Excellent Outcomes with Surgery or Radiotherapy in the Management of Castleman Disease Including a Case of Oligocentric Disease.

BACKGROUND: Castleman disease (CD) is a rare polyclonal lymphoproliferative disorder of unclear etiology. Standard therapy for unicentric CD is surgical resection. Radiotherapy can be used; however, its efficacy is poorly characterized. PATIENTS AND METHODS: We reviewed patients with histologically confirmed CD undergoing definitive local therapy at our institution between 1990 and 2017. Overall survival was determined from the date of diagnosis. Local progression-free survival and distant failure-free survival were determined from the date of first definitive therapy. The Kaplan-Meier method was used to analyze survival. RESULTS: Forty-four patients (29 female and 15 male) were identified with a median age at diagnosis of 40 years (range, 14-70 years). Thirty-five (80%) patients received surgery alone, 3 (7%) had surgery followed by radiotherapy, and 6 (14%) had radiotherapy alone. Thirty-nine (89%) patients had a single area of involvement, and 3 (7%) patients had limited regional involvement. Two (5%) patients had multicentric CD and received consolidative radiotherapy. The 3-year overall, local progression-free, and distant failure-free survival were 92%, 100%, and 100%, respectively. No distant failures were observed. The median radiation dose was 3960 cGy (range, 3600-5940 cGy) in 22 fractions (range, 18-33 fractions). CONCLUSIONS: Unicentric CD is readily amenable to cure with local therapy. Surgical excision is preferred, but radiation appears to be an effective alternative for patients when surgery is high risk or not feasible. Patients with oligo- or multi-centric CD may experience prolonged disease-free survival with consolidative radiotherapy after partial response to systemic therapy.

Castleman's disease and radiotherapy: a single center experience.

CONTEXT: The role of radiotherapy (RT) in the management of Castleman'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.

A case of cervical multicentric Castleman disease treated with intensity-modulated radiation therapy using helical tomotherapy.

Castleman disease (CD) is a rare lymphoproliferative disorder. Two clinical entities are described: a unicentric form with disease confined to a single lymph node region and a multicentric form characterized by generalized lymphadenopathy and systemic symptoms. Although surgery is regarded as standard therapy for the unicentric form, no consensus has been reached concerning the standard treatment for multicentric CD. We report here a case of cervical multicentric CD treated with intensity-modulated radiation therapy (IMRT), using helical tomotherapy to minimize xerostomia in comparison with conventional radiotherapy. A 29-year-old woman complained of neck swelling. Computed tomography showed lymphadenopathy in both sides of the neck. The patient was diagnosed with the plasma cell subtype of CD on biopsy. After initial treatment with prednisone, IMRT was planned to avoid normal structures, for example the parotid gland. The cervical lymphadenopathy shrank gradually during IMRT with 44 Gy in 22 fractions. Four years and 3 months after IMRT, regrowth of cervical lymph nodes has not been detected. The parotid function improved dramatically on quantitative salivary scintigraphy between 3 and 12 months after IMRT. Radiotherapy could be an option for multicentric CD, and IMRT is an effective means of minimizing xerostomia in head and neck lesions.

Castleman's disease in childhood: report of three cases and review of the literature.

Castleman's disease (CD) is a rare, localized or generalized, lymphoproliferative disorder with a frequent mediastinal location, but possible in any lymph node or extra nodal site. It usually appears in young adults whilst it rarely occurs in childhood. There are only about 100 pediatric cases published, five of them in Italy. We report 3 cases of localized Castleman's disease, investigated in our Department in a 3 years period and reviewed the literature.

Radiotherapy of unicentric mediastinal Castleman's disease.

Castleman's disease is a slowly progressive and rare lymphoproliferative disorder. Here, we report a 55-year-old woman with superior mediastinal Castleman's disease being misdiagnosed for a long term. We found a 4.3 cm mass localized in the superior mediastinum accompanied with severe clinical symptoms. The patient underwent an exploratory laparotomy, but the mass failed to be totally excised. Pathologic examination revealed a mediastinal mass of Castleman's disease. After radiotherapy of 30 Gy by 15 fractions, the patient no longer presented previous symptoms. At 3 months after radiotherapy of 60 Gy by 30 fractions, Computed tomography of the chest showed significantly smaller mass, indicating partial remission. Upon a 10-month follow-up, the patient was alive and free of symptoms.

Neoadjuvant radiotherapy of primary irresectable unicentric Castleman's disease: a case report and review of the literature.

BACKGROUND: Castleman disease (CD) is a rare benign disorder characterised by hyperplasia of lymphoid tissue that may develop at a single site or throughout the body. The etiology of this disorder is unclear, although the histopathological presentation can be differentiated into a hyaline vascular variant, a plasma cell variant and a mixed variant. Clinically, it has been recorded that 3 manifestations of CD are characterized: a localized unicentric type, a generalized multicentric type and a mixed form. Surgery remains the main treatment for resectable unicentric CD, since removal of the large node is possible without further complications. No consensus has been reached concerning the most adequate treatment for irresectable unicentric CD. METHODS: Case report of a 67 year old woman. RESULTS: This report, describes the case of a 67-year-old woman with unicentric Castleman disease located in the right lower abdomen. The patient had symptoms of fatigue, dyspnoea and pain in the right lower abdomen. Computed tomography (CT)- examination revealed a tumour, which had grown to form a close relationship with the common iliac vessels and the sacral bone. A Laparotomy procedure revealed that the tumour was an irresectable mass. Neo-adjuvant radiotherapy (40 Gy) was administered in order to downsize the tumour. Six weeks later a new CT-scan revealed a major reduction of the tumour, which enabled a successful radical resection of the tumour to be performed. Histopathological analysis of the tumour showed the hyaline vascular type of CD. CONCLUSIONS: Neo-adjuvant radiotherapy should be considered in case of an irresectable unicentric CD.

A case of POEMS syndrome with mixed hyaline vascular and plasma cell type Castleman's disease.

We report a 43-year-old man who developed progressive ascending sensorimotor polyneuropathy, a solitary bone plasmacytoma originating from a rib and an axillary lymph node with features of Castleman's disease, mixed hyaline vascular and plasma cell type. He was diagnosed of having non-Hodgkin's lymphoma seven years back and was treated. Examination revealed a smooth goitre, thickened skin, white nails, small testes and ankle oedema. Investigations revealed a monoclonal para-proteinaemia of 7.83 g/l and elevated levels of serum TSH and prolactin. In this case the Castleman's disease is localised and the lymphoma preceded the Castleman's disease.

Castleman disease of the spine mimicking a nerve sheath tumor. Case report.

Castleman disease is a rare lymphoproliferative disease of unknown cause. In most cases, afflicted patients present with a mediastinal mass although the disease may manifest in numerous other sites, including intracranially and rarely intraspinally. The authors report on the case of a 19-year-old woman who presented with a large paraspinal mass emanating from the T7-8 neural foramen. The morphological and signal characteristics revealed on magnetic resonance imaging were similar to those of nerve sheath tumors. The patient underwent open biopsy sampling of the lesion, and results of a pathological evaluation revealed hyaline-vascular type Castleman disease. She underwent radiotherapy and remains symptom-free with a radiographically stable lesion 1 year later. Although the disease has been reported to mimic a meningioma when encountered in intracranial locations, to the authors' knowledge, this is the first case of the disorder mimicking a nerve sheath tumor. When the diagnosis of Castleman disease is made, good results can be obtained with partial resection and radiotherapy.

Outcome and late complications of radiotherapy in patients with unicentric Castleman disease.

Castleman disease is a rare lymphoproliferative disorder. Surgery is considered standard therapy for the unicentric type. However, case reports have documented favorable responses to radiotherapy. The aim of this study was to analyse the clinical outcomes of five patients with unicentric Castleman disease treated with radiotherapy between 1991 and 2005. Mediastinal lymph nodes were the most common site of disease (four patients). Three patients were treated with radiotherapy alone, two patients with surgery and radiotherapy. Patients were treated with radiotherapy doses ranging from 40 Gy to 50 Gy. The median follow-up was 12 months (range, 3-175 months). During follow-up only one patient had progressive disease and died of Castleman disease. At the time of last follow-up two patients were in complete remission, one patient in partial remission, and one patient had stable disease. One patient showed serious acute and late toxicities. At the end of radiotherapy a paraneoplastic pemphigus vulgaris occurred, and eight to 11 months after radiotherapy a stenosis of the esophagus, of the left bronchus, and of the trachea due to scars. The study shows that unicentric Castleman disease is successfully treated with radiotherapy. However, for detection of possible complications as pemphigus vulgaris or stenosis of the esophagus or trachea an accurate follow-up is necessary.

Castleman's disease in children: report of 2 cases and clinicopathologic review.

Castleman's disease (CD), a disorder of lymphoid hyperplasia with an unknown etiology, is rare in children. It usually presents as a localized mass and is often cured by surgery. The authors report two unusual cases of CD in children. One patient had localized CD in the left supraclavicular region, a rare site of unicentric disease. She was ultimately treated by excisional biopsy, and the mass did not recur. Another patient presented with airway compression due to localized infiltrating mediastinal disease, not amenable to surgery. She refused prednisone and was treated with radiotherapy, with slow remission and no relapse for 4 years.

Surgical resection of a solitary plasmacytoma originating in a rib of a patient with Castleman's disease.

Castleman's disease is a rare disorder characterized by lymphoid hyperplasia. It may present as asymptomatic involvement of one lymph node group or as a multicentric disease with systemic features. We report a patient with Castleman's disease who presented with axillary lymphadenopathy associated with a solitary plasmacytoma originating from a rib. The affected rib was surgically resected and radical radiotherapy was subsequently administered to the axillary lymph nodes. In this particular case, a joint surgical and oncologic approach resulted in a successful outcome.

A rapidly expanding lesion in the neck: unusual presentation of Castleman's disease.

We report a painless but rapidly enlarging 9 cm x 4 cm lobulated hard neck mass. CT scanning suggested lymphoid tissue that was not confirmed by cytopathology. Histopathological analysis of the excision specimen detected Castleman's disease, extending to the resection margin. Postoperative radiotherapy was administered. The patient remains disease-free after 44 months.

Treatment of unicentric and multicentric Castleman disease and the role of radiotherapy.

BACKGROUND: Although surgery is considered standard therapy for unicentric Castleman disease, favorable responses to radiotherapy also have been documented. The authors undertook this study to analyze the clinical factors, treatment approaches, and outcomes of patients with unicentric or multicentric Castleman disease, and to report the outcomes of patients with unicentric Castleman disease treated with radiotherapy. METHODS: The authors reviewed the medical records of 22 patients who had received a histologic diagnosis of Castleman disease at the University of Texas M. D. Anderson Cancer Center between 1988 and 1999. One patient with a concurrent histopathologic diagnosis of nonsecretory multiple myeloma was excluded from the study. In all patients, the diagnosis of Castleman disease was based on the results of lymph node biopsies. Disease was categorized as being either unicentric or multicentric and further subdivided into hyaline vascular, plasma cell, or mixed variant histologic types. Clinical variables and outcomes were analyzed according to treatment, which consisted of surgery, chemotherapy, or radiotherapy. RESULTS: Records from 21 patients were analyzed: 12 had unicentric disease, and 9 had multicentric disease. The mean follow-up time for the entire series was 51 months (median, 40 months). Four patients with unicentric disease were treated with radiotherapy alone: 2 remain alive and symptom free, 2 died of causes unrelated to Castleman disease and had no evidence of disease at last follow-up. Eight patients with unicentric disease were treated with complete or partial surgical resection, and all are alive and asymptomatic. All nine patients with multicentric disease were treated with combination chemotherapy: five are alive with no evidence of disease, and four are alive with progressive disease. CONCLUSIONS: Surgery results in excellent rates of cure in patients with unicentric Castleman disease; radiotherapy can also achieve clinical response and cure in selected patients. Multicentric Castleman disease is a more aggressive clinical entity and is most effectively treated with combination chemotherapy, whereas the role of radiotherapy in its treatment remains unclear.

The management of unicentric and multicentric Castleman's disease: a report of 16 cases and a review of the literature.

BACKGROUND: Castleman's disease (CD), or angiofollicular lymph node hyperplasia, creates both diagnostic and therapeutic dilemmas for most physicians. For patients with this rare and poorly understood disease, the optimal therapy is unknown. The authors report their experience during the years 1986-1997 with this uncommon clinicopathologic entity. METHODS: Sixteen patients with a histologic diagnosis of CD were identified in the pathology database. Unicentric disease was defined as a solitary mass. Multicentric disease compromised patients with widespread lymphadenectomy. Clinical, radiologic, and laboratory data were analyzed to evaluate treatment response. RESULTS: The study group consisted of 16 patients classified into 3 clinicopathologic groups: hyaline-vascular, plasma cell, and "mixed." Of those patients who underwent complete surgical excision of a unicentric hyaline-vascular CD mass (n = 8), all remain symptom free without clinical or radiographic recurrence. Two patients remain asymptomatic following partial resection or radiation therapy for an unresectable unicentric hyaline-vascular CD mass. Two patients with multicentric hyaline-vascular CD are currently in complete remission following adjuvant therapy. Multicentric plasma cell CD was present in a single patient. This patient (who underwent surgical and systemic therapy) died of disease within 4 months of presentation. Three patients with unicentric hyaline-vascular/plasma cell-CD remain symptom free following either complete resection or observation. CONCLUSIONS: The authors recommend surgical resection for patients with the unicentric variant of CD. Surgical removal of a unicentric mass of hyaline-vascular or hyaline-vascular/plasma cell type is curative. Partial resection, radiotherapy, or observation alone may avoid the need for excessively aggressive therapy. Patients with multicentric disease, either hyaline-vascular or plasma cell type, do not benefit from surgical management and should be candidates for multimodality therapy, the nature of which has yet to be defined.

Reactivation of Behçet's disease in the course of multicentric HHV8-positive Castleman's disease: long-term complete remission by a combined chemo/radiation and interferon-alpha therapy regimen.

We used a new combined chemo- (COP/ABVD), radiation and interferon-alpha (10 x 10(6) IU s.c. 3x per week/12 months) therapy regimen to treat severe multicentric Castleman's disease (CD) complicated by relapsing Behcet's disease (BD) manifestations. More than 16 years after diagnosis of CD the patient remains in very good clinical condition, with remission of all CD and BD manifestations 13 months after discontinuation of the interferon-alpha treatment. In addition, our clinicopathological, immunohistological and virological data suggest a pathogenetic link between CD and BD via activation of pre-existing BD-specific plasma cells due to CD-related HHV8-induced overexpression of interleukin-6.

[Castleman disease: 2 unusual cases]. / Maladie de Castleman: deux observations inhabituelles.

Castleman's disease is a localized, rare and benign hyperplasia of the lymph nodes, occurring mainly in the mediastinum. Three histologic types--hyalinvascular, plasmacellular and intermediate--have been recognized. We report on two patients with unusual locations of the disease, i.e. the pelvis and the retroperitoneum respectively. These observations show that diagnosis is difficult. They also indicate that, in selected patients, radiotherapy may induce tumor regression and allow the resection of apparently inoperable lesions.