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Cystic Pleuro-Pulmonary Blastoma

Short summary

2-year-old female had been diagnosed with Pleuropulmonary Blastoma. The disease extent included the lung and pleura with no signs of metastatic spread. It was decided to start with 3 cycles of chemotherapy and afterwards to carry out a CT scan reassessment and surgical operation. The expert agrees that patients treated with combination of chemotherapy and complete surgical resection do better. Achieving total resection of the tumor results in a significantly better prognosis, whereas extrapulmonary involvement at diagnosis results in worse prognosis.

Patient's questions

1) Do you agree with the treatment chosen?

2) Prognosis?

3) Possible options to the surgical operation?

Medical Background

2 years old , female, Italy
Diagnosis: Cystic Pleuro-Pulmonary Blastoma

Case history:
Diagnosis of pneumonia for the appearance of hyperpyrexia and cough treated with antibiotic by oral way. In the absence of clinical response after over 48 hours’ treatment, the patient has been taken to the Emergency Room where a chest radiography was carried out with finding of a right basal opacity with associated effusion and a bullous area located cranially to the lesion, of difficult interpretation.

In May, 2009 Following a parenteral antibiotic therapy at the beginning, and afterwards by oral way, the young patient felt well and 3 weeks later she underwent a clinical and radiological follow-up. The chest CT scan performed on that occasion still showed a non-specific pulmonary cystic area cranially to the area of the flogistic infiltrate put down to pneumonia. As there was a persisting diagnostic doubt, it was decided that the young patient had to undergo repeated radiological follow-ups until definition or resolution of the clinical picture.

During June examination a picture of hypertensive pneumothorax with the mediastinum mobility was found, with the patient always being asymptomatic. The patient underwent pleural drainage and afterwards to exeresis operation of the pulmonary lesion via thoracoscopy. The tissue taken has been, therefore, subject to hystopathologic analysis that is indicative of cystic pleuro-pulmonary blastoma.

During hospitalization in DH dated 8/2009 at admission to ward, the young patient showed good general conditions, she was lively and reactive; at the thorax level a slight reduction in the inspiratory airflow at the right lung base was appreciated. Clear thoracoscopy scars. Remaining examinations within normal limits. The hematochemical examinations performed showed: WBC: 9 750/mrnc; N: 3i00/mmc. Platelets: 864 000, Hb: 10.5 g/dI; °ERS: 58 mm/h; APC: 0.26 mg/dl; ° Ferritin: 71.36 ng/ml; ° Haepatic and renal function, electrolytes within normal limits; ° LDH:336U/L; ° IgG: 946 rng/dl; IgA: 110 mgldl; IgM: 172 mg/dl; ° CA 125: 39.14 U.LmL ° CA 19-9: 4.25 U.L1’ml; ° CEA: 1,19 ng/ml;

Staging of neoplasia has been carried out by means of:
- Bone scintigraphy: Negative;

- Cranial, neck, chest and abdomen CT scan: Some small lymph nodes of around a centimeter size are observed in the laterocervical area, posterior to the carotid space bilaterally and in left inguinal fossa. At the right chest level various ovalar air bubbles are observed. Showing a maximum diameter of 2.5 cm, they are next to each other and located both in pleural and parenchymal area, of triangle morphology as a whole, encircled by hypodense material that extendes, thickening it, to the pleura in lateral and posterior costal margin area, in intratissular and mediastinal area, at the level of the right hilus and between the right cardiac margin and paravertebral region. Focal pleural thickening hypodense at the inferior middle third level in the rear and in right area over the diaphragm. Transverse mega-apophysis on the right side at L5. Therefore, the disease seems to be located at the pleural/pulmonary level without any sign of long-term metastasis.

The attending doctors talked about the case with their surgery colleagues and with the person in charge of the TREP protocol (Rare Paediatric Tumors) and, in accordance with the parents, it was decided to start with 3 cycles of chemotherapy as per the protocol (VAIA) and afterwards to carry out a CT scan reassessment and surgical operation with radical attempt.

The first cycle envisages the infusion of Ifosfamide, Vincristine and Actinomycin D on the first day, in hospitalization; afterwards Vincristine in Day Hospital once a week for 2 weeks.

Medical opinion

It is important to know if the tumor was completely cystic or had some solid elements. This may have prognostic implications. From the description I would suspect that we are dealing with type II tumor.

Pleuropulmonary blastoma is a rare, malignant intrathoracic pediatric tumor. It arises from the lung, pleura, or mediastinum and its pathogenesis and relationship to other pediatric solid tumors is not well understood.

It is subclassified as type I (purely cystic), type II (both cystic and solid elements), and type III (completely solid). Type II and type III may be associated with metastasis, with the brain being the most common metastatic site.

The diagnosis in this case is completely obvious in view of the pathology report, the clinical picture and the imaging results.

Pleuropulmonary Blastoma is a high risk disease, however with modern therapy cure can be achieved in about 40-50% of patients.

Until today no adequate therapy has been defined.

The protocol the patient has been offered is one of the accepted protocols at this time, and even though we do not have results of large studies in this rare disease, it is obvious that patients treated with combination of chemotherapy and complete surgical resection do better.

Some cases have been treated with radiotherapy, however this may be accompanied by substantial sequelae.

According to the proposed protocol the patient will be re evaluated at the end of the treatment and will hopefully proceed to complete surgical resection which is of utmost importance.

Achieving total resection of the tumor at any time of treatment results in a significantly better prognosis, whereas extrapulmonary involvement at diagnosis results in worse prognosis.

Dr Priest JR from the International Pleuropulmonary Blastoma Registry, Children's Hospitals and Clinics of Minnesota, St. Paul, Minnesota, is involved in collecting information on these patients worldwide.

I would be happy to see this patient in my practice if the parents wish to travel to Israel.

I would stress the point that the most experienced chest surgeon should be involved in the evaluation of resectability, timing, and the performance of the operation.

Our chest surgeon is definitely highly experienced, but I believe an equally experienced one can be found in Italy.