title: Primary Effusion Lymphoma-like Lymphoma in a Patient with Neurofibromatosis Type 1.
abstract: To date, there are only 15 case reports of lymphoma in patients with neurofibromatosis type 1 (NF1), a common autosomal dominant tumor predisposition syndrome. Here, we present the first report of a primary effusion lymphoma (PEL)-like lymphoma (PEL-L), which is a human herpes virus 8/Kaposi sarcoma herpes virus-unrelated PEL, in a 73-year-old woman with NF1. The woman presented with pleural effusion following surgery for a small intestinal gastrointestinal stromal tumor and a malignant peripheral nerve sheath tumor. We prepared cellblocks to accurately differentiate between PEL, PEL-L, and pyothorax-associated lymphoma, for establishing a starting point for treatment and for prolonging survival. Attention should be paid to malignant neoplasms in NF1 patients. Diffuse large B-cell lymphoma may not be a rare complication in these patients, although how NF1 promotes its development remains to be determined. PEL-L should be suspected when body cavity effusion is observed in elderly patients. If feasible, it should be treated via rituximab-containing chemotherapy, which according to the literature, results in longer survival times than does drainage or regimens consisting of cyclophosphamide, doxorubicin, vincristine, and prednisone.
authors.affiliation: Division of General Internal Medicine, Department of Medicine, Tokai University School of Medicine, 143 Shimokasuya, Isehara, Kanagawa 259-1193, Japan. okidoki@is.icc.u-tokai.ac.jp.
authors.forename: Masayuki
authors.initials: M
authors.lastname: Oki
authors.forename: Tomihisa
authors.initials: T
authors.lastname: Nanao
authors.forename: Takuma
authors.initials: T
authors.lastname: Shinoda
authors.forename: Ayumi
authors.initials: A
authors.lastname: Tsuda
authors.forename: Atsushi
authors.initials: A
authors.lastname: Yasuda
authors.forename: Toshiro
authors.initials: T
authors.lastname: Seki
authors.forename: Hideki
authors.initials: H
authors.lastname: Ozawa
authors.forename: Naoya
authors.initials: N
authors.lastname: Nakamura
authors.forename: Atsushi
authors.initials: A
authors.lastname: Takagi
mesh_terms: D000368:Aged; D000971:Antineoplastic Combined Chemotherapy Protocols / Q000627:therapeutic use; D003937:Diagnosis, Differential; D005260:Female; D005770:Gastrointestinal Neoplasms / Q000601:surgery; D046152:Gastrointestinal Stromal Tumors / Q000601:surgery; D019288:Herpesvirus 8, Human; D006801:Humans; D054685:Lymphoma, Primary Effusion / Q000175:diagnosis* / Q000188:drug therapy / Q000401:mortality; D009378:Neoplasms, Multiple Primary; D009442:Neurilemmoma / Q000601:surgery; D009456:Neurofibromatosis 1; D010996:Pleural Effusion / Q000209:etiology; D011183:Postoperative Complications / Q000209:etiology; D000069283:Rituximab / Q000008:administration & dosage; D015996:Survival Rate; D016896:Treatment Outcome
chemical_list: D000069283:Rituximab