Thymic neuroblastoma in adults

Report of three cases with special emphasis on its association with the syndrome of inappropriate secretion of antidiuretic hormone

Pedram Argani, Robert A. Erlandson, Juan Rosai

Research output: Contribution to journalArticle

Abstract

We report three cases of neuroblastoma arising within the thymus of elderly patients. All tumors consisted of primitive neuroblasts showing focal gangliocytic differentiation within nests of neuropil. All stained for neuroendocrine markers but were negative for cytokeratins and for the MIC2 gene product. One tumor was associated with the syndrome of inappropriate secretion of antidiuretic hormone, an endocrinopathy we found in three of five cases reports of thymic neuroblastoma in adults. Immunohistochemical stains confirmed production of antidiuretic hormone by this tumor. One patient died of progressive disease, one patient is disease free at 18 months, and the other patient died of unrelated causes, a spectrum that reflects the variable clinical behavior others have reported. The possible histogenesis of these purely neural tumors includes malignant transformation of a mediastinal teratoma, aberrantly located sympathetic ganglia, neuroectodermal cells native to the normal thymus, and precursors of thymic epithelial cells that have differentiated along neural lines.

Original languageEnglish (US)
Pages (from-to)537-543
Number of pages7
JournalAmerican Journal of Clinical Pathology
Volume108
Issue number5
StatePublished - Nov 1997

Fingerprint

Inappropriate ADH Syndrome
Neuroblastoma
Thymus Gland
Neoplasms
Sympathetic Ganglia
Neuropil
Teratoma
Keratins
Vasopressins
Coloring Agents
Epithelial Cells
Genes

Keywords

  • Antidiuretic hormone [ADH]
  • Neuroblastoma
  • Thymus

ASJC Scopus subject areas

  • Pathology and Forensic Medicine

Cite this

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abstract = "We report three cases of neuroblastoma arising within the thymus of elderly patients. All tumors consisted of primitive neuroblasts showing focal gangliocytic differentiation within nests of neuropil. All stained for neuroendocrine markers but were negative for cytokeratins and for the MIC2 gene product. One tumor was associated with the syndrome of inappropriate secretion of antidiuretic hormone, an endocrinopathy we found in three of five cases reports of thymic neuroblastoma in adults. Immunohistochemical stains confirmed production of antidiuretic hormone by this tumor. One patient died of progressive disease, one patient is disease free at 18 months, and the other patient died of unrelated causes, a spectrum that reflects the variable clinical behavior others have reported. The possible histogenesis of these purely neural tumors includes malignant transformation of a mediastinal teratoma, aberrantly located sympathetic ganglia, neuroectodermal cells native to the normal thymus, and precursors of thymic epithelial cells that have differentiated along neural lines.",
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