The Peutz-Jeghers Syndrome (PJS) is an autosomal dominant neoplastic syndrome defined by hamartomatous polyps through the gastrointestinal tract, development of characteristic mucocutaneous pigmentations, and an elevated lifetime cancer risk. The majority of cases are due to a mutation in the STK11 gene located at 19p13.3. The estimated incidence of PJS ranges from 1:50,000 to 1:200,000. PJS carries an elevated risk of malignancies including gastrointestinal, breast, lung, and genitourinary (GU) neoplasms. Patients with PJS are at a 15- to 18-fold increased malignancy risk relative to the general population. Radiologists have an integral role in the diagnosis of these patients. Various imaging modalities are used to screen for malignancies and complications associated with PJS. Awareness of various PJS imaging patterns, associated malignancies, and their complications is crucial for accurate imaging interpretation and patient management. In this manuscript, we provide a comprehensive overview of PJS, associated malignancies, and surveillance protocols.
Mastocytosis is a rare disorder due to the abnormal proliferation of clonal mast cells. Mast cells exist in most tissues, mature in situ from hematopoietic stem cells and develop unique characteristics of local effector cells. Mastocytosis develops by activation mutation of the KIT surface receptor which is involved in the proliferation of a number of cell lines such as mast cells, germ cells, melanocytes, and hematopoietic cells. It manifests as two main categories: cutaneous mastocytosis and systemic mastocytosis. Imaging can play an important role in detection and characterization of the disease manifestation, not only by radiography and bone scans, but also magnetic resonance imaging and computed tomography, which can be more sensitive in the assessment of distinctive disease patterns. Radiologists should be aware of various appearances of this disease to better facilitate diagnosis and patient management. Accordingly, this review will discuss the clinical presentation, pathophysiology, and role of imaging in detection and extent estimation of the systemic involvement of the disease, in addition to demonstration of appearance on varying imaging modalities. Familiarity with the potential imaging findings associated with mastocytosis can aid in early disease diagnosis and classification and accordingly can lead directing further work up and better management.
The lymphatic system is an anatomically complex vascular network that is responsible for interstitial fluid homeostasis, transport of large interstitial particles and cells, immunity, and lipid absorption in the gastrointestinal tract. This network of specially adapted vessels and lymphoid tissue provides a major pathway for metastatic spread. Many malignancies produce vascular endothelial factors that induce tumoral and peritumoral lymphangiogenesis, increasing the likelihood for lymphatic spread. Radiologic evaluation for disease staging is the cornerstone of oncologic patient treatment and management. Multiple imaging modalities are available to access both local and distant metastasis. In this manuscript, we review the anatomy, physiology, and imaging of the lymphatic system.
Gastrointestinal bleeding (GIB) among cancer patients is a major source of morbidity and mortality. Although a wide variety of etiologies contribute to GIB, special considerations should be made for cancer-related factors such as the type of malignancy, location and extent of disease, hemostatic parameters, and treatment effects. Key imaging modalities used to evaluate GIB include computed tomography angiography (CTA), radionuclide imaging, and catheter-based angiography. Understanding the cancer and treatment history and recognizing the associated imaging manifestations are important for identifying the source and potential causes of GIB in cancer patients. This article will review the common clinical presentations, causes, imaging manifestations, and angiographic management of GIB in cancer patients.