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PubMed This is a summary of 22 peer-reviewed journal articles Updated
Oncology

Signs, Symptoms, and the Road to Diagnosis

At a Glance

Unicentric Castleman disease (UCD) is often a silent condition discovered by accident on scans. When symptoms do occur, they are typically caused by the enlarged lymph node pressing on nearby organs, though rare variants can cause systemic issues like fevers, night sweats, and weight loss.

Unicentric Castleman Disease (UCD) is often called a “silent” condition because many people do not feel sick when it is discovered [1][2]. In most cases, the mass is found incidentally—meaning it shows up on an X-ray or CT scan that was being done for a completely different reason [1][3].

However, when symptoms do occur, they usually fall into one of two categories: local pressure from the mass itself or systemic inflammation caused by the disease.

Local “Mass Effect” Symptoms

Most UCD symptoms are caused by the mass physically pressing on nearby structures. These vary depending on where the enlarged lymph node is located:

  • Chest (Mediastinum): A mass in the chest can press against the lungs or airways, leading to a persistent cough, shortness of breath, or chest discomfort [4][5].
  • Abdomen: In the belly, UCD can cause a feeling of fullness, non-specific abdominal pain, or pressure on the bladder or intestines [6][7].
  • Neck (Head/Neck): A mass in the neck is often felt as a painless, firm lump. If it grows large enough, it may cause trouble swallowing or a hoarse voice [2][8].

The Plasmacytic Variant and Systemic Symptoms

While most UCD is the hyaline vascular type (which rarely causes body-wide symptoms), a less common version called the plasmacytic variant can behave differently [9][1]. This variant can trigger systemic inflammation, causing:

  • B-Symptoms: These include unexplained fevers, drenching night sweats, and unintentional weight loss [10][11].
  • AA Amyloidosis: In rare cases, prolonged inflammation can lead to AA amyloidosis, a condition where inflammatory proteins build up in organs, particularly the kidneys, which can cause damage [9].

Extremely Rare but Serious Warning Signs

In very rare cases (affecting a tiny fraction of patients), UCD is associated with “paraneoplastic” syndromes—conditions where the body’s immune response to the mass attacks healthy tissue:

  • Paraneoplastic Pemphigus (PNP): This causes severe, painful blisters and sores in the mouth and on the skin that are resistant to normal treatments [12][13].
  • Bronchiolitis Obliterans (BO): This is a life-threatening lung condition that can occur alongside PNP, causing severe shortness of breath [14][15].

The Challenge of Misdiagnosis

Because UCD looks like many other conditions on a scan, it is frequently misdiagnosed before surgery. It is often mistaken for:

  • Lymphoma: This is the most common misdiagnosis because both involve enlarged lymph nodes [16][17].
  • Location-Specific Tumors: Depending on its location, UCD can mimic other tumors. For example, a mass in the chest may look like a thymoma, while UCD near the spine or brain can occasionally be mistaken for a schwannoma or meningioma [18][19].
  • Other conditions: It can also mimic neuroendocrine tumors or inflammatory conditions like IgG4-related disease [20][21].

A definitive diagnosis of UCD can usually only be made by a pathologist examining the entire lymph node after it has been surgically removed [22][1].

Next Steps:

Common questions in this guide

Does Unicentric Castleman Disease always cause symptoms?
No, UCD is often a silent condition. In many cases, it does not cause any noticeable symptoms and is found accidentally on an X-ray or CT scan performed for a completely different medical reason.
What are the most common symptoms of UCD?
When symptoms occur, they are usually caused by the enlarged lymph node pressing on nearby structures. This 'mass effect' can cause chest discomfort, a persistent cough, shortness of breath, abdominal fullness, or a painless lump in the neck depending on its location.
How is the plasmacytic variant of UCD different?
Unlike the more common hyaline vascular type, the plasmacytic variant can trigger systemic inflammation. This may cause body-wide symptoms, often called B-symptoms, which include unexplained fevers, drenching night sweats, and unintentional weight loss.
Why is UCD frequently misdiagnosed as lymphoma?
UCD is often mistaken for lymphoma because both conditions involve significantly enlarged lymph nodes that look very similar on imaging scans. A definitive diagnosis requires removing the entire lymph node so a pathologist can examine the tissue under a microscope.
What is paraneoplastic pemphigus in UCD?
Paraneoplastic pemphigus is an extremely rare but serious complication where the body's immune system attacks healthy tissue. It causes severe, painful blisters and sores in the mouth and on the skin that are highly resistant to normal treatments.

Questions to Ask Your Doctor

Curated prompts to bring to your next appointment.

  1. 1.Where exactly is my mass located, and is it pressing against any nearby organs or nerves?
  2. 2.Is my pathology 'hyaline vascular' or 'plasmacytic'? How does that change my expected symptoms?
  3. 3.Do I need to be screened for AA amyloidosis or paraneoplastic syndromes based on my symptoms?
  4. 4.My imaging looked like lymphoma; what specific features on the pathology confirmed this is UCD instead?
  5. 5.If I am having systemic symptoms like fever, are these expected to resolve immediately after surgery?

Questions For You

Tap a prompt to share your answer — we'll use it plus this page's context to start a tailored conversation.

References

References (22)
  1. 1

    Unicentric Castleman disease presenting as a longstanding axillary and chest wall mass: A case report.

    Zhou W, Liu X, Qiu A, et al.

    Clinical case reports 2023; (11(12)):e8258 doi:10.1002/ccr3.8258.

    PMID: 38054194
  2. 2

    A retrospective study of 44 patients with head and neck Castleman's disease.

    Wang T, Chen X, Chen W, et al.

    European archives of oto-rhino-laryngology : official journal of the European Federation of Oto-Rhino-Laryngological Societies (EUFOS) : affiliated with the German Society for Oto-Rhino-Laryngology - Head and Neck Surgery 2022; (279(5)):2625-2630 doi:10.1007/s00405-021-07065-0.

    PMID: 34480599
  3. 3

    [The clinical features of Castleman disease in the head and neck].

    Liu D, Sun F, Wei H, et al.

    Lin chuang er bi yan hou tou jing wai ke za zhi = Journal of clinical otorhinolaryngology head and neck surgery 2022; (36(7)):545-548 doi:10.13201/j.issn.2096-7993.2022.07.013.

    PMID: 35822384
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    Mediastinal Castleman disease presenting as a paraspinal mass causing back pain and shortness of breath in a young adult.

    Chen HY, Wu SH, Chew FY, Lee SY

    Heliyon 2024; (10(24)):e40792 doi:10.1016/j.heliyon.2024.e40792.

    PMID: 39759295
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    Rare Unicentric Intrapulmonary Castleman Disease: A Systematic Review and Report of a Case.

    Pannu MK, Ehrsam JP, Adamenko OM, et al.

    The open respiratory medicine journal 2025; (19()):e18743064348696 doi:10.2174/0118743064348696250107092627.

    PMID: 40322498
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    Ultrasound and radiological features of abdominal unicentric castleman's disease: A case series study.

    Lv K, Zhao Y, Xu W, et al.

    Medicine 2020; (99(18)):e20102 doi:10.1097/MD.0000000000020102.

    PMID: 32358399
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    Castleman disease: a case report of a pediatric incidental case in Saudi Arabia.

    Alessa HA, Alshayeb AN, Alqurashi TM, et al.

    Journal of surgical case reports 2025; (2025(9)):rjaf712 doi:10.1093/jscr/rjaf712.

    PMID: 40979424
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    Unicentric Castleman Disease: A Rare Diagnosis of Radiological and Histological Correlation.

    Maqbool S, Javed A, Idrees T, Anwar S

    Indian journal of otolaryngology and head and neck surgery : official publication of the Association of Otolaryngologists of India 2023; (75(2)):975-978 doi:10.1007/s12070-022-03253-4.

    PMID: 36373120
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    Epidemiology of Castleman disease associated with AA amyloidosis: description of 2 new cases and literature review.

    Fayand A, Boutboul D, Galicier L, et al.

    Amyloid : the international journal of experimental and clinical investigation : the official journal of the International Society of Amyloidosis 2019; (26(4)):197-202 doi:10.1080/13506129.2019.1641078.

    PMID: 31364863
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    Castleman's disease: Laparoscopic resection of unicentric disease in the hepatic hilum: A case report.

    Serpa Carmoma F, Cueva M, Serpa Carmoma F, et al.

    Annals of medicine and surgery (2012) 2021; (67()):102494 doi:10.1016/j.amsu.2021.102494.

    PMID: 34188910
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    Cutaneous manifestations of Castleman disease.

    Chavez-Alvarez S, Villarreal-Martinez A, Ocampo-Candiani J, et al.

    International journal of dermatology 2020; (59(10)):1226-1240 doi:10.1111/ijd.15043.

    PMID: 32686150
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    Clinical Analysis of Unicentric Castleman's Disease with Paraneoplastic Pemphigus and Bronchiolitis Obliterans.

    Zhen JF, Zhang L, Cao XX, et al.

    Zhongguo yi xue ke xue yuan xue bao. Acta Academiae Medicinae Sinicae 2017; (39(4)):492-498 doi:10.3881/j.issn.1000-503X.2017.04.006.

    PMID: 28877826
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    Pulmonary small lymphocytic lymphoma combined with paraneoplastic pemphigus and bronchiolitis obliterans: a case report.

    Sun S, Hu J, Zheng Y, et al.

    BMC pulmonary medicine 2025; (25(1)):500 doi:10.1186/s12890-025-03935-z.

    PMID: 41168701
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    Bronchiolitis Obliterans in Unicentric Castleman Disease Without Paraneoplastic Pemphigus Requiring Bilateral Lung Transplantation.

    Yanagihara T, Kayawake H, Tsuji K, et al.

    Cureus 2025; (17(9)):e92458 doi:10.7759/cureus.92458.

    PMID: 41111743
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    Unicentric castleman disease complicated by paraneoplastic bronchiolitis obliterans and pemphigus.

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    PMID: 30128272
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    Pathology of Castleman Disease.

    Wu D, Lim MS, Jaffe ES

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    An Unusual Presentation of Multiple Sclerotic Bone Lesions in Unicentric Castleman's Disease.

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    Challenging diagnosis: unusual presentation of Unicentric Castleman disease-a case report.

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    Intracranial Castleman's Disease Mimicking Dural-based Pathologies: A Case Report.

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    Development of Castleman Disease in the Paravertebral Space Mimicking a Neurogenic Tumor.

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This page explains the signs, symptoms, and diagnostic process for Unicentric Castleman disease for educational purposes only. It does not replace professional medical advice, and you should always consult your doctor or oncologist regarding your specific symptoms.

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