Case Of Unicentric Castleman's Disease Research Articles

A rare case report of hyaline vascular variant of Castleman\\\'s disease

Castleman's disease, is a heterogenous spectrum of lymphoproliferative disorder also known as angiofollicular lymph node hyperplasia or giant lymph node hyperplasia, offers a diagnostic dilemma enriched with histological differences. Its manifestations, range from localized to systemic manifestation with progression to fatal disease involving multiple organs. It has 3 histological types- hyaline vascular variant (HVCD), plasma cell variant (PCCD) and mixed hyaline vascular and plasma cell variant. Clinically it can be classified as unicentric and multicentric. Here we report a case of unicentric Castleman disease in a 55 year old woman who presented with a swelling in the posterior aspect of the neck which was radiologically diagnosed as lipoma and histologically to proven to be a unicentric hyaline vascular type of Castleman's disease.

CASTLEMAN DISEASE : A CASE REPORT OF RARE CAUSE OF CERVICAL LYMPHADENOPATHY

Castleman disease (CD) is a rare lymphoproliferative disorder characterized by enlargement of lymph nodes with two primary subtypes that vary in presentations and course. Unicentric Castleman disease (UCD) presents as a solitary mass, most commonly in the mediastinum, and rarely in the head and neck . In contrast to multicentric Castleman disease (MCD),which features generalized lymphadenopathy and numerous systemic symptoms, a more aggressive clinical course and relatively poorer prognosis .The most common histopathological subtype is the hyaline vascular variant .We present the case of an 14 years old female presented with a slowly expanding painless left sided neck swelling. Physical examination, coagulation tests, infectious disease screening (HIV, HCV and HBsAg), ESR test and chest X-ray were all normal. Both computed tomography (CT) and Ultrasonography (USG) ndings showed a well-dened solid enhancing mass lesion in the left supraclavicular fossa, which was completely excised and diagnosed to have hyaline vascular variant of unicentric Castleman disease .This disorder carries a an excellent prognosis and does not require further therapy if complete excision of the involved lymph node has been done. We report a case of unicentric Castleman disease is a rare cause of cervical lymphadenopathy , diagnosis by excision biopsy and surgical resection is curative. However ,Histopathological examination rules out lymphoma and concluded that it is unicentric Castleman disease, hyaline-vascular variant .

Unicentric Castleman disease: multidetector computed tomography classification with surgical and pathologic correlation.

This study aimed to correlate multidetector computed tomography (MDCT) classification of unicentric Castleman disease with the surgical and pathologic features. The imaging manifestations of 63 cases of unicentric Castleman disease confirmed by pathology were retrospectively analyzed. Every patient underwent an MDCT examination. Classification based on imaging manifestations, surgical, histopathological, and imaging features were simultaneously reviewed and analyzed by two radiologists, with any disagreements resolved by consensus. Sixty-three patients with unicentric Castleman disease were divided into I-IV types by imaging manifestations: type I, single mass with smooth margin (n=5); type II, single mass with irregular or lobulated margin (n=33); type III, single invasive mass with blurred margin (n=20); and type IV, multiple fused masses (n=5). Thirty-eight cases of type I and type II were diagnosed as hyaline-vascular type by pathology after complete surgical resection; 20 cases were type III, in which eight cases were partially resected, 17 cases were pathologically diagnosed as hyaline-vascular type, and the remaining three cases were a mixed type. In five cases of type IV that could not be completely resected, four cases were hyaline-vascular type, and one case was plasma cell type. MDCT is an excellent tool for the detection and diagnosis of unicentric Castleman disease. Classification by MDCT features is a reliable method for evaluating tumor infiltration and growth mode, which helps surgical optimization.

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.

Unicentric Castleman Disease with High Inflammatory State: Surgical Treatment Is a Potential Cure

Objective: Castleman disease (CD) is a group of rare lymphoproliferative disorders which are divided into unicentric CD (UCD) and multicentric CD (MCD) according to the number of lymph node regions involved. In the case of UCD, no or only local compressive symptoms are present with normal biochemistry, and prognosis is great after complete surgical resection. However, we have noticed that a small portion of UCD patients presenting with severe constitutional symptoms accompanied by high inflammatory state might resemble MCD in clinical presentations and biochemical features. Since our current understanding of this group of patients is far from enough, we are here to summarize the characteristics, treatments and outcomes of these high inflammatory patients and compare them with other UCD patients to see whether surgery still serves well as first-line treatment, so as to illuminate the biological behavior of these hyper-inflammatory UCD patients. Method: We retrospectively analyzed 123 cases of UCD diagnosed between Jan 2000 and Dec 2019 in Peking Union Medical College Hospital. The patients with high inflammatory state who met the laboratory and clinical criteria for idiopathic MCD (Blood, 2017) were included in the hyper-inflammatory group (Figure 1). We summarized their clinical characteristics, treatments and outcomes. Response criteria of iMCD defined by Castleman Disease Collaborative Network (CDCN) were cited to evaluate treatment response in symptoms and biochemistry. Kaplan-Meier analysis were used to analyze the progress-free survival (PFS) and overall survival (OS), and comparison was made with other UCD patients through Log-rank test. Result: Among 24 UCD patients with high inflammatory state (19.5%), when compared to others without, constitutional symptoms, organomegaly, fluid retention and complication of paraneoplastic pemphigus (PNP) or bronchiolitis obliterans (BO) were more common. ESR or CRP elevation, anemia, abnormal platelet count, albumin decrease, renal function impairment and IgG increase were also more usually discovered (Table 1). Meanwhile, plasmacytic (PC) histopathology was more frequently seen (45.8% vs 14.1%, P=0.001) (Table 2), but no statistical significance was shown on the lymph node region involved. As for first-line treatments (Figure 1), 18 UCD patients with high inflammatory state (75.0%) underwent complete surgical excision alone; there was no recurrence of lymphadenopathy after surgery, and the inflammation resolved completely except 2 cases of disease progression (both complicated by PNP), achieving a response rate of 88.9%. 2 patients (8.3%) received chemotherapy together with surgery, and all disease completely resolved. Among the 3 patients (12.5%) treated only with chemotherapy, 1 experienced symptomatic and biochemical progression of disease. Finally, there was one patient (4.17%), after a watch-and-wait period, experienced progression in symptoms. In contrast, disease progression was not seen in UCD without high inflammatory state. There was no statistical difference between median follow-up time of the two groups. UCD patients without high inflammatory state have a better PFS curve [P<0.001, HR=0.005 (95%CI: 0.001-0.043)], but median PFS was not reached in either group of patients (Figure 2). The overall survival was not significantly different between the two groups [P=0.225, HR=4.722 (95%CI: 0.123-181)]. Conclusion: UCD patients with high inflammatory state are not uncommon. Although they resemble MCD with more PC histopathology, more severe symptoms and abnormal lab results, the good response to surgical treatment suggested that patients with high inflammatory state still shared common biological behavior with other UCD. However, recurrence of symptoms and progression of inflammation might still happen in patients with PNP or unresectable disease, so treatment options other than surgery should be considered in these patients. Figure Disclosures No relevant conflicts of interest to declare.

Unicentric Castleman disease and lymphoepithelial cyst of the parotid: A case report and review of the literature

AbstractObjectivesPresent the first reported case of unicentric Castleman disease and lymphoepithelial cyst of the parotid in an HIV‐negative adult.MethodsRetrospective case presentation.ResultsCastleman disease is an uncommon disorder of benign hypervascular lymphoid hyperplasia with an estimated incidence rate of 15.9‐19.1 cases per million person‐years (Leukemia & Lymphoma, 56, 2015: 1252). Parotid involvement is extremely rare, with less than 30 cases reported in the literature (Case Reports in Otolaryngology, 2015, 2015: 1). Benign lymphoepithelial cysts (BLECs) are another rare parotid lesion presenting most commonly in HIV‐positive patients. Here we present the case of a 42‐year‐old HIV‐negative male who presented with 3 cm parotid mass. The patient underwent superficial parotidectomy with final pathology demonstrating BLEC with the hyaline‐vascular subtype of Castleman disease. The patient has had no recurrence of symptoms in a 3‐month follow‐up period.ConclusionsThe practitioner should consider unicentric Castleman disease and BLEC in HIV‐negative patients presenting with a new head and neck mass.

Unicentric castleman disease in the differential diagnosis of a paravesical mass: A case report

Castleman disease is a rare condition with variable forms of clinical presentation. Diagnosis is therefore difficult and the ideal management of the disease has yet to be defined. Prognosis varies as a function of histologic type. Treatment can range from curative surgery for the unicentric form of the disease to a combination of treatments for the multicentric form. This paper describes an unusual case of unicentric Castleman disease in which the differential diagnosis included several gynaecological malignancies. The lesion was resected, with histopathology and immunohistochemistry confirming the diagnosis. The surgical procedure was completed without complications and follow-up of the patient over one year has shown no signs of recurrence. A literature review was performed, and the possible conditions to be included within the differential diagnoses are discussed.

A Case of Unicentric Castleman Disease Diagnosed by FDG-PET/CT

A Case of Unicentric Castleman Disease Diagnosed by FDG-PET/CT

Castleman Disease in the Buccal Space Mimicking Lymphoma: A Case Report

Castleman disease is a benign lymphoproliferative disorder of unknown etiology, more commonly appearing in the mediastinum. Craniofacial involvement is rare. Surgical excision is the only intervention needed for the unicentric type, while systemic treatment would be necessary for the multicentric type. Here, we report a rare case of unicentric castleman disease of the buccal space of a 23-year-old woman. Additionally, we review the radiological and histopathological features of this lesion.

Treatment and outcome of Unicentric Castleman Disease: a retrospective analysis of 71 cases.

We retrospectively analysed 71 cases of Unicentric Castleman disease, a rare, usually asymptomatic, benign lymphoproliferative disorder presenting as a unique nodal mass. Although surgery is considered as the gold standard therapy, only 38 patients (54%) underwent initial surgical resection and 95% were cured. An additional 9 patients had surgery after an attempt at medical reduction. Reduction therapy was used in 21 patients with a 55% response rate, but without evidence for an optimal regimen. Radiotherapy was limited to 8 patients because of associated toxicity. Watch and wait was considered in 13 asymptomatic patients and 11 of these remained stable for up to 17years.

Unicentric Castleman disease in the paediatric neck: an unusual case of a rare diagnosis

We present a rare case of unicentric Castleman disease (CD) in the paediatric neck. A 12-year-old Caucasian girl was referred to us due to a slowly enlarging, painless right-sided neck mass. Our thorough investigative approach identified unicentric CD, with previously undocumented CD-like features occurring simultaneously in a separate lesion from the lymph node group involved in unicentric CD. Recognition of CD in the paediatric patient is essential, despite the condition’s rarity, to ensure adequate diagnostic work-up and management. We discuss features of unicentric CD, current concepts surrounding histology of the two separate variants of unicentric and multicentric CD, and the implications for management.

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.

A Palpable Painless Axillary Mass as the Clinical Manifestation of Castleman's Disease in a Patient with Hepatitis C Disease.

Introduction. Castleman's disease (CD) is a rare lymphoproliferative disorder. CD is divided into two clinical subtypes: the most common unicentric and the less usual multicentric subtype. The majority of unicentric CD affects the mediastinum, while neck, abdomen, and axilla are less common locations. Case Presentation. Herein, we describe a rare case of unicentric CD in the right axilla in a 36-year-old white male with a medical history of hepatitis C virus infection admitted to our hospital due to palpation of a painless mass in the right axilla. Complete excision of the lesion was performed and, one year after the diagnosis, patient was free of the disease. Conclusions. Although infrequent, it is important to include CD in the differential diagnosis when evaluating axillary lymphadenopathy particularly in young patients with a low-grade inflammation process and chronic disease even in the absence of an abnormal blood picture or organomegaly.

Unicentric Castleman disease at an unusual site

We report a rare case of unicentric Castleman disease (UCD) in a 56-year-old lady, presenting as a soft-tissue mass in an unusual site - mid-dorsal aspect of the arm. She was otherwise asymptomatic. Excision biopsy confirmed the diagnosis. The probable etiological factors, clinical and morphological types, and histological diagnosis are discussed.

Intensity modulated radiation therapy (IMRT) for the treatment of unicentric Castlemans disease: a case report and review of the use of radiotherapy in the literature

BackgroundSurgical resection is considered standard therapy for cases of resectable unicentric Castleman’s disease (UCD). Unresectable cases of UCD do not have a consensus regarding the optimal treatment approach, but have utilized steroids, observation, chemotherapy, and radiotherapy. Here we discuss a patient presentation of UCD treated with an advanced radiotherapy technique, IMRT.Case reportA 47 year old female was found to have an intra-thoracic posterior UCD and was determined not to be a good surgical candidate. She was referred for radiotherapy and was treated using IMRT to a total dose of 4320 cGy in 180 cGy fractions including a scheduled 10 day break. Following the break, the patient’s treatment was replanned at which the initial treatment volume was reduced by 50.9% for the duration of the treatment course. Radiation Therapy Oncology Group (RTOG) grade III pneumonitis developed which was managed medically. Neither disease progression nor late effects have occurred.ConclusionsThe use of IMRT and planned treatment break was successful in the treatment of a case of UCD, and should be considered for other unresectable cases.

Rare Case of Castleman Disease Involving the Pancreas

Castleman disease, also known as angiofollicular lymph node hyperplasia, is an uncommon tumor involving lymph node tissues. The most common location is in the thorax presenting as a mediastinal mass. In the abdomen, most of the lesions are located in the pelvis, mesentery, and retroperitoneum. Only nine other cases of Castleman disease involving the pancreas have been described. We report a case of Castleman disease (hyaline vascular type) presenting as a pancreatic mass and an enlarged spleen. A 23-year-old woman presented with a 2-month history of abdominal pain localized to the left upper quadrant. Ultrasound and CT demonstrated a large retrogastric pancreatic mass and an enlarged spleen. Distal pancreatectomy and splenectomy were performed. Histopathologic examination revealed a markedly enlarged lymph node showing germinal centers with hyalinization and prominent follicular dendritic cells meshwork surrounded by concentric layers of small lymphocytes. Within the interfollicular area were regions of increased vascularity. BCL2, CD20, and CD79a show expanded mantle zones with a concentric targetoid arrangement of small lymphocytes. This is a case of unicentric Castleman disease of the hyaline-vascular type, which presented with signs and symptoms of a primary pancreatic neoplasm.

Unicentric and multicentric Castleman's disease

Castleman's disease (CD) appears at ubiquitous lymph nodes. To date, detection of the lesion focus for CD has mainly been carried out by physical examination and radiological findings, such as X-ray analysis, CT and MRI. 18F-FDG PET visualizes the active focus of glucose metabolism and the clinical value has been investigated for many different tumours. Previous studies of 18F-FDG PET for CD have only reported four cases of unicentric CD and no cases of multicentric CD. In this paper, we report two cases of CD, one with unicentric CD and one with multicentric CD. We demonstrate that the use of 18F-FDG PET for the detection and monitoring of patients with CD, especially multicentric CD, would be effective.