1130-0108/2016/108/1/43-47
Revista Española de Enfermedades Digestivas
Copyright © 2016 Arán Ediciones, S. L.
Rev Esp Enferm Dig (Madrid)
Vol. 108, N.º 1, pp. 43-47, 2016
CASE REPORT
Pelvic inflammatory myofibroblastic tumor mimicking a rectal cancer
Lídia Roque-Ramos1, António P. Matos2, Pedro Pinto-Marques1, Gabriela Machado3, Joana Nogueira4 and Miguel Ramalho2
Gastroenterology, 2Radiology, 3Surgery, and 4Pathology Departments. Hospital Garcia de Orta. Almada, Portugal
1
ABSTRACT
CASE REPORT
We report a case of a 50-year-old woman who presented to the
emergency department with large bowel obstruction and anemia.
The initial imaging study suggested an inoperable rectal tumor with
involvement of surrounding structures. In this paper, we discuss
the diagnostic work-up of this patient with a diagnosis of pelvic/
perirectal inflammatory myofibroblastic tumor (IMT). IMT is a rare
tumor with intermediate malignant potential that frequently mimics
clinical and imaging features of malignancy. Additionally, to the best
of our knowledge, this is the first case of a pelvic IMT that regressed
without surgical excision.
A 50-year-old woman presented to the emergency
department with lower abdominal pain, fever, anorexia,
progressive constipation and loss of 11 kg for one month.
She had a history of depression and iron deficient chronic anemia and denied smoking and drinking habits. The
patient was medicated with estazolam and oral iron. There
was no family history of cancer.
On physical examination, lower abdomen tenderness
and abdominal distension were noted. Gynecological
examination revealed extrinsic bulging of both anterior and
posterior vaginal sacs, which appeared hard and painful,
with no evidence of mucosal lesions.
The laboratory panel was remarkable for microcytic
anemia (hemoglobin 9.4 g/dl, reference 11.5-18); low
mean corpuscular volume (69.4 fl, reference 76-96), elevated white blood cell (19,800/mm3, reference 4-11,000,
84% neutrophils) and platelet (924,000/mm3, reference
140-400,000) counts and a C-reactive protein of 23.3 mg/
dl (reference < 0.2). The renal function was within the
normal limits. There was no elevation of tumor markers,
including carcinoembryonic antigen and carbohydrate antigen 19.9.
Contrast-enhanced abdominopelvic computed tomography revealed an obstructive transmural rectal tumor,
centered at the right rectum wall, extending anteriorly to
the mesorectum and with ill-defined boundaries with the
uterus and the vaginal dome (Fig. 1).
Fibrosigmoidoscopy revealed a rectal stenosis with normal underlying mucosa and passable with moderate pressure. A pelvic magnetic resonance imaging was ordered
to further characterize the local extent of the tumor and
showed a transmural rectal tumor with 9.5 cm length.
Key words: Inflammatory myofibroblastic tumor. Rectal cancer.
Endoscopic ultrasound. Magnetic resonance imaging.
INTRODUCTION
We report a case of a pelvic/perirectal inflammatory
myofibroblastic (IMT) in which the initial imaging study
suggested an inoperable rectal cancer and illustrate the
challenges of pre-operative diagnosis. In addition, we present the endoscopic ultrasound and fine needle aspiration
findings of this tumor.
IMTs are rare mesenchymal tumors that can involve
virtually any organ, with the lung being the most frequent
location (1). Due to its paucity and misuse of the term
“inflammatory pseudotumor” as synonym for IMTs, its
true prevalence and incidence are unknown (2). According
to the World Health Organization classification IMTs are
considered to have intermediate biological potential due
to the 25% rate of local recurrence and up to 5% of distant
metastasis (3). Surgical resection is usually the definite
treatment option, in rare cases complemented with chemotherapy and/or radiation (4).
Received: 20-03-2015
Accepted: 28-03-2015
Correspondence: Lídia Roque Ramos. Gastroenterology Department. Hospital Garcia de Orta. Avenida Torrado da Silva, 2801-951 Almada, Portugal
e-mail: [email protected]
Roque-Ramos L, Matos AP, Pinto-Marques P, Machado G, Nogueira J, Ramalho M. Pelvic inflammatory myofibroblastic tumor mimicking a rectal cancer.
Rev Esp Enferm Dig 2016;108:43-47.
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L. ROQUE-RAMOS ET AL.
Fig. 1. Post contrast (iopromide [Ultravist 370®]) pelvic axial computed
tomography image (BrightSpeed® 16 slice, GE Healthcare, Milwaukee,
Wisconsin, USA) shows a right-sided heterogeneous and enhancing
tumor (arrows) extending from the rectum with contact with the uterus.
Rev esp enfeRm Dig (maDRiD)
The tumor extensively contacted the posterior wall of the
vagina and uterus, ovaries and sacrum, suggesting direct
invasion of the surrounding structures. Lymphadenopathies were observed in the mesorectum and at the right
internal iliac chain (Fig. 2). Subsequent chest computed
tomography was unremarkable.
Considering the presumed diagnosis of a locally
advanced rectal tumor and the symptomatic bowel obstruction, an emergent derivative colostomy was performed.
Intra-operatively, a rectal tumor adhering to the uterus and
pre-sacral area was observed.
To further characterize this rectal stenosis, an endoscopic ultrasound (Olympus UCT140 AL5) was performed.
The normal five-layered appearance of the rectal wall was
preserved while an ill-defined extrinsic hypoecoic mass
infiltrated the perirectal fat. These features were suspicious
for extrinsic tumor infiltration and fine needle aspiration
with a 19 G procore needle was performed (Fig. 3). The
Fig. 2. Pelvic magnetic resonance imaging (1.5-T, GE-Signa HDxÒ, GE Healthcare) after derivative colostomy. A. Axial contrast-enhanced [gadoterate meglumine
(Dotarem®, Guerbet)] fat-suppressed T1-weighted image shows heterogeneous tumor enhancement with rectal encasement (asterisk), spreading along the
mesorectum and abutting the uterus (arrow). B. Axial T2-weighted image shows a tumor with intermediate signal arising from the right rectal wall. Right-sided mesorectal enlarged lymph nodes are seen (curved arrow). The hipointense line of the mucosa seems preserved, suggesting a deeper origin rather than
the mucosa (arrow). C. Sagittal T2-weighted image shows the longitudinal extent of the tumor, and its relation with the sacrum, vaginal dome and cervix.
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PELVIC INFLAMMATORY MYOFIBROBLASTIC TUMOR MIMICKING A RECTAL CANCER
Fig. 3. Linear endoscopic ultrasound (Olympus UCT 140 AL5) depicted
an ill-defined hypoecoic mass infiltrating the peri-rectal fat. Fine-needle
aspiration was performed with a 19 G procore needle.
cellblock obtained muscle cells and stroma with an exuberant inflammatory cell infiltrate composed of B and T
lymphocytes and histiocytes, consistent with an inflammatory process.
During the follow-up, the patient maintained abdominal pain, low-grade fever and elevated inflammatory
markers, despite being medicated with intravenous cefuroxime and ciprofloxacin. Since a definitive diagnosis
was lacking a second-look surgery was performed, which
revealed a whitish infiltrating mass extending from the
pre-sacral region to the uterus, involving the ovary, rectum and ileo-cecal appendix. Hysterectomy, bilateral salpingo-oophorectomy and appendectomy were performed.
Surgical macrobiopsies revealed a spindle cell proliferation with numerous inflammatory cells, predominantly
lymphocytes and plasma cells, in an eosinophilic collagenized stroma. No atypia, cellular mitoses or necrosis
was observed. Immunohistochemical staining for smooth
muscle actin antibody was positive in the spindle cells.
There was no expression of anaplastic lymphoma kinase
(ALK), CD34, CD117 or S100 (Fig. 4). These findings
were consistent with inflammatory myofibroblastic tumor
in the cellular phase. Additionally, a chronic right-sided
salpingitis with acute inflammatory infiltrate was identified and the sample was negative for fungi and acid fast
bacilli.
Shortly after the procedure the patient became asymptomatic with gradual normalization of laboratory parameters, including hemoglobin (14 g/dl), leucocytes (5,700/
mm3), platelets (291,000/mm3) and C-reactive protein (0.1
mg/dl). Due to the favorable course of the disease and
histopathological findings, the clinical decision was to follow up without further therapy. Bowel reconstruction was
successfully performed. The magnetic resonance imaging
exam repeated after one year showed complete regression
of the pelvic mass (Fig. 5).
Rev esp enfeRm Dig 2016; 108 (1): 43-47
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Fig. 4. Histological examination of the peri-rectal mass: A. The tumor
is composed of a spindle cell proliferation with a collagenized stroma
and a diffuse inflammatory infiltrate, with scattered aggregates (arrow),
and with numerous lymphocytes and plasma cells. B. Some epithelioid
tumor cells with eosinophilic nucleoli and amphophilic cytoplasm can
be seen intermingled with the lymphoplasmacytic inflammation. C. The
tumor has positive immunoreactivity for smooth muscle actin. D. No
immunoreactivity for anaplastic lymphoma kinase protein.
DISCUSSION
Inflammatory pseudotumor (IPT) is a term first used in
1985 and describes the histological appearance of a bland
spindle cell proliferation with prominent inflammatory
infiltrate. Over the last decades it has became clear that
this morphological terminology was broad and included
several clinic-pathological entities with different cell lineage and biological behavior. For this reason some authors
advocate dividing IPT in secondary, when these lesions
are believed to represent a reparative reaction to infection,
inflammation or trauma, and primary or idiopathic when
the etiology is unknown (5). Idiopathic IPT probably represent true IMT, a neoplastic entity that emerged from the
heterogeneous group of IPT for it’s distinct clinical, pathological and molecular features (2). IMT have an intermediate biological behavior and should be distinguished from
benign reactive secondary IPT and malignant tumors with
a prominent inflammatory component such as leiomyosarcomas. Although IMT are more frequent in children and
adolescents they can occur in older patients such as seen in
our patient. Virtually any organ can be involved, with the
most common locations being the lung, abdominopelvic
region and retroperitoneum (2).
Patients may be asymptomatic, particularly when the
tumor is a small solitary mass; present with local mass
effects such as abdominal pain, vomiting, constipation,
chest pain or cough; and/or have systemic manifestations, including fever, weight loss and malaise, reported in 15-30% of cases. Laboratory work-up reflects an
inflammatory process and includes anemia, thrombo-
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L. ROQUE-RAMOS ET AL.
Rev Esp Enferm Dig (Madrid)
Fig. 5. Follow-up pelvic magnetic resonance imaging 2 years after presentation. A. Axial T2-weighted image shows a resolution of the previously described
tumor, with minimal thickening of the rectal wall. B. Sagittal T2-weighted image confirms the macroscopic resolution of the tumor.
cytosis, leucocytosis, increased C-reactive protein and
sedimentation rate, hypoalbuminemia and polyclonal
hypergammaglobulinemia (1,2,5). Imaging features, are
variable and nonspecific and may mimic malignant processes with aggressive features such as mural infiltration
and extravisceral extension (1,6). Indeed, the reported
patient presented with constitutional symptoms and bowel obstruction, as well as increased serum inflammation
markers, anemia and an apparent rectal tumor invading
surrounding organs on both computed tomography and
magnetic resonance imaging.
As in most malignancies the definitive diagnosis of IMT
relies on histology (6). There are only 3 cases of abdominal
IMT reported where endoscopic ultrasound was performed
(7-9). In one case, the IMT was located in the spleen (10),
and fine needle aspiration allowed definite histological
diagnosis. In the remaining two cases, gastric hypoecoic
subepithelial lesions were evaluated with endoscopic ultrasound without fine needle aspiration and the final diagnosis
was obtained after gastrectomy (7) and endoscopic submucosal resection (8). In our case, the endoscopic ultrasound
evaluation demonstrated a preserved five-layer rectal wall
structure, suggesting an extramural process. The fine needle aspiration pointed towards an entity characterized by
the presence of abundant inflammatory cells and stroma.
However, since soft tissue sarcomas can be accompanied
by an inflammatory reaction and the patient had an exuberant clinical presentation, malignancy couldn’t be confidently ruled out and the final diagnosis was obtained after
intra-operative macrobiopsies.
Surgical excision is considered the treatment of choice
(9) and adjuvant therapy with chemotherapy and/or radiation is controversial (4). In our case the infiltrating and
diffuse nature of the pelvic mass precluded a complete
excision and a decision to follow-up was taken. Zhao et
al. recently published a literature review with 38 patients
with abdominal IMT that regressed without surgery, 31%
of whom were treated with NSAIDs, steroids or antibiotics. The liver was the most common location (11). Interestingly, shortly after surgery, our patient became asymptomatic and the serum inflammatory markers normalized.
The magnetic resonance imaging performed one year later
showed complete resolution of the pelvic mass. Regression
without surgical tumor resection is uncommon (1). To the
best of our knowledge this is the first case of a pelvic IMT
that regressed without surgery, with short-term intravenous
antibiotics being the only therapies given considering the
suspicious of infection.
Recurrence rates range from less than 2% in the lung up
to 25% in extrapulmonary locations. Distant metastases
are rare (< 5%) and the most common sites are lung, brain,
liver and bone (2). Prognostic factors are still undefined.
Tumor size and morphologic features are not reliable
prognostic factors, however aneuploidy may indicate a
more aggressive course (3). Rearrangements involving the
ALK locus on chromosome 2p23 have been demonstrated
in approximately 50% of IMTs, based on immunohistochemical analysis. ALK-positive IMTs may have higher
recurrence rates, while ALK-negative IMTs seem to occur
in older patients and have higher metastatic rate (2,12).
In our case, the tumor had two poor predictive factors,
including the infiltrating characteristics at presentation
and negativity for ALK; nevertheless, regression without
surgical resection was observed. At 2 years follow-up the
patient remains clinically well and with normal inflammatory parameters.
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PELVIC INFLAMMATORY MYOFIBROBLASTIC TUMOR MIMICKING A RECTAL CANCER
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