Prenatal management of congenital infantile fibrosarcoma: unexpected outcome

Abstract

We report the case of a large leg tumor in a fetus at 36 weeks of gestation. The tumor 's location, ultrasound CASE REPORTA 31-year-old woman, gravida 2 para 0, was referred to our hospital after a third ultrasound examination at 36 weeks of gestation revealed a large tumor on the right leg of the fetus (Figure 1a). The patient had conceived spontaneously and had no family history of any malformation. This anomaly was not present on previous ultrasound examinations. The tumor measured 90 × 80 mm and was poorly vascularized. The fetal middle cerebral artery peak systolic velocity was normal. A male fetus of normal size was observed in the footling breech presentation, with no other structural abnormalities. Magnetic resonance imaging (MRI) examination using 1.5 Tesla (IRM MAGNETOM Avanto, Siemens, Erlangen, Germany) at 37 weeks of gestation showed a 107 × 73 × 86-mm soft tissue mass around the tibia and fibula (Figure 1b,c). The tumor presented as a well-demarcated low-signal-intensity soft tissue mass on T1-weighted imaging and as an inhomogeneous high-signal-intensity soft tissue mass on T2-weighted imaging and TrueFISP sequences. The mass lay adjacent to the bones and deformed the foot and leg, but there was no cortical destruction or marrow involvement. No evidence of metastatic spread was found on whole-body MRI 1,2 . All ultrasound and MRI findings were compatible with an infantile fibrosarcoma. The parents were informed and initially decided on termination of pregnancy. We submitted the medical records to the pediatric oncology department and to the prenatal diagnosis center. Termination of pregnancy was not accepted because of the excellent prognosis of such a tumor, with complete remission in 80% of patients and a 5-year survival rate of 90% after treatment 3 .The same operator performed another ultrasound examination 1 week later to evaluate the tumor progression: the vascularized mass had grown to 120 mm without fetal anemia. The fetus was still in the footling breech position. Because the pregnancy was now at 38 weeks of gestation, a Cesarean section was scheduled. On the day of admission, the patient reported no fetal movements. Fetal heart rate had slowed markedly. A male infant in the cephalic position was delivered by emergency Cesarean. Birth weight was 3380 g, the 1-and 5-min Apgar scores were 2 and 4, respectively, and the umbilical artery pH was 7.21. The amniotic fluid was bloody. Hemorrhagic shock caused by an oval-shaped red-blue tumor, not covered with skin, surrounding the right leg (Figure 1d) was treated immediately. During the first day (hour 6 after delivery) and despite appropriate care, it was decided to amputate the lower leg because of uncontrolled bleeding. Seven days later, neurological

Emergency surgical treatment of an ulcerative and hemorrhagic congenital/infantile fibrosarcoma of the lower leg

“…Dumont et al [12] reported a case of a fibrosarcoma found in a 36 week gestational age fetus. Ulceration in utero may cause various complications including polyhydramnios, torsion, anemia, hemorrhagic shock, hemolysis, as well as compression of adjacent organs and cardiac insufficiency; any of which could threaten intrauterine life.…”

Congenital-infantile fibrosarcoma masquerading as sacrococcygeal teratoma

“…It is locally aggressive with an increased rate of local recurrence, but systemic metastasis is very rare. Most infantile fibrosarcomas are diagnosed postnatally, but there are increasing reports of antenatally diagnosed cases [11,12]. There appear to be no advantage to preterm delivery of these patients except if there is associated hydrops and delivery by cesarean section is indicated only in those with large tumors that are likely to obstruct delivery.…”

Congenital infantile fibrosarcoma in a very low-birth-weight infant

“…With the widespread use of routine antenatal ultrasonography, reported cases with fibrosarcoma detected prenatally have increased (Table 1) [3][4][5][6][7][8][9][10][11][12][13][14][15][16]. Unlike postnatally diagnosed cases, prenatal CIFs are located in various sites, and only 3 of 14 cases occurred in limbs.…”

“…Thus, CIF should be considered in the differential diagnosis of any soft tissue mass found during fetal sonography. Recently, the combination of ultrasound and magnetic resonance imaging has allowed for the prenatal diagnosis of CIF [5]. Congenital infantile fibrosarcoma is usually successfully treated with surgery.…”