Publication: Renal hybrid oncocytic/chromophobe tumors. A review
Authors
Hes, Ondrej ; Petersson, Fredrik ; Kuroda, Naoto ; Hora, Milan ; Michal, Michal
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Publisher
F. Hernández y Juan F. Madrid. Universidad de Murcia. Departamento de Biología Celular e Histología
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DOI
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info:eu-repo/semantics/article
Description
Abstract
Hybrid oncocytic/chromophobe tumors
(HOCT) occur in three clinico-pathologic situations; (1)
sporadically, (2) in association with renal
oncocytomatosis and (3) in patients with Birt-HoggDubé
syndrome (BHD). There are no specific clinical
symptoms in patients with sporadic or HOCT associated
with oncocytosis/oncocytomatosis. HOCT in patients
with BHD are usually encountered on characteristic
BHD clinicopathologic background. Sporadic HOCT are
composed of neoplastic cells with eosinophilic oncocytic
cytoplasm. Tumors are usually arranged in a solidalveolar
pattern. Some neoplastic cells may have a
perinuclear halo, with no raisinoid nuclei present. HOCT
occurring in patients with oncocytomatosis are
morphologically identical to sporadic HOCT. HOCT in
BHD frequently display 3 morphologic patterns, either
in isolation or in combination; (1) An admixture of areas
typical of RO and CHRCC, respectively, (2) Scattered
chromophobe cells in the background of a typical RO,
(3) Large eosinophilic cells with intracytoplasmic
vacuoles. The immunohistochemical profiles of HOCT
in all clinicopathologic and morphologic groups differ
slightly. The majority of tumors express parvalbumin,
antimitochondrial antigen and CK 7. CD117 is
invariably positive. HOCT show significant molecular
genetic heterogeneity. The highest degree of variability
in numerical chromosomal changes is present in
sporadic HOCT. HOCT in the setting of oncocytomatosis
have revealed a lesser degree of variability in
the chromosomal numerical aberrations. HOCT in
patients with BHD display FLCN gene mutations, which
are absent in the other groups. HOCT (all three
clinicopathologic groups) seem to behave indolently, as
no evidence of aggressive behavior has been
documented. However, no report with follow up longer
than 10 years has been published.
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Citation
Histology and Histopathology, vol. 28, nº 10 (2013)
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