Castleman's disease and spinal cord compression: case report

OBJECTIVE AND IMPORTANCE: Castleman's disease is a rare lymphoproliferative disorder most often found in the mediastinum. Localized forms are usually benign, whereas multicentric forms may be aggressive. We report a patient with Castleman's disease who presented with spinal cord compression, and we review previously published cases of Castleman's disease involving the central nervous system. To our knowledge, this is only the second case of Castleman's disease presenting as a spinal epidural mass with cord compression. CLINICAL PRESENTATION: A 44-year-old otherwise healthy woman presented acutely with difficulty walking. Examination revealed mild myelopathy in her legs. Magnetic resonance imaging revealed a posterior epidural mass compressing the thoracic spinal cord at T3-T5. INTERVENTION: Thoracic laminectomy and gross total resection of the lesion were performed. Pathological examination of the lesion identified the hyaline-vascular type of Castleman's disease. The patient's symptoms resolved postoperatively. CONCLUSION: Castleman's disease presenting as a spinal epidural mass lesion with cord compression is rare. Surgical treatment can result in an excellent outcome.     

Castleman's disease: unusual case of inflammatory spinal dorsal epidural mass: case report

OBJECTIVE AND IMPORTANCE: Castleman's disease (giant lymph node hyperplasia) is a rare, heterogeneous, lymphoproliferative disorder of unknown cause and pathogenesis. Most cases occur as mediastinal masses, although extrathoracic involvement, including nodal and extranodal locations, has been reported. The localized variants (solitary lesions) respond well to surgical excision. CLINICAL PRESENTATION: We report the case of a 34-year-old man with headache, intermittent febrile sense, progressive weakness of the legs, and urinary incontinence. Magnetic resonance imaging disclosed an enhancing dorsal extradural mass with impingement on the spinal cord at the T2-T3 level. Other abnormal laboratory findings were increased erythrocyte sedimentation rate and mild leukocytosis. TECHNIQUE: The mass lesion was removed; the histopathological findings included diffuse lymphoid cell infiltration and vascular proliferation, which are compatible with Castleman's disease. CONCLUSION: Although the dorsal epidural site of this case is very unusual, Castleman's disease was considered the most appropriate diagnosis on the basis of the associated systemic findings. This patient with dorsal epidural Castleman's disease may be the first reported case in the literature.     

胸部局限性Castleman病的临床特征及外科治疗

目的 探讨胸部局限性Castleman病的临床特征及外科治疗方法.方法 回顾性分析1996年6月至2008年11月收治的32例胸部局限性Castleman病患者的临床资料,探讨该病的临床和病理特点,总结其外科治疗效果.32例患者中男性14例,女性18例,年龄16～48岁,平均34.2岁.其中8例患者表现为胸闷、气短;3例患者表现为刺激性干咳;2例患者表现为胸痛;14例患者无临床症状,为体检时偶尔发现.CT检查均发现纵隔或肺门单发肿瘤.结果 除1例因术中麻醉意外死亡外,其余患者全部行手术完整切除肿瘤,病理改变符合Castleman病,病理类型以透明血管型为主.5例患者伴有副肿瘤性天疱疮,其中3例出现闭塞性细支气管炎;常规激素治疗均尤效,肿瘤切除后药物治疗1～2个月皮损明显好转,但肺部病变持续加重.术后随访全部患者均未见肿瘤复发.结论 胸部局限性Castleman病是一种罕见的胸部疾病,副肿瘤性天疱疮和肺部异常为Castleman病的少见而严重的合并症,手术切除肿瘤为首选治疗.     

Pancreatic Castleman's tumour: an unusual case

We present an uncommon case of hyaline vascular type Castleman's disease mimicking a pancreatic tumour. A 56-year-old woman with constitutional symptoms was investigated. Pre-operative interventions failed to produce a definitive diagnosis. Surgical excision was performed and the tumour was diagnosed to be the hyaline vascular type of Castleman's disease histopathologically. Pancreatic Castleman's disease should remain a consideration in the differential diagnosis of a pancreatic mass.     

Hyaline vascular Castleman's disease of the mediastinum.

Castleman's disease is rare and can be present in many sites and with a variety of symptoms. Surgery is always recommended for localized lesions to remove the mass as completely as possible, reserving other treatment modalities for unresectable cases.     

腹部局限性Castleman病副肿瘤性天疱疮八例临床分析

目的 探讨腹部局限性Castleman病副肿瘤性天疱疮的临床表现和临床治疗方法。方法 对我院收治的8例伴有腹部局限性Castleman病的副肿瘤性天疱疮患者的临床资料进行分析,探讨该病的临床和病理特征,总结其外科治疗效果。结果 8例患者均有典型天疱疮皮肤损害,伴有难治性口唇黏膜溃疡、糜烂;病理改变表现为表皮内疱、基底细胞液化变性,表皮内散在坏死的角质形成细胞及真皮浅层以淋巴细胞浸润为主。8例患者CT检查均发现内脏单发肿瘤。5例出现阻塞性细支气管炎。所有患者常规激素治疗无效,肿瘤切除后药物治疗1～2个月皮肤及黏膜损害治愈,肺部病变缓解,切除的肿瘤位于腹膜后,病理改变符合Castleman病。结论 腹部局限性Castleman病副肿瘤性天疱疮是一罕见疾病,发现和切除合并的内脏肿瘤是治疗的关键。     

A case of incidentally detected Castleman's disease with retroperitoneal paravertebral localization.

Castleman's disease, or angiofollicular lymph node hyperplasia, is a fairly rare benign tumor of lymphoid origin. The expected localization is mediastinum and rarely retroperitoneum. An asymptomatic case is reported with multimodality imaging and postoperative findings. The lesion was detected incidentally on routine chest radiogram. Surgical exploration revealed a retroperitoneal mass and the mass was resected successfully through a thoracoabdominal flank incision. Castleman's disease should be included in the list of differential diagnosis of retroperitoneal masses which are mostly malignant tumors.     

Hyaline vascular type of Castleman's disease confined to the kidney

A 59-year-old female was referred to our department for a renal mass which was incidentally found during pneumonia treatment. Computed tomography and magnetic resonance imaging showed a 1.5-cm enhancing mass in the posterior midportion of the left kidney. Subsequently, an open partial nephrectomy was performed on the diagnosis of renal cell carcinoma. Pathological examinations revealed a hyaline-vascular type of Castleman's disease. Castleman's disease confined to the kidney is exceptionally rare. We present this rare case and review the literature concerning this unusual disorder.     

Castleman's disease in children

A three and one-half-year-old girl and a 12-year-old boy presented with features of the two clinical presentations of Castleman's disease or giant lymph node hyperplasia. The girl presented with anemia, fever, night sweats, hypergammaglobulinemia, and a palpable abdominal mass. Her symptoms were consistent with those seen in the plasma-cell type of this disease. The boy presented with acute appendicitis and a left hilar mass was noted on his chest radiograph. His asymptomatic presentation was typical of the hyaline-vascular form. Both patients are well without evidence of recurrence four years following resection. Castleman's disease is a benign disorder of lymph nodes that occurs rarely in children. Since the original report in 1954 we could find only 18 cases in the 16-year and younger age group and our patient with the symptomatic form represents the youngest patient reported in the English literature. The management requires surgical resection of the enlarged nodes both for diagnosis and therapy since the enlarged nodes can mimic malignant tumors of the lymphoid system. No recurrences have been reported in pediatric patients.     

Membranous nephropathy associated with giant lymph node hyperplasia. A case report with histological and ultrastructural studies

A young woman presented with nephrotic syndrome due to membranous nephropathy and a localized form of giant lymph node hyperplasia (Castleman's disease) occurring as a large intra-abdominal mass. Five months after surgical removal of the mass, only mild proteinuria persisted. Twenty months later a second kidney biopsy showed a near-normal morphology. Six years later the patient was free of symptoms and had normal urinalysis. Reviewing the pertinent literature, it seems to be the first case of a biopsy-proven cure of membranous nephropathy in a patient with membranous nephropathy and Castleman's disease.     

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.     

Multicentric Castleman's disease found by a renal sinus lesion: a case report

A 79-year-old man complained of anorexia and weight loss. Computed tomography and magnetic resonance imaging showed a solitary mass in the left renal sinus. Left nephrectomy was carried out under a clinical diagnosis of renal pelvic tumor. The pathological diagnosis was plasma cell type of Castleman's disease. The patient suffered from lymph node swelling in the right neck at age 75 and the lymph node was resected. The pathological diagnosis was lymphadenitis but it proved to be plasma cell type of Castleman's disease. The patient also had had a lymphadenectomy in the supraclavicular fossa and in the supraorbital area at age 76. Both had been diagnosed as lymphadenitis pathologically but they proved to be plasma cell type of Castleman's disease. The final diagnosis was multicentric Castleman's disease. We reviewed multicentric Castleman's disease.     

Castleman's disease: an unusual retroperitoneal mass

Castleman's disease (angiofollicular lymph node hyperplasia) rarely presents as an isolated retroperitoneal mass radiographically indistinguishable from retroperitoneal malignancies. We report a case of the hyaline vascular variant of Castleman's disease presenting as a calcified pararenal mass. Computerized tomography and nuclear magnetic resonance imaging of this lesion are presented, and its clinical behavior and management are discussed.     

Pseudo-adrenal incidentaloma: magnetic resonance imaging in a patient with para-adrenal Castleman's disease.

We report a case of para-adrenal angiofollicular lymph node hyperplasia (Castleman's disease) of the hyaline-vascular type. The mass could not be differentiated from an adrenal tumor by ultrasonography and computed axial tomography (CT). However, magnetic resonance imaging (MRI) suggested the possibility of an extra-adrenal origin of the mass. The intensity of the mass by MRI was homogeneous and of a higher intensity in the T2-weighted image than in the T1-weighted image, a finding similar to lymphadenopathy, lymphatic tumorous mass or metastatic tumor of the lymph node. Ultrasonography, CT and MRI may not be useful in characterizing Castleman's disease, but MRI was useful to distinguish asymptomatic para-adrenal masses from those of adrenal origin.     

腹膜后Castleman病（附3例报告）

目的提高对腹膜后Castleman病的认识,探讨其诊治方法。方法报告北京友谊医院收治的3例腹膜后Castleman病患者的临床资料,并结合有关文献进行分析。结果3例均成功行手术切除并经病理证实。术后随访12～16个月未发现肿瘤复发。结论腹膜后Castleman病临床上少见,肿块可发生在淋巴组织的任何部位。CT扫描是重要的影像学诊断方法,手术切除肿瘤为首选治疗手段。     

Castleman's disease in the left suprarenal region, mimicking an adrenal neoplasm

We present a rare case of a 16-year-old male patient with Castleman's disease (CD) manifest by a suspicious mass within the left suprarenal region abutting the left adrenal gland, mimicking an adrenal neoplasm. CD is an uncommon and poorly understood lymphoproliferative disorder that, when observed, typically occurs as a mediastinal mass. Two percent of cases reportedly occur in the pararenal region. Although rare, Castleman's disease should be considered in the differential diagnosis of a retroperitoneal mass.     

Unicentric Castleman''s disease with leiomyosarcoma: A rare association

A 54-year-old asymptomatic woman was first diagnosed with unicentric Castleman's disease at 51 years of age. The disease was identified incidentally behind the inferior vena cava after a computed tomography (CT) scan. She was followed conservatively and no enlargement of the mass was observed. However, a follow-up CT scan revealed another solid mass in the left retroperitoneal area. Both tumors were surgically removed in the same session. The retrocaval mass was histologically confirmed to be Castleman's disease of the hyaline vascular type. Histological examination of the left peritoneal mass revealed low-grade leiomyosarcoma. We present here a case of leiomyosarcoma associated with unicentric Castleman's disease, both of which were localized in the retroperitoneum.     

Hyaline Vascular-Type Castleman's Disease in the Hepatoduodenal Ligament: Report of a Case

Castleman's disease is an uncommon lymphoproliferative disorder, which occurs most commonly in the mediastinum. Sporadic reports of its occurrence in the hepatoduodenal ligament and other extrathoracic locations have also been documented. Ultimately, Castleman's disease can develop anywhere lymphoid tissue is found, and the preoperative diagnosis is often difficult. We report a case of hyaline vascular-type Castleman's disease in the hepatoduodenal ligament. An asymptomatic 26-year-old woman was admitted to our hospital for further examination of a mass found on ultrasonography. A contrast-enhanced computed tomography scan confirmed a hypervascular mass in the hepatoduodenal ligament, and angiography showed that the mass was receiving its blood supply from various arteries. These findings suggested a diagnosis of Castleman's disease and we removed the tumor without resecting any other organs. Computed tomography and angiography were helpful in establishing a correct preoperative diagnosis, which minimized the magnitude of surgery.     

Castleman's Disease with Myasthenia Gravis

Castleman's disease is a rare disorder characterized by benign tumors that may develop in the lymph node tissue throughout the body. Castleman's disease associated with myasthenia gravis is an especially rare disease. Only less than 10 cases have been reported in the world literature. The cause of Castleman's disease is associated with immune mediated reaction, and myasthenia gravis also develops due to an antibody-mediated process. The cause of myasthenia gravis is the immune activity of Castleman's disease, which may be the promoter of the antibody-mediated process. We report here a case of Castleman's disease, which was incidentally found in a patient diagnosed with myasthenia gravis.     

Retroperitoneal Castleman's disease. A report of two cases and analysis of the literature

Castleman's disease is a rare disorder of the lymphoid tissue with three possible histological variants--the hyalin-vascular type, with a good prognosis, the plasma-cellular type and the mixed type; the latter two are both more aggressive than the hyalin-vascular type. Two clinical types of this disease have already been described: the localized or unifocal type and the multicentric or multifocal type. The aetiology of Castleman's disease remains unclear due to polymorphic clinical features that give rise to many diagnostic and treatment problems. Its diagnosis, therefore, can only be confirmed by histological examination. Surgical treatment is the treatment of choice in patients with Castleman's disease, but radical removal of the tumour mass, especially in the multicentric type, is not always possible. We are still in no position to draw definitive conclusions as to treatment, because there are only a few reports with different regimens regarding patients with multicentric Castleman's disease. A better understanding of the pathogenesis of this rare disorder may help in deciding the best treatment approach. In this study, we report two cases of Castleman's disease, one hyalin-vascular and the other plasma-cellular, both of which were unifocal and located in a retroperitoneal-pararenal site. We also analyse the main clinical, diagnostic and treatment problems associated with this rare condition, with an overview of the literature.