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Tumorsphere Derivation and Treatment from Primary Tumor Cells Isolated from Mouse Rhabdomyosarcomas
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[Importance of heart rabdomioma in pediatric population. A 39 year experience. Case series]

Nadia G Chía-Vázquez1, Gerardo Fuentes-Ramos2, Emilia J Patiño-Bahena1

  • 1Departamento de Cardiología Pediátrica, Insituto Nacional de Cardiología Ignacio Chávez, Ciudad de México, México.

Archivos De Cardiologia De Mexico
|December 17, 2020
PubMed

Insights

Pediatric rhabdomyomas, common benign heart tumors, are often associated with tuberous sclerosis (ET). Early detection and management are crucial, as outcomes vary, with some cases showing spontaneous regression and others requiring intervention.

Area of Science:

  • Pediatric Cardiology
  • Oncology
  • Genetics

Background:

  • Rhabdomyomas are the most common benign cardiac tumors in fetuses and children.
  • Their association with tuberous sclerosis (ET) necessitates thorough evaluation and management.

Purpose of the Study:

  • To present a 39-year experience with pediatric rhabdomyoma cases.
  • To highlight the importance of early detection, study, and monitoring due to the ET association.

Main Methods:

  • Retrospective and descriptive study from January 1980 to March 2018.
  • Analysis of 24 pediatric patients with rhabdomyoma, including clinical data, diagnostic studies, treatment, and long-term follow-up via phone.

Main Results:

  • 24 of 51 cardiac tumor patients were diagnosed with rhabdomyoma.
  • Diagnosis occurred prenatally (8), at birth (5), and within the first year (11).
  • Frequent symptoms included murmurs, arrhythmias, cyanosis, dyspnea, and diaphoresis; 17 patients had ET. Outcomes included stability (17), spontaneous regression (5), and mortality (2).

Conclusions:

  • Rhabdomyoma is a rare benign tumor with potentially malignant evolution.
  • Association with tuberous sclerosis significantly impacts prognosis, underscoring the need for comprehensive care.

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