Case Reports © 1990 S. Kargcr AG. Basel 0025-7931/90/0576-0395 S 2.75/0
Respiration 1990;57:395-397
Intravascular Bronchoalveolar Tumor M.S. Prats ', C. Cáliz*. F.J. Sancho''. R. Cornudellaa Departments of 1Pneumology and h Pathology, Hospital de la Sta. Creu i St. Pau, Barcelona. Spain
Key Words. Intravascular bronchoalveolar tumor • Bronchoalveolar lavage • Hepatic nodules Abstract. We report the case of a young woman presenting productive cough and arthralgia in the left ankle. Chest radiography revealed multiple bilateral pulmonary nodules and abdominal echography and computer ized axial tomography demonstrated various hepatic nodules. Definitive diagnosis of an intravascular bron choalveolar tumor was reached by an open pulmonary biopsy. Liver involvement was confirmed by laparos copy and biopsy.
Introduction Since the first description of an intravascular bron choalveolar tumor by Dail and Liebow [1], approxi mately 60 further cases have been published. This tu mor appears to be more frequent in women, and the age group ranges between 12 and 71. Most patients appear asymptomatic. Chest radiographs show bilat eral multiple pulmonary nodules. Survival ranges be tween 4 months and 12 years.
Case Report
blood gases were within the reference ranges. Abdominal echogra phy showed a nodule of 28 mm diameter with a peripheric halo in each hepatic lobe. Computerized axial tomography revealed pul monary nodules disseminated in both hemithoraxes and a right paratracheal adenopathy (fig. 1). Bone and thyroid gammagraphy, barium oesophagogastroduodenal study, mammography, bone marrow biopsy and gynecologic examination were normal. A pul monary biopsy was performed by thoracotomy. Macroscopy
A fragment of pulmonary parenchyma of 2.5 x 2 x I cm was studied. The pleural surface was slightly depressed in two small areas of increased consistency. Sectioning at these points showed nodular areas with a mean diameter of 0.4 cm, consisting of clearly differentiated firm whitish tissue.
The lesion consisted of round or oval nodules, clearly delin eated from the neighboring pulmonary parenchyma, but not encapsulated (fig. 2). Peripherally they consisted of cells of large polygonal cytoplasm, occasionally with vacuoles, and only slightly pleomorphic vesicular nuclei. No mitosis was observed. The cells appeared to be covered with an eosinophilic matrix, forming poly poid masses, occupying the alveolar spaces. Centrally, the nodules became progressively more fibrotic and acellular, obliterating the alveolar spaces without destroying their walls, as demonstrated in the reticulin and elastic fibers. Vascular invasion was observed in various areas with tumoral exclusion of the small arteries and veins. There was also bronchiolar invasion with partial destruction of the lining epithelium (fig. 3).
Downloaded by: Temple University 155.247.166.234 - 2/3/2019 7:45:20 AM
Microscopy
A 17-year-old woman was admitted to our hospital in May 1987 for investigation of bilateral pulmonary nodules discovered 4 weeks previously on a chest radiogram taken because she had a productive cough and pain in the left ankle. There was no history of pulmonary tuberculosis, exposure to dusts, recent travel or allergy. She had been smoking 6 cigarettes a day. Menarche was at 12 and her menstrual cycles were 28/3-4 days. On admission, physical ex amination, routine laboratory tests were normal with the exception of a gammaglobulinemia of 19.3 g/1 (normal 8.2-14 g/1). The eye fundis were normal. PPD 5-UT was negative. Bronchoscopy, bron choalveolar lavage (macrophages 90%, lymphocytes 5%, polymorphonuclears 5%) and transbronchial biopsy were normal. Microbi ologie and cytologic studies were negative. Spirometry and arterial
Prats/Câli7./Sancho/Cornudella
396
No acid-alcohol-resistant bacilli or fungi were identified and cul tures were negative. Pathologic diagnosis was intravascular bronchoalveolar tumor. Laparoscopy showed a normal-sized liver with lenticular images and tiny nodular plaques in both lobes. Focal substitution of the liver parenchyma due to tumoral proliferation similar to that of the lung was observed in the biopsy.
Fig. 1. Computerized axial tomography of chest with multiple bilateral nodules.
Fig. 2. Periphery of one of the tumoral nodes. Alveolar spaces are filled with polypoid masses with hyalin centers and cellular pe riphery. HE. x 400.
Fig. 3. Invasion with partial destruction of the wall of a bron chiole. HH. < 100.
The pathology in a patient with pulmonary nod ules and liver nodules is most likely tumoral. From a morphologic point of view the microscopic image of an intravascular bronchoalveolar tumor is suffi ciently specific to eliminate other primary or metas tatic neoplasms with the exception of the rare possi bility of métastasés of epitheloid-hemangioendothelioma [2]. The morphology of this infrequent tumor is similar to that of intravascular bronchoalveolar tu mors and it is a vascular sarcoma with a very similar histogenesis. The symptomatology may vary from isolated ar thralgia, as in the case described by Sherman et al. [3], to right upper-quadrant pain, as reported by Emery et al. [4]. Clinical presentation is usually masked and the evolution sometimes of long duration [5] although it may be acute and aggressive, as an expression of hemopathy [6]. The radiological features described to date are multiple pulmonary nodules which may grow slowly or calcify [5], Bronchoscopy is normal. One case with atypical cells isolated in bronchial washing has been reported [7], There appears to be no experience regarding cellularity obtained by bronchoalveolar lavage in these pa tients. We performed this examination and observed no cellular abnormalities. Adenopathies have been described in the mediasti num and in the abdomen; they were also found to in vade the pleura and to involve other organs, such as the liver and kidney [3, 6, 8]. In spite of their name, which suggests an epithelial origin, the most widely accepted histogenesis is that of a multicentric sarcoma originating in mesenchymal vasoformative reserve cells [9, 10]. This origin might explain the simultane ous presence of multiple pulmonary nodules and a possible hepatic lesion. The latter may be metastatic, particularly if not found at diagnosis. In our patient, the hepatic involvement was diagnosed simulta neously with the pulmonary involvement. There is no specific treatment for these patients. The present trend is to provide symptomatic treat-
Downloaded by: Temple University 155.247.166.234 - 2/3/2019 7:45:20 AM
Discussion
397
Intravascular Bronchoalveolar Tumor
ment when required. At the moment this patient re mains without treatment. Regarding the factors of poor prognosis, it has been seen that it worsens when more than one organ is involved and also in cases of respiratory insufficiency [5, 11]. According to published cases, patients with in volvement of the lung and other organs do not live more than 12 months. Nevertheless, our patient, with liver involvement and without respiratory insuffi ciency, remains alive and asymptomatic 2.5 years af ter diagnosis. Her thorax radiography, respiratory function tests and hematologic and biochemical stud ies have not changed. We think that, perhaps, poor prognosis depends more on respiratory insufficiency than on the involvement of several organs. Acknowledgement We thank Dr. J.Castella for his collaboration and Ms. Nuria Martinez for typing the manuscript.
5 Sicilian L, Warson F, Carrington ChB, Hayes J, Gaensler EA: Intravascular bronchioloalveolar tumor (IVSBAT). Respira tion 1983:44:387-394. 6 Delcourt A, Le Charpentier Y. Raphael M. Blétry O, Lovahlia S. Godeau P. Chomette G: Tumeur sclérosante intravasculaire bronchiolo-alvéolaire (IVSBAT). Arch Anal Cytol Path 1988: 36:96-99. 7 Dail DH, Liebow AA, Gmelich JT, Friedman PJ, Miyai K, Myer W, Patterson SD, Hammar SP: Intravascular, bronchiolar and alveolar tumor of the lung (IVSBAT). Cancer 1983:51: 452-464. 8 Corrin B, Harrison WJ, Wright D: The so-called intravascular bronchioalveolar tumor of the lung (low grade sclerosing angio sarcoma) presentation with extrapulmonary deposits. Diagn Histopathol 1983:6:229-237. 9 Azumi N, Churg A: intravascular and sclerosing bronchio alveolar tumor. Am J Surg Pathol 1981;5:587-596. 10 Weldon-Linne CM, Victor TA, Chist ML, et al: Angiogenic na ture of the intravascular bronchioloalveolar tumor of the lung. Arch Pathol Lab Med 1981:105:174-179. 11 Kantelip B, Champiat B, Mignot P, Fonck Y, Molina CL: «In travascular bronchioloalveolar tumor» ou IVSBAT. A propos d’un cas et revue de la littérature. Rev Pneumol Clin 1985:41: 273-282.
References
Received: March 27, 1990 Accepted after revision: September 26, 1990 M.S. Prats Aparell Respiratori Hospital Sant Pau Av. St. Antoni M. Claret, 167 E-08025 Barcelona (Spain)
Downloaded by: Temple University 155.247.166.234 - 2/3/2019 7:45:20 AM
1 Dail DH. Liebow AA: Intravascular bronchioloalveolar tumor (abstract). Am J Pathol 1975:78:6a. 2 Verbeken E. Beyls J, Moerman P. et al: Lung metastasis of ma lignant epithelioid hemangioendothelioma mimicking a pri mary intravascular bronchioalveolar tumor. Cancer 1985;55: 1741-1746. 3 Sherman JL, Rykwalder PJ, Tashkin DP: Intravascular bron chioloalveolar tumor. Am Rev Respir Dis 1981:123:468-470. 4 Emery RW, Fox A, Raab DE: Intravascular bronchioloalveolar tumor. Thorax 1982:37:472-473.
Respiration 1990;57:395-397
Intravascular Bronchoalveolar Tumor M.S. Prats ', C. Cáliz*. F.J. Sancho''. R. Cornudellaa Departments of 1Pneumology and h Pathology, Hospital de la Sta. Creu i St. Pau, Barcelona. Spain
Key Words. Intravascular bronchoalveolar tumor • Bronchoalveolar lavage • Hepatic nodules Abstract. We report the case of a young woman presenting productive cough and arthralgia in the left ankle. Chest radiography revealed multiple bilateral pulmonary nodules and abdominal echography and computer ized axial tomography demonstrated various hepatic nodules. Definitive diagnosis of an intravascular bron choalveolar tumor was reached by an open pulmonary biopsy. Liver involvement was confirmed by laparos copy and biopsy.
Introduction Since the first description of an intravascular bron choalveolar tumor by Dail and Liebow [1], approxi mately 60 further cases have been published. This tu mor appears to be more frequent in women, and the age group ranges between 12 and 71. Most patients appear asymptomatic. Chest radiographs show bilat eral multiple pulmonary nodules. Survival ranges be tween 4 months and 12 years.
Case Report
blood gases were within the reference ranges. Abdominal echogra phy showed a nodule of 28 mm diameter with a peripheric halo in each hepatic lobe. Computerized axial tomography revealed pul monary nodules disseminated in both hemithoraxes and a right paratracheal adenopathy (fig. 1). Bone and thyroid gammagraphy, barium oesophagogastroduodenal study, mammography, bone marrow biopsy and gynecologic examination were normal. A pul monary biopsy was performed by thoracotomy. Macroscopy
A fragment of pulmonary parenchyma of 2.5 x 2 x I cm was studied. The pleural surface was slightly depressed in two small areas of increased consistency. Sectioning at these points showed nodular areas with a mean diameter of 0.4 cm, consisting of clearly differentiated firm whitish tissue.
The lesion consisted of round or oval nodules, clearly delin eated from the neighboring pulmonary parenchyma, but not encapsulated (fig. 2). Peripherally they consisted of cells of large polygonal cytoplasm, occasionally with vacuoles, and only slightly pleomorphic vesicular nuclei. No mitosis was observed. The cells appeared to be covered with an eosinophilic matrix, forming poly poid masses, occupying the alveolar spaces. Centrally, the nodules became progressively more fibrotic and acellular, obliterating the alveolar spaces without destroying their walls, as demonstrated in the reticulin and elastic fibers. Vascular invasion was observed in various areas with tumoral exclusion of the small arteries and veins. There was also bronchiolar invasion with partial destruction of the lining epithelium (fig. 3).
Downloaded by: Temple University 155.247.166.234 - 2/3/2019 7:45:20 AM
Microscopy
A 17-year-old woman was admitted to our hospital in May 1987 for investigation of bilateral pulmonary nodules discovered 4 weeks previously on a chest radiogram taken because she had a productive cough and pain in the left ankle. There was no history of pulmonary tuberculosis, exposure to dusts, recent travel or allergy. She had been smoking 6 cigarettes a day. Menarche was at 12 and her menstrual cycles were 28/3-4 days. On admission, physical ex amination, routine laboratory tests were normal with the exception of a gammaglobulinemia of 19.3 g/1 (normal 8.2-14 g/1). The eye fundis were normal. PPD 5-UT was negative. Bronchoscopy, bron choalveolar lavage (macrophages 90%, lymphocytes 5%, polymorphonuclears 5%) and transbronchial biopsy were normal. Microbi ologie and cytologic studies were negative. Spirometry and arterial
Prats/Câli7./Sancho/Cornudella
396
No acid-alcohol-resistant bacilli or fungi were identified and cul tures were negative. Pathologic diagnosis was intravascular bronchoalveolar tumor. Laparoscopy showed a normal-sized liver with lenticular images and tiny nodular plaques in both lobes. Focal substitution of the liver parenchyma due to tumoral proliferation similar to that of the lung was observed in the biopsy.
Fig. 1. Computerized axial tomography of chest with multiple bilateral nodules.
Fig. 2. Periphery of one of the tumoral nodes. Alveolar spaces are filled with polypoid masses with hyalin centers and cellular pe riphery. HE. x 400.
Fig. 3. Invasion with partial destruction of the wall of a bron chiole. HH. < 100.
The pathology in a patient with pulmonary nod ules and liver nodules is most likely tumoral. From a morphologic point of view the microscopic image of an intravascular bronchoalveolar tumor is suffi ciently specific to eliminate other primary or metas tatic neoplasms with the exception of the rare possi bility of métastasés of epitheloid-hemangioendothelioma [2]. The morphology of this infrequent tumor is similar to that of intravascular bronchoalveolar tu mors and it is a vascular sarcoma with a very similar histogenesis. The symptomatology may vary from isolated ar thralgia, as in the case described by Sherman et al. [3], to right upper-quadrant pain, as reported by Emery et al. [4]. Clinical presentation is usually masked and the evolution sometimes of long duration [5] although it may be acute and aggressive, as an expression of hemopathy [6]. The radiological features described to date are multiple pulmonary nodules which may grow slowly or calcify [5], Bronchoscopy is normal. One case with atypical cells isolated in bronchial washing has been reported [7], There appears to be no experience regarding cellularity obtained by bronchoalveolar lavage in these pa tients. We performed this examination and observed no cellular abnormalities. Adenopathies have been described in the mediasti num and in the abdomen; they were also found to in vade the pleura and to involve other organs, such as the liver and kidney [3, 6, 8]. In spite of their name, which suggests an epithelial origin, the most widely accepted histogenesis is that of a multicentric sarcoma originating in mesenchymal vasoformative reserve cells [9, 10]. This origin might explain the simultane ous presence of multiple pulmonary nodules and a possible hepatic lesion. The latter may be metastatic, particularly if not found at diagnosis. In our patient, the hepatic involvement was diagnosed simulta neously with the pulmonary involvement. There is no specific treatment for these patients. The present trend is to provide symptomatic treat-
Downloaded by: Temple University 155.247.166.234 - 2/3/2019 7:45:20 AM
Discussion
397
Intravascular Bronchoalveolar Tumor
ment when required. At the moment this patient re mains without treatment. Regarding the factors of poor prognosis, it has been seen that it worsens when more than one organ is involved and also in cases of respiratory insufficiency [5, 11]. According to published cases, patients with in volvement of the lung and other organs do not live more than 12 months. Nevertheless, our patient, with liver involvement and without respiratory insuffi ciency, remains alive and asymptomatic 2.5 years af ter diagnosis. Her thorax radiography, respiratory function tests and hematologic and biochemical stud ies have not changed. We think that, perhaps, poor prognosis depends more on respiratory insufficiency than on the involvement of several organs. Acknowledgement We thank Dr. J.Castella for his collaboration and Ms. Nuria Martinez for typing the manuscript.
5 Sicilian L, Warson F, Carrington ChB, Hayes J, Gaensler EA: Intravascular bronchioloalveolar tumor (IVSBAT). Respira tion 1983:44:387-394. 6 Delcourt A, Le Charpentier Y. Raphael M. Blétry O, Lovahlia S. Godeau P. Chomette G: Tumeur sclérosante intravasculaire bronchiolo-alvéolaire (IVSBAT). Arch Anal Cytol Path 1988: 36:96-99. 7 Dail DH, Liebow AA, Gmelich JT, Friedman PJ, Miyai K, Myer W, Patterson SD, Hammar SP: Intravascular, bronchiolar and alveolar tumor of the lung (IVSBAT). Cancer 1983:51: 452-464. 8 Corrin B, Harrison WJ, Wright D: The so-called intravascular bronchioalveolar tumor of the lung (low grade sclerosing angio sarcoma) presentation with extrapulmonary deposits. Diagn Histopathol 1983:6:229-237. 9 Azumi N, Churg A: intravascular and sclerosing bronchio alveolar tumor. Am J Surg Pathol 1981;5:587-596. 10 Weldon-Linne CM, Victor TA, Chist ML, et al: Angiogenic na ture of the intravascular bronchioloalveolar tumor of the lung. Arch Pathol Lab Med 1981:105:174-179. 11 Kantelip B, Champiat B, Mignot P, Fonck Y, Molina CL: «In travascular bronchioloalveolar tumor» ou IVSBAT. A propos d’un cas et revue de la littérature. Rev Pneumol Clin 1985:41: 273-282.
References
Received: March 27, 1990 Accepted after revision: September 26, 1990 M.S. Prats Aparell Respiratori Hospital Sant Pau Av. St. Antoni M. Claret, 167 E-08025 Barcelona (Spain)
Downloaded by: Temple University 155.247.166.234 - 2/3/2019 7:45:20 AM
1 Dail DH. Liebow AA: Intravascular bronchioloalveolar tumor (abstract). Am J Pathol 1975:78:6a. 2 Verbeken E. Beyls J, Moerman P. et al: Lung metastasis of ma lignant epithelioid hemangioendothelioma mimicking a pri mary intravascular bronchioalveolar tumor. Cancer 1985;55: 1741-1746. 3 Sherman JL, Rykwalder PJ, Tashkin DP: Intravascular bron chioloalveolar tumor. Am Rev Respir Dis 1981:123:468-470. 4 Emery RW, Fox A, Raab DE: Intravascular bronchioloalveolar tumor. Thorax 1982:37:472-473.
