Castleman Disease: A Rare and Intriguing Malady: A Case Series

Castleman disease (CD), first described by Benjamin Castleman as angiofollicular mediastinal lymph杗ode hyperplasia, is a rare benign lymphoproliferative disorder with varied modes of presentation. Its common presentation within the mediastinum misleads the clinician and merits special attention since it is essentially a diagnosis of exclusion. We are sharing our experience with three patients, within a relatively short period of 2 years. All three presented with a mediastinal mass, however, each of them came with an entirely different clinical scenario and diagnosis. All three were successfully operated and Castleman disease [hyaline杤ascular (HV) type] was diagnosed only after the final histopathology.

Clinicopathological Features of Follicular Dendritic Cell Sarcoma / 中国医学科学院学报

To explore the clinicopathological and immunohistochemical characteristics of follicular dendritic cell sarcoma(FDCS)and the expressions of IgG and IgG4. We retrospectively analyzed the clinicopathological and immunohistochemical data of 9 pathologically confirmed FDCS cases in Peking Union Medical College Hospital from January 2005 to December 2018.Immunohistochemical staining of IgG and IgG4 were performed,and Epstein-Barr virus(EBV)-encoded RNA(EBER)in situ hybridization were carried out. Nine cases of FDCS included 4 men and 5 women aged 16-53 years [mean(38.2±9.7)years].The clinical manifestations included masses,lymph node enlargement,rash,and fever.The tumors were located in lymph node,retroperitoneal region,adrenal gland,neck,axillary region,and liver,respectively.Ultrasound showed clear boundary cystic or solid mass with maximum diameters of 1.5-15.0 cm.Microscopically,the spindle tumor cells were arranged in solid and storiform patterns with abundant and slightly stained cytoplasm,vacuolated nuclei,and small nucleoli.The mitosis was 1-3/10 high power fields,and necrosis was found in 5 cases.Immunohistochemically,the tumor cells were positive for CD21(6/9),CD35(6/9),and CD23(7/9). FDCS is a rare malignant tumor,which is easy to be missed.The combination of CD21,CD35,and CD23 is helpful for diagnosis.Hyaline-vascular type Castleman's disease may be the precursor of FDCS,and there may be only a small number of IgG4-positive plasma cells in FDCS.Surgical resection remains the main treatment for FDCS.

Unicentric Castleman’s disease presenting as retroperitoneum lump.

Castleman disease is a rare lymphoproliferative disorder, also known as giant lymph node hyperplasia or angiofollicular lymph node hyperplasia. Clinically, Castleman disease is of two types: localised/unicentric type and multicentric/systemic type. Unicentric or localised Castleman disease affect a single lymph node or group of lymph nodes. The multicentric type affects two or more groups of lymph node in different part of the body. It can also affect organs containing lymphoid tissue. Histologically it is classified as hyaline vascular variant, plasma cell variant and a mixed variant. Clinical symptoms may vary from asymptomatic to symptomatic lymphadenopathy accompanied by fever, anaemia fatigue, abdominal or thoracic pain and weight loss. There is no specific test to diagnose Castleman disease. We report a case of 16 years old male who presented with a painless lump in left lumbar region without any constitutional symptoms. CECT suggested a retroperitoneal lump with calcification. Laparotomy was done and complete excision of mass done. Histopathological examination of excised tissue suggested Castleman disease of hyaline vascular variant. After six month of follow up, the patient has no complain.

Cases report of unicentric Castleman's disease: revisit of radiotherapy role

Castleman's disease or angiofollicular lymph node hyperplasia is a rare lymphoproliferative disorder. Complete surgical resection was recommended in unicentric Castleman's disease. Radiotherapy was considered alternative therapeutic option. However, there have been consistent favorable responses to radiotherapy. We also experienced two cases of uncentric Castleman's disease salvaged successfully with radiotherapy. This paper described these cases and reviewed the literature about Castleman's disease treated with radiotherapy. Reviewed cases showed that radiotherapy is a successful treatment option in unicentric Castleman's disease. Furthermore, our report confirms the radiotherapy role in uncentric Castleman's disease.

Castleman disease / 대한혈액학회지

Castleman and Towne described a disease presenting as a mediastinal mass resembling thymoma. It is also known as "giant lymph node hyperplasia", "lymph node hamartoma", "angiofollicular mediastinal lymph node hyperplasia", and "angiomatous lymphoid hyperplasia". The pathogenesis is unknown, but the bulk of evidence points toward faulty immune regulation, resulting in excessive B-lymphocyte and plasma-cell proliferation in lymphatic tissue. In addition to the mediastinal presentation, extrathoracic involvement in the neck, axilla, mesentery, pelvis, pancreas, adrenal gland, and retroperitoneum also have been described. There are 2 major pathologic variations of Castleman disease: (1) hyaline-vascular variant, the most frequent, characterized by small hyaline-vascular follicles and capillary proliferation; and (2) the plasma-cell variant, in which large lymphoid follicles are separated by sheets of plasma cells. The hyaline-vascular cases usually are largely asymptomatic, whereas the less common plasma-cell variant may present with fever, anemia, weight loss, and night sweats, along with polyclonal hypergamma-globulinemia. Castleman disease is a rare lymphoproliferative disorders. Few cases have been described world widely. In this article we reviewed the classification, pathogenesis, pathology, radiological features and up to date treatment with special emphasis on the role of viral stimulation, recent therapeutic modalities and the HIV-associated disease.

Hyaline Vascular Castleman Disease Involving Renal Parenchyma and a Lymph Node: A Case Report

Castleman disease is a rare lymphoproliferative lesion that is predominantly found in the mediastinum. Retroperitoneal and pararenal localizations are very rare. We describe a 36-year-old man with a hyaline vascular type of Castleman disease involving renal parenchyma and a paraaortic lymph node. Most reported renal Castleman disease was plasma cell type with systemic symptoms. Herein, we report the first Korean case of the hyaline vascular type of Castleman disease involving the renal parenchyma and the paraaortic lymph node simultaneously.

Castleman's disease - hyaline vascular type - clinical, cytological and histological features with review of literature.

Background and Aims: Castleman's disease (CD) is a rare disease of lymph node with two identified forms - the hyaline vascular (HV) type or angiofollicular type and plasma cell (PC) type. It usually presents as localized or systemic lymphadenopathy or even as extra nodal mass and may give rise to several differential diagnoses. Fine needle aspiration cytology (FNAC), as the initial investigation, may be misleading. Excision biopsy usually gives the diagnosis. This study analyzes the clinical, cytological and histological features of CD of HV type Materials and Methods: All the cases of CD reported between 2001 and March 2008 have been reviewed. Cytology and histopathology slides of all cases were reviewed by two reporting pathologists independently and the clinical records were analyzed. Result: We had five cases of CD all of which presented as cervical lymphadenopathy of 1 to 5 cms in young patients. Patients were mostly asymptomatic with all relevant investigations within normal limits. The cytology findings mostly showed a predominance of small lymphocytes with capillary fragments. On excision biopsy, all cases were diagnosed as CD of HV type. Conclusion: CD should be kept as a differential of lymphadenopathy, especially in asymptomatic and young patients.

Características clinicopatológicas de la enfermedad de Castleman: experiencia en el Instituto Nacional de Cancerología / Clinical and pathological features of Castleman's disease: experience at the Instituto Nacional de Cancerología, Mexico City

Introducción: La enfermedad de Castleman es una entidad patológica poco comprendida, descrita originalmente en pacientes europeos. Informamos nuestra experiencia con esta entidad clinicopatológica en pacientes del Instituto Nacional de Cancerología de la Ciudad de México. Material y métodos: Analizamos retrospectivamente los expedientes de pacientes con enfermedad de Castleman de 1996 a 2003. La enfermedad fue monocéntrica si había solo un ganglio o multicéntrica si se encontraba linfoadenopatía generalizada. Además, se dividió en las variantes histológicas hialinovascular y de células plasmáticas. Resultados: Once pacientes con enfermedad de Castleman fueron diagnosticados en el periodo referido, seis tenían enfermedad monocéntrica y cinco multicéntrica. La mediana de seguimiento fue de 40 meses. Todos los pacientes con enfermedad monocéntrica tenían la variante hialinovascular. De los cinco con multicéntrica, cuatro tenían la variante de células plasmáticas y uno la hialinovascular. Cinco pacientes con enfermedad monocéntrica se trataron con cirugía y uno con quimioterapia; al momento de este informe todos permanecían vivos y sin enfermedad. Tres pacientes con enfermedad multicéntrica recibieron quimioterapia y dos, quimioterapia más radioterapia por enfermedad residual; a dos pacientes se les prescribió quimioterapia de segunda línea, con buena respuesta. Dos pacientes con una condición asociada evolucionaron desfavorablemente. Conclusiones: Las características clínicas, patológicas y los resultados del tratamiento son similares a los señalados en otras poblaciones.

BACKGROUND: Castleman's disease (CD) is a rare, poorly understood pathological entity. We report our experience with this clinicopathological entity. METHODS: We retrospectively analyzed records of all patients with CD from 1996 to 2003. The disease was classified as unicentric if a solitary mass was present or multicentric if generalized lymphadenopathy was present. We further subdivided the disease into hyaline vascular (HV) and plasma cell (PC) histological variants. RESULTS: We found 11 patients with CD. Six patients had unicentric disease and five had multicentric disease. Median follow-up was 40 months. All patients with unicentric disease had the HV variant. Of the five patients with multicentric disease, four had the PC variant and one had the HV. Five patients with unicentric disease were treated surgically with complete resection, and only one patient was treated with chemotherapy. All remain alive without disease. Three patients with multicentric disease were treated with chemotherapy, and two patients received chemotherapy plus radiotherapy for residual disease. Two patients received second-line chemotherapy with a favorable outcome. Two patients with a comorbid condition had a poor outcome. CONCLUSIONS: Clinical characteristics, pathological features and treatment results are similar to that reported in other populations.

Laparoscopic Treatment of Mesenteric Castleman's Disease

Castleman's disease (CD) is an uncommon lymphoproliferative disorder of unknown origin. There are two histological types: hyaline-vascular type and plasma cell type. CD is usually located in the mediastinum, but may be seen in any site including the neck, axilla, mesentery, and retroperitoneum. A 52-year-old male complained of vague lower abdominal pain. There was no palpable mass and all laboratory data showed nonspecific findings. Abdominal computed tomography scan showed a solitary homogenous, well-defined mass in the mesentery. The laparoscopic complete resection was performed without complications. Histologic examination of resected lesion revealed the hyaline-vascular type of CD. In the hyaline-vascular type of CD, laparoscopic approach constitutes a complete treatment. We present here the case of laparoscopic treatment of isolated mesenteric CD.

Two Cases of Orbital Castleman's Disease

PURPOSE:To report two cases of multicentric Castleman's disease involving the orbital area. CASE SUMMARY: A 52-year-old man presented with discomfort of the right eye on eyeball movement that began 4~5 months ago. He had been diagnosed as Castleman's disease upon the biopsy of his neck lymph nodes 2 years ago. Proptosis was found in his right eye, and orbital computed tomography revealed an enlarged mass in the inferior orbit compared to the former image. An incisional biopsy confirmed Castleman's disease. A 57-year-old man presented with left eyelid swelling that began 3 months ago. Orbital and chest computed tomography revealed a mass in the left superotemporal orbit and the enlarged lymph nodes of the mediastinal area. Castleman's disease was confirmed by biopsy of the lymph nodes in the mediastinal area. CONCLUSIONS: Steroid therapy was prescribed for the 52-year-old man. The 57-year-old man was placed under close observation.

Enfermedad de castleman: a propósito de un caso / Castleman disease: a purpose of a case

La enfermedad de Castleman o hiperplasia angiofolicular se caracteriza por hiperplasia linfoide reactiva, crecimiento de tumores benignos del tejido linfático y una mayor predisposición a padecer linfomas. Descrita por Benjamín Castleman en 1956, de etiología desconocida, con probable relación con el herpes virus tipo 8, fallo en la inmunorregulación, expresión aumentada del gen codificador de interleukina-6. En pediatría es excepcional. Clínicamente se distinguen las formas multicéntrica y la localizada (70 por ciento de los casos), de buen pronóstico, localizada en mediastino, cuello, abdomen, menos frecuente en axila, pelvis y páncreas; la resección de la lesión es curativa. Histológicamente se clasifica en dos tipos: hialinovascular (la más frecuente), y variedad de células plasmáticas. Se revisó la literatura y se presentan dos casos clínicos. Caso nº 1: escolar de 6 años, quien desde los 18 meses de vida presentaba masa tumoral en axila izquierda de 0,5 cm. la cual fue resecada a los 4 años. A los 6 años recidivó hasta medir 7 x 4 cms, realizándose exéresis. Presentó además hipergammaglobulinemia, bajo nivel de células NK y del índice CD4/CD8. Caso nº 2: pre-escolar masculino de 4 años, con masa tumoral en axila derecha de 1 cm. de 6 meses de evolución. Se le realizó biopsia excisional. En ambos casos el estudio anatomopatológico e inmunohistoquímico reportó Enfermedad de Castleman de variedad hialinovascular. Los pediatras y cirujanos pediatras debemos maximizar la vigilancia de adenomegalias que puedan ser lesiones centinelas de afección inmunológica o neoplásica curables si son tratadas precozmente.

Castleman’s disease or angiofolicular hiperplasy is characterized by reactive lymphoid hyperplasia, benign tumors of lymphoid tissue and predisposition to develop lymphomas. Described by Benjamin Castleman in 1956, it is of unknown etiology, probably related to herpes virus type 8, immunoregulation failure, increased expression of 6-interleukin gene. Very rare in childhood, the disease has two different clinical types: a multicentric type, and a localized type (70% of the cases). The latter with good prognosis, located inmediastinum, neck, abdomen, and less frequently in axila, pelvis and pancreas. Treatment consists in the resection of the lesion. The histological types are the hyaline-vascular type (most frequent) and the plasma cells type. Literature was reviewed and two clinical cases are reported: Case nº1: 6 year old child, who presents at 18 months of age with a 0.5 cm bulk in his left axila. The lesion was removed surgically at 4 years of age, with reappearance of a 7 x 4 lesion which was removed at 6 years of age. This child had also hipergammaglobulinemia, low levels of NK cells and of the CD4/CD8 index. Case nº2: 4 year old child, who presented with a 1 cm mass in his right axila of 6 months of evolution. An excisional biopsy was performed. In both cases the histological study reported Castleman’s disease of hialinovascular variety. Pediatricians and pediatric surgeons must follow very closely the growth of lymphoid tissue that may represent immunological. or neoplastic lesions, potentially curable if diagnosed and treated early.

Giant Lymph Node Hyperplasia (Castleman's Disease) as a Rare Cause of Back Pain

Giant lymph node hyperplasia (Castleman's disease) is a nonneoplastic lymphoproliferative disorder of unknown etiology that usually occurs in the chest. Its morphological recognition is based on a composition of various histological features. The mass is often asymptomatic, but it can cause nonspecific thoracic symptoms, such as regional pain. This disease can be found wherever lymph nodes are present, but two-thirds of these tumors are found in the chest, along the tracheobronchial tree in the mediastinum or lung hilus. However, we experienced an unusual case of Castleman's disease as a cause of back pain that was localized in the posterior mediastinum bordering the chest wall.

Ileal Mesenteric Castleman's Disease

Castleman's disease is a rare disorder characterized by tumorous masses that may develop in the lymph node tissue throughout the body. Most common location is mediastinum, but it can also affect retroperitoneum, neck, pelvis, and/or axilla. It may exceptionally affect extranodal sites like striated muscle, thoracic wall, lungs, skull, larynx, and/or vulva. The presentation is varied and diagnosis is difficult. There are two main types of Castleman's disease: hyaline-vascular type and plasma cell type. The hyaline vascular type accounts for approximately 90% of the cases. Most individuals exhibit no symptoms of this form of the disorder or they may develop non-cancerous growths in the lymph nodes. The plasma cell type is often associated with fever, weight loss, skin rash, early destruction of red blood cells, leading to unusually low levels of circulating red blood cells (hemolytic anemia), and/ or abnormally increased amounts of certain immune factors in the blood (hypergammaglobulinemia). We here report a case of Castleman's disease of ileal mesentery in 30-years old female patient. Abdominal mass, 4.7x3.6 cm in size, was completely removed from ileal mesentery without complication, and confirmed histologically mesenteric Castleman's disease of the mixed type.

A Case of Mesenteric Castleman's Disease with Systemic Involvement / 대한소아혈액종양학회지

Castleman's disease (CD) is a lymphoproliferative disorder of unknown origin and rarely occurs in children. It has been further classified into two major subgroups: solitary or localized and multicentric CD. Furthermore, there are two main histological variants: hyaline-vascular, plasma cell types. Clinically, hyaline-vascular type is rarely associated with systemic symptoms, but the plasma cell type is frequently associated with the constitutional symptoms of fever, malaise, night sweat and the abnormal laboratory markers. Surgical excision of the affected lymph node plays an important role in the treatment of this disease. We encountered a case of the hyaline-vascular type CD located in the mesentery with systemic involvement. The clinical and biochemical abnormal findings improved after surgical resection of the involved lymph node.

Axillary Castleman's Disease

Castleman's disease is a giant lymph node hyperplasea that was initially described by Castleman in 1956. Although the mediastinum was the site of disease in the majority of patients, these tumors may develop wherever lymph nodes are present; specifically, the retroperitoneum as well as the cervical, pelvic, and axillary regions. Microscopically, two distinct histologic entities have been described; (1) the hyaline vascular type characterized by small hyaline follicles and interfollicular capillary proliferation; and (2) the plasma cell type characterized by large follicles with sheets of plasma cells. A 3-year old male patient was admitted due to a palpable right axillary mass of 45 days duration. An axillary mass, 5X3X2.5 cm in size, was completely removed from the right axillary region without complication, and was comfirmed histopathologically as axillary Castleman's disease of the hyaline vascular type.

Retroperitoneal Castleman's Disease with Pure Red Cell Aplasia

Castleman's disease is a distinct lymphoproliferative disorder of unknown origin, which creates both a diagnostic and therapeutic dilemma for most physicians. Here, we present a case of hyaline-vascular and solitary Castleman's disease associated with pure red cell aplasia. A 49-year old woman was admitted suffering from severe anemia. A bone marrow biopsy showed marked erythroid hypoplasia. A solitary retroperitoneal mass was excised and proven to be Castleman's disease with hyaline-vascular type histology. Removal of the mass led to a rapid reversal of anemia. No evidence of recurrence was found 1 year after the excision.

Castleman's Disease (Giant Lymph Node Hyperplasia): Report of 2 cases and review of the literature

Two cases of Castleman's disease (Giant lymph node hyperplasia) are reported. The first case was a multicentric cervical giant lymph node hyperplasia. Excisional biopsy confirmed the diagnosis of Castleman's disease of the plasma-cell type. Despite repeated dissection of the cervical lymph nodes, complete removal of the lymph nodes was impossible, and the type was changed into mixed type, and regrowth of the tumor was noted. Prednisolone was of no benefit. The patient is now living with cervical masses in situ without general symptoms, 4 years after the initial diagnopsis. The second case was a solitary retroperitoneal giant lymph node hyperplasia, incidentally found. Complete excision was performed and biopsy confirmed the diagnosis of Castleman's disease of hyaline-vascular type. No recurrence was found at 2 years after the excision.

CT Diagnosis of Hyaline-vascular Type of Giant Lymph Node Hyperplasia(A Report of 4 Cases and A Review of Literature) / 实用放射学杂志

Objective To expore CT diagnostic values for hyaline-vascular type of giant lymph node hyperplasia(GLNH).Methods CT findings in 4 patients with hyaline-vascular type of GLNH confirmed by surgery and pathology were respectively analyzed and relevant literatures were reviewed.CT examination included plain and enhanced scan with contrast medium bolus injection.Results Lesions were located 2 at the posterior mediastinum,1 at the restroperitonum and 1 at the neck.The characteristic CT appearances were a large soft tissue mass round or oval in shape with homogenous density,well and regular margin.The masses were all markedly enhanced after contrast,their attenuation were similar to but slightly lower than the larger arteries in the same section.Conclusion GLNH is an uncommon disease and it′s clinical diagnosis is difficult.But CT scanning could showed some features.When CT found a mass with benign characters locating in lymph node regions,especially marked enhancement,Hyaline-vascular type of GLNH should be considered.

Angiofollicular Lymph Node Hyperplasia(=Castleman's Disease): Report of A Case / 대한치료방사선과학회지

Angiofollicular lymph node hyperplasia (AFLNH) with well marginated lymphoid masses, is a rare benign disease of unknown etiology. The majority of the disease develop intrathoracically. Histologically this disease can be divided into the hyaline-vascular and the plasma cell types with the hyaline-vascular type prevailing. The plasma cell variant has been associated with nephritic syndrome, anemia, growth failure, fever, hyperglobulinemia, peripheral neuropathy, and hypoalbuminemia. Surgical resection is known to be treatment of choice in most cases, and radiotherapy is reserved for advanced, unresectable lesions. We report a complete remission of AFLNH in a case treated by surgical excision followed by irradiation.