Retroperitoneal malignant teratoma is a rare congenital tumor, representing 1%–10% of retroperitoneal tumors in children.1 2 A pathological migration of primitive germ cells to the genital ridges can stop at various levels, which explains the localizations of these tumors.3 The diagnosis is done either prenatally, or later in front of an abdominal mass.4 5 We reported a case treated in our department and reviewed the diagnostic and therapeutic aspects of its management.
It was a 3-year-old child, weighing 12 kg, without any particular history, examined during a foreign Non-Governmental Organisation (NGO) surgical mission. The child had an abdominal mass that gradually increased in volume over a period of 2 years. The general condition was moderately impaired.
Physical examination, found a swollen abdomen with collateral venous circulation, a voluminous mass occupying almost the entire abdominal cavity except the hypogastrium, the left side and iliac fossa, without digestive or urinary disorders. Abdominal ultrasonography (figure 1) and abdominal CT (figure 2) revealed a mixed, heterogeneous right retroperitoneal tumor containing cystic areas and calcifications with a normal kidney. The alpha-fetoprotein level was up to 301 ng/mL.
Ultrasound image showing a solid component mass (a) and fluid (b).
CT image showing, a normal right kidney (a), well separated from a mixed heterogeneous right retroperitoneal mass (b) with presence of calcifications.
Surgical treatment consisted of a complete tumor resection (figure 3) with some cystic pouches puncture.
Solid portion of teratoma in a cup after opening and aspiration of cysts.
Macroscopic examination of the specimen weighing 4 kg, revealed a mixed tumor with solid tissue and cysts, and its standard X-ray (figure 4) confirmed the presence of bone tissue. Immediate postoperative follow-up was simple and the child was discharged. The histology concluded to retroperitoneal teratoma with a contingent of malignant vitelline cells.
X-ray of teratoma after excision and opening with bone structures (a).
The parents refused a new hospitalization and the proposed free chemotherapy. Three months after surgery she presented a poor general condition with parietal and peritoneal nodules that evoked peritoneal carcinomatosis. With the persistent refusal of chemotherapy by the parents, the child was lost of sight and died two months later.
The discovery of a retroperitoneal malignant teratoma is often fortuitous.1 It is either discovered prenatally by obstetrical ultrasound,2 6 or at an advanced age4 5 with a first pic before three years among female.7 8 The latter is consistent with our observation.
Clinically, asymptomatic in 15%–30% of cases, it is most often seen as an abdominal mass that often lays on the right.1 8 The general status may change with a significant loss of weight1 9 with signs of compression of neighbouring organs resulting in collateral venous circulation.1 8
The dosage of alphafoetoprotein (AFP) and the gonadotropin hormone chorionic markers1 10 11 are crucial for therapy and monitoring.12 13 The high AFP level in our patient confirmed the malignancy of the tumor.
Standard X-rays may reveal calcifications, suggesting the presence of teeth or bone.6 14 This exam of the operative specimen, showed calcifications and bone structures.
The ultrasound analyzes various components of the tumor mass, as cystic zones or shadow cone related to the hair, teeth or calcifications.6 14 In this case, ultrasonography only a revealed a heterogeneous mixed mass, with solid tissue and fluid components. The CT scan performed confirmed the heterogeneous and mixed appearance of the tumor mass and showed a normal right kidney.
Magnetic resonance imaging (MRI), which is not performed, reveal more details of the mass, and can highlight its pedicle.1 6
Histological examination specifies the anatomopathological type and provides the therapeutic approach.1 7 It indicated a retroperitoneal teratoma with a malignant contingent of vitelline cells in this case.
In the treatment of a mixed tumor with a malignant contingent, neo and/or adjuvant chemotherapy is required.13 15 The purpose of adjuvant chemotherapy is to destroy the tumor cells seeded in the peritoneal cavity during surgery. This would be indicated for puncture-aspiration manipulations of cystic pouches performed intraoperatively.
In this case, there were no complications during tumor removal, contrary to Rattan and al,13 who reported an inferior vena cava intraoperative lesion that was repaired immediately.
The evolution is generally good when the treatment is well conducted and well monitored.15 However, the proposed chemotherapy following the histological results was refused by the parents. A tumor relapse was observed with appearance of peritoneal and parietal nodules. The child lost sight before dying two months later. Mouad et al16 reported a case of death as well.
Various sociological factors can be evoked during care, including late access to care due to poverty. The refusal of free chemotherapy could be associated with the manifestations of religious beliefs and ignorance. These factors were obstacles to the malignant tumor management protocol.
Retroperitoneal malignant teratomas are rare tumors in children. In our case the diagnosis was late. Multidisciplinary treatment was handicapped by socio-economic factors. Early diagnosis associated with preoperative and postoperative chemotherapy is of great meaning for a good prognosis.
