intralobar pulmonary sequestration ( ilps ) with an anomalous systemic arterial blood supply represents a mass of nonfunctioning parenchyma surrounded by visceral pleura ( 1 , 2 ) . unlike extralobar sequestration ,
radiographically demonstrated calcification in the ilps has been reported in some case series , but primary sequestration associated with typical hamartoma signs is really rare and has been described only once ( 3 , 4 ) . in this report , we present a rare case of giant hamartoma originated from primary ilps in a 39-year - old woman who had intracranial cholesteatoma .
a 39-year - old woman with an abnormal mass in the right cerebellopontine angle was referred to neurosurgery department of our hospital .
a giant mass in the right thoracic cavity was detected accidentally by x - ray in a routine preoperative screening process .
subsequent thoracic computed tomography scan demonstrated a complex lesion ( size 15.1  12.3  15.7 cm ) occupying more than two - thirds of the thoracic cavity on the right side .
the blood supply to the area of the lesion was from a systemic artery arising from the abdominal aorta , with venous drainage through the inferior pulmonary vein , suggesting an ilps ( figure 1 a - 1c ) .
three months later , an open thoracic surgery for the intrathoracic lesion was performed under general anesthesia .
intraoperatively , lesion appeared hypervascular , because of abundant systemic vascularization ( figure 2 a ) .
the massive lesion was compressing the right lung and there was no communication between the tracheobronchial tree and sequestered lesion .
based on the findings of 3d ct angiography , we explored the aberrant artery at the bottom of the chest and carefully ligated it with an absorbable silk suture .
after the pulmonary ligament had been completely divided , the lower pulmonary vein was found to be widened .
the lesion was adherent to adjacent pulmonary vein branches and was not easily dissected . to avoid major intraoperative blood loss ,
pathologic analysis showed a predominant fatty differentiation with fibroblastic , chondroid and osseous differentiation making the rest ( figure 2b - 2d ) .
the postoperative recovery was uneventful and the patient was discharged from the hospital on the eighth postoperative day .
ilps is usually diagnosed later than extralobar sequestration ( els ) , being found in childhood or adulthood when the patient presents with an infection ( 5 ) . up to 15% of patients , particularly in adult population , are asymptomatic when the sequestration is discovered incidentally ( 6 ) . in our case , the sequestration surrounded by visceral pleura , characteristically receives its blood supply from a systemic artery with venous drainage through pulmonary veins and was consistent with ilps .
the lesions of ilps may be solid , fluid or hemorrhagic or may contain mucus . primary sequestration associated with typical hamartoma signs
final histology was pulmonary hamartoma with predominantly adipose and cartilage differentiation , which is an unusual complication originated from primary sequestration .
pulmonary hamartoma is a rare lung neoformation , usually symptomless and discovered incidentally , of a probable dysontogenetic origin with prevailing cartilaginous tissue and adult onset age ( 7 ) . in a series of 24 patients , gabrail and zara (
8) concluded that pulmonary hamartomas are frequently accompanied by other developmental abnormalities and benign tumors .
however , the entity described ( pulmonary hamartoma syndrome ) seems to be distinct from well - established multiple hamartoma syndrome ( cowden s syndrome ) .
many interesting features characterize the case report : histopathological nature of the pulmonary hamartoma , the typical vascular structure related to intrapulmonary sequestration and intracranial cholesteatoma as shown by neurosurgery operation .
. the combination of sequestration , hamartoma and cholesteatoma may be only an incidental combination , but this could indicate both possibilities as hamartomatosis change on underlying sequestration or sequestration associated with hamartomatosis syndrome .
 the radiologic features of intralobar pulmonary sequestration ( ilps ) have been describe and include the identification of a feeding systemic artery with venous drainage through pulmonary veins . 
 primary sequestration associated with typical hamartoma signs is really rare and has been described only once . 
 we describe a patient with ilps whose radiographic findings were unusual for two reasons . 
 first , computed tomography ( ct ) demonstrated a bulky mass in the pulmonary sequestration . 
 the size of lesion and histopathology made it an unusual presentation . 
 final histology study demonstrated pulmonary hamartoma with predominantly adipose and cartilage differentiation , which is an unusual complication originated from ilps . 
 another sign also explains the second unusual feature , intracranial cholesteatoma , occurring concurrently with ilps . 
1. Introduction
2. Case Presentation
3. Discussion