Abstract
Congenital infantile fibro-sarcomas (CIFS) are rare, locally aggressive mesenchymal tumors that usually develop from soft tissue of distal extremities. CIFS arising from paraspinal region are extremely rare and only few cases have been reported. A left thoracic paraspinal swelling in a female fetus was noted on 20-week antenatal scan of a primigravida. The swelling was monitored with regular antenatal scans. The baby was born at term and was well at birth. Serum AFP and Beta HCG were within normal age limits. MRI scan confirmed an aggressive tumor infiltrating the paraspinal muscles but not the spinal cord. The mass grew very rapidly in size within a few days, and was excised on day 7 of life. Histopathology and Immunohistochemistry diagnosed the mass to be a Fibrosarcoma. No chemotherapy or radiotherapy was given. The baby is well with no recurrence or residual deformity or neurological deficit after 18 months of follow up.Paraspinal CIFS are extremely rare but can be detected antenatally as early as 20 weeks of gestation. Complete surgical excision is the treatment of choice.
Highlights
Congenital infantile fibro-sarcomas (CIFS) was first recognized in 1962.[1]
We report a case of CIFS arising from the thoracic paraspinal region
The three cases of CIFS reported in the thoraco-lumbar paraspinal region by Ainsworth [4] so far had presented between 1 week and 1 month of life but none of these cases were detected antenatally
Summary
CIFS was first recognized in 1962.[1]. This tumor arises from mesenchymal cells and composed of malignant fibroblasts within a collagen background. A large left thoracic paraspinal swelling in a female fetus was noted on routine 20-week antenatal scan of a primigravida. Fetal MRI showed no infiltration of the spinal cord. The swelling was monitored with regular antenatal scans but no significant growth was noted. The paraspinal swelling was firm to hard and measured about 5x10x10cms.The baby was nursed in lateral and prone positions. Ultrasound scan was initially done showed soft tissue swelling but the margins could not be delineated clearly. MRI confirmed an aggressive tumor, infiltrating the paraspinal muscles but not the spinal cord (Fig.2a,2b). After adequate counselling of parents, in view of rapid growth of tumor along with the potential risk of bursting and subsequent bleeding, surgical excision was done on day 7 of life without any prior biopsy.
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