Review of hereditary leiomyomatosis renal cell carcinoma with focus on clinical and pathobiological aspects of renal tumors

Naoto Kuroda, Chisato Ohe, Ikuma Kato, Mitsuko Furuya, Masaya Baba, Yoji Nagashima, Yukio Nakatani, Ichiro Murakami, Ming Zhou, Michal Michal, Ondrej He, Mahul Amin

Research output: Contribution to journalReview article

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Abstract

The entity of hereditary leiomyomatosis renal cell carcinoma (HLRCC)-associated RCC has been proposed and integrated into the recent International Society of Urologic Pathology (ISUP) of renal tumors. This tumor is characterized by presence of cutaneous and/or uterine leiomyomas and RCC and autosomal dominant hereditary form. Grossly, HLRCC arising in the kidney show the solid tumor with frequent partial cystic area. Microscopically, the tumor typically shows papillary RCC, type 2, with eosinophilic large nucleoli reminiscent of cytomegaloviral inclusion and perinuclear clearing/haloes. Immunohistochemically, tumor cells show the overexpression for 2SC and reduced expression of FH. Germline mutation of fumarate hydratase (FH) gene, the HLRCC responsible gene mapped to chromosome 1q43, has been identified in patients with HLRCC. As the renal cancer in patients with HLRCC generally behave aggressively even in a small size, complete surgical resection and retroperitoneal lymph node resection should be performed promptly when the tumor is discovered. The surveillance of renal tumor in FH gene germline mutation-positive patients should be started from the early age using ultrasound sonography or magnetic resonance imaging.

Original languageEnglish (US)
Pages (from-to)284-290
Number of pages7
JournalPolish Journal of Pathology
Volume68
Issue number4
DOIs
StatePublished - Jan 1 2017

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Leiomyomatosis
Renal Cell Carcinoma
Kidney
Fumarate Hydratase
Neoplasms
Germ-Line Mutation
Genes
Kidney Neoplasms
Leiomyoma
Ultrasonography
Chromosomes
Lymph Nodes
Magnetic Resonance Imaging
Pathology
Skin

All Science Journal Classification (ASJC) codes

  • Pathology and Forensic Medicine

Cite this

Review of hereditary leiomyomatosis renal cell carcinoma with focus on clinical and pathobiological aspects of renal tumors. / Kuroda, Naoto; Ohe, Chisato; Kato, Ikuma; Furuya, Mitsuko; Baba, Masaya; Nagashima, Yoji; Nakatani, Yukio; Murakami, Ichiro; Zhou, Ming; Michal, Michal; He, Ondrej; Amin, Mahul.

In: Polish Journal of Pathology, Vol. 68, No. 4, 01.01.2017, p. 284-290.

Research output: Contribution to journalReview article

Kuroda, N, Ohe, C, Kato, I, Furuya, M, Baba, M, Nagashima, Y, Nakatani, Y, Murakami, I, Zhou, M, Michal, M, He, O & Amin, M 2017, 'Review of hereditary leiomyomatosis renal cell carcinoma with focus on clinical and pathobiological aspects of renal tumors', Polish Journal of Pathology, vol. 68, no. 4, pp. 284-290. https://doi.org/10.5114/pjp.2017.73920
Kuroda, Naoto ; Ohe, Chisato ; Kato, Ikuma ; Furuya, Mitsuko ; Baba, Masaya ; Nagashima, Yoji ; Nakatani, Yukio ; Murakami, Ichiro ; Zhou, Ming ; Michal, Michal ; He, Ondrej ; Amin, Mahul. / Review of hereditary leiomyomatosis renal cell carcinoma with focus on clinical and pathobiological aspects of renal tumors. In: Polish Journal of Pathology. 2017 ; Vol. 68, No. 4. pp. 284-290.
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