Castleman’s disease is a rare lymphoproliferative disorder. It typically presents as mediastinal masses and causes a wide range of clinical symptoms. Histologically, Castleman’s disease is classified as either a hyalinic vascular or plasma cell variant. The prognosis mainly depends on the histological type and broadly varies. We herein report our sonographic findings in a patient with Castleman’s disease, including gray-scale ultrasonography, color Doppler ultrasonography, and sonoelastography ultrasonography, which have not been previously reported in the literature. These findings allowed for a preoperative diagnosis and avoidance of overly aggressive therapy.
A 28-year-old European female patient with unicentric Castleman’s disease of hyalinic vascular type (HV) restricted to the axilla was referred to us because of a 4-month history of a painless, solitary mass located in the left axilla. The patient’s medical history was unremarkable.
Castleman’s disease is a pathologic entity of unknown etiology and pathogenesis. In this case report of unicentric HV-type CD, we demonstrate that typical sonographic findings can lead to a preoperative diagnosis of Castleman’s disease. Core needle biopsy usually allows for a final diagnosis and helps to avoid unnecessary operations and overtreatment.
Castleman’s disease; Giant lymph node hyperplasia; Ultrasonography; Core needle biopsy
Castleman’s disease is a type of non-neoplastic lymphoproliferative disease having lymph nodal hyperplasia. It has two distinct microscopic types: hyaline-vascular type and plasma cell type. Clinically, it may present either as a solitary mass, most commonly in the mediastinum, or as a multicentric form whose features are generalized lymph-adenopathy, splenomegaly and involvement of other organs like the lungs and kidneys. Here we report a case of isolated retroperitoneal Castleman’s disease, which presented as a lump in the iliac fossa in a young female. A clinico-radio-logical diagnosis of retroperitoneal soft tissue tumour was made and the patient underwent complete surgical excision. The exact diagnosis was only obtained at histopathology and there is no evidence of recurrence at six months follow-up.
Retroperitoneal; Castleman’s disease; Isolated; Hyaline vascular
Three histological variants (hyaline vascular, plasma cell, and mixed) and two clinical types (localized and multicentric) of Castleman's disease have been described. The risk of progression to lymphoma is higher in multicentric Castleman's disease and is associated with poorer outcomes and higher mortality rate. Multicentric Castleman's disease often requires systemic therapy. Complete resection of the involved node in localized Castleman's disease is curative, with no reported recurrences.
We report a case of a 66-year-old female with systemic symptoms and bilateral cervical lymph nodes which were initially diagnosed as the hyaline vascular variant of Castleman's disease and two years later after the initial diagnosis she was confirmed to B cell lymphoma. Following the treatment with radiation therapy to the cervical area and combination chemotherapy complete response was achieved.
Although it has rarely been reported, the malignant potential of the Castleman's disease must be kept in mind.
Castleman's disease is a rare benign lymphoproliferative disorder that frequently affects lymph nodes of the mediastinal thorax and the neck. It very rarely affects the renal sinus. We report a case of Castleman's disease arising in the renal sinus in a 64-year-old man. The patient visited the hospital with the chief complaint of hematuria. Abdominal computed tomography revealed a homogeneous mass in the sinus of the left kidney, radiologically interpreted as a malignant urothelial tumor. Subsequently, nephroureterectomy was performed, after which microscopic examination of the specimen revealed a diffuse lymphoproliferative lesion with reactive lymphoid follicles of various sizes and prominent plasma cell infiltration of interfollicular spaces, highlighted by immunohistochemical staining for CD138. The lesion was diagnosed as Castleman's disease of the plasma cell type. Although preoperative diagnosis of Castleman's disease is difficult and the incidence is exceedingly rare, it should be considered in the differential diagnosis of renal sinus tumors.
Giant lymph node hyperplasia; Plasma cell type; Renal sinus; Urothelial malignancy; Kidney
Castleman's disease is a distinct form of lymph node hyperplasia divided into a solitary and a multicentric type. The solitary type occurs most commonly in the mediastinum and is usually asymptomatic. We present a patient with Castleman's disease of the hyaline-vascular solitary type located in the retroperitoneum.
The patient was a 38-year-old male, who presented to our hospital with fever. The imaging workup revealed a retroperitoneal mass, measuring 4 × 6 cm, located lateral to the aorta, inferior to the left renal artery and vein, and posterior to the left testicular vein. At workup, a solid hepatic lesion, 3 cm in diameter, located in the left lobe of the liver, segment IV, was also identified. Both lesions were surgically excised. The retroperitoneal tumor had the features of angiofollicular hyperplasia (Castleman's disease), hyaline-vascular type, whereas a diagnosis of focal nodular hyperplasia was made for the hepatic lesion. The patient is well at fourty months followup postoperatively. Surgical excision is the treatment of choice for unifocal Castleman's disease.
A 78-year-old woman with B-symptoms was referred for a left adrenal incidentaloma of 5 cm. Imaging revealed features compatible with adrenal cancer. The authors excluded excess production of catecholamines or adrenal steroids. The tumour was removed by en bloc radical left retroperitonectomy with adrenalectomy, nephrectomy, interaortocaval lymphadenectomy and splenectomy. Histology demonstrated periadrenal hyaline vascular Castleman’s disease with local infiltration and 14 positive lymph nodes. The lymphoid infiltrate spilled into the adjacent renal cortex. HHV8 was negative. The Ki67 proliferative index was 30–40% in germinal centres. There was no syn- or metachronous disease on extended imaging including fluorodeoxyglucose positron emission tomography-CT and narrow follow-up at 3 years. This is a rare case of unicentric hyaline vascular Castleman’s disease with documented locoregional lymph node involvement. The case exemplifies the transition from unifocal unicentric disease into disseminated disease with involvement of multiple lymph node stations (multicentric disease). The authors demonstrate surgical cure by oncological resection.
Castleman's Disease (CD) is a rare lymphoproliferative disorder accompanied by marked systemic inflammatory response. Morphological diagnosis of CD requires biopsy of the whole of the involved lymph node tissue. Three histologic variants have already been described in CD morphology (hyaline vascular, plasma-cell, and mixed). In this study, we report a case of a multicentric Castleman's disease of the plasma cell variant type with negative Herpes Virus 8. The clinical presentation of this patient was of systemic amyloidosis as a result of both a delayed diagnosis and medical management. Previously described cases of CD with secondary amyloidosis have been of the localized type. Regardless, long-standing clinical remission of CD by cytotoxic drugs and anti-CD20 antibody therapy was achieved, but the nephrotic syndrome remained irreversible.
Castleman's disease is a rare entity with an unknown etiology which was first described by Castleman in 1954. It is a lymphoproliferative disorder histologically classified into three types; hyaline-vascular, plasma cell type and mixed type. It might be localized or multicentric and usually involves the mediastinum. We report a case of Castleman's disease discovered accidentally in a case of blunt chest trauma which caused a challenging diagnostic process and management.
acute respiratory distress; Castleman's disease; extra luminal airway obstruction; mediastinal mass.
Castleman’s disease (CD) is a rare lymphoproliferative disorder of unknown aetiology presenting with different clinical manifestations in young adults. There are two main types, unicentric occurring in a younger age group and multicentric occurring in older people. We report the case of 22-year-old man who initially presented with acute appendicitis. During his admission he had a routine chest radiograph which demonstrated a mass lesion, later diagnosed as Castleman’s disease of hyaline vascular type. He underwent a right pneumonectomy for complete excision and made an unremarkable recovery.
Castleman's disease or angiofollicular lymph node hyperplasia is a rare clinical entity that may present in many sites and with a variety of symptoms. We report here a case of unicentric Castleman's disease (hyaline vascular pattern) located in the mediastinum as a solitary mass. The patient was a Caucasian female 58 years old presented after incidental discovery of the mass in a x-ray. In Castleman's disease surgery is generally recommended for localised lesions to remove the mass as completely as possible reserving other treatment modalities for unresectable cases or multicentric disease.
Castleman’s disease, also known as angiofollicular hyperplasia, is a disease of unsettled complexity and morbidity, usually occurs in the mediastinum and less frequently in the neck as a solitary mass and is often associated with other illnesses. Two variants have been recognized, the hyaline vascular type which is mare common and the less common plasma cell type with a third hybrid or intermediate type that shares features with both. It has been described with AIDS and Kaposi’s sarcoma as well as follicular dendritic cell tumours.
We report a case of Castleman’s disease of the hyaline vascular type arising in the left supra-clavicular region in 41 year old male without any associated systemic illness. The neck is an uncommon location for this rare disease.
Giant lymphnode hyperplasia; Hyalin; Plasma cell
Castleman disease (CD) is a rare lymphoproliferative disorder of unknown etiology with different clinical manifestations. A previous healthy 50 year-old man was hospitalized for right upper quadrant (RUQ) abdominal pain. He had jaundice and a 1 cm-sized lymph node in the right supraclavicular area. Pancreas and biliary computed tomography (CT) showed masses at the right renal hilum and peripancreatic areas. Positron emission tomography (PET) showed widespread systemic lymphadenopathy. Excisional biopsy of the right supraclavicular node revealed a hyaline vascular variant of CD. Corticosteroid therapy was started and the extent of disease decreased. We here report a case of multicentric CD, the hyaline vascular variant, presenting with jaundice, diagnosed by excisional biopsy and successfully treated with corticosteroids.
Lymphoproliferative Disorder; Jaundice; Obstructive; Castleman Disease
Castleman'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.
Here, 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.
In 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.
Castleman's disease; unicentric; mixed cellularity type; follicular dendritic cells
Most of the previously reported cases of Castleman’s disease (CD) in the neck were of the hyaline vascular type and the most common sign was an asymptomatic neck mass. To achieve a more general recognition of CD in the neck, we present four cases of CD in the neck with unusual presentations.
Patients and Methods
We describe four cases of CD in the neck with unusual clinical manifestations, different histopathologic subtypes and discuss the problems arising in their clinical findings.
Two cases were plasma cell type with multiple bilateral enlarged lymph nodes and systemic manifestations. Two cases presented as a solitary mass were hyaline vascular type. One case underwent twice local recurrence and evolved from the hyaline vascular CD to follicular dendritic cell sarcoma.
Cervical plasma cell CD may have a tendency to present as multiple masses on the both sides with systemic manifestations. It is necessary to pay more attention on the overgrowth of follicular dendritic cells in the hyaline vascular CD, which might represent a precursor of follicular dendritic cell sarcoma.
Castleman’s disease; Neck; POEMS syndrome; Follicular dendritic cell sarcoma; Clinical manifestation
Castleman's disease is an uncommon lymphoproliferative disorder of unknown etiology, most often involving the mediastinum. It has 2 distinct clinical forms: unicentric and multicentric. Unicentric Castleman's disease arising from an intrapulmonary lymph node is rare, and establishing a preoperative diagnosis of this disease is very difficult mainly due to a lack of specific imaging features. We report a case of intrapulmonary unicentric Castleman's disease in an asymptomatic 19-year-old male patient who was accurately diagnosed by preoperative computed tomography (CT). The mass was incidentally found on a routine chest X-ray. A subsequent dynamic CT showed a well-defined, hypervascular, soft-tissue mass with small calcifications located in the perihilar area of the right lower lung. Three-dimensional CT (3D-CT) angiography indicated that the mass was receiving its blood supply through a vascular network at its surface that originated from 2 right bronchial arteries. The clinical history and CT findings were consistent with a diagnosis of unicentric Castleman's disease, and we safely and successfully removed the tumor via video-assisted thoracoscopic surgical lobectomy. This case shows that the imaging characteristics of these rare tumors on contrast-enhanced CT combined with 3D-CT angiography can be helpful in reliably establishing a correct preoperative diagnosis.
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.
Castleman disease; Hyaline vascular variant; Plasma cell variant; Unicentric; Multicentric Castleman disease; Human immunodeficiency virus
Castleman's disease is a rare nonclonal proliferative disorder of the lymph nodes with an unknown etiology. Common locations of Castleman's disease are mediastinum, neck, axilla, and abdomen. Castleman's disease of a peripancreatic location masquerading as pancreatic neoplasm is an even rarer entity. On search of published data, we came across about 17 cases published on peripancreatic Castleman's disease until now. Here we are reporting a case of retropancreatic Castleman's disease masquerading as retroperitoneal neoplasm in a 46-year-old male patient.
Castleman's disease, also known as angiofollicular hyperplasia, usually occurs in the mediastinum and rarely in cervical region as solitary mass. Histopathologically four variants have been recognized (Castleman et al. Cancer 1954;9:822–30) hyaline vascular type—most common type (Keller et al. Cancer 1972;29:670–83) plasma cell type (Salisbury, Pediatric Pathol 1990;10:609–15) transitional type (Shahidi, Mayo Clinic Proc 1995;70:969–77) stromal rich type. We report a case of Castleman's disease of typical histopathological picture of angio follicular hyperplasia arising from the right carotid triangle of neck in 12-year-old child without any associated systemic illness.
Castlman's disease; angio follicular hyperplasia; neck
Castleman disease (CD) is an uncommon lymphoproliferative disorder and is especially rare in the retroperitoneum or perirenal area. We report the case of a 42-year-old woman in whom we found on routine physical examination a mass localized in the right abdomen without clinically important constitutional symptoms for 30 days. Abdominal ultrasound, magnetic resonance images and computed tomography scans revealed a solid mass localized just below the right kidney in the retroperitoneum. The patient subsequently underwent an exploratory laparotomy. Pathological examination revealed a right retroperitoneal mass of CD (hyaline vascular type). The patient completely recovered after surgery. Castleman disease is commonly misdiagnosed as malignant lymphoma, lymphadenitis or ectopic thymoma. So far, its diagnosis is mainly achieved via histopathological examination of surgically obtained tissue. After removal of the lesion, local CD has a good prognosis.
Castleman’s disease (CD), also called giant lymph nodal hyperplasia, is a lymphoproliferative disorder secondary to lymphoid follicle hyperplasia and marked capillary proliferation with endothelial hyperplasia. It presents as enlarged lymph nodes in the mediastinum, neck, groin, axilla and other sites. On clinical examination as well as gross examination, this disease mimics lymphomas and tuberculosis. Since cytological appearances vary depending on the type and extent of hyperplasia, fine needle aspiration cytology (FNAC) findings may not always be conclusive in all cases. We studied aspirates in two cases of CD, one of which presented with multiple enlarged axillary lymph nodes and the other with enlarged cervical lymph node. Cytology revealed reactive lymphadenitis with hyalinized capillaries and other features. Lymph node excision in both cases confirmed the diagnosis.
Cytology; Castleman’s disease; lymph node; fine needle aspiration cytology
Castleman disease (CD) is an uncommon benign lymphoproliferative disorder, which usually presents as solitary or multiple masses in the mediastinum. Peripancreatic CD was rarely reported. Herein, we report two cases of unicentric peripancreatic CD from our center. A 43-year-old man and a 58-year-old woman were detected to have a pancreatic mass in the routine medical examinations. Both of them were asymptomatic. The computed tomography and ultrasonographic examination revealed a mild enhancing solitary mass at the pancreatic head/neck. No definite preoperative diagnosis was established and Whipple operations were originally planned. The intraoperative frozen section diagnosis of both patients revealed lymphoproliferation. Then the local excisions of mass were performed. Histological examination revealed features of CD of hyaline-vascular type. No recurrence was found during the follow-up period. CD should be included in the differential diagnosis of pancreatic tumors. Local excision is a suitable surgical choice.
Castleman disease; Peripancreatic tumor; Hyaline-vascular type
Giant lymph node hyperplasia is a rare, benign disease involving lymph nodes in various locations, predominantly in the mediastinum. There are two variants: plasma cell (earlier and/or acute) and hyaline-vascular, more chronic with an intermediate transitional type. The usual presentation is a solitary well-circumscribed asymptomatic mass lesion, often attaining large size, with infrequent associated hematologic manifestations. A case of giant lymph node hyperplasia involving the paravertebral superior mediastinum is reported.
Surgical excision was the treatment of choice in a 65-year-old man, and at thoractomy, an encapsulated mass was excised from the posterior superior mediastinum. The patient had an uneventful postoperative course and was discharged on the tenth postoperative day.
Three years later, he is well and employed as a carpenter.
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's disease; Hyaline-vascular; Radiotherapy
Castleman’s disease is a rare disease characterized by lymph node hyperplasia. Its occurrence in the retroperitoneal space has rarely been reported, making its preoperative diagnosis difficult. Here, we report a case of retroperitoneal Castleman’s disease, which radiologically resembled paraspinal schwannoma.
A 33-year-old Japanese man with epigastric discomfort underwent abdominal ultrasonic examination revealing a solid mass next to the right kidney. Computed tomography demonstrated a well-circumscribed mass with central calcification in the right psoas muscle. Because the mass presented a dumbbell-like shape extending to the intervertebral foramen, neurogenic tumor was suspected. Both iodine-123 metaiodobenzylguanidine and gallium-67 scintigraphies were negative in the mass, whereas thallium-201 mildly accumulated in the tumor, suggesting blood flow to the tumor. Positron emission tomography revealed accumulation of fluorine-18-2-fluoro-2-deoxy-d-glucose in the tumor at a standard uptake value of 4.7, whereas no other abnormal uptake suggestive of metastatic lesion was noted. On the basis of imaging studies, we mostly suspected paraspinal schwannoma, although malignancy was not completely excluded. Angiography showed feeding vessels from the right lumbar arteries, which were embolized with porous gelatin particles in order to reduce intraoperative bleeding. Surgical resection was performed using a retroperitoneal approach, which revealed the tumor in the swollen psoas muscle. Intraoperative pathological examination of a frozen section revealed no evidence of malignancy; thus, marginal excision of the tumor was performed. The tumor adhered tightly to surrounding muscle tissues, resulting in 940 g of intraoperative blood loss. The pathological examination demonstrated infiltration of lymphocytes surrounding small germinal centers with extensive capillary proliferation. Immunostaining revealed that proliferated lymphocytes were CD3-negative and CD79a-positive.
Although a dumbbell-shaped mass in a paraspinal region is indicative of a schwannoma for orthopedic surgeons, the possibility of Castleman’s disease should be considered if a central low-signal area in fissured and a radial pattern is detected on computed tomography or magnetic resonance imaging. Appropriate preparation for massive bleeding during the treatment of Castleman’s disease, including angiography and embolization, would be helpful for performing surgical procedures safely.
Castleman’s disease; Dumbbell shape; Paraspinal schwannoma; Soft-tissue sarcoma
We report a rare case of multicentric Castleman's disease that presented with ophthalmic involvement, along with a review of the literature. A 63-year-old male presented with decreased visual acuity in both eyes. Both eyes had serous elevations of the retinas with shifting subretinal fluid and annular choroidal detachment. No retinal breaks were found. Laboratory tests revealed pancytopenia, hypergammaglobulinemia, and an increased erythrocyte sedimentation rate. Chest and abdominal computed tomographies showed multiple lymphadenopathies in the mediastinum, abdomen, and in both inguinal areas. Histological examination of the inguinal lymph node biopsy was consistent with Castleman's disease. After combination chemotherapy, the serous elevations of both retinas and the annular choroidal detachments of both eyes disappeared. Ophthalmic involvement in Castleman's disease is very rare, and to the authors' knowledge, this is the first report of ophthalmic involvement of Castlemans's disease in Korea.
Choroidal detachment; Giant lymph node hyperplasia; Uveal effusion