Surgical Science, 2011, 2, 52-56
doi:10.4236/ss.2011.22011 Published Online April 2011 (
Copyright © 2011 SciRes. SS
Angiomyolipoma of the Jejunum Mimicking Metastatic
Disease in a Patient with Colonic Adenocarcinoma
Spyridon Miliaras1, Dimosthenis Miliaras2
1Surgical Department , Papageorgiou Hospital, Medical School, Aristotle University, Thessaloniki, Greece
2Laboratory of Histology and Embryology, Med i c a l School, Aristotle University, Thessaloniki, Greece
Received July 27, 2010; revised January 5, 2011; accepted March 9, 2011
Angiomyolipoma is a benign tumor most commonly arising in the kidney. Very few cases have been re-
ported to be located in the small intestine. Here we report the first case located in the jejunum in a patient
who was diagnosed with a colonic adenocarcinoma. In the preoperative evaluation this benign lesion was
thought it might represent a metastatic nodule.
Keywords: Angiomyolipoma, Colon Adenocarcinoma, Jejunum
1. Introduction
Angiomyolipoma (AML) is a benign neoplasm com-
posed of blood vessels, smooth muscle cells, and mature
fat cells [1]. AML most commonly arises in the kidney.
Other locations include the liver (most frequent extrare-
nal site) [2], the uterus[3], the lungs [4], the skin [5], the
subgaleal space [6], the anterior mediastinum [7], the
urinary bladder [8], and mucosal membranes such as the
lips [9] and the nasal cavity [10]. Very few cases have
been reported in the gastrointestinal tract, including nine
cases arising from the colon [11-19], and four cases aris-
ing from the small bowel [20-23]. Two of the latter cases
were located in the duodenum [20,21], and the other two
in the ileum [22,23]. In this paper we report a fifth case
of AML of the small intestine, this time located at the
jejunum. The benign small intestinal tumor was mas-
querading a metastatic nodule in the preoperative imag-
ing evaluation, since our patient also presented a tumor
in the ileocecal region.
2. Case Report
An 80 years old male was urgently admitted to our sur-
gical department with abdominal pain, vomiting and ab-
dominal distension. On clinical examination the patient
was afebrile and the abdomen was moderately distended,
with mild tenderness on palpation of the right iliac fossa,
but no signs of peritonism. Bowel sounds were reduced
and digital examination of the rectum was normal. Plain
abdominal films confirmed small bowel obstruction and
full blood count and biochemistry were unremarkable.
Conservative treatment was initiated, including intrave-
nous fluids, nasogastric suction and monitoring of the
vital organs. After three days of conservative treatment,
although there was no clinical deterioration, the obstruc-
tive ileus was not resolved and a colonoscopy was per-
formed, to further investigate the etiology of obstruction,
which revealed an obstructive tumor of the ileocecal
valve. A preoperative CT scan of the abdomen per-
formed the next day for staging purposes showed re-
gional lymphadenopathy of the mesentery of the ileoce-
cal region, but no hepatic metastases. However, a soft
tissue mass was found in the proximal jejunum with most
probable diagnosis to be metastatic implant (Figure 1(a)).
In order to resolve the persisting ileus, an exploratory
laparotomy was decided and performed through a high
suprasubumbilical incision. A large obstructive tumor of
the ileocecal valve with regional lymphadenopathy were
detected with no signs of hepatic or other metastatic dis-
ease. However, a round, well circumscribed tumor of the
antimesenteric border of the proximal jejunum was de-
tected (Figure 1(b)), which was excised in healthy tis-
sues macroscopically through a limited small bowel en-
terectomy. A typical right hemicolectomy was then per-
formed with ileocolic anastomosis to the transverse colon.
Following an uneventful postoperative period, and after
gradual mobilization and feeding, the patient was dis-
charged home on the seventh postoperative with instruc-
tions for regular follow-up and referral to the medical
Figure 1. (a) CT scan of the abdominal area showing a nod-
ule attached to the jejunum (arrow); (b) The surgical spe-
cimen of the nodule of the jejunum as found at the opera-
tion theater (arrow).
oncology department for the colon cancer. The patient
refused any further treatment, but still today, 12 months
after operation, is alive and well.
2.1. Pathological Findings
The hemicolectomy specimen consisted of a segment of
the terminal segment of ileum 22 cm in length, the ileo-
cecal valve, the cecum along with the appendix, and a
segment of the ascending colon 16 cm in length. A large
exophytic and invasive tumor, 4 cm in diameter was
found in the ascending colon, close to the ileocecal valve.
20 lymph nodes, 0.3-1.2 cm in diameter were removed
from the surrounding pericolic fat. On microscopy this
tumor proved to be a well-differentiated adenocarcinoma
of the large intestine, invading the whole thickness of the
intestinal wall, and extending to the pericolic fat. One of
the removed lymph nodes presented metastasis from the
aforementioned adenocarinoma. The tumor of the jeju-
num was reddish, and soft, 2 cm in diameter. It was
composed by spindle cells arranged in fascicles, vacuo-
lated (fat) cells and many blood vessels (Figure 2(a)).
No significant nuclear atypia, mitotic activity, necroses
or increased cellularity were detected. In order to sustain
a definitive diagnosis, immunohistochemical evaluation
Immunohistochemistry was performed on formalin-
fixed, paraffin-embedded tissue sections, 3-μm in thick-
ness. High temperature antigen unmasking in electric
pressure cooker, and TrilogyTM solution (Cell Marque,
Rocklin, CA, USA) pretreatment preceded the main pro-
cedure. A standardised automated (Nexes, Ventana,
Tuscon, AZ, USA), streptavidin-biotin method (I-VIEW
Paraffin DAB, Ventana) followed the application of the
monoclonal antibodies. The latter were directed against
HMB-45 (dilution 1:50, Dako, Glostrup, Denmark), Me-
lan-A (dilution 1:50, Dako), a-smooth muscle actin
(SMA, dilution 1:40, Novocastra, Newcastle, UK), Des-
min (dilution 1:100, Novocastra), S-100 (dilution 1:300,
Dako), CD117 (dilution 1:50, Dako), and CD34 (dilution
1:50, Dako). The tumor cells were positive to HMB-45
(Figure 2(b)), Melan-A, SMA (Figure 2(c)), and desmin,
while they were negative to CD117, and CD34 (except
of the endothelial cells of the vessels, Figure 2(d)). Our
findings in total were consistent with a diagnosis of an-
3. Discussion
Traditionally, AML was considered as hamartoma.
However, AML today is regarded as a true neoplasm,
since it has been found to present clonal nature [24].
Moreover, AML is thought to arise from the perivascular
epithelioid cell (PEC), which has certain morphologic
and immunohistochemical characteristics, even though
no known normal cell counterpart has been found until
now [1]. Immunohistochemically, PEC expresses myo-
genic and melanocytic markers, such as HMB-45,
HMSA-1, MelanA/Mart1, microophtalmia transcription
factor (Mitf), actin and, less commonly, desmin. Other
(rare) tumors which arise from PEC include clear-cell
“sugar” tumor of the lung and extrapulmonary sites,
lymphangioleiomyomatosis, clear-cell myomelanocytic
tumor of the falciform ligament/ligamentum teres, and
rare clear-cell tumors of other anatomical sites. In addi-
tion, some PEComas are related to the tuberous sclerosis
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(a) (b)
(c) (d)
Figure 2. (a) The tumor is composed of fascicles of spindle cells, interspersed with groups of vacuolated fat cells and numer-
ous blood vessels (H&E, X100); (b) The spindle cells of the lesion are strongly positive to HMB-45 (DAB, Hematoxylin, X100);
(c) The spindle cells of the lesion are strongly positive to smooth muscle actin (DAB, Hematoxylin, X100); (d) Only blood ves-
sels show positive reaction to CD34 (DAB, Hematoxylin, X100).
complex (TSC), an autosomal dominant genetic disease
due to losses of TSC1 (9q34) or TSC2 (16p13.3) genes
[25,26]. This syndrome is characterized by mental retar-
dation, seizures and cellular proliferations (AMLs, sub-
ependymal giant cell tumors, cutaneous angiofibromas,
cardiac rabdomyomas, lymphangioleiomyomatosis,
pulmonary multifocal micronodular hyperplasia). Similar
alterations of the TSC genes have been demonstrated in a
significant number of PEComas, both occurring within
the TSC and in sporadic cases.
Sporadic AMLs occur in older patients, in the fourth
to sixth decades of life, with a female predominance;
they are single, unilateral and larger than those associ-
ated with TSC [27]. Classic AML contains more than
one cell type; if a particular cell type predominates,
AML is consequently named (lipoma-like AML or leio-
myoma-like AML). AMLs arising in the GI tract are
extremely uncommon and usually present with melena,
anemia, diarrhea, abdominal pain, and may even be clini-
cally asymptomatic [11-23]. Radiological diagnosis of
extrarenal AMLs is difficult because of the rarity of the
condition. Even though four other angiomyolipoma cases
have been reported to affect small intestine, our case is
unique, since to our knowledge is the first to involve
jejunum. In addition, in our case the intestinal nodule
was thought to represent metastatic disease in the
pre-operative evaluation, because our patient was having
a colonic tumor as well. So, it represented a critical dif-
ferential diagnostic problem in terms of severity, and
staging of his primary disease. Moreover, we consider
ours to be a genuine AML case, since it is both HMB-45,
and Melan-A positive. At least two cases of small intes-
tinal AML reported in the literature [8,9] were HMB-45
negative (Table 1), when HM-45 positive immureaction B
Table 1. Angiomyolipomas of the small intestine reported in the literature: Immunohistochemical reaction to various anti-
Author SMA Desm KIT CD34 Melan-A HMB-45 S-100 Vim
De Padua et al. + NA NA NA NA + NA NA
Toye and Czarnecki + NA NA NA NA - - NA
Lee et al. + + - + NA - NA NA
Lin et al. + + + + + + NA +
Miliaras and Miliaras + - - - + + - NA
SMA = smooth muscle actin, Desm = desmin, Vim = vimentin, NA = not available.
is currently considered as a prerequisite for such a diag-
nosis [1,14]. In conclusion, intestinal AMLs are very rare,
and may cause various symptoms or mimick other condi-
tions as metastatic disease in our case. For this reason it
is quite important to differentiate such a case from other
mesenchymal small intestinal tumors, and especially
from gastrointestinal stromal tumor, which is the most
frequent tumor in this location and merits specialized
targeted immunotherapy.
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