pleuropulmonary blastoma

What is Pleuropulmonary Blastoma?

Pleuropulmonary blastoma (PPB) is a rare and aggressive cancer that occurs in the lungs or pleural cavity, specifically in children. It is characterized by its rapid growth and potential to spread to other parts of the body.

Types of PPB

There are four subtypes of PPB: type I, II, III, and Ir. Type I, II, and III PPB are typically found in children under the age of 7-8 years, while PPB can also occur in adults [1].

Symptoms and Characteristics

PPB is often diagnosed as a large mass in the thorax with mixed solid-cystic heterogeneous low attenuation, pleural effusion (fluid accumulation in the pleural space) [6]. It may also form in the organs between the lungs, including the heart, aorta, and pulmonary artery, or in the diaphragm [3].

Age Group Affected

PPB mainly occurs in younger children, typically before the age of 5 years [11]. In fact, it is one of the most common primary malignant neoplasms of the lung in childhood, occurring in the same early age group (0–6 years) as other solid tumors like neuroblastoma and Wilms tumor [10].

Treatment Options

PPB is typically treated with surgery, chemotherapy (CTx), and/or radiation therapy (RTx) [14]. The treatment approach may vary depending on the stage and severity of the cancer.

Prognosis

The prognosis for PPB patients varies depending on several factors, including the age at diagnosis, tumor size, and response to treatment. However, with aggressive treatment, some children have been able to achieve long-term survival [14].

References: [1] - Bisogno et al. (2021) [3] - Bisogno et al. (2021) [6] - Bisogno et al. (2021) [10] - Bisogno et al. (2021) [11] - Bisogno et al. (2021) [14] - Bisogno et al. (2021)

Common Signs and Symptoms of Pleuropulmonary Blastoma

Pleuropulmonary blastoma (PPB) is a rare cancer that affects the lungs and pleural cavity, primarily in infants and young children. The symptoms of PPB can vary depending on the age and type of the disease, but here are some common signs and symptoms:

• Respiratory problems: Difficulty breathing, coughing, fever, and chest pain are common symptoms of PPB [2][4][6].
• Cough and respiratory distress: Children with PPB may experience a persistent cough, wheezing, or shortness of breath [7][11].
• Fever and weight loss: Fever, weight loss, anorexia, fatigue, or weakness are also common symptoms of PPB [5][9].
• Pneumonia-like symptoms: Some children with PPB may experience pneumonia-like symptoms, including cough, fever, and pain in the chest [2][11].
• Cysts or tumors: In some cases, PPB can be detected through imaging tests, such as MRI, which may show cysts or tumors in the lungs or pleural cavity [1][3].

It's essential to note that not every child with PPB will experience all of these symptoms, and the severity of the symptoms can vary from one child to another. If you suspect that your child has PPB, it's crucial to consult a doctor for proper diagnosis and treatment.

References:

[1] Context result 3: Pleuropulmonary blastoma (PPB) is a rare cancer originating in the lung or pleural cavity.

[2] Context result

Diagnostic Tests for Pleuropulmonary Blastoma

Pleuropulmonary blastoma (PPB) is a rare and aggressive cancer that affects the lungs and pleura in children. Accurate diagnosis is crucial for effective treatment and management of the disease.

• Biopsy: The only way to correctly diagnose PPB is through a biopsy of tissue from the lung or chest [3]. This involves taking a sample of tissue from the affected area, which is then examined under a microscope by a pathologist.
• Imaging Studies: Imaging tests such as X-rays, CT scans, and MRI may be used to visualize the tumor and assess its size and location. Chest CT usually reveals a heterogeneous low-attenuation mass with pleural effusion and mediastinal shift [6].
• Bronchoscopy and Fine Needle Biopsy: In some cases, bronchoscopy and fine needle biopsy have been useful for diagnosis [7]. This involves using a flexible tube with a camera and light on the end to visualize the airways and take a sample of tissue from the affected area.
• Diagnostic Teams: Diagnostic teams for PPB may include oncology, pulmonology, and other specialists who can help

Chemotherapy for Pleuropulmonary Blastoma

Pleuropulmonary blastoma (PPB) is a rare and aggressive cancer that affects the lungs and pleura in children. While surgery is often the primary treatment, chemotherapy plays a crucial role in managing this disease.

Types of Chemotherapy Used

According to various studies [7][11], chemotherapy drugs used alone or in combination to treat PPB include:

• Dactinomycin
• Doxorubicin
• Ifosfamide
• Vincristine

These chemotherapeutic agents are injected into a vein and work by killing cancer cells or stopping them from dividing.

Treatment Guidelines

The International Pleuropulmonary Blastoma Registry (IPPBR) has initiated a prospective clinical trial to provide treatment guidelines for the various PPB types [10]. This study aims to standardize management by utilizing specific regimens with prospective review and efficacy analysis.

Chemotherapy in Combination with Surgery

Some studies suggest that intensive combination chemotherapy should be used as the initial management of children with advanced PPB, in addition to surgery [9]. This approach may improve treatment outcomes for these patients.

Adaptation of Drug Doses

It is essential to note that drug doses should be adapted in infants, as their physiology and pharmacokinetics differ from those of older children [13].

While chemotherapy is an essential part of treating pleuropulmonary blastoma, it is crucial to remember that each patient's response to treatment may vary. A multidisciplinary approach involving surgeons, oncologists, and other healthcare professionals is often necessary to provide the best possible care for these patients.

References:

[7] Chemotherapy drugs used alone or in combination to treat pleuropulmonary blastoma include: dactinomycin; doxorubicin; ifosfamide; vincristine. [9] We believe intensive combination chemotherapy should be used as the initial management of children with advanced PPB, in addition to surgery. [10] The first and thus-far only prospective clinical trial was opened in 2011 by the International Pleuropulmonary Blastoma Registry (IPPBR) and is currently ongoing. [11] Chemotherapy (also called chemo) uses drugs to stop the growth of cancer cells. Chemotherapy either kills the cancer cells or stops them from dividing. Chemotherapy may be given alone or with other types of treatment. [13] drug doses should be adapted in infants [Level V, Grade B] (Supplemental Material 1).

Differential Diagnosis of Pleuropulmonary Blastoma

Pleuropulmonary blastoma (PPB) is a rare and aggressive tumor that occurs in the lungs of children, typically in the age group of 0-6 years. When diagnosing PPB, it's essential to consider other possible causes or conditions that may present with similar symptoms or imaging findings.

Key Differential Diagnoses:

• Congenital Pulmonary Airway Malformation (CPAM): CPAM is a type of cystic lesion that can be difficult to distinguish from PPB using imaging techniques alone. However, Type I PPB is typically multifocal and occurs postnatally, whereas congenital cystic lesions are usually unifocal and seen in the second trimester antenatal ultrasonography [9].
• Metastasis from Wilms Tumor: PPB can be mistaken for metastatic disease from other primary tumors, such as Wilms tumor. A key pathologic and genetic discriminator is the DICER1 germline mutation found in PPB.
• Macrocystic Congenital Pulmonary Airway Malformation (CPAM): This condition can also present with cystic lesions similar to those seen in PPB.

Other Considerations:

• Pulmonary Blastoma: While not a direct differential diagnosis, pulmonary blastoma is a distinct entity that occurs in adults and presents as a sarcomatoid carcinoma.
• Neuroblastoma and Wilms Tumor: These are more common solid tumors that