Infantile myofibromatosis (IMF) is a rare group of reactive lesions. Their clinical, ra­diological, and pathological features may be confused with those of malignancy. The lung and mediastinum are the most common site of IMF in all pediatric age groups .

We present a case of a 9-year-old boy pre­senting with a history of recurrent ab­dominal pain and chronic diarrhea since the age of one. He was under the third percentile for weight (16 kg) and height (106cm). Physical examination and la­boratory findings were normal. Abdomi­nal computed tomography (CT) showed a nodular lesion of the liver (segment IV), cholelithiasis, and nephrolithiasis. CT of the thorax showed a lesion measuring 3.9x2.2x2.6cm in the inferior left lung with bronchial and pulmonary vein inva­sion and mediastinal lymph nodes.

The patient underwent upper endoscopy and a subepithelial lesion with a central depression was found in the greater cur­vature of the gastric body. Colonoscopy showed multiple subepithelial lesions with a central ulceration, some of them with fibrin, varying in size from 3 mm to 20 mm ( Fig.1). Video capsule endos- copy(MiroCam; Intromedic, Seoul, Korea) showed two lesions in thejejunum similar to those found in the colon and stomach ( Fig.2; Video 1). Endoscopic ultra­sonography of the colon with 15-MHz miniprobes showed a well-delimited, hypoechogenic, homogeneous, fusiform lesion with a central ulceration originat­ing from the muscle layer, measuring 9.2×13.2mm ( Fig.3).


Fig.1 Colonoscopic image of the subepithe­lial lesion.

Fig 2 Video capsule endoscopy showing two subepithelial lesions in the jejunum.

Video capsule endoscopy shows small-bowel tu­mors in multicentric infantile myofibromatosis.

Fig.3 Endoscopic ultrasonography of the colon with 15-MHz miniprobes showing a well- delimited, hypoechogenic, homogeneous fusi­form lesion with a central ulceration, originat­ing from the muscle layer.

Biopsies were taken from the gastric and colonic lesions and a pathological diagno­sis of IMF was made. The peripheral area showed spindle cells (myofibroblasts) with eosinophilic cytoplasm and ovoid nuclei, with less differentiated, rounder cells with pale cytoplasm and basophilic, small round nuclei in the central portion. No cellular anaplasia was present and there were few mitoses. The mesenchy­mal cells stained with vimentin and actin, but were uniformly negative with CD117, CD34, Ki-67, MIB-1, and S-100 antigen. Aerobic, anaerobic, and mycobacterial cultures obtained from the specimen were negative.


Competing interests: None

L. R. Alberti1, P. F. Souto Bittencourt2,

A. Rodrigues Ferreira2,

R. R. Rodrigues Da Silva2,

S. Diniz Carvalho2,

F. M. B. Nunes Cachicolo2, M. Bahia2,

L. P. Fonseca de Castro2

1 Department of Surgery, Federal University of Minas Gerais, Santa Casa de beloHorizonte. Belo Horizonte. brazil

2 Department of Pediatrics, Federal University of Minas Gerais. Santa Casa de beloHorizonte. Belo horizonte. brazil


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2 Smith A, Orchard D. Infantile myofibromato- sis: two families supporting autosomal dominant inheritance. Australas J Dermatol 2011; 52: 214-217

3 Al-Jazaeri A, Al-Zahem A, Al-Maziad H et al. Unilateral breast mass in an infant: a rare presentation of spontaneously regressing myofibromatosis. J Pediatr Surg 2010; 45: 1896-1899

4 Larralde M, Hoffner MV, Boggio P et al. Infan­tile myofibromatosis: report of nine pa­tients. Pediatr Dermatol 2010; 27: 29 - 33




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