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Table of Contents   
IMAGES  
Year : 2017  |  Volume : 10  |  Issue : 2  |  Page : 218-219
Massive biventricular rhabdomyoma in a neonate


1 Department of Cardiology, AIIMS, New Delhi, India
2 Department of Pathology, AIIMS, New Delhi, India

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Date of Web Publication25-Apr-2017
 

   Abstract 


Rhabdomyoma is a well characterised entity in a neonate. Herein, we report a massive biventricular rhabdomyoma in a neonate presenting with cyanosis and congestive heart failure which was confirmed on autopsy. The report is for documentation of an unusually large tumour.

Keywords: Everolimus, rhabdomyoma, tuberous sclerosis

How to cite this article:
Pandey M, Dutta R, Kothari SS. Massive biventricular rhabdomyoma in a neonate. Ann Pediatr Card 2017;10:218-9

How to cite this URL:
Pandey M, Dutta R, Kothari SS. Massive biventricular rhabdomyoma in a neonate. Ann Pediatr Card [serial online] 2017 [cited 2021 May 12];10:218-9. Available from: https://www.annalspc.com/text.asp?2017/10/2/218/205153




Rhabdomyoma as the most common benign cardiac tumor presenting in infancy is a well-characterized entity, and its association with tuberous sclerosis is also well known.[1] Most patients with small tumors are asymptomatic, but some patients may present with arrhythmias, obstruction, and rarely embolism.[2] Spontaneous partial or complete regression of rhabdomyomas during growth is the rule.[1],[3] We recently encountered a 20-day-old neonate in congestive heart failure with cyanosis resulting from massive multiple biventricular rhabdomyoma [Figure 1]a and [Figure 1]b and [Supplementary Video 1]a and [Supplementary Video 1]b and wish to document the same in view of its size. The neonate also had macules over the chest suggestive of tuberous sclerosis, but further evaluation could not be performed. He was planned for debulking surgery but had ventricular fibrillation from which he could not be revived. An autopsy confirmed the diagnosis [Figure 2]a,[Figure 2]b,[Figure 2]c. More recently, salutary effects of everolimus (mammalian target of rapamycin) in regression of rhabdomyomas have been reported and possibly could have been utilized in this neonate.[4],[5]
Figure 1: (a) Subcostal view. (b) Parasternal short axis

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Figure 2: (a) Cardiac rhabdomyoma: Gross picture of the ventricular cavity shows multiple sessile gray-white nodules of varying sizes attached to the ventricular wall (arrows). (b) Microscopy of these nodules revealed a well-demarcated tumorous lesion with adjacent compressed normal myocardium (H and E, ×4). (c) Microscopy of these nodules on high magnification shows classical spider cells (arrow) of rhabdomyoma (H and E, ×20)

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Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
   References Top

1.
Beghetti M, Gow RM, Haney I, Mawson J, Williams WG, Freedom RM. Pediatric primary benign cardiac tumors: A 15-year review. Am Heart J 1997;134:1107-14.  Back to cited text no. 1
    
2.
Black MD, Kadletz M, Smallhorn JF, Freedom RM. Cardiac rhabdomyomas and obstructive left heart disease: Histologically but not functionally benign. Ann Thorac Surg 1998;65:1388-90.  Back to cited text no. 2
    
3.
Günther T, Schreiber C, Noebauer C, Eicken A, Lange R. Treatment strategies for pediatric patients with primary cardiac and pericardial tumors: A 30-year review. Pediatr Cardiol 2008;29:1071-6.  Back to cited text no. 3
    
4.
Mohamed I, Ethier G, Goyer I, Major P, Dahdah N. Oral everolimus treatment in a preterm infant with multifocal inoperable cardiac rhabdomyoma associated with tuberous sclerosis complex and a structural heart defect. BMJ Case Rep 2014;2014. pii: Bcr2014205138.  Back to cited text no. 4
    
5.
Tiberio D, Franz DN, Phillips JR. Regression of a cardiac rhabdomyoma in a patient receiving everolimus. Pediatrics 2011;127:e1335-7.  Back to cited text no. 5
    

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Correspondence Address:
Shyam S Kothari
Department of Cardiology AIIMS, New Delhi
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/apc.APC_164_16

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    Figures

  [Figure 1], [Figure 2]



 

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