Solid-pseudopapillary tumor of the pancreas in a 13-year-old girl-case report

Background: The solid-pseudopapillary tumor (SPT) of the pancreas is a rare type of exocrine pancreatic neoplasm. SPT predominantly affects young women and female children, and is usually discovered incidentally. This tumor is generally benign with a low incidence of malignancy. Case Report: A 13-year-old girl was admitted to the hospital with a few weeks' history of mild abdominal pain and jaundice. On physical examination, there was no palpable mass. The laboratory tests showed increased SR, CRP, high bilirubin, amylase and lipase serum levels. Ultrasound imaging revealed a solid lesion in the region of the pancreatic head. On MRI, precise tumor localization in the head of the pancreas with pancreatic duct dilatation and compression of the common bile duct were visualized. Pancreaticoduodenectomy and cholecystectomy was performed with good clinical outcome. Microscopic and immunhistochemical studies indicated that tumor cells were typical of SPT without any signs of malignancy. After surgical treatment, the patient in good condition was discharged from the hospital. Conclusions: Magnetic resonance imaging is an excellent and safe modality for detection and characterization of SPT

An uncommon cause of well-known symptoms: acute abdomen in a 2-year-old boy with intestinal malrotation. Case report and literature review

Midgut malrotation is the most frequent congenital defect of the small intestine. The clinical manifestationcan vary from being asymptomatic to presenting acutely as volvulus with bilious vomiting.Presented here is a case of a 32-months-old boy with abdominal pain and several emetic episodes beforeadmission. The patient was diagnosed with ileus and needed emergency surgery which showed thepresence of volvulus due to malrotation of the midgut. Extensive partial resections of the jejunum, ileumand colon beginning in the upper part of the rectum up to ascending colon were performed.Intestinal malrotation is rarely a symptomatic abnormality, however, when it occurs severely it can resultin life-threatening complications. Ultrasonography may be a helpful screening tool for early diagnosis,but it needs the experience of the doctor. Treating significant malrotation almost always requires surgery.The timing and urgency depend on the child’s condition

Totally implantable central venous access ports

Całkowicie implantowane systemy dożylnego podawania leków (tzw. porty dożylne) stanowią niezwykle dogodne rozwiązanie u chorych poddanych chemioterapii. Metoda ta znajduje coraz szersze zastosowanie. Rocznie 700-1000 dorosłym chorym w Polsce wszczepia się port donaczyniowy. Z inicjatywy krajowego konsultanta w dziedzinie onkologii klinicznej odbyło się spotkanie uzgodnieniowe w celu ustalenia zasad bezpiecznego stosowania portów w Polsce. W spotkaniu uczestniczyło 26 ekspertów z 17 ośrodków onkologicznych. Rezultatem spotkania stało się uzgodnienie zasad przedstawionych w formie standardów i zaleceń. Uzgodnienia dotyczyły wskazań do zastosowania portów, kwalifikacji chorych, warunków technicznych implantacji i okresu obserwacji po zabiegu, zapobiegania i leczenia powikłań infekcyjnych i powikłań zakrzepowych, szkolenia personelu obsługującego porty, informacji przekazywanej chorym.Totally implantable central venous access ports are very useful in chemotherapic treatment. It gains wider application. In Poland they are implanted in 700-1000 patient annually. National Consultant in Clinical Oncology at meeting with 26 experts from 17 oncologic centers established the principles of safe implantation of ports in Poland. The meeting resulted in report on standards and recommendations containing: recommendation on ports application, patients’ qualification, implantation technics and follow-up observation, prevention and treatment of infection and thrombotic complication, professional staff training and information for patients

Clinically Malignant Breast Lesion in an Adolescent Girl: A Case Report

Diseases of the breast in adolescent women are usually benign, and their treatment is simple using appropriate medical strategy and rarely surgical therapy. The whole team’s real challenge is when the girl presents malignant breast cancer symptoms such as a non-movable tumor, nipple discharge, nipple retraction, retraction of the skin, inflammatory infiltration of the breast, or ulceration. Presented here is a case of a 15-years-old girl with the features of a malignant neoplasm of the breast. There was an observed non-movable tumor, retraction of the nipple, inflammatory infiltration, and pain. The performed ultrasound and magnetic resonance imaging suggested a malignant lesion measuring 84 mm × 66 mm × 50 mm. After many diagnostic difficulties, the lesion evacuated spontaneously, and the abscess was diagnosed. In conclusion, not all features of a malignant breast tumor in adult women are typical for adolescent females. In young girls, breast diseases are usually benign, and appropriate diagnostics and therapy allow for an effective cure. Atypical breast lesions require the extraordinary cooperation of a multidisciplinary team

An Evaluation of the Efficacy of Selective Alpha-Blockers in the Treatment of Children with Neurogenic Bladder Dysfunction—Preliminary Findings

The aim of this study was to assess the usefulness of selective α1-blockers in children with neurogenic urinary tract dysfunctions and increased leak point pressure (LPP). 14 children from age 6 to 16 years with neurogenic urinary tract dysfunctions (neurogenic bladder) and LPP > 40 cm H2O were enrolled in the study. All patients received a selective α1-blocker (doxazosin) for 6–8 weeks with an initial dosage of 0.03 mg/kg. During the observation period the continuation of oral anticholinergics, Clean Intermittent Catheterization (CIC), observation of “urinary dryness” and urinary incontinence periods were recommended. Patients were scheduled for a follow-up visit and urodynamic investigation after 6–8 weeks after the doxazosin therapy was started. In 4 patients, urine leakage occurred at lower pressures; in 9 patients, no significant changes in urine leak point pressures were detected; in 3 patients, there was a significant increase in the bladder capacity; in one patient, deterioration in continence was noted. The differences both in LPP and LPV before and after the treatment were not statistically significant. Our observations are consistent with the conclusions from other studies and showed no evident efficacy of doxazosin in children with neurogenic bladder

Guz Frantz rzadką przyczyną bólu brzucha u dzieci – opis przypadku

The Frantz tumor (solid pseudopapillary neoplasm – SPN) is a rare, usually benign tumor of unclear etiology having a predilection for young women. This usually asymptomatic tumor may, however, be a cause of abdominal pain or discomfort. In our case, the mentioned tumor was a cause of abdominal pain in a 14-year-old, a so far healthy patient. Abdominal imaging – USG and computed tomography scan (CT) were performed showing a large mass in the left epi- and mesogastrium with primary location probably in the pancreas. A laparoscopic biopsy was performed and confirmed the initial diagnosis. A tumor along with the pancreatic tail were removed by laparotomy without complications. The case we described shows that a solid pseudopapillary neoplasm can be a rare cause of abdominal pain in pediatric patients.Guz Frantz (lity pseudobrodawkowaty nowotwór trzustki, guz Hamoudiego) jest rzadkim, zwykle łagodnym guzem o nieznanej etiologii, występującym głównie u młodych kobiet. Guz ten jest zazwyczaj bezobjawowy, może być jednak przyczyną bólu lub dyskomfortu ze strony jamy brzusznej. W opisywanym przez nas przypadku guz był przyczyną bólu brzucha u 14-letniej, zdrowej dotychczas pacjentki. W wykonanych badaniach obrazowych – ultrasonografii oraz tomografii komputerowej – uwidoczniono dużą masę guza w nadbrzuszu i śródbrzuszu lewym, którego punktem wyjścia była najprawdopodobniej trzustka. Wykonano biopsję metodą laparoskopową, potwierdzającą wstępne rozpoznanie. Guz wraz z ogonem trzustki usunięto metodą laparotomii bez powikłań. Opisany przez nas przypadek pokazuje, iż lity pseudobrodawkowaty guz trzustki może być rzadką przyczyną bólu brzucha u pacjentów pediatrycznych

Pentalogy of Cantrell: Complete expression with mediastinal teratoma

Pentalogy of Cantrell (POC) is a rare, and often fatal congenital disorder that is characterized by a pentad consisting of ectopia cordis, omphalocele, sternal cleft, congenital diaphragmatic hernia, and various intra-cardiac defects. Although the hallmark of POC consists of these five anomalies, only a handful of cases have been reported with the full spectrum of this disorder. This case report presents a full term female with complete expression of POC and a mediastinal teratoma. Two days after birth, this infant underwent correction of the omphalocele and diaphragmatic defect, with repositioning of the cardiac apex within the thoracic cavity. Three months later surgical correction of the intra-cardiac defects took place. At initiation of cardiac by-pass a mediastinal mass at the superior cavopulmonary junction was identified and excised. This mass on histopathology was a teratoma, which makes this case unique as the occurrence of POC and mediastinal teratoma is unreported. This infant has survived the series of corrective surgeries, and is now functioning well. Conclusion: when POC is suspected further investigation for associated anomalies is required for a planned multidisciplinary surgical approach combined with neonatal intensive care to afford the opportunity for a successful outcome

The Important Role of the Radiologist in Determining the Indications for the Surgical Treatment of Neuroblastoma with Vascular Image-Defined Risk Factors: A Case Report

The International Neuroblastoma Risk Group Staging System (INRGSS) is based on the age of patients and preoperative imaging, with attention paid to whether the primary tumor is affected by one or more of specific image-defined risk factors (IDRFs). This publication presents a 2.5-year-old boy with neuroblastoma who had an accidental ligation of the celiac trunk during tumor resection. The consequences of this complication were pancreatic and spleen ischemia and necrosis, ischemia, and perforation of the common bile duct, gallbladder, stomach, and duodenum. The aim of this publication was to highlight the great role of the radiologist in determining the indications for neuroblastoma tumor removal, especially with current vascular IDRFs, and to show how the radiologist’s insightful approach can save the patient from irreversible complications

Clinical, Histological, Cytogenetic and Molecular Analysis of Monozygous Twins with Wilms Tumor

The familial occurrence of childhood cancers has been proven for a long time. Wilms’ tumors often do not have a clear germline genetic cause. However, approximately 2% of all nephroblastoma cases are familial. Descriptions of twins with the same cancer are extremely rare, so our aim was to present the background of the available literature of the occurrence of Wilms’ tumor in a pair of monozygotic twin girls with detailed clinical, histological, and molecular analysis. Two twins were born of unrelated Caucasian parents. Family history revealed no known chronic diseases or malformations. At the age of 3.5 years, the first twin was admitted to the emergency department due to hematuria and abdominal pain. Ultrasound examination revealed an enlarged right kidney, 12.8 cm, with a mass in the upper pole measuring 56 × 69 × 78 mm. The second girl was referred for an abdominal ultrasound, which revealed a right kidney measuring 8.6 cm with a central mass measuring 54 × 45 × 41 mm. Both children underwent surgical resection, and the histopathological result showed a mixed form of nephroblastoma, predominantly epithelioid with residual blastemal compartment. Detailed clinical, histological, cytogenetic, and molecular analyses were performed on both sisters. It was also decided to identify environmental factors. Information was obtained that the girls’ parents run a farm and regularly use pesticides and chemical rodenticides. Based on our observations and the available literature, Wilms tumor in monozygotic twins may be present. Both genetic and environmental factors may be involved in the development of tumors. After excluding methylation abnormalities and mutations in the genes studied, we questioned whether the onset of Wilms tumor in both sisters could be the result of exposure of the twins’ parents to pesticides