REFERENCES
2 3 4 5 6
7
Fauci AS. The vasculitis syndromes. In: Wilson JD, Braunwald E, Isselbacher KJ, Petersdorf RG, Martin JB, Fauci AS, et ai, eds. Harrison's principles of internal medicine. 12th ed. New York: McGraw-Hili, 1991:1456-63 Abramovsky CR, Swinehart GL. The nephropathy of cystic fibrosis: a human model of chronic nephrotoxicity. Hum Pathoi 1982; 13:934-39 Soter NA, Mihm MC, Colten HR. Cutaneous necrotizin~ venulitis in patients with cystic fibrosis. J Pediatr 1979; 95:197-201 Finnejtan MJ, Hinchcliffe J, Russell-Jones D, Neill S, Sheffield E, Jayne D, et aI. Vasculitis Iuputed torno~raphil' (el, scan of his chest showed a hu~(' tumor in his ri~ht lun~ extendin~ to the nlt'diastiuurn (Fi~ 1). H.'sults of th.· remainder of the workup were not dia~nostic. Due to tilt' pt'rsisteut hemothorax, the ri~ht side of his chest was t'xplort'd sur~il'ally. The tumor had a dark-red and yellowish appearance with many an>as of Ilt'crosis. The patlllllo~ic findin~s of the tumor showl,d a hiphasil' structure with a major undifft'rentiated mesenchymal and a minor epithelial olmponenl. The epitllt'lial elt>ments st pain and dyspnea. 1"0 other trt'atnll'nt was ~iven to him excl>pt tht· supportive measnrenlt'nts. Ill' died a month later wilh no "vidence of distant metastases. DISCUSSION
Spenct'r was thl' first to su~est thl' term pulmonary l
blastol1U1 for this very rare lung tumor hI'C;\lISl' of his concept of the tumor arisin~ from the pulmonary hlastoma in a CHEST I 102 I 2 I AUGUST, 1992
649
productive cough, chest pain, and ra. ely hemoptysis. not massive and persistent hemothorax as in our patient. The tumor is usually located in the peripheral portion of the lun~, althou~h in one child at least, the lesion arose centrally and it was endobronchial in ori~in.' The treatment of this tumor has been most commonly by sur~ery alone. althou~h both irradiation and chemotherapy have been ~iven both as adjuvants or to treat metastatic disease. The pro~nosis is poor amon~ those patients who have metastases or a primary tumor more than 5 cm in diameter.' There have been patients with prolon~ed survival after a noncurative sur~ery followed hy combination chemotherapy with drugs like cyclophosphamide, vincristine, doxorubicin. and dactinomycin. t.,. The selection of the above combination of chemotherapy was based on putative histo~enic similarities of pulmonary blastoma to Wilms' tumor and emhryonal rhabdomyosarcoma. Our selection of chemotherapy was based mainly on the domination of the sarcomatous over the epithelial elements in the histoloh'Y report. We noticed a distinct subjective response of short duration. which did not correspond to definitive objective response. REFERE~CES
Spencer II. Pulmonary blastomas. J Pathol Bal'lt'rioll961; 112: 16165
manner analo~ous to the ori~in of \Vilm's tnmor from the renal hlastoma. This concept has been Iluestioned recently on the basis of electron microscopic studies;t. the ori~in is now uncertain. ' The presentin~ symptoms of this tumor have heen non-
2 McCann MP. Fu r, Kay S. Pulmonary hlastoma: a li~ht and e1el'lron micros~"pic study. Canct'r 1976; 38:789-97 3 Fun~ CH, Lo J\V, ¥lman TI'\, Milloy FJ, Hakami MM. Chan~us G\\' Pulmonary hlastoma: an ultrastructural study with a hrit'f rt'vit'w of literatnrt' and a discussion of patho~ent'sis. Canct'r 1977; 39:153-63 4 Mt'dhery CA. Bihro MC, Phart's JC, Veach SR, Martin JE, Pasllnale DN. Pulmonar~' hlastoma: cast' rt'port and IHt'raturt' rt'vit'w of chemotht'rapy t'xperienct'. Canct'r 19R4; 5.1:2413-16 5 Leeson TS. Leeson CR. A li~ht and elel'lron microsmpt' study of dt'velopin~ respiratory tissue in tht' rat. J Anat 1964; 911: 1113-93 6 Roth JA, EI~ut'zahal A. Pulmonary hlastoma t'\'olvin~ into carcinosarcoma. Am J Sur~ Pathol197R; 2:407-13
650
Pulmonary Blastoma Presenting with Massive Hemothorax (Vassilopoulos al all
FIr.I'H~: 2. Foci of cartila~t' surroundt'd by tumor tissut' (Ilt'matoxylin-eosin, origiualma~nification x 25).
2 3 4 5 6
7
Fauci AS. The vasculitis syndromes. In: Wilson JD, Braunwald E, Isselbacher KJ, Petersdorf RG, Martin JB, Fauci AS, et ai, eds. Harrison's principles of internal medicine. 12th ed. New York: McGraw-Hili, 1991:1456-63 Abramovsky CR, Swinehart GL. The nephropathy of cystic fibrosis: a human model of chronic nephrotoxicity. Hum Pathoi 1982; 13:934-39 Soter NA, Mihm MC, Colten HR. Cutaneous necrotizin~ venulitis in patients with cystic fibrosis. J Pediatr 1979; 95:197-201 Finnejtan MJ, Hinchcliffe J, Russell-Jones D, Neill S, Sheffield E, Jayne D, et aI. Vasculitis Iuputed torno~raphil' (el, scan of his chest showed a hu~(' tumor in his ri~ht lun~ extendin~ to the nlt'diastiuurn (Fi~ 1). H.'sults of th.· remainder of the workup were not dia~nostic. Due to tilt' pt'rsisteut hemothorax, the ri~ht side of his chest was t'xplort'd sur~il'ally. The tumor had a dark-red and yellowish appearance with many an>as of Ilt'crosis. The patlllllo~ic findin~s of the tumor showl,d a hiphasil' structure with a major undifft'rentiated mesenchymal and a minor epithelial olmponenl. The epitllt'lial elt>ments st pain and dyspnea. 1"0 other trt'atnll'nt was ~iven to him excl>pt tht· supportive measnrenlt'nts. Ill' died a month later wilh no "vidence of distant metastases. DISCUSSION
Spenct'r was thl' first to su~est thl' term pulmonary l
blastol1U1 for this very rare lung tumor hI'C;\lISl' of his concept of the tumor arisin~ from the pulmonary hlastoma in a CHEST I 102 I 2 I AUGUST, 1992
649
productive cough, chest pain, and ra. ely hemoptysis. not massive and persistent hemothorax as in our patient. The tumor is usually located in the peripheral portion of the lun~, althou~h in one child at least, the lesion arose centrally and it was endobronchial in ori~in.' The treatment of this tumor has been most commonly by sur~ery alone. althou~h both irradiation and chemotherapy have been ~iven both as adjuvants or to treat metastatic disease. The pro~nosis is poor amon~ those patients who have metastases or a primary tumor more than 5 cm in diameter.' There have been patients with prolon~ed survival after a noncurative sur~ery followed hy combination chemotherapy with drugs like cyclophosphamide, vincristine, doxorubicin. and dactinomycin. t.,. The selection of the above combination of chemotherapy was based on putative histo~enic similarities of pulmonary blastoma to Wilms' tumor and emhryonal rhabdomyosarcoma. Our selection of chemotherapy was based mainly on the domination of the sarcomatous over the epithelial elements in the histoloh'Y report. We noticed a distinct subjective response of short duration. which did not correspond to definitive objective response. REFERE~CES
Spencer II. Pulmonary blastomas. J Pathol Bal'lt'rioll961; 112: 16165
manner analo~ous to the ori~in of \Vilm's tnmor from the renal hlastoma. This concept has been Iluestioned recently on the basis of electron microscopic studies;t. the ori~in is now uncertain. ' The presentin~ symptoms of this tumor have heen non-
2 McCann MP. Fu r, Kay S. Pulmonary hlastoma: a li~ht and e1el'lron micros~"pic study. Canct'r 1976; 38:789-97 3 Fun~ CH, Lo J\V, ¥lman TI'\, Milloy FJ, Hakami MM. Chan~us G\\' Pulmonary hlastoma: an ultrastructural study with a hrit'f rt'vit'w of literatnrt' and a discussion of patho~ent'sis. Canct'r 1977; 39:153-63 4 Mt'dhery CA. Bihro MC, Phart's JC, Veach SR, Martin JE, Pasllnale DN. Pulmonar~' hlastoma: cast' rt'port and IHt'raturt' rt'vit'w of chemotht'rapy t'xperienct'. Canct'r 19R4; 5.1:2413-16 5 Leeson TS. Leeson CR. A li~ht and elel'lron microsmpt' study of dt'velopin~ respiratory tissue in tht' rat. J Anat 1964; 911: 1113-93 6 Roth JA, EI~ut'zahal A. Pulmonary hlastoma t'\'olvin~ into carcinosarcoma. Am J Sur~ Pathol197R; 2:407-13
650
Pulmonary Blastoma Presenting with Massive Hemothorax (Vassilopoulos al all
FIr.I'H~: 2. Foci of cartila~t' surroundt'd by tumor tissut' (Ilt'matoxylin-eosin, origiualma~nification x 25).
