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  Table of Contents  
LETTER TO THE EDITOR
Year : 2015  |  Volume : 52  |  Issue : 3  |  Page : 452-453
 

Infantile myofibromatosis of uterus: A case report


Department of Pathology, Government Medical College, Aurangabad, India

Date of Web Publication18-Feb-2016

Correspondence Address:
M S Dhawle
Department of Pathology, Government Medical College, Aurangabad
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0019-509X.176725

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How to cite this article:
Bhatkule M A, Dhawle M S, Kumbhakarna N R, Bindu R S. Infantile myofibromatosis of uterus: A case report. Indian J Cancer 2015;52:452-3

How to cite this URL:
Bhatkule M A, Dhawle M S, Kumbhakarna N R, Bindu R S. Infantile myofibromatosis of uterus: A case report. Indian J Cancer [serial online] 2015 [cited 2019 Sep 22];52:452-3. Available from: http://www.indianjcancer.com/text.asp?2015/52/3/452/176725


Sir,

Infantile myofibromatosis is rare and unusual mesenchymal disorder presenting as single or multiple nodules affecting the soft tissues and internal organs. It is the most common fibrous disorder of infancy and early childhood. [1] We found a case of infantile myofibromatosis in uterus of 11 months old female child. This is the first documentation of infantile myofibromatosis in the uterus and so consider it worthwhile to report this case.

An 11 month old female presented with swelling of 5 × 5 cm. in right paraumbilical region. USG showed well defined hypoechoeic mass with calcification of 5.8 × 7 cm in right paraumbilicalregion. During operation a mass of 10 × 6 × 4 cm was seen arising from posterior aspect of fundus of infantile uterus. It was firm and highly vascular. A subtotal hysterectomy with right oophorectomy was done as bleeding could not be controlled. As the mass is solitary, well encapsulated not adherent to any other pelvic structures it is arising from the fundus of uterus only.

On gross was seen a well encapsulated globular mass of 10 × 6 × 4 cm. Cut surface was grayish white and firm [Figure 1].
Figure 1: Gross photograph showing well encapsulated globular mass of 10 × 6 × 4 cm. Cut surface was grayish white and firm

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Microscopic examination showed encapsulated lesion with spindle shaped cells and elongated nuclei having eosinophilic cytoplasm arranged in bundles and fascicles [Figure 2]. IHC revealed positivity for vimentin [Figure 3] and Smooth muscle actin [Figure 4] while Desmin, H-caldesmon, c-kit, CD34, cytokeratin, HMB-45 were negative. S-100 protein was also negative. On the basis of these morphological and immunohistochemical findings a diagnosis of Infantile myofibromatosis was made. Patient came only once for follow up 6 months after operation. At that time patient was apparently alright.
Figure 2: Microphotograph showing encapsulated lesion with spindle shaped cells and elongated nuclei having eosinophilic cytoplasm arranged in bundles and fascicles

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Figure 3: IHC reveals positivity for vimentin

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Figure 4: IHC reveals positivity for smooth muscle actin

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The condition was first described by Stout in 1954 as congenital generalized fibromatosis.[2] Infantile myofibromatosis was the term coined by Chung and Enzinger in 1981.

Three distinct forms are described. Solitary nodules are located in the skin subcutaneous tissue and bones. Multicentric nodules also involve the bones while generalized forms occur in lungs, heart and gastrointestinal tract.[3],[4] Rarly central nervous system is affected.[4] It is characterized by development of firm, discrete nodules that are rubbery. Etiology is unknown. Prognosis is poor when several internal organs are affected.

The diagnosis of infantile myofibromatosis should be considered when evaluating children who present with either solitary or multiple tumors during neonatal period as it is a rare mesenchymal disorder not widely known and has frequently misdiagnosed.[5]

 
  References Top

1.
Wiswell TE, Davis J, Cunningham BE, Solenberger R, Thomas PJ. Infantile myofibromatosis: The most common fibrous tumor of infancy. J PeadiatrSurg 1988;23:315-18.  Back to cited text no. 1
    
2.
Stout AP. Juvenile fibromatosis, Cancer 1954;7:953-78.  Back to cited text no. 2
    
3.
Chung EB, Enzinger FM. Infantile myofibromatosis. Cancer 1981;48:1807-18.  Back to cited text no. 3
[PUBMED]    
4.
Behar PM, Albritton FD, Muller S, Todd NW. Multi-centric infantile myofibromatosis. Int J PediatrOtorhinolaryngol 1998;45:249-54.  Back to cited text no. 4
    
5.
Kotiloğlu E, Göğüş S, Ruacan S, Akyüz C, Büyükpamukçu M, Srialioğlu F. Infantile myofibromatosis: A case with unusual features and review of literature. TurkJPediatr 1996;38;527-32.  Back to cited text no. 5
    


    Figures

  [Figure 1], [Figure 2], [Figure 3], [Figure 4]



 

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