High-grade B-cell lymphoma, not otherwise specified, presenting as primary peritoneal lymphomatosis and successfully treated with dose-adjusted EPOCH-R

• Published in: Journal of Clinical and Experimental Hematopathology Vol. 64; no. 1; pp. 37 - 44
• Main Authors: Fujimi, Akihito, Nagamachi, Yasuhiro, Yamauchi, Naofumi, Onoyama, Naoki, Hayasaka, Naotaka, Matsuno, Teppei, Koike, Kazuhiko, Goto, Yoshiro, Ihara, Kohji, Kato, Junji, Nishisato, Takuji, Kawase, Hiroshi, Yano, Tomoyuki, Kanaseki, Takayuki, Sugita, Shintaro, Kobune, Masayoshi
• Format: Journal Article
• Language: English
• Published: Japan The Japanese Society for Lymphoreticular Tissue Research 01.01.2024; JSLRT
• Subjects: Case Report; DA-EPOCH-R; high-grade B-cell lymphoma; MYC; peritoneal lymphomatosis; high-grade B-cell lymphoma; peritoneal lymphomatosis; MYC; DA-EPOCH-R
• ISSN: 1346-4280; 1880-9952
• DOI: 10.3960/jslrt.23044

Peritoneal lymphomatosis (PL) is a rare lymphoma-associated condition defined as the dissemination of lymphoma cells in the peritoneum. An 82-year-old man presented with abdominal pain, heartburn, and high fever. Radiological findings, including positron emission tomography-computed tomography (PET-CT), and gastrointestinal fiberscopy, showed diffuse thickening of the peritoneum, omentum, and mesentery; however, no lymphadenopathy, hepatosplenomegaly, or gastrointestinal lesions were observed. Under suspicion of peritonitis carcinomatosa of unknown origin, exploratory laparoscopy was performed that revealed multiple white nodules and masses on the surfaces of the peritoneum, mesentery, and intestinal serosa. The histopathological and cytogenetic findings of the peritoneum revealed high-grade B-cell lymphoma, not otherwise specified, and a gain of MYC by fluorescence in-situ hybridization. The patient was treated with two cycles of R-CHOP therapy, followed by six cycles of dose-adjusted EPOCH-R therapy, and a complete metabolic response was confirmed by PET-CT. Since there are no specific radiological findings to confirm the diagnosis of PL, a histopathological diagnosis is usually required. Most PL exhibit an aggressive lymphoma phenotype and can be cured by appropriate chemotherapy. Therefore, early diagnosis and treatment are desirable.