Testicular juvenile granulosa cell tumor: a case report.

The Testicular Juvenile Granulosa Cell Tumor (JGCT) is a rare testicular neoplasm that appears in the first months of life as a painless testicular mass. Following an accurate radiological ultrasound diagnosis, through which the cystic appearance of the lesion is observed, and histological confirmation, showing follicular growth pattern and an immunoreactivity for inhibin, the treatment process involves, when feasible, conservative surgery. We present the case of a 2-months old infant with a bilateral JGCT of the testis and we review the classical findings of the patology.

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.

Congenital tibial pseudarthrosis: A challenge in pediatric radiology.

Congenital pseudarthrosis of the tibia (CPT) is a rare disorder affecting the skeletal system in pediatric population with an estimated incidence of 1:140,000 to 1:250,000 newborns. It is characterized by deformity of the tibia, including anterolateral bowing of the bone diaphysis and/or narrowing of the medullary canal, leading to instability or fracture. CPT can be either idiopathic or associated with underlying conditions such as type 1 neurofibromatosis (NF1), fibrous dysplasia, or Campanacci's osteofibrous dysplasia. Diagnosis is based on clinical and imaging findings, using conventional radiography and magnetic resonance imaging (MRI). The disorder is characterized by recurrent pathological fractures of the tibia or fibula during childhood, often beginning by the age of 2 years. Treatment options include surgical and nonsurgical management.

Pest risk assessment of African Leucinodes species for the European Union.

Following a request from the European Commission, the EFSA Panel on Plant Health performed a quantitative risk assessment for the EU of African Leucinodes species (Lepidoptera: Crambidae), which are fruit and shoot borers, especially of eggplant type fruit. The assessment focused on (i) potential pathways for entry, (ii) distribution of infested imports within EU, (iii) climatic conditions favouring establishment, (iv) spread and (v) impact. Options for risk reduction are discussed, but their effectiveness was not quantified. Leucinodes spp. are widely distributed across sub-Saharan Africa but are little studied and they could be much more widespread in Africa than reported. Much African literature erroneously reports them as Leucinodes orbonalis which is restricted to Asia. The import of eggplant type fruit from sub-Saharan Africa consists of special fruit types and caters mostly to niche markets in the EU. The main pathway for entry is fruit of Solanum aethiopicum and exotic varieties of eggplant (S. melongena). CLIMEX modelling was used with two possible thresholds of ecoclimatic index (EI) to assess establishment potential. Climates favouring establishment occur mostly in southern Europe, where, based on human population, 14% of the imported produce is distributed across NUTS2 regions where EI ≥ 30; or where 23% of the produce is distributed where EI ≥ 15. Over the next 5 years, an annual median estimate of ~ 8600 fruits, originating from Africa, and infested with African Leucinodes spp. are expected to enter EU NUTS2 regions where EI ≥ 15 (90% CR ~ 570-52,700); this drops to ~ 5200 (90% CR ~ 350-32,100) in NUTS2 regions where EI ≥ 30. Escape of adult moths occurs mostly from consumer waste; considering uncertainties in pathway transfer, such as adult emergence, mate finding and survival of progeny, the annual median probability of a mated female establishing a founder population in NUTS regions where EI ≥ 15 was estimated to be 0.0078 (90% CR 0.00023-0.12125). This equates to a median estimate of one founder population ~ every 128 years (90% CR approximately one every 8-4280 years). Using an EI ≥ 30, the median number of founder populations establishing in the EU annually is 0.0048 (90% CR 0.0001-0.0739), equating to a median estimate of one founder population approximately every 210 years (90% CR approximately one every 14-7020 years). Under climate change for the period 2040-2059, the percent of infested produce going to suitable areas would be increased to 33% for EI ≥ 15 and to 21% for EI ≥ 30. Accordingly, the waiting time until the next founder population would be reduced to median estimates of 89 years for EI ≥ 15 (90% CR ~ 6-2980 years) and 139 years for EI ≥ 30 (90% CR 9-4655 years). If a founder population were to establish, it is estimated to spread at a rate of 0.65-7.0 km per year after a lag phase of 5-92 years. Leucinodes spp. are estimated to reduce eggplant yield by a median value of 4.5% (90% CR 0.67%-13%) if growers take no specific action, or 0.54% (90% CR between 0.13% and 1.9%) if they do take targeted action, matching previous estimates made during a risk assessment of L. orbonalis from Asia.

Risk assessment of Retithrips syriacus for the EU.

Following a request from the European Commission, the EFSA Panel on Plant Health performed a quantitative risk assessment for the EU of Retithrips syriacus (Mayet) (Thysanoptera: Thripidae), a polyphagous thrips, regarded as a tropical/subtropical pest occurring in several countries of Africa, South America, Asia and in the EU in Cyprus. The current risk assessment focused on potential pathways for entry, the climatic conditions allowing establishment, the expected spread capacity and the impact considering a time horizon of 10 years (2023-2032). The Panel identified the import of cut roses, persimmons, table grapes, as well as plants for planting of the genera Acalypha and Terminalia from third countries and those of Persea americana (avocado) from Israel as the most relevant entry pathways to consider. Over the next 10 years, an annual median estimate of 95 (90% Certainty Range, CR, ranging from 13 to 1832) potential R. syriacus founder populations per year are expected to successfully transfer to a suitable host in the EU NUTS2 regions where the climatic conditions are predicted as suitable for establishment; this value drops to a median of 4.6 founder populations per year (90% CR: 1 every 1.9 years - 85.6 per year) after considering the actual probability of establishment of a potential founder population. The estimated number of founder population per year is mostly driven by the import of cut roses and plants for planting. If such founder populations were to establish, R. syriacus is estimated to spread at a median rate of 0.05 km/year (90% CR 0.02-2.30 km/year) after a median lag phase of 1.1 years (90% CR 0.3-3.3 years). The overall impact on yield (expressed as % of the total agricultural production) directly attributable to R. syriacus when considering: (i) the main R. syriacus hosts in the EU, (ii) the areas of the EU where establishment is possible, (iii) the current agricultural practices and (iv) the evidence of impact from the countries where the pest is established for a long time, was estimated at 0.065% as the median value of the uncertainty distribution (90% CR 0.001%-0.571%). Options for risk reduction are discussed, but the effectiveness was not quantified.

Pest risk assessment of Leucinodes orbonalis for the European Union.

Following a request from the European Commission, the EFSA Panel on Plant Health performed a quantitative risk assessment of Leucinodes orbonalis (Lepidoptera: Crambidae), the eggplant fruit and shoot borer, for the EU. The assessment focused on potential pathways for entry, climatic conditions favouring establishment, spread and impact. Options for risk reduction are discussed but effectiveness was not quantified. L. orbonalis is a key pest of eggplant (aubergine/brinjal) in the Indian subcontinent and occurs throughout most of southern Asia with records mostly from India and Bangladesh. The main pathway of entry is fruit of solanaceous plants, primarily exotic varieties of eggplant, Solanum melongena and turkey berry, S. torvum. The trade in both commodities from Asia is small but nevertheless dwarfs the trade in other Solanum fruits from Asia (S. aethiopicum, S. anguivi, S. virginianum, S. aculeatissimum, S. undatum). Other Solanum fruits were therefore not further assessed as potential pathways. The trade in eggplant from Asia consists of special fruit types and caters mostly to niche markets in the EU, while most eggplant consumed in Europe is produced in southern European and northern African countries, where L. orbonalis does not occur. Using expert knowledge elicitation (EKE) and pathway modelling, the Panel estimated that approximately 3-670 infested fruit (90% certainty range, CR) of S. melongena or fruit bunches of S. torvum enter into regions of the EU that are suitable for L. orbonalis establishment each year. Based on CLIMEX modelling, and using two possible thresholds of ecoclimatic index (EI) to indicate uncertainty in establishment potential, climates favouring establishment occur mostly in southern Europe, where, based on human population, approximately 14% of the imported produce is distributed across NUTS2 regions where EI ≥ 30; or 23% of the produce is distributed where EI ≥ 15. Escape of adult moths occurs mostly from consumer waste. By analysing results of different scenarios for the proportion of S. melongena and S. torvum in the trade, and considering uncertainties in the climatic suitability of southern Europe, adult moth emergence in areas suitable for establishment is expected to vary between 84 individuals per year and one individual per 40 years (based on 90% CR in different scenarios). In the baseline scenario, 25% of the solanaceous fruit from Asia is S. torvum, 75% is S. melongena and EI ≥ 30 is required for establishment. After accounting for the chances of mating, host finding and establishment, the probability of a mated female establishing a founder population in the EU is less than 1 in 100,000 to about 1 event per 622 years (90% CR in baseline scenario). The waiting time until the first establishment is then 622 to more than 100,000 years (CR). If such a founder population were established, the moth is estimated to spread at a rate of 0.65-7.0 km per year after a lag phase of 5-92 years. The impact of the insect on the production of eggplant is estimated to be 0.67%-13% (CR) if growers take no specific action against the insect and 0.13%-1.9% if they do take targeted actions. Tomato (S. lycopersicum) and potato (S. tuberosum) are hosts of L. orbonalis, but the insect does not develop to maturity in tomato fruit, and it does not feed on potato tubers under field conditions; hence, damage to potato can only occur due to feeding on shoots. Tomato and potato are not preferred hosts; nevertheless, impact can occur if populations of L. orbonalis are high and preferred hosts are not available. The Panel did not assess this damage due to insufficient information.

Assessment of the probability of introduction of Thaumatotibia leucotreta into the European Union with import of cut roses.

Following a request from the European Commission, the EFSA Panel on Plant Health performed a quantitative pest risk assessment to assess whether the import of cut roses provides a pathway for the introduction of Thaumatotibia leucotreta (Lepidoptera: Tortricidae) into the EU. The assessment was limited to the entry and establishment steps. A pathway model was used to assess how many T. leucotreta individuals would survive and emerge as adults from commercial or household wastes in an EU NUTS2 region climatically suitable in a specific season. This pathway model for entry consisted of three components: a cut roses distribution model, a T. leucotreta developmental model and a waste model. Four scenarios of timing from initial disposal of the cut roses until waste treatment (3, 7, 14 and 28 days) were considered. The estimated median number of adults escaping per year from imported cut roses in all the climatically suitable NUTS2 regions of the EU varied from 49,867 (90% uncertainty between 5,298 and 234,393) up to 143,689 (90% uncertainty between 21,126 and 401,458) for the 3- and 28-day scenarios. Assuming that, on average, a successful mating will happen for every 435 escaping moths, the estimated median number of T. leucotreta mated females per year from imported cut roses in all the climatically suitable NUTS2 regions of the EU would vary from 115 (90% uncertainty between 12 and 538) up to 330 (90% uncertainty between 49 and 923) for the 3- and 28-day scenarios. Due to the extreme polyphagia of T. leucotreta, host availability will not be a limiting factor for establishment. Climatic suitability assessment, using a physiologically based demographic modelling approach, identified the coastline extending from the northwest of the Iberian Peninsula through the Mediterranean as area suitable for establishment of T. leucotreta. This assessment indicates that cut roses provide a pathway for the introduction of T. leucotreta into the EU.

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.

Multimodal imaging approach in hyponatremic hypertensive syndrome. A rare case of pediatric unilateral hypoplasia of the main renal artery combined itself with stenosis and review of literature.

Renal artery stenosis (RAS) accounts for approximately 5%-10% of secondary renovascular hypertension in the pediatric population. It can occur as an isolated entity, or as a hypoplasia combined itself with stenosis. Hypoplasia, or long-segment developmental narrowing, is a rare cause of renovascular hypertension. Hyponatremic hypertensive syndrome (HHS) is a malignant complication of unilateral RAS and/or renal artery hypoplasia. Hyponatremia, hypokalemic hypochloremic metabolic alkalosis, nephrotic range proteinuria, polyuria, polydipsia, and weight loss are the most common findings. In particular, hypertension remains refractory despite aggressive antihypertensive therapy. Laboratory findings of elevated plasma levels of renin in most case suggest that the stimulation of renin release from the ischemic kidney plays an important pathophysiologic role. HHS is a diagnostic and therapeutic challenge in children. We report a case of a unilateral right renal artery hypoplasia, complicated by a segmental narrowing, in a 17-month-old male, clinically symptomatic for hypertension. We emphasize the role of ultrasound, computed tomography, and digital subtraction angiography that should be planned as reliable and non-invasive multimodal imaging approach.

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.

The Role of Ultrasound in Chronic Intestinal Diseases in Pediatric Patients.

Chronic inflammatory bowel diseases (IBD) are chronic disorders of the gastrointestinal tract, with an increasing incidence in pediatric populations. Ultrasound of the intestinal wall represents the first-line imaging technique in children since it is a noninvasive method, is free of ionizing radiation, and is inexpensive. Furthermore, the absence of intestinal wall thickening has a good negative predictive value for IBD, which is greater for Crohn's disease than for ulcerative colitis. Ultrasound is used for the diagnosis of disease, for the differential diagnosis in IBD, in the follow-up of known IBD, in the definition of the site and extent of the disease, for the diagnosis of intestinal complications, for the evaluation of disease activity, in the definition of prognostic parameters, and in the post-operative follow-up.

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.

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.

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.

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.

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.

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.