Synovial Cell Sarcoma of the Larynx
Synovial cell sarcoma represents a rare group of cancers, particularly in the head and neck region, that typically affects young individuals and has a male preponderance. Prognosis varies with patient…
Esthesioneuroblastoma
Esthesioneuroblastoma (ENB) is a rare malignancy, representing only 3% to 6% of all sinonasal malignancies. A wide array of treatment options for ENB have been described in the literature, but…
Parathyroid Carcinoma
Parathyroid carcinoma is a malignant neoplasm affecting 0.5% to 5.0% of all patients with primary hyperparathyroidism. Since it was first described by De Quervain in 1904 to this day, it…
New Insights into the Pathogenesis, Diagnosis, and Management of Mastocytosis
This review describes developments in understanding normal mast cell function and genetic changes that predispose to malignant transformation to mastocytosis. Most mastocytosis cases are associated with somatically acquired activating mutations…
Epidemiology and Management of Uveal Melanoma
Uveal melanoma is the most common primary intraocular malignancy in adults. The disease overwhelmingly affects white populations. Other risk factors include fair skin, light iris color, ancestry from northern latitudes,…
What is a Rare Cancer?
Many rare cancers are essentially an enigma, with little to no information in the medical literature. Defining a rare cancer is not as easy as it might seem. As a…
Animal Models of Human Granulocyte Diseases
In vivo animal models have proven very useful to the understanding of basic biologic pathways of the immune system, a prerequisite for the development of innovate therapies. This article addresses…
Clinical and Molecular Pathophysiology of Shwachman–Diamond Syndrome
Shwachman–Diamond syndrome (SDS) is an inherited neutropenia syndrome associated with a significant risk of aplastic anemia and malignant transformation. Multiple additional organ systems, including the pancreas, liver, and skeletal and…
Leukocyte Adhesion Deficiencies
During inflammation, leukocytes play a key role in maintaining tissue homeostasis through elimination of pathogens and removal of damaged tissue. Leukocytes migrate to the site of inflammation by crawling over…
Chronic Granulomatous Disease
Chronic granulomatous disease (CGD) is a paradigm for nonlymphoid primary immune defects, and has guided elucidation of oxygen metabolism in the phagocyte, vasculature, and brain. It has been in the…
