Malignant ovarian germ cell tumors in pediatric patients: The AIEOP (Associazione Italiana Ematologia Oncologia Pediatrica) study.

OBJECTIVE: Malignant ovarian germ cell tumors (MOGCT) carry an excellent prognosis, and the treatment aims to achieve results with the least possible treatment-related morbidity. The aim of this study was to assess the outcomes of pediatric patients with MOGCT. METHODS: Patients were treated according to their stage: surgery and surveillance for stage I; a modified bleomycin-etoposide-cisplatin (BEP) regimen for stages II (three cycles), III, and IV (three cycles) with surgery on residual disease. RESULTS: Seventy-seven patients were enrolled (median age 11.8 years), 26 with dysgerminoma (Dysg), 13 with immature teratoma and elevated serum alpha-fetoprotein levels (IT + AFP), and 38 with nondysgeminoma (Non-Dysg) staged as follows: 27 stage I, 13 stage II, 32 stage III, 5 stage IV. Among evaluable patients in stage I (5-year event-free survival [EFS] 72.1% [95% CI: 56.4-92.1%]; 5-year overall survival [OS] 100%), seven relapsed (three patients with Dysg and four patients with Non-Dysg) and were rescued with chemotherapy (plus surgery in three patients). Among the evaluable patients with stages II-IV, 48 (98%) achieved complete remission after chemotherapy ± surgery, one (IT + AFP, stage IV) had progressive disease. In the whole series (median follow-up 80 months), the 5-year OS and EFS were 98.5% (95% CI: 95.6-100%) and 84.5% (95% CI: 76.5-93.5%). CONCLUSIONS: We confirm the excellent outcome for MOGCT. Robust data are lacking on surgical staging, surveillance for Non-Dysg with stage I, the management of IT + AFP, and the most appropriate BEP regimen. As pediatric oncologists, we support the role of surveillance after proper surgical staging providing cases are managed by experts at specialized pediatric centers.

Laparoscopic management of an isolated left fallopian tube torsion due to twisted Morgagni hydatid in a pre-menarcheal girl.

Isolated torsion of fallopian tube, meanwhile uncommon, should be considered in diagnosis of pelvic and lower abdominal pain. US investigation is an useful diagnostic tool. A prompt diagnosis could avoid salpingectomy and preserve fertility. Laparoscopy, as the first approach, should be preferred. We report a case of isolated tubal torsion occurring in a premenarcheal girl successfully managed by laparoscopy.

Meconial peritonitis in a rare association of partial ileal apple-peel atresia with small abdominal wall defect.

Intestinal atresia type III B (apple peel) and gastroschisis are both congenital malformations who require early surgical correction in neonatal age. Their association is very rare. We present the case of a full term infant with partial apple peel ileal atresia and a small defect of the anterior abdominal wall, complicated by in utero intestinal perforation and subsequent meconial peritonitis. We observed a partial atresia of small intestine, with involvement of terminal ileus savings of jejunum and a large part of the proximal ileum, small anterior abdominal wall defect with herniation of few bowel loops, intestinal malrotation. Paralytic ileus and infections are the main causes of morbidity and mortality at neonatal age. In our case, in spite of the mild phenotype, prognosis has been complicated by the onset of functional bowel obstruction, caused by chemical peritonitis resulting from contact with either amniotic fluid and meconium.

[Predicting factors of vesicoureteral evolution in children]. / Fattori prognostici nell'evoluzione del reflusso vescico-ureterale in età pediatrica.

BACKGROUND: Despite the large number of children with reflux, management among urologists is still controversial. One of the most debated aspects is the choice between observation treatment or surgical treatment. METHODS: We assessed the natural course of children with vesico-ureteral reflux in the period 1990-1995, to correlate factors and identify patients with high risk of renal damage. We retrospectively reviewed the clinical course of 80 children with vesicoureteral reflux. Thirty-two were diagnosed during prenatal ultrasound scan. In the other cases urologic evaluation was requested because of urinary tract infections. The following data were analyzed: medical records, diagnostic and follow-up cystogram, renal imaging, medical therapy or surgical treatment carried out according to the reflux grade, diagnostic age, congenital reflux nephropathy or postnatal acquired scarring, voiding patterns, spontaneous resolution during medical management. Follow up ranged from 5 to 10 years. RESULTS: Vesicoureteral reflux resolved spontaneously in 29 patients: 25 were affected from moderate reflux, 4 from IV grade reflux. Surgical correction was carried out in 32 patients. Endoscopic treatment was performed in 25. Twenty-two children are still receiving prophylaxis and 12 were lost to follow-up. Congenital renal pathology correlate with poor outcome. CONCLUSIONS: The conclusion is drawn that there is a wide clinical variability in children with vescicoureteral reflux. The most important is host's susceptibility to urinary tract infection, but the severity of reflux, age of patients and congenital reflux nephropathy influence prognosis and long-term outcome.

Fibrosarcoma in pediatric patients: results of the Italian Cooperative Group studies (1979-1995).

BACKGROUND AND OBJECTIVES: Fibrosarcoma is a rare soft tissue sarcoma (STS) that has two peaks of incidence in pediatric patients: one in infants and young children (infantile fibrosarcoma), another in older children ("adult type" fibrosarcoma). The purpose of this study was to describe the clinical features and the treatment results in patients affected by fibrosarcoma enrolled in two consecutive studies run by the STS-Italian Cooperative Group (ICG) between 1979 and 1995. PATIENTS AND METHODS: Twenty-five evaluable patients were grouped according the intergroup rhabdomyosarcoma staging (IRS) system: 12 Gr I, 5 Gr II, 8 Gr III. The cut-off point between the two forms was considered the age of 2 years: 14 patients were affected by infantile and 11 by adult type of fibrosarcoma. The therapeutic guidelines were not strict and not different for the two forms: patients with initial macroscopic excision (Gr I-II) were given IVA or VAC; Gr III had VAC or VAIA and subsequent excision, if feasible. Radiation therapy (RT) was delivered in patients > 3 years with microscopic (42 Gy) and macroscopic (54 Gy) residuals. RESULTS: Thirteen patients are alive without evidence of disease: 10 in first complete remission (CR), 7 Gr I, 1 Gr II, 2 Gr III, 3 in second CR after local relapse (LR) (2 Gr I, 1 Gr II). Two Gr I patients are alive with metastatic relapse. Nine patients died: six of progressive disease (PD) (three Gr II, three Gr III), two because of a second tumor (one Gr I, one Gr III) and one because of other causes (Gr III). One Gr III patient was lost at follow up, 2 years from diagnosis. The 10 years overall survival (OS) is 67.4% and the progression-free survival (PFS) is 52.2%. The OS for patients < 2 years is 78.6% (11 of 14), versus 51% for patients > 2 years (5 of 11). CONCLUSIONS: The complete excision at diagnosis was the treatment of choice and was related to the best outcome. Microscopical residuals were difficult to treat with chemo-radiotherapy in both forms of fibrosarcoma. Neoadjuvant chemotherapy (CT) obtained a partial remission (PR) only in three of eight cases, while no conclusions concerning the efficacy of CT for infantile forms are possible. Patients < 2 years had a better outcome than the older ones: most of them had a tumor on extremities which was excised at diagnosis.

Resection of primary tumor at diagnosis in stage IV-S neuroblastoma: does it affect the clinical course?

PURPOSE: To determine whether resection of primary tumor has a favorable influence on outcome of infants (age 0 to 11 months) with stage IV-S neuroblastoma. PATIENTS AND METHODS: Between March 1976 and December 1993, 97 infants with previously untreated neuroblastoma diagnosed in 21 Italian institutions were classified as having stage IV-S disease. Seventy percent were younger than 4 months. Adrenal was the primary tumor site in 64 of 85 patients with a recognizable primary tumor. Liver was the organ most often infiltrated by the tumor (82 patients), followed by bone marrow and skin. RESULTS: The overall survival (OS) rate at 5 years in 80% and event-free survival (EFS) rate 68%. In 24 infants, the effect of resection of primary tumor could not be evaluated because of rapidly fatal disease progression (n = 8), absence of a primary tumor (n = 12), or partial resection (n = 4). Of 73 assessable patients, 26 underwent primary tumor resection at diagnosis: one died of surgical complications, one relapsed locally and died, and two others relapsed (one of these two locally) and survived, for a 5-year OS rate of 92% and EFS rate of 84%. Of the remaining 47 patients who did not undergo primary tumor resection at diagnosis 11 suffered unfavorable events, of whom five died, for an OS rate of 89% and EFS rate of 75% (no significant difference from previous group). Disease recurred at the primary tumor site in only one five who died, and in only one of six survivors of progression or relapse; in these patients, the primary tumor, located in the mediastinum, was successfully resected. CONCLUSION: Infants who underwent resection of the primary tumor at diagnosis had no better outcome than those in whom the decision was made not to operate.

[Neuroblastoma with muscular localization: description of a case]. / Neuroblastoma a localizzazione muscolare: descrizione di un caso.

An unusual case of abdominal neuroblastoma, whose extension involved the psoas muscle, leading to an ultrasonographic and Magnetic Resonance imaging simulating an haematoma, is described. Histology disclosed the malignant nature of the muscle mass. The Authors discuss the value of the new imaging techniques and in particular of Magnetic Resonance in the diagnostic work-up of paediatric malignancies.

[Paratesticular rhabdomyosarcoma: presentation of a case load and considerations on the current therapeutic management]. / I rabdomiosarcomi paratesticolari: presentazione della casistica e considerazioni sugli attuali atteggiamenti terapeutici.

The Authors report their experience in the management of paratesticular rhabdomyosarcomas observed in the last 5 years (3 cases). Clinical and instrumental findings are analyzed; their therapeutic planes and suggestions of National Protocol RMS 88 are compared.