Page 57 - TD-2-3

Tumor Discovery

CASE REPORT
Pleuropulmonary blastoma in a child

masquerading as massive pleural effusion: A
case report

1
1
Anshuman Darbari *, Ishan Jhalani , Meenu Singh , Ajay Kumar ,
3
2
Khushboo Taneja , and Prashant Durgapal 4
2
1 Department of CTVS, AIIMS, Rishikesh, Uttarakhand, India
2 Department of Pediatric Pulmonology, AIIMS, Rishikesh, Uttarakhand, India
3 Department of Anaesthesia, AIIMS, Rishikesh, Uttarakhand, India
4 Department of Pathology, AIIMS, Rishikesh, Uttarakhand, India

Abstract

Pleuropulmonary blastoma (PPB) is a rare dysembryonic lung neoplasm of childhood.
Its aggressive nature is demonstrated by its large size and metastasis at the time of
diagnosis. Here, we present a case of a 5-year-old male with a history of sudden
dyspnea who was initially referred to the primary health-care center for emergency
medical services. His initial chest radiography showed massive hydropneumothorax
in the right pleural cavity, with gross mediastinal displacement. These clinical
findings and rapidly decreasing peripheral saturation prompted the emergency
team to insert a right-sided intercostal drainage tube. Initially, this step was able
to stabilize the condition, but a subsequent computed tomography scan revealed
a vast cystic-solid mass in the right hemithorax. After establishing the diagnosis
*Corresponding author: of PPB by means of histopathology and immunohistochemistry, he was operated
Anshuman Darbari on urgently for cytoreductive purposes. Despite the initial macroscopic clearance,
(darbarianshu@gmail.com)
the mass recurrence occurred after 3 months, contributing to the ultimate fatal
Citation: Darbari A, Jhalani I, outcome for the patient. Due to the cystic-solid nature of type II/III PPB, it could be
Singh M, et al., 2023, Pleuropulmonary
blastoma in a child masquerading as masquerading as hydropneumothorax in the initial chest X-ray, a misleading yet the
massive pleural effusion: A case report. sole initial presentation of this rare entity.
Tumor Discov, 2(3): 1756.
https://doi.org/10.36922/td.1756
Received: September 4, 2023 Keywords: Pleuropulmonary blastoma; Lung neoplasm; Pediatric oncology; Thoracic
Accepted: November 14, 2023 surgery
Published Online: November 22, 2023
Copyright: © 2023 Author(s).
This is an Open-Access article
distributed under the terms of the 1. Background
Creative Commons Attribution
License, permitting distribution, Pleuropulmonary blastoma (PPB) is a rare dysembryonic thoracic tumor of mesenchymal
and reproduction in any medium,
provided the original work is origin in the childhood age group. It was first recognized by Manivel et al. in a study
[1]
properly cited. of 11 patients in 1988 . So far, four types of PBB have been described in the medical
Publisher’s Note: AccScience literature. Type I PPBs are virtuously cystic tumors that occur in young children of less
Publishing remains neutral with than 2 years with a median age of 10 months. Type I tumor may progress to type II or
regard to jurisdictional claims in
published maps and institutional III or regress to another type Ir, where “r” stands for regressing (no malignant cells are
affiliations. present). Type II PPBs are a mix of cystic and solid tumors usually occurring at a median

Volume 2 Issue 3 (2023) 1 https://doi.org/10.36922/td.1756