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Updated: Sep 5, 2025

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Published on: May 26, 2023
[The clinical features of Castleman disease in the head and neck]
Dingding Liu1, Feihu Sun1, Hao Wei1
1Department of Otolaryngology Head and Neck Surgery,Affiliated Drum Tower Hospital of Nanjing University Medical School,Jiangsu Provincial Key Medical Discipline,Research Institute of Otolaryngology,Nanjing,210008,China.
Insights
Castleman disease in the head and neck is typically unicentric, presenting as enlarged lymph nodes. Surgical removal offers a good prognosis for this type, while multicentric forms require ongoing management due to complex symptoms.
Area of Science:
- Head and Neck Oncology
- Pathology
- Clinical Medicine
Background:
- Castleman disease is a rare lymphoproliferative disorder.
- Head and neck involvement presents unique diagnostic and management challenges.
Purpose of the Study:
- To analyze clinicopathological features, treatment strategies, and prognosis of head and neck Castleman disease.
- To differentiate outcomes between unicentric and multicentric forms.
Main Methods:
- Retrospective analysis of 18 patients diagnosed with head and neck Castleman disease between 2007 and 2021.
- Review of clinical data, pathological findings, treatment interventions, and follow-up outcomes.
Main Results:
- Unicentric Castleman disease (14 cases) showed good prognosis after surgical resection with no recurrence.
- Multicentric Castleman disease (4 cases) presented with systemic symptoms like edema and organomegaly, with disease progression despite varied treatments.
- All patients survived follow-up, but multicentric cases exhibited significant progression.
Conclusions:
- Head and neck Castleman disease is predominantly unicentric, effectively treated with surgery offering a favorable prognosis.
- Multicentric Castleman disease requires long-term monitoring and management due to its systemic nature and potential for progression.
Abstract:
Objective:To investigate the clinicopathological features, treatment and prognosis of Castleman disease in the head and neck. Methods:The clinical and pathological data of 18 patients with Castleman disease of the head and neck in Nanjing Drum Tower Hospital from 2007 to 2021 were retrospectively analyzed. There were 14 cases of unicentric type and 4 cases of multicentric type. The clinical characteristics, treatment and prognosis were analyzed. Results:Among the 18 cases of Castleman disease in the head and neck, 1 case was located in the parotid gland, 1 case was behind the ear, 1 case was in the parapharyngeal space, 3 cases were in the neck region Ⅰ, 2 cases were supraclavicular, 2 cases were in the neck region Ⅲ, the rest were located in more than two subregions of the neck. In patients with unicentric type, no tumor recurrence and progression were found in the postoperative re-examination with neck Doppler ultrasound and CT; in the multicentric type, multiple organ dysfunction, such as edema of both lower extremities, hepatosplenomegaly, and cough, were found. Of the 4 patients with multicentric type, only 1 patient received chemotherapy, and the remaining 3 patients refused chemotherapy and only received symptomatic treatment. All patients survived during follow-up, but the disease of multicentric patients progressed significantly, and the number of involved lymph nodes increased, and hepatosplenomegaly were found in some patients. Conclusion:Castleman disease of the head and neck is mostly unicentric type, which is manifested as multiple asymptomatic enlarged lymph nodes in the neck. The surgical resection is effective and the prognosis is good. Multicentric Castleman disease of the head and neck has complex clinical symptoms and involves multiple organs over time, requiring follow-up treatment.
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