From uptodate
Classification of Castleman disease
- Castleman disease (CD) describes a heterogeneous group of lymphoproliferative disorders that share common histopathologic features and abnormal proliferation of morphologically benign lymphocytes. СD is classified based on the number of lymph node regions with enlarged lymph nodes.
- Unicentric CD (UCD) involves one or more lymph node(s) in a single region
- Multicentric CD (MCD) involves multiple lymph node sites and is subclassified into human herpesvirus 8 (HHV-8)-associated MCD and HHV-8-negative/idiopathic MCD.
Clinical presentation
- UСD is most often an isolated lymphoproliferative disorder of children and young adults.
- Patients are commonly asymptomatic and are brought to clinical attention when an enlarged lymph node is noted on physical examination or imaging studies.
- Most lesions are in the mediastinum, but UCD can present in any lymph node location. Laboratory and clinical abnormalities are seen in less than 25 percent of cases.
Diagnosis
- UCD should be suspected in the setting of a single persistently enlarged lymph node associated with moderate to intense post-contrast enhancement on computed tomography (СT).
- 18F-fluorodeoxyglucose positron emission tomography (FDG PET) should establish that the disease is limited to a single site.
- The diagnosis is made upon pathologic review of a complete excisional biopsy of a lymph node.
- Biopsy should demonstrate histopathologic features consistent with the hyaline vascular, plasmacytic, or mixed histopathologic subtypes, though the clinical utility of these histopathologic subtypes is unclear.
- HHV-8 testing via LANA-1 should be performed and be negative in all cases of UCD.
Management
- For most patients, we recommend complete excisional resection of the involved lymph node rather than core biopsy or incomplete resection
- Complete resection is curative in most patients.
- If the involved lymph node cannot be completely removed because of its anatomic location and it is causing compressive symptoms, we proceed with systemic B cell depleting therapy to reduce mass size followed by surgical resection.
- If the involved lymph node cannot be completely removed because of its anatomic location and it is causing inflammatory symptoms, options for systemic therapy include treatments utilized for HHV-8-negative/idiopathic MCD.
- Localized radiation therapy has a relatively limited role in refractory cases with continuing symptoms.