Recommendations for Managing Adults With Histiocytic Neoplasms: New NCCN Guidelines

The histiocytoses, a group of clonal and reactive conditions, arise from monocytic macrophage or dendritic cell lineages. The current NCCN Clinical Practice Guidelines in Oncology (NCCN Guidelines) for Histiocytic Neoplasms reflect the most up-to-date, evidence-based data relating to the evaluation and management of this disease. Specifically, the guidelines focus on adult Langerhans cell histiocytosis, Erdheim-Chester disease, and Rosai-Dorfman disease. Because these disorders are rare, challenges have arisen regarding clinical suspicion, histologic diagnosis, treatment, and molecular subtyping. Future versions of the NCCN Guidelines will address the diagnosis and management of pediatric patients, as well as malignant histiocytosis.

Rosai-Dorfman Disease: A Case Report of Asymptomatic Isolated Renal Involvement

Rosai-Dorfman disease (RDD) is a rare non-malignant histiocytosis disorder, commonly manifesting with massive painless cervical lymphadenopathy. Renal involvement develops in only four percent of patients with RDD. Generally, RDD is self-limiting and has a good prognosis; however, in patients with renal involvement, mortality rate can be as high as 40%.

Malignant histiocytosis of lungs and chest wall: A case report of diagnostic conundrum and treatment challenges in a resource-poor setting

Malignant histiocytosis is a rare condition with variable clinical presentation and prognosis. Its diagnosis requires high index of suspicion and further evaluation. Thus, it poses a diagnostic as well as management challenges especially in resource poor setting. The objective is to highlight the diagnosis and treatment challenges in the management of malignant histiocytosis so that physicians can diagnose it early and initiate appropriate treatment. Our patient is a 35-year-old man who presented with chest wall mass, dyspnoea and right cervical lymphadenopathy He was initially being evaluated for lymphoma. Biopsy of the lymph nodes was done twice in different hospitals and that of the chest wall mass done once. Histological diagnosis was arrived at with the aid of immunohistochemistry three months after his first presentation to the hospital. The patient was then commenced on chemotherapy and showed remarkable improvement on the short-term basis. Malignant histiocytosis is a rare disease laden with management challenges. Despite that, its diagnosis and treatment can be achieved even in resource poor setting with high index of suspicion, team work and good laboratory support.