Disease Of Hyaline Vascular Type Research Articles

Castleman’s disease presenting as persistent asymptomatic cervical lymphadenopathy in a child: A very rare disease with atypical presentation

Castlemans disease is a rare atypical lympho-proliferative disorder characterised by enlarged hyperplastic lymphnodes with striking vascular proliferations. A cervical location has very rarely been reported in paediatric literature. It can be unicentric or multicentric. There are mainly four histological variants, hyaline vascular type being the commonest one.Although unicentric Castleman's disease carries a favorable prognosis in children, surgical excision is recommended to confirm the diagnosis and to rule out other causes. We report a case of unicentric cervical Castlemans disease of neck in a 6-years old male child, who came to paediatric ward with history of right sided neck mass since 2yrs. After thorough clinical examination and investigations, complete excision of the mass was done. Histopathological examination confirmed the diagnosis of Castlemans disease-hyaline vascular type. On follow-up for two years no recurrence has been seen.

膵腫瘍との鑑別が困難であった後腹膜Castleman病の1例

膵腫瘍との鑑別が困難であった後腹膜Castleman病の1例

Castleman Disease of Hyaline Vascular Type in the Infrathyroidal Region: A Masquerader of Parathyroid Adenoma

Castleman Disease of Hyaline Vascular Type in the Infrathyroidal Region: A Masquerader of Parathyroid Adenoma

Castleman disease: an unusual diagnosis of a portal mass in an 8-year-old boy

An 8-year-old boy presented with a mass located in the portal hilum and hepatosplenomegaly, and the presumed initial diagnosis was lymphoma. The pathology result was Castleman disease of hyaline vascular type. Castleman disease is an unusual diagnosis that should be kept in mind in the differential diagnosis of portal masses. In the case of solitary lesions, total excision can be curative.

Multicentric Castleman's disease associated with glomerular microangiopathy and MPGN-like lesion: does vascular endothelial cell-derived growth factor play causative or protective roles in renal injury?

A 52-year-old Japanese man presented with fever spikes, generalized fatigue, anorexia, and anasarca. The patient was referred for the evaluation of fever of unknown origin in association with swelling of cervical, axillary, and inguinal lymph nodes. He also manifested nephrotic syndrome, acute renal failure, hepatosplenomegaly, massive pleural effusion, ascites, disseminated intravascular coagulation, and hypergammaglobulinemia. C-reactive protein was positive and plasma vascular endothelial cell-derived growth factor (VEGF) and serum interleukin-6 levels were markedly elevated. Lymph node biopsy results showed that findings were compatible with Castleman's disease of hyaline vascular type associated with interfollicular plasmacytosis. In conjunction with the clinical findings, a diagnosis of multicentric Castleman's disease was made. The patient underwent renal biopsy because of nephrotic syndrome, and the results showed proliferation of mesangial cells, lobulation of glomeruli, and tram track pattern of the capillary wall without immune complex deposition. Electron microscopy showed widening of the subendothelial space. No electron-dense deposits were present in both mesangial and subendothelial regions. Pathologic features were compatible with glomerular microangiopathy and membranoproliferative glomerulonephritis-like lesions. With corticosteroid therapy, systemic symptoms disappeared; both VEGF and interleukin-6 levels were normalized, and he went into complete remission of nephrotic syndrome. In this article, the role VEGF plays in the pathogenesis of nephrotic syndrome and glomerular microangiopathy is discussed.

Angiomyoid proliferative lesion: an unusual stroma-rich variant of Castleman's disease of hyaline-vascular type.

The stroma-rich variant of Castleman's disease of hyaline-vascular type (CDHV) is a newly identified entity that shows overgrowth of a variety of stromal cells. Six CDHV patients showed proliferation of vasculature and actin-positive myoid cells in the expanded interfollicular (IF) area. There were three women and three men, and the median age was 29.5 years. Of the six lesions, four were located in the retroperitoneum, one in the neck, and one in the mediastinum. All patients were asymptomatic. Microscopically, the degree of widening of the IF area varied from slight overgrowth of the IF area (approximately 55% of the lymph node area) to vague nodularity and finally to the formation of prominent nodules. In the nodular lesions, lymph follicles were compressed and attenuated. In the IF area, there were numerous vessels and proliferation of spindle cells that possessed blunt nuclei. The long, slender, dendritic cytoplasms of the spindle cells were stained by alpha smooth muscle actin. The spindle cells were negative for desmin, CD34, factor VIII-related antigen, S-100, CD21, and CD68. No patient has had recurrence after simple excision. We maintain that these angiomyoid proliferative lesions in CDHV are of a hyperplastic nature. This condition encompasses proliferation of small vessels and myoid cells. Its characteristics include an asymptomatic, solitary nodule that predominantly develops in the retroperitoneum.

Ｔ細胞リンパ腫に移行したキャッスルマン病例

A 57-year-old male was admitted with severe fatigue and systemic lymphadenopathy. Surgical biopsy specimens revealed Castleman's disease of hyaline vascular type. After admission, Guillain-Bane syndrome developed and the ptient was treated with predonisolone and plasma pheresis was given. The patient's symptoms and lymphadenopathy responded and disappeared rapidly. Five months later, systemic lymphadenopathy recurred. Surgical biopsy was performed and the histological features were consistent with a diagnosis of peripheral T-cell lymphoma of diffuse mixed type, probably resulting from the progression of the Castleman's disease.

Angiomyoid and Follicular Dendritic Cell Proliferative Lesions in Castleman's Disease of Hyaline-Vascular Type: A Study of 10 Cases

Angiomyoid and Follicular Dendritic Cell Proliferative Lesions in Castleman's Disease of Hyaline-Vascular Type: A Study of 10 Cases

Angiomyoid and follicular dendritic cell proliferative lesions in Castleman's disease of hyaline-vascular type: a study of 10 cases.

Castleman's disease of hyaline-vascular type (HV CD) may rarely be associated with a confusing variety of stromal cell overgrowths and neoplasms. We report here on the pathologic and clinical findings of 10 such cases. In addition to the usual complex histoimmunophenotype of the stroma of HV CD and some unusual features that mimicked neoplasms, we observed focal proliferations of angiomyoid (five cases) and follicular dendritic cell type (five cases). The former were nonneoplastic growths featuring compact tangles of spindle cells, exhibiting immunoreactivity for smooth muscle actin and interpreted as vessel-related pericytes and myoid cells. The latter were neoplastic growths of oval to spindle cells intermixed with lymphocytes; the tumor cells grew in long, intersecting bundles, featured various degree of atypia, and expressed the markers of follicular dendritic cells (CD21, CD35, KiM4p). The two types were clinically distinct. Four of five patients with angiomyoid proliferations were young women, who presented with an abdominal mass and were cured by surgery; that is, they had a clinical profile similar to that of patients with the stroma-rich variant of HV CD. The follicular dendritic cell proliferations were in older patients of either gender presenting with masses at various sites, recapitulating the profile of follicular dendritic cell tumors arising independently from HV CD; in three patients with long-term follow-up, recurrences or metastases developed at various intervals from the initial diagnosis (1 1/6, 3 1/2, and 11 years), and one patient died as a result. This study confirms the potential for, and the variety of, stromal cell proliferations in HV CD. Because their biologic behavior differs, correct identification of these various proliferative lesions is clinically important.

Bronchiolitis obliterans with pemphigus vulgaris and castleman's disease of hyaline-vascular type: An autopsy case analyzed by computer-aided 3-D reconstruction of the airway lesions

We have experienced an autopsy case of a 20-year-old woman with bronchiolitis obliterans, pemphigus vulgaris, and Castleman's disease. The affected bronchioles showed almost the same histology as previously described. But, the number of the lesions seemed too small to cause her death of respiratory insufficiency. We also wondered whether or not some normal routes to the peripheral pulmonary tissues remained. After reviewing many histological sections from both lungs, one tissue block was chosen to make semiserial sections, and computer-aided three-dimensional (3-D) reconstruction analysis was performed. The 3-D reconstruction clearly showed two points: (1) Obstructive lesions developed within very narrow segments of bronchioles (0.4 to 0.7 mm in diameter; restricted to two to three generations) sparing terminal and respiratory bronchioles. This could explain the paucity of bronchiolar lesions that appeared on random sections; (2) All the affected bronchioles showed a high degree (up to 80–90%) of luminal stenosis. No intact routes to the periphery were left. Thus, respiratory insufficiency in this case became quite understandable.

A Mediastinal Mass

A 32-year-old female was admitted for evaluation of known mass on right suprahilar area. Chest PA showed round and homogenous mass on suprahilar area. No abnormal findings were found in PFT, cytology, bacterial study, and fine needle aspiration biopsy(FNAB). On chest computed tomography, solitary mass was on right suprahilar area and no evidence of intrapulmonary metastasis or lymphnode metastasis was seen. Right upper lobectomy of lung was performed and Castleman's disease of hyaline vascular type was diagnosed based on the histologic findings of multiple and large lymphoid follicles with prominent vascular proliferation and hyalization in the central portion.

Castleman's Disease of the Chest: Magnetic Resonance Imaging Features

The value of magnetic resonance imaging (MRI) in the evaluation of vascular abnormalities in the chest is well established. To our knowledge, however, MRI in Castleman's disease of the chest had not been reported previously. We present a case of Castleman's disease of hyaline vascular type involving the pulmonary hilum where MRI was equal to computed tomography in showing the extent of the disease, but was more informative in demonstrating its relationship with vascular structures.

Radiological presentations of Castleman's disease

The radiographic appearances of three cases of Castleman's disease of hyaline vascular type are reported. CT demonstrated homogeneous masses with marked contrast enhancement. Calcification was seen in one, and associated lymphadenopathy was seen in two. All tumors were hypervascular with prominent tumor blush on angiography. Sonography showed the masses to be hypoechoic with hyperechoic spots. On MR imaging, all tumors had homogeneous architecture with hypointensity on T1-weighted images and hyperintensity on T2-weighted images. In tumors with above characteristics in areas where lymphoid tissue is normally found, Castleman's disease should be added to the list of differential diagnosis.

Development of Vascular Neoplasia in Castlemanʼs Disease

Seven cases of vascular neoplasia arising within lesions of hypervascular follicular hyperplasia (HFH) fulfilling the criteria of Castleman's disease are described. The patients did not have evidence of acquired immunodeficiency syndrome or other immunologic disorders. The masses were solitary and located in the retroperitoneum (five cases), mediastinum (one case), and axilla (one case). Grossly, they measured up to 20 cm and had a variegated appearance. In each case two morphologically distinct processes were present: a mesenchymal spindle-cell neoplasm with evidence of vascular differentiation and Castleman's disease of hyaline vascular type. The two processes blended with each other, with the neoplasm appearing to be continuous with the interfollicular proliferation of small vessels that is typical of Castleman's disease. The lesions behaved aggressively in two cases, both patients having died with metastatic disease. This remarkable association may be viewed as a pathologic manifestation of the intimate functional relationship that exists between the immune and the vascular systems. Other probable examples of this relationship are systemic Castleman's disease associated with Kaposi's sarcoma, localized Castleman's disease associated with vascular hamartoma, histiocytoid hemangioma/angiolymphoid hyperplasia with eosinophilia, and (possibly) angiomatoid malignant fibrous histiocytoma. Perhaps these associations are mediated by the production of angiogenic factors by the activated lymphoid cells.