This is the report of a case of primary leiomyosarcoma of the small intestine, an uncommon primary malignancy of the small intestine

Bafna, UD

Ganguly, S

Guruprasad, B

Rajshekar, S

Rathod, P

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Metastatic Leiomyosarcoma of Small Intestine

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1 
 
  
 
 
Case Report: 
Metastatic Leiomyosarcoma of Small Intestine 
 
Authors 
Rajshekar SK,Department of Gynaec-Oncology, 
Guruprasad B, Post Graduate Trainee, Department of Medical Oncology, 
Sandip Ganguly, Post Graduate Trainee, Department of Medical Oncology, 
Praveen Rathod, Asst Professor, Department of Gynaec-Oncology, 
Bafna UD, Professor and Head, Department of Gynaec-Oncology, 
Kidwai Memorial Iinstitute of Oncology, Bangalre, India. 
 
Address for Correspondence 
Dr Guruprasad B 
PG Hostel Room No 108, 
Kidwai Memorial Institute of Oncology, 
Dr MH Marigowda Road, 
Bangalore - 560029, 
India.  
E-mail: guru02doc@yahoo.co.in 
 
Citation 
Rajshekar S, Guruprasad B, Ganguly S, Rathod P, Bafna UD. Metastatic Leiomyosarcoma of Small Intestine. Online J Health Allied 
Scs. 2013;12(2):18 Available at URL: http://www.ojhas.org/issue46/2013-2-18.html 
 
Open Access Archives 
http://cogprints.org/view/subjects/OJHAS.html 
http://openmed.nic.in/view/subjects/ojhas.html 
Submitted: Jan 4, 2013; Accepted: Jun 2, 2013; Published: Aug 25, 2013 
 
Abstract: This is the report of a case of primary 
leiomyosarcoma of the small intestine, an uncommon 
primary malignancy of the small intestine.  
Key Words: Leiomyosarcoma; Gastro intestinal stromal 
tumor 
 
Introduction 
Small intestine represents 75% of total length of the 
gastrointestinal tract, yet rarely does this region develop 
malignant tumors. Approximately 2% of malignancies in the 
gastrointestinal tract arise from small intestine.(1) Prior to the 
days of immunohistochemistry, leiomyosarcomas used to be 
diagnosed abundantly. However with the advent of 
immunohistochemistry, many leiomyosarcomas are now 
being diagnosed as Gastro intestinal stromal tumor (GIST). 
At present leiomyosarcomas are uncommon and comprise 
only 3% of all primary malignancies in the small intestine.(2) 
Case Report 
A 53 years old multiparous woman presented in the hospital 
with dull aching lower abdominal pain of one week duration. 
The pain was intermittent without any specific aggravating or 
relieving factors. She had undergone total abdominal 
hysterectomy (TAH) one year back in view of 
dysmenorrhoea. Histopathological examination revealed 
multiple leiomyomas of fibroid with chronic endocervicitis. 
She didn’t have any comorbid illness and her family history 
was insignificant. 
On examination, her performance status was 1(ECOG scale). 
General physical examination was unremarkable. Per 
abdominal examination revealed no obvious mass. Pelvic 
examination however revealed a mass measuring about 
10x10x15 cm in right adnexa with extension into the recto 
sigmoid region. Bilateral parametrium were free. Rest of the 
systemic examinations were within normal limits. 
Labs reveal a mildly elevated CA-125 level of 63 IU/ml, 
while CEA was within normal limits. Rest of the blood 
parameters were normal. Computed tomography (CT) scan 
revealed a large solid mass (Figure 1) is seen in pelvis more 
on right side and posterior to urinary bladder. Mass measured 
9.5x9.2 cm with one of the ileal loop is seen adherent to the 
mass. The mass showed heterogenous enhancement and 
small cystic components. There was a small separate nodule 
just superior to the mass on the left side measuring 3.2x3.5 
cm adherent to the bladder. 
A provisional diagnosis of ovarian neoplasm was made and 
she underwent laparotomy. Intra operative findings revealed 
12x10x10 cm solid vascular mass arising from the serosal 
surface of terminal ileum with multiple peritoneal deposits. 
There were tumor deposits in right ovary with rest of the 
ovary being normal. Rest of the findings were essentially 
being normal. The mass was resected following which ileal 
anastomosis was made. She also underwent bilateral 
oophorectomy. 
Histopathological examination revealed high grade spindle 
cell neoplasia with foci of haemorrhage, necrosis and high 
mitotic activity (>10/hpf) and infiltrating into the ileal 
mucosa. Foci of pleomorphic cells were also noted. The 
tumor margin was free of tumor. Right ovary shows tumor 
deposits and fallopian tube was normal. In view of spindle 
cells, immunohistochemistry was advised. 
Immunohistochemistry studies revealed the neoplastic cells 
were positive for desmin, caldesmin and smooth muscle 
antigen (Figure 2). However the tumor cells were negative 
for CD 117 (Figure 3), DOG 1, S100, CD 34 and calretinin. 
Ki-67 showed high positivity of 80%. Features were 
consistent with leiomyosarcoma of the wall of the ileum. 
She was treated with adjuvant chemotherapy (Docetaxel 
80mg/m2 and gemcitabine 1 gm/m2). She received 5 cycles 
of chemotherapy till date and at present is asymptomatic. 
 
This work is licensed under a 
Creative Commons Attribution- 
No Derivative Works 2.5 India License 
Online Journal of Health and Allied Sciences 
Peer Reviewed, Open Access, Free Online Journal 
Published Quarterly :  Mangalore, South India : ISSN 0972-5997 
Volume 12, Issue 2; Apr-Jun 2013 
2 
 
 
Figure 1: CT scan showing tumor arising from small 
intestine 
 
Figure 2: Immunohistochemistry showing smooth muscle 
antigen positivity 
 
Figure 3: CD117 Negative Tumor 
Discussion 
Leiomyosarcoma are rare tumors of small intestine which 
arise from the muscualris mucosa or muscularis propria. The 
common presentations include abdominal mass, abdominal 
pain or overt gastrointestinal bleeding. (3) The most common 
site of LMS in the small intestine is jejunum followed by 
ileum and then duodenum. They are mainly seen in 6th 
decade of life with slight male preponderance. (4-5) 
Preoperative diagnosis of small intestinal tumors is difficult, 
especially differentiating between benign and malignant 
tumors. For LMS in small intestine, recent review of 
literature revealed that CT- and MRI-enterography and 
enteroclysis are good options.(6) Cases of superficial lesion 
which can be missed by both CT and MRI, can however be 
detected by water capsule endoscopy with a detection rate of 
around 80%.(7) 
Histologically LMS resembles like GIST, however they are 
CD117 and CD34 negative by immunohistochemistry and 
positive for smooth muscle antigen (SMA) and desmin.(8) 
When these tumors are more than 5 cm they commonly 
spread hematogenously to liver (65%), other GI organs 
(15%), lung(4%). It also has the capability to spread via 
lymphatics (13%) or via peritoneal route (18%). (9) 
The only effective treatment for small intestine LMS is 
surgery. The primary tumor should be excised radically, 
including a wide resection of the mesentry. Response to 
chemotherapy is doubtful, and there is no role for 
radiotherapy.  Therefore, metastasectomy, if possible, should 
be considered.  Large phase II and III studies combining 
docetaxel and gemcitabine yielded impressive response rates 
in LMSs (mostly of uterine origin). However, others were not 
able to confirm the efficacy of this combination. Recently, 
trabectedin showed response rates up to 56% in LMSs, and it 
appeared to be especially useful in far-advanced and 
metastatic LMSs after failure of the combination of 
anthracyclines and ifosfamide. (10) 
Prognosis of non metastatic small bowel LMS depends upon 
its mitotic grade, with low grade tumors  having a 5 yr 
survival of 55% while high grade variants have a 5 yr 
survival of 5-20%. Outcome of metastatic small bowel LMS 
is mostly poor. The present case highlights the need for high 
index of suspicion for diagnosis of this rare disease and need 
of adequate surgery even when metastatic at presentation for 
better management of these patients 
Acknowledgements: 
We sincerely thank Dr K C Lakshmaiah, Head of Medical 
Oncology department, Dr Clementina Rama Rao, Professor 
of Pathology and Dr Shakuntala PN, Department of Gynaec-
Oncolgy, Kidwai memorial Institute of Oncology, Bangalore, 
for their kind help in preparing this manuscript. 
References 
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4. Akwari OE, Dozois RR, Weiland LH, Beahrs OH. 
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5. Evans HL. Smooth muscle tumors of the gastrointestinal tract: a 
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Metastatic Leiomyosarcoma of Small Intestine

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