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Journal of Indian Association of Pediatric Surgeons
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Year : 2021  |  Volume : 26  |  Issue : 5  |  Page : 342-344

Extrapulmonary pleuropulmonary blastoma in a 3-year-old child: A case report and review of literature

Department of Pediatric Surgery, PGIMER, Chandigarh, India

Date of Submission17-May-2020
Date of Decision27-Jun-2020
Date of Acceptance08-Aug-2020
Date of Web Publication16-Sep-2021

Correspondence Address:
Muneer Abas Malik
Department of Pediatric Surgery, 5421, 5D Advance Pediatric Centre, PGIMER, Chandigarh
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/jiaps.JIAPS_159_20

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Pleuropulmonary blastomas (PPBs) are very rare, highly aggressive, dysembryonic neoplasms of thoracopulmonary mesenchyme. These have been reported in the pediatric population and account for only 0.5%–1% of all primary malignant lung cancers. They normally arise from lung tissue, however rarely the parietal pleura may be the tissue of origin (extra pulmonary PPB) which are extremely rare. Common age of presentation is three to 4 years. The prognosis is poor with distant metastasis to central nervous system and bone with survival rate of approximately 42.9% at 5 years. They are managed by aggressive multimodal therapies including surgery and chemotherapy. We report a case of a 3-year-old male child with Type 2 PPB of the left hemithorax, managed by surgical excision of the mass and adjuvant chemotherapy.

Keywords: Extra pulmonary pleuropulmonary blastoma, multimodal therapy, poor prognosis

How to cite this article:
Ali M, Malik MA, Peters NJ, Reddy M, Samujh R. Extrapulmonary pleuropulmonary blastoma in a 3-year-old child: A case report and review of literature. J Indian Assoc Pediatr Surg 2021;26:342-4

How to cite this URL:
Ali M, Malik MA, Peters NJ, Reddy M, Samujh R. Extrapulmonary pleuropulmonary blastoma in a 3-year-old child: A case report and review of literature. J Indian Assoc Pediatr Surg [serial online] 2021 [cited 2023 Mar 26];26:342-4. Available from: https://www.jiaps.com/text.asp?2021/26/5/342/326062

   Introduction Top

Pleuropulmonary blastomas (PPBs) are dysembryonic neoplasms of thoracopulmonary mesenchyme in childhood. They have been known by various nomenclatures like pulmonary rhabdomyosarcomas, cystic mesenchymal hamartomas, blastomas, or undifferentiated sarcomas. These tumors are commonly seen in children in the age group of 3–4 years. Clinically, these patients present with respiratory symptoms such as cough, fever, and chest pain. Chest radiograph may show pleural effusion, pneumonia, or space occupying lesions (SOLs). Contrast-enhanced computed tomography (CECT) chest is the investigational modality of choice. In spite of poor outcome and aggressive nature of disease, the only hope in these patients is tailored multimodality treatment by combination of chemotherapy, surgery and rarely, radiotherapy. Combination of complete resection with chemotherapy leads to a reasonable long-term outcome.[1]

   Case Report Top

A 3-year-old male child presented with complaints of cough, fever for 2 weeks and respiratory distress for 2 days. On examination, the child had tachypnea (respiratory rate 40/min), suprasternal retractions and distended neck veins. On percussion, he had a dull note on left side, with reduced air entry on auscultation. In view of respiratory distress, the patient was intubated.

Chest radiography showed complete opacification of the left hemithorax with contralateral shift of mediastinum. CECT of chest showed large solid cystic mass lesion in the left hemothorax - 10.5 cm × 9.6 cm × 13.1 cm with multiple enhancing septations seen within cystic component [Figure 1]. This lesion extended into mediastinum with contralateral mediastinal shift. There was loss of fat planes with left atrium, ventricle and aorta. Atelectasis of left lung and inferior displacement of left hemidiaphragm was noted.
Figure 1: Contrast-enhanced computed tomography chest showing large solid cystic lesion in left hemithorax with septations displacing mediastinum to the right

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After adequate preoperative stabilization, patient underwent left posterolateral thoracotomy. A mass of size 14 cm × 10 cm × 8 cm was identified which pushed the heart to the right upper hemithorax with complete collapse of ipsilateral lung [Figure 2]. The mass was well encapsulated tense cystic lesion. Initial decompression was done with needle aspiration. The entire lesion was easily enucleated from the surrounding tissue. There was a distinct area of dense adhesion from the parietal pleura near the costophrenic angle indicating the organ of origin. The lesion was excised completely and the area of origin along with adjoining area was ablated with monopolar diathermy. The left lung expanded well at the end of procedure. Patient was extubated on postoperative day 3. Chest drain was removed the next day and discharged in a satisfactory condition. Histopathology confirmed the tumor to be PPB Type II. Postsurgery, the patient was started on adjuvant chemotherapy follow-up. Computed tomography (CT) scan at 3 months showed no evidence of recurrence. Due to the Covid-19 pandemic, the patient could not continue adjuvant chemotherapy and presented at 5 months follow-up with relapse at the same site.
Figure 2: Intraoperative pictures of large cystic pleuropulmonary blastomas tumor visible just below thoracotomy incision

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   Discussion Top

Extrapulmonary PPBs are extremely rare pediatric primary neoplasms of chest and accounts for 25% of all PPB cases. These highly malignant and aggressive tumors, usually occur in the children <5–6 years of age. PPBs have been classified into three types by Dehenr et al.: type I PPB (cystic variety seen in young children), Type II PPB (mixed solid and cystic) and Type III PPB (solid variety seen mostly in older children). Type I tumors are seen to be associated with preexisting long-standing congenital cystic lesions of lung and can be more aggressive if inadequately managed. The median age of presentation of these tumors depends broadly on their type: for Type I tumors, it is 10 months; for Type II, it is 34 months; and for Type III, it is 44 months. These tumors have no gender predilection and are most commonly detected in right hemithorax.[2] Our case presented at 36 months of age corresponding with that of Type II PPB.

Most patients present with respiratory distress due to lung compression.[3] Other symptoms may include chest pain, fever, cough, hemoptysis, anorexia, malaise, or neurological symptoms resulting from brains metastasis. This tumor is also known to metastasize to bone, liver, lymph nodes, kidney, pancreas, and adrenal glands.[4]

Intrathoracic space occupying lesions (SOL) with septations in children may create a diagnostic dilemma with hydatid cyst or congenital cystic lung lesions often delaying the correct diagnosis. PPB remains a differential diagnosis in these radiological scenarios in spite of its rarity. Surgical excision remains the mainstay of treatment. Adjuvant chemotherapy is recommended to prevent recurrence. Most of the time, response to chemotherapy is poor, so combination of chemotherapy with local radiotherapy is also recommended by some authors. Ifosfamide, Vincristine, Actinomycin D, and Doxorubicin regimen is the regime of choice. However, due to limited literature there is no consensus on duration of chemotherapy.

Extra pulmonary PPBs have poor prognosis. Local recurrence and distant metastasis frequently occur after or during therapy. Patients with pleural, mediastinal or extra pulmonary involvement at the time of diagnosis have worse prognosis than those without such involvement (2 year survival rate, lung parenchyma only: 80.0% ± 11.3%, mediastinum: 37.5% ± 17.1%, and pleura: 47.9% ± 14.9%, P = 0.05).[3] Types II and III PPBs are highly aggressive malignancies with projected overall survival of 42.9% at 5 years,[5] even after multimodality therapy.

High-dose consolidation therapy with autologous stem cell rescue is recommended for recurrence of these tumors. For patients with unresectable or residual tumors postsurgery chemotherapy, radiotherapy is prescribed.[3]

   Conclusions Top

Extra pulmonary PPBs are rare and highly aggressive primary intrathoracic tumors of childhood which require multimodality therapy including radical surgical excision, chemotherapy and rarely radiotherapy. However, even after complete surgical excision, prognosis solely depends on the histopathological type, origin, and biological behavior of tumor. In view of the poor outcomes for patients with Types II and extrapulmonary location, the role of neoadjuvant CT or instillation of hyperthermic intrathoracic chemotherapy must be explored to prevent recurrence.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

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Conflicts of interest

There are no conflicts of interest.

   References Top

Priest JR, McDermott MB, Bhatia S, Watterson J, Manivel JC, Dehner LP. “Pleuropulmonary blastoma: A clinicopathological study of 50 cases”. Cancer 1997;80:147-61.  Back to cited text no. 1
Calabria R, Srikanth MS, Chamberlin K, Bloch J, Atkinson JB. Management of pulmonary blastoma in children. Am Surg 1993;59:192-6.  Back to cited text no. 2
Indolfi P, Casale F, Carli M, Bisogno G, Ninfo V, Cecchetto G, et al. Pleuropulmonary blastoma: Management and prognosis of 11 cases. Cancer 2000;89:1396-401.  Back to cited text no. 3
Kelsey AM, McNally K, Birch J, Mitchell EL. Case of extra pulmonary, pleuro-pulmonary blastoma in a child: Pathological and cytogenetic findings. Med Pediatr Oncol 1997;29:61-4.  Back to cited text no. 4
Bisogno G, Brennan B, Orbach D, Stachowicz-Stencel T, Cecchetto G, Indolfi P, et al. Treatment and prognostic factors in pleuropulmonary blastoma: An EXPeRT report. Eur J Cancer 2014;50:178-84.  Back to cited text no. 5


  [Figure 1], [Figure 2]


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