Journal of Surgical Oncology 46:270-272 ( 1991)
Endobronchial Carcinoid and Mucoepidermoid Carcinoma in Children
Endobronchial neoplastic disease is rarely encountered in patients under 20 years of age. The great majority of these lesions are carcinoids or mucoepidermoid carcinoma. Symptoms are secondary to bronchial irritation and manifest as recurrent pneumonitis, hetnoptysis, persistent cough, reactive airway disease, and chest pain. Early bronchoscopy reliably enables identification and may prevent harmful sequelae resulting from delay in diagnosis. Retrospective data from four cases collected from the Tumor Registry in the Southern California Kaiser Pernianente Medical Group is presented and a review of the literature is discussed. KEYWORDS:bronchial neoplasms, bronchial obstruction, childhood tumor
INTRODUCTION
antibiotics and bronchodilators for presumed asthma. Workup for a possible immunodeficiency and tuberculin Endobronchial tumors are rare in patients under 20 skin test were negative. Chest X-ray showed partial years of ape. In Hartman's review in 1983 only 230 total atelectasis of the left lower lobe. Fiberoptic bronchosprimary pulmonary neoplasms were reported in the copy revealed a 90% stenosing mass of the left lower lobe literature 1 I ] . Of those only 80 were of endobfonchial bronchus. Bronchial washings and biopsy were nondiagorigin. Out of 73 malignant lesions, 49 were carcinoids nostic. and 12 were mucoepidermoid carcinomas. Since that Surgical exploration of the left chest revealed a 4 cm publication 37 carcinoids and 6 mucoepidermoid carcitumor at the bifurcation of the upper and lower lobe nomas have been reported [ 2- 171. bronchus. Left pneunionectomy and mediastinal node Combined with adenoid cystic carcinoma (cyclindissection were performed. Pathology reported a typical droma) these malignant neoplasms have been grouped carcinoid tumor with one out of eight hilar nodes ( N I ) together as bronchial adenonias. Although slow growing, positive for metastatic carcinoid. the term adenonia is misleading due to the inherent Eighteen months after resection the patient is doing malignant nature of these tumors. Therefore, the more well without evidence of recurrent disease. accurate pathological diagnosis should be used when describing and caring for these patients. CASE 2 Symptoms of endobronchial neoplasms in children are An 18 year-old white male presented with a I year very similar to those in their adult counterparts. Howhistory of recurrent dry cough, chest pain, fever, and ever, given the frequency of upper respiratory illnesses episodes of pneumonia. He also reported an episode of and childhood asthma compared to the rarity of these henioptysis 1 month prior to presentation. Physical exam lesions, diagnosis is commonly delayed. revealed wheezes over the right chest. Chest X-ray This report discusses four cases collected from the showed a mass density obliterating the right cardiac Tumor Registry of the Southern California Permanente shadow with segmental atelectasis of the right middle Medical group since 1957.
CASE I A 14 year-old Caucasian female was refered to the
surgical service tor evaluation after a 2 year history of recurrent left lower lobe pneumonia, persistent cough, and wheezing. She had received several courses of
(C 1991 Wiley-Liss, Inc.
Accepted for puhlication August 18. 1990. Addrcss rcprint requests to Dr. I). liauae, Kaiser Permanente Mtttiic;d Center, Depanmcnt of Surgery. 1526 North Edgemont Street, Los Angeles. CA 90027. Dr. Janieh C. Harvey is now at the Department of Surgery, Beth Israel Medical Center, New Ynrk, NY.
Carcinoid and Bronchial Epidermuid Cancer
lobe. There was no hilar adenopathy. Bronchoscopy discovered a fleshy mass occluding the right middle lobe bronchus. Exploration of the right chest revealed total collapse of the right middle lobe with palpable hilar adenopathy. Right middle lobectomy and mediastinal node dissection were performed. Histologic findings were typical carcinoid tumor with no hilar nodes positive for metastasis. Nineteen years later the patient is doing well without cvidence of recurrent disease.
CASE 3 A 13 year-old black male presented with a severalmonth history of wheezing, cough, and chest pain. Tuberculin skin test was negative. On physical examination he had inspiratory wheezes over the right base. Chest X-ray showed a 2.5 cm neoplasm in the medial basal segment of the right lower lobe. Bronchial washings grew out gram-positive diplococci. Bronchograms revealed a right lower lobe lesion with no bronchiectasis. At operation a right lower lobectomy was performed with pathology reporting a 2 cm typical bronchial carcinoid with no evidence of nodal metastasis. Seventeen years after resection he is free from evidence of recurrent disease.
CASE 4 An 1 1 year-old white female with a several-month history of recurrent upper repiratory infections and bronchitis was referred to the surgical service for evaluation. Tuberculin skin test and fungal serologies were negative. Physical examination revealed expiratory wheezes and occasional rhonchi over the left upper lobe. Chest X-ray showed a 4 cm well-circumscribed lesion of the left upper lobe with segmental atelectasis. Exploration of the left chest disclosed a bronchial lesion of the left upper lobe with extention to the perihilar region. Pathologic diagnosis was mucoepidermoid carcinoma with no evidence of nodal matastasis. Twenty-one years later she is in good health with no evidence of recurrent disease.
DISCUSSION Pediatric endobronchial tumors are rare with fewer than 150 cases appearing in the literature. Greater than 90% of these lesions are malignant [ I ] . Benign lesions include hamartomas, leiomyomas, myoblastomas, and mucus gland adenomas. Eighty percent of the malignant neoplasms are carcinoid tumors or mucoepidermoid carcinoma. Although adult patients may be asymptomatic in 20 to 60% of cases [7,8,18,19], children nearly always present with complaints related to airway obstruction. Table 1 lists the most common symptoms with recurrent unifocal pneumonitis being most characteristic. The differential
271
TABLE I. Symptoms of Endobronchial Neoplasms Recurrent unifocal pneumonitis Hemoptysis Wheezing Chest pain Pleural effusion Persistent cough Dyspnea
diagnosis includes foreign bodies, tuberculosis, aspergillosis, bronchiectasis, and cystic fibrosis. Additionally some patients may be mistakenly treated for childhood asthma. Given the frequency of this and other respiratory complaints during childhood, patients may undergo lengthy evaluations for obscure causes of their symptoms prior to definitive diagnosis. Chest radiography is nearly always abnormal with the actual tumor being appreciated in 30% [6,8]. Other signs include lobar atelectasis, infiltrates, and hilar adenopathy. Bronchoscopy is the mainstay of diagnosis and should be pursued in any child with recurrent pneumonitis, hemoptysis, or localized wheezes on auscultation. Early bronchoscopic evaluation will prevent potentially delaterious delay in diagnosis which has been reported from 2 months to 6 years after symptoms developed. Bronchial carcinoids make up approximately 4% of lung neoplasms and only 4% of these occur before the age of 20. They belong to the amine precursor uptake and decarboxylase (APUD) group of tumors and are derived from Kulschitsky cells of the respiratory epithelium. Carcinoid syndrome which manifests as cutaneous flushing spells, hypotension, intestinal hypermotility, bronchial constriction, and development of endocardia1 lesions is rare in childhood bronchial carcinoids. The only reported case developed in a patient with advanced bronchial carcinoid and massive liver metastisis [2]. Bronchial carcinoids may synthesize other active compounds and three cases of hypercortisolism have appeared in the literature [ 11,121, Histologically carcinoid tumors have been subdivided into “typical” and “atypical” variants as described in reports from the Mayo Clinic. Atypical carcinoids were found to be larger, to metastasize more frequently, and to have a much poorer prognosis [ 181. To our knowledge this classification has not been applied to the childhood lesion and therefore, the prognostic significance is unknown. Our practice is to not biopsy endobronchial lesions when encountered in children. Although recent reports suggest that this procedure has a minimal risk of massive hemorrhage, we have not found that confirmed pathologic diagnosis changes our therapeutic approach. Regardless of the specific histology we believe that com-
272
Hause and Harvey
pletc excision is required for symptomatic relief and the best chance for long-term cure. Surgical resection is the appropriate treatment for bronchial carcinoid. Early attempts at endobronchial excision, polypectomy, or enucleation have resulted in recurrence rates of 60 to 7% [8,18]. Therefore formal conservative pulmonary resection with removal of all involved tissue is necessary. In selected patients bronchoplastic procedures have been successful [ 141. Longterm survival of 90% can be expected if all carcinoid tumor is removed. Mediastinal node dissection should be performed in all patients as it gives prognostic information and niay be therapeutic. In adults negative prognostic factors include tumor size greater than 3 cm, atypical histology, and presence of nodal metastasis [ 181. Bronchial carcinoids metastasize
2. 'To prevent a potentially harmful delay in diagnosis, flexible bronchoscopy should be pursued early in the diagnostic workup of children with recurrent pneumonitis, localized wheezes or hemoptysis. 3 . Conservative surgical resection with niediastinal node dkection is the mainstay of treatment and will result in excellent long-term survival in the ma.jority of cases,
REFERENCES 1. Hnrtinan GE, Shochat SJ: Primary pulmonary neoplasms of childhood: A review. Ann Thorac Surg 36:108-119. 1983. 2. Lack EE. Harris GBC, Erlakis AJ, Vawter GP: Primary bronchial tumors in childhood. A clinicomthologic study of six cases. Cancer 51:492497.1083. 3 Torres AM. Rvcknian FC: Childhood tracheohronchial mucoeDidenioid carcinoma: A ca\e report and review of the literature.' J j
,
c
21:13&144, 1987. biopsied or aspirated with a needle. 16. Helin I . ledgard U. Dejmek A , Lindgren S: Mucoepidermoid ~ ~ ~ ~ ) ~carcinoma ~ i d of~ the~ bronchus ~ ~ ) during i d tuniour of the bronchus. Int J Pcdiatr Otorhinol 7:289-295, 1984. childhood has a ]OW malignant potential and conservative 17. Leiberlnan A , Rar-Liv J . Zirkirl HJ: idow grade n1ucoepidermoid tumour of the bronchus in chldhood. Eur J Pediatr 145:130b 132, ~ulmonarvresection incoruoratine all diseased tissue is
CONCLUSIONS 1 . Endobronchial tumors are rare during childhood. The majority of rhesc lcsions are inalignant and are carcinoids or mucoepidermoid carcinomas. c
19, Todd TK, Coopcr JD.Wcissherg I). Delarue NC, Pearson R i :
Bronchial carcinoid tumors. Twenty year's experience. J Thoriic Cardiovasc Surg 79532-536. 1980.
Endobronchial Carcinoid and Mucoepidermoid Carcinoma in Children
Endobronchial neoplastic disease is rarely encountered in patients under 20 years of age. The great majority of these lesions are carcinoids or mucoepidermoid carcinoma. Symptoms are secondary to bronchial irritation and manifest as recurrent pneumonitis, hetnoptysis, persistent cough, reactive airway disease, and chest pain. Early bronchoscopy reliably enables identification and may prevent harmful sequelae resulting from delay in diagnosis. Retrospective data from four cases collected from the Tumor Registry in the Southern California Kaiser Pernianente Medical Group is presented and a review of the literature is discussed. KEYWORDS:bronchial neoplasms, bronchial obstruction, childhood tumor
INTRODUCTION
antibiotics and bronchodilators for presumed asthma. Workup for a possible immunodeficiency and tuberculin Endobronchial tumors are rare in patients under 20 skin test were negative. Chest X-ray showed partial years of ape. In Hartman's review in 1983 only 230 total atelectasis of the left lower lobe. Fiberoptic bronchosprimary pulmonary neoplasms were reported in the copy revealed a 90% stenosing mass of the left lower lobe literature 1 I ] . Of those only 80 were of endobfonchial bronchus. Bronchial washings and biopsy were nondiagorigin. Out of 73 malignant lesions, 49 were carcinoids nostic. and 12 were mucoepidermoid carcinomas. Since that Surgical exploration of the left chest revealed a 4 cm publication 37 carcinoids and 6 mucoepidermoid carcitumor at the bifurcation of the upper and lower lobe nomas have been reported [ 2- 171. bronchus. Left pneunionectomy and mediastinal node Combined with adenoid cystic carcinoma (cyclindissection were performed. Pathology reported a typical droma) these malignant neoplasms have been grouped carcinoid tumor with one out of eight hilar nodes ( N I ) together as bronchial adenonias. Although slow growing, positive for metastatic carcinoid. the term adenonia is misleading due to the inherent Eighteen months after resection the patient is doing malignant nature of these tumors. Therefore, the more well without evidence of recurrent disease. accurate pathological diagnosis should be used when describing and caring for these patients. CASE 2 Symptoms of endobronchial neoplasms in children are An 18 year-old white male presented with a I year very similar to those in their adult counterparts. Howhistory of recurrent dry cough, chest pain, fever, and ever, given the frequency of upper respiratory illnesses episodes of pneumonia. He also reported an episode of and childhood asthma compared to the rarity of these henioptysis 1 month prior to presentation. Physical exam lesions, diagnosis is commonly delayed. revealed wheezes over the right chest. Chest X-ray This report discusses four cases collected from the showed a mass density obliterating the right cardiac Tumor Registry of the Southern California Permanente shadow with segmental atelectasis of the right middle Medical group since 1957.
CASE I A 14 year-old Caucasian female was refered to the
surgical service tor evaluation after a 2 year history of recurrent left lower lobe pneumonia, persistent cough, and wheezing. She had received several courses of
(C 1991 Wiley-Liss, Inc.
Accepted for puhlication August 18. 1990. Addrcss rcprint requests to Dr. I). liauae, Kaiser Permanente Mtttiic;d Center, Depanmcnt of Surgery. 1526 North Edgemont Street, Los Angeles. CA 90027. Dr. Janieh C. Harvey is now at the Department of Surgery, Beth Israel Medical Center, New Ynrk, NY.
Carcinoid and Bronchial Epidermuid Cancer
lobe. There was no hilar adenopathy. Bronchoscopy discovered a fleshy mass occluding the right middle lobe bronchus. Exploration of the right chest revealed total collapse of the right middle lobe with palpable hilar adenopathy. Right middle lobectomy and mediastinal node dissection were performed. Histologic findings were typical carcinoid tumor with no hilar nodes positive for metastasis. Nineteen years later the patient is doing well without cvidence of recurrent disease.
CASE 3 A 13 year-old black male presented with a severalmonth history of wheezing, cough, and chest pain. Tuberculin skin test was negative. On physical examination he had inspiratory wheezes over the right base. Chest X-ray showed a 2.5 cm neoplasm in the medial basal segment of the right lower lobe. Bronchial washings grew out gram-positive diplococci. Bronchograms revealed a right lower lobe lesion with no bronchiectasis. At operation a right lower lobectomy was performed with pathology reporting a 2 cm typical bronchial carcinoid with no evidence of nodal metastasis. Seventeen years after resection he is free from evidence of recurrent disease.
CASE 4 An 1 1 year-old white female with a several-month history of recurrent upper repiratory infections and bronchitis was referred to the surgical service for evaluation. Tuberculin skin test and fungal serologies were negative. Physical examination revealed expiratory wheezes and occasional rhonchi over the left upper lobe. Chest X-ray showed a 4 cm well-circumscribed lesion of the left upper lobe with segmental atelectasis. Exploration of the left chest disclosed a bronchial lesion of the left upper lobe with extention to the perihilar region. Pathologic diagnosis was mucoepidermoid carcinoma with no evidence of nodal matastasis. Twenty-one years later she is in good health with no evidence of recurrent disease.
DISCUSSION Pediatric endobronchial tumors are rare with fewer than 150 cases appearing in the literature. Greater than 90% of these lesions are malignant [ I ] . Benign lesions include hamartomas, leiomyomas, myoblastomas, and mucus gland adenomas. Eighty percent of the malignant neoplasms are carcinoid tumors or mucoepidermoid carcinoma. Although adult patients may be asymptomatic in 20 to 60% of cases [7,8,18,19], children nearly always present with complaints related to airway obstruction. Table 1 lists the most common symptoms with recurrent unifocal pneumonitis being most characteristic. The differential
271
TABLE I. Symptoms of Endobronchial Neoplasms Recurrent unifocal pneumonitis Hemoptysis Wheezing Chest pain Pleural effusion Persistent cough Dyspnea
diagnosis includes foreign bodies, tuberculosis, aspergillosis, bronchiectasis, and cystic fibrosis. Additionally some patients may be mistakenly treated for childhood asthma. Given the frequency of this and other respiratory complaints during childhood, patients may undergo lengthy evaluations for obscure causes of their symptoms prior to definitive diagnosis. Chest radiography is nearly always abnormal with the actual tumor being appreciated in 30% [6,8]. Other signs include lobar atelectasis, infiltrates, and hilar adenopathy. Bronchoscopy is the mainstay of diagnosis and should be pursued in any child with recurrent pneumonitis, hemoptysis, or localized wheezes on auscultation. Early bronchoscopic evaluation will prevent potentially delaterious delay in diagnosis which has been reported from 2 months to 6 years after symptoms developed. Bronchial carcinoids make up approximately 4% of lung neoplasms and only 4% of these occur before the age of 20. They belong to the amine precursor uptake and decarboxylase (APUD) group of tumors and are derived from Kulschitsky cells of the respiratory epithelium. Carcinoid syndrome which manifests as cutaneous flushing spells, hypotension, intestinal hypermotility, bronchial constriction, and development of endocardia1 lesions is rare in childhood bronchial carcinoids. The only reported case developed in a patient with advanced bronchial carcinoid and massive liver metastisis [2]. Bronchial carcinoids may synthesize other active compounds and three cases of hypercortisolism have appeared in the literature [ 11,121, Histologically carcinoid tumors have been subdivided into “typical” and “atypical” variants as described in reports from the Mayo Clinic. Atypical carcinoids were found to be larger, to metastasize more frequently, and to have a much poorer prognosis [ 181. To our knowledge this classification has not been applied to the childhood lesion and therefore, the prognostic significance is unknown. Our practice is to not biopsy endobronchial lesions when encountered in children. Although recent reports suggest that this procedure has a minimal risk of massive hemorrhage, we have not found that confirmed pathologic diagnosis changes our therapeutic approach. Regardless of the specific histology we believe that com-
272
Hause and Harvey
pletc excision is required for symptomatic relief and the best chance for long-term cure. Surgical resection is the appropriate treatment for bronchial carcinoid. Early attempts at endobronchial excision, polypectomy, or enucleation have resulted in recurrence rates of 60 to 7% [8,18]. Therefore formal conservative pulmonary resection with removal of all involved tissue is necessary. In selected patients bronchoplastic procedures have been successful [ 141. Longterm survival of 90% can be expected if all carcinoid tumor is removed. Mediastinal node dissection should be performed in all patients as it gives prognostic information and niay be therapeutic. In adults negative prognostic factors include tumor size greater than 3 cm, atypical histology, and presence of nodal metastasis [ 181. Bronchial carcinoids metastasize
2. 'To prevent a potentially harmful delay in diagnosis, flexible bronchoscopy should be pursued early in the diagnostic workup of children with recurrent pneumonitis, localized wheezes or hemoptysis. 3 . Conservative surgical resection with niediastinal node dkection is the mainstay of treatment and will result in excellent long-term survival in the ma.jority of cases,
REFERENCES 1. Hnrtinan GE, Shochat SJ: Primary pulmonary neoplasms of childhood: A review. Ann Thorac Surg 36:108-119. 1983. 2. Lack EE. Harris GBC, Erlakis AJ, Vawter GP: Primary bronchial tumors in childhood. A clinicomthologic study of six cases. Cancer 51:492497.1083. 3 Torres AM. Rvcknian FC: Childhood tracheohronchial mucoeDidenioid carcinoma: A ca\e report and review of the literature.' J j
,
c
21:13&144, 1987. biopsied or aspirated with a needle. 16. Helin I . ledgard U. Dejmek A , Lindgren S: Mucoepidermoid ~ ~ ~ ~ ) ~carcinoma ~ i d of~ the~ bronchus ~ ~ ) during i d tuniour of the bronchus. Int J Pcdiatr Otorhinol 7:289-295, 1984. childhood has a ]OW malignant potential and conservative 17. Leiberlnan A , Rar-Liv J . Zirkirl HJ: idow grade n1ucoepidermoid tumour of the bronchus in chldhood. Eur J Pediatr 145:130b 132, ~ulmonarvresection incoruoratine all diseased tissue is
CONCLUSIONS 1 . Endobronchial tumors are rare during childhood. The majority of rhesc lcsions are inalignant and are carcinoids or mucoepidermoid carcinomas. c
19, Todd TK, Coopcr JD.Wcissherg I). Delarue NC, Pearson R i :
Bronchial carcinoid tumors. Twenty year's experience. J Thoriic Cardiovasc Surg 79532-536. 1980.
