A rare case of bilateral testicular metastasis from ileocecal NET: multiparametric US detection.

Testicular metastasis are rare findings and bilateral metastasis of testes are extremely rare. Here we are describing for the first time a case of bilateral testicular metastasis in a patient with a known ileocecal valve NET using an in-depth ultra-sound studying including microvascular flow imaging (MV-flow), ultra-sound new technique, able to detect small vessel slow-signal.

Integrated imaging of systemic Langerhans cell histiocytosis in an infant.

Langerhans cell histiocytosis (LCH) is a myeloid neoplasm characterized by a clonal proliferation of CD1a+/CD207+ dendritic cells. Although individuals of any age can be affected, the disease is most common in infants younger than 5 years of age, especially males. A wide range of manifestations, from asymptomatic to aggressive, have been described, along with multiorgan involvement. Even though the majority of bone lesions are observed, skin, lymph nodes, brain and lungs can also be involved. The involvement of hematopoietic system, including bone marrow, liver and spleen, is less frequent yet associated with worse prognosis, due to a worse treatment response. Diagnosis of LCH is based on the integration of clinical, laboratory, and radiological data; however, only histopathological examination might confirm it. As far as the spleen involvement is concerned, according to literature, it has been reported in about 15% patients with multisystem involvement, nonetheless only a few cases show parenchymal lesions. The present study reports the case of an infant with LCH with multisystem involvement, including bone, skin, liver, and spleen, with evidence of parenchymal lesions.

Giant malignant sacrococcygeal germ cell tumor in a newborn: A rare case report.

Malignant germ cell tumors constitute about 3%-4% of all neoplasms occurring before the age of 15. They arise in the ovaries, the testes, and in several other locations, including the lower back, the chest, the brain, and the abdomen. In infants and young children, the sacrococcygeal region is the most common site for extragonadal germ cell tumors, and teratomas account for the vast majority of sacrococcygeal germ cell tumors. Neonatal sacrococcygeal teratomas are usually benign and rarely they may contain a malignant component that is predominantly a yolk sac tumor. In this article, we describe a rare case of a male newborn with a giant sacrococcygeal mixed germ cell tumor composed of grade 3 immature teratoma and malignant yolk sac elements.

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.

Early diagnosis of Meigs syndrome in children A case report and a review of the literature.

Meigs syndrome is a rare disease defined by the coexistence of benign ovarian neoplasm, ascites and hydrothorax, which mainly affects women over the age of 30. This clinical condition refers only to cases in which the ovarian neoformation is a fibroid, a thecoma, a granulosa cell tumor or a Brenner tumor with disappearance of symptoms and effusions after removal of the neoplasm. Meigs syndrome is most frequently characterized by the presence of an ovarian fibroid, which in childhood is very rare and not commonly associated with the disease. In this article we report the case of an 11- year-old girl who came to our observation for a high fever for five days accompanied by cough and abdominal pain; imaging methods revealed bilateral hydrothorax, ascites, and a voluminous expansive right ovarian formation. On histological examination, the mass showed a cellular fibroid and the diagnosis of Meigs syndrome was made. Furthermore, we present a review of the literature aimed at detecting the state of knowledge on this disease in pediatric age, giving particular emphasis to the condition for which, in the presence of pleural effusion and ascites, an ovarian neoformation is not necessarily malignant. KEY WORDS: CT, Meigs syndrome, Pediatric, Pelvic mass, Ultrasounds.

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.

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.

Pneumatosis cystoides intestinalis, a rare case in a pediatric patient following allogeneic hematopoietic stem cell transplantation: CT findings and literature review.

Pneumatosis cystoides intestinalis (PCI) is a rare condition characterized by the presence of gas-filled cysts in the subserosa or submucosa of the bowel wall. It is associated with various disorders including chronic obstructive pulmonary diseases, autoimmune disorders, and organ transplantation. PCI has also been observed following Hematopoietic Stem Cell Transplantation (HSCT), associated with chemotherapy, acute Graft versus Host Disease (GvHD), immunosuppression, and infections. Computed tomography (CT) provides an easy diagnosis because it highlights the presence of air bubbles in the intestinal wall and possible pneumoperitoneum. We report the case of a patient with severe acquired medullary aplasia undergoing allogeneic HSCT with subsequent development of cutaneous GvHD and an incidental finding of PCI during a CT scan of the chest in absence of gastrointestinal symptoms. Our work aims at clarifying a possible complication in pediatric patients undergoing HSCT to guide young or non-pediatric radiologists in the identification of this rare condition, helping the clinician in the correct conservative management of these patients and reserving the surgical treatment only to specific complications.

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.