Pediatric Pulmonology 12:260-262 (1992)
Bronchial Adenoid Cystic Carcinoma With Saccular Bronchiectasis as a Cause of Recurrent Pneumonia in Children Vladimir Ahel,
MD,
Ivan Zubovic,
INTRODUCTION
The causes of recurrent or chronic pneumonia in childhood are numerous and include congenital anomalies of the tracheobronchial tree, immunological disorders of secretory or serum immunoglobulins, the usual lower respiratory tract infections, and (very rarely) newly formed masses like benign or malignant tumors. Newly formed masses, depending on their dimensions, cause disturbances of ventilation, atelectasis, or emphysema and they are facilitating factors for recurrent pneumonias, which after some time can present as saccular bronchiectases. In these situations it is important to carry out bronchologic examination to make the correct diagnosis. This is especially true in the presence of a bronchial submucosal gland tumor, which, has a tendency toward malignant alteration in a high percentage of cases.'-' We present the case of a 13-year-old girl with recurrent pneumonias. Bronchologic examination verified the existence of a bronchial submucosal gland tumor (adenoid cystic carcinoma-cylindroma) with saccular bronchiectases in the left lower lobe. We found no report describing this entity in the accessible pediatric literature. CASE REPORT A 13-year-old girl was admitted to the Pediatric Clinic
for bronchologic examination. During the last 4 to 5 years she had recurrent pneumonias on the left side, usually twice to three times a year. On admission the patient was normally developed for age and feeling very well. Moist wheezes were heard over the left basal part of the lungs with normal vesicular sound over the other lung areas. Somatic and neurological status was normal. Routine laboratory tests gave normal results. Radiological examination of the heart and lungs showed no significant change except increased vascular markings in the left hilum. Bronchoscopic examination of the larynx, trachea, and carina showed normal conditions. The right main stem bronchus and lobar and segmental bronchi on the same 0 1992 Wiley-Liss, Inc.
MD,
and Vojko Rozmanic, MD
side were normal. The left main bronchus and left upper lobar bronchus were normal. The left lower lobe bronchus was narrowed to a third of its lumen by a roundish tumorous pile of light rosy color. This rather compact formation was situated with its wide basis against the wall of the bronchus. Touching it produced minimal bleeding. It was necessary to perform a biopsy and extraction. Basally, the left segmental bronchial orifices were visibly filled with an abundant, purulent substance. Bronchography verified saccular bronchiectases in the left lower lobe (Fig. 1). The histopathological report indicated adenoid cystic carcinoma (cylindroma) (Figs. 2, 3). Based on the diagnostic reports the patient underwent left lower lobectomy. The postoperative course and a 5-year follow-up was normal. DISCUSSION
Primary tumors of the tracheobronchial tree and pulmonary parenchyma are rare and their occurrence in childhood is unusual. Such tumors are either benign or malignant, with intrabronchial and parenchymatous localizations. For histological analysis and origin, they can be classified as f01lows:~ Benign Inflammatory pseudotumor Hamartoma Neurogenic tumor Leiomyoma Mucous gland adenoma Myoblastoma From the Pediatric Clinic, Pulmonary Unit, Faculty of Medicine. University of Rijeka, Rijeka, Yugoslavia. Received February 25, 1991: (revision) accepted for publication July 1 1 . 1991. Address correspondence and reprint requests to Dr. V . Ahel, Pediatric Clinic, Faculty of Medicine, U1.Bratstva i jedinstva 61,51000 Rijeka, Yugoslavia.
Bronchial Cystic Carcinoma and Bronchiectasis
Fig. 1. Bronchographic picture. Saccular bronchiectases are visible in the left lower lobe.
Malignant Bronchial “adenoma” Pulmonary blastoma Rhabdornyosarcoma Lymphoma Plasmacytoma Bronchogenic carcinoma Leiomyosarcoma Hemangiopericy toma Teratoma Myxosarcoma. The number of cases described in the literature is small, and their frequency depends upon the type of tumor. In most cases bronchial submucosal gland tumors in childhood are described as primary tumors. Sixty-six such cases have been reported in the pediatric literature.’-’ They were known previously as bronchial adenomas, i.e., of benign nature. However, owing to the localization, which involves the submucosal glands of the trachea and bronchi, the term bronchial submucosal gland tumor has recently been i n t r ~ d u c e d . ~ At’ ~the
261
Fig. 2. Adenoid cystic carcinoma (cylindroma). ~ 3 2 .
present time a definite tendency toward malignant transformation is also a ~ c e p t e d . ~ - ’ ~ ’ ~ * ’ ~ Bronchial submucosal gland tumors usually involve the primary and secondary bronchi, as in our case, and less often the trachea. Lesions of this type, located more peripherally in adults, have not been found in children. They are slow-growing, and regularly cause protrusion of the mass into the lumen of the respiratory t r a ~ t . ~ - ~ This group of benign tumors is histopathologically classified into four different Carcinoid tumors Adenoid cystic carcinomas (cylindroma) Mucoepidermoid carcinomas Pleomorphic carcinomas. More than 90% of bronchial submucosal gland tumors belong to the carcinoid Of 66 patients with benign bronchial submucosal gland tumors, 59 had carcinoid tumors. Seven patients had mucoepidermoid carcinomas with good and benign prognosis. 9 Pleomorphic adenomas, until now, have only been described in adults.
262
Ahel et al.
changes pointing to pneumonia that appear repeatedly in the same area. This was also the case in our patient. Bronchoscopy is the key diagnostic method, making possible simultaneous biopsy for histological analysis. There is also a possibility of extracting the tumor if its size permits. Bronchography enables identification of the morphological condition of the tracheobronchial tree. We used both methods for making the diagnosis in our Surgical treatment, i.e., lobectomy or segmentectomy, is recommended because there is a possibility of recurrence of lesions and of malignant transformation and additional lesions of the lung parenchyma and bronchial tree, as was the case in our patient with saccular bronchiectases in the left lower l ~ b e . ~ * ~ * " * ' * REFERENCES I . Wellons HA Jr, Eggelston P, Golden GT. Allen MS. Bronchial
adenoma in childhood: Two case reports and review of literature. Am J Dis Child. 1976; 130:301-307. 2. Bean DM, Haldenby DA. Endobronchial adenomata in children. 1 Otolaryngol. 1976; 5519-524. 3. Verska JJ, Connolly JE. Bronchial adenoma in children. J Thorac Cardiovasc Surg. 1968; 55:411416. 4. McDougall JC, Gorenstein A. Unni K, O'Conell EJ. Carcinoid and mucoepidermoid carcinoma of bronchus in children. Ann Otolaryngol. 1980; 89:425430. 5 . Mak H, Metz SK, Stokes DC, Moser RL, Wang KP, Turner CS. Recurrent wheezing and massive atelectasis in an adolescent. J Fig. 3. High-power view of tumor mass. ~ 3 5 0 . Pediatr. 1983; 102:955-962. 6. Griese M, Reihardt D, Reifenhause A, Irlich G. Chronisch rezidivierende pneumonien bei ossifizirendem bronchuscarcinoid. Monatsschr Kinderheilkd. 1987; 135:511-513. 7 . Hartman GE, Shochar SJ. Primary pulmonary neoplasm of childhood: A review. Ann Thorac. Surg. 1983; 36:108-119. 8. Siegelman SS, Khouri NF. Scott WW. Pulmonary hamartoma: Adenoid cystic carcinoma (cylindroma) has so far not CT findings. Radiology. 1986; 160:313-317. been described in This form of tumor 9. Condon VR, Philips EW. Bronchial adenoma in children. AJR. shows a more aggressive attitude, tending toward local 1962; 88543-550. relapse with metastases in the regional lymph nodes, 10. Salyer DC, Salyer WR, Eggelston JC. Bronchial carcinoid tumors. Cancer. 1975; 36:1522-1534. which we did not observe in our patient. The clinical symptomatology of bronchial submucosal 11. Wisniewski M, Fayemi AO. Bronchial carcinoid: A malignant tumor. Chest. 1972; 62:760-771. gland tumor depends on the mechanical effect on the 12. Shermeta DW. Chondroma of the bronchus in childhood. A case bronchial tree and bleeding of the tumor surface. Cough report illustrating problems in diagnosis and management. J is the leading symptom in most cases and 50% of patients Pediatr Surg. 1975; 10:545-550. are affected by it. It is often followed or accompanied by 13. Haller JA, Goladay ES, Pickard LR, Tepas JJ, Shorter NA. Shermeta DW. Surgical management of lung bud anomalies: hemoptysis. Two-thirds of the reported patients had Lobar emphysema, bronchogenic cyst, cystic adenomatoid malrecurrent pneumonias, as in our patient. Some patients formation, and intralobar pulmonary sequestration. Ann Thorac felt chest pain and had shortness of breath, owing to Surg. 1978; 28:3341. decreased vital capacity, and displacement caused by the 14. Ahel V, Bosnar B, MohorovitiC D. Recidivni obstruktini bronhitis refrakteran na terapiju uzrokovan benignim polipoidnim mediastinal mass. In case of hoarseness and dysphagia, intrabronhijalnim tumorom u dvogodignjeg djeteta. Jug Pedij. tracheal involvement must be s ~ s p e c t e d . ~ ~ - ' ~ - ' ~ 1982; 25~221-224. Radiological examination can help in the diagnosis. 15. Kumis FD. Conn JH. Endobronchial hamarthoma. J Thorac Surg. Often observed changes relate to emphysema or atelecta1965; 50: 138-1 47. sis in a certain area with displacement of the mediasti- 16. Taylor TL, Miller DR. Leyomioma of the bronchus. J Thorac Cardiovasc Surg. 1969; 57:28&296. num, obstruction of the airways, and inflammatory
Bronchial Adenoid Cystic Carcinoma With Saccular Bronchiectasis as a Cause of Recurrent Pneumonia in Children Vladimir Ahel,
MD,
Ivan Zubovic,
INTRODUCTION
The causes of recurrent or chronic pneumonia in childhood are numerous and include congenital anomalies of the tracheobronchial tree, immunological disorders of secretory or serum immunoglobulins, the usual lower respiratory tract infections, and (very rarely) newly formed masses like benign or malignant tumors. Newly formed masses, depending on their dimensions, cause disturbances of ventilation, atelectasis, or emphysema and they are facilitating factors for recurrent pneumonias, which after some time can present as saccular bronchiectases. In these situations it is important to carry out bronchologic examination to make the correct diagnosis. This is especially true in the presence of a bronchial submucosal gland tumor, which, has a tendency toward malignant alteration in a high percentage of cases.'-' We present the case of a 13-year-old girl with recurrent pneumonias. Bronchologic examination verified the existence of a bronchial submucosal gland tumor (adenoid cystic carcinoma-cylindroma) with saccular bronchiectases in the left lower lobe. We found no report describing this entity in the accessible pediatric literature. CASE REPORT A 13-year-old girl was admitted to the Pediatric Clinic
for bronchologic examination. During the last 4 to 5 years she had recurrent pneumonias on the left side, usually twice to three times a year. On admission the patient was normally developed for age and feeling very well. Moist wheezes were heard over the left basal part of the lungs with normal vesicular sound over the other lung areas. Somatic and neurological status was normal. Routine laboratory tests gave normal results. Radiological examination of the heart and lungs showed no significant change except increased vascular markings in the left hilum. Bronchoscopic examination of the larynx, trachea, and carina showed normal conditions. The right main stem bronchus and lobar and segmental bronchi on the same 0 1992 Wiley-Liss, Inc.
MD,
and Vojko Rozmanic, MD
side were normal. The left main bronchus and left upper lobar bronchus were normal. The left lower lobe bronchus was narrowed to a third of its lumen by a roundish tumorous pile of light rosy color. This rather compact formation was situated with its wide basis against the wall of the bronchus. Touching it produced minimal bleeding. It was necessary to perform a biopsy and extraction. Basally, the left segmental bronchial orifices were visibly filled with an abundant, purulent substance. Bronchography verified saccular bronchiectases in the left lower lobe (Fig. 1). The histopathological report indicated adenoid cystic carcinoma (cylindroma) (Figs. 2, 3). Based on the diagnostic reports the patient underwent left lower lobectomy. The postoperative course and a 5-year follow-up was normal. DISCUSSION
Primary tumors of the tracheobronchial tree and pulmonary parenchyma are rare and their occurrence in childhood is unusual. Such tumors are either benign or malignant, with intrabronchial and parenchymatous localizations. For histological analysis and origin, they can be classified as f01lows:~ Benign Inflammatory pseudotumor Hamartoma Neurogenic tumor Leiomyoma Mucous gland adenoma Myoblastoma From the Pediatric Clinic, Pulmonary Unit, Faculty of Medicine. University of Rijeka, Rijeka, Yugoslavia. Received February 25, 1991: (revision) accepted for publication July 1 1 . 1991. Address correspondence and reprint requests to Dr. V . Ahel, Pediatric Clinic, Faculty of Medicine, U1.Bratstva i jedinstva 61,51000 Rijeka, Yugoslavia.
Bronchial Cystic Carcinoma and Bronchiectasis
Fig. 1. Bronchographic picture. Saccular bronchiectases are visible in the left lower lobe.
Malignant Bronchial “adenoma” Pulmonary blastoma Rhabdornyosarcoma Lymphoma Plasmacytoma Bronchogenic carcinoma Leiomyosarcoma Hemangiopericy toma Teratoma Myxosarcoma. The number of cases described in the literature is small, and their frequency depends upon the type of tumor. In most cases bronchial submucosal gland tumors in childhood are described as primary tumors. Sixty-six such cases have been reported in the pediatric literature.’-’ They were known previously as bronchial adenomas, i.e., of benign nature. However, owing to the localization, which involves the submucosal glands of the trachea and bronchi, the term bronchial submucosal gland tumor has recently been i n t r ~ d u c e d . ~ At’ ~the
261
Fig. 2. Adenoid cystic carcinoma (cylindroma). ~ 3 2 .
present time a definite tendency toward malignant transformation is also a ~ c e p t e d . ~ - ’ ~ ’ ~ * ’ ~ Bronchial submucosal gland tumors usually involve the primary and secondary bronchi, as in our case, and less often the trachea. Lesions of this type, located more peripherally in adults, have not been found in children. They are slow-growing, and regularly cause protrusion of the mass into the lumen of the respiratory t r a ~ t . ~ - ~ This group of benign tumors is histopathologically classified into four different Carcinoid tumors Adenoid cystic carcinomas (cylindroma) Mucoepidermoid carcinomas Pleomorphic carcinomas. More than 90% of bronchial submucosal gland tumors belong to the carcinoid Of 66 patients with benign bronchial submucosal gland tumors, 59 had carcinoid tumors. Seven patients had mucoepidermoid carcinomas with good and benign prognosis. 9 Pleomorphic adenomas, until now, have only been described in adults.
262
Ahel et al.
changes pointing to pneumonia that appear repeatedly in the same area. This was also the case in our patient. Bronchoscopy is the key diagnostic method, making possible simultaneous biopsy for histological analysis. There is also a possibility of extracting the tumor if its size permits. Bronchography enables identification of the morphological condition of the tracheobronchial tree. We used both methods for making the diagnosis in our Surgical treatment, i.e., lobectomy or segmentectomy, is recommended because there is a possibility of recurrence of lesions and of malignant transformation and additional lesions of the lung parenchyma and bronchial tree, as was the case in our patient with saccular bronchiectases in the left lower l ~ b e . ~ * ~ * " * ' * REFERENCES I . Wellons HA Jr, Eggelston P, Golden GT. Allen MS. Bronchial
adenoma in childhood: Two case reports and review of literature. Am J Dis Child. 1976; 130:301-307. 2. Bean DM, Haldenby DA. Endobronchial adenomata in children. 1 Otolaryngol. 1976; 5519-524. 3. Verska JJ, Connolly JE. Bronchial adenoma in children. J Thorac Cardiovasc Surg. 1968; 55:411416. 4. McDougall JC, Gorenstein A. Unni K, O'Conell EJ. Carcinoid and mucoepidermoid carcinoma of bronchus in children. Ann Otolaryngol. 1980; 89:425430. 5 . Mak H, Metz SK, Stokes DC, Moser RL, Wang KP, Turner CS. Recurrent wheezing and massive atelectasis in an adolescent. J Fig. 3. High-power view of tumor mass. ~ 3 5 0 . Pediatr. 1983; 102:955-962. 6. Griese M, Reihardt D, Reifenhause A, Irlich G. Chronisch rezidivierende pneumonien bei ossifizirendem bronchuscarcinoid. Monatsschr Kinderheilkd. 1987; 135:511-513. 7 . Hartman GE, Shochar SJ. Primary pulmonary neoplasm of childhood: A review. Ann Thorac. Surg. 1983; 36:108-119. 8. Siegelman SS, Khouri NF. Scott WW. Pulmonary hamartoma: Adenoid cystic carcinoma (cylindroma) has so far not CT findings. Radiology. 1986; 160:313-317. been described in This form of tumor 9. Condon VR, Philips EW. Bronchial adenoma in children. AJR. shows a more aggressive attitude, tending toward local 1962; 88543-550. relapse with metastases in the regional lymph nodes, 10. Salyer DC, Salyer WR, Eggelston JC. Bronchial carcinoid tumors. Cancer. 1975; 36:1522-1534. which we did not observe in our patient. The clinical symptomatology of bronchial submucosal 11. Wisniewski M, Fayemi AO. Bronchial carcinoid: A malignant tumor. Chest. 1972; 62:760-771. gland tumor depends on the mechanical effect on the 12. Shermeta DW. Chondroma of the bronchus in childhood. A case bronchial tree and bleeding of the tumor surface. Cough report illustrating problems in diagnosis and management. J is the leading symptom in most cases and 50% of patients Pediatr Surg. 1975; 10:545-550. are affected by it. It is often followed or accompanied by 13. Haller JA, Goladay ES, Pickard LR, Tepas JJ, Shorter NA. Shermeta DW. Surgical management of lung bud anomalies: hemoptysis. Two-thirds of the reported patients had Lobar emphysema, bronchogenic cyst, cystic adenomatoid malrecurrent pneumonias, as in our patient. Some patients formation, and intralobar pulmonary sequestration. Ann Thorac felt chest pain and had shortness of breath, owing to Surg. 1978; 28:3341. decreased vital capacity, and displacement caused by the 14. Ahel V, Bosnar B, MohorovitiC D. Recidivni obstruktini bronhitis refrakteran na terapiju uzrokovan benignim polipoidnim mediastinal mass. In case of hoarseness and dysphagia, intrabronhijalnim tumorom u dvogodignjeg djeteta. Jug Pedij. tracheal involvement must be s ~ s p e c t e d . ~ ~ - ' ~ - ' ~ 1982; 25~221-224. Radiological examination can help in the diagnosis. 15. Kumis FD. Conn JH. Endobronchial hamarthoma. J Thorac Surg. Often observed changes relate to emphysema or atelecta1965; 50: 138-1 47. sis in a certain area with displacement of the mediasti- 16. Taylor TL, Miller DR. Leyomioma of the bronchus. J Thorac Cardiovasc Surg. 1969; 57:28&296. num, obstruction of the airways, and inflammatory
