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]
Entire Body System
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Fatigue
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]
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]
[…] 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]
Symptoms of ALL Bone and joint pain Fatigue Weakness Bleeding Fever Weight loss Brain Tumors Brain tumors and other nervous system tumors make up about 27 percent of childhood cancers. [roswellpark.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]
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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]
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Pediatric Disease
Dehner, Acquired Neonatal and Pediatric Diseases, Pulmonary Pathology, 10.1007/978-1-4757-3935-0_8, (191-254), (1994). David H. Dail, Uncommon Tumors, Pulmonary Pathology, 10.1007/978-1-4757-3935-0_33, (1279-1461), (1994). [doi.org]
Respiratoric
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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]
Gastrointestinal
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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]
Musculoskeletal
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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]
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]
X-Ray
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Pleural Mass
Types of Pleuropulmonary Blastoma Pleuropulmonary blastoma is a rare and highly aggressive pulmonary malignancy that can present as a pulmonary or pleural mass. [cancer.gov]
Skip Navigation Types of Pleuropulmonary Blastoma Pleuropulmonary blastoma is a rare and highly aggressive pulmonary malignancy that can present as a pulmonary or pleural mass. [healthy.kaiserpermanente.org]
Other Pathologies
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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]
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]
Prognosis
Recurrences after type I PPB are usually advanced type II or type III neoplasms with a poor prognosis. [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]
[…] life (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]
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]
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]
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]
The role of chemotherapy remains uncertain for the prevention of progression in the pure cystic PPB Type I or Ir. Upfront chemotherapy and progression. [ascopubs.org]