title: Sarcoidal granulomas in the mediastinal lymph nodes after treatment for marginal zone lymphoma of the esophagus: report of a case with review of the concept of the sarcoidosis-lymphoma syndrome.
abstract: Patients with sarcoidosis have a high risk of development of malignant lymphoma, and this association was coined the term "sarcoidosis-lymphoma syndrome". Extranodal marginal zone lymphoma of mucosa-associated lymphoid tissue (MALT lymphoma) is a distinct clinicopathological entity, and the stomach is the most common site. The occurrence of this type of lymphoma in the esophagus is extremely rare. In this report, we describe the first documented case of sarcoidal granulomas in the mediastinal lymph nodes after treatment for MALT lymphoma of the esophagus. A 60-year-old Japanese female was found to have a submucosal tumor in the esophagus. Histopathological study revealed proliferation of small- to medium-sized lymphoid cells with convoluted nuclei, and immunohistochemically, these lymphoid cells were diffusely positive for CD20, bcl-2, and MUM1. R-CHOP therapy was performed, which led to tumor remission. Two years later, swelling of the mediastinal lymph nodes was detected. Histopathological study of the lymph nodes revealed presence of variably-sized epithelioid granulomas without caseating necrosis but no malignant lymphoma was noted. Sarcoidal granulomas can be observed in patients with malignant tumors including malignant lymphoma and carcinoma without history of systemic sarcoidosis. It is important to recognize that systemic sarcoidosis and sarcoidal reaction without evidence of systemic disease can occur after development of malignant lymphoma, therefore, sarcoidal reaction must be included in the differential diagnostic consideration of recurrent malignant lymphoma.
authors.affiliation: Department of Clinical Laboratory Medicine, Shiga University of Medical Science Shiga, Japan ; Division of Diagnostic Pathology, Shiga University of Medical Science Shiga, Japan.
authors.forename: Mitsuaki
authors.initials: M
authors.lastname: Ishida
authors.affiliation: Department of Hematology, Shiga University of Medical Science Shiga, Japan.
authors.forename: Keiko
authors.initials: K
authors.lastname: Hodohara
authors.affiliation: Department of Hematology, Shiga University of Medical Science Shiga, Japan.
authors.forename: Aya
authors.initials: A
authors.lastname: Furuya
authors.affiliation: Department of Clinical Laboratory Medicine, Shiga University of Medical Science Shiga, Japan.
authors.forename: Hiroko
authors.initials: H
authors.lastname: Okuno
authors.affiliation: Department of Clinical Laboratory Medicine, Shiga University of Medical Science Shiga, Japan.
authors.forename: Miyuki
authors.initials: M
authors.lastname: Yoshii
authors.affiliation: Department of Clinical Laboratory Medicine, Shiga University of Medical Science Shiga, Japan.
authors.forename: Akiko
authors.initials: A
authors.lastname: Horinouchi
authors.affiliation: Department of Clinical Laboratory Medicine, Shiga University of Medical Science Shiga, Japan.
authors.forename: Ayaka
authors.initials: A
authors.lastname: Shirakawa
authors.affiliation: Division of Diagnostic Pathology, Shiga University of Medical Science Shiga, Japan.
authors.forename: Muneo
authors.initials: M
authors.lastname: Iwai
authors.affiliation: Division of Diagnostic Pathology, Shiga University of Medical Science Shiga, Japan.
authors.forename: Akiko
authors.initials: A
authors.lastname: Kagotani
authors.affiliation: Department of Clinical Laboratory Medicine, Shiga University of Medical Science Shiga, Japan.
authors.forename: Takashi
authors.initials: T
authors.lastname: Yoshida
mesh_terms: D058846:Antibodies, Monoclonal, Murine-Derived; D000971:Antineoplastic Combined Chemotherapy Protocols; D014408:Biomarkers, Tumor / Q000032:analysis; D003520:Cyclophosphamide; D004317:Doxorubicin; D004938:Esophageal Neoplasms / Q000150:complications* / Q000188:drug therapy; D005260:Female; D006099:Granuloma / Q000209:etiology / Q000473:pathology*; D006801:Humans; D007150:Immunohistochemistry; D008198:Lymph Nodes / Q000473:pathology*; D018442:Lymphoma, B-Cell, Marginal Zone / Q000150:complications* / Q000188:drug therapy; D008875:Middle Aged; D011241:Prednisone; D000069283:Rituximab; D012507:Sarcoidosis / Q000150:complications*; D014750:Vincristine
chemical_list: D058846:Antibodies, Monoclonal, Murine-Derived; D014408:Biomarkers, Tumor; C571759:R-CHOP protocol; D000069283:Rituximab; D014750:Vincristine; D004317:Doxorubicin; D003520:Cyclophosphamide; D011241:Prednisone
keywords: Sarcoidosis; esophagus; marginal zone lymphoma; sarcoidosis-lymphoma syndrome