J Gastrointest Canc DOI 10.1007/s12029-014-9612-0

CASE REPORT

Mesenteric Liposarcoma: Report of Two Cases with Review of Literature Pankaj Kumar Garg & Bhupendra Kumar Jain & Davinder Dahiya & Shuchi Bhatt & Vinod Kumar Arora

# Springer Science+Business Media New York 2014

Introduction

Case Presentations

Liposarcomas are the most common sarcomas of adulthood [1]. They usually arise in the deep soft tissues of the extremities and retroperitoneum. There are five histopathological types of liposarcoma: well differentiated, myxoid, round cell, dedifferentiated, and pleomorphic [2]. They are notorious to grow relentlessly into largesized tumors when they become clinically symptomatic in view of sheer size or compression on adjacent organs. Though liposarcoma are the most common tumors of the retroperitoneum, bowel mesentery is an uncommon site for them. There are a few case reports of mesenteric liposarcoma (ML) in the English literature. There are no standard guidelines for the management of mesenteric liposarcomas in view of rarity of tumor. We present our experience of two cases of dedifferentiated mesenteric liposarcoma and discuss the reported clinicopathological features and employed management strategies.

Case 1

P. K. Garg (*) : B. K. Jain : D. Dahiya Department of Surgery, University College of Medical Sciences and Guru Teg Bahadur Hospital, University of Delhi, Dilshad Garden, Delhi 110095, India e-mail: [email protected] P. K. Garg Department of Surgical Oncology, All India Institute of Medical Sciences, New Delhi, India

A 47-year-old lady presented with progressively increasingly lump in the abdomen of 8 months’ duration and breathlessness of 3 months’ duration. She denied any history of jaundice, diarrhea, constipation, or urinary complaints. Her general physical examination revealed pallor and bilateral pitting pedal edema. Abdominal examination showed a large, firm, non-tender, intraabdominal lump occupying the whole of the abdomen. Contrast-enhanced computed tomography (CECT) scan of the abdomen and pelvis revealed a well-defined heterogeneously hypodense mass in the peritoneal cavity predominantly on the left side; mass contained few hypodense areas of HU-35 suggestive of fat, and there was no calcification or necrotic area (Fig. 1). Exploratory laparotomy with complete excision of the tumor with resection of jejunal segment with primary anastomosis was undertaken. The postoperative period was unremarkable except for superficial surgical site infection. Histopathological examination was dedifferentiated liposarcoma (Fig. 2). She was advised adjuvant chemo-radiotherapy in view of an extremely large tumor size, dedifferentiated pathology, and a high mitotic count (10–12/HPF) to lessen locoregional and systemic relapse. She developed pneumonia with severe sepsis with neutropenia following first cycle of chemotherapy (doxorubicin, cisplatin, and ifosfamide) and succumbed to it.

S. Bhatt Department of Radiology, University College of Medical Sciences and Guru Teg Bahadur Hospital, University of Delhi, Delhi, India

Case 2

V. K. Arora Department of Pathology, University College of Medical Sciences and Guru Teg Bahadur Hospital, University of Delhi, Delhi, India

A 63-year-old man presented with progressively increasing abdominal distention of 4 months’ duration. He denied any history of bowel/urinary complaints. His general physical

J Gastrointest Canc Fig. 1 a Axial section of CT abdomen shows a well-defined heterogeneously hypodense mass in the peritoneal cavity predominantly on the left side; its few hypodense areas of HU-35 are suggestive of fat. There is no calcification or necrotic area. b Sagittal section shows the mass reaching up to the left flank

examination revealed pallor. Abdominal examination showed a large, firm, non-tender intraabdominal lump in the lower half of the abdomen. CECT abdomen and pelvis revealed multiple well-defined heterogeneously hypodense mass in the peritoneal cavity, just posterior to the anterior abdominal wall, not compressing the bowel loops suggesting them to be intraperitoneal (Fig. 3). He underwent exploratory laparotomy with “R0” resection of the tumor. The postoperative period was unremarkable. Histopathological examination confirmed the diagnosis of dedifferentiated liposarcoma (Fig. 4). He was advised adjuvant chemo-radiotherapy in view of multiple large mesenteric tumors, dedifferentiated pathology, and a high mitotic count to lessen loco-regional and systemic relapse; however, he refused adjuvant treatment. He again returned after 1 year with a recurrence. CECT abdomen revealed multiple mesenteric masses. Exploratory laparotomy and resection of multiple mesenteric masses were done. He was again advised adjuvant therapy; however, he did not turn up for follow-up visits.

Fig. 2 (H&E, 100×) Photomicrograph shows dedifferentiated liposarcoma with fascicle of oval to spindle-shaped cells with fibroblastic differentiation. Inset (H&E, 200×) shows lipoblasts with multiple vacuoles

Discussion A Pubmed Search with key words strategy “liposarcoma and mesenter*; filters: English” done on March 3, 2013 yielded only 65 articles; however, a careful review of them revealed only 22 well-described cases of ML. Table 1 displays the clinicopathological features of 13 cases that we could retrieve and two of our cases (total of 15 cases). The mean age of the patients was 58.4± 13.9 years (range 30–70). Male to female ratio was 3:2. The common presentations were presence of abdominal distention (8/15) and abdominal mass (3/15). There was single tumor in 10 cases, while there were multiple in 5 cases of ML. The sites involved were small bowel in 7 patients, large bowel in 4 patients, and both in 2 patients; site was not mentioned in 2 patients. Various histopathological type were dedifferentiated (6/15), well differentiated (5/15), mixed (2/15), myxoid (1/15), and pleomorphic (1/15). Primary modality of treatment in all

J Gastrointest Canc Fig. 3 a Axial section of CT abdomen shows a well-defined heterogeneously hypodense mass in the peritoneal cavity. b Coronal section shows a large mass just posterior to the anterior abdominal wall which is not compressing the bowel loops; it suggests an intraperitoneal mass

patients was surgical excision; 1 patient received adjuvant chemotherapy, and 1 patient received neoadjuvant chemotherapy and postoperative radiotherapy. Five out of nine patients (where recurrence was mentioned in the article) developed recurrence. Liposarcoma is one of the most common soft tissue sarcoma affecting adults. Most common intraabdominal sarcoma is also liposarcoma; however, it rarely involves mesentery of the bowel. The presence of only 22 welldescribed reported cases of ML till March 2013 is a testimony of this fact [1–13]. As mean age of the patients was 58.4 years (30–70), ML involves mainly middle- to old- aged people. Male to female ratio is 3:2; it suggests slight male predominance of ML. As seen in retroperitoneal sarcoma, ML increases in size relentlessly and presents with features of abdominal distension and lump. Rare manifestations of ML in the form of granulocyte colony production-causing leukocytosis has been reported [9]. Though most of the published reports

Fig. 4 Dedifferentiated liposarcoma shows transition between characteristic liposarcoma areas and dedifferentiated fibroblastic areas

did not report duration of symptoms of the patients, a careful analysis of Table 1 indicates a slow-growing nature of tumor (duration of symptoms, range 3 weeks to 1 year). Most of the tumors were of a huge size; a maximum size was seen in our patients (45×35 and 38 × 28 cm); this explains why most of the patients presented with progressive distension of abdomen. ML tend to involve both small and large bowel mesentery equally, and at times both. There are five described histopathological types of liposarcoma: well differentiated, myxoid, round cell, dedifferentiated, and pleomorphic [2]. Table 1 shows that dedifferentiated (6/15) and well-differentiated (5/15) types were the most common histopathological types. It is to be noted from Table 1 that the two patients had more than one type of histopathological picture (mixed type), and there was no case of pleomorphic variety. CECT and magnetic resonance imaging (MRI) plays an important role in the preoperative diagnosis of

Calo et al. [5]

Winn et al. [6]

Hirakoba et al. [7] 65

Ishiguro et al. [8] 30

Nakamura et al. [9] Goel et al. [10]

Yuri et al. [11]

Cha [12]

Benedict [13]

Garg et al. (the 47 present report) 63

5

6

7

8

9

10

11

12

13

14

56

76

73

48

77

59

43

55

61

Abdominal pain

2 months

NS

NS

NS

NS

3 weeks

NS

NS

Female Abdominal 3 months distension Male Abdominal 4 months distension

Abdominal 1 year mass Female Abdominal 3 months mass Female Constipation NS

Male

Male

Female Abdominal distension Male Abdominal distension Female Altered bowel habits Male Abdominal distension Female Abdominal mass Male Abdominal distension Male Pyrexia

Male

Multiple

Single

Single

Single

Single

Single

Multiple

Single

Single

Single

Single

Multiple

Multiple

Single

Multiple

Number (single/ multiple)

a

The patient died on the eighth postoperative day

NACT neoadjuvant chemotherapy, PORT postoperative radiotherapy, NS not specified

15

4

Korukluoglu et al. [3] Khan et al. [4]

3

45

Gupta et al. [2]

2

Duration of symptoms

Abdominal NS distension Abdominal 1 year distension

74

Jukić et al. [1]

1

Male

Age Gender Clinical (years) presentation

S.N. Reference

Table 1 Clinicopathological features of 15 cases of mesenteric liposarcoma

38×28

45×35

NS

13.2×6.5

12.4×9.6

NS

NS

16×13×9

19×17×12

20×16

19

25

30×30×5

35×15×15

Small bowel

Small and large bowel Jejunum

Small bowel

Splenic flexure of colon Duodenum

Ileocaecal

Colon

Sigmoid colon Jejunum

Small bowel

NS

Splenic flexure of colon NS

Small bowel

Size (of largest in case Involved of multiple), cm bowel

Excision

Excision

Excision

Excision

Excision

Excision

NACT followed by excision and PORT Excision

Excision

Excision

Excision and adjuvant chemotherapy

Excision

Excision

Excision

Excision

Treatment

Dedifferentiated

Dedifferentiated

Dedifferentiated

Well differentiated Dedifferentiated

Well differentiated

Pleomorphic

Well differentiated Myxoid

Dedifferentiated

Well differentiated

Mixed type

Dedifferentiated

Well differentiated

Mixed type

No

NS

No

Yes

a

Recurrence

1 year

2 months

6 years

NS

6 months

NS

NS

26 months

NS

Yes

No

Yes

NS

No

NS

NS

Yes

NS

26.5 months Yes

33 months

NS

1 year

5 years

a

Histopathological Follow up type

J Gastrointest Canc

J Gastrointest Canc

liposarcoma; they are of immense value to assess size of the tumor and involvement of adjacent organs [7]. In a review of abdominal fatty masses, Friedman et al. [14] suggested sharply marginated and homogenous abdominal masses that show CT numbers less than or equal to the patient’s normal fat can be considered benign; an extra-renal abdominal fatty mass that displays one or more of the following characteristics: inhomogeneity, infiltration or poor margination, CT numbers greater than the patient’s normal fat, or contrast enhancement, should be suspected to be a malignant neoplasm. In a review of 20 cases of pathologically confirmed extra-parenchymal intraabdominal lipomatous tumors, Waligore et al. [15] reported that the CT appearance (the density) of these tumors closely correlated with their histopathological findings; more importantly, for management point of view, CT accurately conveyed their relationship to adjacent structures. Surgical excision is the recommended primary treatment of localized liposarcoma, whenever feasible; wide excision with negative margins (R0) is the standard surgical procedure. Table 1 also clearly confirms this fact; surgical excision was performed in all the patients. Ishiguro et al. [8] reported success of neoadjuvant chemotherapy (doxorubicin, cisplatin, and ifosfamide) in an initially unresectable colonic ML which could be resected, though R2, postchemotherapy. The patient also received postoperative radiotherapy and remained recurrence-free for 26 months following treatment. Calo et al. [5] reported to give adjuvant chemotherapy (cyclophosphamide, vincristine, adriamycin, and dacarbazine) to a patient with small bowel ML. It is striking to note the high local recurrence rate; five out of nine patients (where recurrence was mentioned in the article) developed local recurrence. This clearly indicates that there is urgent need for research to define the role of adjuvant therapy in ML. We believe adjuvant therapy should be offered to patients having R1 or R2 resection, extremely large or multiple tumors, and highgrade tumors.

Conclusion Surgical excision of mesenteric liposarcoma is the primary modality of treatment; however, role of neoadjuvant and

adjuvant therapies needs to be defined in further studies in view of high local recurrence. Conflict of Interest The authors declare they have no conflict of interest.

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Mesenteric liposarcoma: report of two cases with review of literature.

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