Unicentric Castleman's Disease Arising from an Intrapulmonary Lymph Node

Abstract

Castleman's disease is an uncommon lymphoproliferative disorder of unknown etiology, most often involving the mediastinum. It has 2 distinct clinical forms: unicentric and multicentric. Unicentric Castleman's disease arising from an intrapulmonary lymph node is rare, and establishing a preoperative diagnosis of this disease is very difficult mainly due to a lack of specific imaging features. We report a case of intrapulmonary unicentric Castleman's disease in an asymptomatic 19-year-old male patient who was accurately diagnosed by preoperative computed tomography (CT). The mass was incidentally found on a routine chest X-ray. A subsequent dynamic CT showed a well-defined, hypervascular, soft-tissue mass with small calcifications located in the perihilar area of the right lower lung. Three-dimensional CT (3D-CT) angiography indicated that the mass was receiving its blood supply through a vascular network at its surface that originated from 2 right bronchial arteries. The clinical history and CT findings were consistent with a diagnosis of unicentric Castleman's disease, and we safely and successfully removed the tumor via video-assisted thoracoscopic surgical lobectomy. This case shows that the imaging characteristics of these rare tumors on contrast-enhanced CT combined with 3D-CT angiography can be helpful in reliably establishing a correct preoperative diagnosis.

Castleman Disease of the Thorax: Clinical, Radiologic, and Pathologic Correlation:From the Radiologic Pathology Archives

“…However, since UCD represents a nodal proliferation, it can occur anywhere where lymphatic tissue is present; other common sites in the thorax include the hila, axillae, pleural space, chest wall, and extrapleural soft tissues (43,44,46,47). In rare instances, UCD has been reported in the trachea, esophagus, and even the lungs (41,42,48). Fortunately, although the location in the thorax can vary, the imaging manifestations of UCD are relatively consistent, as the majority of patients present with a solitary mass that demonstrates avid enhancement.…”

Isolated Intrapulmonary Castleman’s Disease: A Case Report, Review of the Literature

“…CD is clinically classified into unicentric (localized) and multicentric (systemic) and then further subdivided histologically into the hyaline vascular (90% of cases), plazma cell (8%-9% of cases), and mixed types (1%-2% of cases). [1][2][3][4] The localized type is a rare form and usually detected incidentally in asymptomatic cases as in our case.…”

“…The value of the PET/CT in the preoperative diagnosis is low because it is inadequate to differentiate the malign diseases and benign diseases. [2][3][4][5] Castleman disease can be a cause of false-positive findings by mimicking metastatic adenopathy. The diagnosis is very difficult in these patients, and a thoracotomy is usually needed for the diagnosis and treatment as our case.…”

“…Intrapulmonary CD is exceedingly rare. [1][2][3] Herein, we report an asymptomatic case of unicentric, intrapulmonary CD, who underwent curative surgery.…”

Intrapulmonary Castleman’s Disease Pretending to Be a Lung Cancer—Work Up of an Intrapulmonary Tumour

“…Intrapulmonary manifestation of CD is even less common, as only 6 cases have been reported in the literature. [1][2][3][4][5][6] CD histologically presenting as the plasma cell type (95%) is associated with severe systemic symptoms such as weakness (81%), fever (71%), weight loss (58%), anorexia and nausea (42%) and signs as splenomegaly (79%), hepatomegaly (63%), oedema and effusion, hypergammaglobulinemia, anaemia and multicentric lymphadenopathy. 9) The localized variant mostly presents as the hyalinevascular type with typical onion-skin arrangement of the lymphatic tissue.…”