Journal Name: International Journal of Hepatobiliary … · Chandramohan SM. Embryonal...

Manuscript Accepted Early View Article

of 13

Early View Article: Online published version of an accepted article before publication in the final form.

Journal Name: International Journal of Hepatobiliary and Pancreatic Diseases (IJHPD)

Type of Article: Case Report

Title: Embryonal Rhabdomyosarcoma of liver in a 16 year old male - Rare case report

Authors: Rajamahendran Rajendran, Amudhan A, Prabhakaran R, Kannan D,

Chandramohan SM

doi: To be assigned

Received: 11th August 2014

Accepted: 3rd September 2014

How to cite the article: Rajendran R, Amudhan A, Prabhakaran R, Kannan D, Chandramohan SM. Embryonal Rhabdomyosarcoma of liver in a 16 year old male - Rare case report. International Journal of Hepatobiliary and Pancreatic Diseases (IJHPD). Forthcoming 2014.

Disclaimer: This manuscript has been accepted for publication. This is a pdf file of the Early View Article. The Early View Article is an online published version of an accepted article before publication in the final form. The proof of this manuscript will be sent to the authors for corrections after which this manuscript will undergo content check, copyediting/proofreading and content formatting to conform to journal’s requirements. Please note that during the above publication processes errors in content or presentation may be discovered which will be rectified during manuscript processing. These errors may affect the contents of this manuscript and final published version of this manuscript may be extensively different in content and layout than this Early View Article.


of 13

TYPE OF ARTICLE: Case Report 1

2

TITLE: Embryonal Rhabdomyosarcoma of liver in a 16 year old male - Rare case 3

report 4

5

AUTHORS: 6

Rajamahendran Rajendran1, Amudhan A2, Prabhakaran R3, Kannan D4, 7

Chandramohan SM5 8

9

AFFILIATIONS: 10

1Post Graduate in Surgical Gastroenterology, Institute of Surgical Gastroenterology, 11

Rajiv Gandhi Government General Hospital, Chennai, Tamil Nadu, India. Email ID: 12

[email protected] 13

2Assistant Professor in Surgical Gastroenterology, Institute of Surgical 14

Gastroenterology, Rajiv Gandhi Government General Hospital, Chennai, Tamil 15

Nadu, India. Email ID: [email protected] 16

3Assistant Professor in Surgical Gastroenterology, Institute of Surgical 17



4Professor of Surgical Gastroenterology, Institute of Surgical Gastroenterology, Rajiv 20

Gandhi Government General Hospital, Chennai, Tamil Nadu, India. Email ID: 21

[email protected] 22

5Head of Department of Surgcial Gastroenterology, Institute of Surgical 23



26

CORRESPONDING AUTHOR DETAILS 27

Dr. Rajamahendran Rajendran, 28

No. 245, Tower 2, 4th Floor, Rajiv Gandhi Government General Hospital, Chennai-29

600003, Tamil Nadu, India. 30

Contact Phone Number: 919787387183 31

Contact Email ID: [email protected] 32


of 13

Short Running Title: 33

34

Guarantor of Submission: The corresponding author is the guarantor of 35

submission. 36

37

38

39

40

41

42

43

44

45

46

47

48

49

50

51

52

53

54

55

56

57

58

59

60

61

62

63


of 13


report 65

66

ABSTRACT 67

Introduction: 68

Primitive mesenchymal tumors represent about 9%-15% of all hepatic tumors in 69

children. Only about 150 cases have been reported in the literature so far. Hereby 70

we report a case of Ruptured Embryonal Rhabdomyosarcoma. 71

72

Case Report: 73

A boy aged 16 years old with weight of 26 kg presented to us with sudden onset of 74

abdominal pain and fever. Clinical examination revealed a tender hepatomegaly 75

about 6 cm below Right costal margin. CECT abdomen revealed 15x 11x 9 cm 76

heterogenous multi septated loculated mass lesion involving right lobe of liver 77

involving segments 5,6,7,8. Diagnostic laparoscopy was done which showed a tumor 78

occupying the entire right lobe of liver with no other deposits. The tumor capsule was 79

seen ruptured on the right lateral side for which we did emergency right 80

Hepatectomy. Weight of the specimen was 2.8 kilograms. 81

Histopathology revealed the lesion as Embryonal Rhadomyosarcoma with co-82

existing mesenchymal hamartoma. Immunohistochemistry showed Vimentin and 83

Desmin positive. Patient was on carboplatin and Ifosfamide chemotherapy. Patient 84

expired on 30th postoperative day due to acute respiratory distress. 85

86

Conclusion: 87

Improved survival can be expected only if we detect the disease early. This case 88

report will enlighten this type of rare tumor in young children and an early referral to 89

the specialist. 90

91

Keywords: Primary Embryonal Rhabdomyosarcoma liver, Heterogenous mass in 92

liver, Ruptured capsule, Right Hepatectomy. 93

94


of 13


report 96

97

INTRODUCTION 98

Primary liver tumors rank the third amongst the solid malignant tumors in children 99

following Wilms tumor and Neuroblastoma. They account for 2% of total solid 100

malignancies in paediatric cases [1]. Primitive mesenchymal tumors rank the fourth 101

among the malignant tumors of paediatric age group following Hepatoblastoma, 102

Infantile hemangio endothelioma and Hepatocellular carcinoma. 103

Rhabdomyosarcoma is the malignant solid tumor arising from mesenchymal tissues 104

which normally differentiates to form striated muscle. It is most common tumor in the 105

age group less than 15 years. There are four types of Rhabdomyosarcoma-106

Pleomorphic, Embryonal, Alveolar and Botryoidal. The 5 year survival rate of 107

localised disease is 80% and for metastatic disease- 5% 108

Primitive mesenchymal tumors represent about 9%-15% of all hepatic tumors in 109

children. So far only 150 cases of rhabdomyosarcoma liver have been reported in 110

the literature. Hereby we report a case of ruptured primary Embryonal 111

Rhabdomyosarcoma of liver in a 16 year old male boy. Embryonal type of 112

Rhabdomyosarcoma of liver is very rare and only 12 cases were reported in the 113

literature so far. 114

115

CASE REPORT 116

16 years male with body weight of 26 kilograms presented to us with a mass in the 117

right hypochondrium past 1 month with history of abdominal pain, fever with chills 118

and rigors. He had no history of jaundice, weight loss or loss of appetite. On 119

elaborating the history, the mass was present for the past 1 month and rapidly 120

increased for the past 1 week with severe intolerable pain past 4 days. On clinical 121

examination abdomen was soft and minimal guarding was present. A firm tender 122

heptomegaly was present 6 cm below right costal margin. Surface found to be 123

smooth and edges were rounded. CECT abdomen revealed 15x 11x 9 cm hetero 124

genous multi septated loculated mass lesion involving right lobe of liver involving the 125

segments 5,6,7,8 (Figure 1 and 2). Portal venous Doppler study showed displaced 126


of 13

and stretched right portal vein with normal flow. Laboratory investigations at the time 127

of admission were Hemoglobin 8 gm/dl, Platelets- 4 lakhs/cubic mm, Blood sugar – 128

80 mg/dl, Blood urea- 22 mg/dl and Serum creatinine- 0.8 mg/dl. Two units of 129

packed cells were transfused and the preoperative hemoglobin was 10 mg/dl. Tumor 130

markers AFP and CA 19-9 were done. Alpha fetoprotein value was 4 ng/ml and CA 131

19-9 was found to 20 U/ml. Liver biopsy was not taken to avoid the risk of seedling of 132

an operable tumor. 133

Diagnostic laparoscopy revealed a tumor occupying the segments 5,6,7,8 with 134

ruptured capsule in the right lateral lobe of liver (Figure 3). There was no other 135

peritoneal or omental mets. The left lobe of liver was found normal with other lesions. 136

So we decided to proceed with right hepatectomy. The inflow control was done with 137

Pringles manoeuvre. Parenchymal dissection was done with Harmonic scalpel and 138

Kelly crush clamp technique (Figure 4, 5). The bleeding vessels from raw area in the 139

left lobe were ligated with 3’0 Silk sutures. Specimen retrieved and complete 140

haemostasis was achieved in the remnant liver raw area. Weight of the specimen 141

was 2.8 kilograms (Figure 6). 142

Histopathology showed liver parenchyma with tumor composed of round to 143

polyhedral and spindle shaped cells with bizarre nuclei, arranged in sheets and 144

clusters with vague lobular pattern in some places. Tumor cells inhibit 145

intracytoplasmic globules and multinucleate giant cells with atypical mitosis. 146

Entrapped bile ductules are seen. Stroma shows extensive haemorrhagic areas with 147

areas of necrosis. The above features are suggestive of Embryonal variety of 148

Rhabdomyosarcoma probably coexisting with mesenchymal hamartoma (Figure 7 a, 149

7b), resected margins were free of tumor. 150

IHC markers are positive for Vimentin showed focal positivity, Desmin showed 151

diffuse strong positivity, CK 7 glands showed positivity. CD 34 was found negative. 152

All these confirmed the diagnosis of Embryonal variety of Rhabdomyosarcoma. 1st 153

cycle of chemotherapy started with Ifosfamide at 800 mg/day and Carboplatin at 300 154

mg/day. 155

Patient expired on 30th postoperative day suddenly because of respiratory distress 156

and pleural effusion. Post mortem examination was not done. 157

158


of 13

DISCUSSION 159

Having an incidence of 9-15% in children, Hepatic Rhabdomyosarcoma liver needs a 160

lot of experience to diagnose. Rhadomyosarcoma was classified into four types- 161

Pleomorphic, Alveolar, Embryonal and Botryoidal types. The predominant age of 162

presentation are Pleomorphic type in male adults, Alveolar type in adolescents and 163

young adults, Embryonal type in infants and children and Botryoidal type in young 164

children. The different types of Rhabdomyosarcoma can be differentiated on the 165

basis of histopathological appearance. The tumor cells are more elongated and less 166

densely cellular in Embryonal type. In alveolar type the tumor cells tend to be smaller 167

and rounder, often with a denser cellularity, and are so named because of their 168

resemblance to the appearance of the small air sacs in the lungs (the 169

"alveoli").Rhabdomyosarcomas have very poor prognosis with 5 year survival rate of 170

80% for localised disease and 5% for metastatic disease. 171

These cases have a great challenge in treatment because of the rarity of the disease 172

and absence of clinical trials and standard treatment guidelines. A lot of case reports 173

published showed that most patients expired within 5 years of the diagnosis. 174

Neoadjuvant chemotherapy was found to extend the survival of these patients [5]. 175

Postoperative chemotherapy is advised for all patients. Various chemotherapy 176

regimens are applied for these patients like Vinorelbine and Cyclophosphamide, 177

Gemcitabine and Docetaxel, Rapamycin, Topotecan and Vincristine [3]. Our patient 178

received Ifosfamide and carboplatin for 1 cycle, but unfortunately patient suddenly 179

died of acute respiratory distress and pleural effusion. 180

With this case report we would like to stress the aggressive nature of this disease 181

and the necessity for early detection of the disease. A multidisciplinary approach is 182

needed in managing these patients as there is no protocol available. An extensive 183

work-up is essential because these tumours have the potential to grow rapidly. PET 184

CT scan may be of beneficial in these patients before surgery [2] and may help in 185

diagnosing the metastasis before taking up for hepatectomy. 186

187

188

189

190


of 13

CONCLUSION 191

The poor prognosis and early death of most cases reported so far implies the 192

necessity of early detection and evaluation of the disease. Surgical resection along 193

with chemotherapy provides improved survival only if given in the early stage of the 194

disease. 195

196

CONFLICT OF INTEREST 197

Authors declare no conflict of interest 198

199

REFERENCES 200

1. Hepatic rhabdomyosarcoma in an adult: A rare primary malignant liver tumor. 201

Case report and literature review. Schoofs G, Braeye L, Vanheste R, 202

Verswijvel G, Debiec-Rychter M, Sciot R. Acta Gastroenterol Belg 2011 203

Dec;74(4):576-81. 204

2. Primary embryonal rhabdomyosarcoma of the liver in a young male. Haider N, 205

Nadim MS, Piracha MN. J Coll Physicians Surg Pak 2013 Oct;23(10):750-1. 206

3. Management of undifferentiated embryonal sarcoma of the liver in children: A 207

case series and management review. Geel JA, Loveland JA, Pitcher GJ, 208

Beale P, Kotzen J, Poole JE. S Afr Med J 2013 Jun 27;103(10):728-31. 209

4. Undifferentiated Embryonal sarcoma of liver- Combination treatment by 210

surgery and chemotherapy. Dae-Yeon kim, Ki- Hong Kim, Seoul Korea, 211

presented in 34th annual meeting of pacific associations of paediatric 212

surgeons, Japan. 213

5. Almogy G, Lieberman S, Gips M, Pappo O, Edden Y, Jurim O, Simon Slasky 214

B, Uzieli B, Eid A: Surgical outcomes of surgical resections for primary liver 215

sarcomas in adults: Results from a single center. Eur J Surg Oncol 216

2004;30:421-427. 217

218

TABLES 219

NIL 220

221

222


of 13

FIGURE LEGENDS 223

Figure 1: CECT abdomen showing heterogenous mass lesion in right lobe of liver. 224

Figure 2: Coronal view of CECT abdomen showing the lesion occupying the entire 225

right lobe. 226

Figure 3: Intraop picture showing ruptured capsule of tumor. 227

Figure 4: Rhabdomyosarcoma – During surgery – Tying the Falciparum ligament. 228

Figure 5: Parenchymal resection using Harmonic Scalpel. 229

Figure 6: Post operative specimen of Rhabdomyosarcoma liver. 230

Figure 7: (a) HPE showing pleomorphic nuclei and spindle cells. (b) HPE showing 231

liver parenchyma with mitotic figures. 232

233

FIGURES 234

235

Figure 1: CECT abdomen showing heterogenous mass lesion in right lobe of liver. 236

237

238


of 13

239

Figure 2: Coronal view of CECT abdomen showing the lesion occupying the entire 240

right lobe. 241

242

243


of 13

244

Figure 3: Intraop picture showing ruptured capsule of tumor. 245

246

247

Figure 4: Rhabdomyosarcoma – During surgery – Tying the Falciparum ligament. 248

249

250


of 13

251

Figure 5: Parenchymal resection using Harmonic Scalpel. 252

253

254

Figure 6: Post operative specimen of Rhabdomyosarcoma liver. 255

256


of 13

a 257

b 258

Figure 7: (a) HPE showing pleomorphic nuclei and spindle cells. (b) HPE showing 259

liver parenchyma with mitotic figures. 260

Journal Name: International Journal of Hepatobiliary … · Chandramohan SM. Embryonal...

Documents

Transcript of Journal Name: International Journal of Hepatobiliary … · Chandramohan SM. Embryonal...