Focal nodular hyperplasia of the liver: an emerging complication of hematopoietic SCT in children.

Hepatic focal nodular hyperplasia (FNH) is a nonmalignant condition rarely affecting children previously treated for cancer, especially those who received hematopoietic SCT (HSCT). Some aspects of its pathogenesis still remain unclear and a strong association with specific risk factors has not yet been identified. We report here a single institution's case series of 17 patients who underwent HSCT and were diagnosed with FNH, analyzing retrospectively their clinical features and the radiological appearance of their hepatic lesions. We aimed to compare the diagnostic accuracy of ultrasound (US) and magnetic resonance imaging (MRI) and to explore the role of transient elastography (FibroScan) to evaluate the degree of hepatic fibrosis in FNH patients. Our analysis showed an association of FNH with age at transplant ⩽12 years (hazard ratio (HR) 9.10); chronic GVHD (HR 2.99); hormone-replacement therapy (HR 4.02) and abdominal radiotherapy (HR 4.37). MRI proved to be a more accurate diagnostic tool compared with US. Nine out of 12 patients who underwent FibroScan showed hepatic fibrosis. Our study points out that FNH is an emerging complication of HSCT, which requires a lifelong surveillance to follow its course in cancer patients.

CT analysis of ovarian tumors: correlations with laparoscopical and surgical findings.

This study concerns a selected group of 21 patients with ovarian carcinoma in whom CT, laparotomy, and laparoscopy findings could be compared. Considering the loco-regional and distant abdominal sites of involvement, the limits and possibilities of CT in diagnosis and follow-up are outlined.

Further experience with OK-432 for lymphangiomas.

This study includes all the children treated with OK-432 for lymphangioma at our institute. Twenty-nine children treated between 1999 and 2003 are reported for the first time: twelve cases regressed completely, eight cases regressed more than 50% and seven remained unchanged; two cases were lost at follow-up. The outcome was related to the size of the cysts, the larger ones having a better prognosis. The adverse reactions are discussed and the methods of treatment are described in detail. Fifteen children, treated before 1999 and already reported, are reviewed after a long-term follow-up. Four had a recurrence: one regressed spontaneously and three needed further treatment. The other 11 had no complaints. Even considering the risk of recurrence, OK-432 therapy remains our first line therapy for lymphangiomas, avoiding surgery in most cases.

Neonatal ovarian cysts: management and follow-up.

Twenty-seven neonatal ovarian cysts were diagnosed in utero during a 13-year period; 9 were complicated and 18 were initially simple, but 8/18 showed evidence of complications in utero or soon after birth, leaving only 10 simple cysts. One simple and 4 complicated cysts were surgically excised early in the study period; 2 simple cysts were treated by needle aspiration at birth because of their large size. The remaining 20 cases were initially managed conservatively. All simple cysts and 10/13 complicated cysts regressed completely within 12 months; 3 that failed to regress were surgically excised. Nineteen patients who did not undergo a laparotomy had ultrasonographic (US) examinations from 3 months to 9 years after birth. Echography showed both ovaries in all 9 girls who had simple cysts at birth and in 2 of the 10 with complications. In the other 8 complicated cases only one ovary was detected at follow-up. Pre- or post-natal aspiration of simple cysts 4 cm or more in diameter is recommended, while conservative management seems appropriate in the absence of symptoms for simple cysts of less than 4 cm and complicated cysts of any size tending to involute after birth.

[Diagnostic role of echography in acute appendicitis in children]. / Ruolo diagnostico dell'ecografia nell'appendicite acuta del bambino.

Eighty-seven pediatric patients with suspected acute appendicitis underwent high-resolution US with graded abdominal compression. The study was limited to the patients with a questionable clinical diagnosis, accounting for about 40% of the patients examined for acute appendicitis in our Institution. US had 87.3% accuracy, 81.5% sensitivity, and 90% specificity. The main US findings in the positive cases were: visualization of the appendix as a tubular non-compressible structure, with a diameter of 5 mm or more, symmetric/asymmetric wall thickening, possible presence of appendicoliths and variable appearance of the central echogenic layer (preserved, doubled for lumen dilatation, partially/totally lost). The above US findings were grouped in 3 basic patterns: type I (thickened appendix with no structural abnormalities) appeared to be related to non-suppurative and phlegmonous acute appendicitis; type II (detectable appendiceal abnormalities) was observed both in phlegmonous and in suppurative acute appendicitis; type III (pericecal complex mass, frequently with appendicoliths) was found in all cases of periappendiceal abscess. In our experience, the use of US in the diagnosis of acute appendicitis in children allowed a reduction by about 2/3 in the rate of unnecessary laparotomies. Such a finding emerges from the comparison with the results obtained in the 2 years prior to the use of US. The technique also allowed an unquestionable diagnosis of acute appendicitis or periappendiceal abscess to be made in a number of clinically equivocal cases, thus avoiding potentially harmful delays in diagnosis. On the other hand, the incidence of false-negatives on US is not negligible, which calls for a cautious clinical and US evaluation of all equivocal cases following no typical US pattern.

Pancreatic cancer: CT scan.

The results of 72 cases of histologically confirmed pancreatic neoplasm are reviewed. By applying a semeiological scheme, which considers intra and extrapancreatic alterations, it is shown that the possibility of a pre-invasive diagnosis on CT is still limited, and requires further confirmation. CT examination is unique for demonstrating the extension of the lesion, and provides truly probative findings for a specific diagnosis, especially when signs of direct local or distant invasion appear.

[Behavior of T1 relaxation time in normal and neoplastic pulmonary tissue. An in vitro study]. / Comportamento del tempo di rilassamento T1 nel tessuto polmonare normale e neoplastico. Studio in vitro.

The aim of the study was to look for a difference between normal and neoplastic lung tissue by means of Magnetic Resonance Spectroscopy in vitro. A 2.1 Tesla intensity spectrometer was used. From 23 pneumonectomized patients, two samples from the lung were taken immediately after surgery: one from the center of the neoplasm, one from the healthy surrounding tissue; on each of them T1 relaxation time was measured. Twenty patients out of 23 had a higher T1 relaxation time in the pathologic tissue than in the healthy surrounding tissue; only in 3 cases the T1 of neoplastic tissue was lower than that of the healthy tissue. A correlation was also sought between the T1 value and the histologic type of the neoplasm. In epidermoid carcinoma, the T1 relaxation time increased with the malignity of the neoplasm.

Fetal gallstones: sonographic and clinical observations.

Fetal gallstones were visualized sonographically in two cases at 30 and 37 weeks' gestation; follow-up scans at 2 and 3 months, respectively, disclosed their spontaneous resolution. The hypotheses advanced to explain the formation of echogenic material in the fetal gallbladder are reviewed, and the clinical utility of postnatal long-term sonographic follow-up is stressed.

Testicular cystic dysplasia: evaluation of 3 new cases treated without surgery.

PURPOSE: We describe 3 cases of testicular cystic dysplasia that were diagnosed only by sonography to avoid an invasive approach. MATERIALS AND METHODS: Three patients 5, 8 and 12 years old, respectively, had increased testicular volume and/or intermittent pain. Sonographic examination of the testis by high frequency (7.5 mHz.) probes showed the typical onset of testicular cystic dysplasia, characterized by several small focal or diffuse intraparenchymal cystic formations. RESULTS: Biopsy or orchiectomy was not considered. At 16, 18 and 24 months of followup, respectively, testicular pain was absent in our 3 cases and sonographic findings were unchanged. CONCLUSIONS: Clinical and sonographic followup is considered sufficient to evaluate possible changes in the clinical course of this pathological condition which, although benign, still remains to be defined.

Congenital absence of the portal vein associated with focal nodular hyperplasia of the liver and cystic dysplasia of the kidney.

Congenital absence of portal vein is a rare malformation. To date, 16 cases have been reported--all in association with other anomalies, i.e. benign or malignant hepatic neoplasms in 6 cases and cardiac malformations in 12. This case report described a girl with congenital absence of portal vein, focal nodular hyperplasia of the liver and cystic kidney dysplasia. Angiography showed the splenic vein and superior mesenteric vein joining to form a common trunk that entered the inferior vena cava directly above the liver. A review of the other cases in the literature is provided and the clinical aspects of our patient are discussed.