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Case Report
Case report of pleuropulmonary blastoma: The heterogenous features of a rare primitive neoplasm of the thorax
1 Department of Pathology and Microbiology, School of Medicine, New York Medical College, 40 Sunshine Cottage Rd, Valhalla, NY 10595, USA
2 Office of Undergraduate Medical Education, School of Medicine, New York Medical College, 40 Sunshine Cottage Rd, Valhalla, NY 10595, USA
Address correspondence to:
Elayna Maxx Shanker
Westchester Medical Center, 100 Woods Rd, Valhalla, NY 10595,
USA
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Article ID: 100068Z11LV2023
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Vemulakonda LA, Astudillo Y, Shanker EM. Case report of pleuropulmonary blastoma: The heterogenous features of a rare primitive neoplasm of the thorax. J Case Rep Images Pathol 2023;9(1):7–11.ABSTRACT
Introduction: Pleuropulmonary blastoma (PPB) is a rare primary neoplasm of the thorax frequently diagnosed in early childhood. Pleuropulmonary blastoma arises from primitive intrathoracic mesenchyme—either pulmonary and pleural, or both. Patients with pleuropulmonary blastoma usually present with respiratory symptoms and are often misdiagnosed as pneumonia in the early clinical course. Pleuropulmonary blastoma can be further classified into types I, II, and III based on histological components. Such histological distinctions have significant implications for the treatment of these lesions as well as prognosis. Currently, the treatment of pleuropulmonary blastoma is surgical resection with or without chemotherapy, based on the histological type.
Case Report: We highlight the histopathological presentation of pleuropulmonary blastoma in a 3-year-old male who presented with nonspecific symptoms. After identifying an intrathoracic mass on imaging, frozen section demonstrated primarily mature cartilaginous tissue without rhabdomyosarcomatous differentiation or anaplasia. Surgical resection specimens, however, contained small areas of proliferating spindle cells with skeletal muscle features as well as primitive blastema-like cells. Following ancillary testing, the patient was diagnosed with a type II PPB.
Conclusion: This case illustrates the challenges of using frozen sections to diagnose heterogenous neoplasms such as PPB. The histologic features of this patient’s tumor identified on frozen section were largely mature and benign-appearing. Only when considering the histology of the surgical specimens in combination with immunohistochemistry and gross morphology, we were able to make an accurate assessment about the type of PPB and corresponding prognosis and treatment plan. Therefore, we urge pathologists to consider PPB for large thoracic masses in pediatric patients.
Keywords: Embryonal rhabdomyosarcoma, Lung cyst, Pediatric pathology, Pleuropulmonary blastoma
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Author Contributions
Lakshmisree Akhila Vemulakonda - Conception of the work, Design of the work, Acquisition of data, Analysis of data, Drafting the work, Revising the work critically for important intellectual content, Final approval of the version to be published, Agree to be accountable for all aspects of the work in ensuring that questions related to the accuracy or integrity of any part of the work are appropriately investigated and resolved.
Yaritzy Astudillo - Conception of the work, Design of the work, Acquisition of data, Analysis of data, Drafting the work, Revising the work critically for important intellectual content, Final approval of the version to be published, Agree to be accountable for all aspects of the work in ensuring that questions related to the accuracy or integrity of any part of the work are appropriately investigated and resolved.
Elayna Maxx Shanker - Conception of the work, Design of the work, Acquisition of data, Analysis of data, Drafting the work, Revising the work critically for important intellectual content, Final approval of the version to be published, Agree to be accountable for all aspects of the work in ensuring that questions related to the accuracy or integrity of any part of the work are appropriately investigated and resolved.
Guarantor of SubmissionThe corresponding author is the guarantor of submission.
Source of SupportNone
Consent StatementWritten informed consent was obtained from the patient for publication of this article.
Data AvailabilityAll relevant data are within the paper and its Supporting Information files.
Conflict of InterestAuthors declare no conflict of interest.
Copyright© 2023 Salvatore Chirumbolo et al. This article is distributed under the terms of Creative Commons Attribution License which permits unrestricted use, distribution and reproduction in any medium provided the original author(s) and original publisher are properly credited. Please see the copyright policy on the journal website for more information.
