. Ital. Chir
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A. Panarese, et. al.
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Ann. Ital. Chir - Published online (EP) 24 February 2014

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