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Multilocular thymic cysts in children with human immunodeficiency virus infection: clinical and pathologic aspects.

Publication ,  Journal Article
Kontny, HU; Sleasman, JW; Kingma, DW; Jaffe, ES; Avila, NA; Pizzo, PA; Mueller, BU
Published in: J Pediatr
August 1997

BACKGROUND: Children with human immunodeficiency virus (HIV) infection have an increased susceptibility to severe and unusual infections, malignancies, and disorders characterized by abnormal lymphoproliferation (e.g., lymphoid interstitial pneumonitis). We report a novel disease entity associated with pediatric HIV infection that is characterized by massive enlargement of the thymus as a result of lymphoid hyperplasia and multicystic changes. METHODS: Eight patients with HIV infection and cystic enlargement of the thymus are subject of this report. The status of their HIV disease and its clinical and radiologic manifestations at the time of diagnosis of the mediastinal mass are described. Tissue specimens were obtained from six patients and examined by microscopy and immunohistochemistry. The specimens were also evaluated for the evidence of HIV and Epstein-Barr virus by in situ hybridization. RESULTS: Patients were between 2.1 and 12.1 years of age, with CD4+ cell counts between 102 and 733 cells/mm3. In all eight cases an anterior mediastinal mass was discovered incidentally on radiography of the chest, and computed tomography of the chest revealed a multicystic appearance. Histologic examination demonstrated distortion of the thymic architecture by focal cystic changes, lymphoid follicular hyperplasia, diffuse plasmacytosis, and multinucleated giant cells. In situ hybridization revealed HIV particles on the surface of follicular dendritic cells. Further, results of in situ hybridization for EBV were positive in lymphoid cells from biopsy samples of four patients. The patients were followed between 8 months and 4.8 years. In five patients the mass either decreased in size or resolved completely. CONCLUSIONS: We describe a series of children with HIV infection and multilocular thymic cysts. We hypothesize that aberrant immunoregulation in these HIV-infected children leads to follicular hyperplasia and multicystic changes in the thymus, causing massive enlargement. EBV infection might also contribute to the pathogenesis of this process. Because none of our patients had symptoms from the mass, and there was no evidence of malignancy in the examined biopsy samples, it seems prudent to manage such children with careful follow-up examinations.

Duke Scholars

Published In

J Pediatr

DOI

ISSN

0022-3476

Publication Date

August 1997

Volume

131

Issue

2

Start / End Page

264 / 270

Location

United States

Related Subject Headings

  • Tumor Virus Infections
  • Tomography, X-Ray Computed
  • Thymus Gland
  • Radiography, Thoracic
  • Plasma Cells
  • Pediatrics
  • Mediastinal Cyst
  • Male
  • Lymphoproliferative Disorders
  • Lymphoid Tissue
 

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Kontny, H. U., Sleasman, J. W., Kingma, D. W., Jaffe, E. S., Avila, N. A., Pizzo, P. A., & Mueller, B. U. (1997). Multilocular thymic cysts in children with human immunodeficiency virus infection: clinical and pathologic aspects. J Pediatr, 131(2), 264–270. https://doi.org/10.1016/s0022-3476(97)70164-1
Kontny, H. U., J. W. Sleasman, D. W. Kingma, E. S. Jaffe, N. A. Avila, P. A. Pizzo, and B. U. Mueller. “Multilocular thymic cysts in children with human immunodeficiency virus infection: clinical and pathologic aspects.J Pediatr 131, no. 2 (August 1997): 264–70. https://doi.org/10.1016/s0022-3476(97)70164-1.
Kontny HU, Sleasman JW, Kingma DW, Jaffe ES, Avila NA, Pizzo PA, et al. Multilocular thymic cysts in children with human immunodeficiency virus infection: clinical and pathologic aspects. J Pediatr. 1997 Aug;131(2):264–70.
Kontny, H. U., et al. “Multilocular thymic cysts in children with human immunodeficiency virus infection: clinical and pathologic aspects.J Pediatr, vol. 131, no. 2, Aug. 1997, pp. 264–70. Pubmed, doi:10.1016/s0022-3476(97)70164-1.
Kontny HU, Sleasman JW, Kingma DW, Jaffe ES, Avila NA, Pizzo PA, Mueller BU. Multilocular thymic cysts in children with human immunodeficiency virus infection: clinical and pathologic aspects. J Pediatr. 1997 Aug;131(2):264–270.
Journal cover image

Published In

J Pediatr

DOI

ISSN

0022-3476

Publication Date

August 1997

Volume

131

Issue

2

Start / End Page

264 / 270

Location

United States

Related Subject Headings

  • Tumor Virus Infections
  • Tomography, X-Ray Computed
  • Thymus Gland
  • Radiography, Thoracic
  • Plasma Cells
  • Pediatrics
  • Mediastinal Cyst
  • Male
  • Lymphoproliferative Disorders
  • Lymphoid Tissue