|LETTER TO THE EDITOR
|Year : 2015 | Volume
| Issue : 3 | Page : 452-453
Infantile myofibromatosis of uterus: A case report
MA Bhatkule, MS Dhawle, NR Kumbhakarna, RS Bindu
Department of Pathology, Government Medical College, Aurangabad, India
|Date of Web Publication||18-Feb-2016|
M S Dhawle
Department of Pathology, Government Medical College, Aurangabad
Source of Support: None, Conflict of Interest: None
|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
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.  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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The condition was first described by Stout in 1954 as congenital generalized fibromatosis. 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., Rarly central nervous system is affected. 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.
| » References|| |
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[Figure 1], [Figure 2], [Figure 3], [Figure 4]