Primary hepatic Castleman disease.

Castleman disease (CD) is a rare, benign lymphoproliferative disorder with characteristic histopathological features, but variable aetiology, presentation, treatment and prognosis. It is broadly classified based on its location and histopathological features, with unicentric hyaline vascular disease presenting most commonly. We present a case of primary, unicentric hepatic CD that was incidentally found on imaging and managed with laparoscopic resection.

Hodgkin Lymphoma-related Inflammatory Modification-displayed Castleman Disease-like Histological Features and Positron Emission Tomography/Computed Tomography Usefulness for the Differential Diagnosis.

Hodgkin lymphoma (HL) and idiopathic multicentric Castleman disease (iMCD) are markedly different conditions. However, in some cases, histological similarities caused by elevated cytokines, including interleukin-6, can lead to a misdiagnosis of HL as Castleman disease (CD). We herein report a patient with HL who had been diagnosed with CD by an expert panel and for whom an additional biopsy was useful for determining the correct diagnosis. Furthermore, we analyzed the positron emission tomography/computed tomography findings at the diagnosis and found that the maximum standardized uptake value was useful for distinguishing HL from iMCD.

[Left pneumonectomy for intrapulmonary unicentric Castleman disease]. / Bal oldali teljes tüdoeltávolítás intrapulmonalisan elhelyezkedo Castleman betegség miatt.

Castleman disease is an uncommon lymphoproliferative disorder, located most commonly in the mediastinal lymph nodes. The intrapulmonary presentation is extremely rare, with 14 published cases in the English literature. The clinical presentation of the disorder is not specific and the diagnosis is often challenging. The main goal is to achieve an R0 surgical resection which gives the potential chance for a recurrence-free survival. We present the case of a symptomless, 15-year-old female patient with left-sided tumor mass. Transthoracal invasive tissue biopsy confirmed Castleman disease. The central mass involved the main structures in the left hilus and therefore left pulmonectomy was necessary to reach the complete, tumor-free resection margins. The patient had no local or distant relapse during the 7-year follow-up. Although Castleman disease treated by complete surgical resection provides excellent results, radical and extended lung resection is sometimes inevitable to reach tumor-free margins. Orv Hetil. 2023; 164(37): 1476-1483.

Mesenteric Castleman Disease Misdiagnosed as Lymph Node Metastasis of Rectal Cancer on 18 F-FDG PET/CT.

ABSTRACT: Two years ago, a 64-year-old man underwent an 18 F-FDG PET/CT for staging rectal cancer. Besides the hypermetabolic rectal lesion, the image revealed a mesenteric lymph node with intense activity and multiple lung nodules with slight FDG uptake, which were highly suspected of metastases. After surgery and multiple cycles of chemotherapy, the follow-up 18 F-FDG PET/CT showed remission of all lesions except for the enlarged mesenteric lymph node with higher metabolic activity. Serum CEA remained normal during the follow-up. Postoperative pathology of the mesenteric lymph node confirmed Castleman disease.

Castleman disease masquerading as a paraganglioma showing uptake on DOTATATE PET.

A man in his 50s presented with shortness of breath and was found to have a large paracaval mass, which on further evaluation with CT, showed a large heterogeneously enhancing mass lesion adjacent to right renal hilum. His 24-hour urinary metanephrines and nor-metanephrines were normal. In view of location and features on CT, paraganglioma was considered as a possibility. A 68Ga-DOTATATE positron emission tomography/CT demonstrated avidity in the lesion with maximum standardised uptake value of 16.5. He underwent right laparoscopic converted to open mass excision along with nephrectomy. Histopathological examination was suggestive of hyaline vascular Castleman disease.

A Rare Case of Orbital Castleman Disease with Overlapping IgG4-Related Disease.

Multicentric Castleman disease (MCD) is a systemic lymphoproliferative disorder that can lead to mass lesions in various body parts, including the lungs, kidneys, and extranodal sites. Meanwhile, orbital Castleman disease is extremely rare. Immunoglobulin G4-related disease (IgG4-RD) is a recently recognized fibroinflammatory disorder and is characterized by the formation of tumor-like lesions with lymphoplasmacytic infiltrates, which are enriched in IgG4-positive plasma cells and may present with a characteristic storiform pattern of fibrosis to variable degrees. In this study, we report a case of a 67-year-old Taiwanese man with a 7-year history of bilateral eyelid swelling and proptosis. Orbital magnetic resonance imaging revealed soft tissue lesions in the bilateral intraconal region, demonstrating strong enhancement in the lacrimal glands, and extension into the bilateral infraorbital foramen, suggesting an orbital lymphoproliferative disease. The histopathological results of the intraorbital tumor excision were suggestive of a plasma-cell-predominant mixed-cell variant of MCD. However, the patient also showed definitive signs of IgG4-RD, including lacrimal gland enlargement and histopathological results of plasmacytosis, fibrosis, and germinal centers, with an increased ratio of IgG4 cells and elevated serum IgG4 levels. This case suggests a potential interacting pathway between these two disease entities that needs further studies.

Imaging of Castleman Disease.

Castleman disease (CD) is a group of rare and complex lymphoproliferative disorders that can manifest in two general forms: unicentric CD (UCD) and multicentric CD (MCD). These two forms differ in clinical manifestation, imaging appearances, treatment options, and prognosis. UCD typically manifests as a solitary enlarging mass that is discovered incidentally or after development of compression-type symptoms. MCD usually manifests acutely with systemic symptoms including fever and weight loss. As a whole, CD involves lymph nodes throughout the chest, neck, abdomen, pelvis, and axilla and can have a wide variety of imaging appearances. Most commonly, lymph nodes or masses in UCD occur in the chest, classically with well-defined borders, hyperenhancement, and possible characteristic patterns of calcification and/or feeding vessels. Lymph nodes affected by MCD, while also hyperenhancing, tend to involve multiple nodal chains and manifest alongside anasarca or hepatosplenomegaly. The polyneuropathy, organomegaly, endocrinopathy, monoclonal plasma cell disorder, and skin changes (POEMS) subtype of MCD may demonstrate lytic or sclerotic osseous lesions in addition to features typical of MCD. Since a diagnosis of CD based solely on imaging findings is often not possible, pathologic confirmation with core needle biopsy and/or surgical excision is necessary. Nevertheless, imaging plays a crucial role in supporting the diagnosis of CD, guiding appropriate regions for biopsy, and excluding other potential causes or mimics of disease. CT is frequently the initial imaging technique used in evaluating potential CD. MRI and PET play important roles in thoroughly evaluating the disease and determining its extent, especially the MCD form. Complete surgical excision is typically curative for UCD. MCD usually requires systemic therapy. ©RSNA, 2023 Quiz questions for this article are available in the supplemental material.

Ultrasound findings of Castleman's disease of the parotid gland.

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Enfermedad de Castleman unicéntrica, una causa infrecuente de masa cervical / Unicentric Castleman disease, an infrequent cause of cervical mass

La enfermedad de Castleman (EC) describe una serie de desórdenes linfoproliferativos de patrones histológicos similares, pero de etiologías, presentaciones clínicas y enfrentamientos notoriamente variables. Se presenta el caso de un paciente que consultó por masa cervical, cuyo estudio final concluyó EC unicéntrica, la cual se resolvió de forma quirúrgica. Además, se presenta una revisión actualizada del tema, con foco en la enfermedad unicéntrica.

Castleman's disease (CD) describes several lymphoproliferative disorders with similar histological patterns, but with notoriously variable etiologies, clinical presentations, and management. We present the case of a patient who consulted with cervical mass, whose final study concluded with unicentric CD, which was treated surgically. In addition, an updated review of the subject is presented, focusing on unicentric disease.

Radiological and Pathological Features of Cyst Formation in Idiopathic Multicentric Castleman Disease.

INTRODUCTION: Idiopathic multicentric Castleman disease (MCD) has been reported to form lung cysts at a relatively high rate. However, the radiological and pathological features of cystic formation in MCD are unclear. METHODS: To clarify these questions, we retrospectively investigated the radiological and pathological findings of cysts in MCD patients. Eight consecutive patients who underwent surgical lung biopsies in our center from 2000 to 2019 were included. RESULTS: The median age was 44.5 years, with three males and five females. On the initial computed tomography, cyst formation was found in seven patients (87.5%). All of the cysts were multiple, round, and thin walled, accompanying ground-glass attenuation (GGA) around cysts. In six patients (75%), cysts increased during their clinical courses, and the new cysts had emerged from GGA, although GGA was improved by treatment. In all four cases, whose pulmonary cysts could be pathologically evaluated, a marked plasma cell infiltration around the cyst wall, and loss of elastic fibers of the alveolar wall were observed. CONCLUSIONS: Pulmonary cysts emerged in the area of GGA pathologically consistent with plasma cell infiltration. Cysts in MCD may be formed by the loss of elastic fibers due to marked plasma cell infiltration and may be considered irreversible changes.

A case of idiopathic multicentric Castleman disease with pulmonary hyalinizing granuloma-like multiple pulmonary nodules.

A 41-year-old man presented with chronic cough and chest pain. Laboratory tests revealed anemia, inflammation, hypoalbuminemia, polyclonal hypergammaglobulinemia, and elevated interleukin-6 levels. Computed tomography revealed diffuse bilateral pulmonary nodules and multicentric lymphadenopathy. Histopathology of the pulmonary nodule resembled pulmonary hyalinizing granuloma (PHG), whereas lymph node histopathology was consistent with idiopathic multicentric Castleman disease (iMCD). The patient was diagnosed with iMCD involving PHG-like pulmonary nodules. Little is known about the association between these two diseases, and the present case provides insights regarding the relationship between PHG and iMCD.

[Challenging Case of Primary Renal Castleman's Disease Accompanied with Bacterial Pyelonephritis].

Castleman's disease is a rare lymphoproliferative disease, mostly found in the mediastinum. The number of Castleman's disease cases involving the kidneys is still limited. We report a case of primary renal Castleman's disease sporadically detected during a regular health check-up as pyelonephritis with ureteral stones. In addition, computed tomography showed renal pelvic and ureteral wall thickening with paraaortic lymphadenopathy. A lymph node biopsy was performed, but it did not confirm either malignancy or Castleman's disease. The patient underwent open nephroureterectomy for diagnostic and therapeutic purposes. The pathological diagnosis was renal and retroperitoneal lymph node Castleman's disease with pyelonephritis.

Castleman disease: a rare case in a young woman.

Castleman disease is a rare lymphoproliferative disorder characterized by benign enlargement of lymph nodes. It is divided into unicentric disease, which involves a single enlarged lymph node, and multicentric disease, which affects multiple lymph node stations. In this report, we describe a rare case of a 28-year-old female patient with an unicentric Castleman disease. Computed tomography and magnetic resonance imaging revealed a well-circumscribed large mass in the left neck, characterized by intense homogenous enhancement and suspected for a malignant disease. The patient underwent an excisional biopsy for definitive diagnosis of unicentric Castleman disease and ruled out malignant conditions.