Pleuropulmonary Blastoma in Pediatric Age Group: A Very Rare Case Report and Review of Literature

Pleuropulmonary blastoma (PPB) is a very rare and very aggressive malignant tumor that affects children and adults. Though its presentation in children has been reported in literature, incidence is very low as compared to adults. This neoplasm is characterized on histology by primitive blastema and a malignant mesenchymal stroma that often demonstrates multidirectional differentiation. Even though availability of multimodal therapy, the prognosis of patients with PPB remains poor.

DICER1 mutations in twelve Chinese patients with pleuropulmonary blastoma

Our aim is to examine the impact of DICER1 mutations on the pathogenesis of pleuropulmonary blastoma(PPB) by evaluating the mutation frequency and investigating the family history of Chinese patients with PPB. The family histories of 12 children with PPB recruited consecutively were surveyed. Blood samples from patients and their first-degree relatives were tested for DICER1 mutations. Whole-genome sequencing of blood samples and formalin-fixed and paraffin-embedded(FFPE) tumor tissue was performed in one family with twins. Twelve patients with PPB included six type II and six type III cases. Seven of the12 patients harbored DICER1 mutations, six of which were frameshift or nonsense mutations. Another case carried a germline DICER1 mutation affecting the splice site. FFPE sample had a nonsense mutation in TDG and missense mutations in DICER1.In addition, two cases with DICER1 mutations were found to have lung cysts preceding the diagnosis of PPB. Furthermore, one patient had a family history remarkable for thyroid diseases. Our results indicate that the germline mutation frequency in Chinese patients with PPB is similar to the ones reported for patients from USA, UK, and Japan. Moreover, our study strongly suggests that investigating the family history and detecting germline DICER1 mutations might be of benefit to increasing awareness and improving the accuracy of the differential diagnosis of PPB from non-malignant lung cysts.

Contrast-enhanced ultrasonography in peripheral lung consolidations: What's its actual role?

AIM:To evaluate the diagnostic accuracy of contrastenhanced ultrasonography(CEUS)in the differential diagnosis between neoplastic and non-neoplastic peripheral pleuro-pulmonary lesions.METHODS:One hundred patients with pleural or peripheral pulmonary lesions underwent thoracic CEUS.An 8 microliters/mL solution of sulfur hexafluoride microbubbles stabilized by a phospholipid shell(SonoVue)was used as US contrast agent.The clips were stored and independently reviewed by two readers,who recorded the following parameters:presence/absence of arterial enhancement,time to enhancement(TE),extent of enhancement(EE),pattern of enhancement(PE),presence/absence of wash-out,time to wash-out,and extent of wash-out.After the final diagnosis(based on histopathologic findings or follow-up of at least 15 mo)was reached,sensitivity,specificity,positive predictive value(PPV),negative predictive value(NPV),positive likelihood ratio(PLR),negative likelihood ratio(NLR)of each CEUS parameter in the differential diagnosis between neoplastic and nonneoplastic lesions were calculated.Furthermore,an arbitrary score based on the ratio between the PPVs of each CEUS parameter was calculated,to evaluate if some relationship could exist between overall CEUS behaviour and neoplastic or non-neoplastic nature of the lesions.

Amebic liver abscess:Clinico-radiological findings and interventional management

In its classic form,amebic liver abscess(ALA)is a mild disease,which responds dramatically to antibiotics and rarely requires drainage.However,the two other forms of the disease,i.e.,acute aggressive and chronic indolent usually require drainage.These forms of ALA are frequently reported in endemic areas.The acute aggressive disease is particularly associated with serious complications,such as ruptures,secondary infections,and biliary communications.Laboratory parameters are deranged,with signs of organ failure often present.This form of disease is also associated with a high mortality rate,and early drainage is often required to control the disease severity.In the chronic form,the disease is characterized by low-grade symptoms,mainly pain in the right upper quadrant.Ultrasound and computed tomography(CT)play an important role not only in the diagnosis but also in the assessment of disease severity and identification of the associated complications.Recently,it has been shown that CT imaging morphology can be classified into three patterns,which seem to correlate with the clinical subtypes.Each pattern depicts its own set of distinctive imaging features.In this review,we briefly outline the clinical and imaging features of the three distinct forms of ALA,and discuss the role of percutaneous drainage in the management of ALA.

Adult pulmonary blastoma: Report of an unusual malignant lung tumor

Pulmonary blastoma is an uncommon lung malignancy,usually presenting itself as a large chest mass causing pain, hemoptysis, cough and dyspnea; however, it is asymptomatic in up to 40% of patients. We present the case and suggestive images of a 37-year-old non-smoking lady with a monophasic pulmonary blastoma located in the lower lobe of the left lung who underwent a left posterolateral thoracotomy with lower lobectomy, hilar and mediastinal node dissection, followed by chemo and radiation therapy. After 36 mo, there is no disease progression and the patient is in good health, clinically stable and without significant chest pain.

Spectrum of Non-Typhoidal <i>Salmonella</i>Infections among Immunocompetent Individuals

Non-typhoidal salmonellosis has emerged as an invasive infection in industrialized countries. Bacteremia and pleuropulmonary involvement usually occur in the setting of chronic illnesses such as diabetes, malignancies and HIV. We present three cases of non-typhoidal salmonellosis in immunocompetent patients presenting over a year period. A 66-year-old female presented with septic shock. She was started on vancomycin, doripenem and ciprofloxacin. Her blood cultures grew Salmonella enteridis that was also isolated from her urine and bronchoalveolar lavage fluid. Stool cultures were negative for Salmonella species. She had no epidemiologic risk factors for invasive Salmonella infection. Her immunodeficiency workup was negative. CT scan abdomen was unremarkable. She became hemodynamically stable and completed a two week course of Ceftriaxone without complications. A 58-year-old female presented with sub-acute onset of fever and dyspnea. CT chest showed bilateral pleural effusions. Left sided thoracentesis revealed purulent fluid consistent with empyema. Pleural fluid cultures grew Salmonella Group D. Blood, sputum and stool cultures remained negative. She denied consumption of raw eggs or exposure to pets, farm animals or reptiles. She was treated with Ceftriaxone for two weeks along with pigtail catheter drainage. Her immunodeficiency workup was negative. A 62-year-old man presented with acute left lower quadrant abdominal pain. CT Abdomen revealed a focal abnormality in abdominal aorta consistent with mycotic aneurysm. Blood cultures grew Salmonella Group D resistant to cephalosporins and bactrim, hence treatment with Meropenem was started. Subsequent blood, respiratory tract, urine and stool cultures remained negative. Due to high risk of perioperative complications, it was decided to follow the mycotic aneurysm expectantly. Antibiotics were deescalated to six weeks of Ertapenem followed by long term quinolone prophylaxis. He also did not have any epidemiologic risk factors for salmonellosis and his immunodeficiency workup was negative. These cases highlight the pathogenesis of this invasive organism that is transmitted though food borne route and causes bacteremic seeding of various sites such as lungs and pleura. It is extremely important to consider this organism in patients presenting with gram negative bacteremia leading to septic shock as well as endovascular infections.