Management of Symptomatic Mesenchymal Hamartoma of the Chest Wall: Surgical Resection Only in Symptomatic Cases

Abstract

Mesenchymal hamartoma of the chest wall (MHCW) is a rare neonatal benign tumor with an estimated incidence of 1 in 3 000 among primary bone tumors, and 1 in one million in the general population. Traditionally, the treatment of choice was an “en bloc” resection, but surgery limited to symptomatic cases, is now suggested by most authors due to the numerous cases of spontaneous regressions. We report 2 patients of symptomatic MHCW, characterized by progressive respiratory distress, who underwent surgical treatment with prompt resolution of symptoms. Surgeons and neonatologists should be aware of this rare condition and its possible fatal or nearly-fatal complications.

Zusammenfassung

Das mesenchymale Hamartom der Brustwand (MHCW) ist ein seltener gutartiger Tumor bei Neugeborenen mit einer geschätzten Inzidenz von 1:3 000 unter primären Knochentumoren und 1:1 000 000 in der gesamten Bevölkerung. ­Traditionell war die Therapie eine „en bloc“-Resektion, aber von den meisten Autoren wird nun ­aufgrund der zahlreichen Fälle mit spontaner Regression eine Resektion ausschließlich bei symptomatischen Fällen vorgeschlagen. Wir berichten über 2 Fälle von symptomatischer MHCW, gekenn­zeichnet durch progressive, schwere Atemnot, die nach Operation beschwerdefrei sind. Chirurgen und Neonatologen sollten sich dieser seltenen, fast immer heilbaren Erkrankung bewusst sein, die auch schwerwiegende oder tödliche Kom­plikationen haben kann.

Key words

mesenchymal chest wall hamartoma - benign soft-tissue tumors - thoracic tumors - neonatal tumors

Schlüsselwörter

mesenchymales Brustwand– Hamartom - gutartige Weichteiltumoren - Brusttumoren - Neugeborenentumoren

• References

• 1 Amstalden EM, Carvalho RB, Pacheco EM et al. Chondromatous hamartoma of the chest wall: description of 3 new cases and literature review. Int J Surg Pathol 2006; 14: 119-126
• 2 Bertocchini A, Falappa P, Accinni A et al. Radiofrequency thermoablation in chest wall mesenchymal hamartoma of an infant. Ann Thorac Surg 2007; 84: 2091-2093
• 3 Bisogno G, Ferrari A, Bien E et al. Rare cancers in children – the EXPeRT initiative: a report from the european cooperative study group on pediatric rare tumors. Klin Padiatr 2012; 224: 416-420
• 4 Braatz B, Evans R, Kelman A et al. Perinatal evolution of mesenchymal hamartoma of the chest wall. J Pediatr Surg 2010; 45: e37-e40
• 5 Brecht IB, Graf N, Dv Schweinitz et al. Networking for children and adolescents with very rare tumors: foundation of the GPOH Pediatric Rare Tumor Group. Klin Padiatr 2009; 221: 181-185
• 6 Cameron D, Ong TH, Borzi P. Conservative management of mesenchymal hamartomas of the chest wall. J Pediatr Surg 2001; 36: 1346-1349
• 7 Castellano VM, Fiaño C, Vargas J et al. Mesenchymal hamartoma of the chest wall. Cytopathology 1997; 8: 215-217
• 8 Chu L, Seed M, Howse E et al. Mesenchymal hamartoma: prenatal diagnosis by MRI. Pediatr Radiol 2011; 41: 781-784
• 9 Cohen MC, Drut R, Garcia C et al. Mesenchymal hamartoma of the chest wall: a cooperative study with review of the literature. Pediatr Pathol 1992; 12: 525-534
• 10 Crocoli A, Fruhwirth R, De Vito R et al. Microwave thermoablation treatment of chest wall chondroid hamartoma in a child. J Vasc Interv Radiol 2011; 22: 1051-1052
• 11 Donahoo JS, Miller JA, Lal B et al. Chest wall hamartoma in an adult: an unusual chest wall tumor. Thorac Cardiovasc Surg 1996; 44: 110-111
• 12 Dounies R, Chwals WJ, Lally KP et al. Hamartomas of the chest wall in infants. Ann Thorac Surg 1994; 57: 868-875
• 13 Groom KR, Murphey MD, Howard LM et al. Mesenchymal hamartoma of the chest wall: radiologic manifestations with emphasis on cross-sectional imaging and histopathologic comparison. Radiology 2002; 222: 205-211
• 14 Haase R, Merkel N, Milzsch M et al. Mesenchymal chest wall hamartoma – surgery is preferred. Arch Perinat Med 2007; 13: 56-61
• 15 Hopkins SM, Freitas EL. Bilateral Osteochondroma of the ribs in an infant: an unusual case of cyanosis. J Thorac Cardiovasc Surg 1965; 49: 247-249
• 16 Jung AL, Johnson DG, Condon VR et al. Congenital chest wall mesenchymal hamartoma. J Perinatol 1994; 14: 487-491
• 17 Kerrey B, Reed J. A neonate with respiratory distress and a chest wall deformity. Pediatr Emerg Care 2007; 23: 565-569
• 18 Lisle DA, Ault DJ, Earwaker JW. Mesenchymal hamartoma of the chest wall in infants: report of three cases and literature review. Australas Radiol 2003; 47: 78-82
• 19 McLeod RA, Dahlin DC. Hamartoma (mesenchymoma) of the chest wall in infancy. Radiology 1979; 131: 657-661
• 20 Nash A, Stout AP. Malignant mesenchymomas in children. Cancer 1961; 14: 524-533
• 21 Nicholson SA, Hill DA, Foster KW et al. Fine-needle aspiration cytology of mesenchymal hamartoma of the chest wall. Diagn Cytopathol 2000; 22: 33-38
• 22 Oakley RH, Carty H, Cudmore RE. Multiple benign mesenchymomata of the chest wall. Pediatr Radiol 1985; 15: 58-60
• 23 Odaka A, Takahashi S, Tanimizu T et al. Chest wall mesenchymal hamartoma associated with a massive fetal pleural effusion: a case report. J Pediatr Surg 2005; 40: e5-e7
• 24 Odell JM, Benjamin DR. Mesenchymal hamartoma of chest wall in infancy: natural history of two cases. Pediatr Pathol 1986; 5: 135-146
• 25 Rose NC, Coleman BG, Wallace D et al. Prenatal diagnosis of a chest wall hamartoma and sternal cleft. Ultrasound Obstet Gynecol 1996; 7: 453-455
• 26 Shimotake T, Fumino S, Aoi S et al. Respiratory insufficiency in a newborn with mesenchymal hamartoma of the chest wall occupying the thoracic cavity. J Pediatr Surg 2005; 40: e13-e16
• 27 Sodhi KS, Aiyappan SK, Menon P et al. Unilateral multifocal mesenchymal hamartoma of the chest wall: a case report and review of literature. J Pediatr Surg 2009; 44: 464-467
• 28 Soyer T, Karnak I, Ciftci AO et al. The results of surgical treatment of chest wall tumors in childhood. Pediatr Surg Int 2006; 22: 135-139
• 29 Szadowska A, Sitkiewicz A, Jarosik N. Mesenchymal hamartoma of the chest wall in infancy – a case report of this rare entity with cytologic findings. Cytopathology 1996; 7: 211-217
• 30 Tsuji Y, Maeda K, Tazuke Y et al. Mesenchymal hamartoma of the bilateral chest wall in neonates. Pediatr Surg Int 2012; 28: 939-942
• 31 van den Berg H, van Rijn RR, Merks JH. Management of tumors of the chest wall in childhood: a review. J Pediatr Hematol Oncol 2008; 30: 214-221