Unicentric Castleman Research Articles

Unicentric castleman disease located in the left popliteal fossa: a case report

BackgroundCastleman disease (CD) is a lymphoproliferative disease of unknown etiology, it can affect any lymph nodes of the body but rarely affects the popliteal fossa.Case presentationWe present a 67-year-old woman with touching solitary painless mass in the left popliteal fossa for one week. Imaging showed multiple soft-tissue masses of different sizes in the left popliteal muscle space, the T1 weighted image showed hypointense to isointense, the fat-suppressed T2 weighted images showed subtle hypersignal intermingled with linear of hypointense,and displayed homogeneous contrast enhancement after administration of gadolinium. Complete surgical resection was performed. Pathologically demonstrated plasma cell type CD.ConclusionWe described a rare case plasma cell type of UCD located in the popliteal fossa which might help to enrich the clinical spectrum of this rare site and unique subtype of UCD. This case illustrates that CD should be considered in the differential diagnosis of every hypervascularity soft tissue tumor in any anatomic location, especially when they occur in the region of lymph node distribution.

Unicentric castleman's disease of interpectoral (rotter's) lymph nodes mimicking a breast tumor.

Unicentric castleman's disease of interpectoral (rotter's) lymph nodes mimicking a breast tumor.

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

This study aimed to improve the diagnostic accuracy of abdominal unicentric Castleman's disease (UCD) by retrospectively summarizes the relatively specific imaging features of UCD.This study retrospectively collected fifteen patients with abdominal UCD confirmed by pathology. All patients were underwent ultrasound (US), computed tomography (CT), or magnetic resonance imaging (MRI) examination. The imaging findings of UCDs were analyzed by senior radiologists.Fifteen patients included 7 males and 8 females, aged 30 to 68 years old, with an average age of 51.73 ± 13.57. In the 15 cases, 7 were located around the mesentery, 4 were located in the retroperitoneal space, and 4 in the liver. Fifteen cases contained solid masses, of which 13 had clear margins and 2 had blurred margins. The size of the mass ranged from 1.5 to 14.2 cm, with an average of 6.49 ± 4.16 cm. US showed that 9 lesions were presented with hypo-echogenicity while 5 lesions presented with hyper-echogenicity spots. Unenhanced CT showed that the lesions were comprised of soft tissue while calcified lesions were found in 10 of the cases (66.67%, 10/15). T1-weighted imaging (T1WI) suggested the lesions as iso/hypo-signal, and mildly hyper-signal on T2-weighted imaging (T2WI). Diffusion-weighted imaging (DWI) showed different degrees of hyper-signal. Contrast-enhanced US and CT/MRI showed obvious enhancement at the arterial phase in 12 cases (85.71%, 12/14), most of which (50%, 7/14) showed continuous enhancement at the delayed phase. Feeding vessel could be seen within, or around the lesion in 5 cases (35.71%, 5/14).The study suggests that abdominal UCD commonly manifests as well-defined, homogeneous, solid, and hypervascular masses. Calcification and the presence of feeding vessel in the tumors are relatively specific features of abdominal UCD.

Castleman Disease in Children from Histopathology to Therapy

Background: Castleman disease (CD) describes a group of rare lymphoproliferative disorder with characteristic histopathology. It presents with heterogeneous clinical features whether unicentric (UC) or multicentric disease (MCD). The aim of the work was to describe clinic-pathological characteristics, management and outcome of different types of castleman disease in CCHE. It is a retrospective study, all children with (CD) treated from July 2007 till end of 2017 were analyzed as regard diagnosis, management and outcome. The results showed that, twelve patients with a median age of 11.5 years (ranging from 4 - to 17 years) were enrolled. Eight of them (66.6%) were males and 4 (33.3%) were females (33.3%). Histopathology was either hyaline vascular in 8 patients (66.6%) or plasma cell variant in 4 patients (33.3%). Nine patients (75%) had uni-centric disease (UC) with lymphadenopathy. Three patients (25%) were multi-centric disease (MCD); out of them one patient had immune bi-cytopenia, small intestinal thickening and splenomegaly and another one had extensive pulmonary involvement and respiratory distress, while the third patient had pleural effusion and ascites. HIV antibody was negative for all patients. Regarding the three MCD patients, IL-6 was normal in one patient, and extremely high in second one, while it hasn’t been done for the third patient. All UC patients underwent surgical excision and are alive with no active disease till now. All MCD patients received systemic steroids as initial therapy, followed by R- CHOP (Rituximab-cyclophosphamide-doxorubicin-vincristine-prednisone) as salvage therapy, except for one patient with pulmonary involvement who didn’t achieved clinical response and received single agent Rituximab with Anti IL-6. Unfortunately this patient is still having uncontrolled systemic manifestation, while the other two patients showed partial response. In conclusion, Uni-centric castleman disease is a localized surgically cured disease. MCD treatment remains challenging, and the outcome is controversial, so Uniform treatment guidelines are mandatory.

Unicentric castleman disease in the differential diagnosis of a paravesical mass: A case report

Castleman disease is a rare condition with variable forms of clinical presentation. Diagnosis is therefore difficult and the ideal management of the disease has yet to be defined. Prognosis varies as a function of histologic type. Treatment can range from curative surgery for the unicentric form of the disease to a combination of treatments for the multicentric form. This paper describes an unusual case of unicentric Castleman disease in which the differential diagnosis included several gynaecological malignancies. The lesion was resected, with histopathology and immunohistochemistry confirming the diagnosis. The surgical procedure was completed without complications and follow-up of the patient over one year has shown no signs of recurrence. A literature review was performed, and the possible conditions to be included within the differential diagnoses are discussed.

2571 Unicentric Castleman Disease as a Cause of Ileal Perforation: A Case Report

INTRODUCTION: Castleman disease is describes a group of heterogenous lymphoproliferative disorders that share histopathologic features. It is divided into Unicentric castleman disease (UCD) and Multicentric Castleman disease. We present a case of a patient with one year history of recurrent small bowel obstruction (SBO), which was found to be due to UCD affecting the mesentery. CASE DESCRIPTION/METHODS: A 75 years old gentleman initially presented one year ago with an episode of SBO. He had no history of abdominal or pelvic surgery. CT scan showed a transition point near the terminal ileum and mesenteric/retroperitoneal lymphadenopathy. Patient underwent a colonoscopy which showed a stenotic ileocecal valve with active colitis in the terminal ileum and ascending colon suggestive of resolving intestinal ischemia. This time, patient was admitted with decreased oral intake due to SBO. He was found to have a low albumin of 0.8 and an AKI on CKD. He also had bilateral pleural effusions and new ascites. He was treated conservatively initially, but he developed new abdominal pain with X ray showing free air under the diaphragm concerning for intestinal perforation. Patient underwent emergent laparotomy, where he had a perforated terminal ileum and multiple large fibrotic lymph nodes in the associated mesentery. Pathology showed proliferation of atretic follicles. There were occasional penetrating capillaries and the mantle lymphocytes have an onion-skin pattern. The interfollicular zones showed increased plasma cells, but not prominent vascular proliferation. CD3 and CD20 stains showed an intact immune-architecture. The overall findings showed a mixed pattern of Castleman lymphadenopathy, with some features of both the hyaline vascular and plasma cell variants. He had IgA of 510 mg/dl and an abnormal SPEP with IgG lambda paraprotein of 0.39 g/dl. His hospital course was complicated by septic shock due to bacterial and fungal peritonitis. Patient eventually went into cardiac arrest and died. DISCUSSION: Mixed variant CD is mostly multicentric, however, some patients with UCD reveal features of both hyaline vascular type and plasma cell type. In this case, apart from abdominal pain and recurrent SBO episodes, there were no other complaints. Acute phase reactants, CBC, and serological testing for HIV, HHV-8, Hepatitis B, Hepatitis C, RPR and PPD were all unremarkable. UCD is usually asymptomatic and generally curable using surgical resection but in this case led to severe complication ending in patient's demise.

Castleman Disease in the Buccal Space Mimicking Lymphoma: A Case Report

Castleman disease is a benign lymphoproliferative disorder of unknown etiology, more commonly appearing in the mediastinum. Craniofacial involvement is rare. Surgical excision is the only intervention needed for the unicentric type, while systemic treatment would be necessary for the multicentric type. Here, we report a rare case of unicentric castleman disease of the buccal space of a 23-year-old woman. Additionally, we review the radiological and histopathological features of this lesion.

Retroperitoneal castleman’s disease: A rare case report

Introduction: Castleman’s disease is a rare lymphoproliferative disorder which may be confused with other cause of lymphadenopathy.Case report: Here, we report a case of unicentric Castleman’s disease presenting with retroperitoneal lymphadenopathy. This case has been reported for its rarity.Conclusion: Though castleman’s disease is a relatively rare entity. This case report brings to light the importance of obtaining definitive histological diagnosis in patients presenting with lymphadenopathy and systemic symptoms. This case emphasis the importance of histology to seal the diagnosis from the other lymphoproliferative conditions.

Recurrence in unicentric castleman\u2019s disease postoperatively: a case report and literature review

BackgroundOur case describe a rare recurrence case of Unicentric Castleman’s disease (UCD) with hyaline vascular type 14 years after surgery.Case presentationA 35-year-old Chinese female was admitted to hospital with one and half months history chest distress and chest pain. Patient reports a history of thoracic operation for mediastinal mass 14 years ago, and it was diagnosed UCD with hyaline vascular type after postoperative pathological examination. At this time, the imaging examination showed a mediastinal mass once again. Combining the medical history, postoperative microscopically examination and immunoperoxidase staining, patient was again diagnosed UCD with hyaline vascular type again. The hyaline vascular type is the most common type and usually presents as a UCD. Most patients with UCD have no clinical symptoms. The diagnosis of UCD is generally achieved with histological and immunohidtochemical findings postoperatively. Currently, the standard treatment of UCD is the complete surgical resection, with almost no relapse postoperative. The recurrence in UCD with hyaline vascular type postoperative have not previously been reported. Therefore, herein we describe a recurrence case of UCD with hyaline vascular type 14 years after surgery.ConclusionOur case is the first case which reports the relapse of UCD with hyaline vascular type after completely surgery. It indicates that long term follow-up is necessary for patient who is diagnosed UCD after surgery.

Laparoscopic treatment of abdominal unicentric castleman\u2019s disease: a case report and literature review

BackgroundCastleman’s disease is a rare lymphoproliferative disorder of unknown etiology that most commonly presents as a mediastinal nodal mass. It is exceptionally uncommon for Castleman’s disease to present in the mesentery and, only 53 cases have ever been described in the literature. Standard treatment for this lymphoproliferative disorder involving a single node is a complete “en bloc” surgical resection which has proven to be a curative approach in almost all cases without recurrence after 20 years of follow up. All 53 reported cases of mesenteric Castleman’s disease, except one, were treated with laparotomy.Case presentationWe report on a case of mesenteric Castleman’s disease localized in the mesentery which is the second reported case if its kind and was treated by a laparoscopic-assisted procedure. Our female patient had an uneventful postoperative course and was discharged in the 5th post-operative day. No signs of recurrence were present as evidenced by physical examination and total body CT scan 24 months after the operation. We compare our case with the other reported cases in which Castleman’s disease presented as an isolated mass in the abdomen.ConclusionAlthough a rare disease, Unicentric Castleman’s disease should always be considered when a solid asymptomatic abdominal mass is occasionally presented. The laparoscopic approach (LA) allows for the achievement of better results than open surgery, including a reduction in postoperative pain and length of hospital stay. In cases of masses of an uncertain nature, LA must be considered the last diagnostic tool and the first treatment one.

Unicentric castleman’s disease of neck node in old age- rare presentation of a rare disease

Castleman’s disease is an uncommon lymphoproliferative disorder that may be unicentric or multicentric disease. We are reporting a case of 75 year old female presented with left level 3 cervical lymph node, whose final diagnosis was unicentric castleman’s disease. This presentation is uncommon because unicentric castleman’s disease is usually seen in younger adults and it usually affects lymph nodes in chest or abdomen. People with localized Castleman’s disease are usually cured by surgical removal of the affected lymph nodes.

Castleman's Disease as a Rare Differential Diagnosis of Lymphadenopathy: 2 Case Reports and Review of Literature

ntroduction: Lymphadenopathy is a common complaint in children. It's defined as abnormality in size or character of lymph node. It may be localized or generalized. It may be part of a group of signs and symptoms or the sole finding and chief complaint. Several reasons can lead to Lymphadenopathy. Castleman's disease is a rare differential diagnosis of generalized or localized Lymphadenopathy. It's a rare lymph proliferative disorder that rarely occurs in children. Case presentation: We reported two cases, the first one was 13 years old female with multicenteric castleman's disease associated with constitutional manifestations and unusual hyaline vascular pathology successfully treated with monthly combination chemotherapy, the second one was 2.5 years old male with unicentric hyaline vascular castleman's disease with unusual young age at presentation successfully treated with surgical excision. Conclusion: Castleman's disease is a rare cause of Lymphadenopathy. Although, it's rare in children, it can occur at any age. Most of cases of multicenteric castleman's disease are plasma cell variant, yet hyaline vascular type may occur. Multicenteric castleman's disease can be successfully treated with monthly combination chemotherapy. Most cases of unicentric castleman's disease are hyaline vascular type. Although, surgical removal of unicentric type is usually sufficient to achieve complete remission, recurrence can still occur.

Castleman’s Disease of the Pleura: Case Presentation and Review of Literature

Purpose. Castleman’s Disease is a very rare medical disease and the presence of this condition in the pleura is even rarer and there are only few reported cases. The presence of symptoms is also unusual and has been mostly described in multicentric cases of castleman’s disease instead of solitary lesions (unicentric castleman). We report a case of unicentric pleural castlema’s disease with presence of constitutional symptoms. Methods. A 36-year-old male without any significant past medical history who presented to the emergency due to an incidental large pleural effusion seen in chest radiograph, but referring chronic constitutional symptoms consistent of significant weight loss and chronic cough. Results. Chest computed tomography revealed a mass and several attemps with minimally invase approach failed to identify the lesion. The mass was excised through VATS but due to its significant vascularity, it had to be converted to open thoracotomy. Conclusion. The treatment of unicentric lesions is mainly complete surgical excision and the prognosis is excellent with some chances of recurrence. There are less than one dozen of reported pleural castleman reported in the literature. This case evidence than unicentric lesion may exhibit constitutional symptoms and confirmed than lesions above 5 cm can be removed by VATS but the chances for conversion to an open thoracotomy are high.

Unicentric castleman's disease located in the lower extremity: a case report

BackgroundCastleman's disease is a rare form of localized lymph node hyperplasia of uncertain etiology. Although the mediastinum is the most common site of involvement, rare cases occurring in lymph node bearing tissue of other localization have been reported, including only a few intramuscular cases. Unicentric and multicentric Castleman's disease are being distinguished, the latter harboring an unfavorable prognosis.Case PresentationHere, we present a case of unicentric Castleman's disease in a 37-year-old woman without associated neoplastic, autoimmune or infectious diseases. The lesion was located in the femoral region of the right lower extremity and surgically resected after radiographic workup and excisional biopsy examinations. The tumor comprised lymphoid tissue with numerous germinal centers with central fibrosis, onion-skinning and rich interfollicular vascularization. CD23-positive follicular dendritic cells were detected in the germinal centers and numerous CD138-positive plasma cells in interfollicular areas. The diagnosis of mixed cellularity type Castleman's disease was established and the patient recovered well.ConclusionsIn conclusion, the differential diagnosis of Castleman's disease should be considered when evaluating a sharply demarcated, hypervascularized lymphatic tumor located in the extremities. However, the developmental etiology of Castleman's disease remains to be further examined.