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Tumor Discovery Pleuropulmonary blastoma in child

A B is favored for debulking and macroscopic clearance. This
technique is usually narrowed down to non-anatomical
or wedge resection, lobectomy or pneumonectomy, and
removal of infiltrated structures such as pericardium,
diaphragm, or parietal pleura. In general speaking, gross
surgical resection of the tumors may improve survival of
Figure 4. (A) Immediate post-operative computed tomography scan of the patients. Aggressive mediastinal dissection should be
the chest shows clear bilateral lung fields without residual macroscopic weighed against surgical morbidity and mortality [9,10] . In
lesion. (B) A computed tomography scan of the chest shows gross local
recurrence of mass in the right hemithorax with mediastinal shift at the type II and type III PPBs, chemotherapy is mandatory
time of readmission. for maximizing the chance of curing the patients. If
metastatic disease is present, neoadjuvant chemotherapy
Type I (median age at diagnosis 46.5 months) is a subtype should always be included in the treatment regimen, and
that lacks primitive cell components. In the documented doxorubicin is an important agent for type II and type III
registry, most cases of type I and Ir cysts are unilateral PPB. Multidrug regimens for rhabdomyosarcoma, such as
(74%), with only 55% larger than 5 cm in size. Type II ifosfamide-vincristine, actinomycin D (IVA), and IVA with
tumors have cystic and solid areas of blastomatous or doxorubicin (IVADo) or cyclophosphamide for replacing
sarcomatous components with anaplasia in up to 60% ifosfamide, can been considered for treatment, but curative
of these cases, with the median age during diagnosis evidence and optimal duration of chemotherapy have not
at 35 months, and distant metastases happen in 7% of been established. Radiotherapy has a minimal impact on
patients at the time of diagnosis. Type III tumors consist of this tumor. The significant international effort promoted
purely solid components (blastomatous and sarcomatous), by the EXPeRT/PARTNER group proposed possible
with anaplasia occurring in 70% of these cases. The median overall strategies to treat patients with PBB. However,
age at diagnosis of type III tumor is 41 months, and distant more corroborative evidence is needed to answer some
metastases reportedly happen in 10% of patients at the open questions.
time of diagnosis [3,4] . Type II and type III tumors are Prognosis is determined mainly based on the type
more metastasizable at early stage and can metastasize to of tumor, resection margin, metastasis, and post-
the brain, lungs, and bone, with the brain being the most operative adjuvant chemotherapy . A recent report by
[11]
common site of metastasis . Therefore, pre-operative Duc et al. showed that despite tumoral resection and
[5]
staging using magnetic resonance imaging or CT scan chemotherapy, local recurrence and spinal metastasis
of the brain, chest, abdomen, and sometimes, a bone still occurred in a 2-year-old girl with type III PPB after
scan, is strongly recommended . A study by Priest et al. 5 months of treatment , indicating the challenges in the
[6]
[12]
documented an overall survival rate of 45% at 5 years with treatment and control of PBB. It is recommended that
an event-free survival rate of 49% at 2 years among PBB treatment plan should be personalized according to the
patients . Type II and type III PPBs manifest aggressive specific conditions of each patient after multidisciplinary
[7]
behavior, with 2-year and 5-year overall survival rates of
62% and 42%, respectively, despite multimodal therapy. tumor board discussion since the standard consensus
and guidelines surrounding the treatment of PBB are
Due to gross enlargement, these masses usually cause evolving .
[13]
compression of vital structures in the mediastinum. In
some patients with severe respiratory distress, a needle 4. Conclusion
decompression can be performed or a chest tube can be The initial chest X-ray findings may misconstrue type II
inserted as a lifesaving procedure. However, chest tube and type III PPB as hydropneumothorax due to the cystic-
placement is not favored due to possible contamination solid nature of these subtypes, and this may be the sole
of the pleura, which might result in tumor upstaging initial presentation of this rare entity as in our case. Further,
and increase the risk for en bloc resection . Although after establishing the diagnosis, cytoreductive surgery was
[8]
three treatment modalities (surgery, chemotherapy, and performed with the primary goal of complete removal
radiotherapy) have been tested for their efficacy in treating of all macroscopic disease, but the patient succumbed
PBB, no clear consensus can be drawn due to the rarity, to death due to the rapid local recurrence within a short
peculiarity and late presentation of the tumor. duration, possibly caused by the lack of chemotherapy not
Complete surgical resection is cited as the standard administered in this case. Hence, multimodality treatment
management strategy for type I tumors because it delivers should always be considered for more prolonged survival
good patient outcomes. For type II and type III, surgery in advanced cases of PPB.

Volume 2 Issue 3 (2023) 4 https://doi.org/10.36922/td.1756