Unicentric Castleman disease: A case report of an atypical presentation and successful management.

Castleman Disease (CD) is a rare benign disorder characterized by abnormal lymphoid tissue growth. It can be classified as unicentric CD (UCD) affecting a single lymph node region or multicentric CD (MCD) involving multiple regions. While UCD typically occurs in the mediastinum, MCD is associated with inflammatory symptoms and abnormal lab findings. We present a case of a young Syrian male with pelvic UCD, exhibiting unusual symptoms. Surgical excision led to complete recovery. A 27-year-old male presented with a well-defined pelvic mass discovered during routine ultrasonography. Symptoms included fever, fatigue, weight loss, and lower extremity numbness. Laboratory analysis revealed lymphocytopenia. Imaging identified a retroperitoneal pelvic mass, raising concerns of lymphoma. Surgical resection confirmed Castleman disease, specifically the hyaline vascular variant, dispelling malignancy concerns. Post-surgery, the patient demonstrated complete recovery, with normalized lymphocyte count and resolution of symptoms, affirming the effectiveness of the treatment. Castleman Disease (CD) is characterized by abnormal lymphoid tissue growth. It presents as Unicentric (UCD) or Multicentric (MCD) forms. UCD cases are usually asymptomatic or show compressive symptoms due to mass effect, while MCD is associated with systemic symptoms. The estimated UCD incidence is 16 cases per million person-years, with median age at diagnosis around 30-34 years. Histologically, UCD is categorized into hyaline vascular (HV) and plasma cell (PC) variants. In this case, systemic symptoms and lymphocytopenia deviated from typical UCD presentation, leading to initial lymphoma suspicions. Surgical intervention facilitated recovery without adjuvant therapies. This study emphasizes the spectrum of Castleman Disease (CD) manifestations, distinguishing Unicentric (UCD) and Multicentric (MCD) forms crucial for accurate management. It highlights atypical pelvic UCD presentation and successful surgical treatment's importance.

Muhammad T ，Alkheder A ，Mazloum A ，Almooay A ，Naziha L ，Shaheen M ... -  《International Journal of Surgery Case Reports》

Castleman disease: A single-center case series.

Castleman disease (CD) is a rare lymphocytic disorder. Unicentric CD (UCD) has an excellent long-term prognosis after surgical excision; however, multicentric CD (MCD) has a severe clinical course with poor outcomes. We analyzed the clinical presentation of 28 patients treated at a single institution from 1995 to 2017. Demographics, clinical variables, anatomical site, centricity, histopathology, immunochemistry, and surgical approach were reviewed. We evaluated the 5-year recurrence and survival for patients with UCD and MCD. Of the 28 patients, 57 % (n = 16) were female, with a mean age of 41.6 ± 15.6 years. CD was asymptomatic in 57 % (n = 16) of patients, 21 % (n = 6) presented with local symptoms such as pain, and 21 % (n = 6) of patients also had systemic symptoms, including weight loss and fever. CD was unicentric in 64 % (n = 18) and multicentric in 36 % (n = 10). The hyaline vascular variant was noted in 57 % (n = 16) of the tumors, plasmacytoid variant in 36 % (n = 10), and mixed variants in 7% (n = 2) of tumors. Anatomical distributions included: head and neck (20 %), thorax and axilla (24 %), retroperitoneal (13 %), abdominopelvic (30 %) regions, and other (13 %). Complete surgical resection was performed in 95 % of patients with UCD. Surgical biopsy and medical therapy were provided to all patients with MCD. The recurrence rate for UCD and MCD was 6 % (n = 1) and 14 % (n = 1), respectively. The five-year disease-free survival rate for UCD was 95 % (n = 19) and MCD was 33 % (n = 2). We found 100 % survival in patients with UCD and histology demonstrating the HV variant. CD is rare and often misdiagnosed due to the absence of specific clinical symptoms. Surgeons should include CD in their differential diagnoses when evaluating patients with lymph node hyperplasia. Surgery can be curative in nearly all patients with UCD. Patients with MCD require a combination of surgical therapy, chemotherapy, and immunotherapy; however, cytoreductive surgery benefits for patients with MCD have not been established.

Pribyl K ，Vakayil V ，Farooqi N ，Arora N ，Kreitz B ，Ikramuddin S ，Linden MA ，Harmon J ... -  《-》

Complete resection of unicentric Castleman disease in the superior mediastinum: A case report.

Castleman disease (CD) is a rare benign lymphoproliferative disorder characterized by benign lymph node hyperplasia in a single site (unicentric CD [UCD]) or in multiple sites (multicentric CD [MCD]). Patients with UCD are usually asymptomatic; however, those with MCD usually develop fever, weight loss, and peripheral lymphadenopathy. We describe a case of surgically resected UCD in the superior mediastinum in which the involved lymph node was surrounded by important vessels and trachea, necessitating a median sternotomy for complete resection of the tumor. Preoperative diagnosis of UCD in the thorax is very difficult, and surgical resection or excision is necessary. Complete resection is recommended for UCD because it is curative and has a 5-year survival rate of 100%. UCD should be included in the differential diagnosis of asymptomatic mediastinal tumors. Surgical resection or excision is preferred to ensure an accurate diagnosis and appropriate treatment if malignant disease cannot otherwise be ruled out.

Haro A ，Kuramitsu E ，Fukuyama Y 《-》

Hyaline Vascular Type of Unicentric Castleman Disease in a Kidney with End-Stage Renal Disease: A Case Report of a Rare Entity at an Unusual Location and a Special Clinical Setting.

Chen CH ，Li HN 《Diagnostics》

The French paediatric cohort of Castleman disease: a retrospective report of 23 patients.

Borocco C ，Ballot-Schmit C ，Ackermann O ，Aladjidi N ，Delaleu J ，Giacobbi-Milet V ，Jannier S ，Jeziorski E ，Maurier F ，Perel Y ，Piguet C ，Oksenhendler E ，Koné-Paut I ，Galeotti C ... -  《Orphanet Journal of Rare Diseases》