Sandro Bosco

Intraneural synovial cyst of the peroneal nerve: Report of two cases and review of the literature

We report two cases of peroneal nerve compression caused by an intraneural synovial cyst and discuss the pathogenesis, clinical and electrophysiological diagnosis, and treatment of these uncommon lesions in the light of the salient published work on the subject.

Clinical relevance of DNA ploidy and proliferative activity in childhood rhabdomyosarcoma: a retrospective analysis of patients enrolled onto the Italian Cooperative Rhabdomyosarcoma Study RMS88.

PURPOSE Evaluation of the possible clinical relevance of DNA ploidy and proliferative activity assessed as S-phase fraction (SPF) in childhood rhabdomyosarcoma (RMS). PATIENTS AND METHODS We conducted a retrospective study on 59 RMS patients enrolled onto the ICS-RMS88 protocol (seven botryoid, 35 embryonal, and 17 alveolar RMS), for which formalin-fixed paraffin-embedded (FFPE) tissue was available. Nuclear suspensions for cytometric investigation were obtained using a mechanical disaggregation. Tumors were distinguished according to their DNA index (DI) value as follows: diploid (0.9 < DI < 1.1), hyperdiploid (1.1 < or = DI < 1.8 or DI > or = 2.2), and tetraploid (1.8 < or = DI < 2.2); for analysis of SPF, a cutoff value of 14% was used. RESULTS DNA histograms were diploid in 19 (33%) cases, hyperdiploid in 29 (49%), and tetraploid in 10 (32%). One patient showed both a hyperdiploid and a tetraploid peak. The 5-year overall survival (OS) rate by ploidy status was 73% in hyperdiploid patients as compared with 33% and 25% in diploid and tetraploid patients, respectively (P = .0012). A striking difference emerged when the 5-year OS for the combined diploid and tetraploid RMS groups was compared with survival of the hyperdiploid RMS group: 30% versus 73%, respectively (P = .0006). In addition, the SPF was prognostically relevant: 5-year OS by SPF less than or greater than 14% was 70% and 36%, respectively (P = .009). Multivariate analysis confirmed the importance of DNA content (P = .0006) and SPF (P = .034) in predicting survival. CONCLUSION These findings confirm that ploidy and SPF are important new prognostic factors that are able to identify selected groups of patients at high risk of treatment failure, even if the tumors presentation is favorable according to standard criteria.

Prenatal testicular torsion: sonographic appearance in the newborn infant

The prenatal diagnosis of spermatic cord torsion is often really difficult and the diagnosis is usually retrospective. Herein, we report a case of a male newborn baby who presented at delivery with an enlarged, swollen and tender scrotum. US showed an enlarged right testis, with dishomogeneous texture, fluid collection between the testis and the tunica vaginalis and large hydrocele. Differential diagnosis included hydrocele complicated by infection or hemorrhage, testicular tumor or postnatal testicular torsion. Color and power Doppler did not reveal any flow signal, and the diagnosis of antenatal torsion with initial necrosis was made. The role of color Doppler US is emphasized in directing the patient to emergency surgical exploration, when testicular salvage may be possible. Delayed surgical treatment can be proposed, when the diagnosis of antenatal torsion has a high degree of certainty. However, the Doppler examination of a newborn babys testis is a very difficult challenge even for an experienced radiologist.

Adenomyomatosis of the Gallbladder in Childhood: Report of a Case and Review of the Literature

ABSTRACTAdenomyomatosis of the gallbladder is considered an acquired pathologic condition that affects adults, with only 2 cases previously described in childhood. We report on a 5-year-old boy with incidental detection of this lesion by ultrasonography. Pathologic and clinical assessments are discussed in addition to treatment decision-making, including current insights into the relation between adenomyomatosis and malignancy of the gallbladder. The liberal use of ultrasonography in children with abdominal pain may result in detection of an increasing number of asymptomatic patients. Caregivers should be aware of this condition and its therapeutic implications in the pediatric population.

Inflammation of the gastric cardia in children with symptoms of acid peptic disease

OBJECTIVES To assess the severity and causes of inflammation of the gastric cardia in children undergoing endoscopy for symptoms of acid peptic disease. STUDY DESIGN Patients undergoing upper gastrointestinal endoscopy for symptoms of acid peptic disease had biopsies from gastric cardia, gastric, and esophageal sites, and 24-hour intraesophageal pH monitoring. Gastric cardia was defined at endoscopy as the anatomic zone from the squamocolumnar junction to 0.5 cm below it. Severity of gastric cardia inflammation was scored 0 to 9 according to densities of inflammatory cells and epithelial abnormalities in surface and pit epithelium. A score > or =2 was considered positive. RESULTS Forty-seven children (median age, 6.5 years; range, 3-15) had Helicobacter pylori infection, gastroesophageal reflux disease (GERD), or both. In 22 patients, H pylori was detected in cardiac biopsies by rapid urease test and histology; it was detected also in the corpus and antrum in only seven of the 22. No patient had H pylori in gastric corpus/antrum without having the organism at the cardia as well. In 12 H pylori-positive patients, GERD was also diagnosed. Twenty-five patients had GERD and no H. pylori infection. Severity score was 3.8+/-0.8 in the H pylori group and 2.08+/-0.9 in the GERD alone group (P<.001); however, there was no difference in reflux index (24-hour % of gastroesophageal reflux) between the two groups. In neither group was correlation found between reflux index and severity score (H pylori, r=0.22; GERD alone, r=0.31; NS) nor between cardia inflammation and esophagitis grade (H pylori, r=0.37; GERD alone, r=0.22; NS). CONCLUSIONS In children with symptoms of acid peptic disease, inflammation of the gastric cardia does occur. It is more severe when the cardiac zone is infected with H pylori than in its absence. Of major practical significance is the finding that the gastric cardia is a highly sensitive site for the detection of H pylori infection.

MDCT classification of steatotic liver: a multicentric analysis.

Purpose Fatty liver disease is the most common cause of chronic liver disease in the western world. The aim of this study was to analyze steatotic liver characteristics using multidetector row computed tomography (CT) to identify reliable criteria to identify the steatosis and quantify its severity. Patients and methods Multiphasic CT scans of 51 consecutive adults (36 men, mean age 57.1±9.9 years), who underwent ultrasound-guided liver biopsy, were analyzed. In all patients, the Hounsfield units (HU) value was determined for each hepatic segment and for each contrast phase. Also, the splenic attenuation was quantified and the differences in the liver–spleen (DLS). Steatosis was graded according its severity into four grades. Receiver operating characteristic (ROC) curve analysis was carried out to calculate the sensitivity and specificity for the specific HU threshold. Pearson’s &rgr; correlation was also calculated. A P value of 0.05 was considered statistically significant. Results We found that 14 individuals (10 men, mean age 56±9.8 years) did not have hepatic steatosis. Only the nonenhanced CT scans showed a statistically significant association with liver steatosis (with the only exception of region-of-interest selected in the Couinaud segment VII, where a P value of 0.0513 was obtained). For grades 1, 2, 3, and 4, we identified 50, 45, 35, and 20 HU as thresholds. A statistically significant association was found between steatosis and DLS in the nonenhanced and the arterial phase (P=0.0192 and 0.001, respectively). Conclusion The result of our study indicates that the nonenhanced value of the liver can be used to identify steatosis of the liver and to grade its severity. Moreover, the DLS in the arterial phase represents another reliable parameter.

Liver splenosis mimicking hepatocellular carcinoma in cirrhotic liver.

Hepatocellular carcinoma (HCC) is the sixth most common cancer and the third cause of cancer-related death (1). Approximately 90% of HCCs are associated with an underlying risk factor, including chronic viral hepatitis (types B and C) (2). Splenosis is a heterotopic implantation of splenic fragments into exposed vascularised peritoneal and intrathoracic surfaces, following splenic injury or elective splenectomy (3). We present a case of a 54-year-old Caucasian man admitted in our unit with the diagnosis of suspect HCC developed on HBV-related cirrhosis. At the age of 29, the patient has been already operated, undergoing an urgent spenectomy owing to blunt trauma after a traffic accident. A computed tomography (CT) scan reported a lesion located at the level of segment III, with the typical radiological characteristics of HCC (Fig. 1A,B). Atypical hepatic resection of the lesion was performed, and patient was discharged after an uneventful post-operative course at day 7. Histopathology showed a 4.5 9 3.5 9 1.5-cm brownish mass with the microscopical aspects of a capsulated spleen (Fig. 2A,B). Autotransplant of splenic tissue may occur anywhere in the body, but is most commonly observed in the peritoneal cavity. Splenosis is easily misinterpreted as a tumour condition. There are few previous reports of hepatic splenosis mimicking hepatocellular carcinoma. Treatment usually requires surgery and confirmation by pathology. In case of patients waiting for liver transplantation, particular attention must be paid in patients with a history of accidental splenectomy.

The use of Tachosil® as hemostatic sealant in nephron sparing surgery for Wilms tumor: Preliminary observations

BACKGROUND TachoSil, which is an absorbable collagen sponge coated with human fibrinogen and thrombin, has proven to enhance hemostasis and promote optimal wound healing in adults undergoing nephron sparing surgery (NSS). We report our preliminary experience using this hemostatic agent during NSS in children. METHODS Prospective analysis of 3 patients with Wilms tumor (synchronous bilateral in one) undergoing NSS at our institution over an 18-month period. Primary outcome measures were intraoperative bleeding control and surgery-related postoperative sequelae. RESULTS Overall, 4 kidneys underwent 5 NSS procedures, which included 3 partial nephrectomies and multiple tumor excisions performed on another kidney at initial and second-look surgery. After control of major bleeding and suturing of the collecting system, which was entered during 2 of the 5 NSS procedures, TachoSil was applied to the parenchymal surface of the kidney, achieving immediate hemostasis. All NSS procedures were performed without hilar clamping, drainage or stent placement. None of the patients required blood transfusion. Small perirenal fluid collections were documented postoperatively, and all spontaneously resolved within 4 weeks of surgery. CONCLUSION In children undergoing NSS, TachoSil represents an effective and safe tool for control of mild to moderate bleeding, and also facilitates sealing and wound dressing.

Intrahepatic Paratesticular Cyst: Unique Presentation of Vestigial Remnants of Wolffian Duct

To report on a case of a progressively enlarging intrahepatic cyst in a 1-year-old boy who also presented with bilateral undescended testes. At surgery, the lesion emerged from the anterior surface of the liver but was unexpectedly found to arise from the epididymis of the right testis, which was located intra-abdominally. The histologic findings were consistent with a paratesticular cyst of vestigial remnants derived from the Wolffian duct. Such an unusual event has not been previously reported among the possible location of a paratesticular cyst, nor has it been described in the differential diagnosis of cystic lesions of the liver.

Immunohistochemical profile of neurotrophins and MIB-1 in jugulotympanic paragangliomas: prognostic value and review of the literature.

Jugulo-tympanic paragangliomas are the most common primary neoplasm of the middle ear, but little is still known about the histological features differentiating the benign and malignant forms. We investigated, with an immunohistochemical procedure, the expression of neurotrophins with their receptors, in fifteen samples of paragangliomas, and MIB-1 in order to consider them as prognostic factors of malignancy. We observed a general positivity for NGF – TrKA – NT4 – TrKC in the cytoplasm, and a strong expression for BDNF in the extracellular space. MIB-1 was moderate in the nucleus of neoplastic cells, weak in the cytoplasm and totally absent in the extracellular space. The comparison between the clinical recurrences and the rate of cytoplasmatic neurotrophins showed strong immunoreactivity in recurrent patients. It should be emphasized that 2 of the 3 recurrences had a wider distribution of the neutrophins, leading to hypothesize the involvement of these substances in the cell proliferation of glomus tumors. Malignant forms of these rare glomus tumors cannot be clearly identified using MIB-1 as a prognostic marker, although we can affirm that neurotrophins and their receptors can be considered as a panel of potential diagnostic markers to monitor the development of such malignancies. Although the small number of patients does not allow definitive conclusions to be made, our findings showed a possible trend towards significance which requires a more powerful study to evaluate this further.