Case Report
1 Department of Radiology, Children’s Hospital, Mohamed V University, Rabat, Morocco
2 Department of Anatomic Pathology, Children’s Hospital, Mohamed V University, Rabat, Morocco
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Daoud Ali Mohamed
Department of Radiology, Rabat Children′s Hospital, Mohamed V University, Agdal, Rabat,
Morocco
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Article ID: 101125Z01DM2020
Introduction: The rhabdoid tumor (RT) is a malignant tumor, rare and aggressive, with a poor prognosis. It affects the young children, especially the infants. Described for the first time in 1978 by Beckwith and Palmer, it develops mainly in the kidney but other localizations like cerebral and extra-cerebral have been described. The mediastinal and pulmonary localizations remain rare.
Case Report: We report a case of RT of an 18-month-old male infant admitted for pleural effusion syndrome, without any past medical history. The chest X-ray revealed a large opacity of the left hemithorax compressing heart and the mediastinum structures to the right. It was associated with a lysis of the sixth rib. Thoracic computed tomography (CT*) showed a large lobulated heterogeneously parietal mass delimiting liquid hypodense area with pleural effusion. The mass compressed the homolateral lung with reduction of left lobar bronchus and mediastinal structures to the right. It was associated with a lysis of the fifth and sixth ribs with soft tissue extension. Histopathological examination revealed richly vascularized fibrous tissue composed of large cell ranges with reduced cytoplasm and oval nuclei with vesicular chromatin, and cell ranges with eccentric nuclei and deep eosinophilic cytoplasmic inclusion. On immunohistochemistry, integrase interactor 1 (INI-1) protein labeling was negative. The diagnosis of the RT was retained. A chemotherapy was started, but despite chemotherapy the patient’s condition was deteriorated on the respiratory with dependence on oxygen therapy. The patient died after 2 months and 10 days of diagnosis.
Conclusion: The RT is a rare entity occurring usually in young children very frequently in infants. It develops mainly in the kidney but other localizations have been described including the mediastinal and pulmonary localizations which are very rare. It is extremely aggressive in nature and quickly progresses. The imaging does not provide pathognomonic diagnostic signs. The Histopathological and immunohistochemical study with immunolabeling makes it possible to clarify the diagnosis by the absence of labeling of the protein integrase interactor. Generally, the prognosis remains very poor despite the chemotherapy and radiotherapy until now.
*CT scan with 16 slice of General Electric.
Keywords: Chest wall, Extra-renal tumor, INI-1, Malignant rhabdoid tumor
Daoud Ali Mohamed - Substantial contributions to conception and design, Acquisition of data, Analysis of data, Drafting the article, Revising it critically for important intellectual content, Final approval of the version to be published
H Essaber - Substantial contributions to conception and design, Drafting the article, Revising it critically for important intellectual content, Final approval of the version to be published
Amarkak A Waiss - Substantial contributions to conception and design, Drafting the article, Revising it critically for important intellectual content, Final approval of the version to be published
Fabrice Diekouadio - Substantial contributions to conception and design, Drafting the article, Revising it critically for important intellectual content, Final approval of the version to be published
S El Haddad - Substantial contributions to conception and design, Drafting the article, Revising it critically for important intellectual content, Final approval of the version to be published
A Fekkar - Substantial contributions to conception and design, Drafting the article, Revising it critically for important intellectual content, Final approval of the version to be published
N Lamalmi - Substantial contributions to conception and design, Drafting the article, Revising it critically for important intellectual content, Final approval of the version to be published
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.
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