Hyaline-vascular Type Castleman's Disease Research Articles

Analysis of immunophenotypic features in hyaline vascular type Castleman disease

BackgroundStudies suggested that the immunophenotype of hyaline vascular type Castleman disease (HVCD) lacked characteristics, which was easy to be confused with other diseases.MethodsFrom January 2010 to June 2022, 17 cases of HVCD were selected from the Department of Pathology of Shaanxi Provincial People’s Hospital and the Department of Pathology of Shaanxi Provincial Cancer Hospital. 13 cases of reactive hyperplastic lymph nodes (RHL) and 11 cases of follicular lymphoma (FL) were selected as the control group. All cases were performed CD3, CD20, CD21 and BCL2 immunohistochemical staining.Results(i) In 17 cases of HVCD, the negative area of BCL2 of germinal center was significantly smaller than the negative area of CD3 of germinal center. However, in 13 cases of RHL, the negative area of CD3 of germinal center was basically consistent with the negative area of germinal center of BCL2 of germinal center. In 11 cases of FL, in neoplastic follicles, the negative area of CD3 was basically consistent with the positive area of BCL2. The difference between HVCD group and other two groups of diseases was statistically significant (P < 0.05). (ii) In 17 cases of HVCD, the negative area of BCL2 of germinal center was significantly smaller than the follicular dendritic cell (FDC) meshworks expressed by CD21. However, in 13 cases of RHL, the FDC meshworks expressed by CD21 were basically consistent with the negative area of BCL2 of germinal center. In 11 cases of FL, in neoplastic follicles, the FDC meshworks expressed by CD21 was basically consistent with the positive area of BCL2. The difference between HVCD group and other two groups of disease was statistically significant (P < 0.05).ConclusionsHVCD has unique immunophenotypic characteristics. The negative area of BCL2 of germinal center is significantly smaller than the negative area of CD3. The negative area of the BCL2 of germinal center is significantly smaller than the FDC meshworks expressed by CD21. These two immunophenotypic features in HVCD are very important in diagnosis and differential diagnosis.

Unicentric Castleman Disease: A Rare Diagnosis of Radiological and Histological Correlation.

Castleman disease is an uncommon nonneoplastic, lymphoproliferative disorder of that is associated with lymphadenopathy and nonclonal lymph nodes hyperplasia. It is further subdivided into two types: Unicentric Castleman disease and Multicentric Castleman disease. Unicentric Castleman disease is rare to be reported in patients having AIDS, because HIV infected patients most commonly presented with Multicentric Castleman disease and they mostly coinfected with HHV-8. Herein we reported a rare presentation of Unicentric Castleman disease with hyaline vascular variety evident Histopathologically as a preoperative diagnosis. The patient was initially managed conservatively and surgical excision (excisional biopsy) of the left cervical lymph node was than performed and sent for histopathology. The Immunohistochemistry findings were also consistent with the diagnosis of Castleman disease Hyaline vascular type as evident from left cervical lymph node excisional biopsy.

Calcifying fibrous tumor of the gastrointestinal tract: A clinicopathologic review and update

Calcifying fibrous tumor (CFT) is a rare mesenchymal lesion that has been documented throughout the gastrointestinal tract. Gastrointestinal CFTs may occur at virtually any age, with a predilection for adults and for females. They occur most commonly in the stomach and the small and large intestines. CFTs are most often found incidentally, cured by local resection, and have a low risk of recurrence. Histology shows three characteristic features: Spindle cell proliferations within a densely hyalinized stroma, scattered calcifications, and lymphoplasmacytic inflammation. CFTs are immunoreactive for CD34, vimentin and factor XIIIa, helping to distinguish them from other benign mesenchymal neoplasms. The differential diagnosis of CFTs includes sclerosing gastrointestinal stromal tumor, leiomyoma, schwannoma, solitary fibrous tumor, inflammatory myofibroblastic tumor, plexiform fibromyxoma, fibromatosis, sclerosing mesenteritis, and reactive nodular fibrous pseudotumor. The pathogenesis of CFTs remains unclear, but some have hypothesized that they may be linked to IgG4-related disease, inflammatory myofibroblastic lesions, hyaline vascular type Castleman disease, sclerosing angiomatoid nodular transformation of the spleen, or trauma.

Temporal dispersion in demyelination of POEMS syndrome and Castleman disease

ObjectiveWe detailed the electrophysiological patterns of peripheral nerve temporal dispersion across spectrum of POEMS syndrome and Castleman disease (CD). MethodsCompound muscle action potentials (CMAP) duration of 3 patients with POEMS syndrome and 2 with hyaline vascular type CD without clonal plasma cell dyscrasia were retrospectively analysed. ResultsMedian and ulnar nerves distal CMAP duration were prolonged in all patients irrespective of plasma cell dyscrasia or M protein. All lower limbs distal CMAP responses were absent. Greatest distal CMAP duration prolongation was observed in median nerves for POEMS syndrome (17.0 ms, 158% upper limit normal) and in ulnar nerves for CD (9.8 ms, 47% upper limit normal). Distal/proximal CMAP duration ratio of <0.7 were seen in 33% of median and ulnar nerves studied among POEMS syndrome. Among nerves with ratio >0.7, all had distal CMAP duration prolongation (Range 7%–158% of upper limit normal). ConclusionsAbnormal distal CMAP dispersion is not uncommon in POEMS syndrome and CD without clonal plasma cell dyscrasia or M protein. POEMS syndrome has greater distal CMAP duration in median and ulnar nerves, particularly in median nerve that can reach up to 150% of upper limit normal, compared to <50% in CD. SignificanceDetailed electrophysiological analysis of distal CMAP duration may help in distinguishing POEMS syndrome and CD.

PB2068 THE CASTLEMAN DISEASE: HISTOLOGICAL VARIANTS, POEMS‐SYNDROME AND PRODUCTIVE PLASMA CELL VASCULITIS

Background:The Castleman disease (CD) is a rare, little‐studied disease characterized by lymphadenopathy with follicles hyperplasia and active neoangiogenesis. Occurs in the form of two histological variants: plasma cell and hyaline‐vascular. The total vasculitis in the vessels of internal organs, previously not noted by researchers, was revealed in the analysis of 2 sectional cases of CD.Aims:To study the state of microcirculatory vessels in patients with plasma cell and hyaline‐vascular type of CD in sections of biopsied lymph nodes.MethodsSections of biopsied lymph nodes were used. It was used hematoxylin‐eosin and reaction to antibodies to CD138 to identify plasma cells. In all patients with plasma cell variant cells were painted with antibodies to HSV‐8. Vessels estimated in perinodular tissue of capsule and fat. We examined 15 patients with CD. According to histology and CD138 content, the plasma cell variant was diagnosed in 8 patients, 7 men and 1 woman aged from 40 to 70 years. In 5 patients approved POEMS syndrome by the presence of M‐protein, disorders of neuro‐muscular conduction according to the electro‐neuromyography, and other symptoms. In 7 patients was diagnosed hyaline vascular type of CD, including 5 women and 2 men aged from 30 to 60 years.Results:In 1 patient with plasma cell variant of CD revealed HSV8 reaction with intranuclear staining antibodies. Vasculitis in the form of vascular walls thickening of the microcirculatory bloodstream with a decrease or obliteration of their lumen, wall fibrosis and plasma cell infiltration was found in all 15 patients. The most pronounced changes in the form of fibrosis, complete vascular obliteration and massive perivascular infiltration CD138+ cells by type of massive colonies of hundreds of cells were found in patients with the POEMS‐ syndrome. They also noted the location of single CD138 + cells inside the intima of the sclerosal vessels. Fibroplastic vascular changes in patients with POEMS‐syndrome were weirdly shaped in the form of “mood masks”. It coincided with massive osteosclerotic changes of the vertebrae on radiographs. Massive development of fibrosis corresponds to the POEMS pathogenesis. All patients with its presence later died.Summary/Conclusion:The Castleman disease, regardless of the histological variant, includes in its morphological pattern productive plasma cell vasculitis, most pronounced in the plasma‐cell variant with POEMS‐ syndrome.

Unicentric hyaline vascular type of castleman disease of the renal hilum with diagnostic dilemma: A case report and review of literature

Castleman disease (CD) represents a morphologically distinct variety of benign proliferation of lymphoid tissue. Renal hilum involvement is rare and may be confused with renal malignancy or granulomatous diseases. Here, we report a rare case of unicentric CD of renal hilum. A 56-year-old male patient presented with flank pain and voiding symptoms. Contrast-enhanced computed tomography scan showed a left renal hilar tumor mimicking a renal malignancy which led to nephrectomy. Our case is the first report of the unicentric hyaline vascular type of CD of the renal hilum reported from India. Awareness of such cases is important for radiologists and surgeons to prevent the misdiagnosis of a renal malignant tumor and to avoid nephrectomy.

Multicentric Castleman disease of hyaline vascular variant presenting with unusual systemic manifestations: a case report

BackgroundCastleman disease is a rare lymphoproliferative disorder presenting with localized or disseminated lymphadenopathy and systemic manifestations. It can be categorized in numerous ways, such as unicentric versus multicentric, histopathological variants (hyaline-vascular, plasma cell, and mixed), or subtypes based on causative viral infections (human immunodeficiency virus, human herpesvirus-8, or Kaposi sarcoma herpesvirus). Presentation ranges from asymptomatic to symptoms involving multiple organs. Even though the exact mechanism of pathogenesis is unknown, treatment is directed toward possible etiologies such as interleukin-6, cluster of differentiation 20, and viral agents.Case presentationA 36-year-old Sri Lankan woman presented with generalized body swelling and foamy urine of 2 weeks’ duration. Examination revealed pallor; generalized edema; axillary, cervical, and inguinal lymphadenopathy; hypertension; and hepatomegaly. Investigations showed bicytopenia, nephrotic range proteinuria with hypoalbuminemia, hypogammaglobulinemia, and features of hyaline-vascular type Castleman disease in a lymph node biopsy. She was managed with rituximab and had good clinical improvement.ConclusionsCastleman disease has a broad spectrum of clinical manifestations, disease pathogeneses, and associations and/or complications. Medical professionals need to be familiar with this spectrum because timely diagnosis and aggressive targeted therapy are the cornerstones of managing these patients.

Clinical characteristics and treatment of unicentric retroperitoneal Castleman's disease

Objective:To investigate the clinical characteristics and treatment analysis of unicentric retroperitoneal Castleman's dis-ease (CD) and to improve its levels of diagnosis and treatment. Methods:The clinical data of 18 patients with unicentric retroperitone-al CD were retrospectively reviewed from January 2002 to December 2016. We analyzed the demographic characteristics, clinical fea-tures, histological diagnosis, treatment, and prognostic characteristics of these patients. Results:The patients comprised 4 males and 14 females with a median age of 44.1 years old (ranging from 24 years old to 70 years old). The tumor was located in the kidney in one case, while it was located in the adrenal region in eight cases. In the other cases, the tumor was in the retroperitoneal region. Seven patients had a clinical atypical abdominal pain, and one patient had moderate anemia. All patients underwent surgical resection. The mean operation time was 153.3 min with a range of 60 min to 260 min. The mean blood loss was 447.2 mL (ranging from 10 mL to 3000 mL). Two cases had blood transfusion after operation, and one had urinary fistula complication. CD was confirmed by histopathol-ogy. Hyaline vascular type of CD was observed in 16 cases, and mixed type of CD was observed in 2 cases. The median follow-up was 25 months. One patient had tumor recurrence after 2 years of operation and died. Other patients remained alive without recurrence. Conclusion:Unicentric retroperitoneal CD is a rare disease that is often misdiagnosed because of the absence of specific clinical mani-festations. The final diagnosis depends on the results of a pathologic examination. Complete surgical resection offers a favorable result for unicentric retroperitoneal CD.

顎下部に発生した硝子血管型Castleman’s diseaseの1例

Castleman's disease (CD) usually occurs in the mediastinum and the hilar region and relatively rarely occurs in the head and neck region. We report a case of CD arising in the right submandibular region in a 26-year-old woman. The patient had symptoms of swelling in the right submandibular region that had been present for half a year. On examination, a nontender movable mass of 25 × 20 mm was palpated in the right submandibular region. Computed tomography revealed a well-demarcated mass measuring 30 × 20 mm in the right submandibular region, and all laboratory data were within normal range. The mass was removed with the patient under general anesthesia. Histopathologically, the mass was diagnosed as a hyaline-vascular type of CD. There was no recurrence at follow-up 7 years after surgery.

Mulberry cells in the thyroid: warthin–finkeldey‐like cells in hashimoto thyroiditis‐associated lymphoma

Warthin-Finkeldey type giant cells were first described in autopsies performed on young children who died during the highly lethal measles epidemic in Palermo during the winter of 1908. The cells had 8-15 nuclei without identifiable cytoplasm within the germinal centers of lymphoid organs resembling megakaryocytes. We describe a case of Hashimoto thyroiditis with an enlarging substernal throid mass. The resection specimen contained many Warthin-Finkeldey-Like Cells (WFLC) in an extranodal marginal zone lymphoma (MALT type) with focal transformation to diffuse large B-cell lymphoma. The WFLC showed nuclear features similar to those of neighboring follicular dendritic cells (FDCs), favoring the hypothesis that these cells might be the product of fusion of FDCs. This is supported by immunostaining results and the occurrence of similar cells in follicular dendritic cell sarcomas and in "dysplastic" FDCs in hyaline vascular type Castleman disease, a possible precursor of follicular dendritic cell tumors. Diagn. Cytopathol. 2017;45:212-216. © 2016 Wiley Periodicals, Inc.

Follicular dendritic cell tumour/sarcoma: a commonly misdiagnosed tumour in the thorax

Follicular dendritic cell sarcoma is a rare tumour reported to occur occasionally in association with the hyaline-vascular type of Castleman's disease (HVCD). Most cases arise in lymph nodes, although extranodal presentation is described. Clinical, radiological and histological characteristics, including diagnosis on pre-resection material, were assessed in seven intrathoracic cases from five males and two females with a median age of 38years. Clinical symptoms were related to mass location, six cases presenting within central and/or posterior mediastinal compartments and one within the lungs. Positron emission tomography-computed tomography demonstrated marked fluoro-deoxy-glucose avidity and the prominent vessels traversing the lesions. Four of six cases (67%) were misdiagnosed initially. HVCD was present in three cases. Two cases with high mitotic rates recurred after resection. All were positive for at least one of the follicular dendritic cell markers (CD21, CD35 and CD23). Six of seven cases (86%) show cyclin D1 expression ranging from 5% to 90%. Follicular dendritic cell sarcoma is often misdiagnosed on biopsy and pathologists need to be aware of the tumour to request the relevant immunohistochemistry, especially in masses presenting in the central/posterior mediastinum with high vascularity and standardized uptake values. Background HVCD appears more common than previously thought.

Multicentric Hyaline-Vascular Castleman Disease: A Case Report

Castleman disease is a rare lymphoproliferative disorder. Contrary to its closest differential of lymphoma, the disease tends to run a benign course. However the multicentric variety, which is usually of the plasma cell type, has a more sinister prognosis. It is commonly associated with HIV infection. We report a case of a 65 year old man presenting with recurrent episodes of swelling of the whole body and diarrhoea for 3 years. Physical examination revealed generalized lymphadenopathy with hepato-splenomegaly and ascites. Lymph node biopsy revealed histopathological changes consistent with hyaline-vascular type of Castleman disease (multicentric). He was HIV negative. Patient was treated with high dose corticosteroids and discharged with follow up advice. DOI: http://dx.doi.org/10.3329/jom.v16i1.22402 J MEDICINE 2015; 16 : 48-50

TdT+ T-Lymphoblastic Proliferation in Castleman Disease.

TdT+ T-Lymphoblastic Proliferation in Castleman Disease.

Budd-Chiari syndrome, a rare complication of multicentric Castleman disease: A case report.

A 39-year-old female presented to The First Affiliated Hospital of Jishou University (Jishou, Hunan) with a fever of unknown origin and progressive abdominal distension. Physical examination revealed generalized lymphadenopathy, multiple non-tender cutaneous nodules, hepatomegaly, splenomegaly and abdominal edema. An axillary lymph node biopsy indicated hyaline vascular type Castleman disease, and color Doppler and computed tomography scans suggested Budd-Chiari syndrome (BCS). Based on the abdominal distension and impairments of the liver and kidneys, an inferior vena cavography and balloon dilatation were performed, confirming the diagnosis of BCS and leading to symptomatic improvement. The patient commenced a combination chemotherapy regimen of cyclophosphamide (0.4 g; days 1-3), vindesine (4 mg; day 1) and prednisolone (100 mg; days 1-5), with no melioration of symptoms. Theprubicin was added to suppress the aggravation of the disease on day six of the chemotherapy cycle. The patient exhibited symptomatic remission for one week, however, she subsequently succumbed to intracranial hemorrhage and infections of the lung and intestine due to long-term myelosuppression following chemotherapy. To the best of our knowledge, this is the first report of BCS in a patient with multicentric Castleman disease without human immunodeficiency virus infection.

Transformation of Castleman's Disease into Follicular Dendritic Cell Sarcoma, Presenting as an Asymptomatic Intra-abdominal Mass

Follicular dendritic cell (FDC) sarcoma is an extremely rare malignant neoplasm arising from FDCs. The exact origin of FDCs remains unclear; both a hematopoietic lineage origin and a stromal cell derivation have been proposed. Proliferation of FDCs can lead to benign reactive lesions or generate neoplastic conditions. The lesions are most commonly found in lymph nodes and usually involve the head and neck area. Castleman's disease is a rare non-neoplasitic lymphoproliferative disorder. Rare cases of hyaline-vascular Castleman's disease have been associated with FDC sarcoma, but a clonal relationship has not been convincingly demonstrated. A pathway toward tumor evolution, beginning with hyperplasia and dysplasia of FDCs, has been proposed. Despite this known association between Castleman's disease and FDC sarcoma, there have only been few reported cases of sarcoma arising as a complication of pre-existing Castleman's disease, especially in abdominal lesions. We describe here a 51-year-old female with an FDC sarcoma arising from unicentric, hyaline-vascular type Castleman's disease in an intra-abdominal mass. Pathologically, the lesion showed a series of changes during the process of transformation from Castleman's disease to FDC sarcoma. (Korean J Gastroenterol 2013;62:131-134).

Atypical Hyaline Vascular-Type Castleman's Disease With Thrombocytopenia, Anasarca, Fever, and Systemic Lymphadenopathy

Recently, atypical Castleman's disease (CD) was reported in Japan. This disease is considered as TAFRO syndrome or non-idiopathic plasmacytic lymphadenopathy (IPL), a constellation of clinical symptoms, namely, thrombocytopenia, anasarca, fever, reticulin fibrosis, and organomegaly without hyper-γ-globulinemia. Histopathologically, this disease is similar to hyaline vascular (HV)-type CD. Here, we present a 43-year-old Japanese woman meeting the clinical criteria of TAFRO syndrome who was successfully treated with combined corticosteroid therapy. She showed a rapidly progressive course of thrombocytopenia, systemic lymphadenopathy, fever, anasarca, and increase in acute inflammatory proteins without hyper-γ-globulinemia. Lymph node biopsy was performed and revealed HV-type CD without human herpes virus 8 infection, which was clinicopathologically compatible with non-IPL. The association of these atypical features with well-known multicentric Castleman's disease (MCD), namely, HV-type histology with systemic lymphadenopathy, marked thrombocytopenia even with a high level of interleukin-6, and increased acute inflammatory proteins without hyper-γ-globulinemia, suggests that TAFRO syndrome as presented in our case is a novel entity, which may have been diagnosed as MCD in the past. To define this novel entity more clearly and to demonstrate its etiology, further nationwide surveys of this syndrome and MCD are needed.

Clinical Features and Outcomes of Head and Neck Castleman Disease

To increase the understanding of head and neck Castleman disease (CD) and to improve its diagnosis and management. A retrospective study was performed on the medical records of 14 patients with cervical CD treated at the Sun Yat-sen University Cancer Center from January 2000 through December 2009. The predictor variables were age, gender, site, size, and treatment modality. The outcome variables were survival time and recurrence. Neck level II (9/14) was the most common site for CD. On computed tomogram, all 14 cases appeared as nodular and cylindrical-shaped lesions growing along the neck. Computed tomogram showed a uniform density and clear margins of the lesions. Thirteen cases with hyaline-vascular type CD showed significant enhancement on enhancing computed tomographic scans. One case with plasma-cell type CD accompanied by Hodgkin lymphoma showed mild heterogeneous enhancement and a strong vascular shadow inside the lesion. Thirteen patients with unicentric CD underwent regional resection. Follow-up time ranged from 14 to 132 months, during which none of the patients relapsed. The results of this study suggest that head and neck CD has a low incidence and that the most common site is unilateral level II. Regional resection was the first choice for the treatment of unicentric CD. Overall, chemotherapy was associated with a poor prognosis in patients with multicentric CD. Future studies will focus on the early diagnosis and treatment of multicentric CD. Long-term follow-up is also necessary.

Clinical characteristics and surgical treatment for localized Castleman's disease

To investigate the clinical characteristics and treatment of localized Castleman's disease (CD), and review the literatures to improve the diagnosis and management of this disease. The clinical symptoms, histopathology, CT, MRI findings and results of surgery in 20 patients with localized CD were evaluated retrospectively. The average age of the patients was 37.7 years. The lesions were located in the retroperitoneal space (9 cases), mediastinum (7 cases), pelvic cavity (1 case), neck (1 case), upper arm (1 case), and axillary (1 case). All patients underwent surgical resection, including 9 cases for retroperitoneal resection (6 cases had open operation and 3 cases laparoscopic resection) and 7 cases for mediastinal resection (open operation in 5 cases and thoracoscopic resection in 2 cases). The Castleman's disease was confirmed by histopathology. There were hyaline vascular type of CD in 17 cases, plasma cell type of CD in 1 case, and mixed cellularity type of CD in 2 cases. The duration of follow-up ranged from 12 to 165 months for 16 cases. Among them 15 patients were alive without recurrence, and 1 case had recurrence in the primary site at 47 months after the operation. Patients with Castleman's disease have no typical clinical symptoms and have normal laboratory results. The majority of patients are of hyaline vascular type of the disease. Imaging examination is helpful to diagnosis, and the final diagnosis depends on pathologic examination. Complete surgical resection of the tumor is the best treatment for localized Castleman's disease.

Follicular Dendritic Cell Sarcoma Arising in Hyaline-Vascular Castleman Disease of Mediastinum: A Case Report

Follicular dendritic cell (FDC) sarcoma is a rare neoplasm that is grouped as histiocytic and dendritic cell neoplasm. It can occur primarily in lymph nodes but also in extranodal sites. Follicular dendritic cells are nonlymphoid, nonphagocytic accessory cell in the immune system. Their dendritic cell nature can be demonstrated by the various FDC markers, such as CD21, CD23, CD35, R4/23, and Ki-4p. FDC sarcoma may occur in association with Castleman disease in 10 - 20% cases. We experienced a case of FDC sarcoma associated with hyaline vascular type-Castleman disease (HVCD). A 62-year-old woman presented a posterior mediastinal mass shown by computed tomography. The resected mass was an encapsulated solid mass, measuring 5.5 x 4 cm in size. Microscopically, the tumor was composed of spindle cells showing a fascicular and storiform arrangement. There are some areas reminiscent of HVCD. The tumor cells were positive for the FDC marker CD21. doi:10.4021/jmc215w

CT and Orbital Ultrasound Findings in a Case of Castleman Disease

A 53-year-old man with a 2-month history of left periorbital swelling was found to have a large solid intraconal mass on CT scan. Orbital ultrasound showed that the lesion had a cavernous pattern of internal reflectivity. Histopathology revealed hyaline-vascular type Castleman disease (CD). This article represents the first reported orbital ultrasound findings in CD. The findings of CT scan and ultrasound may be useful in the preoperative evaluation of orbital hyaline-vascular type CD.