Malignant spindle cell tumour (fibrosarcoma) in sequestration of lung—a case report

Pulmonary sequestration is an uncommon congenital anomaly of respiratory system and usually diagnosed in childhood but can present at any at age. It frequently presents as a mediastinal mass with symptoms from vascular shunting, or associated anatomic defects. This case report describes an adult who presented with chest pain due to extralobar sequestration of right lung extending into the mediastinum. Per-operatively it was found to be an extra pulmonary mass lesion with a peduncle attached to right lung suggestive of extralobar sequestration. Histopathological examination of the specimen showed it to be spindle cell tumor (fibrosarcoma) in sequestrated lung. The patient had an uneventful recovery and is under regular follow up without any further problems.     

Intraabdominal pulmonary sequestration

Bronchopulmonary sequestration is an uncommon developmental abnormality that frequently presents as an incidental mass. Differential diagnosis includes malignancy. Reports of extralobar abdominal pulmonary sequestration in adults are sparse. Only 2.5% of all pulmonary sequestrations are detected below the diaphragm. The authors present a case of a 45-year-old woman and a literature review. Computed tomography, magnetic resonance imaging, and operative and pathologic findings are provided. A review of diagnosis and therapy is presented.     

Pulmonary sequestration in older child and in adults.

Pulmonary sequestration is often found in children with recurrent pneumonia, but is not rare in adults. During the last 6 years (1985-1990), 6 patients with pulmonary sequestration were surgically treated. One 40-year-old patient had an extralobar sequestration, and 5 patients ranging in age from 14 to 26 years had an intralobar sequestration. The patient with extralobar sequestration was asymptomatic. In cases of intralobar sequestration, 4 patients were symptomatic. All 6 patients had an abnormal chest roentgenogram. Preoperative arteriography revealed anomalous blood supply came from a systemic artery in 4 patients. All cases with intralobar sequestration had a lower lobectomy, and one case with extralobar sequestration had an extirpation. Even in cases without any symptoms, there was the possibility of a microscopic infection in the sequestrated lesion with the occurrence of later symptoms. Therefore, patients diagnosed with pulmonary sequestration should be considered for surgical resection.     

Infarction of extralobar pulmonary sequestration after blunt trauma

Pulmonary sequestration is a rare congenital lung malformation that occurs in either an intralobar or extralobar location and is generally asymptomatic. We report an unusual case of a child who presented with infarction of a previously asymptomatic extralobar pulmonary sequestration after blunt trauma, eventually requiring surgical excision.     

Pulmonary sequestration.

Pulmonary sequestration is a congenital anomaly in which an aberrant systemic artery arising from the thoracic or upper abdominal aorta supplies part of the lungs, usually the lower lobe. The sequestered lung may be anatomically distinct from the remainder of the lobe (extralobar), or may be included in the substance of the lobe, in which case it may or may not have bronchial communication with the rest of the bronchial tree. The patients present, often in the first two decades of life, with recurrent and severe bronchopulmonary infections. Associated anomalies are present, especially in the extralobar variety. Nine cases of sequestration are reviewed, stressing significant clinical, radiological, and arteriographic findings. Preoperative demonstration of the anomalous vessel by aortography has contributed significantly to the planning and safety of the surgical procedure, which was generally a lower lobectomy. Eight out of nine patients survived the procedure.     

Pulmonary sequestration

The author discusses pulmonary sequestration which is a rare developmental anomaly difficult for diagnosis. Eleven patients were under observation and underwent operation for pulmonary sequestration (8 with intralobar and 3 with extralobar) which accounted for 2.4% of patients with congenital developmental anomalies of the lungs and for 0.48% of those with chronic unspecific disease of the lung. Complete clinical radiological examination, including aortography, made it possible to recognize the anomaly before the operation in 6 patients. In the remaining patients sequestration of the pulmonary tissue was an operative finding. Preoperative functional study of pulmonary circulation revealed a marked left-to-right shunt of the blood, increased oxygenation of blood in the superior vena cava and saturation of arterial and venous blood with oxygen (to 94 and 91%, respectively, on the average). Analysis of clinical, radiological, and morphological data allowed three main forms of pulmonary sequestration to be distinguished: cystic-abscessed, pseudotumorous, and fibrous-atelectatic.     

Extralobar pulmonary sequestration

Extralobar sequestration of pulmonary tissues is relatively rare and seldom produces symptoms. An incidental finding is described of extralobar sequestration of the lung in a Nigerian child, presenting as an anterior mediastinal polycystic mass. The mass was attached to the right pulmonary artery by a fibrovascular pedicle which contained a small elastic artery, veins, and nerve bundles. Sequestrated lobes are usually situated in the posterior basal lobes of the lungs and may be associated with other congenital abnormalities, but the case reported here differs in these respects. Death was due to enterocolitis unrelated to the congenital abnormality.     

Thoracoscopic resection of an apical extralobar pulmonary sequestration in an infant

Pulmonary sequestration is a form of bronchopulmonary-foregut malformation that is treated with surgical resection, either via a thoracotomy or the thoracoscopic approach. Apical extralobar pulmonary sequestrations are rare. We report a case of an apical pulmonary sequestration in an infant that was amenable to thoracoscopic resection. An 8-month-old girl, weighing 10.3 kg, with a left apical extralobar pulmonary sequestration underwent thoracoscopic resection. The pulmonary sequestration was identified and dissected free from surrounding tissue. The vascular peduncle was secured with an endo-GIA loaded with 2-mm vascular staples. The infant was discharged on postoperative day two without complications. In spite of the patient's small size and the apical nature of the sequestration, the operation was easily performed via a thoracoscopic approach. Thoracoscopic resection of an apical extrapulmonary sequestration in a small infant is feasible and may minimize the morbidity of this procedure.     

Upper thoracic extralobar pulmonary sequestration with anomalous blood supply from the subclavian artery

The authors report on a newborn boy with extralobar pulmonary sequestration in the right upper thoracic region. Preoperative angiography showed an anomalous large vessel from the right subclavian artery, supplying the sequestrated lobe. Right thoracotomy was performed to resect the sequestrated lobe, which was diagnosed as extralobar form. This is an extremely rare case of extralobar pulmonary sequestration in which anomalous blood supply from the subclavian artery was seen preoperatively on radiographs. The authors recommend angiographic examination, particularly in case of diagnostic difficulty.     

Lung sequestration: report of seven cases and review of 540 published cases

We present an analysis of 400 intralobar and 133 extralobar sequestrations, six cases presenting intra- and extralobar sequestration simultaneously, five sequestrations of one whole lung, and two bilateral intralobar sequestrations.     

Spectrum of Pulmonary Sequestration

Bronchopulmonary sequestration was diagnosed in 17 patients ranging in age from newborn to 64 years. The sequestration was intralobar in 14 patients and extralobar in 3. The spectrum of symptoms could be divided into three patterns: no symptoms (6 patients), respiratory problems (8 patients), and cardiovascular problems (3 patients). Cardiovascular problems usually manifest themselves in the first few weeks or months of life and often have a respiratory component. In older patients the sequestration is first manifested by recurrent pulmonary infections or, if it remains uninfected, an asymptomatic density on chest roentgenogram.The definitive diagnostic study is arteriography. Operative treatment for the intralobar variety consists of segmental resection or, if the inflammatory process is more extensive, lobectomy. An extralobar sequestration may simply be excised.     

Laparoscopic removal of a retroperitoneal extralobar pulmonary sequestration in an adult

A 41-year-old male patient presented with dull left flank pain. A computed tomography (CT) showed a subdiaphragmatic retroperitoneal extra-adrenal mass. A magnetic resonance image demonstrated it was hypointense on T1 weighted and hyperintense on T2. Serum analyses for adrenal hormones revealed no abnormality. [I131]metaiodo-benzylguanidene scintigraphy showed no abnormal uptake. The patient underwent laparoscopic removal to manage the symptoms and to make a pathological diagnosis, which was an extralobar pulmonary sequestration. Another congenital lung malformation was not detected on chest CT scan. The symptom was relieved postoperatively.     

Intrapericardial extralobar pulmonary sequestration detected as an intrathoracic cystic mass by using prenatal ultrasonography: Case report and review of the literature

Intrapericardial extralobar pulmonary sequestration is a very rare congenital lung anomaly. We report a case of this condition, detected as an intrathoracic cystic lesion by using prenatal ultrasonography. The neonate was born at 38 weeks of gestation with no progression of the lesion and no respiratory or cardiac symptoms. Ultrasonography and computed tomography (CT) revealed a 40 × 17 × 17-mm intrapericardial lesion, composed of cystic components and a solid component. Intrapericardial extrapulmonary sequestration was suspected largely because CT showed a vague aberrant artery. At the age of 3 months, elective surgery was performed, and the postoperative course was uneventful.     

Management of antenatally diagnosed pulmonary sequestration associated with congenital cystic adenomatoid malformation

BACKGROUND: Sequestration with associated cystic adenomatoid malformation is rare. A study was undertaken to determine whether pulmonary sequestration associated with congenital cystic adenomatoid malformation has a more favourable natural history than that of sequestration without associated cystic adenomatoid malformation. METHODS: An outline of the postnatal work up leading to the management of extralobar or intralobar pulmonary sequestration with congenital cystic adenomatoid malformation diagnosed antenatally as pulmonary malformation is presented and the indications for surgical intervention are discussed. RESULTS: In five infants in whom an antenatal ultrasound scan had detected a congenital lung malformation at 18-19 weeks gestation a final diagnosis of extralobar or intralobar pulmonary sequestration with congenital cystic adenomatoid malformation was made postnatally. Postnatal ultrasound and computerised axial tomographic scans confirmed the diagnosis of sequestration by delineating anomalous vascular supply. Cystic changes were also observed in the basal area of the sequestration in all patients. Four children remained asymptomatic and one infant presented at 10 months of age with pneumonia. The mean age at surgical resection was 6.8 months (range 2-10). Histopathological examination confirmed intralobar pulmonary sequestration with associated Stocker type 2 congenital cystic adenomatoid malformation in two patients and extralobar pulmonary sequestration with associated Stocker type 2 congenital cystic adenomatoid malformation in three patients. The mean period of follow up was four years (range 1-8). The children remain well and are developing normally. CONCLUSIONS: The importance of seeking an anomalous blood supply in children with congenital lung lesions is emphasised. Pulmonary sequestration and congenital cystic adenomatoid malformation probably share a common embryogenesis despite diverse morphology. The natural history of antenatally diagnosed lung masses is variable. Early postnatal surgical resection of pulmonary sequestration with cystic adenomatoid malformation is recommended. Surgical excision should be conservative, sparing the normal lung parenchyma.     

Extralobar pulmonary sequestration mimicking neuroblastoma

Extralobar pulmonary sequestration is a congenital pulmonary malformation, which rarely may be present in an intra-abdominal location. We describe a 1-week-old newborn with an intra-abdominal sequestration, which presented to us as an antenatally diagnosed suprarenal mass. Intra-abdominal extralobar sequestration should also be kept in differential diagnosis in cases of masses in the suprarenal location.     

Video-Assisted Thoracic Surgery Lobectomy for Extralobar Pulmonary Sequestration in a Child: Report of a Case

Pulmonary sequestration in infants and children is conventionally treated by resecting the sequestered lung parenchyma (sequestrectomy) or by performing lobectomy through a standard thoracotomy. We performed lobectomy by video-assisted thoracic surgery, using an original tracheal tube that we designed, in a 6-year-old boy with extralobar pulmonary sequestration and bronchiectasis in the left lower lobe.     

Congenital cystic adenomatoid malformation associated with pulmonary sequestration

The association of congenital cystic adenomatoid malformation with pulmonary sequestration is infrequent. Has been reported less than 60 cases. We describe two patients with this association. One of this with extralobar pulmonary sequestration, and the other with an intralobar one.     

Late revelation of a subphrenic extralobar pulmonary sequestration as a suprarenal mass

Pulmonary sequestrations are some rare congenital anomalies. The incidence was estimated of 0.15% to 1.7%. They are characterized by a mass of non functioning pulmonary tissue that has no communication to the normal bronchial tree. The vascularisation is supplied by systemic arteries. They are classified further as intralobar and extralobar types. Extralobar sequestration, so-called accessory lung, is separated from the normal lung. We present a rare case of subphrenic extralobar pulmonary sequestration in a 57 years old patient. The lesion was initially presented as a non-typical suprarenal mass discovered on CT scan. The approach by laparatomy permitted the resection and the definitive diagnosis.     

Communicating bronchopulmonary foregut malformation involving a mixed sequestration/cystic adenomatoid malformation: a case report

The authors report the case of a baby girl with an unusual communicating bronchopulmonary foregut malformation consisting of extralobar pulmonary sequestration and cystic adenomatoid malformation. A well-formed bronchus was the communication between the sequestration and lower esophagus.     

Extralobar pulmonary sequestration with aberrant lobulation of the lower lobe; report of a case

An adult case of extralobar pulmonary sequestration with aberrant lobulation of the right lower lobe is reported. A 32-year-old woman admitted to our hospital with a right intrathoracic tumor in the chest computed tomography (CT). Chest CT showed a mass lesion on the right diaphragm of a diameter 5 cm and abnormal fissure of the right lower lobe. Under the diagnosis of pulmonary sequestration without clearly thick vessels, video-assisted thoracoscopic surgery was performed. The mass existed between the right diaphragm and posterior lower lobe with a thin stalk, and the lower lobe had aberrant lobulation. The resected mass was histopathologically diagnosed as extralobar pulmonary sequestration.