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Pleuropulmonary Blastoma


Presentation

  • Now, we present another case of PPB showing unusual features. A 3-yr-old boy presented with a pleural- or chest wall-based tumor.[ncbi.nlm.nih.gov]
  • Here we present a case report of an unusual presentation of type III pleuropulmonary blastoma.[ncbi.nlm.nih.gov]
  • We present a case of PPB in a child, which presented to the emergency department with an extensive pleural effusion. We discuss the radiological features, pathology, classification and treatment of this condition.[ncbi.nlm.nih.gov]
  • This report presents a rare case of pleuropulmonary blastoma with cervical lymph node metastasis at the time of presentation.[ncbi.nlm.nih.gov]
  • The typical histopathological presentation includes the aggregation of malignant primitive small cells, usually observed in sheets.[ncbi.nlm.nih.gov]
Fatigue
  • Side effects of chemotherapy, which may include nausea, vomiting, hair loss, decreased appetite, mouth sores, fatigue, low blood cell counts, and a higher chance of developing infections The treatment can also cause infertility in men and women.[dovemed.com]
  • .............................................................. 20 Intermittent fatigue, malaise, and anorexia, or; incapacitating episodes (with symptoms such as fatigue, malaise, nausea, vomiting, anorexia, arthralgia, and right upper quadrant pain)[militarydisabilitymadeeasy.com]
  • […] with the highest potential for distant metastasis, mainly seen in pediatric patients with a mean age of 44 months old. [7], [8], [9], [10] Respiratory distress syndrome, progressive shortness of breath, or pneumonia-like symptoms, such as fever, cough, fatigue[ccij-online.org]
  • […] due to fatigue -intubation required A-a gradient Normal v increased alveolar-arterial oxygen difference Normal gradient - when hypoventilation is cause of hypoxemia Increased in most other causes: V/Q mismatch, shunt Mechanical Ventilation: decrease oxygenation[quizlet.com]
  • Some developed a mild nonproductive cough associated with fatigue, myalgia, and low grade fever over 72 hours, followed by a rapid onset of severe dyspnea with diaphoresis and cyanosis.[web.duke.edu]
Soft Tissue Mass
  • The chest radiograph and a contrast enhanced CT scan revealed a cystic cavity in the left upper lobe with an endobronchial soft tissue mass in left bronchus.[ncbi.nlm.nih.gov]
  • Preoperative (a) axial computed tomography (CT) image showing a large heterogenously enhancing soft tissue mass lesion in the right hemithorax.[lungindia.com]
Episodic Pain
  • Stiff person syndrome is a rare autoimmune, neurological disorder characterized by progressive rigidity and episodic painful spasms, predominantly affecting the proximal limbs and axial muscles, and leading to progressive disability.[ncbi.nlm.nih.gov]
Respiratory Disorders
  • The clinical presentation includes signs and symptoms associated with various respiratory disorders. To make a definitive diagnosis of PPB, an examination of the cystic fluid or solid tumor is required.[ncbi.nlm.nih.gov]
Recurrent Abdominal Pain
  • Can İhsan Öztorun, Rabia Demir, Esra Karakuş, Ceyda Kırsaçlıoğlu Tuna, Süleyman Arif Bostancı, Atilla Şenaylı, Müjdem Nur Azılı and Emrah Şenel, WITHDRAWN: Multiseptate Gallbladder, a rare cause of recurrent abdominal pain: A case report and review of[doi.org]
Macrocephaly
  • Stewart, Macrocephaly associated with the DICER1 syndrome, Genetics in Medicine, 10.1038/gim.2016.83, 19, 2, (244-248), (2016). David J.[doi.org]
Withdrawn
  • Can İhsan Öztorun, Rabia Demir, Esra Karakuş, Ceyda Kırsaçlıoğlu Tuna, Süleyman Arif Bostancı, Atilla Şenaylı, Müjdem Nur Azılı and Emrah Şenel, WITHDRAWN: Multiseptate Gallbladder, a rare cause of recurrent abdominal pain: A case report and review of[doi.org]

Workup

  • […] newer quinolones: levofloxacin, moxifloxacin (not cipro) OR -3rd gen ceph macrolide Outpt treatment of community acquired pneumo -likely strep pneumo -azithromycin or doxycycline painful skin lesions on shins bilateral mediastinal adenopathy Management/workup[quizlet.com]
Multilocular Cyst
  • Type I PPB is a delicate multilocular cyst with variable numbers of primitive mesenchymal cells beneath a benign epithelial surface. Rhabdomyoblasts and cartilage nodules are seen in 49% and 40% of cases, respectively.[ncbi.nlm.nih.gov]
  • From a multilocular cyst, PPB has the potential to progress to a high-grade multipatterned primitive sarcoma. More than 65% of all affected children have a heterozygous germline mutation in DICER1.[ncbi.nlm.nih.gov]

Treatment

  • Two patients with type I PPB and 4 patients with type II PPB underwent surgical and chemotherapeutic treatment and all are alive and without recurrence 1 to 13 years after treatment.[ncbi.nlm.nih.gov]
  • This finding supports early resection of these lesions rather than observation or treatment with nonoperative strategies.[ncbi.nlm.nih.gov]
  • The girl died 4 1/2 months after initial evidence of disease because of brain metastasis, indicating a very aggressive neoplasm unresponsive to treatment.[ncbi.nlm.nih.gov]
  • We report a case of PPB in a young adult and its recurrence in the pancreas after primary surgical treatment and adjuvant chemotherapy.[ncbi.nlm.nih.gov]
  • Here we described the main characteristics of these patients, the diagnostic methods, and treatment modalities used.[ncbi.nlm.nih.gov]

Prognosis

  • Recurrences after type I PPB are usually advanced type II or type III neoplasms with a poor prognosis.[ncbi.nlm.nih.gov]
  • T'he prognosis is extremely poor with frequent metastasis to the brain and bone. A 4-year-old boy successfully treated with multimodal therapy despite unfavorable prognostic factors is presented.[ncbi.nlm.nih.gov]
  • This case reiterates the importance of considering this diagnosis prior to surgical intervention, to improve the long term prognosis of this aggressive disease.[ncbi.nlm.nih.gov]
  • Despite the use of chemotherapy with or without radiotherapy, the prognosis is poor. Here we report on the case of a 2.5 year-old boy presenting with pneumothorax who turned out to have a right hemithoracic mass.[ncbi.nlm.nih.gov]
  • Keeping in mind the moderate prognosis of PPB in children, accurate assessment and treatment of PPB require a team approach of oncology, radiology and surgery to establish new therapeutic guidelines in the future.[ncbi.nlm.nih.gov]

Etiology

  • The etiology and treatment of the uncommon combination of pleuropulmonary blastoma and thyroid carcinoma is discussed.[ncbi.nlm.nih.gov]
  • It has been hypothesized that loss of heterozygosity of DICER1 in lung epithelium is a non-cell autonomous etiology of PPB and a critical pathway that regulates lung development; however, there are no known direct targets of epithelial microRNAs (miRNAs[ncbi.nlm.nih.gov]
  • Pathogenic germline and somatic mutations of DICER1 in NCMH establishes that the genetic etiology of NCMH is similar to PPB, despite the disparate biological potential of these neoplasms.[ncbi.nlm.nih.gov]
  • (90% are between 0 - 2 years old) Type I may be found in very young children (birth to 2 years of age) Types II and III tend to be found after 2 years of age ( Pediatr Dev Pathol 2015;18:504 ) Sites PPB may arise in the lung, mediastinum and pleura Etiology[pathologyoutlines.com]

Epidemiology

  • Author information 1 Center for Clinical Epidemiology & Evidence-based Medicine, Beijing Children's Hospital, Capital Medical University, National Center for Children's Health, Beijing, 100045, China. 2 Hematology Oncology Center, Beijing Children's Hospital[ncbi.nlm.nih.gov]
  • Author information 1 Clinical Genetics Branch, Division of Cancer Epidemiology and Genetics, National Cancer Institute, NIH, Rockville, MD, USA, drstewart@mail.nih.gov.[ncbi.nlm.nih.gov]
  • BEIR VII (model VII proposed by the Committee on the Biological Effects of Ionizing Radiation) risk tables were used to predict excess cancer mortality for each strategy, and the corresponding loss of life expectancy was calculated using Surveillance Epidemiologic[ncbi.nlm.nih.gov]
  • Epidemiology PPB is encountered in childhood, mostly in the first years of life (90% in those between 0-2 years old). Associations PPBs are associated with type 4 CPAM. PPB is associated with PPB family tumour and dysplasia syndrome in 33% of cases.[deeprad.de]
  • […] thickening areas (nodules) within this cystic lesion Type III shows solid masses Terminology PPB Pneumoblastoma Mesenchymal cystic hamartoma Cystic mesenchymal hamartoma Pulmonary rhabdomyosarcoma Rhabdomyosarcoma in lung cyst Pediatric pulmonary blastoma Epidemiology[pathologyoutlines.com]
Sex distribution
Age distribution

Pathophysiology

  • Pathophysiologically, PPB evolves from a cystic to solid state over time. It is subclassified as type I (purely cystic), type II (both cystic and solid elements), and type III (completely solid).[ncbi.nlm.nih.gov]
  • Grasp the nuances of the pathophysiology of today’s major respiratory conditions—including pulmonary hypertension, COPD, asthma, environmental lung disease, sleep disorders, infections of the immunocompromised, neonatal breathing disorders, and drug-resistant[books.google.de]
  • CLINICAL DATA OBTAINED AT THE PATIENT’S BEDSIDE The Physical Examination Vital Signs Increased Respiratory Rate (Tachypnea) Several pathophysiologic mechanisms operating simultaneously may lead to an increased ventilatory rate: • Stimulation of peripheral[clinicalgate.com]
  • The pathophysiology of ergoline-induced pneumonitis also remains an unsettled issue.[mafiadoc.com]

Prevention

  • Abstract Pleuropulmonary blastomas (PPB) are rare intrathoracic tumors that can develop in children with preexisting pulmonary cystic lesions, inferring that the prophylactic resection of such cysts might prevent the onset of these malignant tumors.[ncbi.nlm.nih.gov]
  • Three courses of postoperative chemotherapy with 2 different regimens were ineffective in preventing multiple metastases of the lung. We then instituted a protocol with irinotecan (CPT-11) and vincristine.[ncbi.nlm.nih.gov]
  • This supports the theory that PPB I may progress to a more aggressive type II with time, and highlights the importance of the adequately treating the PPB I to prevent this transformation.[ncbi.nlm.nih.gov]
  • As complete tumour ablation is essential to prevent local recurrence and allow any chance of survival, the favourable outcome of our patient is probably due to the radical resection of the neoplasm.[ncbi.nlm.nih.gov]
  • Every attempt should be made to remove completely all cystic remnants to prevent reccurence of PPB. In case of reccurence, Type I recurrences frequently progress to more serious Type II or III disease.[telemedicina.med.muni.cz]

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