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Primary mesenteric rhabdomyosarcoma in paediatric patient
Radiography xxx (2015) 1e3
Contents lists available at ScienceDirect
Radiography journal homepage: www.elsevier.com/locate/radi
Case report
Primary mesenteric rhabdomyosarcoma in paediatric patient Alberto Martínez-Martínez*, Jade García-Espinosa, Elena Pastor Pons Diagnostic Imaging Department, “Virgen de las Nieves” University Hospital, Granada, Spain
a r t i c l e i n f o
a b s t r a c t
Article history: Received 19 January 2015 Received in revised form 26 May 2015 Accepted 27 May 2015 Available online xxx
Primary mesenteric rhabdomyosarcoma is an exceptional entity in adult and paediatric patients. We report and discuss the imaging findings in a 2 year-old child with an abdominal mass. Our case showed similar imaging findings to rhabdomyosarcoma in other locations. © 2015 The College of Radiographers. Published by Elsevier Ltd. All rights reserved.
Keywords: Rhabdomyosarcoma Imaging Magnetic resonance imaging Pediatrics
Introduction Rhabdomyosarcoma (RMS) is the most common soft tissue sarcoma of childhood, representing 3e5% of all paediatric cancers.1 It arises from the same immature mesenchymal cells committed to skeletal muscle differentiation and can occur in many tissues, including those that lack striated muscle. There are very few cases described in mesenteric location. For its extreme rarity we report the case of a primary embryonal RMS in the mesentery. Case report A two year old male child with no relevant medical history was brought to the emergency department with symptoms of abdominal pain of 3 days duration and temperature of 39 C without leukocytosis and normal urinary sediment. Suspecting septic urinary focus, abdominal ultrasound was requested which revealed the existence of a large and hetero-echoic abdominal mass with significantly increased flow and hypo-echoic cystic/necrotic central areas. The location of the mass seemed intraperitoneal causing bowel loop displacement and bilateral renal ectasia (Fig. 1). Abdominal magnetic resonance imaging (MRI) revealed a, 15 12 7.5 cm, lobulated intraabdominal mass arising from the root of the mesentery with extension to the right flank and pelvis.
* Corresponding author. Tel.: þ34 645558225. E-mail address: [email protected] (A. Martínez-Martínez).
All margins were well-defined and without evidence of organic infiltration. The tumour demonstrated low and homogeneous signal similar to muscle in the T1-weighted sequence. On T2weighted sequences the mass demonstrated high and heterogeneous signal with markedly hyper-intense central areas suggesting necrosis or cystic areas. In the contrast-enhanced T1-weighted sequence, the tumour showed heterogeneous, high and progressive enhancement (Figs. 2e4). The patient underwent surgery through median laparotomy and a large mesenteric multiloculated mass was extracted with a pathologic diagnosis made of embryonal RMS, arising in the mesentery following immunoistochemical analysis: the tumour cells were positive for Vimentin, Desmin and Myogenin (Fig. 5).
Discussion RMS is a very rare primary malignant tumour in mesenteric location. It has been reported to arise from, and metastasise to, nearly all body organs; however the head and neck (40%) and the genitourinary region (20%) account for most primary RMS.1 There is only one case series of intraperitoneal RMS in children in the literature, which found an incidence of 11% (6/55) over the course of the disease, and of 7% (4/55) at the time of initial diagnosis.2,3 This, however, included both primary and metastatic disease (only one case with peritoneal spread without a determined primary). Chung et al.3 the most common imaging finding in peritoneal RMS is ascites, intraperitoneal nodules and masses and
http://dx.doi.org/10.1016/j.radi.2015.05.007 1078-8174/© 2015 The College of Radiographers. Published by Elsevier Ltd. All rights reserved.
Please cite this article in press as: Martínez-Martínez A, et al., Primary mesenteric rhabdomyosarcoma in paediatric patient, Radiography (2015), http://dx.doi.org/10.1016/j.radi.2015.05.007
2
A. Martínez-Martínez et al. / Radiography xxx (2015) 1e3
Figure 1. Doppler-colour abdominal ultrasound imaging. Large and hetero-echoic central abdominal mass with significantly increased flow.
mesenteric nodules. Oto et al.4 presented a case of pelvic RMS and intraperitoneal neoplastic involvement without evidence of ascites, mesenteric nodules or omental caking. To date, only five cases of RMS in mesenteric location have been reported in the literature, only one of which was in a child.5e8 There are no references for the radiologic findings of mesenteric RMS, however Chung et al.2, Leung et al.3 and Oto et al.4
Figure 2. Abdominal Magnetic Resonance Imaging (MRI). Coronal MRI T2 fat saturation sequence: abdominal mass arising from the root of the mesentery with extension to right flank and pelvis (white arrow). The tumour demonstrated high and heterogeneous signal with no infiltration of the bladder (arrow head).
Figure 3. Abdominal Magnetic Resonance Imaging (MRI). Axial T1-weighted fast spinecho sequence without intravenous contrast. The tumour (white arrow) showed low and homogeneous signal similar to muscle (arrow-head).
described the imaging characteristics of intraperitoneal and omental rhambdomyosarcoma. The imaging findings of rhabdomyosarcoma are well known but nonspecific. It has been described as a well-defined solid heterogeneous mass with variable location. In very rare instances the tumour may show a predominantly cystic appearance.1 With its superior ability to depict soft-tissue changes, MRI is the primary imaging modality in RMS, being mandatory contrastenhanced series. Like most soft-tissue tumours they have an intermediate-low signal intensity in the T1-weighted sequence and intermediate-to-high signal in the T2-weighted sequence. The tumour in general has strong enhancement on postcontrast imaging with non-enhanced areas suggesting necrotic or cystic components. Our case showed similar imaging findings to RMS in other locations, although is important to note that these characteristics are totally nonspecific and very similar to those of other paediatric soft tissue masses. We have not identified any pathognomonic sign or peculiarity that allows its radiological diagnosis of certainty. The site of the tumour determines the differential diagnosis, however
Figure 4. Abdominal Magnetic Resonance Imaging. Axial T1-weighted fast spin-echo sequence with intravenous contrast. The tumour (white arrow) showed strong enhancement with non-enhanced central areas suggesting necrotic or cystic components (arrow head).
Please cite this article in press as: Martínez-Martínez A, et al., Primary mesenteric rhabdomyosarcoma in paediatric patient, Radiography (2015), http://dx.doi.org/10.1016/j.radi.2015.05.007
A. Martínez-Martínez et al. / Radiography xxx (2015) 1e3
3
We describe what we belive to be the second case of primary mesenteric RMS in a child reported in the literature. RMS should be considered in the differential diagnosis of an apparently malignant mesenteric mass in children because of its possibility to occur in any anatomical region. Conflict of interest statement Authors declare that there is no conflict of interest regarding the publication of this article. Permission statement Authors declare that the permission to publish this case report was obtained from the parents. References
Figure 5. Inmunohistochemical analysis: tumour cell were positive for Myogenin (black arrow).
certain tumours such as haemangiomas, other soft tissue sarcomas, infantile fibrosarcoma, aggressive fibromatosis, Primitive Neuroectodermal Tumour (PNET), rhabdoid tumour and even, nonosseous Ewing sarcoma, should be kept in mind in the differential diagnosis of soft-tissue tumours in childhood.1
1. Van Rijn RR, Wilde JC, Bras J, Oldenburger F, McHugh KM, Kerks JH. Imaging findings in noncraniofacial childhood rhabdomyosarcoma. Pediatr Radiol 2008;38:617e34. 2. Chung CJ, Fordham L, Little S, Rayder S, Nimkim K, Kleinmann PK, et al. Intraperitoneal rhabdomyosarcoma in children: incidence and imaging characteristics on CT. AJR Am J Roentgenol 1998;170(5):1385e7. 3. Leung RS, Calder A, Roebuck D. Embryonal rhabdomyosarcoma of the omentum: two cases occurring in children. Pediatr Radiol 2009;39:865e8. 4. Oto A, Basgün N, Kultuk T, Eryilmaz M, Oran M, Besim A. Intraperitoneal involvement in rhabdomyosarcoma CT findings in a child. Turk J Pediatr 2001;43(4):342e4. 5. Petit ML, Leteurtre E, Truant S, Herjean M, Triboulet JP, Lecomte-Houcke M. Alveolar rhabdomyosarcoma: a case report of a rare location. Ann Pathol 2001;21:51e4. 6. Urai S, Ashida C, Kida H, Yamashita M, Kubota S, Murao S. An autopsy case of an old-aged mesenteric rhabdomyosarcoma. Nihon Shokakibyo Gakkai Zasshi 2002;99(8):946e50. 7. Agarwald K, Kulshrestha R, Pahuja S, Chadja R. Botryoid rhabdomyosarcoma of mesentery-a case report. Indian J Pathol Microbiol 2003;46(3):457e9. 8. Agarwald K, Kumar Goel R, Puri V. Primary “botryoid” embryonal rhabdomyosarcoma in mesentery. Turk Patoloji Derg 2011;27(1):84e6.
Please cite this article in press as: Martínez-Martínez A, et al., Primary mesenteric rhabdomyosarcoma in paediatric patient, Radiography (2015), http://dx.doi.org/10.1016/j.radi.2015.05.007
Contents lists available at ScienceDirect
Radiography journal homepage: www.elsevier.com/locate/radi
Case report
Primary mesenteric rhabdomyosarcoma in paediatric patient Alberto Martínez-Martínez*, Jade García-Espinosa, Elena Pastor Pons Diagnostic Imaging Department, “Virgen de las Nieves” University Hospital, Granada, Spain
a r t i c l e i n f o
a b s t r a c t
Article history: Received 19 January 2015 Received in revised form 26 May 2015 Accepted 27 May 2015 Available online xxx
Primary mesenteric rhabdomyosarcoma is an exceptional entity in adult and paediatric patients. We report and discuss the imaging findings in a 2 year-old child with an abdominal mass. Our case showed similar imaging findings to rhabdomyosarcoma in other locations. © 2015 The College of Radiographers. Published by Elsevier Ltd. All rights reserved.
Keywords: Rhabdomyosarcoma Imaging Magnetic resonance imaging Pediatrics
Introduction Rhabdomyosarcoma (RMS) is the most common soft tissue sarcoma of childhood, representing 3e5% of all paediatric cancers.1 It arises from the same immature mesenchymal cells committed to skeletal muscle differentiation and can occur in many tissues, including those that lack striated muscle. There are very few cases described in mesenteric location. For its extreme rarity we report the case of a primary embryonal RMS in the mesentery. Case report A two year old male child with no relevant medical history was brought to the emergency department with symptoms of abdominal pain of 3 days duration and temperature of 39 C without leukocytosis and normal urinary sediment. Suspecting septic urinary focus, abdominal ultrasound was requested which revealed the existence of a large and hetero-echoic abdominal mass with significantly increased flow and hypo-echoic cystic/necrotic central areas. The location of the mass seemed intraperitoneal causing bowel loop displacement and bilateral renal ectasia (Fig. 1). Abdominal magnetic resonance imaging (MRI) revealed a, 15 12 7.5 cm, lobulated intraabdominal mass arising from the root of the mesentery with extension to the right flank and pelvis.
* Corresponding author. Tel.: þ34 645558225. E-mail address: [email protected] (A. Martínez-Martínez).
All margins were well-defined and without evidence of organic infiltration. The tumour demonstrated low and homogeneous signal similar to muscle in the T1-weighted sequence. On T2weighted sequences the mass demonstrated high and heterogeneous signal with markedly hyper-intense central areas suggesting necrosis or cystic areas. In the contrast-enhanced T1-weighted sequence, the tumour showed heterogeneous, high and progressive enhancement (Figs. 2e4). The patient underwent surgery through median laparotomy and a large mesenteric multiloculated mass was extracted with a pathologic diagnosis made of embryonal RMS, arising in the mesentery following immunoistochemical analysis: the tumour cells were positive for Vimentin, Desmin and Myogenin (Fig. 5).
Discussion RMS is a very rare primary malignant tumour in mesenteric location. It has been reported to arise from, and metastasise to, nearly all body organs; however the head and neck (40%) and the genitourinary region (20%) account for most primary RMS.1 There is only one case series of intraperitoneal RMS in children in the literature, which found an incidence of 11% (6/55) over the course of the disease, and of 7% (4/55) at the time of initial diagnosis.2,3 This, however, included both primary and metastatic disease (only one case with peritoneal spread without a determined primary). Chung et al.3 the most common imaging finding in peritoneal RMS is ascites, intraperitoneal nodules and masses and
http://dx.doi.org/10.1016/j.radi.2015.05.007 1078-8174/© 2015 The College of Radiographers. Published by Elsevier Ltd. All rights reserved.
Please cite this article in press as: Martínez-Martínez A, et al., Primary mesenteric rhabdomyosarcoma in paediatric patient, Radiography (2015), http://dx.doi.org/10.1016/j.radi.2015.05.007
2
A. Martínez-Martínez et al. / Radiography xxx (2015) 1e3
Figure 1. Doppler-colour abdominal ultrasound imaging. Large and hetero-echoic central abdominal mass with significantly increased flow.
mesenteric nodules. Oto et al.4 presented a case of pelvic RMS and intraperitoneal neoplastic involvement without evidence of ascites, mesenteric nodules or omental caking. To date, only five cases of RMS in mesenteric location have been reported in the literature, only one of which was in a child.5e8 There are no references for the radiologic findings of mesenteric RMS, however Chung et al.2, Leung et al.3 and Oto et al.4
Figure 2. Abdominal Magnetic Resonance Imaging (MRI). Coronal MRI T2 fat saturation sequence: abdominal mass arising from the root of the mesentery with extension to right flank and pelvis (white arrow). The tumour demonstrated high and heterogeneous signal with no infiltration of the bladder (arrow head).
Figure 3. Abdominal Magnetic Resonance Imaging (MRI). Axial T1-weighted fast spinecho sequence without intravenous contrast. The tumour (white arrow) showed low and homogeneous signal similar to muscle (arrow-head).
described the imaging characteristics of intraperitoneal and omental rhambdomyosarcoma. The imaging findings of rhabdomyosarcoma are well known but nonspecific. It has been described as a well-defined solid heterogeneous mass with variable location. In very rare instances the tumour may show a predominantly cystic appearance.1 With its superior ability to depict soft-tissue changes, MRI is the primary imaging modality in RMS, being mandatory contrastenhanced series. Like most soft-tissue tumours they have an intermediate-low signal intensity in the T1-weighted sequence and intermediate-to-high signal in the T2-weighted sequence. The tumour in general has strong enhancement on postcontrast imaging with non-enhanced areas suggesting necrotic or cystic components. Our case showed similar imaging findings to RMS in other locations, although is important to note that these characteristics are totally nonspecific and very similar to those of other paediatric soft tissue masses. We have not identified any pathognomonic sign or peculiarity that allows its radiological diagnosis of certainty. The site of the tumour determines the differential diagnosis, however
Figure 4. Abdominal Magnetic Resonance Imaging. Axial T1-weighted fast spin-echo sequence with intravenous contrast. The tumour (white arrow) showed strong enhancement with non-enhanced central areas suggesting necrotic or cystic components (arrow head).
Please cite this article in press as: Martínez-Martínez A, et al., Primary mesenteric rhabdomyosarcoma in paediatric patient, Radiography (2015), http://dx.doi.org/10.1016/j.radi.2015.05.007
A. Martínez-Martínez et al. / Radiography xxx (2015) 1e3
3
We describe what we belive to be the second case of primary mesenteric RMS in a child reported in the literature. RMS should be considered in the differential diagnosis of an apparently malignant mesenteric mass in children because of its possibility to occur in any anatomical region. Conflict of interest statement Authors declare that there is no conflict of interest regarding the publication of this article. Permission statement Authors declare that the permission to publish this case report was obtained from the parents. References
Figure 5. Inmunohistochemical analysis: tumour cell were positive for Myogenin (black arrow).
certain tumours such as haemangiomas, other soft tissue sarcomas, infantile fibrosarcoma, aggressive fibromatosis, Primitive Neuroectodermal Tumour (PNET), rhabdoid tumour and even, nonosseous Ewing sarcoma, should be kept in mind in the differential diagnosis of soft-tissue tumours in childhood.1
1. Van Rijn RR, Wilde JC, Bras J, Oldenburger F, McHugh KM, Kerks JH. Imaging findings in noncraniofacial childhood rhabdomyosarcoma. Pediatr Radiol 2008;38:617e34. 2. Chung CJ, Fordham L, Little S, Rayder S, Nimkim K, Kleinmann PK, et al. Intraperitoneal rhabdomyosarcoma in children: incidence and imaging characteristics on CT. AJR Am J Roentgenol 1998;170(5):1385e7. 3. Leung RS, Calder A, Roebuck D. Embryonal rhabdomyosarcoma of the omentum: two cases occurring in children. Pediatr Radiol 2009;39:865e8. 4. Oto A, Basgün N, Kultuk T, Eryilmaz M, Oran M, Besim A. Intraperitoneal involvement in rhabdomyosarcoma CT findings in a child. Turk J Pediatr 2001;43(4):342e4. 5. Petit ML, Leteurtre E, Truant S, Herjean M, Triboulet JP, Lecomte-Houcke M. Alveolar rhabdomyosarcoma: a case report of a rare location. Ann Pathol 2001;21:51e4. 6. Urai S, Ashida C, Kida H, Yamashita M, Kubota S, Murao S. An autopsy case of an old-aged mesenteric rhabdomyosarcoma. Nihon Shokakibyo Gakkai Zasshi 2002;99(8):946e50. 7. Agarwald K, Kulshrestha R, Pahuja S, Chadja R. Botryoid rhabdomyosarcoma of mesentery-a case report. Indian J Pathol Microbiol 2003;46(3):457e9. 8. Agarwald K, Kumar Goel R, Puri V. Primary “botryoid” embryonal rhabdomyosarcoma in mesentery. Turk Patoloji Derg 2011;27(1):84e6.
Please cite this article in press as: Martínez-Martínez A, et al., Primary mesenteric rhabdomyosarcoma in paediatric patient, Radiography (2015), http://dx.doi.org/10.1016/j.radi.2015.05.007