Crystal-storing Histiocytosis in the Stomach: A Clue to Subtle Hematolymphoid Malignancies

Crystal-storing histiocytosis (CSH) is an under-recognized entity with a striking association with lymphoproliferative disorders. To study the typical morphologic features of gastric CSH, all lymphomas diagnosed on in-house gastric specimens at The Ohio State University between January 1, 2008 and January 1, 2017 were retrieved. This search yielded 66 specimens from 51 unique patients. All cases were reviewed with CSH identified in 7 stomach biopsies from 4 patients (2 men:2 women; average age, 69 y; range, 56 to 82 y). Endoscopic findings were all abnormal: diffuse nodularity and white discoloration (n=1), patchy nodularity (n=1), and malignant-appearing fundic mass with lymphadenopathy (n=2). We report the typical gastric CSH lesion displays full-thickness expansion of the lamina propria by a lymphohistiocytic infiltrate that distorts the usual gastric glandular architecture. On high power, all cases were defined by the presence of macrophages with abundant eosinophilic cytoplasm containing nonrefractile, nonpolarizable fibrillary cytoplasmic inclusions. Three of the 4 patients had a kappa-restricted lymphoma; the 1 patient with a lambda-restricted lymphoma had the fewest macrophages. Follow-up data were available up to 228 weeks. All 4 patients had persistent/recurrent lymphoma, and 2 patients died of lymphoma-related complications. None of the CSH cases were prospectively recognized as CSH, and 1 case was initially misdiagnosed as a xanthoma. In summary, CSH is an under-recognized lesion historically associated with lymphoproliferative disorders and we found associated with a high mortality in this small series. Since CSH can be so florid as to obscure the concomitant lymphoma, awareness is crucial for accurate diagnosis.