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Primary esophageal CD30-positive ALK-positive anaplastic large cell lymphoma with MUM1 expression

机译：具有MUM1表达的原发性食管CD30阳性ALK阳性间变性大细胞淋巴瘤

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摘要

We report a case of primary esophageal CD30~+/ALK~+/MUM~+ anaplastic large cell lymphoma (ALCL) and discuss its diagnosis and treatment, specifically in regard to the unusual expression of the B cell marker, MUM1. A 29-year-old Mexican male presented with an esophageal mass with stenosis and dysphagia. Endoscopic samples were sent for hematoxylin-eosin staining and immunohistochemical analysis. Pathologic and immunohistochemical examination resulted in a diagnosis of primary ALCL of the esophagus with CD30~+/ ALK~+/MUM1~+ phenotype. This diagnosis was confirmed by molecular analysis [fluorescence in situ hybridization (FISH)]. Initially, two cycles of cyclophosphamide, doxo-rubicin, vincristine, and prednisone (CHOP) chemotherapy were given, but the patient exhibited progressive disease. Subsequent treatment consisted of four cycles of ifosfa-mide, carboplatin, and etoposide (ICE). As of the 12-month follow-up examination, the patient is in complete remission. This case suggests that ALCL of the esophagus should be considered in the differential diagnosis of esophageal neoplasms. Biopsy through esophagoscopy or surgical exploration is recommended, and treatment with chemotherapy or radiotherapy can achieve long-term survival.
机译：我们报告一例原发性食管CD30〜+ / ALK〜+ / MUM〜+间变性大细胞淋巴瘤（ALCL），并讨论其诊断和治疗，特别是针对B细胞标记物MUM1的异常表达。一名29岁的墨西哥男性患者出现食管肿块并伴有狭窄和吞咽困难。将内窥镜样品送去进行苏木精-伊红染色和免疫组织化学分析。病理和免疫组化检查诊断为食管原发性ALCL，表现为CD30〜+ / ALK〜+ / MUM1〜+表型。该诊断通过分子分析[荧光原位杂交（FISH）]得到证实。最初，给予了两个周期的环磷酰胺，多柔比星，长春新碱和泼尼松（CHOP）化疗，但患者表现出进行性疾病。后续治疗包括异环磷酰胺，卡铂和依托泊苷（ICE）的四个周期。截至12个月的随访检查，患者已完全缓解。该病例提示在食管肿瘤的鉴别诊断中应考虑食道ALCL。建议通过食道镜或手术探查进行活检，而化学疗法或放射疗法可以实现长期生存。

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