Undifferentiated pleomorphic sarcoma

Case contributed by Ammar Ashraf
Diagnosis certain

Presentation

Swelling right upper medial thigh for 3 months. Gradually increasing in size, and associated with intermittent pain. No history of trauma, fever, or weight loss.

Patient Data

Age: 55 years
Gender: Male
ultrasound
Longitudinal
Longitudinal
Longitudinal
Longitudinal
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Info
  • a well-defined oval shape mildly heterogeneous hypoechoic mass lesion is seen between the muscle layers of the right upper medial thigh. Mild internal vascularity is seen in it on color Doppler ultrasound examination

  • differential diagnosis includes peripheral nerve sheath tumor (like neurofibroma), or soft tissue tumor (myxoma, or soft tissue sarcoma)

Frontal
Frontal
Lateral
Lateral
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A partially circumscribed soft tissue swelling/bulge is seen along the medial aspect of the right upper thigh. No soft tissue calcifications are seen. Morphology of the visualized bones is intact. 

6 months later

mri
This study is a stack
Axial
T1
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Axial T2
SPAIR
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Coronal
T1
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Coronal
STIR
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Sagittal
STIR
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Axial T1
C+ fat sat
This study is a stack
Coronal T1
C+ fat sat
This study is a stack
Sagittal T1
C+ fat sat
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Findings: There is a large well-defined, oval-shape soft tissue lesion in the right upper medial thigh within the adductor longus muscle. The lesion is isointense to the muscles on T1 and hyperintense on T2 –weighted images. The lesion shows marked enhancement after the contrast administration. No significant cystic/necrotic changes, fat component, or calcifications are seen in it. No extra-muscular extension is seen.  There is no invasion of myofascial planes, subcutaneous soft tissues, neurovascular bundle, or underlying femur. No skip lesion or significant lymphadenopathy is seen.  Differential diagnosis includes neurofibroma, myxoma, or soft tissue sarcoma.

Case Discussion

  • the patient lost to follow up after initial sonographic and radiographic evaluation and 6 months later, presented with worsening symptoms. He underwent an MRI of the right thigh followed by a biopsy

  • histopathology: High grade undifferentiated pleomorphic sarcoma (previously known as malignant fibrous histiocytoma). Immunohistochemically, tumor cells react positively for Vimentin (V9) while negatively for Desmin (D33), SMA (1A4), Myo-D1 (5.8), S100 (polyclonal), CD34 (Qbend10), SOX10 (EP268), and PAN CK (AE1/AE3)

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