ABSTRACT
Rosai-Dorfman disease is an idiopathic histiocytic disorder of lymph nodes and extranodal sites. Central nervous system manifestations of this disease are rare, and to our knowledge only 16 cases of intracranial involvement have been reported previously. Intracranial Rosai-Dorfman disease clinically and radiologically resembles meningioma, and histologic examination is essential for a definitive diagnosis. We report two cases of isolated, intracranial, dural-based Rosai-Dorfman disease, review the literature, and discuss the differential diagnoses of this lesion.
Subject(s)
Dura Mater/pathology , Histiocytosis, Sinus/pathology , Adult , Diagnosis, Differential , Dura Mater/chemistry , Dura Mater/ultrastructure , Histiocytes/pathology , Histiocytosis, Sinus/diagnostic imaging , Histiocytosis, Sinus/immunology , Humans , Immunohistochemistry , Male , Meningioma/diagnosis , Microscopy, Electron , S100 Proteins/analysis , Tomography, X-Ray ComputedABSTRACT
OBJECTIVE AND IMPORTANCE: Granulocytic sarcomas involving the spine in patients without myelogenous leukemia are rare. We report three cases and review the literature. CLINICAL PRESENTATION: Three patients presented with spinal epidural tumors, which caused spinal cord compression in one and cauda equina compression in two. INTERVENTION: All patients underwent surgery, and biopsies revealed histological features of granulocytic sarcomas. Bone marrow aspirates and biopsies showed no evidence of acute leukemia at initial presentation, for all three patients. CONCLUSION: Granulocytic sarcomas in nonleukemic patients are rare, and when they affect the spine they are frequently misdiagnosed. Appropriate therapy for these tumors requires early identification.
Subject(s)
Cauda Equina/surgery , Epidural Neoplasms/surgery , Leukemia, Myeloid/surgery , Nerve Compression Syndromes/surgery , Spinal Cord Compression/surgery , Adolescent , Adult , Bone Marrow/pathology , Bone Marrow Transplantation , Cauda Equina/pathology , Chemotherapy, Adjuvant , Combined Modality Therapy , Diagnosis, Differential , Epidural Neoplasms/diagnosis , Epidural Neoplasms/pathology , Fatal Outcome , Granulocytes/pathology , Humans , Leukemia, Myeloid/diagnosis , Leukemia, Myeloid/pathology , Magnetic Resonance Imaging , Male , Middle Aged , Neoplasm Recurrence, Local/diagnosis , Neoplasm Recurrence, Local/pathology , Neoplasm Recurrence, Local/surgery , Nerve Compression Syndromes/diagnosis , Nerve Compression Syndromes/pathology , Radiotherapy, Adjuvant , Spinal Cord Compression/diagnosis , Spinal Cord Compression/pathology , Tomography, X-Ray ComputedABSTRACT
Gastrocolic fistula in primary non-Hodgkin's lymphoma (NHL) of the stomach is rare; in a review of the literature we found only four cases, all in association with disseminated (stage IV) disease. We describe the first case of a gastrocolic fistula in a patient with stage IE lymphoma. The diagnosis was suggested by feculent vomiting, and the fistula was located using barium enema and CT scan. Therapy consisted of local resection followed by combination chemotherapy.
Subject(s)
Colonic Diseases/etiology , Gastric Fistula/etiology , Intestinal Fistula/etiology , Lymphoma, Non-Hodgkin/complications , Stomach Neoplasms/complications , Aged , Humans , Male , Stomach Neoplasms/pathology , Stomach Neoplasms/therapyABSTRACT
Eighteen patients with spinal cord compression caused by previously undiagnosed lymphoma were treated at our institution between 1976 and 1991. There were 14 male and 4 female patients (mean age, 58.2 years). The absence of bony involvement on radiographic images was a feature in 16 of the cases. All patients underwent laminectomy for decompression and tissue diagnosis, after which 5 underwent radiotherapy, 3 underwent chemotherapy, and 10 underwent combined-modality treatment. The functional outcome was improvement in 8 patients and no change in 10; no patient worsened after surgery. Eleven had advanced disease at diagnosis, while seven had limited disease, including three patients with localized extradural lymphoma. There were 16 cases of non-Hodgkin's lymphoma and 2 of Hodgkin's disease. Two patients had T-cell lymphoma and were among the longest survivors. DNA flow cytometry identified the low-grade tumors as diploid with very low proliferative indices, while the high-grade tumors all had high indices. At a mean observation time of 41.7 months, five patients have died of their disease, and seven remain in complete remission. Survival is markedly better than that reported for other malignant extradural tumors; however, even limited stage lymphoma can behave aggressively. Similarities in age, sex distribution, histological features, and the results of flow cytometry suggest behavior similar to extranodal lymphoma at other sites. Surgery to provide a tissue diagnosis, followed by combined radiotherapy and chemotherapy, is indicated for all cases.
