Hepatoid carcinoma colliding with a liposarcoma of the left colon serosa presenting as an abdominal mass.

Abstract

Hepatoid adenocarcinoma (HAC) is a peculiar type of extrahepatic adenocarcinoma generally characterized by adenocarcinomatous and hepatocellular carcinoma (HCC)-like foci. Stomach is the most frequent site where hepatoid adenocarcinoma occurs, although it has been described in many other organs. On the other side, liposarcoma is a rare, malignant tumor that develops from fat cells.
We describe here a case of hepatoid carcinoma in collision with a liposarcoma of the left colon serosa in a 71-year-old man. It presented as an abdominal mass involving several organs, falsely mimicking metastatic colonic adenocarcinoma. Recognition of this entity was evident on microscopic evaluation following surgery. The patient had an objective response following liposomal antracycline chemotherapy, with a 3-year overall survival.
To our knowledge, this is the first case of a hepatoid tumor colliding with a liposarcoma of the left colon serosa reported to date.

Full text

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World Journal of Surgical Oncology
Open Access
Case report
Hepatoid carcinoma colliding with a liposarcoma of the left colon
serosa presenting as an abdominal mass
Michele Orditura*2,5, Eva Lieto3, Francesca Ferraraccio4, Giuseppe De
Cataldis1, Teresa Troiani2, Paolo Castellano3, Giuseppe Catalano2,
Fortunato Ciardiello2, Gennaro Galizia3 and Ferdinando De Vita2
Address: 1Dipartimento Medico-Chirurgico di Internistica Clinica e Sperimentale "F. Magrassi – A. Lanzara" Naples, Italy, 2Cattedra di Oncologia
Medica, Seconda Università degli Studi di Napoli °Ospedale "Da Procida", Salerno, Naples, Italy, 3Cattedra di Chirurgia Generale ed Epatobiliare,
Seconda Università degli Studi di Napoli Ospedale "Da Procida", Salerno, Naples, Italy, 4Cattedra di Anatomia e Istologia Patologica – Seconda
Università degli Studi di Napoli °Ospedale "Da Procida", Salerno, Naples, Italy and 5Department of Clinical and Experimental Medicine, Second
University of Naples School of Medicine, Naples, Italy c/o II Policlinico Via S. Pansini, 5 80131, Naples, Italy
Email: Michele Orditura* - michele.orditura@unina2.it; Eva Lieto - eva.lieto@unina2.it; Francesca Ferraraccio - francesca.ferraraccio@unina2.it;
Giuseppe De Cataldis - gdecataldis@libero.it; Teresa Troiani - teresa.troiani@unina2.it; Paolo Castellano - paolo.castellano@unina2.it;
Giuseppe Catalano - giuseppe.catalano@unina2.it; Fortunato Ciardiello - fortunato.ciardiello@unina2.it;
Gennaro Galizia - gennaro.galizia@unina2.it; Ferdinando De Vita - ferdinando.devita@unina2.it
* Corresponding author
Abstract
Background: Hepatoid adenocarcinoma (HAC) is a peculiar type of extrahepatic adenocarcinoma
generally characterized by adenocarcinomatous and hepatocellular carcinoma (HCC)-like foci.
Stomach is the most frequent site where hepatoid adenocarcinoma occurs, although it has been
described in many other organs. On the other side, liposarcoma is a rare, malignant tumor that
develops from fat cells.
Case presentation: We describe here a case of hepatoid carcinoma in collision with a
liposarcoma of the left colon serosa in a 71-year-old man. It presented as an abdominal mass
involving several organs, falsely mimicking metastatic colonic adenocarcinoma. Recognition of this
entity was evident on microscopic evaluation following surgery. The patient had an objective
response following liposomal antracycline chemotherapy, with a 3-year overall survival.
Conclusion: To our knowledge, this is the first case of a hepatoid tumor colliding with a
liposarcoma of the left colon serosa reported to date.
Background
Hepatoid adenocarcinoma (HAC) is a type of extrahepatic
adenocarcinoma, which shows a striking morphologic
similarity to hepatocellular carcinoma. It is generally char-
acterized by adenocarcinomatous and hepatocellular car-
cinoma (HCC)-like foci. This variant of adenocarcinoma
has been demonstrated to be an alpha-fetoprotein (AFP)-
producing carcinoma arising in extra hepatic organs, and
it mimics hepatocellular carcinoma in morphological and
functional terms.
In most instances, hepatoid carcinoma has been found in
the stomach [1,2], but it also occurs in many other organs.
Recently, HAC occurrence has been reported in such
Published: 22 April 2007
World Journal of Surgical Oncology 2007, 5:42 doi:10.1186/1477-7819-5-42
Received: 14 November 2006
Accepted: 22 April 2007
This article is available from: http://www.wjso.com/content/5/1/42
© 2007 Orditura et al; licensee BioMed Central Ltd.
This is an Open Access article distributed under the terms of the Creative Commons Attribution License (http://creativecommons.org/licenses/by/2.0),
which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.

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organs as ileum, gallbladder [3], ovary [4,5], pancreas
[6,7], lung [8,9], renal pelvis [10], uterus [11], and urinary
bladder [12].
A prompt and accurate diagnosis of hepatoid adenocarci-
noma is important because prognosis is very poor com-
pared with that of both
adenocarcinoma [13] and AFP-producing adenocarcino-
mas [14].
common types of
Liposarcoma is a rare, malignant tumor that develops
from fat cells. It is the most common soft tissue sarcoma
and accounts for 20% of all malignant mesenchymal
tumors. This tumor typically appears in the deep fat tis-
sues of the thigh or abdomen in people aged 50 to 70.
Prognosis varies depending on site of origin, type of can-
cer cell, tumor size, depth, and proximity to lymph nodes.
Metastases are common and the 5-year survival rate for a
deep and high-grade liposarcoma is less than 50%. This
paper describes the unusual occurrence of a hepatoid car-
cinoma colliding with a liposarcoma.
Case presentation
A 71-year-old man was admitted because of abdominal
pain in January 2003. His past medical history was signif-
icant for a monoclonal gammopathy and surgery for hip
prosthesis 2 years before the current admission. At
another hospital an abdominal computed tomography
(CT) scan had revealed a left-sided mass (10 × 9 cm)
involving the diaphragm, spleen, and, from the outside,
the left colon flexure. Tumor marker (CEA and CA19-9)
serum levels were normal, with the exception of serum
AFP, which was markedly elevated (44074.6 ng/ml).
However, no CT evidence of primary hepatocellular carci-
noma was found. Endoscopy of the upper and lower gas-
trointestinal tract did not show any primary tumor. A
FNAB was performed and cytologic analysis was consist-
ent with a moderately differentiated adenocarcinoma.
Since surgery was deemed not to be feasible, 5-fluorour-
acil and oxaliplatin-based chemotherapy was adminis-
tered for a total of 6 cycles, resulting in stable disease.
CT scan before the treatment showing the mass lesion
Figure 1
CT scan before the treatment showing the mass lesion.

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As the abdominal mass was shown to have slightly shrunk
(Figure 1), a laparotomy was scheduled with the aim to
perform a radical operation in July 2003. Unfortunately,
along with spleen and left colon, the tumor was found to
infiltrate a large part of the left diaphragm thus making it
difficult to remove the entire mass. A segmental resection
of the left colon (left transverse colon, splenic flexure, and
proximal part of the descendent colon) along with
splenectomy was performed. A small part of the tumor
was left in place (figure 2).
The gross appearance of the operated specimen was that
of a lobulated, compact, yellow-greenish mass immersed
in an adipose-like stromal tissue. Included in the patho-
logical evaluation were also the spleen (12.5 × 6.0 cm), a
7-cm intestinal tract, and tissue samples from colon serosa
(6.0 cm), omentum (19 × 18 cm), diaphragm (4.5 × 3.0
cm), peritoneum (8.5 × 3.5 cm), transverse mesocolon
(2.5 × 2.0 cm), and pancreatic tail lymph nodes. The
spleen had an irregular surface due to the presence of mul-
tiple, small gray-whitish nodules, which were also
observed in the context of the other sampled tissues. The
maximum diameter of the nodules was 2.5 cm. Micro-
scopically, each nodule appeared to be made of single or
multiple layers of cells, sometimes with branchlike divi-
sions or in islet-like configuration. Cells in the nodules
had polygonal appearance, acidophilic cytoplasm, and
large, single or multiple hyperchromatic nuclei, often
with vacuolization. These cells stained positive for AFP on
immunohistochemistry (Figure 3). In addition, features
of well-differentiated liposarcoma were found on colon
serosa. In particular, lobules of adipocytes with large and
vacuolized cytoplasm were observed. At higher magnifica-
tion, interspersed lipoblasts with large, hyperchromatic
nuclei could be identified. On immunohistochemistry,
lipoma-like cells stained positive for S100, vimentin and
calretinin but not for p53, p21 WaF1, and c-kit, indicating
a well-differentiated liposarcoma.
Finally, a collision zone between the two tumors was
identified, consisting of a large area infiltrated by islets or
layers of AFP-positive, acidophilic, polygonal cells in con-
CT scan after the treatment
Figure 2
CT scan after the treatment.

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tiguity and continuity with islets of lipoblast-rich fat tissue
(well-differentiated liposarcoma).
The serum AFP level decreased after surgery (9000 ng/ml).
The patient received first line chemotherapy with lipo-
somal doxorubicin for 8 cycles resulting in disease control
for 2 years. The patient then had hepatic metastases and
died 3 years after the first diagnosis.
Discussion
The unusual presentation of two entirely different primary
malignancies in close proximity to each other is defined as
a "collision tumor". To our knowledge, this is the first case
of a hepatoid tumor colliding with a liposarcoma of the
left colon serosa reported to date. This collision tumor
presented as an abdominal mass with extensive involve-
ment of abdominal organs, thus falsely mimicking meta-
static dedifferentiated colonic adenocarcinoma. In the
absence of a primary identifiable liver disease, this was
consistent with a hepatoid carcinoma arising from the
peritoneum. The dual nature of the mass was revealed on
histologic analysis; it has to be stressed that the two
tumors collided at a clear boundary with no evidence of
transition both grossly and microscopically; in addition,
the immunohistochemical staining pattern for AFP was
quite different between the two tumors [[2,9] 15]. In cases
such this one, it would be interesting to find out whether
these are true collision tumors or single neoplasms with
divergent differentiation. However, this was beyond the
scope of this report in our case because of the need of
complex molecular analyses. Finally, it is uncommon for
a liposarcoma to respond to antracycline-based chemo-
therapy, given its chemo-resistance. A further peculiarity
of this case was the unusual presentation of the hepatoid
carcinoma, which showed itself as an abdominal extraco-
lonic mass, whereas in other reported cases it would arise
in such definite organs as the ovary, the lung or the blad-
der.
Conclusion
To data, this is the first case of hepatoid carcinoma collid-
ing with a liposarcoma of the left colon serosa. The unu-
sually good response of this tumor to chemotherapy is
worth reporting, especially because systemic chemother-
apy is considered little or non effective. Although this is a
single case report, patient's response shows that lipo-
somial antracycline therapy is possible for this type of
tumor.
Competing interests
The author(s) declare that they have no competing inter-
ests.
Authors' contributions
MO: Primary author in producing and revising the manu-
script. TT, GC, FDV: carried out second line chemotherapy
and provided data about follow-up of the patient; Helped
to draft the manuscript
FC: helped in scrutiny of manuscript. EL, PC, GG: carried
out surgical procedure and provided data about surgical
procedure. GDC: carried out the first line of chemother-
apy. All authors read and approved the final manuscript.
Acknowledgements
The written consent of the patient was obtained for publication of this case
report.
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