[Infantile fibrosarcoma: a clinicopathological and molecular study of five cases].
We report 5 cases of infantile fibrosarcoma (4 boys and 1 girl) whose average age was 5, 7 months (range 0 days to 14 months). The tumor was congenital in 4 cases. All tumors presented in the extremities (forearm, hand, thigh: 1 case, lower leg: 2 cases). Treatment was based on surgery (3 cases: amputation, 2 cases: local excision) with a favorable course in all cases, even those with marginal excision (follow-up ranging from 5 to 21 years). The lesions were characterized by dense monotonous cells growing in a fascicular pattern, with small necrotic areas and scattered lymphocytes. The mitotic index was high (average 8/10 high-power fields). ETV6-NK3 chimeric RNA was detected by reverse transcriptase polymerase chain reaction in two cases out four cases from paraffin-embedded tissue blocks. The infantile fibrosarcoma is a good prognosis tumor characterized by particular histological features and ETV6-NK3 gene fusion.
Mrad
K
K
Service d'anatomie et cytologie pathologiques, Institut Salah Azaiez, Bab Saadoun, 1006 Tunis.
Dubus
P
P
Bougrine
F
F
Ben Salah
H
H
Sassi
S
S
Rammeh
S
S
Driss
M
M
Ben Ghachem
M
M
Merlio
J P
JP
Ben Romdhane
K
K
D005260:Female; D005354:Fibrosarcoma / Q000235:genetics* / Q000473:pathology* / Q000601:surgery; D005542:Forearm; D006225:Hand; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D007866:Leg; D008297:Male; D011379:Prognosis