Revisit on congenital bronchopulmonary vascular malformations: a haphazard branching theory of malinosculations and its clinical classification and implication.

We propose a haphazard branching theory to support the concept of bronchopulmonary malinosculations, and we apply this theory to classify congenital bronchopulmonary vascular malformation (BPVM) based on the anatomical results we have found. Between January 1990 and December 1997, a total of 22 pediatric patients (10 male and 12 female), aged 2 days to 14 years (median, 19.6 months), with congenital BPVM were enrolled in this retrospective study. Study modalities include the clinical features and plain chest films (n = 22) plus at least two of the following: echocardiography (n = 13), barium esophagraphy (n = 2), bronchoscopy (n = 4), contrast bronchography (n = 8), high-resolution direct coronal CT (n = 1) and electron beam or ultrafast CT (n = 1) of the chest, MRI (n = 10), MRA (n = 1), contrast cineangiocardiography (n = 9), surgery (n = 11), or autopsy (n = 2). The salient clinical features were recurrent lung infections in 14 patients, acute respiratory distress in 13, associated cardiovascular malformations in 8, dextroversion in 7, congestive heart failure in 7, dextrocardia in 4, and complex congenital heart diseases in 4. There were abnormal openings (malinosculations) of the pulmonary airway in 20 patients: to an artery in 12, to a vein in 8, and to the lung parenchyma in 9. These 22 patients with congenital BPVM can be classified into bronchial malinosculation (10 cases), arterial malinosculation (2 cases), and bronchoarterial malinosculation (10 cases). Congenital BPVM can be classified in terms of bronchopulmonary malinosculation based upon a haphazard branching theory, in which abnormal communications between two independent systems (primitive foregut system and aortic-pulmonary arch system) occurred coincidentally rather than causally.     

Right pneumonectomy syndrome: Report of two cases

We report herein the cases of two infants who developed right pneumonectomy syndrome, both of whom were born with gross C-type esophageal atresia (EA/TEF), and a hypoplastic right lung arising from the lower esophagus, being a bronchopulmonary foregut malformation (BPFM). Appropriate and well-timed treatments for a variety of sequelae primarily caused by the mediastinal shift must be considered after right pneumonectomy in early childhood.     

Ciliated hepatic cyst leading to squamous cell carcinoma of the liver – A case report and review of the literature

INTRODUCTIONCiliated hepatic foregut cysts (CHFC) are rare, typically benign liver lesions. Primary squamous cell carcinoma (SCC) of the liver is also a rare entity with only approximately 25 reported cases in the literature. Recently, there have been four reports of malignant transformation of CHFC into primary squamous cell carcinoma of the liver. Here we report a fifth with unique presentation and review the literature.PRESENTATION OF CASEA 34 year-old man, with a history of ulcerative colitis, was incidentally found to have a 10 cm lesion in the right anterior sector plus left medial section of the liver on computerized tomography (CT) scan. The patient was asymptomatic at presentation and neoplastic markers were not elevated. Sequential transarterial chemoembolization (TACE) and portal vein embolization (PVE) allowed for left lateral section plus segment 1 hypertrophy and subsequent resection. Histology later revealed the cyst to be a CHFC and showed its malignant transformation. At 6 month follow-up, the patient has lung and abdominal recurrence.DISCUSSIONWith now the fifth case of malignant transformation of CHFC being reported, approximately 5% of all reported CHFC have undergone malignant transformation. This frequency, taken together with the aggressive disease course and poor prognosis, suggests that CHFC must not be presumed benign and should be regarded with clinical suspicion.CONCLUSIONAccurate diagnosis of CHFC is mandatory given its potential malignant transformation. Even in asymptomatic CHFC, surgical excision is recommended. In addition, in cases of otherwise unresectable lesions, sequential TACE and PVE may provide optimal hypertrophy of future liver remnant.     

Ciliated hepatic foregut cyst in a young child

Ciliated hepatic foregut cysts are a rare entity usually found in adults. We present a case of a 3-year-old boy incidentally noted to have a radiographically complex liver cyst on computed tomographic scan. Given the complex appearance, the cyst was excised. Pathology revealed a ciliated hepatic foregut cyst. This is the second child and youngest patient affected with this lesion reported in the literature. The etiology of the lesion and an argument for surgical removal in pediatric patients are presented.     

Foregut duplication cyst of the stomach

Foregut duplication cyst of the stomach is an extremely rare disease entity. A 35-year-old Korean man presented with epigastric pain. An abdominal cystic mass, measuring 7 x 6 x 5 cm, was found in the lesser curvature of the stomach. The cyst was unilocular with a grey-white, rubbery wall. Microscopically, the cyst wall was lined by pseudostratified ciliated, columnar epithelium and gastric mucosa with a complete lining of smooth muscle bundles. Although the origin of this lesion remains uncertain, this case suggests that the gastric cyst arose from the embryonic foregut and showed differentiation toward respiratory and gastric structures.     

Esophageal duplication cyst causing unilateral hyperinflation of the lung in a neonate

Esophageal duplication cysts are rare congenital anomalies. Frequently asymptomatic, they may cause respiratory distress and feeding difficulties in infants. Unilateral hyperinflation of the lung due to compression of the bronchus by the cyst is rare. We report a case of a 4-day-old male neonate presenting with respiratory distress who had an esophageal duplication cyst causing obstructive hyperinflation of the right lung. The nature of the cyst was confirmed after surgery.     

Criteria for malignancy in gastrointestinal endocrine tumors

In contrast with the large amount of data generated from endocrine tumors of the pancreas, sparse and mostly unconfirmed data are available on the criteria for the assessment of malignancy risk and patient outcome in endocrine tumors of the gastrointestinal tract. In these conditions the 2000 WHO classification with its standardized scheme of pathologic report constitutes a framework facilitating the assessment of tumor malignancy and has been regarded as useful for clinical purposes, providing the basis for proper management of the patients and for the design of treatment protocols. The classification is based on a combination of pathological and clinical features with parameters specific for each organ in which the endocrine tumors originate. Three main categories, one further subdivided into two subgroups, are considered: (1) well-differentiated endocrine tumors, further subdivided into tumors with benign and with uncertain behavior; (2) well-differentiated endocrine carcinomas, low grade; and (3) poorly differentiated endocrine carcinomas, high grade. In this review the differential tumor characteristics between the different categories are summarized. Moreover, the relevance of additional features with respect to tumor prognostication, chiefly the Ki-67 proliferation index and malignancy-associated genetic changes, is discussed with emphasis on the discrepancies emerging between tumors of foregut and of midgut origin.     

Subphrenic bronchopulmonary foregut malformation with pulmonary-sequestration-like features

A retroperitoneal bronchopulmonary foregut malformation in a 62-year-old man is reported. The lesion was composed of mature lung tissue with randomly distributed bronchial structures and ciliated epithelium-lined cysts, some of which were lined with gastric mucosa. The histological features of this lesion were of both pulmonary sequestration and a bronchogenic, or foregut, cyst, and thus were a unique example of bronchopulmonary foregut malformation with pulmonary differentiation. This case is important in understanding the pathogenesis of foregut anomalies (i.e. bronchopulmonary foregut malformations), which range from pulmonary sequestrations to bronchogenic cysts and foregut duplication cysts.