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Undifferentiated sarcoma of the liver
The American Journal of Surgery (2009) 198, e7-e9
Clinical Images
Undifferentiated sarcoma of the liver Satoshi Ida, M.D.a, Hideaki Okajima, M.D., Ph.D.b,*, Shintarou Hayashida, M.D.b, Takayuki Takeichi, M.D., Ph.D.b, Katsuhiro Asonuma, M.D., Ph.D.b, Hideo Baba, M.D., Ph.D.a, Yukihiro Inomata, M.D., Ph.D.b a
Department of Gastroenterological Surgery, Graduate School of Medical Sciences, Kumamoto University, Kumamoto, Japan; bDepartment of Pediatric Surgery/Transplantation, Graduate School of Medical Sciences, Kumamoto University, 1-1-1 Honjo, Kumamoto City, Kumamoto 860-8556, Japan
KEYWORDS: Undifferentiated sarcoma of the liver; Trauma; Rupture; Hepatic resection; Chemotherapy
Abstract. Undifferentiated sarcoma of the liver (USL) is one of the rare malignant liver tumors of mesenchymal origin in children and adolescents. USL is difficult to diagnose in its early stages and sometimes the tumor ruptures. We present the case of a 13-year-old boy with USL that ruptured because of abdominal trauma. This was initially diagnosed as a post-traumatic hematoma. A brief review of USL and its treatment principles are discussed. © 2009 Elsevier Inc. All rights reserved.
A 13-year-old boy was hit in the right upper abdomen while playing rugby. He had dull pain at his right upper quadrant and back. Enhanced computed tomography (CT) scan was performed (Figure 1), which showed a well-limited, heterogeneous, low-density mass in the liver measuring 20 cm in diameter. T1-weighted magnetic resonance imaging (MRI) showed a high-intensity, heterogeneous, multilocular mass that was compatible with intrahepatic hematoma secondary to the abdominal trauma (Figure 2). Severe abdominal pain occurred and an abdominal examination showed prominent distention, as well as a huge, well-defined mass that was elastic and hard at the upper abdomen. Abdominal ultrasound showed a heterogeneous hypoechoic mass. Angiography showed a hypovascular mass. Based on these findings, undifferentiated sarcoma of the liver was suspected rather than intrahepatic hematoma. Emergency laparotomy was performed. * Corresponding author. Tel.: ⫹81-96-373-5616; fax: ⫹81-96-3715616. E-mail address: [email protected] Manuscript received May 8, 2008; revised manuscript August 11, 2008
0002-9610/$ - see front matter © 2009 Elsevier Inc. All rights reserved. doi:10.1016/j.amjsurg.2008.08.019
The tumor was located in the right lobe of the liver; part of the tumor had ruptured. Right lobectomy of the liver and resection of the diaphragm where the tumor invaded were performed (Figure 3A). The tumor was a heterogeneous, multilobular cystic mass (Figure 3B) and measured 20 cm ⫻ 20 cm in diameter. Pathological diagnosis confirmed an undifferentiated sarcoma of the liver. The patient received adjuvant chemotherapy for 6 months, with vincristine, actinomycin-D, cyclophosphamide, doxorubicin, and etoposide, beginning 2 weeks postoperatively according to regimen 36 of the Third Intergroup Rhabdomyosarcoma Study (IRS).1 The patient is doing well and has been disease-free for 52 months. Undifferentiated sarcoma of the liver (USL) is a rare malignant hepatic neoplasm of mesenchymal origin that has been recognized as a unique clinicopathologic entity since the series of 31 cases published by Stocker and Ishak in 1978.2 USL accounts for 9% to 13% of pediatric hepatic tumors in various series and occurs primarily in patients between the ages of 5 years and 10 years, with an average of 8 years of age.3–5 The clinical presentation is variable. In Japan, 6 cases have been diagnosed after trauma, including
e8
The American Journal of Surgery, Vol 198, No 1, July 2009
Figure 1
CT findings. Enhanced CT scan showed a well-limited, heterogeneous, multilobular cystic mass in the liver.
our case.4 Hematoma is often diagnosed after trauma. However, based on the cases already reported, it is important to include USL in the differential diagnosis of children.
USL is difficult to diagnose in its early stages and sometimes a rupture of the tumor may be the presenting event. We analyzed details of pediatric USL in 18 patients with tumor rupture.3– 6 Fourteen patients were treated with tumor resection and chemotherapy, 3 patients underwent biopsy followed by chemotherapy, and details were uncertain in 1 patient. Six of 14 were alive without evidence of recurrence, while 8 died of recurrence. In our case, resection for the ruptured USL was followed by systemic chemotherapy. The patient is doing well and has been disease-free for 52 months. In conclusion, USL should be considered as a differential diagnosis in children after abdominal trauma with cystic mass in the liver. Moreover, tumor resection and adequate chemotherapy are effective even after tumor rupture.
References
Figure 2 MRI findings. T1-weighted MRI showed a high-intensity, heterogeneous, multilobular cystic mass.
1. Crist W, Gehan EA, Ragab AH, et al. The Third Intergroup Rhabdomyosarcoma Study. J Clin Oncol 1995;13:610 –30. 2. Stocker JT, Ishak KG. Undifferentiated (embryonal) sarcoma of the liver. Cancer 1978;42:336 – 48.
Figure 3 Operative findings and specimen. (A) Tumor in the right lobe of the liver (arrows) already ruptured. (B) Macroscopic findings of the resected specimen.
S. Ida et al.
Ruptured undifferentiated sarcoma of the liver
3. Bisogno G, Pilz T, Perilongo G, et al. Undifferentiated sarcoma of the liver in childhood. Cancer 2002;94:252–7. 4. Nakamura H, Hirano K, Nakazaki H, et al. Undifferentiated embryonal sarcoma of the liver. Report of an infantile case and a review of 48 cases reported in Japan (in Japanese with English abstract). Nihon Syonigeka Gakkaizasshi (Jpn J Pediatric Surg) 1999;35:237– 45.
e9 5. Uchiyama M, Iwafuchi M, Yagi M, et al. Treatment of ruptured undifferentiated sarcoma of the liver in children: a report of two cases and review of the literature. J Hepatobiliary Pancreat Surg 2001;8:87–91. 6. Te-Yu Hung, Lu D, Min-Chang Liu, et al. Undifferentiated (embryonal) sarcoma of the liver complicated with rupture in a child. J Pediatr Hematol/Oncol 2007;29:63–5.
Clinical Images
Undifferentiated sarcoma of the liver Satoshi Ida, M.D.a, Hideaki Okajima, M.D., Ph.D.b,*, Shintarou Hayashida, M.D.b, Takayuki Takeichi, M.D., Ph.D.b, Katsuhiro Asonuma, M.D., Ph.D.b, Hideo Baba, M.D., Ph.D.a, Yukihiro Inomata, M.D., Ph.D.b a
Department of Gastroenterological Surgery, Graduate School of Medical Sciences, Kumamoto University, Kumamoto, Japan; bDepartment of Pediatric Surgery/Transplantation, Graduate School of Medical Sciences, Kumamoto University, 1-1-1 Honjo, Kumamoto City, Kumamoto 860-8556, Japan
KEYWORDS: Undifferentiated sarcoma of the liver; Trauma; Rupture; Hepatic resection; Chemotherapy
Abstract. Undifferentiated sarcoma of the liver (USL) is one of the rare malignant liver tumors of mesenchymal origin in children and adolescents. USL is difficult to diagnose in its early stages and sometimes the tumor ruptures. We present the case of a 13-year-old boy with USL that ruptured because of abdominal trauma. This was initially diagnosed as a post-traumatic hematoma. A brief review of USL and its treatment principles are discussed. © 2009 Elsevier Inc. All rights reserved.
A 13-year-old boy was hit in the right upper abdomen while playing rugby. He had dull pain at his right upper quadrant and back. Enhanced computed tomography (CT) scan was performed (Figure 1), which showed a well-limited, heterogeneous, low-density mass in the liver measuring 20 cm in diameter. T1-weighted magnetic resonance imaging (MRI) showed a high-intensity, heterogeneous, multilocular mass that was compatible with intrahepatic hematoma secondary to the abdominal trauma (Figure 2). Severe abdominal pain occurred and an abdominal examination showed prominent distention, as well as a huge, well-defined mass that was elastic and hard at the upper abdomen. Abdominal ultrasound showed a heterogeneous hypoechoic mass. Angiography showed a hypovascular mass. Based on these findings, undifferentiated sarcoma of the liver was suspected rather than intrahepatic hematoma. Emergency laparotomy was performed. * Corresponding author. Tel.: ⫹81-96-373-5616; fax: ⫹81-96-3715616. E-mail address: [email protected] Manuscript received May 8, 2008; revised manuscript August 11, 2008
0002-9610/$ - see front matter © 2009 Elsevier Inc. All rights reserved. doi:10.1016/j.amjsurg.2008.08.019
The tumor was located in the right lobe of the liver; part of the tumor had ruptured. Right lobectomy of the liver and resection of the diaphragm where the tumor invaded were performed (Figure 3A). The tumor was a heterogeneous, multilobular cystic mass (Figure 3B) and measured 20 cm ⫻ 20 cm in diameter. Pathological diagnosis confirmed an undifferentiated sarcoma of the liver. The patient received adjuvant chemotherapy for 6 months, with vincristine, actinomycin-D, cyclophosphamide, doxorubicin, and etoposide, beginning 2 weeks postoperatively according to regimen 36 of the Third Intergroup Rhabdomyosarcoma Study (IRS).1 The patient is doing well and has been disease-free for 52 months. Undifferentiated sarcoma of the liver (USL) is a rare malignant hepatic neoplasm of mesenchymal origin that has been recognized as a unique clinicopathologic entity since the series of 31 cases published by Stocker and Ishak in 1978.2 USL accounts for 9% to 13% of pediatric hepatic tumors in various series and occurs primarily in patients between the ages of 5 years and 10 years, with an average of 8 years of age.3–5 The clinical presentation is variable. In Japan, 6 cases have been diagnosed after trauma, including
e8
The American Journal of Surgery, Vol 198, No 1, July 2009
Figure 1
CT findings. Enhanced CT scan showed a well-limited, heterogeneous, multilobular cystic mass in the liver.
our case.4 Hematoma is often diagnosed after trauma. However, based on the cases already reported, it is important to include USL in the differential diagnosis of children.
USL is difficult to diagnose in its early stages and sometimes a rupture of the tumor may be the presenting event. We analyzed details of pediatric USL in 18 patients with tumor rupture.3– 6 Fourteen patients were treated with tumor resection and chemotherapy, 3 patients underwent biopsy followed by chemotherapy, and details were uncertain in 1 patient. Six of 14 were alive without evidence of recurrence, while 8 died of recurrence. In our case, resection for the ruptured USL was followed by systemic chemotherapy. The patient is doing well and has been disease-free for 52 months. In conclusion, USL should be considered as a differential diagnosis in children after abdominal trauma with cystic mass in the liver. Moreover, tumor resection and adequate chemotherapy are effective even after tumor rupture.
References
Figure 2 MRI findings. T1-weighted MRI showed a high-intensity, heterogeneous, multilobular cystic mass.
1. Crist W, Gehan EA, Ragab AH, et al. The Third Intergroup Rhabdomyosarcoma Study. J Clin Oncol 1995;13:610 –30. 2. Stocker JT, Ishak KG. Undifferentiated (embryonal) sarcoma of the liver. Cancer 1978;42:336 – 48.
Figure 3 Operative findings and specimen. (A) Tumor in the right lobe of the liver (arrows) already ruptured. (B) Macroscopic findings of the resected specimen.
S. Ida et al.
Ruptured undifferentiated sarcoma of the liver
3. Bisogno G, Pilz T, Perilongo G, et al. Undifferentiated sarcoma of the liver in childhood. Cancer 2002;94:252–7. 4. Nakamura H, Hirano K, Nakazaki H, et al. Undifferentiated embryonal sarcoma of the liver. Report of an infantile case and a review of 48 cases reported in Japan (in Japanese with English abstract). Nihon Syonigeka Gakkaizasshi (Jpn J Pediatric Surg) 1999;35:237– 45.
e9 5. Uchiyama M, Iwafuchi M, Yagi M, et al. Treatment of ruptured undifferentiated sarcoma of the liver in children: a report of two cases and review of the literature. J Hepatobiliary Pancreat Surg 2001;8:87–91. 6. Te-Yu Hung, Lu D, Min-Chang Liu, et al. Undifferentiated (embryonal) sarcoma of the liver complicated with rupture in a child. J Pediatr Hematol/Oncol 2007;29:63–5.