Extrarenal testicular Wilms' tumor in a 3-year-old child.

We report an extremely rare case of extrarenal testicular Wilms' tumor in a 3-year-old boy with intrabdominal undescended left testis. The patient was admitted because of pain and vomiting, with evidence of a huge abdominal mass. At surgery a large tumor arising from the intrabdominal testis was found. Histology showed the classical triphasic Wilms' tumor elements: epithelial, mesenchymal and blastemal areas. Extrarenal Wilms' tumors account for only 3% of all Wilms' tumors and just -100 cases have been reported in literature. Testicular origin is anecdotic. We present histomorphological, histogenetic, clinical, diagnostic, prognostic and therapeutic features of this rare tumor.

[Cryptorchidism: medium- and long-term follow-up].

AIM: Cryptorchidism represents the most frequent male genital anomaly in paediatric population and may potentially interfere with fertility and determine neoplastic testicular diseases. We wanted to evaluate the correlation between age at orchiopexy and follicle-stimulating hormone (FSH), luteinizing hormone (LH) and testosterone levels in adulthood, determining the long-term complications of surgical treatment. METHODS: Fifty-seven patients (mean age 19 years, range 18-27) surgically treated for cryptorchidism in pediatric age were included in a medium and long-term follow-up (10-19 years). We divided this population into four groups: A) monolateral cryptorchidism operated on before 36 months of age (15); B) monolateral cryptorchidism operated on over 36 months (32); C) bilateral cryptorchidism operated on before 36 months (5); and D) bilateral cryptorchidism operated on over 36 months (5). All patients underwent andrological examination, testosterone, FSH and LH dosage, measurement of testicular volume and spermiogram. RESULTS: Significant different FSH levels were found between group A and C and between A and D (P<0.01), while groups A and D presented also different mean testicular volume (P<0.01). In addition group D showed an abnormal morphology of spermiogram. The main complications found in follow-up were hydrocele (17,5%), varicocele (8,7%) and epididymal cysts (3.6%). CONCLUSION: Monolateral cryptorchidism is associated with normal fertility when treated early (group A). Subjects in Group D, on the contrary, have a rise of FSH, a reduction of testicular volume and semen abnormalities. The long-term follow-up of these patients can also detect associated.

Surgical treatment of toraco-abdominal teratoma diagnosed in utero, approached with mini-invasive technique: a case report.

Thoracic tumours of childhood arise either in the mediastinum, or from the chest wall and rarely from the lung parenchyma. Mediastinal teratoma occur in the anterior mediastinum and are usually very large at presentation; initial treatment is under discussion, but it appears that surgical excision and histological examination are the treatments of choice and it will determine whether the lesion is benign or malignant. The authors report a case of thoracic-abdominal teratoma, diagnosed in utero, and treated successfully with thoracoscopy and laparoscopy. No complication was described in short follow up, and the child was discharged in day 8th postoperative day. Histology showed mature teratoma. Follow-up at one year: no evidence of recurrence. Due to the rarity of multifocal teratoma in newborns, author believe that mini-invasive technique was useful to stage histological the mass, giving the opportunity to plans a second stage in treatment of the patient. Day of hospitalization resulted shorter in both surgical approaches, and discomfort less for the thoracic approach. This staging must be anyway planned, in the way of prenatal counselling and in the perinatal period, with the team involved in the procedure, that include anaesthetists, oncology staff, obstetricians, neonatologists and pediatric surgeons. It is mandatory in cases like the one reported that a multisciplinary team manage the patients and expert surgeons, especially in minimal invasive surgery, make the adequate plans.

Delayed meconium passage in very low birth weight infants.

BACKGROUND: Delayed meconium passage, typical of premature newborns, is a predisposing condition for bowel perforation with a significant risk of morbidity and mortality. AIM OF THE STUDY: A retrospective study was undertaken to verify the entity of the disease, assess the average time to meconium passage in a neonatal population of very low birth weight (VLBW) infants, and identify associated risk factors. METHODS: The time of first stool passage was studied in 110 VLBW infants (weighing less than 1500 g at birth). Their perinatal features, clinical course, and treatment were reviewed and studied retrospectively. RESULTS: Delayed meconium passage was recorded in 81% of this group. Patent ductus arteriosus, mechanical ventilation and uteroplacental insufficiency were significantly associated with delayed passage. An inverse relationship between gestational age, birth weight and meconium passage was found. Bowel perforation occurred in 4.5% of this neonatal population with a mortality of 50%. CONCLUSIONS: In very low birth weight infants delay in the passage of the first stool is common. Perforation in these patients may represent a fatal event, and procedures such as daily rectal enemas, which can prevent this complication, must be applied.

Ectopic thymus presenting as neck mass in a neonate: a case report.

Neck masses are rare in newborns and can represent a big challenge for the paediatric surgeons. Different histological types are reported in the recent literature; while 80% of neck masses are benign, the pediatric surgeon is often called upon to assist in the evaluation, diagnosis and treatment. A two-month old baby was evaluated for an asymptomatic left sided cervical mass, presented at birth, which slowly increased in size. A magnetic resonance imaging scan was performed, with evidence of capsulated solid neck mass, lateral to the sternocleidomastoid muscle, not including the major vassel structures of the neck. The patient underwent surgical excision in the criteria to define the histology of the mass. A well capsulated mass was detected at surgery. At histology, an ectopic cervical thymus tissue was reported. The ectopic cervical thymus is an uncommon entity with only 91 cases reported; only nine occurred in infants, two in the neonatal period. According to the authors, in case of enlarging solid neck mass with unclear imaging or clinical data, complete surgical resection is the only and safe procedure to perform.

Fulminant multiorgan failure due to varicella zoster virus and HHV6 in an immunocompetent adult patient, and anhepatia.

Varicella is a well-known contagious disease of childhood that can also affect both immunodepressed and immunocompetent adults. The present observations concern a previously healthy adult patient who presented with a fulminant hepatitis evolving in multiorgan failure (MOF), associated with an atypical papulo-ethemateous cutaneous rash without fever. An hepatic biopsy showed massive necrosis. Because of the persistent MOF and severe hemodynamic instability, total hepatectomy was performed as a bridge to urgent liver transplantation (OLT). Despite temporary improvement, the patients condition progressively deteriorated and he died 11 hours after the hepatectomy, i.e. 7 days after admission to the intensive care unit. High viral loads of varicella zoster virus (VZV) and human herpes virus 6 (HHV6) were demonstrated in the blood and in DNA at post mortem examination of the liver, kidneys, lung, and heart. We hypothesize that VZV infection may occasionally occur in immunocompetent patients due to extremely virulent strains that can be rapidly fatal. The clinical influence of simultaneous infection with HHV6 is not clear. Moreover, the role of a previous steroid treatment as a trigger for a temporary immunodepressed state must be considered. The diagnosis of liver disease from VZV should always be clinically suspected in the presence of concurrent atypical skin lesions and a temporarily immunocompromised state. Therapy with acyclovir was ineffective in our patient. Based on the wide spectrum of VZV infections, fulminant MOF in immunocompetent adults must raise the possibility of VZV with simultaneous HHV6 infection with early listing of the patient for a urgent OLT, possibly with a total hepatectomy as a bridge, due to the therapeutic uncertainty of medical treatments.