Primary neuroendocrine tumors of the kidney: Morphological and molecular alterations of an uncommon malignancy

Phyu P Aung, Keith Killian, Carrie O. Poropatich, W. Marston Linehan, Maria J. Merino

Research output: Contribution to journalArticle

21 Citations (Scopus)

Abstract

Primary neuroendocrine (NE) tumors of the kidney (PNRTs) are rare and frequently mistaken for other renal and urothelial cancers. We evaluated morphological and molecular findings of 11 PNRTs classified according to the World Health Organization classification of lung NE tumors. Patients included 5 men and 6 women with a median age of 50 years. These tumors occurred in the left (5/11), right (3/11), and horseshoe (1/11) kidney. The histologic patterns were predominantly solid, trabecular, and pseudoglandular. Lymphovascular invasion and calcification were found in 3 and 1 cases, respectively. There were 2 atypical and 9 typical carcinoids. At the time of surgery, 2 patients with atypical carcinoids had hepatic metastasis, and 1 of the typical carcinoid patients had lymph node metastasis. All cases showed<1% proliferative rate, except 2 cases with hepatic metastasis, which showed 3% to 5% with MIB1/Ki-67 immunostaining. Immunostainings were frequently positive for synaptophysin, chromogranin, CD56, CD99, and neuron-specific enolase. Follow-up data (average 4 years) were available for 6 patients. Two patients with distant metastasis were alive with disease, and four patients with no metastasis were alive without disease. We evaluated the association of PNRT and loss of heterozygosity (LOH) on chromosome 3p21 and found LOH in 2 of 3 cases. However, the comparative genomic hybridization study (2/2) did not demonstrate significant chromosomal imbalances. We conclude that PNRTs are positive for NE markers and may have LOH on chromosome 3p21. PNRTs should be classified as NE tumors in other sites, and proliferative rate can be an indicator of aggressive behavior/metastasis.

Original languageEnglish (US)
Pages (from-to)873-880
Number of pages8
JournalHuman Pathology
Volume44
Issue number5
DOIs
StatePublished - May 1 2013

Fingerprint

Neuroendocrine Tumors
Neoplasm Metastasis
Kidney
Loss of Heterozygosity
Carcinoid Tumor
Neoplasms
Chromosomes
Chromogranins
Synaptophysin
Comparative Genomic Hybridization
Phosphopyruvate Hydratase
Kidney Neoplasms
Liver
Lymph Nodes
Lung

Keywords

  • Comparative genomic hybridization analysis
  • Immunohistochemical stain
  • Loss of heterozygosity analysis
  • Primary neuroendocrine tumors of the kidney

ASJC Scopus subject areas

  • Pathology and Forensic Medicine

Cite this

Primary neuroendocrine tumors of the kidney : Morphological and molecular alterations of an uncommon malignancy. / Aung, Phyu P; Killian, Keith; Poropatich, Carrie O.; Linehan, W. Marston; Merino, Maria J.

In: Human Pathology, Vol. 44, No. 5, 01.05.2013, p. 873-880.

Research output: Contribution to journalArticle

Aung, Phyu P ; Killian, Keith ; Poropatich, Carrie O. ; Linehan, W. Marston ; Merino, Maria J. / Primary neuroendocrine tumors of the kidney : Morphological and molecular alterations of an uncommon malignancy. In: Human Pathology. 2013 ; Vol. 44, No. 5. pp. 873-880.
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