- Email: [email protected]
Endometriosis of the lung
Respiratory Medicine (1989) 83, 255-258
Endometriosis of the lung S. DI PALO, G. MAR1, R. CASTOLDI, C. STAUDACHER, G. TACCAGNI* AND V. DI CARLO
Cattedra di Patologia Chirurgica e * Cattedra di Anatomia e lstologia Patologica dell" Universith, Studi, lstituto Scientifico S. Raffaele, V. Olgettina 60, 20132 Milano, Italy
Introduction
The intrathoracic location of ectopic endometrial tissue is rare. In 1912 Hart (1) described, for the first time, multiple pulmonary lesions with endometrial glands and stroma which were interpreted as hamartomatous. Schwartz (2) in 1938 reported a case of recurrent haemoptysis occurring during the menstrual cycle that was associated with endometriosis of inguinal lymph nodes. The first well proven case of pleural endometriosis was reported by Buengeler (3) in 1939. Foster et al. (4) in 1981 reviewed the literature reporting 65 cases of pulmonary endometriosis: parenchymal in 11 cases and pleural in 54 cases. A further four cases have been recently reported (5-7). Intrathoracic endometriosis is sometimes associated, particularly in pleural sites, to spread of pelvic or peritoneal_ endometriosis. Different hypotheses have been given for explaining the presence of endometrial tissue in such ectopic sites. There is sometimes an association with previous surgical procedures on the uterus. We report a case of pulmonary parenchymal endometriosis in which symptoms (dyspnea and blood-streaked sputum) were not related to menses. Case Report A 42-year-old woman, smoker, gravida-two, paratwo, was admitted to our department in June 1983 for episodes of exertional dyspnea and blood-streaked sputum during the three months prior to admission. Physical examination was negative, menses were regular and dysmenorrhea was not experienced. A pulmonary tomogram (Plate l) showed in the left paramediastinum, posterior to and compressing the main bronchus, a well defined oval density which was considered to be a benign neoplasm. Broncoscopy confirmed narrowing of the origin of the left main bronchus. CAT scanning (Plate 2) showed that the Received October 1986 and in revised form 28 December 1988 0954-6111/89/030255 + 04 $03.00/0
density was solid and not dissociable from the pulmonary artery. Digital Angiography showed it to be parenchymatous with its own vascularization. Fineneedle X-ray guided biopsy was not diagnostic and sputum cytology was repeatedly negative. During the hospital stay she had two episodes of blood-streaked sputum not chronologically related to menses. The radiologic findings of a pulmonary density and the persistence of symptoms indicated an exploratory thoracotomy due to suspicion of a pulmonary neoplasm. During surgery a mass of 3-4 cm was found by the left inferior lobar bronchus which was of soft consistency, encapsulated, partially intraparenchymal and communicating with lumen of the bronchus. The mass was removed and the initial histology was compatible with hamartoma. Histologic sections showed glandular structures of various size and shape, lined by a single layer of cuboidal epithelium and cystic zones containing eosinophilic, amorphous granular material (Plate 3a). The glandular structures were scattered throughout a stroma rich in spindle cells and arterioles, which were sometimes spiral and were surrounded by radial layers of pre-decidual cells. In some areas the stroma showed decidual changes. Some hemosiderin-laden histiocytes were observed. The tumour appeared to be encapsulated, limited by a thick hyaline layer which at intervals was interrupted (Plate 3b). Some little nests of endometrial stromal cells were scattered in the lung parenchyma. They were not encapsulated and were independent from the main lesion. Occasional alveoli were lined with glandular endometrial cells. The histological diagnosis was pulmonary endometriosis. Treatment with danazol was started and continued for four months. Six months after surgery the patient had a sudden episode of dyspnoea and chest X-ray showed a complete collapse of the left lung (Plate 4). A bronchoscopic examination revealed a tumour completely occluding the left main bronchus. This was subsequently endoscopically treated with © 1989 Baillirre Tindall
256
S. di Palo et al.
Plate 3a Irregular glandula lumina lined by cuboidal cells with monomorphic nuclei and vacuolated cytoplasm containing cellular debis and amorphous material (EmatossilinEosine x 137•5).
A poorly defined capsule surrounds the nodule: the lung parenchima shows a thickening of the alveolar septa. (Ematoxilin-Eosine × 31-3). Plate 3b
Plate 1 Pulmonary tomogram film showing an ovalar density (N) in the left paramediastinal area, posteriorly to the left mean bronchus (B).
/
Plate 2 A rounded solid density ( > ) was shown in the left bronchus just posteriorly to the superior lobar bronchus and to pulmonary artery.
Plate 4 Chest X-ray film six months after the operation showing a complete atelectasia of the left lung.
laser, obtaining a complete reexpansion of the lung. The patient was then treated with buserelin, an LHRH analogue. After the treatment there were two more episodes of ateleetasis which were successfully treated
endoscopically with laser• The persisting symptoms and the frequent relapses led to a hystero-salpingooophorectomy being performed in August 1985. Since then the patient has been symptom free.
Endometriosis o f the lung
Discussion
Thoracic endometriosis may present as episodes of haemoptysis concurrent with menses (catamenial haemoptysis) (4), as recurrent episodes of pneumothorax (8) or as an asymptomatic pulmonary density. Chest pain, exertional dyspnea or pleural effusion may be associated with the primary symptom. In reviewing the published cases, Foster et al. (4) described the different clinical characteristics of the two types of thoracic involvement. The pleural localization is found in a younger age group (mean age 33-6 years), is associated more frequently with pelvic endometriosis, is usually seen in the right lung and is more often associated with a pneumothorax. The parenchymal localization appears later (mean age 39 years) and is associated with pelvic endometriosis in only 10% of cases, catamenial haemoptysis in 82% of cases and catamenial pain and dyspnea is found in 18% of cases. In our case report the age of the patient (42 yearsold), the blood-streaked sputum unrelated to menses and the lack of pelvic symptoms or pathology, are consistent with the clinical characteristics of parenchymal localization, as described in literature. According to Lattes (9), Mobbs (10) and Williams (I 1) different pathogenic mechanisms can be proposed for thoracic endometriosis. One hypothesis postulates the haematogenous metastases of viable endometrial tissue (12) subsequent to surgical procedures on the uterus or to previous caesarean sections. According to this hypothesis, the inferior lobes of the lung would be more frequently involved because of the higher blood flow. The hypothesis of hematogenous pathogenesis is more likely to explain parenchymal lesions whereas the hypothesis of coelomic metaplasia and of tubaric regurgitation is more frequently involved in the pathogenesis of pleural localization. In the Ivanoff hypothesis of coelomic metaplasia (4) there is a metaplasia of pleurai epithelium which embryologically originates, as does the endometrial epithelium, from the primitive coelome. In the Sampson hypothesis the transdiaphragmatic passage of endometrial stroma or glandular elements, due to hydrostatic abdominal pressure follows the tubaric regurgitation o f such elements during menses. The finding ofcribriform fenestrations of the diaphragm, prevailing on the fight side, gives further support to this theory. There may also be lymphatic diffusion (4). In the case reported, the pulmonary localization could probably be explained by a hematogenous mechanism, especially considering the previous surgical procedures on the uterus of the patient. Relapses could be more than satisfactorily explained by the
257
presence of endometrial tissue not detectable by the low resolution ofthe diagnostic methods. The lack o f a clear chronological association between the episodes of blood-streaked sputum and menses, which made the diagnosis more difficult, could be related to the lack of direct communication of endometrial ectopic tissue with the bronchial lumen or from an imperfect or delayed response by ectopic endometrial tissue to hormonal variations. A symptom free course has also been reported elsewhere 00,13,14). Elliot et al. (15) reported the effectiveness of danazol in treating pulmonary endometriosis by blocking secretion of gonadotropins and confirmed the results o f previous experiences (16,17). Buserelin, an L H R H analogous, has been recently experienced in the tratment of this pathology (18). The possibility of an effective medical therapy presumes a proper preoperative diagnosis. Lacking clinical suspicious features as catamenial haemoptysis, only a histological examination could lead to a successful diagnosis. Medical therapy, reported usually as effective, has been in this case unsuccessful.
References
1. Hart C. Histologisch benigne Metastasen vom bau eines Adenymioms 22 jahre nach Extirpation eines tumors der Genitalien. FrankfZ Pothol. 1912; 10: 78. 2. Schwarz OH. Endometriosis of the lung. Am J Obstet Gynecol 1938; 36: 887-890. 3. Buengeler W, Fleury Silveira D. Consideracones sobre a patogenia das endometrioses. Arq Cirurg Clin (1939); 3: 169-187. 4. Foster DC, Stern JL, Buscema J, Rock JA, WoodruffJD, Pleural and parenchymal pulmonary endometriosis. Obstet Gynecol 1981; 58: 552-556. 5. Yamamoto H, et ol. A case of pulmonary endometriosis with recurrent catamenial hemoptysis. Nippon Naika Gokkai Zasshi 1980; 69: 1650-1654. 6. Hibbard LT, et al. Thoracic endometriosis: a review and report of two cases. Am J Obstet Gynecol 1981; 140: 227232. 7. lbuki S, et al. A case of pulmonary endometriosis. Nippon Naiko Gakkai Zasshi 1981; 70: 1408-1413. 8. Litlington GA, Mitchell SP, Wood GA. Catamenial pneumothorax. JAMA 1972; 219: 1328-1332. 9. Lattes R, Shepard F, Tovell H, Wylie R. A clinical and pathologic study of endometriosis of the lung. Surg Gynecol Obstet 1956, 103: 552-558. 10. Mobbs GA, Pfranner DW. Endometriosis of the lung. Lancet 1963; i: 472-474. i I. Williams JF, Williams JB, Harper JW. Thoracic endometriosis. Am J Obstet Gynecol 1962; 84: 1512. 12. Rodman MH, Jones CW. Catamenial hemoptysis due to bronchial endometfiosis. N Engl J Med 1962; 266: 805808. 13. Sttlrzenegger H. Lungendometriose under dem Bild des Rundschattens. Schwiez. Z. Tuberk 1960; 17: 259-264. 14. Jelihovsky T, Grant AF. Endometriosis of the lung. A
258
S. di Palo et al.
case report and brief review of the literature. Thorax 1968; 23: 434-437. 15. Elliot DL, Barker AF, Dixon LM. Catamenial hemoptysis. New methods of diagnosis and therapy. Chest 1985; 87: 687-688. 16. Ronnberg L, Ylostalo P. Treatment of pulmonary endometriosis with danazol. Acta Obstet Gynecol Scand 1981; 60: 67-68.
17. Rosenberg SM, Riddick DH. Successful treatment of catamenial hemoptysis with danazol. Obstet Gynecol 1981; 57: 130-131. 18. Lemay A, Maheux R, Faure N, Jean C, Fazekas ATA. Reversible hypogonadism induced by a luteinizing hormone-releasing hormone (LH-RH) agonist (Buserelin) as a new therapeutic approach for endometriosis. Fert Steril 1984; 41: 863-871.
Endometriosis of the lung S. DI PALO, G. MAR1, R. CASTOLDI, C. STAUDACHER, G. TACCAGNI* AND V. DI CARLO
Cattedra di Patologia Chirurgica e * Cattedra di Anatomia e lstologia Patologica dell" Universith, Studi, lstituto Scientifico S. Raffaele, V. Olgettina 60, 20132 Milano, Italy
Introduction
The intrathoracic location of ectopic endometrial tissue is rare. In 1912 Hart (1) described, for the first time, multiple pulmonary lesions with endometrial glands and stroma which were interpreted as hamartomatous. Schwartz (2) in 1938 reported a case of recurrent haemoptysis occurring during the menstrual cycle that was associated with endometriosis of inguinal lymph nodes. The first well proven case of pleural endometriosis was reported by Buengeler (3) in 1939. Foster et al. (4) in 1981 reviewed the literature reporting 65 cases of pulmonary endometriosis: parenchymal in 11 cases and pleural in 54 cases. A further four cases have been recently reported (5-7). Intrathoracic endometriosis is sometimes associated, particularly in pleural sites, to spread of pelvic or peritoneal_ endometriosis. Different hypotheses have been given for explaining the presence of endometrial tissue in such ectopic sites. There is sometimes an association with previous surgical procedures on the uterus. We report a case of pulmonary parenchymal endometriosis in which symptoms (dyspnea and blood-streaked sputum) were not related to menses. Case Report A 42-year-old woman, smoker, gravida-two, paratwo, was admitted to our department in June 1983 for episodes of exertional dyspnea and blood-streaked sputum during the three months prior to admission. Physical examination was negative, menses were regular and dysmenorrhea was not experienced. A pulmonary tomogram (Plate l) showed in the left paramediastinum, posterior to and compressing the main bronchus, a well defined oval density which was considered to be a benign neoplasm. Broncoscopy confirmed narrowing of the origin of the left main bronchus. CAT scanning (Plate 2) showed that the Received October 1986 and in revised form 28 December 1988 0954-6111/89/030255 + 04 $03.00/0
density was solid and not dissociable from the pulmonary artery. Digital Angiography showed it to be parenchymatous with its own vascularization. Fineneedle X-ray guided biopsy was not diagnostic and sputum cytology was repeatedly negative. During the hospital stay she had two episodes of blood-streaked sputum not chronologically related to menses. The radiologic findings of a pulmonary density and the persistence of symptoms indicated an exploratory thoracotomy due to suspicion of a pulmonary neoplasm. During surgery a mass of 3-4 cm was found by the left inferior lobar bronchus which was of soft consistency, encapsulated, partially intraparenchymal and communicating with lumen of the bronchus. The mass was removed and the initial histology was compatible with hamartoma. Histologic sections showed glandular structures of various size and shape, lined by a single layer of cuboidal epithelium and cystic zones containing eosinophilic, amorphous granular material (Plate 3a). The glandular structures were scattered throughout a stroma rich in spindle cells and arterioles, which were sometimes spiral and were surrounded by radial layers of pre-decidual cells. In some areas the stroma showed decidual changes. Some hemosiderin-laden histiocytes were observed. The tumour appeared to be encapsulated, limited by a thick hyaline layer which at intervals was interrupted (Plate 3b). Some little nests of endometrial stromal cells were scattered in the lung parenchyma. They were not encapsulated and were independent from the main lesion. Occasional alveoli were lined with glandular endometrial cells. The histological diagnosis was pulmonary endometriosis. Treatment with danazol was started and continued for four months. Six months after surgery the patient had a sudden episode of dyspnoea and chest X-ray showed a complete collapse of the left lung (Plate 4). A bronchoscopic examination revealed a tumour completely occluding the left main bronchus. This was subsequently endoscopically treated with © 1989 Baillirre Tindall
256
S. di Palo et al.
Plate 3a Irregular glandula lumina lined by cuboidal cells with monomorphic nuclei and vacuolated cytoplasm containing cellular debis and amorphous material (EmatossilinEosine x 137•5).
A poorly defined capsule surrounds the nodule: the lung parenchima shows a thickening of the alveolar septa. (Ematoxilin-Eosine × 31-3). Plate 3b
Plate 1 Pulmonary tomogram film showing an ovalar density (N) in the left paramediastinal area, posteriorly to the left mean bronchus (B).
/
Plate 2 A rounded solid density ( > ) was shown in the left bronchus just posteriorly to the superior lobar bronchus and to pulmonary artery.
Plate 4 Chest X-ray film six months after the operation showing a complete atelectasia of the left lung.
laser, obtaining a complete reexpansion of the lung. The patient was then treated with buserelin, an LHRH analogue. After the treatment there were two more episodes of ateleetasis which were successfully treated
endoscopically with laser• The persisting symptoms and the frequent relapses led to a hystero-salpingooophorectomy being performed in August 1985. Since then the patient has been symptom free.
Endometriosis o f the lung
Discussion
Thoracic endometriosis may present as episodes of haemoptysis concurrent with menses (catamenial haemoptysis) (4), as recurrent episodes of pneumothorax (8) or as an asymptomatic pulmonary density. Chest pain, exertional dyspnea or pleural effusion may be associated with the primary symptom. In reviewing the published cases, Foster et al. (4) described the different clinical characteristics of the two types of thoracic involvement. The pleural localization is found in a younger age group (mean age 33-6 years), is associated more frequently with pelvic endometriosis, is usually seen in the right lung and is more often associated with a pneumothorax. The parenchymal localization appears later (mean age 39 years) and is associated with pelvic endometriosis in only 10% of cases, catamenial haemoptysis in 82% of cases and catamenial pain and dyspnea is found in 18% of cases. In our case report the age of the patient (42 yearsold), the blood-streaked sputum unrelated to menses and the lack of pelvic symptoms or pathology, are consistent with the clinical characteristics of parenchymal localization, as described in literature. According to Lattes (9), Mobbs (10) and Williams (I 1) different pathogenic mechanisms can be proposed for thoracic endometriosis. One hypothesis postulates the haematogenous metastases of viable endometrial tissue (12) subsequent to surgical procedures on the uterus or to previous caesarean sections. According to this hypothesis, the inferior lobes of the lung would be more frequently involved because of the higher blood flow. The hypothesis of hematogenous pathogenesis is more likely to explain parenchymal lesions whereas the hypothesis of coelomic metaplasia and of tubaric regurgitation is more frequently involved in the pathogenesis of pleural localization. In the Ivanoff hypothesis of coelomic metaplasia (4) there is a metaplasia of pleurai epithelium which embryologically originates, as does the endometrial epithelium, from the primitive coelome. In the Sampson hypothesis the transdiaphragmatic passage of endometrial stroma or glandular elements, due to hydrostatic abdominal pressure follows the tubaric regurgitation o f such elements during menses. The finding ofcribriform fenestrations of the diaphragm, prevailing on the fight side, gives further support to this theory. There may also be lymphatic diffusion (4). In the case reported, the pulmonary localization could probably be explained by a hematogenous mechanism, especially considering the previous surgical procedures on the uterus of the patient. Relapses could be more than satisfactorily explained by the
257
presence of endometrial tissue not detectable by the low resolution ofthe diagnostic methods. The lack o f a clear chronological association between the episodes of blood-streaked sputum and menses, which made the diagnosis more difficult, could be related to the lack of direct communication of endometrial ectopic tissue with the bronchial lumen or from an imperfect or delayed response by ectopic endometrial tissue to hormonal variations. A symptom free course has also been reported elsewhere 00,13,14). Elliot et al. (15) reported the effectiveness of danazol in treating pulmonary endometriosis by blocking secretion of gonadotropins and confirmed the results o f previous experiences (16,17). Buserelin, an L H R H analogous, has been recently experienced in the tratment of this pathology (18). The possibility of an effective medical therapy presumes a proper preoperative diagnosis. Lacking clinical suspicious features as catamenial haemoptysis, only a histological examination could lead to a successful diagnosis. Medical therapy, reported usually as effective, has been in this case unsuccessful.
References
1. Hart C. Histologisch benigne Metastasen vom bau eines Adenymioms 22 jahre nach Extirpation eines tumors der Genitalien. FrankfZ Pothol. 1912; 10: 78. 2. Schwarz OH. Endometriosis of the lung. Am J Obstet Gynecol 1938; 36: 887-890. 3. Buengeler W, Fleury Silveira D. Consideracones sobre a patogenia das endometrioses. Arq Cirurg Clin (1939); 3: 169-187. 4. Foster DC, Stern JL, Buscema J, Rock JA, WoodruffJD, Pleural and parenchymal pulmonary endometriosis. Obstet Gynecol 1981; 58: 552-556. 5. Yamamoto H, et ol. A case of pulmonary endometriosis with recurrent catamenial hemoptysis. Nippon Naika Gokkai Zasshi 1980; 69: 1650-1654. 6. Hibbard LT, et al. Thoracic endometriosis: a review and report of two cases. Am J Obstet Gynecol 1981; 140: 227232. 7. lbuki S, et al. A case of pulmonary endometriosis. Nippon Naiko Gakkai Zasshi 1981; 70: 1408-1413. 8. Litlington GA, Mitchell SP, Wood GA. Catamenial pneumothorax. JAMA 1972; 219: 1328-1332. 9. Lattes R, Shepard F, Tovell H, Wylie R. A clinical and pathologic study of endometriosis of the lung. Surg Gynecol Obstet 1956, 103: 552-558. 10. Mobbs GA, Pfranner DW. Endometriosis of the lung. Lancet 1963; i: 472-474. i I. Williams JF, Williams JB, Harper JW. Thoracic endometriosis. Am J Obstet Gynecol 1962; 84: 1512. 12. Rodman MH, Jones CW. Catamenial hemoptysis due to bronchial endometfiosis. N Engl J Med 1962; 266: 805808. 13. Sttlrzenegger H. Lungendometriose under dem Bild des Rundschattens. Schwiez. Z. Tuberk 1960; 17: 259-264. 14. Jelihovsky T, Grant AF. Endometriosis of the lung. A
258
S. di Palo et al.
case report and brief review of the literature. Thorax 1968; 23: 434-437. 15. Elliot DL, Barker AF, Dixon LM. Catamenial hemoptysis. New methods of diagnosis and therapy. Chest 1985; 87: 687-688. 16. Ronnberg L, Ylostalo P. Treatment of pulmonary endometriosis with danazol. Acta Obstet Gynecol Scand 1981; 60: 67-68.
17. Rosenberg SM, Riddick DH. Successful treatment of catamenial hemoptysis with danazol. Obstet Gynecol 1981; 57: 130-131. 18. Lemay A, Maheux R, Faure N, Jean C, Fazekas ATA. Reversible hypogonadism induced by a luteinizing hormone-releasing hormone (LH-RH) agonist (Buserelin) as a new therapeutic approach for endometriosis. Fert Steril 1984; 41: 863-871.