Neonatal testicular tumors and intrauterine testicular torsions are very rare. The presented case is the first describing intrauterine torsion of a descended testis with a teratomatous tumor. Immediately after birth, right hemiscrotal swelling was seen in a preterm male newborn. Surgical intervention showed extravaginal testicular torsion and a highly differentiated testicular teratoma with haemorrhagic infarction. The testis was removed (orchiectomy). Over a period of twelve months no signs of tumor recurrence were found. While being extremely rare, testicular tumors should be included in the differential diagnosis of neonatal scrotal swelling.
The synergistic occurrence of intrauterine testicular torsion and neoplasia of this testis has so far only been described in two newborns with undescended testes; in both cases, a teratoma was found on abdominal testes.1, 2
The presented case report is the first description of an intrauterine torsion of a descended testis associated with a teratomatous tumor. Clinical and paraclinical findings, diagnostic imaging and therapeutic approach are considered and discussed.
A boy weighing 3890 g was delivered by caesarean section after 36 weeks gestation. The 31-year-old Vietnamese mother had a pregnancy complicated by insulin-dependent gestational diabetes and recurring pyelonephritis. The newborn's Apgar score was 9/8/9 after 1, 5 and 10 minutes. Postpartum the baby showed signs of neonatal pneumonia with pronounced cyanosis, respiratory acidosis, oxygen requirement. Antibiotic therapy with cefotaxim, ampicillin, and erythromycin was initiated.
The left testis was enlarged and indurated. Sonography of this testis showed inhomogeneous echoes (Figure 1). No blood circulation was detected by duplex sonography, so intrauterine testicular torsion with tissue necrosis was presumed. By the 7th day of life, the left scrotum had gained only slightly in size and pulmonary function was stable, allowing operative intervention by an inguinal approach. Fixed extravaginal torsion by 360° and diffuse tissue necrosis in a testis measuring about 3 cm was observed. High inguinal left-sided semicastration and contralateral scrotal orchipexy were performed. Histopathology of the specimen revealed hemorrhagic infarction of a highly differentiated testicular teratoma with cartilage and bone tissue. The borders of the specimen were free of tumor cells (Figure 2). Two postoperatively measured alpha fetoprotein (AFP) serum levels were within the physiological range of newborns. The wounds healed without complications and the child was discharged on the 10th postoperative day in good general condition. At 12 months of age, no tumor recurrence was found.
Das and Singer3 divide neonatal testicular torsion into a prenatal (72%) and a postnatal (28%) form, the latter term specifying a period of 30 days postpartum. These authors describe a loss of the testis in 80 to 90% of neonatal testicular torsions in spite of immediate surgical intervention. In addition to usually painless and indurated scrotal swelling with livid discoloration of the scrotal skin, sonographic findings can lead to the diagnosis. Color-coded duplex sonography generally demonstrates absence of blood circulation in the testis, and testicular sonography demonstrates a heterogeneous echo pattern with central necrotic areas and an ipsilateral thickening of the tunica albuginea.4
In regards to treatment, some authors suggest a conservative approach if prenatal testicular torsion is suspected, as the benefit from an operation may be limited and anesthesia risks can be avoided.5 Other authors present the advantages of immediate surgical exploration, which include salvaging of the testis in some newborns, prevention of the development of autoantibodies against spermatogenesis, ability to perform a simultaneous contralateral orchidopexy, and the definitive exclusion of a diagnostically missed testicular tumor.3 In a study by Sorensen et al.6 on 10 newborns with extravaginal testicular torsion, 40% of the concerned testes were salvaged by immediate surgical intervention.6 Meanwhile, a general consent has been reached to perform contralateral orchidopexy in children with unilateral testicular torsion, as otherwise anorchidy might be risked in view of a contralateral torsion rate of 5%, even though literature documents cases of testicular atrophy as a result of orchidopexy.7
Testicular tumors mostly appear as painless scrotal swelling and resemble testicular torsion in the neonatal period. Scrotal sonography can almost always be used to verify a testicular mass, whereas a differentiation between torsion and tumor can only be made by duplex sonography. In addition to inhomogeneous echoes, cystic areas and calcifications may be seen as sonographic evidence of testicular tumors.7 With an incidence between 0.5 and 2 in 100,000 children, testicular tumors in prepuberty are rare, showing a maximum occurrence in the second to third year of life.8 Of 338 pediatric testicular tumors which were documented by the “Prepubertal Testis Tumor Registry of the American Academy of Pediatrics” over a period of 12 years, 22 (6.5%) were found in newborns in the first month of life.9 Of these 22 neoplasias, six were yolk sac tumors, six gonadal stroma tumors, six juvenile granulosa tumors, two gonadoblastomas, one hamartoma, and one teratoma.9 Teratomas characteristically contain tissue of all three embryological blastodermic layers with parts of differentiated tissue like bone, teeth, and hair. In childhood, this type of tumor is mostly seen as a mature, benign teratoma, lacking undifferentiated or unripe tissue.7 Mature teratomas never show AFP-expression. Raised AFP levels may therefore indicate a malignant teratoma. Nevertheless, the diagnostic meaning of this tumor marker is reduced in the first year of life by the physiological production of this onco-fetal antigen in the gastrointestinal tract, the liver, and the yolk sac of the fetus.10 There are no other useful tumor markers for pediatric testicular tumors. Although testicular tumors in children are mostly benign, radical orchiectomy with high inguinal ligation is still seen as the standard primary therapy.7 In recent studies on prepubertal testicular tumors, parenchymal sparing resections are propagated in benign tumors with intraoperative histopathological control of specimen borders and funiculus spermaticus; first data show no deterioration of prognosis in a follow-up of 96 months.11, 12 These procedures cannot altogether be widely recommended, as incorrect histological diagnoses, tumor cell distribution, multilocular tumor growth and problems with intraoperatively undetected blood vessel invasions as well as lack of prospective randomized data do not allow evidence-based recommendations. If a mature teratoma is histologically proven, there is no need for extensive postoperative diagnostic measures as there is no known case of a mature teratoma causing metastases. In addition, there is no indication for adjuvant therapeutic procedures in cases with similar histological findings and documented R 0-resection.7
In conclusion, this first case report of a torsion of an orthotope descended testis with a mature teratoma shows that testicular tumors must be included in the differential diagnosis of neonatal testicular swelling even if testicular torsion seems probable according to clinical symptoms and signs. According to the recent state of data, even if the rate of organs salvaged is only about 40%, in cases of suspected neonatal testicular torsion, immediate surgical intervention is recommended, especially in newborns presenting with normal diagnostic findings on first clinical examination of the testes.
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Pediatric Surgery International (2016)