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Infantile Myofibromatosis Presenting With Scalp Dermoid Cyst
Infantile Myofibromatosis Presenting With Scalp Dermoid Cyst Ching-Ju Huang, MD*, Kuang-Lin Lin, MD*, Shih-Ming Jung, MD†, Chieh-Tsai Wu, MD‡, and Huei-Shyong Wang, MD* Infantile myofibromatosis is a disorder of infancy and early childhood, typically presenting as a solitary lesion or multiple widespread nodular tumors localized to skin, subcutaneous tissue, muscle, bone, viscera, or central nervous system. We present a case of infantile myofibromatosis, multicentric type, in a 4-month-old male infant who initially presented with an occipital scalp mass and other skin-colored nodular mass lesions over his face, trunk, and four limbs. He received tissue biopsy to establish a definite diagnosis. Craniotomy was also arranged for tumor removal due to dural involvement with internal extension and compression of adjacent sigmoid sinus. The patient eventually died of cardiopulmonary failure secondary to primary pulmonary hypertension at age 11 months. Before his death, he had suffered from abdominal distention and frequent vomiting, followed by aggravated respiratory distress and cyanosis. Aggressive surveillance for cardiopulmonary or gastrointestinal involvement is recommended in such cases because prognosis varies according to the involvement of vital organs. © 2005 by Elsevier Inc. All rights reserved. Huang C, Lin K, Jung S, Wu C, Wang H. Infantile Myofibromatosis Presenting With Scalp Dermoid Cyst. Huang C-J, Lin K-L, Jung S-M, Wu C-T, Wang H-S. Infantile myofibromatosis presenting with scalp dermoid cyst. Pediatr Neurol 2005;33:296-299.
From *Division of Pediatric Neurology, Chang Gung Children’s Hospital; †Department of Pathology, Chang Gung Memorial Hospital; and ‡Department of Neurosurgery, Chang Gung Memorial Hospital, Chang Gung University Medical College, Taoyuan, Taiwan.
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Introduction Infantile myofibromatosis is a rare benign disorder of infancy and childhood, characterized by solitary or multicentric mesenchymal tumors of the skin, soft tissue, bone, viscera or central nervous system. It was first described as “congenital fibrosarcoma” in 1951 by Williams and Schrum [cited in 1]. In 1981, Chung and Enzinger reviewed 61 cases, and gave the disease its current name on the basis of histologic features [2]. Infantile myofibromatosis may mimic other conditions, such as fibrosarcoma, leiomyosarcoma, rhabdomyosarcoma, or histiocytosis X, and must be prudently differentiated from these malignancies to avoid the morbidity of unnecessary treatment. It could be categorized into two subtypes, according to the presence or absence of visceral involvement. Radiologically, this condition may be evaluated using roentgenography, ultrasound, computed tomography, or magnetic resonance imaging. Both the solitary and the multicentric variety without visceral involvement carry a good prognosis with spontaneous regression of the lesions in most of the cases, whereas patients with involvement of vital viscera have a high mortality. Surgical intervention may be considered in the event of coexisting vital organ compression. This report presents a case of multicentric infantile myofibromatosis, diagnosed by tissue biopsy of a left limb nodular mass and left occipital skull lesion in a 4-month-old male infant, who eventually succumbed to primary pulmonary hypertension. Case Report The 4-month-old male infant was born by normal vaginal delivery at term, after an uneventful pregnancy, weighing 2800 gm, without perinatal insults. Newborn screening detected no specific abnormalities. Growth and developmental milestones were appropriate for his age, but one soft mass lesion (3 cm ⫻ 3 cm) in the left occipital area, and multiple palpable skin-colored nodules over his face, trunk, and legs were noticed since birth. His father had an unidentified mass on his left occipital scalp, which was excised at 1 year of age, and two paternal cousins also suffered from unidentified solitary limb masses. Neurosonography was arranged on his first visit to our clinic, which revealed one subcutaneous echolucency (22 mm ⫻ 13 mm) without apparent blood flow. Brain magnetic resonance imaging was performed later, and two sites of abnormal findings were disclosed: (1) a subcutaneous cystic structure at the left occipital scalp region with marginal wall enhancement after contrast administration, hypointensity on T1-weighted image, and hyperintensity on T2-weighted image; (2) a soft tissue–like nodular mass over the left adjacent occipital calvarium diploe, with internal extension and compression to the adjacent sigmoid sinus, but no
Communications should be addressed to: Dr. Lin; Division of Pediatric Neurology; Chang Gung Children’s Hospital; 5 Fu-Shin Street; Kwei-Shan, Taoyuan, 333; Taiwan. Received February 2, 2005; accepted May 2, 2005.
© 2005 by Elsevier Inc. All rights reserved. doi:10.1016/j.pediatrneurol.2005.05.009 ● 0887-8994/05/$—see front matter
Figure 2. Chest roentgenography including four extremities disclosed multiple extensive osteolytic lesions in the ribs, humerus, radius, ulna, femur, tibia, and fibula, with prominent loss of cortical boarders.
of this histopathologic examination was also suggestive of infantile myofibromatosis. During hospitalization, abdominal ultrasonography was arranged for surveillance of visceral involvement. A well-defined, walled-off, and central hypoechoic homogenous mass (11 mm ⫻ 8 mm) was observed at the right lower quadrant of the abdominal wall. The findings of spinal neurosonography were negative. He was discharged in stable condition. Unfortunately, he had no follow-up since age 5 months. Six months later, he was brought to our emergency room because of abdominal distention and intermittent postprandial nonbilious vomiting (observed for 2 weeks before admission), aggravated tachypnea, grunting, and lip cyanosis. Frequent irritable crying was also evident. Chest roentgenography indicated cardiomegaly and mildly increased pulmonary infiltration. Echocardiography disclosed a remarkable dilatation of
Figure 1. T1-weighted brain magnetic resonance imaging with and without contrast enhancement revealed: a subcutaneous cystic structure (white arrow) at the left occipital scalp region with marginal wall enhancement after contrast enhancement, hypointensity on T1-weighted image (A: TR/TE: 504/15 ms) and hyperintensity on T2-weighted image (B: TR/TE: 3500/99 ms); a soft tissue-like nodular mass (white arrowhead) over the left adjacent occipital calvarium dipole, with internal extension and compression to the adjacent sigmoid sinus, but no evidence of connection to brain parenchyma.
evidence of connection to brain parenchyma (Fig 1). The pathology findings of a skin biopsy through the left limb mass lesion revealed a dermal biphasic tumor composed of bundles of smooth muscle-like spindle cells, accompanied by focal apoptosis and areas of hyaline sclerosis that were compatible with infantile myofibromatosis. However, chest roentgenography disclosed multiple osteolytic lesions at the ribs and four extremities, especially at the metaphyses of long bones (Fig 2). The bone window view on brain computed tomography also revealed multiple radiolucency areas over the calvarium (Fig 3). Left occipital craniotomy to remove the skull tumor was accomplished. The final report
Figure 3. Bone window view on brain computed tomography revealed multiple osteolytic lesions.
Huang et al: Infantile Myofibromatosis 297
the right atrium and right ventricle, moderate degree of tricuspid regurgitation, and prominent shift in the interatrial and interventricular septum toward the left side, making apparent volume restriction of the left atrium and left ventricle. Therefore, severe pulmonary artery hypertension was suggested. However, there were no other cardiogenic defects. Abdominal ultrasonography and neurosonography disclosed no specific abnormalities. The findings of septic evaluation, peripheral blood smear, coagulation function, electrolytes, alanine aminotransferase, aspartate aminotransferase, creatine kinase, and autoimmune study (complement 3, complement 4, and antinuclear antibody) were all negative. The patient expired 12 hours after admission.
Discussion Infantile myofibromatosis is a mesenchymal tumor, most commonly observed in infancy and childhood. Sporadic or familial cases with male predominance have been reported [3]. Though autosomal dominant or autosomal recessive inheritance has been suggested, cytogenetic and fluorescent in situ hybridization analyses reveal a pseudodiploid karyotype with an interstitial deletion of the long arm of one chromosome 6, del (6) (q12 q15), as the sole anomaly [4]. Hereditary tendency and male predominance were evident in this patient’s family. Infantile myofibromatosis may occur in two distinct forms: solitary type and multicentric type. The solitary type is defined by the presence of one nodule in the skin, muscle, bone, or subcutaneous tissue; and the multicentric type can be divided into two subtypes, according to whether there is visceral involvement [5,6]. The solitary form manifests itself in more than half of the cases, mostly involving the skin and bones, and rarely the central nervous system and viscera. Brain myofibromas are usually intracranial and in the proximity of the dura mater, with infiltration of the calvarial bones and secondary brain compression [6]. Spine myofibromatosis is exceptional. Other localizations (i.e., of the orbital region, gingiva, bone, muscle, viscera [heart, lungs, liver, gastrointestinal tract, bile ducts, pancreas, and endocrine organs], and skin) are inconclusive. The natural history of infantile myofibromatosis is characterized by a period of rapid growth, subsequent stabilization, and spontaneous regression in many cases, especially of the solitary type [6]. In the present case, the patient’s multiple lesions (i.e., osteolytic bone lesion, skin lesion, and abdominal wall mass) indicate the multicentric type of infantile myofibromatosis without visceral involvement. Nevertheless, the gastrointestinal and cardiopulmonary complications may have indicated the involvement of visceral organs at the end of his life. Radiologic evaluation of infantile myofibromatosis is important in determining the extent of the disease and in monitoring disease progression or regression. Infantile myofibromatosis may be evaluated by roentgenography, ultrasound, computed tomography, or magnetic resonance imaging, and has been identified prenatally by ultrasound examination [7]. On plain film, the lesions appear as multiple areas of osteolysis with a sclerotic margin.
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Ultrasound reveals hyperechoic nodules with peripheral hyperperfusion on color Doppler. Computed tomography, in particular, is able to demonstrate calcification in soft tissues, but it may underestimate the true extent of the disease. The signal intensity on magnetic resonance imaging is variable, but lesions are usually demonstrated as either hypointense or isointense to the surrounding musculature on T1-weighted images and as high signal intensity on T2-weighted images [8]. Moreover, gadoliniumenhanced magnetic resonance imaging may be useful in documenting dural involvement. The excellent soft tissue differentiation afforded by magnetic resonance imaging allows the location and size of subcutaneous and intramuscular tumors to be clearly delineated. However, small nodules on the surface of the stomach and the colon, which are identified at surgery, are not visualized on magnetic resonance imaging. In our case, frequent postprandial vomiting, with the initial presentations of respiratory distress and cyanotic change, preceded his unexpected downhill course. Even though there was no evidence of gastrointestinal involvement, the presence of bowel obstruction was still a cause for suspicion. Consequently, further effort must be made to depict lesions in the gastrointestinal tract because they dramatically increase infant mortality. As infants with this condition are often monitored for evidence of progression or regression, magnetic resonance imaging has the important advantage over roentgenography and computed tomography of allowing imaging follow-up without incurring a radiation dose to the patient. The prognosis of this disease depends on the vital organs involved, as death from infantile myofibromatosis results from complications caused by cardiopulmonary or gastrointestinal involvement. Our review of the published literature revealed that infantile myofibromatosis complicated with primary pulmonary hypertension has been rarely reported. Could this underlying disease be associated with the possibility of progressive change in cardiopulmonary vasculature? This likelihood may be prudently taken into consideration. According to the study of Wiswell et al., the multicentric form with involvement of the gastrointestinal, cardiac, or pulmonary system can increase the mortality as high as 73% when one of these three systems is involved [9,10]. Infants with a solitary lesion or multiple lesions without visceral involvement generally have benign courses, usually with spontaneous regression within 1 to 2 years of diagnosis. Visceral lesions are associated with a significant morbidity and mortality, resulting from vital organ obstruction, failure to thrive, or infection. Death in these cases often occurs at birth, or soon after, and is usually the result of cardiopulmonary or gastrointestinal complications. Once the diagnosis is confirmed, observation is the treatment of choice [11]. In addition to medical treatment, surgical intervention may be postponed and dealt with conservatively unless life-threatening pressure on vital organs is present.
Complete excision is often curative, with a reported recurrence rate of 7-10% [1]. In conclusion, because these lesions may not be easily discernible and most spontaneously resolve, the condition is underdiagnosed and underreported. Infantile myofibromatosis must be considered in any child who presents with either solitary or multiple tumors, particularly those occurring in the neonatal period. Especially, a cystic mass on the scalp may mimic a dermoid cyst. Close surveillance of the visceral organs is important because of the attendant risk of fatal complications. References [1] Stout AP. Juvenile fibromatosis. Cancer 1954;7:953-78. [2] Chung EB, Enzinger FM. Infantile myofibromatosis. Cancer 1981;48:1807-18. [3] Michel M, Ninane J, Claus D, Gosseye S, Wese FX, Moulin D. Major malformations in a case of infantile myofibromatosis. Eur J Pediatr 1990;149:251-2.
[4] Stenman G, Nadal N, Persson S, Gunterberg B, Angervall L. del (6)(q12q15) as the sole cytogenetic anomaly in a case of solitary infantile myofibromatosis. Oncol Rep 1999;6:1101-4. [5] Johnson GL, Baisden BL, Fishman EK. Infantile myofibromatosis. Skeletal Radiol 1997;26:611-4. [6] Tamburrini G, Gessi M, Colosimo C Jr, Lauriola L, Giangaspero F, Di Rocco C. Infantile myofibromatosis of the central nervous system. Childs Nerv Syst 2003;19:650-4. [7] Liew SH, Haynes M. Localized form of congenital generalized fibromatosis. A report of 3 cases with myofibroblasts. Pathology 1981; 13:257-66. [8] Kubota A, Imano M, Yonekura T, et al. Infantile myofibromatosis of the triceps detected by prenatal sonography. J Clin Ultrasound 1999;27:147-50. [9] Davies RS, Carty H, Pierro A. Infantile myofibromatosis—a review. Brit J Radiol 1994;67:619-23. [10] Wiswell TE, Davis J, Cunningham BE, Solenberger R, Thomas PJ. Infantile myofibromatosis: The most common fibrous tumor of infancy. J Pediatr Surg 1988;23:315-8. [11] Moore JB, Waldenmaier N, Potchen EJ. Congenital generalized fibromatosis: A new management strategy provided by magnetic resonance imaging. Am J Dis Child 1987;141:714-6.
Huang et al: Infantile Myofibromatosis 299
From *Division of Pediatric Neurology, Chang Gung Children’s Hospital; †Department of Pathology, Chang Gung Memorial Hospital; and ‡Department of Neurosurgery, Chang Gung Memorial Hospital, Chang Gung University Medical College, Taoyuan, Taiwan.
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Introduction Infantile myofibromatosis is a rare benign disorder of infancy and childhood, characterized by solitary or multicentric mesenchymal tumors of the skin, soft tissue, bone, viscera or central nervous system. It was first described as “congenital fibrosarcoma” in 1951 by Williams and Schrum [cited in 1]. In 1981, Chung and Enzinger reviewed 61 cases, and gave the disease its current name on the basis of histologic features [2]. Infantile myofibromatosis may mimic other conditions, such as fibrosarcoma, leiomyosarcoma, rhabdomyosarcoma, or histiocytosis X, and must be prudently differentiated from these malignancies to avoid the morbidity of unnecessary treatment. It could be categorized into two subtypes, according to the presence or absence of visceral involvement. Radiologically, this condition may be evaluated using roentgenography, ultrasound, computed tomography, or magnetic resonance imaging. Both the solitary and the multicentric variety without visceral involvement carry a good prognosis with spontaneous regression of the lesions in most of the cases, whereas patients with involvement of vital viscera have a high mortality. Surgical intervention may be considered in the event of coexisting vital organ compression. This report presents a case of multicentric infantile myofibromatosis, diagnosed by tissue biopsy of a left limb nodular mass and left occipital skull lesion in a 4-month-old male infant, who eventually succumbed to primary pulmonary hypertension. Case Report The 4-month-old male infant was born by normal vaginal delivery at term, after an uneventful pregnancy, weighing 2800 gm, without perinatal insults. Newborn screening detected no specific abnormalities. Growth and developmental milestones were appropriate for his age, but one soft mass lesion (3 cm ⫻ 3 cm) in the left occipital area, and multiple palpable skin-colored nodules over his face, trunk, and legs were noticed since birth. His father had an unidentified mass on his left occipital scalp, which was excised at 1 year of age, and two paternal cousins also suffered from unidentified solitary limb masses. Neurosonography was arranged on his first visit to our clinic, which revealed one subcutaneous echolucency (22 mm ⫻ 13 mm) without apparent blood flow. Brain magnetic resonance imaging was performed later, and two sites of abnormal findings were disclosed: (1) a subcutaneous cystic structure at the left occipital scalp region with marginal wall enhancement after contrast administration, hypointensity on T1-weighted image, and hyperintensity on T2-weighted image; (2) a soft tissue–like nodular mass over the left adjacent occipital calvarium diploe, with internal extension and compression to the adjacent sigmoid sinus, but no
Communications should be addressed to: Dr. Lin; Division of Pediatric Neurology; Chang Gung Children’s Hospital; 5 Fu-Shin Street; Kwei-Shan, Taoyuan, 333; Taiwan. Received February 2, 2005; accepted May 2, 2005.
© 2005 by Elsevier Inc. All rights reserved. doi:10.1016/j.pediatrneurol.2005.05.009 ● 0887-8994/05/$—see front matter
Figure 2. Chest roentgenography including four extremities disclosed multiple extensive osteolytic lesions in the ribs, humerus, radius, ulna, femur, tibia, and fibula, with prominent loss of cortical boarders.
of this histopathologic examination was also suggestive of infantile myofibromatosis. During hospitalization, abdominal ultrasonography was arranged for surveillance of visceral involvement. A well-defined, walled-off, and central hypoechoic homogenous mass (11 mm ⫻ 8 mm) was observed at the right lower quadrant of the abdominal wall. The findings of spinal neurosonography were negative. He was discharged in stable condition. Unfortunately, he had no follow-up since age 5 months. Six months later, he was brought to our emergency room because of abdominal distention and intermittent postprandial nonbilious vomiting (observed for 2 weeks before admission), aggravated tachypnea, grunting, and lip cyanosis. Frequent irritable crying was also evident. Chest roentgenography indicated cardiomegaly and mildly increased pulmonary infiltration. Echocardiography disclosed a remarkable dilatation of
Figure 1. T1-weighted brain magnetic resonance imaging with and without contrast enhancement revealed: a subcutaneous cystic structure (white arrow) at the left occipital scalp region with marginal wall enhancement after contrast enhancement, hypointensity on T1-weighted image (A: TR/TE: 504/15 ms) and hyperintensity on T2-weighted image (B: TR/TE: 3500/99 ms); a soft tissue-like nodular mass (white arrowhead) over the left adjacent occipital calvarium dipole, with internal extension and compression to the adjacent sigmoid sinus, but no evidence of connection to brain parenchyma.
evidence of connection to brain parenchyma (Fig 1). The pathology findings of a skin biopsy through the left limb mass lesion revealed a dermal biphasic tumor composed of bundles of smooth muscle-like spindle cells, accompanied by focal apoptosis and areas of hyaline sclerosis that were compatible with infantile myofibromatosis. However, chest roentgenography disclosed multiple osteolytic lesions at the ribs and four extremities, especially at the metaphyses of long bones (Fig 2). The bone window view on brain computed tomography also revealed multiple radiolucency areas over the calvarium (Fig 3). Left occipital craniotomy to remove the skull tumor was accomplished. The final report
Figure 3. Bone window view on brain computed tomography revealed multiple osteolytic lesions.
Huang et al: Infantile Myofibromatosis 297
the right atrium and right ventricle, moderate degree of tricuspid regurgitation, and prominent shift in the interatrial and interventricular septum toward the left side, making apparent volume restriction of the left atrium and left ventricle. Therefore, severe pulmonary artery hypertension was suggested. However, there were no other cardiogenic defects. Abdominal ultrasonography and neurosonography disclosed no specific abnormalities. The findings of septic evaluation, peripheral blood smear, coagulation function, electrolytes, alanine aminotransferase, aspartate aminotransferase, creatine kinase, and autoimmune study (complement 3, complement 4, and antinuclear antibody) were all negative. The patient expired 12 hours after admission.
Discussion Infantile myofibromatosis is a mesenchymal tumor, most commonly observed in infancy and childhood. Sporadic or familial cases with male predominance have been reported [3]. Though autosomal dominant or autosomal recessive inheritance has been suggested, cytogenetic and fluorescent in situ hybridization analyses reveal a pseudodiploid karyotype with an interstitial deletion of the long arm of one chromosome 6, del (6) (q12 q15), as the sole anomaly [4]. Hereditary tendency and male predominance were evident in this patient’s family. Infantile myofibromatosis may occur in two distinct forms: solitary type and multicentric type. The solitary type is defined by the presence of one nodule in the skin, muscle, bone, or subcutaneous tissue; and the multicentric type can be divided into two subtypes, according to whether there is visceral involvement [5,6]. The solitary form manifests itself in more than half of the cases, mostly involving the skin and bones, and rarely the central nervous system and viscera. Brain myofibromas are usually intracranial and in the proximity of the dura mater, with infiltration of the calvarial bones and secondary brain compression [6]. Spine myofibromatosis is exceptional. Other localizations (i.e., of the orbital region, gingiva, bone, muscle, viscera [heart, lungs, liver, gastrointestinal tract, bile ducts, pancreas, and endocrine organs], and skin) are inconclusive. The natural history of infantile myofibromatosis is characterized by a period of rapid growth, subsequent stabilization, and spontaneous regression in many cases, especially of the solitary type [6]. In the present case, the patient’s multiple lesions (i.e., osteolytic bone lesion, skin lesion, and abdominal wall mass) indicate the multicentric type of infantile myofibromatosis without visceral involvement. Nevertheless, the gastrointestinal and cardiopulmonary complications may have indicated the involvement of visceral organs at the end of his life. Radiologic evaluation of infantile myofibromatosis is important in determining the extent of the disease and in monitoring disease progression or regression. Infantile myofibromatosis may be evaluated by roentgenography, ultrasound, computed tomography, or magnetic resonance imaging, and has been identified prenatally by ultrasound examination [7]. On plain film, the lesions appear as multiple areas of osteolysis with a sclerotic margin.
298
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Ultrasound reveals hyperechoic nodules with peripheral hyperperfusion on color Doppler. Computed tomography, in particular, is able to demonstrate calcification in soft tissues, but it may underestimate the true extent of the disease. The signal intensity on magnetic resonance imaging is variable, but lesions are usually demonstrated as either hypointense or isointense to the surrounding musculature on T1-weighted images and as high signal intensity on T2-weighted images [8]. Moreover, gadoliniumenhanced magnetic resonance imaging may be useful in documenting dural involvement. The excellent soft tissue differentiation afforded by magnetic resonance imaging allows the location and size of subcutaneous and intramuscular tumors to be clearly delineated. However, small nodules on the surface of the stomach and the colon, which are identified at surgery, are not visualized on magnetic resonance imaging. In our case, frequent postprandial vomiting, with the initial presentations of respiratory distress and cyanotic change, preceded his unexpected downhill course. Even though there was no evidence of gastrointestinal involvement, the presence of bowel obstruction was still a cause for suspicion. Consequently, further effort must be made to depict lesions in the gastrointestinal tract because they dramatically increase infant mortality. As infants with this condition are often monitored for evidence of progression or regression, magnetic resonance imaging has the important advantage over roentgenography and computed tomography of allowing imaging follow-up without incurring a radiation dose to the patient. The prognosis of this disease depends on the vital organs involved, as death from infantile myofibromatosis results from complications caused by cardiopulmonary or gastrointestinal involvement. Our review of the published literature revealed that infantile myofibromatosis complicated with primary pulmonary hypertension has been rarely reported. Could this underlying disease be associated with the possibility of progressive change in cardiopulmonary vasculature? This likelihood may be prudently taken into consideration. According to the study of Wiswell et al., the multicentric form with involvement of the gastrointestinal, cardiac, or pulmonary system can increase the mortality as high as 73% when one of these three systems is involved [9,10]. Infants with a solitary lesion or multiple lesions without visceral involvement generally have benign courses, usually with spontaneous regression within 1 to 2 years of diagnosis. Visceral lesions are associated with a significant morbidity and mortality, resulting from vital organ obstruction, failure to thrive, or infection. Death in these cases often occurs at birth, or soon after, and is usually the result of cardiopulmonary or gastrointestinal complications. Once the diagnosis is confirmed, observation is the treatment of choice [11]. In addition to medical treatment, surgical intervention may be postponed and dealt with conservatively unless life-threatening pressure on vital organs is present.
Complete excision is often curative, with a reported recurrence rate of 7-10% [1]. In conclusion, because these lesions may not be easily discernible and most spontaneously resolve, the condition is underdiagnosed and underreported. Infantile myofibromatosis must be considered in any child who presents with either solitary or multiple tumors, particularly those occurring in the neonatal period. Especially, a cystic mass on the scalp may mimic a dermoid cyst. Close surveillance of the visceral organs is important because of the attendant risk of fatal complications. References [1] Stout AP. Juvenile fibromatosis. Cancer 1954;7:953-78. [2] Chung EB, Enzinger FM. Infantile myofibromatosis. Cancer 1981;48:1807-18. [3] Michel M, Ninane J, Claus D, Gosseye S, Wese FX, Moulin D. Major malformations in a case of infantile myofibromatosis. Eur J Pediatr 1990;149:251-2.
[4] Stenman G, Nadal N, Persson S, Gunterberg B, Angervall L. del (6)(q12q15) as the sole cytogenetic anomaly in a case of solitary infantile myofibromatosis. Oncol Rep 1999;6:1101-4. [5] Johnson GL, Baisden BL, Fishman EK. Infantile myofibromatosis. Skeletal Radiol 1997;26:611-4. [6] Tamburrini G, Gessi M, Colosimo C Jr, Lauriola L, Giangaspero F, Di Rocco C. Infantile myofibromatosis of the central nervous system. Childs Nerv Syst 2003;19:650-4. [7] Liew SH, Haynes M. Localized form of congenital generalized fibromatosis. A report of 3 cases with myofibroblasts. Pathology 1981; 13:257-66. [8] Kubota A, Imano M, Yonekura T, et al. Infantile myofibromatosis of the triceps detected by prenatal sonography. J Clin Ultrasound 1999;27:147-50. [9] Davies RS, Carty H, Pierro A. Infantile myofibromatosis—a review. Brit J Radiol 1994;67:619-23. [10] Wiswell TE, Davis J, Cunningham BE, Solenberger R, Thomas PJ. Infantile myofibromatosis: The most common fibrous tumor of infancy. J Pediatr Surg 1988;23:315-8. [11] Moore JB, Waldenmaier N, Potchen EJ. Congenital generalized fibromatosis: A new management strategy provided by magnetic resonance imaging. Am J Dis Child 1987;141:714-6.
Huang et al: Infantile Myofibromatosis 299