case_images: PMC10937079_01
case_images: Clinical course and presentation of Castleman disease with retroperitoneal mass: (A) course of patient diagnosis and treatment. (B)-(E) physical findings from April 2019 showing (B) papular rash on the patient's trunk, (C) ulcerative stomatitis, (D) violaceous sanguineous cuticle encrustations, and (E) genital ulcers; (F) chest CT with red arrows indicating signet rings; (G, H) abdominal CT with red dotted circles indicating the retroperitoneal tumor.
case_images: CRII2024-9382107.001.jpg
case_images: PMC10937079_01_CRII2024-9382107.001.jpg
case_images: fig1
case_images: A previously healthy 19-year-old male presented to the clinic with stomatitis in March 2018 (Figure 1(A)). The physical findings were consistent with mucocutaneous pemphigus vulgaris (Figure 1(B)-1(E)). Chlamydia pneumoniae were positive, while the PCR detection of Mycoplasma pneumoniae was negative. CT of the chest, abdomen, and pelvis revealed ground glass opacities and bronchiectasis with signet rings (Figure 1(F)) and a 12 x 7.2 cm retroperitoneal mass with associated lymphadenopathy (Figures 1(G) and 1(H)).
case_images: Diagnosis of unicentric hyaline vascular type Castleman disease: (a)-(d) immunohistochemical staining of the retroperitoneal mass showing (a) onionin-skinning and CD21 positive lymphocytes, (b) paracortical vascular penetration, (c) pan-IgG staining, and (d) flow cytometric immunophenotyping tumor biopsy and resection; (e) patient pulmonary function tests; (f) clinical data collected before and after surgery including weight, hemoglobin, white blood cell count, red blood cell count, and platelet count. Vertical dashed red line indicates the date of surgery; (g, h) white blood cell differential by (g) percent and (h) absolute number throughout the clinical course. Green designates to segmented neutrophils. Blue designates lymphocytes. Yellow designates monocytes. Red designates eosinophils. Purple designates basophils. Gray designates immature granulocytes.
case_images: CRII2024-9382107.002.jpg
case_images: PMC10937079_01_CRII2024-9382107.002.jpg
case_images: fig2
case_images: The tumor was surgically resected and revealed atretic follicles with paracortical vascular penetration and onion-skinning, consistent with unicentric hyaline vascular type CD (HV-CD) (Figure 2(a)-2(c)). IgG were identified using pan-IgG immunohistochemical staining. Immunophenotyping of the biopsy and resected tumor samples supported a B-cell predominant lymphocyte population in the HV-CD tumor (Figure 2(d)). Repeated pulmonary function tests revealed persistent severe obstruction consistent with bronchiolitis obliterans seen in the Castleman triad even after surgical resection of the tumor (Figure 2(e)). Over the disease course, the patient lost 16.8 kg, and prior to tumor removal, the patient had microcytic anemia and lymphopenia (Figure 2(f)-2(h)).