Pediatric Pulmonology 11:181-186 (1991)
Epithelioid Hemangioendothelioma of the Lung (Intravascular Bronchioloalveolar Tumor) in a Young Girl Michael J. Rock, MD,’ Robert A. Kaufman, MD,* Thom E Lobe, M D , ’ , ~ Samuel D. Hensley, M D , ~and Max L. Moss, M D ~ Summary. This report describes a 7 year old girl who presentedwith scoliosis and a small right hemithorax. Findings on spine roentgenographs were pleural thickening on the right and parenchymal densities. Computed tomography of the chest and abdomen revealed multiple pulmonary nodules and two hypervascular lesions within the liver. The diagnosis established by lung biopsy was intravascular bronchioloalveolar tumor (epithelioid hemangioendotheliomaof the lung). This is the youngest reported case of this rare tumor. Pediatr Pulmonol. 1991; 11:181-186. Key words: Lung tumor, computed tomography, epithelioid hemangioendotheliorna
INTRODUCTION
upper lobe, blunting of the right costophrenic angle, and decreased volume of the right lung. Her discharge An unusual tumor of the lung consisting of micropolydiagnoses from the emergency room were viral gastropoid masses filling alveoli and bronchioles was described enteritis and “probable pulmonary scarring secondary to by Dail and Liebow in 1975.’ Because the cells at the a remote pneumonia.” She was brought to her pediatriperiphery resembled type I1 alveolar cells and because of cian’s office 4 months later with the mother’s complaint the high rate of vascular invasion, this tumor was named that the child’s shoulder blades and chest were asymmetintravascular bronchioloalveolar tumor (IVBAT). Furric, the right side of the chest being smaller than the left. ther investigation by electron microscopy revealed occaOn examination, the child was found to have a thoracic sional pinocytotic vesicles at the cell membrane and scoliosis, and spine X-ray films again revealed that the electron dense microtubular bodies known as Weibellungs were abnormal. She was referred to the pulmonolPalade bodies.2 This suggested an endothelial origin for ogy clinic at Le Bonheur Children’s Medical Center. The these tumors, supported further by positive staining for past medical history was unremarkable. There was no factor-VIII-related antigen (FVIII-RAG) .3 Although it is coughing, fever, or pulmonary symptoms; the child was a misnomer, the popular term IVBAT continues to be able to run and keep up with her peers. There was no used to refer to this tumor. exposure to tuberculosis or other infectious illnesses, and Dail and Liebow expanded on their original abstract‘ no history of previous pneumonias. Chest roentgenograby pubishing a series of 20 cases of the tumor in 1983.4 phy performed at 11 months of age, because of a septic In this series, 80% of the cases were women in the age arthritis, was normal. range of 12-61 years at the time of diagnosis. Nine On physical examination, this was a slender female, patients presented without respiratory symptoms and between the 2Sh.and 50th percentile for both height and three had tumor involvement of liver. We report a 7 year weight, breathing comfortably with a respiratory rate of old child who had an unusual presentation of this slow 24iminute. When she was bent over touching her shoes growing tumor. with her finger tips, it was apparent that the left hemiCASE REPORT
From the Departments of Pediatrics,’ Radiology,’ S ~ r g e r y ,and ~
~ of Tennessee and Le Bonheur Children’s The 7 year, 5 month old black female presented to the P a t h ~ l o g y ,University emergency room in her local community with a history of Medical Center, Memphis, Tennessee. vomiting twice at school and diarrhea for 2 days. On Received December 10, 1990; (revision) accepted for publication April auscultation of the chest, rales were heard in the right 11, 1991. base and the remainder of the lungs was clear. A chest roentgenograph revealed pleural thickening in the apex of Address correspondence and reprint requests to Dr. R.A. Kaufman, the right hemithorax, increased opacities in the right 848 Adams Avenue, Memphis, TN 38103.
0 1991 Wiley-Liss, Inc.
182
Rock et al.
Fig. 1. Chest roentgenogram 5 months after presentation shows mild scoliosis, a smaller right hemithorax, and extensive pleural thickening. Note nodular parenchymal densities throughout both lungs.
thorax was larger than the right hemithorax. When sitting, her right shoulder was depressed 3 cm lower than the left shoulder. Auscultation of the lungs revealed decreased breath sounds throughout the entire right hemithorax. Roentgenography of the chest was unchanged compared with the findings of 5 months previously (Fig. 1). Spirometry was attempted; however the patient could not adequately perform a forced vital capacity maneuver.
Laboratory studies revealed: negative PPD 5 TU skin test; hemoglobin level, 13.4 grddL; hematocrit, 37%; leukocyte count, 6,300/mm3 with 57% neutrophils, 33% lymphocytes, and 10%monocytes; erythrocyte sedimentation rate, 40 mm/hr (normal 0-13 m d h r ) . Computed tomography (CT) of the chest and upper abdomen were performed first without contrast and then with a bolus injection of 40 mL of Omnipaque-300 (iohexol, Winthrop Pharmaceuticals) contrast medium, administered intravenously. Findings in the chest showed a small right hemithorax with mediastinal shift to the right and acquired scoliosis. There was a rind of thickened irregular pleura on the right with circumferential involvement, and adjacent ribs had a thickened periosteum. Multiple pulmonary nodules varying in size with a mean size of 5 mm were seen. These nodules were bilateral, but more numerous on the right than on the left, located both centrally and peripherally (Fig. 2). There were two low attenuation masses within the liver. The most inferior lesion was rounded and well circumscribed, measuring 2.8 cm in diameter. The more superior and anterior lesion was oblong and measured 3.3 by 1.7 cm (Fig. 3). Images after bolus injection of contrast showed that the low density lesions had peripheral enhancement and on delayed scanning filled in in a centripetal fashion, thus demonstrating the contrast dynamics of a hemangioma. With a diagnosis still not established, she was taken to the operating room for open lung and diaphragmatic biopsies. A thoracotomy through the right sixth intercostal space exposed the lung, the upper lobe of which was contracted and scarred with a firm, gray to white plaque-like tissue. Multiple nodules of the same firm tissue were scattered throughout the remaining lung, and plaques and nodules lined the parietal pleura over the chest wall and diaphragm. Biopsies of the lung and
Fig. 2. Chest computed tomography. A: Lung window demonstrates multiple, non-calcified parenchymal nodules greater in number on the right, associated hilar adenopathy, and right-sided volume loss. 8: Bone window at level of carina shows pleural thickening and smaller right hemithorax. Note also extensive periosteal reaction deposited about the ribs on right.
lntravascular Bronchioloalveolar Tumor
Fig. 3. Computed tomography section through liver during bolus contrast enhancement. Peripheral, wedge-shaped, low attenuation lesion in medial segment of left lobe demonstrated subsequent centripetal fill-in on delayed scans (not shown).
183
diaphragmatic nodules were obtained. Histologically, the tumor nodules showed a peripheral growth zone and cellular balls filling alveoli and small vessels. The centers of the nodules were less cellular and often sclerotic (Fig. 4). The nodule from the diaphragm was mostly sclerotic but it contained the same process. Immunoperoxidase staining for FVIII-RAG was positive. Electron microscopy showed large polygonal cells that were closely opposed, with occasional intracellular junctions. The cytoplasm was filled with fine filaments, and some cells had pinocytotic vesicles along the cell membrane (Fig. 5). The patient recovered from the biopsy without complication. She is currently in school and is doing well, except that she occasionally complains of dull abdominal and chest pain. D~SCUSS~ON Although this tumor received the name IVBAT from Dail and Liebow in 1975, it had been previously reported in the literature as primary chondrosarcoma of the lungs5
Fig. 4. The advancing border of this tumor nodule shows balls of tumor cells filling an alveolus. The center of some cellular balls exhibits typical sclerosis. Hematoxylin-eosin,formalin fixation, X 400.
184
Rock et al.
Fig. 5. Electron micrograph shows a tumor cell filled with fine filaments and pinocytoticvesicles (arrows) along the cell membrane. Inset shows Weibel-Palade bodies. Magnification 6,960 x 2; inset 10,182 ~ 2 ) .
and as pulmonary deciduosis.6 Other suggested names for this neoplasm include sclerosing angiogenic tumor,' sclerosing interstitial vascular sarcoma,8 sclerosing epithelioid angiosarcoma of the lung,' and epithelioid hemangioendothelioma of the lung.'" This last term was coined by Weiss and Enzinger in 1982 and is the preferred pathologic nomenclature for this tumor. Additionally, it has become increasingly apparent that epithelioid hemangioendothelioma also occurs in soft tisuse, bone, and liver." In addition to the series of 20 patients reported by Dail and L i e b ~ wother , ~ cases had been Many pdtients were without respiratory symptoms and the tumors were found incidentally on chest roentgenograms. Radiologically, the most characteristic feature was the presence of many small nodular densities in both lungs. Histologically, these tumors had sparsely cellular hyaline cores with a more cellular periphery that projected into adjacent alveoli in a contiguous micropolypoid fashion.
Alveolar septa were compressed and the lung structure was not evident within the tumors in hematoxylin and eosin stained sections; however, special stains showed that the elastin framework of the alveoli and blood vessels was preserved. In Dail and Liebow's ~ e r i e sthe , ~ visceral pleura and immediate subpleural zones were involved in over one half of the cases. Immunoperoxidase staining for FVIII-RAG was frequently positive. Electron microscopic observations have revealed polygonal cells with central nuclei and occasional prominent nucleoli. The cytoplasm always contained abundant microfilaments. Pinocytotic vesicles were occasionally observed, clustered at the cell membrane, and the presence of WeibelPalade bodies has been variably reported. In addition to the 3/20 cases reported by Dail and Liebow with liver i n ~ o l v e m e n t numerous ,~ other cases with tumor in the lung and liver have been p ~ b l i s h e d . ~ ~ ~ ' Controversy exists regarding the origin of the tumor in these two locations. Emery et al.25 stated that the hepatic
Intravascular BronchioloalveolarTumor
tumor was metastatic from the IVBAT tumor, but Echevarria proposed that this entity is not primary to the lungs and that liver tumors have been consistently o ~ e r l o o k e dIn . ~our ~ case, CT of the abdomen was done because of a liver lesion discovered incidentally on the lower sections of the chest CT. Although these lesions were not biopsied, they had enhancement characteristics of liver hemangiomas and it is presumed that they also are epithelioid hemangioendotheliomas. This tumor is slow growing, its clinical course being intermediate between a hemangioma and an angiosarcoma. Multiple chemotherapeutic regimens have been tried, but no improvement documented. Death occurs by extensive lung involvement and slow respiratory compromise. Survival has ranged from within several months of diagnosis to 24 years, with a mean survival of 4.6 years in Dail and Liebow’s ~ e r i e sThe . ~ longest reported surviving patient was a 17 year old girl who initially had surgical excision of a solitary tumor, followed over the next 24 years by 11 additional surgeries for recurrent multiple lung tumors.23 A poor prognosis is associated with extensive intravascular, endobronchial, interstitial, and pleural spread. The outcome is also worse with peripheral lymphadenopathy and hepatic involvement. In conclusion, we report the youngest patient with epithelioid hemangioendothelioma of the lung (IVBAT). Although the tumor was bilateral, there was more extensive involvement of the right lung with loss of volume and resultant scoliosis. Such preferential involvement of one lung has been reported once before in a 24 year old patient whose left lung was airless and contracted on a ~ t o p s yThe . ~ bilateral nature of our patient’s disease was more readily apparent on computed tomography, which graphically demonstrated the tumor nodules in both lungs. Biopsy of the tumor and light microscopy were necessary for diagnosis, with immunoperoxidase staining and electron microscopy as useful diagnostic adjuncts.
REFERENCES 1. Dail D, Liebow A. Intravascular bronchioloalveolar tumor. Am J Pathol. 1975; 78:6-7a (abstract). 2. Corrin B, Manners B, Millard M, Weaver L. Histogenesis of the so-called “intravascular bronchioloalveolar tumour.” J Pathol. 1979; 128:163-167. 3. Weldon-Linne CM, Victor TA, Christ ML. Immunohistochemical identification of factor VIII-related antigen in the intravascular bronchioloalveolar tumor of the lung. Arch Pathol Lab Med. 1981; 105:628429. 4. Dail DH, Liebow AA, Gmelich JT, Friedman PJ, Miyai K, Myer W, Patterson SD, Hammar SP. Intravascular, bronchiolar, and alveolar tumor of the lung (IVBAT). Cancer. 1983; 51:452-464. 5 . Smith EAC, Cohen RV, Peale AR. Primary chondrosarcoma of the lung. Ann Intern Med. 1960; 53338-846. 6. Farinacci CJ, Blauw AS, Jennings EM. Multifocal pulmonary lesions of possible decidual origin (so-called pulmonary deciduosis). Am J Clin Pathol. 1973; 59:508-514.
185
7. Weldon-Linne CM, Victor TA, Christ ML, Fry WA. Angiogenic nature of the ‘intravascular bronchioloalveolar tumor’ of the lung. Arch Pathol Lab Med. 1981; 105:174-179. 8. Azumi N, Churg A. Intravascular and sclerosing bronchioloalveolar tumor: A pulmonary sarcoma of probable vascular origin. Am J Surg Pathol. 1981; 5587-596. 9. Bhagavan BS, Dorfman HD, Murthy MSN, Eggleston JC. Intravascular bronchioloalveolar tumor (IVBAT): A low-grade sclerosing epithelioid angiosarcoma of lung. Am J Surg Pathol 1982; 6:41-52. 10. Weiss SW, Enzinger FM. Epithelioid hemangioendothelioma: A vascular tumor often mistaken for a carcinoma. Cancer 1982; 50:97&981. 11. Weiss SW, Ishak KG, Dail DH, Sweet DE, Enzinger FM. Epithelioid hemangioendothelioma and related lesions. Semin Diagn Pathol. 1986; 3:259-287. 12. Ferrer-Roca 0. Intravascular and sclerosing bronchio-alveolar tumor. Am J Surg Pathol. 1980; 4:375-381. 13. Wenisch HJC, Lulay M. Lymphogenous spread of an intravascular bronchioloalveolar tumour. Virchows Arch. 1980; 387: 117123. 14. Sherman JL, Rykwalder PJ, Tashkin DP. Intravascular bronchioloalveolar tumor. Am Rev Respir Dis. 1981; 123:468470. 15. Marsh K, Kenyon WE, Earis JE, Pearson MG. Intravascular bronchioloalveolar tumour. Thorax. 1982; 37:474475. 16. Sicilian L. Warson F, Carrington CB, Hayes J, Gaensler EA. Intravascular bronchioloalveolar tumor (IV-BAT). Respiration. 1983; 44:387-394. 17. Taguchi T, Tsuji K, Matsuo K, Takebayashi S, Kawahara K, Hadama T. Intravascular bronchioloalveolar tumor: Report of an autopsy case and review of literature. Acta Pathol Jpn. 1985; 35:631-642. 18. Verbeken E, Beyls J, Moerman P, Knockaert D, Goddeeris P, Lauweryns JM. Lung metastasis of malignant epithelioid hemangioendothelioma mimicking a primary intravascular bronchioalveolar tumor: A histologic, ultrastructural, and imniunohistochemical study. Cancer 1985; 55:1741-1746. 19. Borlee-Hermans G , Bury T, Grand JL, Jardon-Jeghers C, Radermecker M. Intravascular bronchioloalveolar tumour. Eur J Respir Dis. 1985; 66:341-346. 20. Teo SK, Chiang SC, Tan KK. Intravascular bronchioloalveolar tumour: A 20-year survival. Med J Aust. 1985; 142:22&222. 21. Eggleston JC. The intravascular bronchioloalveolar tumor and the sclerosing hemangioma of the lung: Misnomers of pulmonary neoplasia. Semin Diagn Pathol. 1985; 2:27&280. 22. Yousem SA, Hochholzer L. Unusual thoracic manifestations of epithelioid hemangioendothelioma. Arch Pathol Lab Med. 1987; 11 1:459463. 23. Miettinen M, Collan Y, Halttunen P, Maamies T, Vilkko P. Intravascular bronchioloalveolar tumor. Cancer. 1987; 60:24712475. 24. Echevamia RA, Arean VM, Galindo L. Hepatic tumors of long duration with eventual metastases: Two cases of leiomyosarcomatosis possibly arising from hamartomas of liver. Am J Clin Pathol. 1978; 69:624-631. 25. Emery RW, Fox AL, Raab DE. Intravascular bronchioloalveolar tumour. Thorax. 1982; 37:472473. 26. Comn B, Harrison WJ, Wright DH. The so-called intravascular bronchioloalveolar tumour of lung (low grade sclerosing angiosarcoma): Presentation with extrapulmonary deposits. Diagn Histopathol. 1983; 6:229-237. 27. Fukayama M, Nihei Z, Takizawa T, Kawaguchi K, Harada H, Koike M. Malignant epithelioid hemangioendothelioma of the liver, spreading through the hepatic veins. Virchows Arch. 1984; 404~275-287. 28. Gledhill A, Kay JM. Hepatic metastases in a case of intravascular bronchioloalveolar tumour. J Clin Pathol. 1984; 37:279-282.
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29. Ishak KG, Sesterhenn IA, Goodman ZD, Rabin L, Stromeyer FW. Epithelioid hemangioendothelioma of the liver: A clinicopathologic and follow-up study of 32 cases. Hum Pathol. 1984; 15:839-852. 30. Nakatani Y, Aoki I, Misugi K . Immunohistochemical and ultrastructural study of early lesions of intravascular bronchioloalveolar tumor with liver involvement. Acta Pathol Jpn. 1985; 35:1453-1465.
31. Ekfors TO, Joensuu K, Toivio I, Launnen P, Pelttari L. Fatal epithelioid haemangioendothelioma presenting in the lung and liver. Virchows Arch. 1986; 410:9-16. 32. Echevania RA. Angiogenic nature of “intravascular bronchioloalveolar tumor.’ Arch Pathol Lab Med. 1981; 105:627-628.
Epithelioid Hemangioendothelioma of the Lung (Intravascular Bronchioloalveolar Tumor) in a Young Girl Michael J. Rock, MD,’ Robert A. Kaufman, MD,* Thom E Lobe, M D , ’ , ~ Samuel D. Hensley, M D , ~and Max L. Moss, M D ~ Summary. This report describes a 7 year old girl who presentedwith scoliosis and a small right hemithorax. Findings on spine roentgenographs were pleural thickening on the right and parenchymal densities. Computed tomography of the chest and abdomen revealed multiple pulmonary nodules and two hypervascular lesions within the liver. The diagnosis established by lung biopsy was intravascular bronchioloalveolar tumor (epithelioid hemangioendotheliomaof the lung). This is the youngest reported case of this rare tumor. Pediatr Pulmonol. 1991; 11:181-186. Key words: Lung tumor, computed tomography, epithelioid hemangioendotheliorna
INTRODUCTION
upper lobe, blunting of the right costophrenic angle, and decreased volume of the right lung. Her discharge An unusual tumor of the lung consisting of micropolydiagnoses from the emergency room were viral gastropoid masses filling alveoli and bronchioles was described enteritis and “probable pulmonary scarring secondary to by Dail and Liebow in 1975.’ Because the cells at the a remote pneumonia.” She was brought to her pediatriperiphery resembled type I1 alveolar cells and because of cian’s office 4 months later with the mother’s complaint the high rate of vascular invasion, this tumor was named that the child’s shoulder blades and chest were asymmetintravascular bronchioloalveolar tumor (IVBAT). Furric, the right side of the chest being smaller than the left. ther investigation by electron microscopy revealed occaOn examination, the child was found to have a thoracic sional pinocytotic vesicles at the cell membrane and scoliosis, and spine X-ray films again revealed that the electron dense microtubular bodies known as Weibellungs were abnormal. She was referred to the pulmonolPalade bodies.2 This suggested an endothelial origin for ogy clinic at Le Bonheur Children’s Medical Center. The these tumors, supported further by positive staining for past medical history was unremarkable. There was no factor-VIII-related antigen (FVIII-RAG) .3 Although it is coughing, fever, or pulmonary symptoms; the child was a misnomer, the popular term IVBAT continues to be able to run and keep up with her peers. There was no used to refer to this tumor. exposure to tuberculosis or other infectious illnesses, and Dail and Liebow expanded on their original abstract‘ no history of previous pneumonias. Chest roentgenograby pubishing a series of 20 cases of the tumor in 1983.4 phy performed at 11 months of age, because of a septic In this series, 80% of the cases were women in the age arthritis, was normal. range of 12-61 years at the time of diagnosis. Nine On physical examination, this was a slender female, patients presented without respiratory symptoms and between the 2Sh.and 50th percentile for both height and three had tumor involvement of liver. We report a 7 year weight, breathing comfortably with a respiratory rate of old child who had an unusual presentation of this slow 24iminute. When she was bent over touching her shoes growing tumor. with her finger tips, it was apparent that the left hemiCASE REPORT
From the Departments of Pediatrics,’ Radiology,’ S ~ r g e r y ,and ~
~ of Tennessee and Le Bonheur Children’s The 7 year, 5 month old black female presented to the P a t h ~ l o g y ,University emergency room in her local community with a history of Medical Center, Memphis, Tennessee. vomiting twice at school and diarrhea for 2 days. On Received December 10, 1990; (revision) accepted for publication April auscultation of the chest, rales were heard in the right 11, 1991. base and the remainder of the lungs was clear. A chest roentgenograph revealed pleural thickening in the apex of Address correspondence and reprint requests to Dr. R.A. Kaufman, the right hemithorax, increased opacities in the right 848 Adams Avenue, Memphis, TN 38103.
0 1991 Wiley-Liss, Inc.
182
Rock et al.
Fig. 1. Chest roentgenogram 5 months after presentation shows mild scoliosis, a smaller right hemithorax, and extensive pleural thickening. Note nodular parenchymal densities throughout both lungs.
thorax was larger than the right hemithorax. When sitting, her right shoulder was depressed 3 cm lower than the left shoulder. Auscultation of the lungs revealed decreased breath sounds throughout the entire right hemithorax. Roentgenography of the chest was unchanged compared with the findings of 5 months previously (Fig. 1). Spirometry was attempted; however the patient could not adequately perform a forced vital capacity maneuver.
Laboratory studies revealed: negative PPD 5 TU skin test; hemoglobin level, 13.4 grddL; hematocrit, 37%; leukocyte count, 6,300/mm3 with 57% neutrophils, 33% lymphocytes, and 10%monocytes; erythrocyte sedimentation rate, 40 mm/hr (normal 0-13 m d h r ) . Computed tomography (CT) of the chest and upper abdomen were performed first without contrast and then with a bolus injection of 40 mL of Omnipaque-300 (iohexol, Winthrop Pharmaceuticals) contrast medium, administered intravenously. Findings in the chest showed a small right hemithorax with mediastinal shift to the right and acquired scoliosis. There was a rind of thickened irregular pleura on the right with circumferential involvement, and adjacent ribs had a thickened periosteum. Multiple pulmonary nodules varying in size with a mean size of 5 mm were seen. These nodules were bilateral, but more numerous on the right than on the left, located both centrally and peripherally (Fig. 2). There were two low attenuation masses within the liver. The most inferior lesion was rounded and well circumscribed, measuring 2.8 cm in diameter. The more superior and anterior lesion was oblong and measured 3.3 by 1.7 cm (Fig. 3). Images after bolus injection of contrast showed that the low density lesions had peripheral enhancement and on delayed scanning filled in in a centripetal fashion, thus demonstrating the contrast dynamics of a hemangioma. With a diagnosis still not established, she was taken to the operating room for open lung and diaphragmatic biopsies. A thoracotomy through the right sixth intercostal space exposed the lung, the upper lobe of which was contracted and scarred with a firm, gray to white plaque-like tissue. Multiple nodules of the same firm tissue were scattered throughout the remaining lung, and plaques and nodules lined the parietal pleura over the chest wall and diaphragm. Biopsies of the lung and
Fig. 2. Chest computed tomography. A: Lung window demonstrates multiple, non-calcified parenchymal nodules greater in number on the right, associated hilar adenopathy, and right-sided volume loss. 8: Bone window at level of carina shows pleural thickening and smaller right hemithorax. Note also extensive periosteal reaction deposited about the ribs on right.
lntravascular Bronchioloalveolar Tumor
Fig. 3. Computed tomography section through liver during bolus contrast enhancement. Peripheral, wedge-shaped, low attenuation lesion in medial segment of left lobe demonstrated subsequent centripetal fill-in on delayed scans (not shown).
183
diaphragmatic nodules were obtained. Histologically, the tumor nodules showed a peripheral growth zone and cellular balls filling alveoli and small vessels. The centers of the nodules were less cellular and often sclerotic (Fig. 4). The nodule from the diaphragm was mostly sclerotic but it contained the same process. Immunoperoxidase staining for FVIII-RAG was positive. Electron microscopy showed large polygonal cells that were closely opposed, with occasional intracellular junctions. The cytoplasm was filled with fine filaments, and some cells had pinocytotic vesicles along the cell membrane (Fig. 5). The patient recovered from the biopsy without complication. She is currently in school and is doing well, except that she occasionally complains of dull abdominal and chest pain. D~SCUSS~ON Although this tumor received the name IVBAT from Dail and Liebow in 1975, it had been previously reported in the literature as primary chondrosarcoma of the lungs5
Fig. 4. The advancing border of this tumor nodule shows balls of tumor cells filling an alveolus. The center of some cellular balls exhibits typical sclerosis. Hematoxylin-eosin,formalin fixation, X 400.
184
Rock et al.
Fig. 5. Electron micrograph shows a tumor cell filled with fine filaments and pinocytoticvesicles (arrows) along the cell membrane. Inset shows Weibel-Palade bodies. Magnification 6,960 x 2; inset 10,182 ~ 2 ) .
and as pulmonary deciduosis.6 Other suggested names for this neoplasm include sclerosing angiogenic tumor,' sclerosing interstitial vascular sarcoma,8 sclerosing epithelioid angiosarcoma of the lung,' and epithelioid hemangioendothelioma of the lung.'" This last term was coined by Weiss and Enzinger in 1982 and is the preferred pathologic nomenclature for this tumor. Additionally, it has become increasingly apparent that epithelioid hemangioendothelioma also occurs in soft tisuse, bone, and liver." In addition to the series of 20 patients reported by Dail and L i e b ~ wother , ~ cases had been Many pdtients were without respiratory symptoms and the tumors were found incidentally on chest roentgenograms. Radiologically, the most characteristic feature was the presence of many small nodular densities in both lungs. Histologically, these tumors had sparsely cellular hyaline cores with a more cellular periphery that projected into adjacent alveoli in a contiguous micropolypoid fashion.
Alveolar septa were compressed and the lung structure was not evident within the tumors in hematoxylin and eosin stained sections; however, special stains showed that the elastin framework of the alveoli and blood vessels was preserved. In Dail and Liebow's ~ e r i e sthe , ~ visceral pleura and immediate subpleural zones were involved in over one half of the cases. Immunoperoxidase staining for FVIII-RAG was frequently positive. Electron microscopic observations have revealed polygonal cells with central nuclei and occasional prominent nucleoli. The cytoplasm always contained abundant microfilaments. Pinocytotic vesicles were occasionally observed, clustered at the cell membrane, and the presence of WeibelPalade bodies has been variably reported. In addition to the 3/20 cases reported by Dail and Liebow with liver i n ~ o l v e m e n t numerous ,~ other cases with tumor in the lung and liver have been p ~ b l i s h e d . ~ ~ ~ ' Controversy exists regarding the origin of the tumor in these two locations. Emery et al.25 stated that the hepatic
Intravascular BronchioloalveolarTumor
tumor was metastatic from the IVBAT tumor, but Echevarria proposed that this entity is not primary to the lungs and that liver tumors have been consistently o ~ e r l o o k e dIn . ~our ~ case, CT of the abdomen was done because of a liver lesion discovered incidentally on the lower sections of the chest CT. Although these lesions were not biopsied, they had enhancement characteristics of liver hemangiomas and it is presumed that they also are epithelioid hemangioendotheliomas. This tumor is slow growing, its clinical course being intermediate between a hemangioma and an angiosarcoma. Multiple chemotherapeutic regimens have been tried, but no improvement documented. Death occurs by extensive lung involvement and slow respiratory compromise. Survival has ranged from within several months of diagnosis to 24 years, with a mean survival of 4.6 years in Dail and Liebow’s ~ e r i e sThe . ~ longest reported surviving patient was a 17 year old girl who initially had surgical excision of a solitary tumor, followed over the next 24 years by 11 additional surgeries for recurrent multiple lung tumors.23 A poor prognosis is associated with extensive intravascular, endobronchial, interstitial, and pleural spread. The outcome is also worse with peripheral lymphadenopathy and hepatic involvement. In conclusion, we report the youngest patient with epithelioid hemangioendothelioma of the lung (IVBAT). Although the tumor was bilateral, there was more extensive involvement of the right lung with loss of volume and resultant scoliosis. Such preferential involvement of one lung has been reported once before in a 24 year old patient whose left lung was airless and contracted on a ~ t o p s yThe . ~ bilateral nature of our patient’s disease was more readily apparent on computed tomography, which graphically demonstrated the tumor nodules in both lungs. Biopsy of the tumor and light microscopy were necessary for diagnosis, with immunoperoxidase staining and electron microscopy as useful diagnostic adjuncts.
REFERENCES 1. Dail D, Liebow A. Intravascular bronchioloalveolar tumor. Am J Pathol. 1975; 78:6-7a (abstract). 2. Corrin B, Manners B, Millard M, Weaver L. Histogenesis of the so-called “intravascular bronchioloalveolar tumour.” J Pathol. 1979; 128:163-167. 3. Weldon-Linne CM, Victor TA, Christ ML. Immunohistochemical identification of factor VIII-related antigen in the intravascular bronchioloalveolar tumor of the lung. Arch Pathol Lab Med. 1981; 105:628429. 4. Dail DH, Liebow AA, Gmelich JT, Friedman PJ, Miyai K, Myer W, Patterson SD, Hammar SP. Intravascular, bronchiolar, and alveolar tumor of the lung (IVBAT). Cancer. 1983; 51:452-464. 5 . Smith EAC, Cohen RV, Peale AR. Primary chondrosarcoma of the lung. Ann Intern Med. 1960; 53338-846. 6. Farinacci CJ, Blauw AS, Jennings EM. Multifocal pulmonary lesions of possible decidual origin (so-called pulmonary deciduosis). Am J Clin Pathol. 1973; 59:508-514.
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