Case of the Week # 131

Patrick Bailleul, MD and Corinne Jeanne Pasquier, MD

November 5-18, 2004

France.

This is a routine ultrasound scan performed at 35 weeks of gestation. These are picture of the leg (the rest of the fetus is normal)

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Answer

This is a routine ultrasound scan performed at 35 weeks of gestation. These are pictures of the leg (the rest of the fetus is normal).

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The diagnosis was not established until the fetus was delivered and a post-natal biopsy revealed a spindle cell tumor that was confirmed to be a fibrosarcoma by immunohistochemistry. The translocation t (12 ;15) ( p13 ;q25) was absent. The antenatal images demonstrate a vascular pedicle leading to the mass:

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Postnatal photograph: 

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In the immediate post partum, the tumor grew massively requiring partial resection. The child did not have an amputation and was also treated by chemotherapy. The following are the histopathological photographs:

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Currently the child is doing well, walks with a limp and the calf is supple without palpable tumor.

Discussion: Congenital fibrosarcoma is a pediatric spindle-cell tumor of soft tissues that usually presents before the age of 2 years. Although these tumors display histological features of malignancy and frequently recur, they have a relatively good prognosis and only rarely metastasize. Soft tissues tumors account for 7% of the malignant tumors in childhood and fibro- and myoblastic tumors account for 12% of the soft tissues tumors in childhood.
A recent study about the follow-up of congenital fibrosarcoma at CHU NECKER – Enfants Malades (Paris) by Dr J-C FOURNET revealed these features of the mass:
Age : newborn to 2 years old with neonatal diagnosis in 80% of the cases.
Sex : M > F ( x 1,7) 
Localization : Head and neck 19%, trunk 31%, abdomen and retroperitoneum 8%, limbs 42 %
Recurrence : 5-50%
Metastasis : 0-25%
Mortality rate : 23%

A prenatal diagnosis is impossible. Diagnosis can only be established by biopsy and t(12 ;15) (p13 ;q25 ) translocation when found, gives a better prognosis.

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