Volvulus of a wandering spleen in a pediatric patient.

Wandering spleen is a rare condition in children that is often caused by the loss or weakening of the splenic ligaments. Its clinical presentation is variable; 64% of children with wandering spleen have splenic torsion as a complication. A 13-year-old boy who had been showing abdominal pain in the hypogastric region accompanied by vomit and an enormous tumefaction in the suprapubic region came to our observation. Considering the ovoid morphology at ultrasound exam, the echostructure and the marked reduction of parenchymal vascularization, suspicion for torsion of an ectopic spleen arose. Ultrasound evaluation has a primary role in the diagnosis of a suspected wandering spleen and, to avoid potentially life-threatening complications, immediate surgery is often times required.

Liposarcoma of the spermatic cord: The state of art and our experience.

Liposarcoma of the spermatic cord is a malignant neoformation so rare that less than 200 cases are reported in the world. It is a tumor that originates from adipose tissue and when it is found in the spermatic cord it can deceptively simulate an inguinal hernia and not be easily identified. The present work describes the case of a 37-year-old man with liposarcoma of the spermatic cord who arrives at our institution with painless swelling of the left testicle. Physical examination revealed a painless swelling in the scrotal sac. The scrotal ultrasound examination revealed a mass, measuring 8 cm (cranio-caudal) × 5.4 cm (latero-lateral) × 8 cm (antero-posterior) and characterized later with a basal CT examination of the abdomen. The patient was subsequently surgically treated with excision of the tumor, plus hernial plastic with plug and mesh. Histological examination revealed a mature adipocyte neoplasm whose morphological and molecular characteristics (amplification of the MDM2 gene) are consistent with the diagnosis of dediferrentiated liposarcoma variety CO-MINGLED, G2 (sec. FNCLCC). The patient is currently under cancer surveillance with no signs of loco-regional recurrence. Spermatic cord liposarcoma is an extremely rare malignancy. It's not easy to identify as it can simulate an inguinal hernia, hydrocele, lipoma, funicular cyst, or testicular tumor. Diagnosis is usually established postsurgery, however, relapses are common and the role of chemo-radiotherapy remains to be defined.

Para-oesophageal hernia in 4-month-old girl: possible role of ultrasound-case report and literature review.

Hiatal hernia is the passage of digestive tract portions into the posterior mediastinum through a defect in the esophageal diaphragmatic hiatus. By guidelines, the diagnosis uses first-level radiographic investigations such as chest X-ray and contrastographic methods. As of today, use of ultrasonography in the suspicion/diagnosis of hiatal hernia is not standardized although it shows advantages such as not using ionizing radiation, which is essential in the small pediatric patient. We report the case of a little 4-month-old patient who came to our attention for dysphagia, vomiting and borborygmus to whom Type II hiatal hernia was suspected by ultrasound investigation, later confirmed by guidelines approved methods.

Pseudopapillary solid tumour of the pancreas in paediatric age: description of a case report and review of the literature.

Pseudopapillary solid tumour of the pancreas is a rare neoplasm that mainly affects young women in the second and third decade of life and less frequently children; originates from the exocrine component of the pancreas; and is characterized by slow growth, low potential for malignancy, and excellent prognosis following complete surgical resection. The tumour often presents as an asymptomatic abdominal mass that is accidentally detected during radiological investigations performed for other reasons. In this article, we report the clinical case of a 10-year-old girl who came to our observation for pain in the left hypochondrium, which had arisen for a week following a trauma; the imaging methods revealed a voluminous expansive pancreatic formation in the abdomen; on histological examination, the mass was a solid pseudopapillary tumour. Furthermore, we present a review of the literature aimed at highlighting the salient features of this neoplasm in paediatric age.

Duodenal hematoma in pediatric age: a rare case report.

In pediatric age, duodenal hematoma is rare and generally occurs following a closed abdominal trauma due to the crushing of the duodenum against the rigid plane of the spine; it rarely follows anticoagulant therapy, pancreatitis, bleeding disorders, vasculitis, tumors or upper digestive endoscopy. Duodenal hematoma is a rare cause of obstruction of the upper gastrointestinal tract and acute pancreatitis, and the diagnosis is sometimes difficult and late. On the other hand, the identification of the pathology in its initial stages allows the young patients to be subjected to a conservative treatment that resolves the issue most of the time, thus avoiding surgery. In this article we describe an unusual case of duodenal hematoma, following esophagus-gastro-duodenoscopy, in a 12-year-old boy with Di George syndrome.

Type II pleuropulmonary blastoma in a 3-years-old female with dyspnea: a case report and review of literature.

Pleuropulmonary blastoma (PPB) is a rare but aggressive pediatric tumor originates from either lung or pleura. It was recently linked to the DICER I mutation as a part of predisposition syndrome for different type of tumor. It is characterized histologically by a primitive, variably mixed blastomatous and sarcomatous tissue. PPB is classified into four subtypes: cystic (type I and type Ir); cystic and solid (type II); solid (type III). PPB has no characteristic imaging findings. Integrated imaging can help to make a differential diagnosis and to recognize the subtypes in order to set up therapy. An early recognition and differentiation from congenital airway malformations and other benign cysts are very important. The treatment consists in a multimodal therapy including surgery and chemoterapy. We report a case of 3 years old female admitted at our hospital with fever, non productive cough and dyspnea, who was diagnosed with type II PPB.

Mediastinal thymoma: A difficult diagnosis in the pediatric age.

Thymoma is a rare neoplasm of the anterior mediastinum, which originates from the epithelium of the thymic gland; it occurs mainly in middle-aged adults and is much less common in children. The tumor has slow growth and is asymptomatic in most pediatric cases, thus resulting in an accidental discovery; one-third of the young patient presents symptoms related to the compression of the tumor mass on the surrounding anatomic structures and/or related to paraneoplastic syndromes. Surgery is the treatment of choice and complete resection of the thymoma achieves excellent long-term results in terms of disease-free survival. In this article, we report the clinical case of a 21-month-old girl who came to our observation for persistent cough for over a month investigated with a chest X-ray, performed in another hospital. The X-ray showed an extensive opacification of the left hemithorax with contralateral dislocation of the mediastinum. The instrumental investigations carried out in our hospital (ultrasound, computed tomography, and magnetic resonance of the chest) showed a voluminous expansive mass of the left antero-superior mediastinum, which occupied the entire ipsilateral hemithorax and not dissociable from the thymus. At the histologic examination, the mass resulted to be a B1 thymoma with a low degree of malignancy according to the histologic classification of thymic tumors of the World Health Organization.

An unusual onset of pediatric acute lymphoblastic leukemia.

Acute lymphoblastic leukemia is the most frequent cancer in children: it represents 80% of leukemias and about 24% of all neoplasms diagnosed between 0 and 14 years. Acute lymphoblastic leukemia mainly affects children between 2 and 5 years old and in this age group the incidence is about 80-90 cases per million per year. In acute lymphoblastic leukemia, cancer cells multiply rapidly and accumulate in the bone marrow and subsequently invade the blood. However, at the time of diagnosis, leukemia rarely occurs outside the bone marrow or blood vessels and the extramedullary involvement happens mostly in patients with refractory or relapsing disease. In this article, we report an unusual clinical presentation of acute B cell lymphoblastic leukemia with intestinal and ovarian localizations in a 5-year-old girl.

A 4-year-old child presenting morning onset of spontaneous tracheal rupture due to bronchial mucous plug occlusion during the nighttime sleep: a case report.

BACKGROUND: Coughing is the most efficient mechanism for clearing mucus and fluid secretions from the airways and its reflex can be suppressed by sleep. Spontaneous tracheal ruptures are believed to result from raised intratracheal pressure against a closed glottis, such as for severe coughing. This is the first reported case of tracheal rupture presented on morning awakening after bronchial mucous plug formation during the nighttime sleep because of an ineffective cough reflex. CASE PRESENTATION: An otherwise healthy white 4-year-old child presented morning onset of dyspnea, chest pain and diffuse swelling of the neck. His history was significant only for nonsevere coughing episodes before his nighttime rest; the child's parents denied any recent fever, weight loss, pains, trauma, bronchial asthma, and sick contacts. A chest X-ray and computed tomography scan revealed pneumomediastinum, obstructive atelectasis of the lower lobe of his left lung, and a small tracheal laceration confirmed by an emergency bronchoscopy. After endoscopic removal of a mucous plug and secretions, the child's pulmonary gas exchange and respiratory rate improved, so our patient was managed conservatively. CONCLUSIONS: This report illustrates an unusual presentation of lung obstructive atelectasis due to a mucous plug manifested by tracheal rupture. This report also highlights the importance of the coughing reflex as one of several defensive mechanisms protecting the airways from the potentially damaging effects of aspirate and accumulated secretions.

Mesenteric lipoblastoma and cervical lipoblastomatosis: ultrasound, elastosonography, and computed tomography findings in two children.

Lipoblastomas are benign tumors of the embryonic lipoid cells mainly occurring in infancy and early childhood. They are clinicopathologically distinguished in two forms: the well-circumscribed and localized type and the diffuse, irregularly confined type with infiltrative growth pattern, also called lipoblastomatosis. We report two pediatric cases of a mesentery localized and cervical diffuse lipoblastomas investigated both with ultrasound and computed tomography examinations.