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Recurrent Massive Hemothorax of Unknown Etiology in an 85-Year-Old Man

2022; Elsevier BV; Volume: 161; Issue: 2 Linguagem: Inglês

10.1016/j.chest.2021.09.012

1931-3543

Yuki Okamatsu, Kazuya Tsubouchi, Takeshi Iwasaki, Tomoya Nakamura, Tadayuki Nakashima, Kousei Nakatsuru, Yuriko Takahata, Taishi Harada,

Eosinophilic Disorders and Syndromes

Case PresentationAn 85-year-old Japanese man, who was taking aspirin and edoxaban for previous myocardial infarction and atrial fibrillation, came to our hospital with a chief complaint of dyspnea for 3 weeks. Chest radiography showed a massive left pleural effusion (Fig 1A). Analysis of pleural fluid showed an elevated hematocrit level at 32.8% (blood hematocrit level, 32.0%), and he was diagnosed with hemothorax. However, he had neither coagulation disorder nor thrombocytopenia, and the pleural effusion was negative for atypical cells. These findings suggested that the antithrombotic and anticoagulant medications might have induced the hemothorax. An 85-year-old Japanese man, who was taking aspirin and edoxaban for previous myocardial infarction and atrial fibrillation, came to our hospital with a chief complaint of dyspnea for 3 weeks. Chest radiography showed a massive left pleural effusion (Fig 1A). Analysis of pleural fluid showed an elevated hematocrit level at 32.8% (blood hematocrit level, 32.0%), and he was diagnosed with hemothorax. However, he had neither coagulation disorder nor thrombocytopenia, and the pleural effusion was negative for atypical cells. These findings suggested that the antithrombotic and anticoagulant medications might have induced the hemothorax. Ten days after pleural fluid drainage with a needle, and discontinuation of aspirin and edoxaban, there was no recurrence of hemothorax; therefore, edoxaban was restarted. However, 13 days after resuming edoxaban, he was hospitalized for exacerbation of dyspnea due to recurrence of hemothorax. Dynamic contrast-enhanced chest CT imaging revealed a massive left pleural effusion and multiple nodular shadows with a halo sign on both lungs (Figs 1B-1D). However, it did not show the possible causes of hemothorax, such as pneumothorax or vascular disease. Thoracoscopy under local anesthesia was performed to confirm the cause of the hemothorax. Although several blood clots were seen in the pleura, no lesion indicating a neoplasm was detected (Fig 2A). The specimens obtained from the random pleural biopsy did not reveal obvious growth of abnormal blood vessels or malignant cells (Fig 2B). Because multiple lung nodular shadows on CT scanning suggested the possibility of malignancy, we performed diffusion-weighted whole-body imaging background body signal suppression (DWIBS), which is MRI that allows acquisition of volumetric diffusion-weighted images of the entire body and is useful for tumor screening. Although the DWIBS images showed abnormal signals in the lumbar spine and pelvic bone (Figs 1E and 1F), the specimens obtained by needle biopsy from the ilium did not show malignant findings. Besides the hemothorax, the bleeding from the ilium was also not controlled after the operation. Furthermore, more than 2 months after the first visit, the patient developed hematuria and bladder tamponade without any triggers. Enhanced CT imaging did not reveal any abnormal findings in the bladder (Fig 1G), and a cytologic examination of the urine did not reveal any atypical cells. Cystoscopy showed only blood clots, but not the source of the bleeding. A bladder biopsy was not performed, to avoid further bleeding. Although bladder perfusion, pleurodesis (Unitalc [Nobelpharma], 4 g; and Picibanil [OK-432; Chugai Pharmaceutical], 5 Klinische Einheit [KE]), and reoperation were performed to control the bleeding, hematuria, hemothorax, and bleeding at the site of biopsy were refractory to treatment. The patient died 4 months after the first visit. An autopsy was performed to investigate the pathology of the disease. What is the diagnosis? Diagnosis•Primary bladder angiosarcoma•Hemothorax due to pleural metastasis•Multiple pulmonary and bone metastases Angiosarcoma is an uncommon malignant tumor of endothelial differentiation observed in approximately 2% of soft-tissue sarcomas. The primary sites are the skin, soft tissue, breast, liver, heart, bone, and spleen.1Young R.J. Brown N.J. Reed M.W. Hughes D. Woll P.J. Angiosarcoma.Lancet Oncol. 2010; 11: 983-991Google Scholar Primary bladder angiosarcomas are extremely rare. Jungano et al2Jungano F. Sur un cas d’angiosarcome de la vessie.Ann Mal Organes Genitourinares. 1907; 25: 1451-1464Google Scholar first described a primary urinary bladder angiosarcoma in 1907. To our knowledge, there are only 38 cases reported in the literature at the present time.3Gerbaud F. Ingels A. Ferlicot S. Irani J. Angiosarcoma of the bladder: review of the literature and discussion about a clinical case.Urol Case Rep. 2017; 13: 97-100Google Scholar, 4Martinez F.J. Villanueva A.G. Pickering R. Becker F.S. Smith D.R. Spontaneous hemothorax: report of 6 cases and review of the literature.Medicine (Baltimore). 1992; 71: 354-368Google Scholar, 5Spina B. Pacella E. Introini C. Boccardo F. Lopez-Beltran A. Ricci R. Primary bladder angiosarcoma with no apparent previous exposure to carcinogens: a case report.Anal Quant Cytopathol Histpathol. 2013; 35: 349-352Google Scholar, 6Nawar N.A. Olsen J. Jelic T.M. He C. Primary urinary bladder angiosarcoma with osteoclast-like multinucleated giant cells: a case report and literature review.Am J Case Rep. 2016; 17: 143-149Google Scholar, 7Wang G. Black P.C. Skinnider B.F. Hayes M.M. Jones E.C. Post-radiation epithelioid angiosarcoma of the urinary bladder and prostate.Can Urol Assoc J. 2016; 10: E197-E200Google Scholar, 8Rallabandi H.B. Swain M. Gowrishankar S. Sinha S. Postradiation angiosarcoma of bladder with extensive osseous metaplasia.Indian J Pathol Microbiol. 2016; 59: 78-80Google Scholar, 9Tynski Z. Barrett A.J. Bastacky S.I. Primary urinary bladder angiosarcoma with ascites.Hum Pathol Case Rep. 2017; 10: 5-9Google Scholar, 10Nizam A. Paquette E.L. Wang B.G. Aragon-Ching J.B. Epithelioid angiosarcoma of the bladder: a case report and review of the literature.Clin Genitourin Cancer. 2018; 16: e1091-e1095Google Scholar The most common symptom is hematuria, and it is usually the first sign of primary bladder angiosarcoma (Table 12Jungano F. Sur un cas d’angiosarcome de la vessie.Ann Mal Organes Genitourinares. 1907; 25: 1451-1464Google Scholar, 3Gerbaud F. Ingels A. Ferlicot S. Irani J. Angiosarcoma of the bladder: review of the literature and discussion about a clinical case.Urol Case Rep. 2017; 13: 97-100Google Scholar, 4Martinez F.J. Villanueva A.G. Pickering R. Becker F.S. Smith D.R. Spontaneous hemothorax: report of 6 cases and review of the literature.Medicine (Baltimore). 1992; 71: 354-368Google Scholar, 5Spina B. Pacella E. Introini C. Boccardo F. Lopez-Beltran A. Ricci R. Primary bladder angiosarcoma with no apparent previous exposure to carcinogens: a case report.Anal Quant Cytopathol Histpathol. 2013; 35: 349-352Google Scholar, 6Nawar N.A. Olsen J. Jelic T.M. He C. Primary urinary bladder angiosarcoma with osteoclast-like multinucleated giant cells: a case report and literature review.Am J Case Rep. 2016; 17: 143-149Google Scholar, 7Wang G. Black P.C. Skinnider B.F. Hayes M.M. Jones E.C. Post-radiation epithelioid angiosarcoma of the urinary bladder and prostate.Can Urol Assoc J. 2016; 10: E197-E200Google Scholar, 8Rallabandi H.B. Swain M. Gowrishankar S. Sinha S. Postradiation angiosarcoma of bladder with extensive osseous metaplasia.Indian J Pathol Microbiol. 2016; 59: 78-80Google Scholar, 9Tynski Z. Barrett A.J. Bastacky S.I. Primary urinary bladder angiosarcoma with ascites.Hum Pathol Case Rep. 2017; 10: 5-9Google Scholar, 10Nizam A. Paquette E.L. Wang B.G. Aragon-Ching J.B. Epithelioid angiosarcoma of the bladder: a case report and review of the literature.Clin Genitourin Cancer. 2018; 16: e1091-e1095Google Scholar, 11Engel J.D. Kuzel T.M. Moceanu M. Oefelein M.G. Schaeffer A.J. Angiosarcoma of the bladder: a review.Urology. 1998; 52: 778-784Google Scholar, 12Casal J. Singer E.D. Monserrat J.M. [Angiosarcoma of the bladder][article in Spanish].Rev Argent Urol Nefrol. 1970; 39: 53-55Google Scholar, 13Stroup R.M. Chang Y.C. Angiosarcoma of the bladder: a case report.J Urol. 1987; 137: 984-985Google Scholar, 14Aragona F. Ostardo E. Prayer-Galetti T. Piazza R. Capitanio G. Angiosarcoma of the bladder: a case report with regard to histologic and immunohistochemical findings.Eur Urol. 1991; 20: 161-163Google Scholar, 15Ravi R. Primary angiosarcoma of the urinary bladder.Arch Esp Urol. 1993; 46: 351-353Google Scholar, 16Navon J.D. Rahimzadeh M. Wong A.K. Carpenter P.M. Ahlering T.E. Angiosarcoma of the bladder after therapeutic irradiation for prostate cancer.J Urol. 1997; 157: 1359-1360Google Scholar, 17Schindler S. De Frias D.V. Yu G.H. Primary angiosarcoma of the bladder: cytomorphology and differential diagnosis.Cytopathology. 1999; 10: 137-143Google Scholar, 18Seethala R.R. Gomez J.A. Vakar-Lopez F. Primary angiosarcoma of the bladder.Arch Pathol Lab Med. 2006; 130: 1543-1547Google Scholar, 19Pazona J.F. Gupta R. Wysock J. Schaeffer A.J. Smith N.D. Angiosarcoma of bladder: long-term survival after multimodal therapy.Urology. 2007; 69: 575.e9-575.e10Google Scholar, 20Kulaga A. Yilmaz A. Wilkin R.P. Trpkov K. Epithelioid angiosarcoma of the bladder after irradiation for endometrioid adenocarcinoma.Virchows Arch. 2007; 450: 245-246Google Scholar, 21Williams S. Romaguera R. Kava B. Angiosarcoma of the bladder: case report and review of the literature.ScientificWorldJournal. 2008; 8: 508-511Google Scholar, 22Tavora F. Montgomery E. Epstein J.I. A series of vascular tumors and tumorlike lesions of the bladder.Am J Surg Pathol. 2008; 32: 1213-1219Google Scholar, 23Warne R.R. Ong J.S. Snowball B. Vivian J.B. Primary angiosarcoma of the bladder in a young female.BMJ Case Rep. 2011; 2011bcr1120103484Google Scholar, 24Abbasov B. Munguia G. Mazal P.R. et al.Epithelioid angiosarcoma of the bladder: report of a new case with immunohistochemical profile and review of the literature.Pathology. 2011; 43: 290-293Google Scholar, 25Beyazal M. Pirinççi N. Yavuz A. Özkaçmaz S. Bulut G. Computed tomography and magnetic resonance imaging findings of primary bladder angiosarcoma: a case report.Clin Imaging. 2014; 38: 212-214Google Scholar, 26Bahouth Z. Masarwa I. Halachmi S. Nativ O. Primary angiosarcoma of urinary bladder: 13th reported patient.Case Rep Oncol Med. 2015; 2015: 652870Google Scholar, 27Matoso A. Epstein J.I. Epithelioid angiosarcoma of the bladder: a series of 9 cases.Am J Surg Pathol. 2015; 39: 1377-1382Google Scholar, 28Ojerholm E. Stripp D. Mamtani R. Van Arsdalen K. Tripp P. Angiosarcoma of the bladder following prostate radiotherapy.Am J. Med. 2015; 128: e11-e12Google Scholar). Dysuria, pain, obstruction, vaginal bleeding, and weight loss are often present. However, in our case, the initial symptom was hemothorax, which is extremely rare. A previous report described one patient, undergoing dialysis, with primary bladder angiosarcoma, which was triggered by hemothorax.4Martinez F.J. Villanueva A.G. Pickering R. Becker F.S. Smith D.R. Spontaneous hemothorax: report of 6 cases and review of the literature.Medicine (Baltimore). 1992; 71: 354-368Google Scholar It was difficult to diagnose primary bladder angiosarcoma as the cause of hemothorax in patients without hematuria.Table 1Characteristics of Patients With Primary Bladder AngiosarcomaStudy/YearAge (y)SexMain SymptomMetastasis at DiagnosisJungano2Jungano F. Sur un cas d’angiosarcome de la vessie.Ann Mal Organes Genitourinares. 1907; 25: 1451-1464Google Scholar/190754MHematuria, urinary obstructionN/ACasal et al12Casal J. Singer E.D. Monserrat J.M. [Angiosarcoma of the bladder][article in Spanish].Rev Argent Urol Nefrol. 1970; 39: 53-55Google Scholar/197085FHematuria, dysuria, weight lossN/AStroup and Chang13Stroup R.M. Chang Y.C. Angiosarcoma of the bladder: a case report.J Urol. 1987; 137: 984-985Google Scholar/198768MHematuriaN/AAragona et al14Aragona F. Ostardo E. Prayer-Galetti T. Piazza R. Capitanio G. Angiosarcoma of the bladder: a case report with regard to histologic and immunohistochemical findings.Eur Urol. 1991; 20: 161-163Google Scholar/199178MHematuria, dysuriaN/AMartinez et al4Martinez F.J. Villanueva A.G. Pickering R. Becker F.S. Smith D.R. Spontaneous hemothorax: report of 6 cases and review of the literature.Medicine (Baltimore). 1992; 71: 354-368Google Scholar/199253MDyspnea, chest discomfortPleural, lungRavi15Ravi R. Primary angiosarcoma of the urinary bladder.Arch Esp Urol. 1993; 46: 351-353Google Scholar/199355MHematuria…Navon et al16Navon J.D. Rahimzadeh M. Wong A.K. Carpenter P.M. Ahlering T.E. Angiosarcoma of the bladder after therapeutic irradiation for prostate cancer.J Urol. 1997; 157: 1359-1360Google Scholar/199778MHematuria…Engel et al11Engel J.D. Kuzel T.M. Moceanu M. Oefelein M.G. Schaeffer A.J. Angiosarcoma of the bladder: a review.Urology. 1998; 52: 778-784Google Scholar/199847MHematuria, flank pain, suprapubic pain…Schindler et al17Schindler S. De Frias D.V. Yu G.H. Primary angiosarcoma of the bladder: cytomorphology and differential diagnosis.Cytopathology. 1999; 10: 137-143Google Scholar/199947MHematuria, dysuria, suprapubic pain…Seethala et al18Seethala R.R. Gomez J.A. Vakar-Lopez F. Primary angiosarcoma of the bladder.Arch Pathol Lab Med. 2006; 130: 1543-1547Google Scholar/200666MHematuriaPeritoneumPazona et al19Pazona J.F. Gupta R. Wysock J. Schaeffer A.J. Smith N.D. Angiosarcoma of bladder: long-term survival after multimodal therapy.Urology. 2007; 69: 575.e9-575.e10Google Scholar/200747MHematuria, suprapubic pain…Kulaga et al20Kulaga A. Yilmaz A. Wilkin R.P. Trpkov K. Epithelioid angiosarcoma of the bladder after irradiation for endometrioid adenocarcinoma.Virchows Arch. 2007; 450: 245-246Google Scholar/200783FMicrohematuriaN/AWilliams et al21Williams S. Romaguera R. Kava B. Angiosarcoma of the bladder: case report and review of the literature.ScientificWorldJournal. 2008; 8: 508-511Google Scholar/200871MHematuria…Tavora et al22Tavora F. Montgomery E. Epstein J.I. A series of vascular tumors and tumorlike lesions of the bladder.Am J Surg Pathol. 2008; 32: 1213-1219Google Scholar/200873FHematuria…77MHematuriaN/A71MHematuriaN/A63FHematuriaN/AWarne et al23Warne R.R. Ong J.S. Snowball B. Vivian J.B. Primary angiosarcoma of the bladder in a young female.BMJ Case Rep. 2011; 2011bcr1120103484Google Scholar/201132FHematuria, left side flank painLungAbbasov et al24Abbasov B. Munguia G. Mazal P.R. et al.Epithelioid angiosarcoma of the bladder: report of a new case with immunohistochemical profile and review of the literature.Pathology. 2011; 43: 290-293Google Scholar/201151MHematuria…Spina et al5Spina B. Pacella E. Introini C. Boccardo F. Lopez-Beltran A. Ricci R. Primary bladder angiosarcoma with no apparent previous exposure to carcinogens: a case report.Anal Quant Cytopathol Histpathol. 2013; 35: 349-352Google Scholar/201367FHematuriaN/ABeyazal et al25Beyazal M. Pirinççi N. Yavuz A. Özkaçmaz S. Bulut G. Computed tomography and magnetic resonance imaging findings of primary bladder angiosarcoma: a case report.Clin Imaging. 2014; 38: 212-214Google Scholar/201420MHematuria, disseminated pelvic pain…Bahouth et al26Bahouth Z. Masarwa I. Halachmi S. Nativ O. Primary angiosarcoma of urinary bladder: 13th reported patient.Case Rep Oncol Med. 2015; 2015: 652870Google Scholar/201589MHematuriaN/AMatoso and Epstein27Matoso A. Epstein J.I. Epithelioid angiosarcoma of the bladder: a series of 9 cases.Am J Surg Pathol. 2015; 39: 1377-1382Google Scholar/201573FHematuriaN/A77MHematuriaN/A71MHematuriaN/A85MHematuriaN/A39MHematuriaN/A64MHematuriaN/A43MHematuriaN/A73MHematuriaN/A64MHematuriaN/AOjerholm et al28Ojerholm E. Stripp D. Mamtani R. Van Arsdalen K. Tripp P. Angiosarcoma of the bladder following prostate radiotherapy.Am J. Med. 2015; 128: e11-e12Google Scholar/201561MHematuria…Nawar et al6Nawar N.A. Olsen J. Jelic T.M. He C. Primary urinary bladder angiosarcoma with osteoclast-like multinucleated giant cells: a case report and literature review.Am J Case Rep. 2016; 17: 143-149Google Scholar/201668MHematuria, urinary retentionN/AWang et al7Wang G. Black P.C. Skinnider B.F. Hayes M.M. Jones E.C. Post-radiation epithelioid angiosarcoma of the urinary bladder and prostate.Can Urol Assoc J. 2016; 10: E197-E200Google Scholar/201679MHematuria, bladder irritation…Rallabandi et al8Rallabandi H.B. Swain M. Gowrishankar S. Sinha S. Postradiation angiosarcoma of bladder with extensive osseous metaplasia.Indian J Pathol Microbiol. 2016; 59: 78-80Google Scholar/201665FHematuriaBoneGerbaud et al3Gerbaud F. Ingels A. Ferlicot S. Irani J. Angiosarcoma of the bladder: review of the literature and discussion about a clinical case.Urol Case Rep. 2017; 13: 97-100Google Scholar/201772MHematuria, urinary obstruction…Tynski et al9Tynski Z. Barrett A.J. Bastacky S.I. Primary urinary bladder angiosarcoma with ascites.Hum Pathol Case Rep. 2017; 10: 5-9Google Scholar/201769MAbdominal distension, urinary obstructionPeritoneumNizam et al10Nizam A. Paquette E.L. Wang B.G. Aragon-Ching J.B. Epithelioid angiosarcoma of the bladder: a case report and review of the literature.Clin Genitourin Cancer. 2018; 16: e1091-e1095Google Scholar/201857MHematuriaBoneOkamatsu et al (current study)85MDyspneaBone, pleural, lungN/A = not available. Open table in a new tab N/A = not available. Bladder angiosarcoma has a poor prognosis, one poorer than that of angiosarcomas in other primary sites.11Engel J.D. Kuzel T.M. Moceanu M. Oefelein M.G. Schaeffer A.J. Angiosarcoma of the bladder: a review.Urology. 1998; 52: 778-784Google Scholar In the literature, the mean overall survival time is 10.6 months (range, 3 days to 6 years).3Gerbaud F. Ingels A. Ferlicot S. Irani J. Angiosarcoma of the bladder: review of the literature and discussion about a clinical case.Urol Case Rep. 2017; 13: 97-100Google Scholar Radical or palliative surgery, radiotherapy, and chemotherapy, or even a combination of these, have been performed to treat bladder angiosarcoma; however, there is still no consensus regarding the optimal treatment.3Gerbaud F. Ingels A. Ferlicot S. Irani J. Angiosarcoma of the bladder: review of the literature and discussion about a clinical case.Urol Case Rep. 2017; 13: 97-100Google Scholar In this case, it was very difficult to diagnose the patient’s condition early. Only pleurodesis and palliative surgery were performed to stop the bleeding. Most cases of hemothorax are related to chest trauma or medical procedures. Spontaneous hemothorax is an uncommon condition and is caused by spontaneous hemopneumothorax, malignancies, anticoagulant medications, vascular ruptures, pulmonary infarctions, and coagulopathy.29Ali H.A. Lippmann M. Mundathaje U. Khaleeq G. Spontaneous hemothorax: a comprehensive review.Chest. 2008; 134: 1056-1065Google Scholar The most common neoplasms associated with hemothorax include vascular abnormalities caused by pleural metastases of soft-tissue tumors such as angiosarcomas and hepatocellular carcinomas, and von Recklinghausen disease. Lung cancer is an uncommon cause of hemothorax even in the setting of pleural extension.29Ali H.A. Lippmann M. Mundathaje U. Khaleeq G. Spontaneous hemothorax: a comprehensive review.Chest. 2008; 134: 1056-1065Google Scholar The treatment of hemothorax depends on its cause and the patient’s condition. Chest tube thoracostomy is usually performed on a stable patient. In a hemodynamically unstable patient or one with persistent massive bleeding, an early surgical approach should be taken into consideration. In the case of anticoagulant-induced bleeding, it is necessary to correct the coagulopathy. Embolization is a valid option to treat hemothorax caused by vascular abnormalities. The early surgical management of residual hemothorax is one option for preventing fibrothorax and restrictive impairment due to a large number of blood clots.29Ali H.A. Lippmann M. Mundathaje U. Khaleeq G. Spontaneous hemothorax: a comprehensive review.Chest. 2008; 134: 1056-1065Google Scholar The clinical presentation of primary or metastatic pleural angiosarcoma is nonspecific, and is often difficult to diagnose. Cytologic examination of pleural fluid is unhelpful in most patients. Most cases have been diagnosed by surgical excision, including debulking excision, pleurectomy, decortication, or at autopsy.30Kao Y.-C. Chow J.-M. Wang K.-M. Fang C.-L. Chu J.-S. Chen C.-L. Primary pleural angiosarcoma as a mimicker of mesothelioma: a case report ∗∗VS∗∗.Diagn Pathol. 2011; 6: 130Google Scholar In the present case, we could not diagnose pleural angiosarcoma by pleural biopsy samples obtained by thoracoscopy under local anesthesia, but autopsy findings revealed metastatic pleural angiosarcoma (Figs 2A-2D). These findings suggest that deep and extensive surgical biopsy should be helpful in confirming the cause of refractory bloody pleural effusion of uncertain etiology. Two cases of pleural angiosarcoma were reported to be diagnosed by performing video-assisted thoracoscopic surgery for repeated hemopneumothorax without apparent pleural mass.31Isowa M. Tanaka S. Nakanobo R. Yamada Y. Date H. Refractory pneumothorax and hemothorax associated with metastatic scalp angiosarcoma.Surg Case Rep. 2020; 6: 272Google Scholar In bladder angiosarcoma, CT imaging and MRI usually demonstrate a large, highly vascular mass with irregular margins. The tumor can protrude into the bladder lumen and extend into the extravesical region. Hydronephrosis and hydroureter are known to be associated with this tumor.32Gaballah A.H. Jensen C.T. Palmquist S. et al.Angiosarcoma: clinical and imaging features from head to toe.Br J Radiol. 2017; 90: 20170039Google Scholar However, in our case, contrast-enhanced CT imaging and MRI of the pelvis did not reveal any abnormal findings suggestive of primary bladder angiosarcoma due to submucosal tumor extension. On the other hand, we were able to consider angiosarcoma on the basis of the chest CT findings (Figs 1C and 1D). Regardless of the origin site, angiosarcoma is particularly likely to metastasize to the lungs.33Tateishi U. Hasegawa T. Kusumoto M. et al.Metastatic angiosarcoma of the lung: spectrum of CT findings.Am J Roentgenol. 2003; 180: 1671-1674Google Scholar Therefore, chest CT imaging may be useful for suspected angiosarcoma. Metastatic pulmonary angiosarcomas exhibit a variety of radiographic appearances. According to a previous report, the most common CT finding of lung metastasis from angiosarcoma was multiple solid nodular lesions, seen in 15 of 24 patients (63%).33Tateishi U. Hasegawa T. Kusumoto M. et al.Metastatic angiosarcoma of the lung: spectrum of CT findings.Am J Roentgenol. 2003; 180: 1671-1674Google Scholar Ground-glass attenuation surrounding solid nodules, which corresponded to alveolar hemorrhage on microscopic observation, was often seen as in the present case. Furthermore, metastatic pulmonary angiosarcomas appear with various chest CT findings. Multiple thin-walled cysts, air-fluid levels in thin-walled cysts, ground-glass attenuation surrounding thin-walled cystic lesions, enlarged multiple thin-walled cysts, and thickened walls of the cysts have been seen in patients with metastatic lung angiosarcoma. The urinary bladder wall was thickened, and the hemorrhagic lesion was accompanied by ulceration on autopsy. An erosion was seen in a 5 × 5 cm2 section of the bladder body proximal to the fundus (Fig 3A). Microscopically, the section showed proliferation of atypical oval to spindle-shaped cells with enlarged hyperchromatic nuclei in fascicular and haphazard patterns, accompanied by vasoformation and lined by atypical endothelial cells. These cells were located primarily in the submucosa of the urinary bladder and had focally invaded the adipose tissue surrounding the urinary bladder (Fig 3B). Immunohistochemically, these cells were positive for ERG, CD31, and CD34 but negative for AE1/AE3, CAM5.2, α-SMA, desmin, GATA-3, MelanA, and HMB-45 (Fig 3C). Therefore, the patient was determined to have had an angiosarcoma. Bilateral massive hemothorax was seen. Multiple hemorrhagic lesions were observed on the visceral pleura of both lungs, predominantly on the left (Fig 2C). Histologically, the hemorrhagic lesions resembled the urinary bladder tumor (Fig 2D). The patient was then determined to have had metastatic lesions from angiosarcoma. A core-needle biopsy specimen of the parietal pleura showed hemorrhage, some reactive mesothelial cells, and fragmented fibrous tissue without atypical vessels. Therefore, this patient’s condition was difficult to diagnose as angiosarcoma by noninvasive pleural biopsy. Metastatic lesions were also observed in the previously biopsied iliac bone, the lumbar vertebrae (Figs 4A and 4B ), and bilateral lung parenchyma. An open biopsy specimen of the iliac bone showed small fragmented inflammatory granulated tissue with proliferation of irregularly shaped anastomosing vessels. We also observed atypical oval endothelial cells with vesicular nuclei and prominent nucleoli, accompanied by hemorrhage and hemosiderin deposits (Fig 4C). Atypical endothelial cells were positive for CD31, CD34, and ERG staining (Fig 4D). However, because neither mitotic figures nor vasoformation was seen in this specimen, it was difficult to distinguish reactive lesions; therefore, we could not make a definitive pathologic diagnosis. The differential diagnosis of angiosarcoma included granulation tissue, and angiosarcoma with bone lesions can be particularly difficult to distinguish from granulation tissue.34Nelson B.L. Thompson L.D.R. Sinonasal tract angiosarcoma: a clinicopathologic and immunophenotypic study of 10 cases with a review of the literature.Head Neck Pathol. 2007; 1: 1-12Google Scholar,35Siddaraju N. Soundararaghavan J. Bundele M.M. Roy S.K. Fine needle aspiration cytology of epithelioid angiosarcoma: a case report.Acta Cytol. 2008; 52: 109-113Google Scholar Because the symptoms of hemothorax manifested first, the possibility of primary angiosarcoma of the pleura and bladder metastasis could not be ruled out. However, the diagnosis of primary bladder angiosarcoma was made because of the following: the tumor was located primarily in the submucosal tissue; no lesion was found in the bladder serosa; the amount of tumor in the bladder was high; and the incidence of bladder metastasis was low. To our knowledge, there has been only one case of pleural angiosarcoma that metastasized to the bladder.36Otsubo S. Tanaka S. Kamiryo Y. Okumur K. Shimokama T. Kinjo M. A case of primary pleural angiosarcoma metastasing to the bladder.Nihon Hinyokika Gakkai Zasshi. 2011; 102: 686-690Google Scholar •We encountered a case of bladder angiosarcoma with spontaneous hemothorax as the first symptom; bladder angiosarcoma is a rare neoplasm with hematuria as the main symptom.•Although primary bladder angiosarcoma is a rare disease, cystoscopy should be done when the primary angiosarcoma lesion is unknown.•Primary or metastatic pleural angiosarcoma is often difficult to diagnose by cytologic examination of pleural effusion and noninvasive pleural biopsy; therefore, surgical or excision biopsy should be considered when pleural angiosarcoma is suspected.