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Imaging findings in noncraniofacial childhood rhabdomyosarcoma.

Van Rijn RR, Wilde JC, Bras J, Oldenburger F, McHugh KM, Merks JH - Pediatr Radiol (2008)

Bottom Line: Rhabdomyosarcoma (RMS) is the most common soft-tissue sarcoma of childhood.This paper is focuses on imaging for diagnosis, staging, and follow-up of noncraniofacial RMS.

View Article: PubMed Central - PubMed

Affiliation: Department of Radiology, Academic Medical Centre Amsterdam, Suite G1-224, Meibergdreef 9, 1105 AZ, Amsterdam Zuid-Oost, The Netherlands. r.r.vanrijn@amc.uva.nl

ABSTRACT
Rhabdomyosarcoma (RMS) is the most common soft-tissue sarcoma of childhood. This paper is focuses on imaging for diagnosis, staging, and follow-up of noncraniofacial RMS.

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Related in: MedlinePlus

Alveolar RMS: cluster of primitive cells with loss of cellular cohesion and bordered by dense fibrous septa, resulting in an alveolar pattern (H&E, original magnification 10×20)
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Fig3: Alveolar RMS: cluster of primitive cells with loss of cellular cohesion and bordered by dense fibrous septa, resulting in an alveolar pattern (H&E, original magnification 10×20)

Mentions: Alveolar RMS is composed of ill-defined, dense aggregates of poorly differentiated round or oval tumour cells that frequently show loss of cohesion (Fig. 3). This loss of cohesion and the presence of thin fibrous septa result in an alveolar pattern. In cases where the tumour cells do not show loss of cohesion, the term ‘solid alveolar RMS’ is used. Alveolar RMS represents about 20% of all RMS and has two specific translocations with specific fusion transcripts that can be detected by RT-PCR. The majority (about 55%) show a t(2;13)(q35;q14) translocation with the corresponding fusion transcript PAX3-FKHR [8]. In about 22% a t(1;13)(p36;q14) translocation is found with fusion transcript PAX7-FKHR. In rare cases, RMS with a more or less alveolar pattern lacks these translocations.Fig. 3


Imaging findings in noncraniofacial childhood rhabdomyosarcoma.

Van Rijn RR, Wilde JC, Bras J, Oldenburger F, McHugh KM, Merks JH - Pediatr Radiol (2008)

Alveolar RMS: cluster of primitive cells with loss of cellular cohesion and bordered by dense fibrous septa, resulting in an alveolar pattern (H&E, original magnification 10×20)
© Copyright Policy
Related In: Results  -  Collection

Show All Figures
getmorefigures.php?uid=PMC2367394&req=5

Fig3: Alveolar RMS: cluster of primitive cells with loss of cellular cohesion and bordered by dense fibrous septa, resulting in an alveolar pattern (H&E, original magnification 10×20)
Mentions: Alveolar RMS is composed of ill-defined, dense aggregates of poorly differentiated round or oval tumour cells that frequently show loss of cohesion (Fig. 3). This loss of cohesion and the presence of thin fibrous septa result in an alveolar pattern. In cases where the tumour cells do not show loss of cohesion, the term ‘solid alveolar RMS’ is used. Alveolar RMS represents about 20% of all RMS and has two specific translocations with specific fusion transcripts that can be detected by RT-PCR. The majority (about 55%) show a t(2;13)(q35;q14) translocation with the corresponding fusion transcript PAX3-FKHR [8]. In about 22% a t(1;13)(p36;q14) translocation is found with fusion transcript PAX7-FKHR. In rare cases, RMS with a more or less alveolar pattern lacks these translocations.Fig. 3

Bottom Line: Rhabdomyosarcoma (RMS) is the most common soft-tissue sarcoma of childhood.This paper is focuses on imaging for diagnosis, staging, and follow-up of noncraniofacial RMS.

View Article: PubMed Central - PubMed

Affiliation: Department of Radiology, Academic Medical Centre Amsterdam, Suite G1-224, Meibergdreef 9, 1105 AZ, Amsterdam Zuid-Oost, The Netherlands. r.r.vanrijn@amc.uva.nl

ABSTRACT
Rhabdomyosarcoma (RMS) is the most common soft-tissue sarcoma of childhood. This paper is focuses on imaging for diagnosis, staging, and follow-up of noncraniofacial RMS.

Show MeSH
Related in: MedlinePlus