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Thus, there are limited evidence-based specific recommendations regarding its diagnosis and treatment. We present a case report of a patient with relapsed primary thyroid DLBCL, followed by a focused literature review covering key epidemiological, diagnostic, and therapeutic aspects. In 2017, a 74-year-old woman was diagnosed with primary thyroid non-GCB-type DLBCL (Ann Arbor IE, IPI 2) after presenting with dyspnoea, hoarseness, and cough. Treatment with R-CHOP and CNS prophylaxis achieved complete remission. Six years later, an isolated relapse occurred in the contralateral thyroid gland. The patient was treated with Pola-R-miniCHP (off-label), resulting in complete remission again. In the focused literature review core needle or surgical biopsy was found to be important for reliable histopathological characterization, while first-line treatment mainly consisted of rituximab-containing chemoimmunotherapy, occasionally combined with radiotherapy. Surgery was largely limited to diagnostic purposes. Reported 5-year overall survival rates ranged from 57% to 88%, whereas relapse or refractory disease and central nervous system involvement were rare. Registry-based studies, especially those focusing on primary extranodal DLBCL, are necessary to develop evidence-based treatment guidelines for these patients.<\/jats:p>","DOI":"10.1007\/s00277-026-07303-7","type":"journal-article","created":{"date-parts":[[2026,10,8]],"date-time":"2026-10-08T22:53:43Z","timestamp":1791500023000},"update-policy":"https:\/\/doi.org\/10.1007\/springer_crossmark_policy","source":"Crossref","is-referenced-by-count":0,"title":["Primary thyroid diffuse large B-cell lymphoma: a case report and focused review of the literature"],"prefix":"10.1007","volume":"105","author":[{"given":"K\u00fcrsat","family":"Kirkg\u00f6z","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Katja","family":"Weisel","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Carsten","family":"Bokemeyer","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-5655-3155","authenticated-orcid":false,"given":"Susanne","family":"von Kroge","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"297","published-online":{"date-parts":[[2026,10,9]]},"reference":[{"key":"7303_CR1","doi-asserted-by":"publisher","first-page":"156","DOI":"10.1093\/annonc\/mdq310","volume":"22","author":"C Onal","year":"2011","unstructured":"Onal C, Li YX, Miller RC et al (2011) Treatment results and prognostic factors in primary thyroid lymphoma patients: A Rare Cancer Network study. 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