first described pleuropulmonary blastoma ( ppb ) as a distinctive tumor in young children , occurring almost exclusively in the first decade of life . since then , individual cases as well as series of similar tumors in children have been reported with the diagnosis of ppb .
international ppb registry is maintaining a record of patients with ppb for last 20 years having published [ n = 50 ] as well as unpublished [ n = 128 ] registry series .
it has a rare occurrence beyond the first decade of life and only a few isolated cases in adults have been reported .
these tumors usually require a multimodality treatment approach for improving the survival including surgery , chemotherapy and radiation therapy .
we herein report a short series of two cases of ppb in adolescence , with one of them having a relatively long survival , an unusual presentation beyond the first decade of life .
a 14-year - old adolescent male presented with complaints of nonproductive cough for three months associated with dyspnea on exertion in july 2009 .
computed tomography ( ct ) of the chest showed an approximately 6  5  3 cm sized heterogenous mass lesion in the right lower lobe [ figure 1a ] .
endobronchial biopsy was done , which showed nests of malignant cells with hyperchromatic , elongated nuclei and moderate cytoplasm .
these cells were positive for vimentin on immunohistochemistry . a possibility of ppb was suggested .
, tumor was seen occupying approximately 90% of lower lobe and was greyish white in color with areas of cystic changes .
on microscopy , plump to spindle - shaped cells arranged in short fascicles as well as storiform pattern were seen .
the cells were showing moderate nuclear atypia , elongated nucleus , some showing nucleoli and moderate amount of cytoplasm with brisk mitoses . in between , there were vague nests of primitive blastemal cells . based on these features , a diagnosis of ppb
preoperative ( a ) axial computed tomography ( ct ) image showing a large heterogenously enhancing soft tissue mass lesion in the right hemithorax .
postoperative and postchemotherapy ( b ) ct image showing a heterogenous paravertebral soft tissue on the right side the patient was referred for radiotherapy , where he received a cumulative dose of 50 gray ( gy ) in 25 fractions .
the follow - up ct showed a heterogenous soft tissue in the right paravertebral location abutting the mediastinal pleura [ figure 1b ] . in view of residual lesion ,
the patient received six cycles of chemotherapy ( 250 mg paclitaxel and 70 mg cisplatin ) at 3-week intervals .
the sixth cycle was completed in march 2010 , and no residual lesion was seen in the follow - up ct .
however , in september 2011 , the patient started complaining of vague abdominal pain on and off .
fine - needle aspiration ( fna ) was done under ultrasound guidance , which showed highly cellular smears and sheets of undifferentiated cells having high nuclear / cytoplasmic ratio .
on immunocytochemistry , cells were positive for vimentin and neuron - specific enolase ( nse ) showed strong focal positivity .
in addition , nuclear positivity for wilms tumor ( wt-1 ) was seen in about 50% of cells .
the patient was referred to the urology department for the surgical consideration , however , was lost to follow up .
an 18-year - old adolescent male patient was referred recently to our institute from a hospital with complaints of abdominal pain with recurrent fever for the last 4 months .
a clinical possibility of a ruptured liver abscess in the right pleural cavity based on sonographic findings was documented in the records .
the ct showed a large solid - cystic mass lesion with thick enhancing septae filling almost the entire right hemithorax and extending inferiorly , indenting upon the right hemidiaphragm and displacing the liver inferiorly .
the mass was having loss of fat planes at places with the mediastinal structures , including trachea , heart , right innominate artery , and was compressing upon right main bronchus .
overlying ribs and soft tissues were normal with no extrathoracic extension [ figure 2 ] .
axial ct image showing a large heterogenous solid - cystic mass lesion occupying the entire right hemithorax .
no definite rib erosion and extrathoracic extension is seen a percutaneous ultrasound ( us)-guided fna was performed .
the aspirate was particulate and the smears were air - dried as well as alcohol - fixed and stained with may - grnwald giemsa and hematoxylin and eosin , respectively . a cell block was also prepared .
on microscopy , the smears were cellular , and showed sheets of small variably shaped poorly differentiated cells .
the cells were predominantly oval to spindle - shaped and a prominent perivascular arrangement was noted .
the tumor cells had high nuclear : cytoplasmic ratio , finely granular chromatin , inconspicuous nucleoli , and scanty eosinophilic cytoplasm with indistinct cell borders .
immunochemically , the tumor cells showed strong positivity for vimentin and focal positivity for smooth muscle actin [ figure 4 ] .
aspiration cytology of pleuropulmonary blastoma : ( a ) cellular smear with prominent perivascular pattern ( hematoxylin and eosin 200 ) ( b ) small ovoid to spindled cells with occasional cell showing cytoplasmic vacuolation , inset : small cell with cytoplasmic vacuolation [ may - grnwald giemsa ( mgg ) 400 ] ( c and d ) cell block showing oval to spindle - shaped cells with scanty eosinophilic cytoplasm with indistinct borders ( mgg 400 ) case 2 .
immunohistochemistry slides showing strong positivity of tumor cells for vimentin ( a - c ) and weak focal positivity for smooth muscle actin ( d ) the patient developed progressive respiratory dysfunction and died approximately seven days after the diagnosis of tumor .
a 14-year - old adolescent male presented with complaints of nonproductive cough for three months associated with dyspnea on exertion in july 2009 .
computed tomography ( ct ) of the chest showed an approximately 6  5  3 cm sized heterogenous mass lesion in the right lower lobe [ figure 1a ] .
endobronchial biopsy was done , which showed nests of malignant cells with hyperchromatic , elongated nuclei and moderate cytoplasm .
these cells were positive for vimentin on immunohistochemistry . a possibility of ppb was suggested .
, tumor was seen occupying approximately 90% of lower lobe and was greyish white in color with areas of cystic changes .
on microscopy , plump to spindle - shaped cells arranged in short fascicles as well as storiform pattern were seen .
the cells were showing moderate nuclear atypia , elongated nucleus , some showing nucleoli and moderate amount of cytoplasm with brisk mitoses . in between , there were vague nests of primitive blastemal cells . based on these features , a diagnosis of ppb
preoperative ( a ) axial computed tomography ( ct ) image showing a large heterogenously enhancing soft tissue mass lesion in the right hemithorax .
postoperative and postchemotherapy ( b ) ct image showing a heterogenous paravertebral soft tissue on the right side the patient was referred for radiotherapy , where he received a cumulative dose of 50 gray ( gy ) in 25 fractions .
the follow - up ct showed a heterogenous soft tissue in the right paravertebral location abutting the mediastinal pleura [ figure 1b ] . in view of residual lesion ,
the patient received six cycles of chemotherapy ( 250 mg paclitaxel and 70 mg cisplatin ) at 3-week intervals .
the sixth cycle was completed in march 2010 , and no residual lesion was seen in the follow - up ct .
however , in september 2011 , the patient started complaining of vague abdominal pain on and off .
fine - needle aspiration ( fna ) was done under ultrasound guidance , which showed highly cellular smears and sheets of undifferentiated cells having high nuclear / cytoplasmic ratio .
on immunocytochemistry , cells were positive for vimentin and neuron - specific enolase ( nse ) showed strong focal positivity .
in addition , nuclear positivity for wilms tumor ( wt-1 ) was seen in about 50% of cells .
the patient was referred to the urology department for the surgical consideration , however , was lost to follow up .
an 18-year - old adolescent male patient was referred recently to our institute from a hospital with complaints of abdominal pain with recurrent fever for the last 4 months .
a clinical possibility of a ruptured liver abscess in the right pleural cavity based on sonographic findings was documented in the records .
the ct showed a large solid - cystic mass lesion with thick enhancing septae filling almost the entire right hemithorax and extending inferiorly , indenting upon the right hemidiaphragm and displacing the liver inferiorly .
the mass was having loss of fat planes at places with the mediastinal structures , including trachea , heart , right innominate artery , and was compressing upon right main bronchus .
overlying ribs and soft tissues were normal with no extrathoracic extension [ figure 2 ] .
axial ct image showing a large heterogenous solid - cystic mass lesion occupying the entire right hemithorax .
no definite rib erosion and extrathoracic extension is seen a percutaneous ultrasound ( us)-guided fna was performed .
the aspirate was particulate and the smears were air - dried as well as alcohol - fixed and stained with may - grnwald giemsa and hematoxylin and eosin , respectively . a cell block was also prepared .
on microscopy , the smears were cellular , and showed sheets of small variably shaped poorly differentiated cells .
the cells were predominantly oval to spindle - shaped and a prominent perivascular arrangement was noted .
the tumor cells had high nuclear : cytoplasmic ratio , finely granular chromatin , inconspicuous nucleoli , and scanty eosinophilic cytoplasm with indistinct cell borders .
immunochemically , the tumor cells showed strong positivity for vimentin and focal positivity for smooth muscle actin [ figure 4 ] .
aspiration cytology of pleuropulmonary blastoma : ( a ) cellular smear with prominent perivascular pattern ( hematoxylin and eosin 200 ) ( b ) small ovoid to spindled cells with occasional cell showing cytoplasmic vacuolation , inset : small cell with cytoplasmic vacuolation [ may - grnwald giemsa ( mgg ) 400 ] ( c and d ) cell block showing oval to spindle - shaped cells with scanty eosinophilic cytoplasm with indistinct borders ( mgg 400 ) case 2 .
immunohistochemistry slides showing strong positivity of tumor cells for vimentin ( a - c ) and weak focal positivity for smooth muscle actin ( d ) the patient developed progressive respiratory dysfunction and died approximately seven days after the diagnosis of tumor .
ppb , which is a rare aggressive malignant primary neoplasm of the pleuropulmonary mesenchyme occurring in early childhood associated with poor prognosis .
. only a few isolated cases of ppbs have been reported in literature in adult population .
table 1 summarizes the key findings of previously published cases in literature in adult population .
there are three pathological types ( type i , ii , and iii ) of ppb based on gross and microscopic features .
the median ages at diagnosis are 10 , 34 , and 44 months for types i , ii , and iii , respectively .
clinicoradiological details of published cases of ppb in adult population the tumor may arise from the lung or it may be extrapulmonary , involving the mediastinum or parietal pleura .
our case showed the tumor filling nearly the entire right hemithorax in the second case , whereas it was central in location in the first case .
it is to be kept in mind that type i ppb can not be differentiated from other benign cystic lung lesions on imaging studies , which include congenital lung cysts such as congenital adenomatoid malformations .
type ii and iii ppb usually present as a large heterogenous masses , which may invade mediastinum , vessels , and diaphragm .
type ii ppb is a solid - cystic tumor , whereas type iii ppb is solid tumor without epithelial - lined cystic spaces . in view of presence of solid - enhancing areas as well as nonenhancing cystic areas , the tumors in our cases were type ii ppb radiologically .
type ii and iii ppb tumors can metastasize to central nervous system , bones , and liver . microscopically , there is presence of primitive blastema and a malignant mesenchymal stroma that often demonstrates heterologous elements such as cartilage differentiation .
blastema components may show numerous mitoses and foci of necrosis , as seen in the resected specimen in the first case .
cystic component is lined by benign respiratory - type epithelium . due to histologic variability in the solid component of the ppb
however , based on the some characteristic features such as cystic architecture , blastemal tissue , and other mesenchymal components including cartilage , ppb can be designated as a distinct entity .
the malignant mesenchymal cells are usually immunoreactive for vimentin , muscle - specific actin and desmin .
the cells were vimentin positive in both of our cases , whereas actin positivity was seen in the second case only .
clinically , the tumor equally affects both genders . in our series , both patients are males .
shortness of breath in the absence of other respiratory tract symptoms is the main symptom in most cases of ppb reported . a suspected pulmonary infection , including an empyema , is the most frequent clinical impression in these patients . our first patient presented with respiratory complaints of nonproductive cough and dyspnea .
however , the second patient presented with abdominal pain with recurrent fever for last 4 months and hence a pulmonary infection was not a clinical consideration .
chemotherapy is based on the institutional practice , which includes either specific chemotherapeutic agents or their combination .
the various agents that have been used include vincristine , actinomycin d , cyclosphosphamide , doxorubicin , ifosfamide , etoposide , cisplatin , carboplatin , epirubicin , methotrexate , and 5-fluorouracil .
however , no specific association has been shown between the survival and the chemotherapy used .
our first patient received cisplatin - paclitaxel combination and showed a complete response ( 100% decrease ) according to response evaluation criteria in solid tumors ( recist ) criteria . types ii and iii ppbs are aggressive tumors and even after a multimodality therapy have a survival rate of 62% at 2 years and 42% at 5 years .
our first patient underwent multimodality treatment and had an overall survival of 27 months ( from the diagnosis to the last contact ) .
however , the second patient died within seven days due to progressive respiratory dysfunction even before the treatment could be started .
ppbs are familial childhood cancer with a high incidence of childhood cancers in close relatives ( 25% ) .
these include ppb , malignant germ cell tumor , medulloblastoma , thyroid neoplasia , and others .
the patients with ppb are also at a higher risk of developing childhood cancers , as seen in the first case in which the patient developed renal wilms tumor during the follow - up period . to conclude , ppbs are primitive dysodontogenic neoplasms of childhood .
 pleuropulmonary blastoma ( ppb ) is a unique dysontogenetic and a primitive neoplasm occuring almost exclusively in the first decade of life , as a pulmonary- and/or pleural - based tumor with cystic , solid , or combined cystic and solid features . 
 it is characterized histologically by a primitive , variably mixed blastematous and sarcomatous tissues . 
 these tumors are usually associated with a poor prognosis . however , with a multimodality treatment approach , the survival of the patient can be prolonged . 
 we herein report two cases of ppb in adolescence , a rare presentation beyond first decade of life with a short review of literature . 
INTRODUCTION
CASE REPORTS
Case 1
Case 2
DISCUSSION