Castleman's Disease: Atypical Manifestation in an 11-Year-Old Gir

Capt Vinod K. Gidvani, USAF, MC, Capt Melissa M. Tyree, USAF, MC, Col Samar K. Bhowmick, USAF, MC, Department of Pediatrics, Keesler Air Force Base, Miss

South Med J. 2001;94(2) 

In This Article

Case Report

For several weeks, an 11-year-old girl had a moderate nonproductive cough, prolonged intermittent fever as high as 102°F, nasal congestion, sinus pressure, dysphagia, intermittent shortness of breath, fatigue, and a 5-lb weight loss. She was examined by a pediatrician, and a trial of albuterol via nebulizer treatments was given without any significant change in symptoms. Her medical history was unremarkable, with no history of tuberculosis exposure or travel outside the United States. The only medication she took was pseudoephedrine as needed. Immunizations were up-to-date. Family history revealed breast, ovarian, and uterine cancer on the maternal side. There was no history of leukemia, lymphoma, autoimmune disease, collagen vascular disease, or thyroid disorders.

Physical examination revealed an ill-appearing 11-year-old girl. Height and weight were at the 75th and 90th percentiles, respectively. Vital signs were temperature 97°F, pulse rate 87/min, blood pressure 130/77 mm Hg, respiratory rate 24/min, and oxygen saturation 98% on room air. Head, ear, eyes, nose, and throat examination was unremarkable except for a 1 cm soft, round mobile lymph node at the inferior portion of the left lower anterior cervical chain. No supraclavicular lymph nodes were palpated. The lungs were clear to auscultation. The remainder of the examination was normal with no evidence of systemic lymphadenopathy, abdominal mass, hepatosplenomegaly, rash, or neurologic deficit.

Laboratory workup was significant for the following values: lactate dehydrogenase 979 U/L (150 to 300 U/L normal for age), erythrocyte sedimentation rate 20 mm/hr (normal, 0 to 20 mm/hr), white blood cell count 5.2 x 109/L (neutrophils 38.1%, lymphocytes 47.0%, monocytes 11.9%), hemoglobin 13.7 g/dL, hematocrit 40.3%, and platelet count 359 x 109/L. Serum electrolytes, serum urea nitrogen, creatinine, serum glucose, prothrombin time, partial thromboplastin time, alanine aminotransferase, alkaline phosphatase, total bilirubin, thyroid stimulating hormone, and serum copper measurements were all within normal limits. A screening urinalysis also yielded normal results. Blood type was A positive. Human immunodeficiency virus and Epstein-Barr virus titers were not indicative of either recent or remote infection. A purified protein derivative was also placed, and no induration was seen.

A chest radiograph was obtained to rule out pneumonia. No infiltrate was found on the radiograph, but it was significant for a left mediastinal mass. The chest radiograph was immediately followed by computed tomography (CT) and magnetic resonance imaging of the neck and chest, both of which showed a 4 to 5 cm anterior mediastinal mass with extension in to the middle mediastinum. A gallium scan with single photon emission CT showed increased uptake at the mass, located retrosternally and at the left mediastinum, with no uptake noted elsewhere. A CT of the abdomen and pelvis showed no abdominal mass or evidence of distant nodal disease. The patient had a thoracoscopy with biopsy, with evidence of hyalinized vessels consistent with the hyaline vascular type of Castleman's disease. This was followed by definitive surgical management via sternotomy with thymectomy and excision of the anterior mediastinal mass without complication. Grossly, the lesion was a 4.5 x 3.5 x 2.0 cm oval, reddish-tan mass with a smooth external surface (Fig 1). Histopathologic examination revealed an enlarged lymph node with prominent mantle zones comprised of small, mature lymphocytes arranged in concentric layers with prominent hyalinized vessels (Fig 2). Vessels extending into the surrounding interfollicular tissue had prominent endothelial cells (Fig 3). Of note, the thymus was normal. Flow cytometric surface marker analysis showed no abnormal hematolymphoid cell population and therefore confirmed the histologic diagnosis of hyaline vascular Castleman's disease.

Figure 1.

Gross specimen, including thymus (pyramidal-shaped pieces of gray-tan soft tissue) and lymphoid mass (4.5 x 3.5 x 2.0 cm oval piece of red-tan soft tissue with smooth external surface).

Figure 2.

(click image to zoom) Enlarged lymph node had prominent mantle zones comprised of small, mature lymphocytes arranged in concentric layers.

Figure 3.

Prominent endothelial cells were seen in vessels that extended into surrounding interfollicular tissue.

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