Head and neck manifestations of indolent T-cell lymphoproliferative disorder of the gastrointestinal tract: a systematic review

Abstract

Indolent T-cell lymphoproliferative disorder of the gastrointestinal tract (iTLPD-GI) is a rare lymphoproliferative disorder with a heterogeneous clinical presentation. Although primarily involving the gastrointestinal tract, head and neck manifestations have been sporadically reported and remain poorly characterized. We conducted a systematic review of case reports and case series, in accordance with PRISMA guidelines, to describe the otorhinolaryngological manifestations of iTLPD-GI and to facilitate early diagnosis and appropriate clinical management. MEDLINE/PubMed, LILACS, Cochrane Library and Web of Science Core Collection were searched to identify case reports and case series published from January 1995 to January 2026 that described head and neck involvement in patients with histologically confirmed iTLPD-GI. Ten studies comprising 13 patients with head and neck manifestations were identified. Patients ranged in age from 22 to 77 years and were predominantly male.

Oral and oropharyngeal ulcerations were observed in all patients with head and neck involvement, most frequently affecting the soft palate, while laryngeal involvement was reported in three cases. In eight patients, head and neck manifestations represented the initial presentation of the disease, including three cases with isolated oral or oropharyngeal lesions.

Lesions were predominantly ulcerative in appearance, whereas pseudotumoral presentations were less common. Most patients experienced recurrent spontaneous resolution of head and neck lesions, although some showed clinical improvement only after systemic treatment.

Head and neck involvement in iTLPD-GI, although rare, may precede gastrointestinal manifestations and should be considered in the differential diagnosis of persistent or treatment-resistant oral or oropharyngeal ulcers.