A large undifferentiated sarcoma of the liver in a 13-year-old girl treated with anatomical resection: a case report and review of the literature.

BACKGROUND: Undifferentiated embryonal sarcoma of the liver (UESL) is a rare liver tumor accounting for 6-13% of primary liver tumors. Accurate preoperative diagnosis is difficult, with a rather high misdiagnosis rate. Herein, we reported a very large UESL treated with anatomical resection. Our case is amongst the largest pediatric UESLs in the literature. CASE PRESENTATION: Herein, we report a 13-year-old girl presenting with right upper quadrant abdominal pain, postprandial vomiting, and abdominal distention, in which radiographic imaging demonstrated a huge UESL (28 × 20 × 12 cm). The patient was treated with partial hepatectomy and the 5 kg tumor was removed. The patient was discharged in good condition, with no significant complaints in her follow-up. CONCLUSIONS: Although different treatment strategies have been reported for UESL cases, anatomical resection is still the main treatment approach, especially for large tumors.

Effects of partial internal biliary diversion on long-term outcomes in patients with progressive familial intrahepatic cholestasis: experience in 44 patients.

PURPOSE: Progressive familial intrahepatic cholestasis (PFIC) is a hereditary disease characterized by cholestasis, which may cause jaundice, severe pruritus, and cirrhosis in the later stages. By the invention of biliary diversion methods, these patients were prevented from undergoing liver transplant. Using biliary diversion techniques, the entero-hepatic cycle was interrupted. This lowers the bile acid pool and resolves the pruritus. Herein, we report 44 cases of PFIC who underwent partial internal biliary diversion (PIBD) and long-term follow-up of these children. This comprises the largest case series of PIBD. METHODS: All patients were diagnosed by liver biopsy as PFIC before the operation. All underwent cholecysto colic bypass by jejunal interposition due to severe pruritus unresponsive to medication. Laboratory blood tests, sonography, and physical exam were done before and after the operation once every 3 months. Besides, a questionnaire was designed to ask the patients about the symptoms after the operation, and a pruritus score was measured using the 5D-itch scale. RESULTS: 44 children (25 boys, 19 girls), between 1.75 and 27.5 years (at the time of this study) were followed for a median period of 54 months. Age at operation ranged from 2 months to 18 years, with a median of 29 months. Of these children, 14 were lost to follow up. Results showed a significant decrease in pruritus and sleep disturbance after the surgery (p < 0.001). Also, jaundice decreased from 82.1 before to 7.1% following the surgery. 50% of the patients became medication-free at follow-up. CONCLUSION: PIBD is a safe procedure which helps non-cirrhotic children preserve their liver function. Therefore, PIBD prevents them from undergoing liver transplant. Effective results were achieved in terms of severe pruritus and jaundice, and children were able to regain their sleep patterns. It also avoided external stoma, which is more convenient from the patient's point of view.

Large congenital cervical mass in a neonate: prenatal diagnosis and postnatal management of teratoma: a case report.

INTRODUCTION: Cervical teratomas are rare congenital neoplasms that can cause neonatal airway obstruction if large. CASE PRESENTATION: The female Persian neonate displayed respiratory distress at birth, with a 7 cm × 8 cm cystic solid mass identified on the left side of the neck. Antenatal ultrasonography revealed polyhydramnios. Despite initial stabilization, the infant required intubation and mechanical ventilation due to persistent respiratory distress. Imaging confirmed a cystic mass compressing the trachea, ruling out cystic hygroma. Surgical resection on postnatal day 17 revealed a 10 cm × 10 cm solid cystic structure, histologically identified as an immature teratoma. CONCLUSION: Despite risks of poor fetal and postnatal outcome from large cervical teratomas, early surgical resection after airway stabilization can result in recovery. Proper multidisciplinary management of respiratory distress from such tumors is paramount.

Large Retroperitoneal Teratoma Presenting with Unilateral Hydronephrosis in an Infant: A Case Report and Review of the Literature.

Teratoma is a type of germ cell tumor layer that appears in the gonadal, sacrococcygeal, mediastinal, and retroperitoneal regions. Primary retroperitoneal teratoma is rare and asymptomatic but can present with symptoms due to a mass effect on neighboring organs. These tumors have to be considered in the differential diagnosis of a mass in the abdominal cavity of children to distinguish between Wilms' tumor, neuroblastoma, and other intra-abdominal lesions. We presented an infant boy with protrusion of the left upper quadrant of the abdomen and a palpable abdominal mass that had progressively enlarged. An abdominal computed tomography scan revealed a large retroperitoneal cystic, solid mass on the left side of the abdominal cavity, causing pressure on the left ureter. Also, hydronephrosis of the left kidney was seen with a decreased enhancement of the left kidney due to obstruction uropathy. The mass was suspicious on imaging for a retroperitoneal teratoma. The patient underwent laparotomy, and excision of the huge retroperitoneal mass was done. The final diagnosis was an immature teratoma grade 3, and the patient was discharged in good condition. Retroperitoneal teratomas are rare tumors in infants. These tumors would be an incident diagnosis or diagnosed by the mass effect of giant tumors on other organs. They must be considered in the differential diagnosis of intra-abdominal tumors in children. Hydronephrosis and obstructive uropathy can be rare consequences of the mass effects of these tumors.

Giant non-parasitic splenic cyst: a case report.

BACKGROUND: Splenic cysts are quite rare and asymptomatic. They may result from infection by a parasite, especially Echinococcus granulosus (hydatid cyst), or from non-parasitic causes. Since primary splenic cysts are not common, simple cysts can be misdiagnosed with a hydatid cyst in endemic areas. CASE PRESENTATION: We reported a 14-year-old Iranian girl initially presented with a vague abdominal pain, which progressed to left shoulder pain, fullness, early satiety, and shortness of breath and remained undiagnosed for 7 months despite seeking medical attention. Finally, imaging revealed a massive splenic cyst measuring 220 mm × 150 mm × 160 mm raising concern for a hydatid cyst due to regional endemicity. Consequently, the patient underwent total splenectomy. However, histopathological examination surprisingly revealed a simple non-parasitic cyst. CONCLUSIONS: Detecting rare simple spleen cysts requires early ultrasonography (US) and careful reassessment of diagnoses for non-responsive or worsening symptoms. Distinguishing them from splenic hydatidosis, especially in endemic areas, demands thorough paraclinical evaluations and patient history regarding potential parasitic exposure. While total splenectomy is the primary treatment for these huge cysts, the optimal surgical approach should be tailored case by case. These insights emphasize a comprehensive diagnostic approach to enhance accuracy and optimize patient care for these uncommon cysts.

Management of Asymptomatic Perforation of a Pediatric Rectal Foreign Body into the Peritoneal Cavity Retrieved with Laparoscopy: A Case Report and Review of the Literature.

Rectal foreign body insertion has had an increasing trend throughout the years, whereas it is rarely reported in pediatrics. The management and treatment of these cases can become challenging, since it also can present with atypical or even no symptoms in physical evaluation. A 14-year-old boy was referred to our hospital with a history of insertion of a paintbrush into his anus four weeks before the admission. The paintbrush had perforated the colon and was in the abdominopelvic cavity; however, no symptoms of peritonitis were observed. Rectal examination, sigmoidoscopy, and colonoscopy were unremarkable. Exploratory laparoscopy was performed, and the paintbrush was taken out completely. The patient was discharged in good condition. In cases with rectal foreign body insertion, perforation without causing peritonitis or acute abdomen is possible. In these conditions, imaging examinations play an essential role in managing the patients, and laparoscopy can be a proper procedure for retrieving the foreign body.

A COVID-19 Patient Presenting with Pneumothorax.

Severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) is a novel virus that has affected millions of individuals across the world. It was officially declared as a pandemic on March 11, 2020. Although most patients with COVID-19 manifest as viral pneumonia characterized by symptoms such as fever, dyspnea, and cough, atypical presentations such as acute respiratory distress syndrome (ARDS) and acute kidney or cardiac injury have been reported amongst COVID-19 patients. Bilateral and peripheral ground-glass and opacities are the hallmarks of COVID-19 infection on imaging exams. Herein, we aim to describe a COVID-19 patient who presented with shortness of breath, neck pain, chest pain, and bilateral pneumothorax in his imaging exam.