Journal of Cancer Research and Therapeutics

: 2015  |  Volume : 11  |  Issue : 1  |  Page : 170--173

Castleman's disease and radiotherapy: A single center experience

Bora Uysal, Selcuk Demiral, Hakan Gamsiz, Ferrat Dincoglan, Omer Sager, Murat Beyzadeoglu 
 Department of Radiation Oncology, Gulhane Military Medical Academy, Istanbul, Ankara, Turkey

Correspondence Address:
Bora Uysal
GATA General Tevfik Saglam Caddesi 06018, Istanbul, Etlik Ankara


Context: The role of radiotherapy (RT) in the management of Castleman«SQ»s disease (CD) is analyzed. Aims: The main goal of this study is to examine the efficiency of RT in the treatment of unresectable and recurrent CD. Settings and Design: Retrospective study. Subjects and Methods: Between 1980 and 2012, 11 CD patients referred and treated at our clinic were studied. Three of the patients were female, and eight of them were male. Four patients had multicentric (MC) and seven patients had unicentric CD. Five patients were managed with incisional biopsy and RT; three unicentric patients underwent total excision followed by RT, and three unicentric patients had total excision and chemotherapy. Patients were retrospectively evaluated. Median follow-up time was 36 (24-60) months with median age 41 (24-52) years and RT dose 30 (30-45) Gy. Statistical Analysis Used: Kaplan-Meier method. Results: About 72.7% of patients were male, and 27.3% were female. 63.6% of the patients were unicentric, and 36.4% were MC CD. 54.5% of the patients were managed with total excision, and 45.5% underwent incisional biopsy. About 63.6% of CD patients received RT and 27.3% were given only chemotherapy, whereas one patient (9.09%) received both RT and chemotherapy. Three-year survival was 83%, and 3-year disease free survival was 91%. No late toxicity was noted with. Acute toxicity was noted in two patients who received 45 Gy and no late radiation-induced toxicity was observed. Conclusions: RT is an effective treatment option for CD recurrences and sole treatment for unresectable CD.

How to cite this article:
Uysal B, Demiral S, Gamsiz H, Dincoglan F, Sager O, Beyzadeoglu M. Castleman's disease and radiotherapy: A single center experience.J Can Res Ther 2015;11:170-173

How to cite this URL:
Uysal B, Demiral S, Gamsiz H, Dincoglan F, Sager O, Beyzadeoglu M. Castleman's disease and radiotherapy: A single center experience. J Can Res Ther [serial online] 2015 [cited 2022 Sep 28 ];11:170-173
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Full Text


Castleman's disease (CD) is an unusual lymphoproliferative disorder described by Benjamin Castleman in 1956. CD is characterized as noncancerous growths developing in single-lymph node (unicentric type) or in multiple lymph nodes (multicentric [MC] type).

Interleukin-6 (IL-6) hypersecretion is considered in the etiology of this disease, but some patients may have microcytic anemia and normal IL-6 levels. IL-6 expression is also associated with Kaposi's sarcoma and human herpes virus-8 (HHV-8). [1] The prominent clinical features of CD are weakness, malaise, fever, local or generalized lymphadenopathy, organomegaly, edema, anemia, pulmonary, and also central nervous system involvement. Histopathological characteristics of CD include diffuse plasma cell infiltration and prominent germinal centers. CD rarely progresses to Kaposi's sarcoma or lymphomas. Median survival for CD is 30 months.

Surgical resection is the mainstay of the management of unicentric CD. Radiotherapy (RT) can be given for unresectable cases or recurrences of unicentric CD. Chemotherapy, immunomodulators, IL-6 antibodies and antiviral drugs against HHV-8 are used for MC type. Thalidomide, tocilizumab, and siltuximab are the other medications used in MC CD. [2]

Castleman's disease may occur in childhood period and it should be kept in mind in the differential diagnosis of lymphoma. [3],[4] The most common involved sites in CD are mediastinum, head and neck, abdomen, mesentery, pelvis, and rarely axilla. [5],[6],[7]] Pancreas is an infrequent site of involvement in the literature. [8]

Chemotherapy is generally accepted to be the standard systemic treatment modality for MC CD, however, a case from Japan with cervical MC CD underwent intensity modulated RT of 44 Gy high with critical structures such as parotid glands being spared resulting in excellent treatment outcome of 4-year follow-up. [9]


Medical records of 11 patients (8 male, 3 female) with CD referred to our clinic between 1980 and 2012 were assessed retrospectively. Seven patients had unicentric and four patients had MC CD. Six unicentric patients underwent complete surgical excision whilst four-MC and one other unicentric patient was managed with incisional biopsy. RT was only used for one unicentric patient due to unresectability. Six unicentric patients were treated with RT for their recurrences in the follow-up period. Three MC patients received systemic chemotherapy. One MC patient underwent RT bulky mediastinal mass followed by chemetoherapy.

Eight of 11 CD patients were given RT in total one unicentric unresectable and one MC bulky CD patient out of right received 45 Gy external RT resulting in substantial downsizing whereas the other six unicentric CD patients were given a 30 Gy. Linac-based RT.

In RT planning procedures, T-bar and ankle-knee support were used for immobilization, followed by computerized tomography (CT) simulation unit. Planning CT images with 2.5 mm slices were acquired and sent to planning unit. Lungs, heart, spinal cord, esophagus, and gross tumor volume (GTV) were contoured, and clinical target volume (CTV) of postoperative cases were delineated as GTV. For unresectable cases and bulky CD, 1 cm. expansion in all diameters was added to GTV for generating the CTV and a 1 cm. additional margin for planning target volume.

Statistical analysis

SPSS (Statistical Package for the Social Sciences) 16.0 program was used for statistical analysis. Gender, age, type of CD, surgery and therapy, follow-up time, survival, and RT dose were registered for percentile and median scores. Median age, RT dose and follow-up times were analyzed using the Kaplan-Meier method.


Patient and treatment characteristics are shown in [Table 1]. Median age was 41 (24-52). Median follow-up time was 36 months (24-60). Median RT dose was 30 Gy (30-45). Karnofsky performance score (KPS) of all patients was recorded. KPS of one patient was 80 because of bulky mediastinal mass. All of the other KPS of remaining patients were 100.{Table 1}

About 72.7% of the patients were male, and 27.3% of them were female. 63.6% of all patients had unicentric CD, and 36.4% of them had MC CD. 54.5% of patients were operated via complete surgical excision, and 45.5% were managed with just insicional biopsy.

63.6% of CD patients were treated by RT, and 27.3% of all were given chemotherapy. 9.1% of patients was delivered RT and then given adjuvant chemotherapy. 81.8% of patients were alive, and 2% of all died.

Nine patients were alive, and two patients died after follow-up time. One of the MC CD patient had a progressive disease and died because of CD. The other patient died because of comorbidities. Six of 11 patients had recurrences after surgery and delivered RT.

Three-year survival was 83%, and 3-year disease free survival (DFS) was 91%. No chronic toxicity was seen in any patient. Acute toxicity was seen in two patients who were delivered 45 Gy. Grade 1 nausea, weakness, dysphagia, and swallowing difficulties were seen in these two patients, and they were given symptomatic medications.


Castleman's disease is an uncommon lymphoproliferative entity with unknown etiology. Unicentric and MC types are the clinical subtypes of CD. Hyaline, vascular, plasmablastic, mixed, plasma cell are histomorphological variants of CD. [10] Although complete surgical excision is the gold standard treatment in unicentric CD, RT can be considered as an alternative and salvage therapy for this rare disease. [11]

Castleman's disease is mostly located in the mediastinum and also outside the mediastinum including head and neck, mesentery, pelvis, axilla, adrenal gland, retroperitoneum, and pancreas. [12] Chemotherapy should be used for MC CD. Rituximab and thalidomide are the drugs of combination therapy for this entity. [13] IL-1 receptor agonist is also used in MC CD. [14] Situximab is a novel anti-IL-6 monoclonal antibody for systemic CD. [15]

Systemic agents still being investigated for the treatment are sirolimus, suramin, and CX-4945. Early in 1990's, one case of CD in the parotid gland was treated with 40 Gy fast electrons successfully. [16] The role of surgery in the management of CD is well-established and reported in various reports. [17],[18] RT for unresectable CD was studied by de Vries et al. and downstaging of tumor was achieved in that study. [19]

Because CD is a rare disease and diagnosed casually, positron emission tomography-CT can be used for staging and treatment response if there are symptoms supporting CD. [20] Increased 18-fluorodeoxyglucose uptake is shown in CD which may be of prognostic importance. [21] The role of RT in unicentric CD is more clearly defined and extensively studied, however, its utility in MC CD is controversial owing to the systemic nature of the disease in this setting. [22]

One of the multinumber analysis of CD from literature was an article by Talat et al. Three-year DFS of unicentric, hyaline vascular and HIV(−) CD was 93%, unicentric, plasma cell, and HIV(−) was 79%, MC, plasma cell, HIV(−) was 46%, HIV(+), MC was 28%. [23] DFS rates of our study are consistent with this study.

The exact etiology of CD is still unknown. Several histopathologic studies about syndromes associated with CD were investigated. These are polyneuropathy organomegaly, endocrinopathy, metabolic syndrome, skin abnormalities and thrombocytopenia, anasarca, fever, reticulin fibrosis, and organomegaly syndrome that were published in the literature. [24],[25],[26]

Radiotherapy is an efficient therapeutic modality for recurrences or unresectable CD patients. One of the limitations of our study is the limited number of cases. Furthermore, our study reflects just single-center experience and it is retrospective. Our center's surgical and RT experience about this occasional entity is the main advantage of our study.


Multicenter prospective studies about this rare disease are needed for further evaluation about RT and future studies will shed light on the algorithms for the management of CD.


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