Hyaline Vascular Variant of Castleman Disease of the Tonsil in an Adolescent: A case Report.

Castleman disease (CD) is a lymphoproliferative disorder classified into two categories as unicentric Castleman disease (UCD) or localized type and multicentric Castleman disease (MCD). A rare case of hyaline vascular variant of tonsil has been presented in which a 14 years old male presented with symptomatic unilateral hypertrophy of right tonsil. A right tonsillectomy was done and surgical pathology report was concluded as hyaline vascular variant of Castleman's disease.Castleman disease (CD) is a rare lymphoproliferative disorder also called as giant lymph node hyperplasia, angiofollicular lymph node hyperplasia (AFH), angiomatous lymphoid hematoma and follicular lymphoreticuloma. The treatment of symptomatic patients with UCD is complete surgical excision (as in present case). In cases with incomplete resection, adjuvant radiotherapy can be given.

Enfermedad de Castleman: un enfoque integral a propósito de un reporte de caso / Castleman's Disease: A Comprehensive Approach Regarding a Case Report

Resumen Se presenta el caso clínico de una paciente del sexo femenino, de 30 años, con adenopatías supraclaviculares y axilares ipsilaterales, quien fue sometida a biopsia excisional con posterior estudio. El análisis histopatológico de la muestra de tejido resecado reveló una serie de características distintivas asociadas con la enfermedad de Castleman variante hialinovascular. La presentación de este caso no solo proporciona información detallada sobre la evolución clínica de la paciente, sino que también sirve como base para ilustrar los aspectos clave del diagnóstico histopatológico y las implicaciones inmunohistoquímicas en la enfermedad de Castleman. Además de hacer una revisión de tema respecto a esta patología poco común, en la cual los informes de casos son fundamentales para aumentar la comprensión de su variabilidad clínica y su abordaje diagnóstico, ilustrando los desafíos en el diagnóstico diferencial y como deben abordarse los mismos.

Abstract The clinical case of a 30-year-old female patient with supraclavicular and ipsilateral axillary lymphadenopathy who underwent excisional biopsy with subsequent study is presented. Histopathological analysis of the resected tissue sample revealed a series of distinctive features associated with hyalinevascular variant Castleman disease. The presentation of this case not only provides detailed information about the clinical evolution of the patient, but also serves as a basis to illustrate Key aspects of histopathological diagnosis and immunohistochemical implications in Castleman disease. In addition to making a review of the topic regarding this rare pathology in which case reports are essential to increase the understanding of its clinical variability and its diagnostic approach, illustrating the challenges in differential diagnosis and how they should be addressed.

Analysis of immunophenotypic features in hyaline vascular type Castleman disease.

BACKGROUND: Studies suggested that the immunophenotype of hyaline vascular type Castleman disease (HVCD) lacked characteristics, which was easy to be confused with other diseases. METHODS: From 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). CONCLUSIONS: HVCD 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.

[Diagnosis and surgical treatment of a patient with Castleman disease]. / Diagnostika i khirurgicheskoe lechenie patsienta s bolezn'yu Kastlemana: klinicheskoe nablyudenie.

Castleman disease (CD) is a benign lymphoproliferative disease. Small prevalence and diverse clinical course of disease makes it difficult to standardize diagnostics and treatment. Currently, the number of CD patients has increased with improvement in the quality of examination. Therefore, differential diagnosis of this disease is important. We present a young patient with CD and retroperitoneal non-organ neoplasm. Despite a thorough preoperative examination, the final diagnosis was established only after histological examination of surgical specimen. We discuss the diagnosis and surgical treatment of a patient with unicentric type of CD.

A Rare Case of Castleman's Syndrome Presenting with a Mass in the Bifurcation of the Celiac Trunk.

INTRODUCTION: Castleman's disease is an extremely rare disease in the abdomen region characterized by benign lymphoepithelial proliferation. CASE PRESENTATION: We report a case of a 63-year-old female who presented with abdominal pain. Abdominal CT Angio and dynamic contrast-enhanced abdominal MRI revealed a mass lesion showing markedly contrast enhancement, no vascular invasion sign, and diffusion restriction lesion in the truncus coelicus bifurcation region. The mass was surgically resected completely. Pathological evaluation showed a hyaline-vascular type of Castleman's disease. CONCLUSION: Castleman's disease should be kept in mind in the differential diagnosis of an isolated intra-abdominal mass.

Hyaline-vascular type unicentric Castleman disease with dysplastic follicular dendritic cell proliferative lesions: a case report.

Castleman disease (CD) is a rare lymphoproliferative disease. Hyaline-vascular type unicentric CD has a good prognosis if completely resected during surgery. However, follicular dendritic cell proliferative lesions have the potential for recurrence and metastasis. A 22-year-old man was referred to our hospital with the chief complaint of nausea and vomiting. These symptoms were caused by a right mesocolonic tumor pushing the duodenum. The patient underwent laparoscopic tumorectomy and complete surgical excision. The postoperative course was uneventful, with no complications. Pathological examination confirmed that the tumor was an enlarged lymph node, typical of hyaline vascular-type CD; however, follicular dendritic cell proliferative lesions were noted. We report a rare case of hyaline-vascular-type CD with follicular dendritic cell proliferative lesions associated with malignancy, as limited case reports exist on this particular disease.

From the archives of MD Anderson Cancer Center Castleman disease involving the thymus gland: Case report and literature review.

Castleman disease is a nodal based disease and very rarely involves the thymus gland. We report a 52-year-old man who was found incidentally to have a single thymic mass by computerized tomography scan. Thymectomy was performed, and the gross specimen showed a well-circumscribed, multi-loculated cystic mass. Histologic examination showed thymus involved by Castleman disease, hyaline-vascular variant. The lesion was characterized by lymphoid follicles with wide mantle zones, variably lymphocyte-depleted germinal centers with sclerotic radial blood vessels, and prominent interfollicular/stromal changes including numerous endothelial venules with sclerotic walls and hyaline sclerosis, scattered and frequent dysplastic follicular dendritic cells and foci of dystrophic calcification. Immunohistochemical analysis showed that the follicle mantle zones were composed of numerous B-cells positive for CD20, PAX5, and IgD. Antibodies specific for CD21 and CD23 highlighted prominent follicular dendritic cell networks within follicles. There was no evidence of human herpes virus 8. We searched the literature and could identify only 10 additional cases of thymic CD. Previously reported cases included 8 unicentric and 2 multicentric, classified pathologically as plasma cell variant (n = 4), hyaline vascular variant (n = 3), and mixed (n = 3). Thymectomy, as was done in the currently reported case, most often leads to the diagnosis of Castleman disease and was a mainstay of treatment in other reported cases.

Case report: The stroma-rich variant of Castleman's disease of hyaline-vascular type with atypical stromal cell proliferation and malignant potential: An exceptional rare case occurred in mediastinal lymph node.

The stroma-rich variant of Castleman disease of hyaline-vascular type (SR-HVCD) is characterized by interfollicular proliferation of the fibroblastic, myofibroblastic, and/or histiocytic-derived stromal cells, occurred in a background of Castleman disease of hyaline-vascular type (HVCD). It has been considered as a hyperplastic disorder by far. Herein, we presented a case of a 40-year-old male suffering from an occupation in the right middle mediastinum. Microscopically, the lesion was characterized by atretic lymphoid follicles and overgrowth of the interfollicular spindle-shaped cells. Those spindle cells were histologically bland in some areas, while exhibited notable cellular atypia and focal necrosis in other areas. SMA and CD68 were immunostained with a subset of the spindle cells in both areas, whereas p53 staining was only perceived in areas with markedly cellular atypia. In addition, indolent T-lymphoblastic proliferation (iT-LBP) was present inside the lesion. The patient developed multiple sites metastases 4 months after surgery, and succumbed to the disease at 7 months. Our case demonstrates for the first time that SR-HVCD have a tumorigenesis potential rather than a simple hyperplastic process. Such disorder should be carefully evaluated to avoid underdiagnosis.

CT of Castleman disease in the mediastinum.

Castleman disease is a rare lymphoproliferative disease commonly occurring as a benign localized mass of lymph nodes in the mediastinum. Given that Castleman disease presents as asymptomatic or through non-specific thoracic symptoms, detection is considered complex. Ultimately, surgical resection is the preferred course of action with a greater than 90% relapse-free survival and no malignant transformation reported. In this article, we describe the case of a 34-year-old male with an unclear smoking history who was diagnosed with hyaline-vascular Castleman disease. We focus on optimizing diagnosis and management through the application of radiological imaging modalities, including computed tomography scans.

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.

The Role of Radiation Therapy in Unicentric Castleman Disease: A Case Report.

Castleman disease (CD) is a rare lymphoproliferative disorder characterized by localized (unicentric) or systemic (multicentric) lymphadenopathy. This study presents a unique case of a 29-year-old female with a rare pelvic presentation of unicentric Castleman disease, specifically the hyaline vascular variant. Despite surgical resection, an unresectable residual lesion prompted adjuvant radiotherapy and subsequent chemotherapy. The literature highlights surgical resection as the primary treatment for localized Castleman disease; however, radiotherapy and combined chemotherapy regimens like cyclophosphamide, doxorubicin, vincristine, and prednisone (CHOP) have shown promise in unresectable cases, emphasizing a multidisciplinary approach. This case underscores the importance of tailoring treatment strategies for uncommon Castleman disease presentations.

A Rare Diagnosis of Nasal Mass:Castleman's Disease.

Castleman disease (CD) is a rare disease of lymph nodes and related tissues. The disease can occur anywhere in the body where lymphoid tissue is normally present. Mediastinum is the most common site of involvement (approximately 70%) followed by the neck (15%) and the abdomen (15%) but extra lymphatic sites such as lungs, larynx, parotid glands, pancreas, meninges can be involved. Localised nasopharyngeal CD is extremely rare and to our knowledge the present case is the only case reported originating from the nasal cavity per se. According to literature only 4 cases of CD in nasopharynx has been reported till date. Microscopic subtypes are hyaline vascular, plasma cell, mixed, plasmablastic type. Hyaline vascular is the most common (90%) histological subtype. Four other cases were reported earlier, in which the nasopharynx was the site of origin. Our case had histopathologic features consistent with a mixture of plasma cell and hyaline vascular variant CD, while the previous four reported cases were of hyaline vascular type, making ours the first of its kind.

Multicentric Castleman Disease: The Hyaline Vascular Type Without HIV Association in a Young Male With Cutaneous Manifestation and Renal Impairment.

Castleman disease (CD), a heterogenous lymphoproliferative disorder resulting from immune dysregulation, is a very rare disease in clinical practice. The clinical spectrum of Castleman disease is wide and its treatment options are mostly based on case reports and case series. To date, two clinical and four histological types have been described. It has recently been successfully demonstrated that the pathogenesis of multicentric Castleman disease (MCD) has a direct association with human immunodeficiency virus (HIV) and human herpes virus 8 (HHV-8) infection which is why further studies are necessary. Here, we report an unusual case of MCD not associated with HIV and having a histological diagnosis of the hyaline vascular type that presented with acute renal impairment and subcutaneous abnormal lymphatic proliferation.

Hyaline Vascular Type of Unicentric Castleman Disease in a Kidney with End-Stage Renal Disease: A Case Report of a Rare Entity at an Unusual Location and a Special Clinical Setting.

Castleman disease (CD) is an unusual heterogeneous lymphoproliferative disorder that has been classified based on either clinical presentation and disease course or histologic features. Clinically, CD is divided into a unicentric CD (UCD) type and multicentric CD (MCD) type according to the extent of lymph node region involvement and the absence or presence of systemic symptoms. Histologically, it can be categorized into hyaline vascular (HV) type, plasma cell (PC) type and mixed type. The majority of HV-type CD involves a solitary lymph node, and excision surgery is often curative. On the contrary, MCD is a progressive and often fatal disease with lymphadenopathy in multiple nodes, and systemic therapy is needed. Herein we report a unique case of HV-type CD presenting as a single renal mass in a patient with end-stage renal disease (ESRD). Despite the rarity, CD should be included in the differential diagnosis of solitary renal mass lesions. An accurate diagnosis is important to avoid unnecessarily risky or extensive operations.

Case report: Castleman's disease involving the renal sinus resembling renal cell carcinoma.

Introduction: Castleman's disease (CD) is a rare benign lymphoproliferative disease that frequently involves the mediastinal thorax and the neck lymph nodes. It rarely affects extrathoracic presentations, with even fewer presentations in the renal sinus. Patient concerns: In this report, we present a case of a 40-year-old woman with no significant past medical history who presented Castleman's disease arising in the renal sinus. Diagnosis and interventions: The patient visited our hospital with the chief complaint of left renal sinus lesion after renal ultrasonography by regular physical examination. Subsequent abdominal computed tomography urography revealed a soft tissue mass with heterogeneous obvious enhancement in the sinus of the left kidney, which was suspected to be a renal malignant tumor. Hence, the patient underwent a left radical nephrectomy. Histological examination revealed hyperplastic lymphoid follicles in the renal sinus and was finally diagnosed as Castleman's disease of the hyaline vascular type. Outcomes: Five days after the surgery procedure, the patient was discharged. Conclusion: Due to the low incidence of Castleman's disease in renal sinus, there is a strong likelihood of missed diagnosis or misdiagnosis, and it is, therefore, important to be aware of the risk. Heightened awareness of this disease and its radiographic manifestations may prompt consideration of this diagnosis. Therefore, we explored the radiologic findings to find out some radiologic features suggesting this condition to help clinicians to schedule nephron-sparing surgery in the future.

Castleman disease: Report of a case and review of literature on role of preoperative embolization.

INTRODUCTION: Castleman disease, which was first described by Dr. Castleman in 1954, is relatively rare and represents a spectrum of heterogeneous lymphoproliferative disorders with characteristic histological features on biopsy. It is classified based on body location and histology with variable clinical presentations. Its treatment depends on the subtype, and preoperative embolization for Castleman disease has rarely been discussed in the literature. PRESENTATION OF CASE: A 22-year old man presented to the ENT clinic with a four-week history of a mass on the left side of the neck, which was associated initially with headache, fever, and fatigue for 2 days. Contrast tomography and magnetic resonance imaging revealed a hypervascular mass located at levels two and three of the left side of the neck with feeding vessels from the external carotid artery. Preoperative embolization was planned; however, the neurointerventionist considered it a lymph node that did not need embolization. Surgical excision was performed with relatively increased operative time and bleeding. A biopsy confirmed a hyaline-vascular type Castleman disease. DISCUSSION: We reviewed the evidence-based management of CD. We reviewed the available literature on the role of preoperative embolization in management. CONCLUSION: Based on published articles and the hypervascular nature of the disease, we believe that preoperative embolization helps decrease morbidity.

Glucocorticoid-induced redistribution lymphocytosis in mantle cell lymphoma with hyaline vascular Castleman disease-like features.

We report a case of mantle cell lymphoma mimicking Castleman disease. A 76-year-old man presented with generalized lymphadenopathy, splenomegaly, anemia, polyclonal gammopathy, and pulmonary infiltrations. Lymph node biopsy revealed histological features of hyaline vascular Castleman disease. Treatment with prednisolone induced lymphocytosis with immunophenotypic and genetic features of mantle cell lymphoma. A detailed immunohistochemical study of the lymph node demonstrated a mantle cell lymphoma-mantle zone growth pattern. Glucocorticoid-induced distribution lymphocytosis has not been reported in mantle cell lymphoma. Careful observation of circulating lymphocytes during steroid treatment may enable diagnosis of the underlying occult lymphoma in a subset of patients exhibiting clinical manifestations of Castleman disease.

Cytology of Castleman's disease (hyaline-vascular type) masquerading as Hodgkin's lymphoma.

Castleman disease (CD) is a rare benign disorder presents as a lymph nodal mass in mediastinum, cervical, axillary or abdomen. Due to the presence of dysplastic dendritic cell in a background mature lymphocyte and plasma cell, it mimics Hodgkin disease (HD). Synchronous and metachronous occurrence in HD and CD can also occur. An 11-year-old male presented with cervical lymphadenopathy (3.5 × 3.5 cm). Fine needle aspiration shows atypical binucleate cell in a background of small lymphocytes, a diagnosis of Hodgkin disease is suggested. Excisional biopsy showed classical features of Hyaline vascular Castleman disease. Careful cytological evaluation and clinical correlation is required for definitive diagnosis.

Mediastinal Castleman disease with supraclavicular extension-a diagnostic dilemma and surgical challenge.

Castleman's disease, also known as angiofollicular lymph node hyperplasia, is a rare lymphoproliferative disorder, with unicentric or multicentric variety. Herein, we present an unusual case of unicentric Castleman disease in a 19-year-old boy presenting as a left posterior mediastinal mass with supraclavicular extension, a rare form of presentation, with very few cases reported in the past. It can be misdiagnosed as other malignant pathology and mismanaged as in our case; hence, a high index of suspicion is necessary.