Unicentric Castleman Disease Research Articles

Complete Remission with Anti-CD20 Therapy for Unicentric, Non-HIV-Associated, Hyaline-Vascular Type, Castleman's Disease

Unicentric Castleman's disease (CD) is a localized lymphoproliferative disorder that is usually treated with surgery.[1] In the last few years, different reports have shown response of Multicentric...

Anti-IL6 Antibody (ab) Based Strategies Improve the Management of HIV Negative Castleman's Disease.

Castleman's disease (CD) is a rare, non-clonal lymphoproliferative disorder featuring histologically hyaline-vascular (HV), plasmacytic (PC) or a mixed variant. Clinically, CD presents with localized lymphadenopathy (unicentric CD: UCD) or generalized lymphadenopathy often associated with constitutional symptoms (multicentric CD: MCD). The etiology of CD in HIV negative patients is not known, but elevated serum levels of IL6 are closely linked to the gravity of systemic manifestations of MCD. We present data on the largest CD series thus far reported with various treatment modalities, including anti-IL6 strategies. Between 1989 and 2004, 37 patients were referred to our institution with HIV-negative CD. None of the patients tested had evidence of HHV8 infection by serology or PCR. Eleven patients had UCD. The median age in UCD was 43 years (range 29–62) with a preponderance of non-white patients (ratio 4.5:1). All patients had HV disease except one who had PC variant. One patient did not receive any specific treatment and is lost to follow up. The other 10 patients all underwent complete surgical resection. Three patients were de-bulked prior to surgery with steroids, rituxan ±cytoxan (n=2) or radiotherapy (n=1). None of these patients has evidence of disease with a median follow up of 43 mths (range 12–140 mths). Twenty-six patients had MCD. The median age was 44 years (range: 19–87) with no racial preponderance. All presented with constitutional symptoms including fatigue, anorexia or low grade fever. MCD was equal distributed amongst histological types (HV versus PC/mixed disease) and was frequently associated with peripheral neuropathy (n=5). Bronchiolitis obliterans and pemphigus, amyloidosis and myeloma were each present in 1 respective patient. The OS in MCD was 8.8 yrs with no significant difference between HVD and PC/mixed variants. A group of 12 MCD patients was treated with steroids, chemotherapy and/or anti-CD20 ab had an OS of 42 months (range: 2–111). Four patients died, whilst 4 are alive and disease free at 48, 50, 96 and 111 months. A group of 5 MCD patients was treated with the anti-trypazonomiasis agent suramin, which has anti-IL6 properties, and 4 died. The fifth patient is a long-term survivor (108 mths) after rescue with anti-IL6 based ab therapy. Nine additional MCD patients received anti-IL6 based ab therapy; 2 died. The median OS survival in this group was 74 months (range: 10–168) with 4 patients in CR at 16, 84, 108 and 168 mths. Anti-IL6 based ab therapy was very effective in abrogating debilitating constitutional symptoms. In recent patients, FDG-PET scanning showed that involved lymph nodes visible by CT scan had differential metabolic activity. PET showed response to therapy earlier than on CT or could demonstrate residual activity in normal nodes. We conclude that 1) MCD often has HV type histology, 2) anti-IL6 based ab therapies hold considerable promise in the management of symptomatic MCD, and 3) PET-scanning is helpful in monitoring disease response and activity. [Display omitted]

Castleman's disease of the urachus

Castleman's disease (angiofollicular lymphoid hyperplasia) is a rare heterogeneous group of lymphoproliferative disorders of uncertain cause. Most cases occur as mediastinal masses, although extrathoracic involvement has been reported. Castleman's disease involving the genitourinary tract, one of the most infrequent organ systems affected, has so far only been described in pararenal and retroperitoneal locations. We describe a patient with an unusual case of unicentric Castleman's disease that was located in a urachal remnant and bore a malignant appearance. The patient initially presented with abdominal pain and underwent laparoscopic radical excision of the urachal mass. Pathologic examination revealed Castleman's disease, hyaline-vascular type, with atypical lymphoid proliferation and follicular dendritic cell dysplasia. No evidence of concomitant lymphoma or sarcoma was found. Two years after surgical removal of the tumor, no recurrence was evident. To our knowledge, this is the first case of Castleman's disease presenting as a urachal tumor.

Pelvic Castleman's disease presenting as an adnexal tumor

Pelvic Castleman's disease presenting as an adnexal tumor

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

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. 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. 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. 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.

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. 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. 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. 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.