EURORAD ESR

Case 12678

Multicentric congenital infantile myofibromatosis in a newborn – a case report

Author(s)
Bruno Araujo, André Carvalho, Margarida Sá Fernandes, Rui Cunha

Hospital de São João
Serviço de Radiologia HSJ
Alameda Prof. Hernâni Monteiro
4200 – 319 Porto, Portugal
Email:Bruno_peixe@hotmail.com
 
Patient
female, 2 week(s)
 
 
  • Figure 1
    Ultrasound image of an intercostal space

    A well-delimited hypoechoic nodule can be identified in the subcutaneous tissue between two ribs (arrows).

     
    Area of Interest: Soft tissues / Skin; Imaging Technique: Ultrasound; Procedure: Diagnostic procedure; Special Focus: Congenital;
     
     
  • Figure 2
    Axial T2-weighted fat saturated (fat-sat) MR image

    Multiple well-delimited hyperintense nodules are identified in the subcutaneous tissue and muscle. Note the largest in the left cubital fossa.

     
    Area of Interest: Musculoskeletal soft tissue; Soft tissues / Skin; Imaging Technique: MR; Procedure: Diagnostic procedure; Special Focus: Congenital;
     
     
  • Figure 3
    Axial T1-wheighted MR image

    The nodules presented low signal in T1-weighted sequences.

     
    Area of Interest: Musculoskeletal soft tissue; Soft tissues / Skin; Imaging Technique: MR; Procedure: Diagnostic procedure; Special Focus: Congenital;
     
     
  • Figure 4
    Coronal T2-weighted MR image

    Multiple hyperintense nodules are identified in the gluteal muscles demonstrating diffuse muscle and subcutaneous involvement.

     
    Area of Interest: Musculoskeletal soft tissue; Paediatric; Imaging Technique: MR; Procedure: Diagnostic procedure; Special Focus: Congenital;
     
     
  • Figure 5
    Axial T1-weighted fat-sat contrast-enhanced MR image

    The nodular lesions demonstrate peripheral enhancement after iv. contrast.

     
    Area of Interest: Musculoskeletal soft tissue; Paediatric; Imaging Technique: MR; Procedure: Diagnostic procedure; Special Focus: Congenital;
     
     
  • Figure 6
    Coronal T1-weighted fat-sat contrast-enhanced MR image

    The multiple dispersed nodules in the subcutaneous tissue and muscle present peripheral contrast enhancement, usually present in myofibromatous lesions.

     
    Area of Interest: Musculoskeletal soft tissue; Paediatric; Imaging Technique: MR; Procedure: Diagnostic procedure; Special Focus: Congenital;
     
     
  • Figure 7
    Photomicrographs (H-E stain 20x magnification) of the excised nodule specimen

    An intramuscular nodule of fibroblastic/myofibroblastic cells is seen with central hyalinization.

     
    Area of Interest: Musculoskeletal soft tissue; Paediatric; Imaging Technique: Percutaneous; Procedure: Biopsy; Special Focus: Pathology;
     
     
  • Figure 8
    Photomicrographs (H-E stain 40x magnification) of the excised nodule specimen

    Note the presence of striated muscle cells in the periphery of the lesion with focal invasion of the adjacent muscular tissue.

     
    Area of Interest: Musculoskeletal soft tissue; Paediatric; Imaging Technique: Percutaneous; Procedure: Biopsy; Special Focus: Pathology;
     
     
  • Figure 9
    Photomicrographs (actin (A) and vimentin (B) stain 100x magnification)

    The cells stained positive to actin and vimentin.

     
    Area of Interest: Musculoskeletal soft tissue; Paediatric; Imaging Technique: Percutaneous; Procedure: Biopsy; Special Focus: Pathology;
     
     
  • Figure 10
    Pelvic X-ray image

    Multiple lytic well-delimited lesions are identified in the metaphysis and diaphysis of both femurs.

     
    Area of Interest: Musculoskeletal bone; Paediatric; Imaging Technique: Digital radiography; Procedure: Diagnostic procedure; Special Focus: Congenital;
     
     
  • Figure 11
    Lower limbs X-ray image

    Lytic lesions are also found in both tibias. Note sclerotic area in the middle diaphysis of the left tibia secondary to a pathologic fracture.

     
    Area of Interest: Musculoskeletal bone; Paediatric; Imaging Technique: Digital radiography; Procedure: Diagnostic procedure; Special Focus: Congenital;
     
     
  • Figure 12
    Ultrasound image of the liver

    A liver hypoechoic nodule is demonstrated in the 8th hepatic segment, compatible with visceral involvement by the known congenital myofibromatosis.

     
    Area of Interest: Abdomen; Paediatric; Imaging Technique: Ultrasound; Procedure: Diagnostic procedure; Special Focus: Congenital;
     
     
  • Figure 13
    Sagittal T1-weighted fat-sat contrast-enhanced MR image

    The hepatic nodule is confirmed (arrow) with imaging features compatible with visceral involvement by the known congenital myofibromatosis. Also note the diffuse multiple nodules in the muscle and subcutaneous tissue.

     
    Area of Interest: Liver; Paediatric; Imaging Technique: MR; Procedure: Diagnostic procedure; Special Focus: Congenital;
     
     
  • Figure 14
    Axial T1-weighted fat-sat contrast-enhanced MR image

    Progression of the nodules was clinically evident and the MRI confirmed with a large lesion in the left adductor minimus. Also note a lesion in the right femur demonstrating osseous involvement (arrow).

     
    Area of Interest: Musculoskeletal bone; Paediatric; Imaging Technique: MR; Procedure: Diagnostic procedure; Special Focus: Congenital;
     
     
  • Figure 15
    Ultrasound image of the liver (post treatment)

    The known liver nodule in the 8th segment (Fig. 12) is not identified, which demonstrates regression of the lesions after chemotherapy with methotrexate and vinblastine was initiated.

     
    Area of Interest: Liver; Paediatric; Imaging Technique: Ultrasound; Procedure: Diagnostic procedure; Special Focus: Congenital;
     
     
  • Figure 16
    Coronal T1-weighted fat-sat contrast-enhanced MR images

    Comparison of MR coronal images before and after chemotherapy. Reduction of the number and size of the multiple myofibromatous nodules is demonstrated, which confirmed the clinical findings suggesting good response to...

     
    Area of Interest: Musculoskeletal soft tissue; Paediatric; Imaging Technique: MR; Procedure: Diagnostic procedure; Special Focus: Congenital;
     
     
A well-delimited hypoechoic nodule can be identified in the subcutaneous tissue between two ribs (arrows).
 
Multiple well-delimited hyperintense nodules are identified in the subcutaneous tissue and muscle. Note the largest in the left cubital fossa.
 
The nodules presented low signal in T1-weighted sequences.
 
Multiple hyperintense nodules are identified in the gluteal muscles demonstrating diffuse muscle and subcutaneous involvement.
 
The nodular lesions demonstrate peripheral enhancement after iv. contrast.
 
The multiple dispersed nodules in the subcutaneous tissue and muscle present peripheral contrast enhancement, usually present in myofibromatous lesions.
 
An intramuscular nodule of fibroblastic/myofibroblastic cells is seen with central hyalinization.
 
Note the presence of striated muscle cells in the periphery of the lesion with focal invasion of the adjacent muscular tissue.
 
The cells stained positive to actin and vimentin.
 
Multiple lytic well-delimited lesions are identified in the metaphysis and diaphysis of both femurs.
 
Lytic lesions are also found in both tibias. Note sclerotic area in the middle diaphysis of the left tibia secondary to a pathologic fracture.
 
A liver hypoechoic nodule is demonstrated in the 8th hepatic segment, compatible with visceral involvement by the known congenital myofibromatosis.
 
The hepatic nodule is confirmed (arrow) with imaging features compatible with visceral involvement by the known congenital myofibromatosis. Also note the diffuse multiple nodules in the muscle and subcutaneous tissue.
 
Progression of the nodules was clinically evident and the MRI confirmed with a large lesion in the left adductor minimus. Also note a lesion in the right femur demonstrating osseous involvement (arrow).
 
The known liver nodule in the 8th segment (Fig. 12) is not identified, which demonstrates regression of the lesions after chemotherapy with methotrexate and vinblastine was initiated.
 
Comparison of MR coronal images before and after chemotherapy. Reduction of the number and size of the multiple myofibromatous nodules is demonstrated, which confirmed the clinical findings suggesting good response to chemotherapy.
 
 
 
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