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Bronchial carcinoid arising in intralobar bronchopulmonary sequestration with vascular supply from the left gastric artery
J
THORAC C ARDIOVASC S URG
90:25-28, 1985
Bronchial carcinoid arising in intralobar bronchopulmonary sequestration with vascular supply from the left gastric artery Case report We report the unique case of a large, nonmetastasizing bronchial carcinoid tumor that arose within an intralobar bronchopulmonary sequestration in a 45-year-old man. The vascular supply to the sequestrated area within the left lower lobe as weD as to the carcinoid tumor originated from atypical branches of the left gastric artery and the thoracic aorta. A left lower lobe lobectomy was performed. Histologically, a typical carcinoid tumor without lymph node metastases was found (1'2 NO MO). Seven years postoperatively, the patient is without signs of recurrence.
F. M. Juettner, M.D., H. H. Pinter, M.D., G. B. Friehs , M.D., and H. Hoefler, M.D., Graz, Austria
Bronchial carcinoids as well as bronchopulmonary sequestrations are rare findings. To our knowledge, no case of bronchial carcinoid arising in a bronchopulmonary sequestration has been described previously.
Case report A 45-year-old nonsmoking man gave a history of multiple episodes of "severe bronchitis" in childhood and adolescence. Recurrent pneumonia of the left lung with intermittent hemoptysis during the past 15 years had developed. Treatment had always been conservat ive (e.g., antibiotics and expectorants). Notwithstanding the recurrrent infections, the patient always had felt well. At the time of admission , decreased resonance in percussion and decreased breath sounds in the left lower lung field were the only pathological physical findings. The posteroanterior plain chest roentgenograms showed total atelectasis of the left lower lobe (Fig. I). Except for an increased erythrocyte sedimentation rate of 23/58, routine laborato ry parameters were within norma l limits. By fiberoptic bronchoscopy a n endobronchial tumor at the lateral wall of the left lower lobe bronchus, 1.5 em distal to the From the Department of Th oracic and Hyperbar ic Surgery of the University Clinics of G raz (Drs. Juettner, Pinter, a nd Friehs) a nd Pathologic-Anatomical Institute of the Un iversity of Graz (Dr. Hoefler), Gra z, Austri a. Received for publicat ion A ug. 27. 1984.
Accepted for publicati on Oct. I J, 1984. Request for reprints: F. M . Ju cttner, M .D., Depa rt ment of T horacic and Hyperbaric Surgery, Un iversity Clinics of Graz, Auenbr uggerplatz, A-8036, Austria.
Fig. I. Plain posteroanterior chest roent genogram. Op acification in the left lower pulmonary field (arrow) well distinguished from the cardiac silhouette and the diaphragm. Left lower lobe atelectasis. lobar carina, was found. Th e tumor had a smooth, grayishyellowish-reddish, slightly lobulated surface and was narrowing the lumen to about 30%. The stenosis could not be passed with the instrument, but massive poststenotic accumulation of purulent mucus was aspirated. A biopsy specimen from the tumor was highly suggestive of bronchial carcinoid. There was no clinical evidence of ectopic endocrine activities. Bone scan and hepatic sonography revealed no signs of metastases.
25
The Journal of Thoracic and Cardiovascular Surgery
2 6 Juettner et al.
Fig. 2. Selective angiogram of the left gastric artery: A large-caliber atypical artery (large arrow) is ascending through the diaphragm. Arborization and tortuosity of the vesselwithin the pulmonary ligament is evident (*). The atypical artery is giving off a small-caliber phrenicoabdominal vessel (small arrow).
Fig. 3. Surgical specimen. The left lower lobe bronchus (arrow) has been opened longitudinally. The almost ballshaped endobronchial portion of the tumor is well distinguishable. Fibrotic lung parenchyma is evident in segment 8 (*). Because of the patient's long history of bronchopulmonary difficulty, however, we performed a catheter angiogram of the aorta to rule out congenital disorders of vascularization. A pulmonary sequestration in the dorsobasal segments of the left
Fig. 4. Injection of specimen with radiopaque medium. The injected atypical arteries form a vascular tuft within the tumor (*). Marked sclerotic changes are visible in the atypical branches (arrow). Venous runoff is through the inferior pulmonary vein (curved arrow). lung was found, supplied by a small-caliber coiled artery from the thoracic aorta and a large-caliber vessel originating from the left gastric artery (Fig. 2). Surgical findings. The lower lobe was of a solid, pseudotumoral consistency and showed massive, tight adherence to the thoracic wall and the diaphragm. Beyond the thickened visceral pleura, numerous atypical coiled arteries with vigorous pulsation were visible. The branches arising from the thoracic aorta extended to the hilus and provided access to the lobe along the bronchus, whereas the abdominal branches passed through the pulmonary ligament. The common basal trunk of the pulmonary artery was hypoplastic. Distal to the origin of the lower lobe bronchus, a tumor, approximately 6 em in diameter, was palpated within the dorsobasal segments. After the abnormal arteries were ligated, left lower lobectomy and meticulous lymphadenectomy were done. At present, 7 years postoperatively, the patient is well without signs of recurrence.
Pathological findings Macroscopic features. The tumor, 6.5 by 5 by 5 em, was located within the sequestrated lung of segments 9 and 10 and was well demarcated. The endobronchial portion of the tumor from which the endoscopic biopsy specimen was obtained was 1.5 by 1 by 1 em. Distal to the stenosis, bronchiectasis with retention of purulent
Volume 90 Number 1 July, 1985
Bronchial carcinoid
27
Fig. 5. Tumor cell with a few dense-cored, round endocrine granules (arrows) and a paranuclear "fibrous body" consisting of intermediate-sized filaments (*). Nucleus (n). mitochondria (m). (Epon glutaraldehyde-fixed, osmium postfixed, Epon-embedded and counterstained with uranylacetate and lead citrate; x 24,OOO.) mucus was found. Lung parenchyma in segment 8 was fibrotic (Fig. 3).
Injection of the atypical arteries with radiopaque medium. The sequestrated basal segments as well as the carcinoid tumor were vascularized by the atypical systemic arteries, whereas the venous drainage was effected by the inferior pulmonary vein (Fig. 4). The hypoplastic pulmonary artery supplied mainly segment 6 and a small area of segment 8.
Histologic findings.
light and electron microscopic features. The growth pattern as seen by light microscopy was predominantly trabecular, partly adenoid. The tumor cells were uni-
form without atypia or mitoses. Invasion of blood or lymphatic vessels by tumor was not observed. A marked fibrosis of the tumor stroma with several foci of calcification was noticed. Seven lymph nodes were without metastases. In lung parenchyma of segments 8, 9, and 10, a chronic, indurative pneumonia had developed. All smalland medium-sized arteries of the sequestration showed marked elastofibrosis and calcification within their walls. By electron microscopy the tumor cells contained round, densely cored vesicles with a diameter ranging from 80 to 110 nm. Most of the tumor cells were rich in
2 8 Juettner et al.
The Journal of Thoracic and Cardiovascular Surgery
mitochondria; in some cells few or no endocrine granules were seen. A striking feature was the occurrence of paranuclear felt-like accumulations of intermediatesized filaments (7 to II nm), which sometimes trapped cell organelles (endocrine granules or mitochondria) (Fig. 5). Discussion Bronchopulmonary sequestration is a rare dysontogenetic disorder, the distinct development of which is still a point of controversy." In the intralobar form a systemic artery supplies blood to an area of lung within one of the lobes, which is covered by the normal pleural surface. Usually venous drainage is into the pulmonary vein, although combined systemic drainage may be present.v' Arterial supply in about 90% of cases is provided by supernumerary branches of the thoracic or abdominal aorta. A branch arising from the celiac artery is a rare finding. 5 Bronchial carcinoid constitutes about 3% of all bronchopulmonary neoplasms." Chronic inflammatory processes have been implicated in the development of bronchial carcinoid. 7 Occurrence of various diseases subsequent to bronchopulmonary sequestration is not uncommon. Especially within the intralobar form, tuberculosis.v'? superinfection with various bacteria that causes lung abscess, II as well as growth of Aspergillus within postinflammatory cavities," have been described. Three reports of bronchial carcinoma in bronchopulmonary sequestration have been described before the current study. In a patient with extralobar sequestration, referred by Hertzog and associates 13 and later by Accard and colleagues," it is not clear whether primary lung cancer or metastatic disease of squamous cell carcinoma were present. In the two other cases reported by Bell-Thompson and colleagues I 5 and Peros and associates," squamous cell bronchogenic carcinoma obviously had developed within intralobar sequestration. To our knowledge, our case of a large, nonmetastasizing bronchial carcinoid arising in bronchopulmonary sequestration is the first to be described. REFERENCES Durnin RE, Lababidi Z, Butler C, Selke A, Flege JB: Bronchopulmonary sequestration. Chest 57:454-459, 1970
2 Holstein P, Helms E: Bronchopulmonary sequestration. J THORAC CARDIOVASC SURG 65:462-466, 1973 3 Sade RM, Clouse MV, Ellis FH Jr: The spectrum of pulmonary sequestration. Ann Thorac Surg 18:644-Ci58, 1974 4 von Windheim K: Pulmonary sequestration. Monatsschr Kinderheilkd 130:233-238, 1982 5 Mielecki T, Garcarek J, Minch K: A case of intralobar sequestration with a rare type of vascularization. Pol Przegl Radiol 47:85-88, 1983 6 Burcharth F, Axelsson CH: Lung carcinoids. Scand J Thorac Cardiovasc Surg 7:72-77, 1973 7 Tragiello P: I carcinoidi della trachea e dei bronchi. Riv Patol Clin Tuberc 42:33-54, 1969 8 Boumghar M, Besson A, Ekoe JM, Saegesser F: Intralobal pulmonary sequestration and tuberculosis superinfection. Schweiz Med Wochenschr 39:1460-1464, 1979 9 Hellstrom PC, Alquist J, Railo J, Niemisto N: Tuberculosis in pulmonary intralobar sequestration. Cardiol Med 16:23-24, 1977 10 Schacter EN, Karpick RJ: Bronchopulmonary sequestration and pulmonary tuberculosis. Chest 62:331-333, 1972 11 Rejou JJ, Albaret A, Bannier M: Pulmonary sequestrations and their pathological associations. Ouest Med 32:1075-1081, 1979 12 Grignet JP, Pagnier F, Faillon JM, Leduc M, Cecile JP, Bauduin JL, Dinnoo V, Demarest P: A case of pulmonary dequestration associated with Aspergillus pathology. Larc Med 10:689-690, 1983 13 Hertzog P, Roujeau J, Marcou J: Cancer epidermoide developpe sur une sequestration. J Franc Med Chir Thorae 17:33-38,1963 14 Accard JL, Toty L, Personne C, Hertzog P: Etude d'une serie de 35 sequestrations avec reference aux formes particulieres. Ann Chir Thorac Cardiovasc 9:501-510, 1970 15 Bell-Thompson J, Missier P, Sommers SC: Lung carcinoma arising in bronchopulmonary sequestration. Cancer 44:334-339, 1979 16 Peros T, Gorecan M, Siobodnjyk Z, Scukanec M: Cancer in a pulmonary sequestrum. Lijec Vjesn 12:694-696, 1981
THORAC C ARDIOVASC S URG
90:25-28, 1985
Bronchial carcinoid arising in intralobar bronchopulmonary sequestration with vascular supply from the left gastric artery Case report We report the unique case of a large, nonmetastasizing bronchial carcinoid tumor that arose within an intralobar bronchopulmonary sequestration in a 45-year-old man. The vascular supply to the sequestrated area within the left lower lobe as weD as to the carcinoid tumor originated from atypical branches of the left gastric artery and the thoracic aorta. A left lower lobe lobectomy was performed. Histologically, a typical carcinoid tumor without lymph node metastases was found (1'2 NO MO). Seven years postoperatively, the patient is without signs of recurrence.
F. M. Juettner, M.D., H. H. Pinter, M.D., G. B. Friehs , M.D., and H. Hoefler, M.D., Graz, Austria
Bronchial carcinoids as well as bronchopulmonary sequestrations are rare findings. To our knowledge, no case of bronchial carcinoid arising in a bronchopulmonary sequestration has been described previously.
Case report A 45-year-old nonsmoking man gave a history of multiple episodes of "severe bronchitis" in childhood and adolescence. Recurrent pneumonia of the left lung with intermittent hemoptysis during the past 15 years had developed. Treatment had always been conservat ive (e.g., antibiotics and expectorants). Notwithstanding the recurrrent infections, the patient always had felt well. At the time of admission , decreased resonance in percussion and decreased breath sounds in the left lower lung field were the only pathological physical findings. The posteroanterior plain chest roentgenograms showed total atelectasis of the left lower lobe (Fig. I). Except for an increased erythrocyte sedimentation rate of 23/58, routine laborato ry parameters were within norma l limits. By fiberoptic bronchoscopy a n endobronchial tumor at the lateral wall of the left lower lobe bronchus, 1.5 em distal to the From the Department of Th oracic and Hyperbar ic Surgery of the University Clinics of G raz (Drs. Juettner, Pinter, a nd Friehs) a nd Pathologic-Anatomical Institute of the Un iversity of Graz (Dr. Hoefler), Gra z, Austri a. Received for publicat ion A ug. 27. 1984.
Accepted for publicati on Oct. I J, 1984. Request for reprints: F. M . Ju cttner, M .D., Depa rt ment of T horacic and Hyperbaric Surgery, Un iversity Clinics of Graz, Auenbr uggerplatz, A-8036, Austria.
Fig. I. Plain posteroanterior chest roent genogram. Op acification in the left lower pulmonary field (arrow) well distinguished from the cardiac silhouette and the diaphragm. Left lower lobe atelectasis. lobar carina, was found. Th e tumor had a smooth, grayishyellowish-reddish, slightly lobulated surface and was narrowing the lumen to about 30%. The stenosis could not be passed with the instrument, but massive poststenotic accumulation of purulent mucus was aspirated. A biopsy specimen from the tumor was highly suggestive of bronchial carcinoid. There was no clinical evidence of ectopic endocrine activities. Bone scan and hepatic sonography revealed no signs of metastases.
25
The Journal of Thoracic and Cardiovascular Surgery
2 6 Juettner et al.
Fig. 2. Selective angiogram of the left gastric artery: A large-caliber atypical artery (large arrow) is ascending through the diaphragm. Arborization and tortuosity of the vesselwithin the pulmonary ligament is evident (*). The atypical artery is giving off a small-caliber phrenicoabdominal vessel (small arrow).
Fig. 3. Surgical specimen. The left lower lobe bronchus (arrow) has been opened longitudinally. The almost ballshaped endobronchial portion of the tumor is well distinguishable. Fibrotic lung parenchyma is evident in segment 8 (*). Because of the patient's long history of bronchopulmonary difficulty, however, we performed a catheter angiogram of the aorta to rule out congenital disorders of vascularization. A pulmonary sequestration in the dorsobasal segments of the left
Fig. 4. Injection of specimen with radiopaque medium. The injected atypical arteries form a vascular tuft within the tumor (*). Marked sclerotic changes are visible in the atypical branches (arrow). Venous runoff is through the inferior pulmonary vein (curved arrow). lung was found, supplied by a small-caliber coiled artery from the thoracic aorta and a large-caliber vessel originating from the left gastric artery (Fig. 2). Surgical findings. The lower lobe was of a solid, pseudotumoral consistency and showed massive, tight adherence to the thoracic wall and the diaphragm. Beyond the thickened visceral pleura, numerous atypical coiled arteries with vigorous pulsation were visible. The branches arising from the thoracic aorta extended to the hilus and provided access to the lobe along the bronchus, whereas the abdominal branches passed through the pulmonary ligament. The common basal trunk of the pulmonary artery was hypoplastic. Distal to the origin of the lower lobe bronchus, a tumor, approximately 6 em in diameter, was palpated within the dorsobasal segments. After the abnormal arteries were ligated, left lower lobectomy and meticulous lymphadenectomy were done. At present, 7 years postoperatively, the patient is well without signs of recurrence.
Pathological findings Macroscopic features. The tumor, 6.5 by 5 by 5 em, was located within the sequestrated lung of segments 9 and 10 and was well demarcated. The endobronchial portion of the tumor from which the endoscopic biopsy specimen was obtained was 1.5 by 1 by 1 em. Distal to the stenosis, bronchiectasis with retention of purulent
Volume 90 Number 1 July, 1985
Bronchial carcinoid
27
Fig. 5. Tumor cell with a few dense-cored, round endocrine granules (arrows) and a paranuclear "fibrous body" consisting of intermediate-sized filaments (*). Nucleus (n). mitochondria (m). (Epon glutaraldehyde-fixed, osmium postfixed, Epon-embedded and counterstained with uranylacetate and lead citrate; x 24,OOO.) mucus was found. Lung parenchyma in segment 8 was fibrotic (Fig. 3).
Injection of the atypical arteries with radiopaque medium. The sequestrated basal segments as well as the carcinoid tumor were vascularized by the atypical systemic arteries, whereas the venous drainage was effected by the inferior pulmonary vein (Fig. 4). The hypoplastic pulmonary artery supplied mainly segment 6 and a small area of segment 8.
Histologic findings.
light and electron microscopic features. The growth pattern as seen by light microscopy was predominantly trabecular, partly adenoid. The tumor cells were uni-
form without atypia or mitoses. Invasion of blood or lymphatic vessels by tumor was not observed. A marked fibrosis of the tumor stroma with several foci of calcification was noticed. Seven lymph nodes were without metastases. In lung parenchyma of segments 8, 9, and 10, a chronic, indurative pneumonia had developed. All smalland medium-sized arteries of the sequestration showed marked elastofibrosis and calcification within their walls. By electron microscopy the tumor cells contained round, densely cored vesicles with a diameter ranging from 80 to 110 nm. Most of the tumor cells were rich in
2 8 Juettner et al.
The Journal of Thoracic and Cardiovascular Surgery
mitochondria; in some cells few or no endocrine granules were seen. A striking feature was the occurrence of paranuclear felt-like accumulations of intermediatesized filaments (7 to II nm), which sometimes trapped cell organelles (endocrine granules or mitochondria) (Fig. 5). Discussion Bronchopulmonary sequestration is a rare dysontogenetic disorder, the distinct development of which is still a point of controversy." In the intralobar form a systemic artery supplies blood to an area of lung within one of the lobes, which is covered by the normal pleural surface. Usually venous drainage is into the pulmonary vein, although combined systemic drainage may be present.v' Arterial supply in about 90% of cases is provided by supernumerary branches of the thoracic or abdominal aorta. A branch arising from the celiac artery is a rare finding. 5 Bronchial carcinoid constitutes about 3% of all bronchopulmonary neoplasms." Chronic inflammatory processes have been implicated in the development of bronchial carcinoid. 7 Occurrence of various diseases subsequent to bronchopulmonary sequestration is not uncommon. Especially within the intralobar form, tuberculosis.v'? superinfection with various bacteria that causes lung abscess, II as well as growth of Aspergillus within postinflammatory cavities," have been described. Three reports of bronchial carcinoma in bronchopulmonary sequestration have been described before the current study. In a patient with extralobar sequestration, referred by Hertzog and associates 13 and later by Accard and colleagues," it is not clear whether primary lung cancer or metastatic disease of squamous cell carcinoma were present. In the two other cases reported by Bell-Thompson and colleagues I 5 and Peros and associates," squamous cell bronchogenic carcinoma obviously had developed within intralobar sequestration. To our knowledge, our case of a large, nonmetastasizing bronchial carcinoid arising in bronchopulmonary sequestration is the first to be described. REFERENCES Durnin RE, Lababidi Z, Butler C, Selke A, Flege JB: Bronchopulmonary sequestration. Chest 57:454-459, 1970
2 Holstein P, Helms E: Bronchopulmonary sequestration. J THORAC CARDIOVASC SURG 65:462-466, 1973 3 Sade RM, Clouse MV, Ellis FH Jr: The spectrum of pulmonary sequestration. Ann Thorac Surg 18:644-Ci58, 1974 4 von Windheim K: Pulmonary sequestration. Monatsschr Kinderheilkd 130:233-238, 1982 5 Mielecki T, Garcarek J, Minch K: A case of intralobar sequestration with a rare type of vascularization. Pol Przegl Radiol 47:85-88, 1983 6 Burcharth F, Axelsson CH: Lung carcinoids. Scand J Thorac Cardiovasc Surg 7:72-77, 1973 7 Tragiello P: I carcinoidi della trachea e dei bronchi. Riv Patol Clin Tuberc 42:33-54, 1969 8 Boumghar M, Besson A, Ekoe JM, Saegesser F: Intralobal pulmonary sequestration and tuberculosis superinfection. Schweiz Med Wochenschr 39:1460-1464, 1979 9 Hellstrom PC, Alquist J, Railo J, Niemisto N: Tuberculosis in pulmonary intralobar sequestration. Cardiol Med 16:23-24, 1977 10 Schacter EN, Karpick RJ: Bronchopulmonary sequestration and pulmonary tuberculosis. Chest 62:331-333, 1972 11 Rejou JJ, Albaret A, Bannier M: Pulmonary sequestrations and their pathological associations. Ouest Med 32:1075-1081, 1979 12 Grignet JP, Pagnier F, Faillon JM, Leduc M, Cecile JP, Bauduin JL, Dinnoo V, Demarest P: A case of pulmonary dequestration associated with Aspergillus pathology. Larc Med 10:689-690, 1983 13 Hertzog P, Roujeau J, Marcou J: Cancer epidermoide developpe sur une sequestration. J Franc Med Chir Thorae 17:33-38,1963 14 Accard JL, Toty L, Personne C, Hertzog P: Etude d'une serie de 35 sequestrations avec reference aux formes particulieres. Ann Chir Thorac Cardiovasc 9:501-510, 1970 15 Bell-Thompson J, Missier P, Sommers SC: Lung carcinoma arising in bronchopulmonary sequestration. Cancer 44:334-339, 1979 16 Peros T, Gorecan M, Siobodnjyk Z, Scukanec M: Cancer in a pulmonary sequestrum. Lijec Vjesn 12:694-696, 1981