Castleman's disease restricted to the infratemporal fossa

Giant lymph node hyperplasia (Castleman's disease) is usually reported as a solitary mediastinal tumour, although involvement of other anatomical sites and a multicentric form have been reported. We describe a rare case of Castleman's disease due to its localisation (the left infratemporal fossa) and histology (plasma-cell variant). A brief review of the main clinico-histological characteristics of Castleman's disease is also presented.     

Long-term follow-up of patients with biliary atresia successfully treated with hepatic portoenterostomy. The importance of sequential treatment

Castleman's disease, also known as benign giant lymph node hyperplasia, is a lymphoproliferative disorder which can occur either in a localized or multicentric form. The latter is characterized by the development of malignant lymphoma, Kaposi's sarcoma or carcinoma in 32% of cases. By contrast, localized Castleman's disease has exceptionally been reported in association with non-Hodgkin's lymphoma and - to the best of our knowledge - never at the same anatomic site. Here we describe the occurrence in the same lymph node of localized Castleman's disease (with monotypic plasma cell component) and an apparently unrelated peripheral small B-lymphocytic lymphoma.     

A transitional variant of Castleman's disease presenting as a chylous pleural effusion

Castleman's disease is an uncommon clinicopathologic entity that results in unregulated growth of lymphoid tissue. It may present as benign involvement of one lymph node group or as multicentric disease with serious systemic symptoms. Pleural effusions are an uncommon manifestation of Castleman's disease. We present a patient with Castleman's disease who initially presented with a chylous pleural effusion.     

Tropism of human herpesvirus 8 for peripheral blood lymphocytes in patients with Castleman's disease

Multicentric Castleman's disease (MCD), also called multicentric angiofollicular lymphoid hyperplasia, is a systemic lymphoproliferative disorder causing fever, lymphadenopathy and splenomegaly. Recently, Kaposi's sarcoma-associated herpesvirus/human herpesvirus 8 (KSHV/HHV-8) DNA sequences have been detected in cases of MCD. We examined HHV-8 DNA sequences in the peripheral blood mononuclear cells (PBMCs) of two HIV-negative patients with MCD and in PBMCs and the lymph node of a HIV-negative patient with localized Castleman's disease (LCD) by the polymerase chain reaction. The novel sequences were detected in all DNA samples. Furthermore, the sequences were detected in only the CD19+ B-lymphocyte fraction of the patient with LCD as previously reported. However, the sequences were detected in CD19+ B-lymphocyte and CD2+ T-lymphocyte fractions of two patients with MCD. These results suggest that HHV-8 has tropisms for both B lymphocytes and T lymphocytes in Castleman's disease.     

An infant with lobar emphysema requiring lobectomy after ventricular septal defect closure]

We report a case of Castleman's disease in a 65-year-old female, revealed by a renal tumor associated with inter-aortico-cava adenopathies and renal chromophobe cell carcinoma. This observation points to the difficulties in differentiating local Castleman's disease, which may be cured by surgical excision, from multicentric disease associated with a dysimmune syndrome of uncertain prognosis. The association of multicentric Castleman's disease with a carcinoma has rarely been described. It could be the emergence of a neoplasia in a context of dysimmunity or Castleman's disease might be related to the production of interleukin 6 by a renal carcinoma.     

Nasopharyngeal Castleman's disease

A 19-year-old male was admitted to our clinic because of nasal obstruction and intermittent postnasal drip of 3 to 4 years' duration. Physical examination revealed a wide-based, smooth-surfaced nasopharyngeal tumor which was suspected to be a nasopharyngeal angiofibroma after examination of computed tomographic scans and an angiogram. However, after the tumor was removed by surgical excision via a transpalatal approach, the pathologic examination revealed Castleman's disease of the hyaline-vascular type. There was neither evidence of recurrence nor nasal problems at 4 years' follow-up. To our knowledge, Castleman's disease, or angiofollicular lymph node hyperplasia, may present as a local or generalized tumor-like condition, usually in the chest or abdomen, and may involve both the lymph nodes and non-nodal tissues. A review of previous articles reveals that there has not been any report of Castleman's disease found in the nasopharynx. This rare disorder is presented and discussed.     

Pancreatic localization of a Castleman's tumour

Castleman's tumour is a rare anatomo-clinical entity. Most cases of Castleman's tumour occur in the mediastinum. The histopathology demonstrates benign angiofollicular lymph node hyperplasia. The pancreatic localization is uncommon and has only been reported once. We describe a new case with literature review.     

Cytology of pigmented renal cell carcinoma. A case report

We used a new combined chemo- (COP/ABVD), radiation and interferon-alpha (10 x 10(6) IU s.c. 3x per week/12 months) therapy regimen to treat severe multicentric Castleman's disease (CD) complicated by relapsing Behcet's disease (BD) manifestations. More than 16 years after diagnosis of CD the patient remains in very good clinical condition, with remission of all CD and BD manifestations 13 months after discontinuation of the interferon-alpha treatment. In addition, our clinicopathological, immunohistological and virological data suggest a pathogenetic link between CD and BD via activation of pre-existing BD-specific plasma cells due to CD-related HHV8-induced overexpression of interleukin-6.     

Human herpesvirus 8 encodes a homolog of interleukin-6

Kaposi's sarcoma is a multifocal lesion that is reported to be greatly influenced by cytokines such as interleukin-6 (IL-6) and oncostatin M. DNA sequences of a novel human gammaherpesvirus, termed human herpesvirus 8 (HHV-8) or Kaposi sarcoma-associated herpesvirus, have been identified in all epidemiological forms of Kaposi's sarcoma with high frequency. The presence of HHV-8 DNA is also clearly associated with certain B-cell lymphomas (body cavity-based lymphomas) and multicentric Castleman's disease. Sequence analysis of a 17-kb fragment revealed that adjacent to a block of conserved herpesvirus genes (major DNA-binding protein, glycoprotein B, and DNA polymerase), the genome of HHV-8 encodes structural homolog of IL-6. This cytokine is involved not only in the pathogenesis of Kaposi's sarcoma but also in certain B-cell lymphomas and multicentric Castleman's disease. The viral counterpart of IL-6 (vIL-6) has conserved important features such as cysteine residues involved in disulfide bridging or an amino-terminal signal peptide. Most notably, the region known to be involved in receptor binding is highly conserved in vIL-6. This conservation of essential features and the remarkable overlap between diseases associated with HHV-8 and diseases associated with IL-6 disregulation clearly suggest that vIL-6 is involved in HHV-8 pathogenesis.     

Castleman disease: 2 unusual cases

Castleman's disease is a localized, rare and benign hyperplasia of the lymph nodes, occurring mainly in the mediastinum. Three histologic types--hyalinvascular, plasmacellular and intermediate--have been recognized. We report on two patients with unusual locations of the disease, i.e. the pelvis and the retroperitoneum respectively. These observations show that diagnosis is difficult. They also indicate that, in selected patients, radiotherapy may induce tumor regression and allow the resection of apparently inoperable lesions.     

Castleman's lymphoma among Japanese population

Since our report on Castlemans lymphoma in 1958, a total of 53 cases by 36 authors have appeared in the Japanese literatures. Among them our first case, whose tumor was found in the pelvic cavity, would be the first patient ever reported in Japan. The outstanding histological features of Castleman's lymphoma consists of variegated morphology of germinal centers due to peculiar capillary proliferation, multicentric germinal centers in a lymph follicle showing a chimerical figure and expansive growth of lymph follicles by compressing interfollicular area. Among these Japanese population, we have noticed the highest age incidence between 10-19 years, with somewhat female predominance, the most preferential site in the upper part of the body, and, as compared to the other ethnical subjects, more frequent tumor multiplicity and less usual plasma cell type of the tumor. A possible pathogenesis that Castleman's lymphoma could be "a benign lymphoma" in contrast to malignant lymphomas is emphasized from various aspects.     

Reticulum cell neoplasms of lymph nodes: a clinicopathologic study of 11 cases with recognition of a new subtype derived from fibroblastic reticular cells

Lymph nodes contain nonlymphoid accessory cells including follicular dendritic cells (FDCs), interdigitating dendritic cells (IDCs) and fibroblastic reticular cells (FBRCs). Neoplasms derived from FDCs are uncommon, and those of IDC origin are even more rare. We report the clinicopathologic features of 11 reticulum cell neoplasms, including 2 of FBRC origin. There were seven male patients and four female patients ranging in age from 13 to 73 years. All cases involved lymph nodes (cervical or supraclavicular-6 cases), (abdominal--2 cases), epitrochlear (1 case); two had more than one site of involvement (cervical lymph node and mediastinum--1 case, cervical and abdominal lymph nodes--1 case). One case of FDC tumor had concomitant Castleman's disease, plasma cell variant. Each neoplasm showed similar histology with oval-to-spindle-shaped cells in a storiform or fascicular pattern. Based on immunophenotypic findings, the neoplasms were classified as FDC (five cases), IDC (two cases), FBRC (three cases), and reticulum cell neoplasm, not otherwise specified (one case). The FDC tumors showed immunoreactivity for CD21 or CD35, vimentin, and CD68. The IDC tumors showed strong positivity for S-100 protein and variable positivity for CD68 and CD1a. The cases derived from FBRCs were positive for vimentin, desmin, and smooth-muscle actin. The neoplasm classified as reticulum cell neoplasm, not otherwise specified had similar morphologic features but showed only equivocal positivity for CD68 and vimentin. Follow-up was available for 9 of 11 (82%) cases with a mean of 3.5 years. Four of five patients with FDC tumors were alive with disease when last seen; the fifth is alive and well with no evidence of disease at 4-year follow-up. One patient with IDC tumor had a recurrence in a different nodal site. Two patients with FBRC tumor were disease free at follow-up of 2 years and 8 years, respectively. The patient with reticulum cell neoplasm, not otherwise specified, was alive and disease free 8 years after diagnosis.     

Intra-articular hyaluronan treatment for osteoarthritis

Castleman's disease, angio-follicular lymph node hyperplasia, is a relatively rare benign tumour. It typically arises in the mediastinum. We report a unique case arising in the erector spinae muscle. The case demonstrates the importance of radiological investigation and embolisation prior to obtaining a tissue diagnosis and subsequent surgical excision.     

Recurrent cerebrovascular insult--manifestation of POEMS syndrome?

The POEMS syndrome is a multisystem disorder characterised by polyneuropathy, organomegaly endocrinopathy, plasma cell dyscrasia and skin changes. Along with many further manifestations this rare syndrome may also include macroangiopathy and acute vascular obliteration. In the literature such vascular problems have received only little attention and most case reports refer to involvement of the coronary and lower limb arteries. To our knowledge, the association with recurrent strokes has not been described. We report on a 59-year-old male with a complete form of POEMS syndrome associated with multicentric Castleman's disease. Despite absence of vascular risk factors, myocardial infarctions and ischemic necroses of toes occurred, and recurrent ischemic strokes appeared during oral anticoagulation. Recurrent strokes may therefore represent a further complication of the POEMS syndrome.     

Giant lymph node hyperplasia (Castleman's disease) presenting with chylous pleural effusion

A case of giant lymph node hyperplasia (Castleman's disease) with an original presentation of a chylous pleural effusion occurred in a female adolescent. CT scans showed mediastinal lymphadenopathy. Lymph node biopsy was consistent with the plasmacytic variant of Castleman's disease.     

The effect of recombinant human erythropoietin on hemostasis, fibrinolysis, and blood rheology in autologous blood donors

We report three cases of Castleman's disease mimicking the features of collagen disease. Case 1: A 39-year-old woman presented with intermittent arthralgia and fever. Laboratory findings were positive results for antinuclear antibody (80x speckled type), the LE test, anti-SSA antibody, anti-RNP antibody, and Coombs test. The patient was suspected to have systemic lupus erythematosus (SLE) or Sj?gren syndrome, but a lymph node biopsy revealed the plasma cell type of Castleman's disease. Steroid treatment led to resolution of her symptoms. Case 2: A 60-year-old man with mixed type Castleman's disease had proteinuria with renal dysfunction, autoimmune thrombocytopenia, antinuclear antibody, anti-RNP antibody, anti-DNA antibody and anti-cardiolipin antibody. The patient was suspected to have SLE but cervical lymph node biopsy revealed the mixed type of Castleman's disease. Symptoms were not controlled with steroid therapy. He developed renal failure that required for hemodialysis and died of gastrointestinal bleeding due to severe thrombocytopenia. Case 3: A 46-year-old woman had Raynaud's phenomenon, sclerodactylia, and nail fold bleeding. Laboratory tests were revealed positive for antinuclear antibody, anti-ENA antibody, and LE cell preparation. Radiographic study showed multiple masses in the retroperitoneal spaces, which necessitated laparotomy. Firstly, the patient was suspected to have systemic sclerosis or mixed connective tissue disease (MCTD). A biopsy revealed the hyaline-vascular type of Castleman's disease. The serum level of IL-6 by ELISA was high in all of three cases. In case 1, symptoms improved and the IL-6 level normalized after steroid treatment.(ABSTRACT TRUNCATED AT 250 WORDS)     

Fibrillary glomerulonephritis in Castleman's disease

Castleman's disease is an uncommon lymph node disorder which can be associated with renal disease. In this report we describe a patient with fever, weight loss, anorexia, increase in inflammatory proteins, anemia and nephrotic syndrome. Castleman's disease, plasma cell type, was diagnosed by histologic analysis after surgical excision of a pelvic lymph node. The disease was considered localized, since further investigations did not show any other pathologic mass. After resection of the pelvic lymphoid mass, clinical remission of systemic symptoms and laboratory abnormalities was observed, with the exception of the nephrotic syndrome. Renal biopsy was performed and showed a pattern compatible with fibrillary glomerulonephritis. Progressive decline in renal function was observed, despite immunosuppressive therapy.     

Retroperitoneal Castleman''s disease of the hyaline vascular type presenting arborizing calcification

Castleman's disease (CD) usually manifests as a solitary mediastinal tumor and only rarely as an isolated retroperitoneal mass. In the latter instances it is difficult to distinguish radiographically from other retroperitoneal masses. We report a 22-year-old female patient with retroperitoneal CD of the hyaline vascular type presenting with arborizing calcification. This characteristic calcification pattern is considered unique to CD, and is useful in diagnosis when present.     

Plasma cell interstitial pneumonia as a manifestation of multicentric Castleman's disease

A 12-year-old boy was admitted to our hospital because of abnormal shadows on a chest radiograph, slight fever, and superficial lymphadenopathy. Laboratory examination showed anemia (Hb 9.9 g/dl) and hyperimmunoglobulinemia (IgG 5469 mg/dl) without M protein. A chest CT scan showed bilateral diffuse shadows and bilateral hilar lymphadenopathy. Biopsy specimens of an inguinal lymph node and a lung showed many lymphoid follicles with germinal centers, and marked infiltration of mature plasma cells in the interfollicular area without destruction of follicular structures. The polyclonality of the plasma cells was confirmed by immunohistochemistry. The patient was not treated because these results excluded malignant disease and he was asymptomatic. At the age of 17 years, he was admitted to our hospital again because of dyspnea and a tendency to bleed. Interstitial pneumonia, hyperimmunoglobulinemia (IgG 13900 mg/dl), and anemia (Hb 6.6 g/dl) were found, along with thrombocytopenia (2.5 x 10(4)/mm3) and proteinuria. The serum interleukin-6 level was high: 177 pg/ml. Bronchoalveolar lavage fluid contained many plasma cells. Therapy with corticosteroids and immunosuppressant medication was effective. Our diagnosis was plasma cell interstitial pneumonia as a manifestation of multicentric Castleman's disease.     

Detection of human herpesvirus 8 in patients with Kaposi's sarcoma or Castleman's disease associated with AIDS

A new herpesvirus provisionally termed as KSHV or HHV-8 has been detected in lesions from AIDS-based Kaposi's sarcoma (KS) and from other KS clinical forms, and also in other tumors such as body cavity-based lymphomas or Castleman's disease (CD). We have assessed the presence of this novel herpesvirus in specimens from patients diagnosed with either AIDS and KS or AIDS and CD. DNA samples from skin lesions and peripheral blood obtained from 8 patients diagnosed with AIDS, seven with KS and one with multicentric CD were analyzed; skin specimens and peripheral blood samples from volunteer blood donors or from KS and CD free HIV seronegative patients were used as controls. Detection of the virus was done by PCR amplification of KS330 region, one of the HHV-8 sequences first reported. All skin lesions analysed tested positive for KS330; peripheral blood samples from 5 of the patients, including the one diagnosed with CD, showed also the virus sequence. All skin specimens and peripheral blood samples from controls were negative. From our results it can be concluded that the novel herpesvirus HHV-8 can also be detected in patients with AIDS-associated KS and AIDS-associated CD in Spain.