Pathak et al.

Surgical Case Reports (2015) 1:73

DOI 10.1186/s40792-015-0077-6

CASE REPORT Open Access

Omental rhabdomyosarcoma (primary

rhabdoid tumor of greater omentum): a
rare case report
Priyank Pathak* , Mayank Nautiyal, P. K. Sachan and Nadia Shirazi

Abstract
The greater omentum is an uncommon location for primary tumors. Metastatic tumors of the omentum are common.
In contrast, primary tumors of the omentum are very rare. Sporadic cases of primary rhabdomyosarcoma (RMS) arising
in the abdomen have been reported, but these cases are limited almost exclusively to the pediatric population. We
report a well-documented case of primary intra-abdominal RMS in a 21-year-old man who presented with complaints
of abdominal pain and lump in left hypochondrium region. Imaging revealed it to be a large mass in the left
hypochondrium region displacing the bowel loops. Further investigations revealed omental RMS. The mass had
originated from the greater omentum and was excised. Our case is doing well and is presently receiving chemotherapy.
Keywords: Rhabdomyosarcoma; Greater omentum

Background Case report

RMS is an uncommon neoplasm in the adult population. A 21-year-old-man was admitted to our hospital with a
The name is derived from the Greek words rhabdo, 1-month history of abdominal pain. Physical examin-
which means rod shape, and myo, which means muscle. ation revealed a palpable mobile lump in the left hypo-
Sporadic cases of primary RMS arising in the abdomen chondrium region and extending up to the epigastrium
have been reported, but these cases are limited almost region. There is no past history of any surgical interven-
exclusively to the pediatric population. Intraperitoneal tions and any chronic illness. No positive family history
RMS, and in particular with omental involvement, in of any hereditary disease or any carcinoma. No abnor-
any age has been rarely reported in literature. RMS usu- malities in routine blood workup. Ultrasound abdomen
ally manifests as an expanding mass. Tumors in superfi- was suggestive of a well defined but irregular hypoechoic
cial locations may be palpable and detected relatively mass lesion in the left hypogastric region and extending
early, but those in deep locations (e.g., retroperitoneum) into the lumbar region. Colonoscopy was done which
may grow large before causing symptoms. Cells are usu- was negative for any intramural growth and no signs of
ally positive for intermediate filaments and other pro- malignancy. Ultrasound guided FNAC from the left
teins typical of differentiated muscle cells, such as hypochondrium region showed deposits of adenocarcin-
desmin, vimentine, myoglobin, actin, and transcription oma. The abdominal CT revealed a large well defined
factor myoD. Treatment responses and prognoses widely mass lesion in the left hypochondrium, measuring 9.8
vary depending on location and histology. While the op- × 7.4 cms, causing displacement of the bowel loops
timal management of this rare tumor is unknown, early (Fig. 1).
recognition and diagnosis, and a prompt multimodality Intraoperative, a large hard, nodular necrotic mass
treatment approach of surgery, chemotherapy, and encased within the greater omentum present at the
radiotherapy offers the best chance of cure. splenic flexure. Some peri splenic and para aortic lymph
nodes were present which were excised in block (Fig. 2).
* Correspondence: [email protected] The histopathological diagnosis of the patient was re-
Department of General Surgery, Himalayan Institute of Medical Sciences, ported as poorly differentiated malignant round cell
Swami Rama Himalayan University (SRHU), Jollygrant, Doiwala, Dehradun
248140, India

© 2015 Pathak et al. Open Access This article is distributed under the terms of the Creative Commons Attribution 4.0
International License (http://creativecommons.org/licenses/by/4.0/), which permits unrestricted use, distribution, and
reproduction in any medium, provided you give appropriate credit to the original author(s) and the source, provide a link to
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Pathak et al. Surgical Case Reports (2015) 1:73 Page 2 of 4

Fig. 1 Showing a large well defined mass of 9.8 × 7.4 cms along with displacement of the bowel loops

tumor with metastasis in regional lymph nodes and peri- period was uneventful. Postoperatively, upper gastro-
nodal extension. Lymph nodes were free of tumor, and intestinal endoscopy was done which was normal. He
there was only reactive hyperplasia. On cross section was discharged on the third postoperative day. Patient
examination, cut surface was homogenous grayish white underwent PET scan, which turned out to be negative
along with many areas of hemorrhage and necrosis. On for any primary disease or other secondary deposits. Pa-
microscopy, tumor cells have round to mildly pleo- tient is undergoing chemotherapy (4 cycles). Patient is
morphic nuclei, with coarse chromatin and indistinct presently on vincristine, dactinomycin, and ifosfamide
cell borders. Large areas of tumor necrosis are seen regimen (vincristine: 1.5 mg/m2 iv on day 1, dactinomy-
(Fig. 3). cin 0.75 mg/m2 iv on day 1 and 2, mesna prior to ifosfa-
Immunohistochemistry was planned in view of mul- mide 1gm/m2 on day 1 and 2, and then ifosfamide
tiple differential diagnoses in the histopathological re- 1.5gm/m2 iv on day 1 and 2). Patient is doing well and
port. Immunohistochemistry revealed strong staining for has completed all the cycles of chemotherapy. Blood in-
Vimentin, EMA, and desmin, thereby confirming the vestigations done routinely are all within normal limits.
diagnosis for RMS (Figs. 4 and 5). The postoperative Patient has been counseled to come for follow up every

Fig. 2 Intra operative finding of a large omental tumor in the left Fig. 3 H and E; 10 × 10 X shows malignant round cell tumor
hypochondrium region with areas of ulceration arranged in sheets admixed with areas of necrosis
Pathak et al. Surgical Case Reports (2015) 1:73 Page 3 of 4

tumors present as abdominal discomfort (45.5 %), ab-

dominal mass (34.9 %), and abdominal distention
(15.2 %). Unfortunately, there are no specific findings
differentiating the origin or nature of the mass in im-
aging studies due to the extent of the omentum and
the adhered organs. A considerable finding is dis-
placement of the stomach, the transverse colon, and
small bowel, by an extrinsic mass [3].
The treatment of omental tumors is complete excision
via omentectomy. We diagnosed the mass as a malig-
nant rhabdoid tumor based on the typical cellular
morphology and immunohistochemical stains. Immuno-
histochemically, the rhabdoid cells express both cytoker-
atin and vimentin, but not myogenic differentiation nor
INI1 protein [4].
Fig. 4 Immunohistochemistry, 10 × 10 X shows tumor cells showing
desmin positivity For treatment, an aggressive operation to achieve total
resection is recommended because the effectiveness of
chemo radiotherapy has not been proven. The classic
6 months. We do not expect any alterations in the ex- combination of ifosfamide, carboplatinum, and etoposide
pected survival age of the patient. is recommended, and multidrug protocols including
topoisomerase I inhibitors are recommended [5].
Vincristine, actinomycin, and cyclophosphamide (VAC)
Discussion compared with vincristine, Actinomycin, and Cyclophos-
Contrary to metastatic tumors of the omentum, pri- phamide with Vincristine, topotecan, and cyclophospha-
mary tumors of the omentum are very rare and mide (VAC/VTC) for intermediate rhabdomyosarcoma
sporadically reported. However, a primary malignant was done at weeks 3, 9, 21, 27, 33, 39 did not show an im-
rhabdoid tumor in the omentum is very rare. RMS provement in outcome for patients with intermediate-risk
rarely affects adults. AS per the Pubmed survey, only RMS. The estimated 4-year FFS rates were 73 % for VAC
three cases have been reported yet. The rhabdoid and 68 % for VAC/VTC, which did not differ significantly
cells show round to tear drop shape with vesicular [6].
nuclei and a single large nucleolus. There are ill- Radiotherapy plays an important role in the treatment
defined round to oval hyaline inclusions composed of RMS and is a major tool in the treatment of children.
of intermediate filaments in cytoplasm [1]. A cumulative dose of between 36 and 41.4 Gy is gener-
Intraperitoneal RMS, and in particular with omental ally sufficient to control microscopic residual disease,
involvement, in any age has been rarely reported in and doses of between 50.4 and 54 Gy are utilized to con-
literature [2]. In general, the symptoms of omental trol gross residual disease. Patient is to be followed every
3 months for the 1st year, then every 6 months for the
2nd and 3rd year and then yearly after [7].

Conclusions
The diagnosis of tumors in the omentum is very difficult
and requires confirmation by excision and histologic
evaluation. A primary tumor originating in the omentum
is rare but possible even as a malignant tumor. The
prognosis depends mainly on tumor biology yet the ma-
lignancy of the omentum might cause severe outcomes
because these masses can easily metastasize to mesen-
tery and peritoneum tissues.

Consent
Fig. 5 Immunohistochemistry 10 × 10 X shows tumor cells are
Written informed consent was obtained from the patient
negative for pancytokeratin
for publication of this Case report and any
Pathak et al. Surgical Case Reports (2015) 1:73 Page 4 of 4

accompanying images. A copy of the written consent is

available for review by the Editor-in-Chief of this
journal.
Abbreviations
RMS: rhabdomyosarcoma.

Competing interests
The authors declare that they have no competing interests.

Authors’ contributions
MN helped in diagnosis and surgery. PP contributed to data collection, post
operative care, and creating the manuscript. PKS was the operating surgeon
and contributed to the review of the manuscript. NS was the Pathologist
incharge of the case and for provided the microphotographs. All authors
read and approved the final manuscript.

Authors’ information
PP is a junior resident and is currently doing his post graduation. MN is an
assistant professor in general surgery with experience of 5 years. PKS is a
professor and incharge of minimal invasive surgery with experience of more
than 20 years in the college institution and with many prestigious awards to
his name. NS is an associate professor in pathology.

Acknowledgements
This case report could not have been possible without the patient’s consent
and the efforts of the faculty of SRH University for their motivation and
guidance.

Received: 26 February 2015 Accepted: 25 August 2015

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