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Pleuropulmonary Blastoma
Pleuropulmonary blastoma family tumor susceptibility syndrome

Presentation

Pleuropulmonary blastomas are rare tumors which present non-specific symptoms. [ncbi.nlm.nih.gov]

[…] with difficulty breathing or other respiratory problems including persistent pneumonitis, coughing or atelectasis 10% may also present with multilocular cystic nephroma and very rarely, Wilms tumor Diagnosis MRI or CT can help diagnose, but biopsy is [pathologyoutlines.com]

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]

    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]

    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]

  • 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]

Gastrointestinal

  • 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Demir, Esra Karakuş, Ceyda Kırsaçlıoğlu Tuna, Süleyman Arif Bostancı, Atilla Şenaylı, Müjdem Nur Azılı and Emrah Şenel, WITHDRAWN: Multiseptate Gallbladder, a rare cause of recurrent abdominal pain: A case report and review of [doi.org]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

  • 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

[…] 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

Treatment of Pleuropulmonary Blastoma For information about the treatments listed below, see the Treatment Option Overview section. [cancer.gov]

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]

[…] 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]

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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