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Pleuropulmonary Blastoma
Pleuropulmonary blastoma family tumor susceptibility syndrome

Presentation

[…] with unusual presentation of tension pneumothorax, with an aim to increase awareness so as to recognize this rare entity in early course. [ijmpo.org]

Here we present a case report of an unusual presentation of type III pleuropulmonary blastoma. [jmedicalcasereports.biomedcentral.com]

PPBs can present insidiously: they have been reported to commonly mimic more common pathologies with non-specific presenting symptoms, such as respiratory distress, cough and chest pain. 6 In some cases, they may present as a recurrent pneumothorax 7 [touchrespiratory.com]

Patients with PPB present with nonspecific symptoms mimicking pneumonia or respiratory distress syndrome. Herein, we report a 3-year-old, female case of PPB presenting with fever, cough, chest pain, and progressive shortness of breath. [ccij-online.org]

At present, well-documented cases or cases of truly precise presentation of either pathological or immunohistochemical findings in PPB are rare. [ncbi.nlm.nih.gov]

Entire Body System

  • 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]

    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]

    […] 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]

    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]

    These children may present with signs of pneumonia or other generalized symptoms such as cough, fever, difficulty breathing, fatigue, loss of energy and decreased appetite. [ncbi.nlm.nih.gov]

Neurologic

  • Agitation

    […] pts with advanced disease, acute exacerbations or cor pulmonale -indication for home O2: -O2 sat -Sat laryngomalacia -congenital abnormality of laryngeal cartilage -inspiratory noises in 2 months of life -noise increases when baby supine, crying or agitation [quizlet.com]

Workup

[…] 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]

Treatment

Because PPB can return after treatment, regular screening for possible recurrence should continue for 48 to 60 months, after diagnosis. [en.wikipedia.org]

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

Type 1 pleuropulmonary blastomas have a good prognosis. [radiopaedia.org]

Recurrences after type I PPB are usually advanced type II or type III neoplasms with a poor prognosis. [ncbi.nlm.nih.gov]

Conclusion The clinical manifestations of PPB are nonspecific, its misdiagnosis rate is high, and it has a poor prognosis. Pediatricians should be aware of the seriousness of PPB. [jpedsurg.org]

Treatment and prognosis Type I tumours have a good prognosis. Complete surgical resection is often the treatment of choice, as it is with CPAM. Tumours larger than 5 cm just like type 1 and 2 PPB carry a worse prognosis. [deeprad.de]

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]

Epidemiology PPB is encountered in child... https://radiopaedia.org/articles/pleuropulmonary-blastoma Pleuroparenchymal fibroelastosis: a rare interstitial lung ... [najdi.si]

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]

Pathophysiology is emphasized throughout, providing a sound basis for discussions of the diagnosis, treatment, and prognosis that follow. [books.google.de]

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]

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