Faculty Bibliography

There is a diverse group of rare, primary benign and malignant ovarian tumors that show characteristic histomorphology and natural history. Some of these tumors may demonstrate typical imaging features. However, due to the rarity of these tumors, imaging characteristics of these diverse lesions are not well described in the literature. Knowledge of these select ovarian entities is essential and facilitates optimal management of patients who often manifest with nonspecific symptoms.

Sclerosing cholangitis can be idiopathic (primary) or secondary to an identifiable cause. Irrespective of cause, sclerosing cholangitis usually progresses to end-stage liver disease and warrants orthotopic liver transplantation. Recent studies provide new insights into the etiopathogenesis, natural history, diagnosis, and management of these different entities.

Hepatocellular adenomas are benign liver neoplasms with specific but varied histopathologic findings and tumor biology. The results from recent studies of the pathologic and genetic basis of hepatocellular adenomas provide important insights into the pathogenesis and molecular changes, as well as the putative oncologic pathways used by diverse adenoma subtypes. On the basis of the genetic and pathologic features, hepatocellular adenomas are categorized into three distinct subtypes: (a) inflammatory hepatocellular adenomas, (b) hepatocyte nuclear factor 1 alpha-mutated hepatocellular adenomas, and (c) beta-catenin-mutated hepatocellular adenomas. Different subtypes show variable clinical behavior, imaging findings, and natural history, and thus the options for treatment and surveillance may vary. Cross-sectional imaging plays an important role in the diagnosis, subtype characterization, identification of complications, and surveillance of hepatocellular adenomas. New schemas for genotype-phenotype classification of hepatic adenomas, as well as management triage of patients with specific subtypes of adenomas, are being proposed in an attempt to improve clinical outcomes.

OBJECTIVE: There is a wide spectrum of nonneoplastic causes of biliary stricture that can pose a significant challenge to clinicians and radiologists. Imaging plays a key role in differentiating benign from malignant strictures, defining the extent, and directing the biopsy. We describe the salient clinical and imaging manifestations of benign biliary strictures that will help radiologists to accurately diagnose these entities. CONCLUSION: Accurate diagnosis and management are based on correlating imaging findings with epidemiologic, clinical, and laboratory data. Cross-sectional imaging modalities permit precise localization of the site and length of the segment involved, thereby serving as a road map to surgery, and permit exclusion of underlying malignancy.

OBJECTIVE: This article provides a comprehensive review of the role of MDCT and MRI in the diagnosis of drug-induced complications in the abdomen and pelvis in adults. A systematic organ-based review of these complications is presented, including but not limited to hepatic changes after chemotherapy, renal complications such as tumor lysis syndrome and lithium nephropathy, gastrointestinal manifestations, various opportunistic infections and secondary neoplasms, mycotic aortic aneurysm from intravesical bacille Calmette-Guerin, complications of anticoagulant therapy, and oral contraceptives. CONCLUSION: Advancements in imaging have led to recognition of radiologic features of previously unsuspected diseases. Occasionally, imaging may also identify effects of treatments instituted for these diseases. Consequently, imaging plays a critical role in the accurate diagnosis of a broad spectrum of drug-induced complications in the abdomen, both in emergent and nonemergent settings. Knowledge of the natural history, clinical manifestations, and salient imaging features of these entities is crucial to facilitate accurate clinical diagnosis in a timely fashion.

Wunderlich syndrome (WS) is a rare condition characterized by acute onset of spontaneous, nontraumatic renal hemorrhage into the subcapsular and perirenal spaces. Wunderlich syndrome is classically characterized by the Lenk's triad: acute flank pain, flank mass, and hypovolemic shock. However, the clinical manifestations can be varied and nonspecific. A wide spectrum of neoplastic and nonneoplastic renal pathologies may result in WS. Renal neoplasms are the most common cause for WS, with angiomyolipoma being the most common benign neoplasm, whereas renal cell carcinoma is the most common malignant neoplasm. Other causative conditions of WS include vascular causes (vasculitis [polyarteritis nodosa being the most common cause], renal artery aneurysms, arteriovenous malformations and fistulas, and venous thrombosis), cystic renal diseases, renal infections, calculus disease, nephritis, and coagulation disorders. Cross-sectional imaging findings help in the detection of the subcapsular and/or perinephric hemorrhage and may identify underlying etiology. Renal angiography not only helps in diagnosis of the underlying cause in select cases but also allows control of active bleeding, which can avoid unnecessary emergent radical surgery.

There is a wide pathological spectrum of kidney sarcomas that show characteristic histology, ontogeny, and clinical-biological behavior. While leiomyosarcomas commonly arise from the capsule, solitary fibrous tumors and clear cell sarcomas typically show renal sinus and medullary epicenter, respectively. Although distribution and imaging findings of some sarcomas may be characteristic, definitive diagnosis warrants histopathological examination following surgery. Renal sarcomas manifest advanced disease at presentation and portend poor prognosis.

Ovarian epithelial carcinoma (OEC), the most common ovarian malignancy, is a heterogeneous disease with several histologic subtypes that show characteristic cytogenetic features, molecular signatures, oncologic signaling pathways, and clinical-biologic behavior. Recent advances in histopathology and cytogenetics have provided insights into pathophysiologic features and natural history of OECs. Several studies have shown that high- or low-grade serous, endometrioid, and clear cell carcinomas are characterized by mutations involving the TP53, K-ras/BRAF, CTNNB1, and PIK3CA genes, respectively. High-grade serous carcinomas, the most common subtype, often manifest with early transcoelomic spread of disease beyond the ovaries, whereas low-grade serous and mucinous carcinomas commonly manifest with early-stage disease, with a resultant excellent prognosis. On the basis of pathogenetic mechanisms, recent findings suggest a dualistic model of ovarian carcinogenesis consisting of types I and II. Type I (low-grade serous, mucinous, and endometrioid) cancers commonly arise from well-described, genetically stable precursor lesions (usually borderline tumors); manifest as large adnexal masses with early-stage disease; and have a relatively indolent clinical course, with an overall good prognosis. In contrast, type II carcinomas (high-grade serous, endometrioid, mixed, and undifferentiated variants) originate de novo from the adnexal epithelia, often demonstrate chromosomal instability, and have aggressive biologic behavior. Better knowledge of hereditary ovarian cancer syndromes and associated cytogenetic abnormalities has led to increased interest in novel biomarkers and molecular therapeutics. Genetic changes, pathologic features, imaging findings, and natural histories of a variety of histologic subtypes of OEC are discussed in this article.

Solitary fibrous tumors (SFTs) are a unique group of mesenchymal neoplasms of fibroblastic or myofibroblastic origin. These tumors were originally described as "benign fibrous mesotheliomas" of the pleural cavity and were erroneously thought to be confined to the serosal surfaces (due to a putative mesothelial or submesothelial origin). It is now established that SFTs are ubiquitous neoplasms with both pleural and extrapleural distribution. Extrapleural SFTs commonly occur in middle-aged adults and manifest as asymptomatic, slow-growing, large tumors. Fewer than 5% of patients with SFTs present with symptomatic hypoglycemia. SFTs are histopathologically diverse with a variable admixture of fibroblasts or myofibroblasts, numerous thin-walled vessels, and dense fibrosis. Tumors previously categorized as hemangiopericytomas are now considered cellular variants of SFTs. At imaging, SFTs demonstrate remarkable heterogeneity, with variable degrees of enhancement, necrosis, or hemorrhage. Although most extrapleural SFTs have a benign clinical course, 10%-15% of these tumors demonstrate aggressive behavior in the form of recurrence or malignancy.