Differential diagnosis in Rosai-Dorfman disease: A rare case of isolated hepatic presentation mimicking a metastatic tumor with positive 18-FDG uptake.

IF 1.1 Q2 MEDICINE, GENERAL & INTERNAL
Carlo Gazia, L. Giordano, M. Diodoro, Iacopo Compalati, R. Avantifiori, G. Grazi
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引用次数: 1

Abstract

Rosai-Dorfman disease (RDD) is also called sinus histiocytosis with massive lymphadenopathy, and it is caused by a histiocytic disorder with unclear etiology. It usually involves cervical lymph nodes, but it may also present with extranodal involvement. We report a rare condition of isolated hepatic RDD without nodal involvement, clinically manifested with three-month abdominal pain and tenderness of the right hypochondrium. CT- and PET-CT scans were compatible with a secondary lesion from an unknown primary tumor. Therefore, the patient underwent an atypical liver resection. Immunohistochemistry and histological results were compatible with a diagnosis of RDD. RDD is characterized by phenomena of emperipolesis, histiocytic proliferation and positive immunostaining for CD14, CD68 and S-100 protein. Cases of isolated gastrointestinal localization of RDD are particularly rare, especially in the liver. Instrumental exams might confuse RDD with other malignancies. RDD is a rare entity, which might be misdiagnosed using PET-CT due to its similarities with malignant tumors. An accurate multidisciplinary approach may help to clear diagnostic clues of this uncommon disease.
Rosai-Dorfman病的鉴别诊断:一例罕见的孤立肝脏表现,模拟转移性肿瘤,18-FDG摄取阳性。
Rosai Dorfman病(RDD)也称为窦性组织细胞增多症伴大量淋巴结病,是由病因不明的组织细胞病引起的。它通常累及颈部淋巴结,但也可能累及结外。我们报告了一种罕见的无淋巴结受累的孤立性肝脏RDD,临床表现为三个月的腹痛和右疑病症压痛。CT和PET-CT扫描与未知原发肿瘤的继发性病变相一致。因此,患者接受了非典型肝切除术。免疫组织化学和组织学结果与RDD的诊断一致。RDD的特征是热斑病、组织细胞增殖和CD14、CD68和S-100蛋白的阳性免疫染色。RDD的孤立性胃肠道定位病例尤其罕见,尤其是在肝脏。仪器检查可能会将RDD与其他恶性肿瘤混淆。RDD是一种罕见的实体,由于其与恶性肿瘤相似,使用PET-CT可能会被误诊。准确的多学科方法可能有助于明确这种罕见疾病的诊断线索。
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来源期刊
Intractable & rare diseases research
Intractable & rare diseases research MEDICINE, GENERAL & INTERNAL-
CiteScore
2.10
自引率
0.00%
发文量
29
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