Journal of Neonatal Surgery 2012;1(2):35

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UNUSUALLY AGGRESSIVE MYOFIBROMATOSIS IN A NEONATE

Jitendra Hazarey*, Shilpa Hazare1, Girish Moghe2 Department of Pediatric surgery*, Pediatrics1, and Pathology2, Getwell Hospital and Research Institute, 20/1, Dhantoli, Nagpur, India. 440012
* Corresponding Author Available at http://www.jneonatalsurg.com This work is licensed under a Creative Commons Attribution 3.0 Unported License

How to cite: Hazarey J, Hazare S, Moghe G. Unusually aggressive myofibromatosis in a neonate. J Neonat Surg 2012; 1: 35

Figure 1: Exophytic masses arising from the knee and ankle. Scar of two excised masses at the hip joint area.

Figure 2: Photomicrograph showing abundant fibroblasts with skeletal muscles. No evidence of malignancy at this time.
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UNUSUALLY AGGRESSIVE MYOFIBROMATOSIS IN A NEONATE

A 15 days old male newborn presented with four exophytic swellings arising from the skin of right lower limb; not restricting its mobility (Fig.1). The lesions were sequentially excised and pathological examination revealed infantile myofibromatosis in these growths. No evidence of malignancy was found (Fig.2). The swelling in the thigh recurred after 6 months and suspecting sarcomatous change, a biopsy was done which confirmed the sarcomatous change. Amputation and chemotherapy was offered, and the poor response to chemoradiotherapy was explained. Parents refused further treatment and child succumbed to the disease after developing inguinal nodes and visceral metastasis.

Infantile myofibromatosis is a rare tumor of infancy. It is mesenchymal in origin and involves superficial structures or may be visceral. The lesions have been found in nearly all kinds of tissues, including the orbit, bone, lip, oral cavity, central nervous system, gastrointestinal tract, lungs, myocardium, liver, and biliary tree. It may present at birth or appears during the first year of life. The exact etiology is unknown and most cases have been reported as sporadic. Both, autosomal dominant and recessive inheritance modes of transmission have been suggested. The prognosis is excellent in solitary or multicentric lesions without visceral involvement, with possibility of spontaneous regression of lesions confined to the skin, soft tissue and bone, and a very low recurrence rate after surgical excision. The prognosis is

poor with visceral involvement [1-3]. In our patient the lesion recurred with a sarcomatous change which is an unusual behavior for a lesion known to be benign with a capacity for spontaneous regression.

REFERENCES
1. 2. Chung EB, Enzinger FM. Infantile myofibromatosis. Cancer 1981;48:1807-18. Inwards CY, Unni KK, Beabout JW, Shives TC. Solitary congenital fibromatosis (infantile myofibromatosis) of bone. Am J Surg Pathol 1991;15:935-41. Wiswell TE, Davis J, Cunningham BE, Solenberger R, Thomas PJ. Infantile myofibromatosis: the most common fibrous tumor of infancy. J Pediatr Surg 1988;23:315-18.

3.

Address for correspondence Dr. Jitendra Hazarey Department of Pediatric surgery, Getwell Hospital and Research Institute, 20/1, Dhantoli, Nagpur, India. 440012.
E mail: jitendrahazare@hotmail.com Hazarey et al, 2012

Submitted on: 11-01-2012 Accepted on: 21-01-2012 Published on: 01-04-2012 Conflict of interest: None Source of Support: Nil

Journal of Neonatal Surgery Vol. 1(2); 2012