TY - JOUR
T1 - Rhabdomyosarcoma of the Adult Prostate
T2 - Two Cases With Molecular and Cytogenetic Analyses
AU - Samarska, Iryna
AU - van den Hout, Mari C. M.
AU - Li, Xiaofei
AU - van Roermund, Joep G. H.
AU - Marcelissen, Tom A. T.
AU - Vanden Bempt, Isabelle
AU - Sciot, Raf
AU - zur Hausen, Axel
N1 - Publisher Copyright:
© The Author(s) 2019.
PY - 2020/2/1
Y1 - 2020/2/1
N2 - Primary rhabdomyosarcoma (RMS) of the adult prostate is a very rare tumor with only 45 cases published to date. The clinical course of RMS of the prostate is very aggressive, and prognosis is very poor. Here we describe two cases of primary RMS of the prostate of adult patients and discuss the differential diagnosis of RMS with other mesenchymal tumors of prostate. The first patient was a 50-year-old man who clinically presented with urinary retention and hematuria, low serum prostate-specific antigen, moderate prostatomegaly, and multiple metastases as shown by computed tomography. Histological examination revealed a diffuse "small round blue cell" proliferation with an intermingled population of larger cells with rhabdomyoblastic differentiation that showed immunoreactivity for desmin and MYF4 (myogenin). Molecular and cytogenetic studies did not reveal recurrent chromosomal translocations associated with RMSs and other sarcomas. The patient underwent doxorubicin (adriamycin) treatment without disease progression during the whole follow-up period of 7 months. The second patient was a 39-year-old man who presented with urinary retention, an increased serum prostate-specific antigen level, extensive prostatomegaly, enlarged abdominal and inguinal lymph nodes, and skeletal metastases. Histological examination revealed a tumoral proliferation with a small round blue aspect and diffuse immunoreactivity for desmin and MYF4 (myogenin). Fluorescence in situ hybridization revealed a FOXO1 translocation, consistent with a diagnosis of alveolar-type RMS. The patient followed a multimodal treatment and died of the disease after its progression.
AB - Primary rhabdomyosarcoma (RMS) of the adult prostate is a very rare tumor with only 45 cases published to date. The clinical course of RMS of the prostate is very aggressive, and prognosis is very poor. Here we describe two cases of primary RMS of the prostate of adult patients and discuss the differential diagnosis of RMS with other mesenchymal tumors of prostate. The first patient was a 50-year-old man who clinically presented with urinary retention and hematuria, low serum prostate-specific antigen, moderate prostatomegaly, and multiple metastases as shown by computed tomography. Histological examination revealed a diffuse "small round blue cell" proliferation with an intermingled population of larger cells with rhabdomyoblastic differentiation that showed immunoreactivity for desmin and MYF4 (myogenin). Molecular and cytogenetic studies did not reveal recurrent chromosomal translocations associated with RMSs and other sarcomas. The patient underwent doxorubicin (adriamycin) treatment without disease progression during the whole follow-up period of 7 months. The second patient was a 39-year-old man who presented with urinary retention, an increased serum prostate-specific antigen level, extensive prostatomegaly, enlarged abdominal and inguinal lymph nodes, and skeletal metastases. Histological examination revealed a tumoral proliferation with a small round blue aspect and diffuse immunoreactivity for desmin and MYF4 (myogenin). Fluorescence in situ hybridization revealed a FOXO1 translocation, consistent with a diagnosis of alveolar-type RMS. The patient followed a multimodal treatment and died of the disease after its progression.
KW - adults
KW - next-generation sequencing
KW - prostate
KW - rhabdomyosarcoma
KW - SOLITARY FIBROUS TUMOR
KW - SPECIALIZED STROMAL TUMORS
KW - EMBRYONAL RHABDOMYOSARCOMA
KW - EPITHELIOID ANGIOSARCOMA
KW - NEUROECTODERMAL TUMOR
KW - SARCOMA
KW - BLADDER
KW - EXPRESSION
U2 - 10.1097/PCR.0000000000000380
DO - 10.1097/PCR.0000000000000380
M3 - (Systematic) Review article
SN - 1082-9784
VL - 25
SP - 133
EP - 137
JO - Pathology Case Reviews
JF - Pathology Case Reviews
IS - 3
ER -