JOURNAL OF MEDICALCASE REPORTS

Chen and Chang Journal of Medical Case Reports 2014, 8:210http://www.jmedicalcasereports.com/content/8/1/210

CASE REPORT Open Access

An unusual timing for symptomatic chest pain inan adult chest wall myofibroma: a case reportChin-Li Chen and Hung Chang*

Abstract

Introduction: Myofibromas are benign mesenchymal neoplasms that can present as solitary and multicentric lesions.They can occur in several locations and can occur at any age from neonates to elderly patients. However, most of thelesions are found in neonates and babies. It rarely occurs in adults.

Case presentation: A 29-year-old Taiwanese man presented with persistent dull chest pain in his right lateral chestwall for 2 weeks. A chest X-ray showed a faint patchy opacity over the periphery of his right upper lung zone.Computed tomography and magnetic resonance imaging showed a lobulated mass at the intercostal spacebetween his right fifth and sixth ribs with contrast enhancement and bone invasion. Malignancy could not beexcluded. A percutaneous needle aspiration biopsy failed due to technique issues, so he underwent a thoracotomyand the tumor was excised with Marlex mesh repairs for the thoracic defect. Pathology confirmed a myofibromawithout malignancy. He recovered uneventfully and no local recurrence was detected at the 1-year follow-upexamination.

Conclusions: Chest wall myofibroma presenting with chest pain has never been reported in adults. It is a challenge todifferentiate myofibroma from malignancy in chest wall preoperatively, such as seen in our patient. To the best of ourknowledge, this has not been previously reported in the scientific literature. Although myofibroma rarely occurs in thechest wall and adults, it must be suspected in any chest wall tumor presenting with chest pain.

Keywords: Chest pain, Chest wall tumor, Myofibroma, Thoracotomy

IntroductionMyofibroma is a benign mesenchymal neoplasm composedof myofibroblasts. Most myofibromas consist of a firm,well-circumscribed mass and grow slowly. Many termshave been used for this neoplasm since the first reportin 1951, including “benign neoplasm” and “malignantneoplasm” [1]. Consequently, it is easily misdiagnosed.Myofibromas can occur in several locations includingskin, subcutaneous tissues, skeletal muscle, oral cavity,head and extremities [2]. Clinically, myofibromas mostcommonly present in children younger than 2-years oldand two-thirds of these lesions present at birth [2].Myofibromas rarely occur in adults and a chest wallmyofibroma has never been reported in an adult. Tothe best of our knowledge, this is the first documented

* Correspondence: hungc888@yahoo.com.twDivision of Thoracic Surgery, Department of Surgery, Tri-Service GeneralHospital, National Defense Medical Center, No.325, Section 2, Cheng-KungRoad, Taipei 114, Taiwan

© 2014 Chen and Chang; licensee BioMed CeCreative Commons Attribution License (http:/distribution, and reproduction in any mediumDomain Dedication waiver (http://creativecomarticle, unless otherwise stated.

case of a chest wall myofibroma in an adult and initiallyit is a challenge to differentiate from a malignancy.

Case presentationA 29-year-old Taiwanese man presented to our emer-gency department with persistent dull pain over his rightchest wall for 2 weeks. His chest pain was characterizedby a persistent dull ache in his right lateral chest wall.No other constitutional symptoms were noticed. Fur-thermore, he did not have a palpable mass over his rightchest wall and the external appearance of his body wasnormal. A physical examination was unremarkable ex-cept for mild tenderness over his right chest wall.A chest X-ray showed a faint patchy opacity over the

periphery of his right upper lung zone (Figure 1). Chestcomputed tomography (CT) showed a lobulated mass ap-proximately 5.5×4.5×4.3cm in size at the intercostal spacebetween his right fifth and sixth ribs. Magnetic resonanceimaging (MRI) of his thorax revealed a 5.2×4.5×4.6cmlobulated mass with bone invasion of the adjacent ribs

ntral Ltd. This is an Open Access article distributed under the terms of the/creativecommons.org/licenses/by/2.0), which permits unrestricted use,, provided the original work is properly credited. The Creative Commons Publicmons.org/publicdomain/zero/1.0/) applies to the data made available in this

Figure 1 Chest X-ray of the patient. A chest X-ray showed a faintpatchy opacity over the periphery of the right upper lung zone(black arrow).

Figure 3 The gross picture of the tumor. A soft tissue massmeasured approximately 5.0×5.0×4.2cm in size with awhite-yellowish appearance.

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(Figure 2). The mass was isointense to hypointense rela-tive to the muscle on T1 pregadolinium, and heteroge-neously hyperintense on fat-suppressed T2 pregadoliniumwith some low-signal spots and tube-like structures. Itshowed inhomogeneous contrast enhancement with gado-linium injection. Malignancy could not be excluded ac-cording to the image studies.We expected to perform a CT-guided needle aspiration

biopsy initially, but it failed due to the barriers of right ser-ratus anterior muscle, latissimus dorsi muscles and scap-ula. Subsequently, we performed a standard thoracotomyand excised the tumor (Figure 3). During surgery, thetumor was found not to adhere to the lung parenchyma.After separating the latissimus dorsi muscles and serratusanterior muscles, we noted a solid and firmly fixed tumormeasuring 5.0×5.0×4.2cm in size over his fifth to sixthintercostal space and it could not be dissected off his fifth

Figure 2 Magnetic resonance imaging of chest shows a 5.2×4.5×4.6cmfifth intercostal space and the subpleural space (white arrow). (A) T1-(B) T2-weighted image shows heterogeneously hyperintense with some low-inhomogeneous contrast enhancement.

and sixth ribs. Subsequently, tumor excision along withsegmental resection of his fifth and sixth ribs was done.The defect of his chest wall was reconstructed withMarlex mesh. Grossly the tumor was white-yellow in colorand had a smooth appearance. On histological examin-ation, the lesion presented as a hypocellular myxoid tumorcomposed of short fascicles, tumor cells and abundantmyxoid stroma (Figure 4). Neither cytologic atypia norpleomorphism was observed. The findings were compat-ible with the diagnosis of a chest wall myofibroma. Aftersurgery, he recovered uneventfully and was discharged onpostoperative day 6. No recurrence was detected at the 1-year follow-up examination.

DiscussionMyofibroma (unifocal) and myofibromatosis (multifocal)are rare spindle-cell neoplasms composed of myofibro-blasts. Typically, these lesions present in children and

mass involving the right serratus anterior muscle, the rightweighted image shows isointense to hypointense relative to the muscle.signal spots. (C) T2-weighted image with gadolinium injection shows

Figure 4 The histopathology of the tumor. (A) Calponin ×400: hypocellular myxoid appearance with short fascicles, tumor cells and abundantmyxoid stroma. (B) Hematoxylin and eosin stain ×200: focal bone invasion.

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babies, which prompted the use of terms such as congenitalfibrosarcoma, congenital generalized fibromatosis, congeni-tal mesenchymal hamartoma and infantile myofibromatosis[2]. Neonates and babies have the highest incidence ofmyofibroma and myofibromatosis: 90% of myofibromatosiscases occur before the age of 2 years, and two-thirds arepresent at birth. Nonetheless, both solitary and multicentriclesions can occur at any age [2]. Adult symptomatic chestwall myofibroma were rarely reported.These lesions mostly involve the head and neck, al-

though some cases of involvement of the trunk and ex-tremities have been reported [2]. The most commonlocations include skin, subcutaneous tissues, skeletalmuscle and oral cavity [2]. Most myofibromas consist ofa firm, well-circumscribed mass and grow slowly. Thedifferential diagnosis includes the possibility of infections(tuberculosis or fungal growths), benign and malignantconnective tissue lesions (fibroma, peripheral giant cellgranuloma, fibrous histiocytoma, fibromatosis, fibrosar-coma or malignant fibrous histiocytoma), benign and ma-lignant neural neoplasms (palisaded encapsulated neuroma,schwannoma, neurofibroma or neurofibromatosis and neu-rofibrosarcoma), benign and malignant muscle neoplasms

Table 1 Chest wall tumors and related symptoms

Common presenting age (years

Myofibroma (the reported case) N/A

Lipoma 50–70

Multiple myeloma >60

Osteosarcoma 10–25, and >40

Ewing sarcoma (Askin’s tumor) 14 (most in children andsometimes in young adults)

Chondrosarcoma >50

Breast carcinoma in men 65–67

Metastatic neoplasm Usually in adults

N/A: Not Applicable.

(leiomyoma, rhabdomyoma, leiomyosarcoma, rhabdo-myosarcoma or alveolar soft-part sarcoma), vascular lesions(hemangiomas), salivary gland pathology (for example,pleomorphic adenoma or mucoepidermoid carcinoma)and lymphoma or chloroma [2,3]. Common chest walltumors and their related symptoms are listed in Table 1.The prognosis for most lesions is excellent, with the ex-ception of generalized myofibromatosis, a condition as-sociated with multicentric visceral lesions of the vitalorgans of babies that has an aggressive and sometimesfatal outcome [2].In this case, the myofibroma does not result in changing

of the external appearance or painful motion. On MRIexamination, a unique postgadolinium feature of myofi-bromas is a central area of hypointensity surrounded byperipheral hyperintense enhancement [4]. However, in ourcase, the mass is heterogeneously hyperintense on T2pregadolinium and inhomogeneous on contrast enhance-ment with gadolinium injection. A malignant neoplasmcannot be totally excluded. Clinically, diagnosis optionsinclude an excisional biopsy, a simple excision, or needleaspiration biopsy. However, needle aspiration biopsy wasdifficult to perform in our case because the mass lesion

old) Typical symptoms

N/A

An asymptomatic, well-circumscribed mobile mass

Bone pain when movement and changing position

A rapidly expanding, painful chest wall mass(usually in ribs and sternum)

Chest wall pain (usually a solitary tumor in ribs,sternum, scapula, clavicle, or paravertebral region)

An enlarging, painful, anterior chest wall mass (commoninvolvement of costochondral arches or sternum)

A painless firm mass

Chest wall pain (might include pathologic fracture)

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was deeply located and hindered by the right serratusanterior muscle, latissimus dorsi muscles and scapula.According to the reports, the myofibromas generallyresponded well to surgical treatment alone [1,2].

ConclusionsIn summary, although myofibroma and myofibromatosistypically present in children and babies, our case intro-duces myofibroma as a lesion located in the chest walland presented with chest pain of an adult man. To thebest of our knowledge, this is the first report of a myofi-broma in an adult man that involved the chest wall. Al-though myofibroma rarely occurs in the chest wall andadults, the possibility of a chest wall tumor presentingwith chest pain must be considered.

ConsentWritten informed consent was obtained from the patientfor publication of this case report and any accompanyingimages. A copy of the written consent is available for re-view by the Editor-in-Chief of this journal.

AbbreviationsCT: Computed tomography; MRI: Magnetic resonance imaging.

Competing interestsThe authors declare that they have no competing interests.

Authors’ contributionsBoth authors participated in the patient’s diagnosis and medical management.C-LC drafted the first version of the manuscript and HC drafted the revisedmanuscript. Both authors have read and approved the manuscript.

AcknowledgementsThere is no substantial direct or indirect commercial financial incentiveassociated with publishing this article.

Received: 28 December 2013 Accepted: 7 April 2014Published: 19 June 2014

References1. Azevedo Rde S, Pires FR, Della Coletta R, de Almeida OP, Kowalski LP, Lopes

MA: Oral myofibromas: report of two cases and review of clinical andhistopathologic differential diagnosis. Oral Surg Oral Med Oral Pathol OralRadiol Endod 2008, 105:e35–e40.

2. Scheper MA, Difabio VE, Sauk JJ, Nikitakis NG: Myofibromatosis: a casereport with a unique clinical presentation. Oral Surg Oral Med Oral PatholOral Radiol Endod 2005, 99:325–330.

3. Marciani RD, White DK, Gotcher JE Jr: Recurrent hard palate lesion in achild. J Oral Maxillofac Surg 1999, 57:1127–1131.

4. Lope LA, Hutcheson KA, Khademian ZP: Magnetic resonance imaging inthe analysis of pediatric orbital tumors: utility of diffusion-weightedimaging. J AAPOS 2010, 14:257–262.

doi:10.1186/1752-1947-8-210Cite this article as: Chen and Chang: An unusual timing for symptomaticchest pain in an adult chest wall myofibroma: a case report. Journal ofMedical Case Reports 2014 8:210.

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