BACKGROUND
Rosai-Dorfman-Destombes disease is a rare and heterogeneous entity that has been associated with autoimmune, hereditary, and malignant diseases. There is controversy about its etiopathogenesis, clinical course, and therapeutic management.
OBSERVATIONS
The authors report a case of a 61-year-old man with a history of progressive headache without any other symptoms. Magnetic resonance imaging of the brain revealed multiple irregular lesions with an initial diagnostic impression of meningiomatosis. An excisional biopsy was performed, and the pathology report stated the finding was Rosai-Dorfman-Destombes disease.
LESSONS
The uniqueness of this case is its rarity. The isolated intracranial location presents many diagnostic and therapeutic challenges, with radiological and clinical characteristics similar to those of other central nervous system tumors. There is currently no clear evidence of the pathogenesis and therapeutic management of this condition. Follow-up of these patients will help elucidate the natural history of this condition and the benefits of various treatment modalities.
Mixed adenoneuroendocrine carcinoma of the ampulla of Vater: Case report Introduction: Mixed adenoneuroendocrine carcinomas (MANEC) are types of biphasic tumors, morphologically recognized in the presence of a neoplastic formation constituted simultaneously by glandular epithelium and neuroendocrine cells. Only 19 cases located in the ampulla of Vater have been reported in the literature. Within the gastrointestinal tract, these neoplasms predominate in the stomach or colon. Aim: Report a case of MANEC; review of the epidemiology, prognosis and treatment of these tumors. Materials and Method: Case presentation of a patient diagnosed with mixed adeno-neuroendocrine carcinoma of the ampullary region. Discussion: The clinical presentation, management and prognosis are similar to ampullary adenocarcinoma. These tumors are diagnosed with a histopathological examination of the resected specimen. Both components must be histologically malignant, and each of them must represent at least 30% of the lesion. Conclusion: MANEC of the ampulla are rare tumors worldwide, being this case the first reported in our institute.
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