castleman 's disease ( cd ) is a rare lymphoproliferative disorder caused by faulty immune regulation resulting in excessive b - lymphocyte and plasma - cell proliferation in lymphatic tissue .
marked interfollicular vascular proliferation leads to mass formation , which can encase bronchi and sometimes cause mural erosion leading to life - threatening hemoptysis .
vascular supply to these lesions have been reported to arise from the bronchial , internal mammary , and intercostal arteries .
we report a case of hilar intrathoracic cd with atypical radiological features where the left inferior phrenic artery and bronchial circulation provided codominant blood supply .
a 24-year - old female with a 5-month history of wheeze and nonproductive cough underwent chest x - ray , which showed a well - defined mass in the left hilar region [ figure 1a  d ] . a contrast - enhanced computed tomography ( ct ) scan of the chest revealed a heterogeneously enhancing 7  5 cm mass containing flecks of calcification at the left hilum . 18 fluorodeoxyglucose positron emission tomography ( 18-fdg pet )
scan demonstrated an inhomogeneous hypermetabolic lesion with no evidence of metastatic uptake . a ct - guided percutaneous core biopsy revealed chronic inflammatory tissue with no evidence of malignancy .
due to worsening cough and wheeze , the patient underwent a thoracotomy and surgical resection . during the operation there was massive blood loss of 2000 ml .
histopathology confirmed the mass to have marked lymphoid infiltrate with irregular b cell follicles and hyaline vascular - like change consistent with cd .
chest x - ray of a 24-year - old female ( a ) showing well - defined left hilar mass .
contrast - enhanced ct scan ( b and c ) shows heterogeneously enhancing mass with coarse calcifications within this hilar mass .
18-fdg pet / ct scan ( d ) shows hypermetabolic uptake within the lesion two months after the thoracotomy , the patient presented to the emergency department with a 1-day history of massive hemoptysis ( > 200 ml ) .
a ct pulmonary angiogram for clinically suspected pulmonary embolism showed no pulmonary embolism , however , multiplanar reformats showed low attenuation soft tissue in the surgical resection bed at the left hilum receiving blood supply through hypertrophied left bronchial and left inferior phrenic arteries [ figure 2a  f ] .
the patient had an embolization through a right common femoral artery approach and the common ( conjoined ) origin of the right broncho - superior intercostal trunk and left bronchial arteries were catheterized with a 5 fr ( cordis , usa ) left amplatz coronary i catheter ( cordis , usa ) .
the sharply angulated left bronchial artery could not be superselectively catheterized with a range of microcatheters .
the main trunk was embolized with 300500 micron polyvinyl alcohol ( pva ) particles ( cook , usa ) .
the medial division of the inferior phrenic artery was seen to supply the mass via its pericardial branch .
this was super - selectively catheterized with the same 5 fr catheter and a renegade microcatheter ( boston scientific , usa ) and embolized with the same pva particles .
no spinal , esophageal , or coronary supply was demonstrated from the bronchial and left inferior phrenic arteries .
there were no procedure - related complications and the patient had an uneventful recovery with no further hemoptysis during the 3 years follow - up period .
contrast - enhanced ct with mip images ( a and c ) showing blood supply to the lesion with bronchial artery and left inferior phrenic artery ( arrows ) .
catheter angiogram demonstrates bronchial artery ( arrow in b ) and left inferior phrenic artery ( arrow in d ) and subsequent embolization with pva ( e and f )
cd may be localized or multicentric and primarily involves the mediastinum , neck , and mesentery .
there are three known histological variates namely ( a ) hyaline - vascular , ( b ) plasma - cell , and ( c ) mixed variant .
classic features on a ct scan is a solitary , intensely , and homogeneously enhancing mass with no local invasion .
the standard treatment of the localized form is surgical resection , however , where there is encasement or invasion of the adjacent structures , preoperative embolization has been advocated . recently ,
embolization alone without surgical resection has been shown to terminate hemoptysis and alleviate shortness of breath due to airway compression in patients with unresectable disease . in our patient ,
preoperative embolization , which may have reduced the large volume intraoperative blood loss , was not considered .
it was only when the patient presented with hemoptysis after surgical resection that the vascular supply was identified and an emergency referral to interventional radiology was made .
previous reports have identified bronchial , internal mammary , and intercostal arteries as feeding vessels to cd .
inferior phrenic supply to cd has not been reported before to the best of our knowledge .
when basal lung segments are suspected as the source of hemoptysis , inferior phrenic supply should always be considered .
inferior phrenic supply to hilar masses is much rarer , but when it occurs , the supply is usually via the pericardial branch of its medial division .
the left inferior phrenic medial branch may supply the esophagus , and it is important to exclude any esophageal supply before particulate embolization .
careful review of multiplanar reformats of the ct scan aided procedure planning and the consent process by identifying the hypertrophied arterial feeders .
cd should be considered in the differential diagnosis for all benign intrathoracic masses . where a mass shows avid enhancement , whether homogeneous or heterogeneous , a ct angiogram ,
 castleman 's disease ( cd ) is a rare lympho - proliferative disorder due to faulty immune regulation resulting in proliferation of lymphatic tissue . 
 the vascular supply to these lesions have been reported to arise from the bronchial , internal mammary and the intercostal arteries . 
 we report a case of hemoptysis secondary to intrathoracic cd with vascular supply arising from the left inferior phrenic artery which was successfully embolised with polyvinyl alcohol ( pva ) particles . 