Pulmonary interstitial glycogenosis in the setting of lung growth abnormality: radiographic and pathologic correlation.

Pulmonary interstitial glycogenosis (PIG) is a rare pediatric interstitial lung disease. We report a case of a term boy presenting with tachypnea at birth requiring supplemental oxygen. Chest radiographs followed by high-resolution CT (HRCT) demonstrated hyperinflation and diffuse interstitial markings interspersed with multiple cystic spaces. An open lung biopsy demonstrated a minor component of PIG superimposed upon poor alveolarization. PIG in the setting of lung growth abnormality might be more common than previously described. Additionally, radiographic findings associated with most pediatric interstitial lung diseases are nonspecific, and histopathologic correlation is essential for diagnosis.

Embolization of a traumatic uterine arteriovenous malformation.

Uterine arteriovenous malformation (AVM) is a rare but potentially life-threatening source of bleeding. A high index of suspicion and accurate diagnosis of the condition in a timely manor are essential because instrumentation that is often used for other sources of uterine bleeding can lead to massive hemorrhage. Although angiography remains the gold standard for diagnosis, ultrasound (US) and magnetic resonance imaging (MRI) are the modalities of choice for the evaluation of a suspected AVM. US and MRI cannot only accurately define a uterine AVM, but they also have the ability to assess the extent of pelvic involvement noninvasively. The definitive treatment of uterine AVM is hysterectomy. However, most women diagnosed with the condition are of childbearing age. Transcatheter uterine artery embolization offers a safe and effective alternative to surgery, with the major advantage of retaining childbearing capacity.

Consistency of primary brain tumor diagnoses and codes in cancer surveillance systems.

High-quality cancer registry data are essential for assessing trends in incidence rates. This study evaluated the consistency of brain tumor surveillance data using a random sample of cases from the Connecticut Tumor Registry. Three neuropathologists independently and blindly reviewed tumor slides from 204 cases and a nosologist blindly reviewed and assigned International Classification of Diseases for Oncology (ICD-O) codes to 326 cases. For the pathology review, absolute concordance was as high as 81% for all primary brain tumors. Absolute concordance rates were high for nerve sheath (89%), meningioma (95%), and pituitary (95%) tumors. Rates were much lower for malignant tumors. ICD-O coding of malignant brain tumors is of relatively high quality with the exception of mixed gliomas and unspecified tumors. A high level of consistency for nonmalignant brain tumor diagnoses suggests that rates for these tumors, when actively reported to a surveillance system, can be of high quality.