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Endobronchial chondroid hamartoma in an infant
Journal of Pediatric Surgery (2009) 44, E21–E23
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Endobronchial chondroid hamartoma in an infant Vishesh Jain a , Prabhud Goel a , Dinesh Kumar b , Aashu Seith b , Chitra Sarkar c , Sushil Kabra d , Sandeep Agarwala a,⁎ a
Department of Pediatric Surgery, All India Institute of Medical Sciences, New Delhi 110029, India Department of Radiodiagnosis, All India Institute of Medical Sciences, New Delhi 110029, India c Department of Pathology, All India Institute of Medical Sciences, New Delhi 110029, India d Department of Pediatrics, All India Institute of Medical Sciences, New Delhi 110029, India b
Received 26 March 2009; revised 9 June 2009; accepted 10 June 2009
Key words: Endobronchial hamartoma; Chondroid hamartoma; Infant
Abstract Endobronchial tumors in infants are uncommon. The clinical and radiologic findings and management of a rare case of endobronchial chondroid hamartoma in an infant is presented along with a review of the literature. © 2009 Elsevier Inc. All rights reserved.
Hamartomas are the most common benign tumor of the lung in adults; however, they are occasionally reported in children as well. Most hamartomas are intraparenchymal, whereas endobronchial hamartomas are less common. The occurrence of an endobronchial hamartoma in an infant is very rare. We describe the clinical presentation and management of this rare tumor in an infant.
1. Case report A three-and-a-half-month-old boy presented with a history of repeated respiratory distress since birth. The antenatal period was uneventful. The child was a full-term infant delivered by normal abdominal delivery. The patient developed respiratory distress soon after birth and required mechanical ventilatory support for 6 days. Thereafter, the
⁎ Corresponding author. Fax: +91 11 26588641. E-mail address: [email protected] (S. Agarwala). 0022-3468/$ – see front matter © 2009 Elsevier Inc. All rights reserved. doi:10.1016/j.jpedsurg.2009.06.011
child did well and was discharged on the 14th day of life. Soon after discharge, he developed tachypnea with moderate subcostal retractions and was brought to our hospital. On physical examination, the trachea was central in position, but air entry was decreased on the right side. The chest radiograph revealed a hyperinflated right lung with no mediastinal shift (Fig. 1A). Contrast-enhanced chest computed tomographic scan showed a solid mass lesion within the right intermediate bronchus with a hyperinflated right upper lobe and collapsed right lower lobe (Fig. 1B and C). The left bronchus was normal. Flexible bronchoscopy showed a mass lesion in right intermediate bronchus. Cytologic examination of the bronchial aspirate was inconclusive. Right thoracotomy was performed and revealed collapsed middle and lower lobes with a hyperinflated upper lobe. Bronchotomy of the right bronchus intermedius revealed the presence of a whitish firm intraluminal mass with a wide base, just distal to the right upper lobe opening. Right middle and lower lobectomy was performed. The histopathologic examination of the lesion was reported as a chondroid hamartoma (Fig. 1D). The patient had an uneventful recovery and was discharged on
E22
V. Jain et al.
Fig. 1 A, Chest radiograph showing hyperinflated lung with no mediastinal shift. B and C, Contrast-enhanced chest computed tomographic scan showing a solid mass lesion within the right intermediate bronchus with hyperinflated right upper lobe and collapsed right lower lobe. D, Photomicrographs of histologic section from the endobronchial mass showing disorganized lobules of cartilage, smooth muscle, and dilated glands of bronchial type (H&E, ×100).
the eighth postoperative day. On follow-up examination, the child remains asymptomatic.
2. Discussion Primary pulmonary neoplasms are unusual in the pediatric age group, and nearly one fourth of these are benign [1]. Inflammatory pseudotumor is the most common benign tumor in children followed by hamartoma. Endobronchial neoplasms make up a very small fraction of these benign neoplasm with bronchial adenoma being the most common endobronchial tumor in children. Other benign endobronchial neoplasms reported are hamartomas, hemangioma, papillomas, leiomyomas, mucous gland tumors, and inflammatory pseudotumor [2]. The term hamartoma was coined in 1904 by Albrecht [3] to describe certain tumorlike malformations resulting from a presumed error in development of tissues normally present in the involved organ. In 1934, Goldsworthy [4] applied this term to benign lung tumors composed of a combination of adipose tissue and cartilage. Pulmonary hamartomas can be parenchymal or endobronchial in location. Endobronchial hamartomas are extremely rare in children and have a low frequency in all the reported series in adults. In the largest review of pulmonary hamartomas in
adults (n = 215), only 1.4% of hamartomas had an endobronchial location; the remainder are located within the parenchyma [5]. Other adult series estimate the incidence of endobronchial hamartomas between 10% and 20% of all lung hamartomas [6-8]. As compared to parenchymal hamartomas, most of the endobronchial hamartomas are symptomatic. Our patient presented with lower respiratory tract infection with atelectasis of the involved lobe. Atelectasis or hyperinflation results from complete or partial occlusion of the airway and may be because of a foreign body, mucous plug, mucosal web, cysts, or tumors. Because of the rarity of endobronchial tumors, it is infrequently considered in the differential diagnosis. Treatment options are diverse. If the diagnosis of hamartoma is confirmed and the tumor is accessible, endoscopy and excision of tumor using ND:YAG laser, cryosurgery, electrosurgery snare, electrocoagulation, or forceps resection is preferred. If endoscopic resection is not possible, then bronchotomy and excision of tumor, bronchoplasty or lobectomy, and pneumonectomy have been done. A thorough review of literature revealed this to be the youngest reported case of endobronchial hamartoma. Only one previous case report of endobronchial hamartoma in an infant 8 months of age who underwent endoscopic resection was found [9]. Endobronchial hamartoma, although rare, should be considered in the differential diagnosis of an endobronchial mass in children.
Endobronchial chandroid hamartoma in an infant
References [1] Hancock BJ, Di Lorenzo M, Youssef S, et al. Childhood primary pulmonary neoplasms. J Pediatr Surg 1993;28:1133-6. [2] Sivanandan S, Lodha R, Agarwala S, Sharma M, et al. Inflammatory myofibroblastic tumor of the trachea. Pediatr Pulmonol 2007;42: 847-50. [3] Albrecht E. Ueber hamartome. Verh Dtsch Ges Pathol 1904;7:153-7. [4] Goldsworthy NE. Chondroma of lung (hamartoma chondromatosum polmonis), with report of case. J Pathol Bacteriol 1934;39:291-8.
E23 [5] Gjevre JA, Myers JL, Prakash UB. Pulmonary hamartomas. Mayo Clin Proc 1996;71:14-20. [6] Sibala JL. Endobronchial hamartomas. Chest 1972;62:631-4. [7] Tomashefski JF. Benign endobronchial mesenchymal tumors: their relationship to parenchymal pulmonary hamartomas. Am J Pathol 1982;6:531-40. [8] Van den Bosch JM, Wagenaar SS, Corrin B, et al. Mesenchymoma of the lung (so called hamartoma): a review of 154 parenchymal and endobronchial cases. Thorax 1987;42:790-3. [9] Abdulhamid I, Rabah R. Endobronchial chondromatous hamartoma in an infant. Pediatr Pulmonol 2003;35:67-9.
www.elsevier.com/locate/jpedsurg
Endobronchial chondroid hamartoma in an infant Vishesh Jain a , Prabhud Goel a , Dinesh Kumar b , Aashu Seith b , Chitra Sarkar c , Sushil Kabra d , Sandeep Agarwala a,⁎ a
Department of Pediatric Surgery, All India Institute of Medical Sciences, New Delhi 110029, India Department of Radiodiagnosis, All India Institute of Medical Sciences, New Delhi 110029, India c Department of Pathology, All India Institute of Medical Sciences, New Delhi 110029, India d Department of Pediatrics, All India Institute of Medical Sciences, New Delhi 110029, India b
Received 26 March 2009; revised 9 June 2009; accepted 10 June 2009
Key words: Endobronchial hamartoma; Chondroid hamartoma; Infant
Abstract Endobronchial tumors in infants are uncommon. The clinical and radiologic findings and management of a rare case of endobronchial chondroid hamartoma in an infant is presented along with a review of the literature. © 2009 Elsevier Inc. All rights reserved.
Hamartomas are the most common benign tumor of the lung in adults; however, they are occasionally reported in children as well. Most hamartomas are intraparenchymal, whereas endobronchial hamartomas are less common. The occurrence of an endobronchial hamartoma in an infant is very rare. We describe the clinical presentation and management of this rare tumor in an infant.
1. Case report A three-and-a-half-month-old boy presented with a history of repeated respiratory distress since birth. The antenatal period was uneventful. The child was a full-term infant delivered by normal abdominal delivery. The patient developed respiratory distress soon after birth and required mechanical ventilatory support for 6 days. Thereafter, the
⁎ Corresponding author. Fax: +91 11 26588641. E-mail address: [email protected] (S. Agarwala). 0022-3468/$ – see front matter © 2009 Elsevier Inc. All rights reserved. doi:10.1016/j.jpedsurg.2009.06.011
child did well and was discharged on the 14th day of life. Soon after discharge, he developed tachypnea with moderate subcostal retractions and was brought to our hospital. On physical examination, the trachea was central in position, but air entry was decreased on the right side. The chest radiograph revealed a hyperinflated right lung with no mediastinal shift (Fig. 1A). Contrast-enhanced chest computed tomographic scan showed a solid mass lesion within the right intermediate bronchus with a hyperinflated right upper lobe and collapsed right lower lobe (Fig. 1B and C). The left bronchus was normal. Flexible bronchoscopy showed a mass lesion in right intermediate bronchus. Cytologic examination of the bronchial aspirate was inconclusive. Right thoracotomy was performed and revealed collapsed middle and lower lobes with a hyperinflated upper lobe. Bronchotomy of the right bronchus intermedius revealed the presence of a whitish firm intraluminal mass with a wide base, just distal to the right upper lobe opening. Right middle and lower lobectomy was performed. The histopathologic examination of the lesion was reported as a chondroid hamartoma (Fig. 1D). The patient had an uneventful recovery and was discharged on
E22
V. Jain et al.
Fig. 1 A, Chest radiograph showing hyperinflated lung with no mediastinal shift. B and C, Contrast-enhanced chest computed tomographic scan showing a solid mass lesion within the right intermediate bronchus with hyperinflated right upper lobe and collapsed right lower lobe. D, Photomicrographs of histologic section from the endobronchial mass showing disorganized lobules of cartilage, smooth muscle, and dilated glands of bronchial type (H&E, ×100).
the eighth postoperative day. On follow-up examination, the child remains asymptomatic.
2. Discussion Primary pulmonary neoplasms are unusual in the pediatric age group, and nearly one fourth of these are benign [1]. Inflammatory pseudotumor is the most common benign tumor in children followed by hamartoma. Endobronchial neoplasms make up a very small fraction of these benign neoplasm with bronchial adenoma being the most common endobronchial tumor in children. Other benign endobronchial neoplasms reported are hamartomas, hemangioma, papillomas, leiomyomas, mucous gland tumors, and inflammatory pseudotumor [2]. The term hamartoma was coined in 1904 by Albrecht [3] to describe certain tumorlike malformations resulting from a presumed error in development of tissues normally present in the involved organ. In 1934, Goldsworthy [4] applied this term to benign lung tumors composed of a combination of adipose tissue and cartilage. Pulmonary hamartomas can be parenchymal or endobronchial in location. Endobronchial hamartomas are extremely rare in children and have a low frequency in all the reported series in adults. In the largest review of pulmonary hamartomas in
adults (n = 215), only 1.4% of hamartomas had an endobronchial location; the remainder are located within the parenchyma [5]. Other adult series estimate the incidence of endobronchial hamartomas between 10% and 20% of all lung hamartomas [6-8]. As compared to parenchymal hamartomas, most of the endobronchial hamartomas are symptomatic. Our patient presented with lower respiratory tract infection with atelectasis of the involved lobe. Atelectasis or hyperinflation results from complete or partial occlusion of the airway and may be because of a foreign body, mucous plug, mucosal web, cysts, or tumors. Because of the rarity of endobronchial tumors, it is infrequently considered in the differential diagnosis. Treatment options are diverse. If the diagnosis of hamartoma is confirmed and the tumor is accessible, endoscopy and excision of tumor using ND:YAG laser, cryosurgery, electrosurgery snare, electrocoagulation, or forceps resection is preferred. If endoscopic resection is not possible, then bronchotomy and excision of tumor, bronchoplasty or lobectomy, and pneumonectomy have been done. A thorough review of literature revealed this to be the youngest reported case of endobronchial hamartoma. Only one previous case report of endobronchial hamartoma in an infant 8 months of age who underwent endoscopic resection was found [9]. Endobronchial hamartoma, although rare, should be considered in the differential diagnosis of an endobronchial mass in children.
Endobronchial chandroid hamartoma in an infant
References [1] Hancock BJ, Di Lorenzo M, Youssef S, et al. Childhood primary pulmonary neoplasms. J Pediatr Surg 1993;28:1133-6. [2] Sivanandan S, Lodha R, Agarwala S, Sharma M, et al. Inflammatory myofibroblastic tumor of the trachea. Pediatr Pulmonol 2007;42: 847-50. [3] Albrecht E. Ueber hamartome. Verh Dtsch Ges Pathol 1904;7:153-7. [4] Goldsworthy NE. Chondroma of lung (hamartoma chondromatosum polmonis), with report of case. J Pathol Bacteriol 1934;39:291-8.
E23 [5] Gjevre JA, Myers JL, Prakash UB. Pulmonary hamartomas. Mayo Clin Proc 1996;71:14-20. [6] Sibala JL. Endobronchial hamartomas. Chest 1972;62:631-4. [7] Tomashefski JF. Benign endobronchial mesenchymal tumors: their relationship to parenchymal pulmonary hamartomas. Am J Pathol 1982;6:531-40. [8] Van den Bosch JM, Wagenaar SS, Corrin B, et al. Mesenchymoma of the lung (so called hamartoma): a review of 154 parenchymal and endobronchial cases. Thorax 1987;42:790-3. [9] Abdulhamid I, Rabah R. Endobronchial chondromatous hamartoma in an infant. Pediatr Pulmonol 2003;35:67-9.