Progressive Nodular Histiocytosis: Report of a Case and Review of the Literature.

Progressive nodular histiocytosis (PNH) is a rare condition characterized by progressive eruption of multiple yellowish-brown papules and nodules on the skin and mucous membranes. We present the case of a 37-year-old Caucasian man with gradually increased appearance of nodular lesions on the forehead and right temple. These lesions were initially diagnosed as xanthomas and did not respond to intralesional injections of triamcinolone. Additional biopsy revealed an intense dermal infiltrate of foamy mononuclear epithelioid cells with a minor admixture of plasma cells, lymphocytes, and scattered multinucleated giant cells. On immunohistochemical staining, the lesional cells were positive for CD163 and CD68 and negative for CD1a, thus confirming a mononuclear-macrophage lineage. The clinical presentation and the histological impression lead to the diagnosis of PNH. This condition could be challenging, mimicking microscopically similar lesions of the non-Langerhans cell histiocytosis group. Although uncommon, PNH stands out due to its clinical and microscopic features and should be taken into consideration in the differential diagnosis of cutaneous histiocytoses.

Esophageal Granular Cell Tumor: A Benign Tumor or an Insidious Cause for Concern?

BACKGROUND: Esophageal granular cell tumors (GCTs) are rare, often benign tumors of neurogenic origin. GCTs most frequently occur in the skin and subcutaneous tissues but are found in the gastrointestinal (GI) tract in 6%-10% of cases, with the distal two-thirds of the esophagus being the most common site. Owing to the insidious nature of GCTs, presentation is typically asymptomatic. In fact, GCTs are often discovered incidentally during investigation of other GI disturbances. CASE REPORT: We report the case of a 36-year-old white male who had a 2.3 × 2.0-cm submucosal mass of the midesophagus found during esophagogastroduodenoscopy (EGD) at an outside hospital for workup of chronic diarrhea. He was referred to us for further evaluation that led to a diagnosis of a large esophageal GCT. CONCLUSION: Because of the rarity of GCTs in clinical practice and their poorly defined malignant classification, proper workup and management are essential to avoid the potential morbidity and mortality associated with large and/or malignant tumors. Although malignancy is uncommon, approximately 1%-2% of esophageal GCTs are malignant. Conservative management is tolerated for benign, asymptomatic lesions <10 mm in diameter, but endoscopic removal is recommended for large, symptomatic tumors or those with features suggestive of malignancy. Routine surveillance often includes EGD and/or esophageal ultrasonography to evaluate tumor size, location, and depth and to exclude malignancy or lymph node involvement.