Spindle cell embryonal rhabdomyosarcoma of the prostate in an adult patient – case report and review of clinicopathological features

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Abstract

Background

Embryonal rhabdomyosarcoma of the prostate in an adult is a very rare event with only a few cases published. Diagnosis usually occurs with advanced disease frequently already with metastatic spread. In adults prognosis is very poor, therefore early diagnosis is crucial. To date, only three cases of spindle cell subtype of embryonal rhabdomyosarcoma of the prostate in an adult have been published.

Case presentation

We report an additional case of prostatic spindle cell embryonal rhabdomyosarcoma subtype in an adult.

Conclusions

We discuss relevant clinicopathological features of spindle cell embryonal rhabdomyosarcoma of the prostate in adult patients in the context of the literature.

Related collections

Most cited references 23

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Chromosomes in Ewing's sarcoma. I. An evaluation of 85 cases of remarkable consistency of t(11;22)(q24;q12).

Claude Turc-Carel, Alain Aurias, Francine Mugneret … (1988)

Since our initial reports on chromosomal studies in eight Ewing's sarcomas (ES), we have carried out similar investigations on 23 additional ES specimens following short-term culture of tumor cells (16 cases), and established in vitro cell lines (three cases) and on xenografted tumors in nude mice (four cases). We demonstrated the presence of the reciprocal t(11;22)(q24;q12) in every case except one that exhibited a complex t(11;22;14)(q24;q12;q11). On the basis of results from these additional 23 cases, we confirm the consistency of the t(11;22)(q24;q12) in ES. Moreover, we reviewed 54 ES cases reported by other investigators; when added to our 31 cases, this brings the total number to 85 unrelated cases of ES available for an evaluation of the frequency of involvement of bands 11q24 and 22q12 in translocations in ES. The standard t(11;22)(q24;q12) proved to be a remarkably consistent event, present in 83% of the cases. Five percent of the cases exhibited complex translocations involving a third chromosome in addition to chromosomes #11 and #22. In 4% of the cases variant translocations involved 22q12 but with a chromosome(s) other than #11. The breakpoint on chromosome 22q12 appears to be the most consistently observed event in 92% of the cases, whereas, the breakpoint at chromosome 11q24 was observed in 88% of the cases.

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Classification of rhabdomyosarcomas and related sarcomas. Pathologic aspects and proposal for a new classification--an Intergroup Rhabdomyosarcoma Study.

W Newton, E Gehan, B Webber … (1995)

There is a need to develop a single prognostically significant classification of rhabdomyosarcomas (RMS) and other related tumors of children, adolescents, and young adults which would be a current guide for their diagnosis, allow valid comparison of outcomes between protocols carried out anywhere in the world, and should enhance recognition of prognostic subsets. Sixteen pathologists from eight pathology groups, representing six countries and several cooperative groups, classified by four histopathologic classification schemes 800 representative tumors of the 999 eligible cases treated on Intergroup Rhabdomyosarcoma Study II. Each tumor was classified according to each of the four systems by each of the pathologists. In addition, two independent subsamples of 200 of the 800 patients were reviewed according to the new system, so that 343 distinct patients were reviewed once, and 57 of these twice. A study of the survival rates of all subtypes in the sample of 800 patients led to the formation of a new system. This was tested on two independent subsets of 200 of the original cases and found to be reproducible and predictive of outcome by univariate analysis. A multivariate analysis of the 343 patients classified according to the new system indicated that a survival model including pathologic classification and known prognostic factors of primary site, clinical group, and tumor size was significantly better at predicting survival than a model with only the known prognostic factors. This new classification, termed International Classification of Rhabdomyosarcoma (ICR) by the authors, was reproducible and predictive of outcome among patients with differing histologies treated uniformly on the Intergroup Rhabdomyosarcoma II protocols. We believe it should be utilized by all pathologists and cooperative groups to classify rhabdomyosarcomas in order to provide comparability among and within multi-institutional studies.

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Spindle cell rhabdomyosarcoma. A prognostically favorable variant of rhabdomyosarcoma.

V Ninfo, P. Harms, Jörn Treuner … (1992)

Twenty-one cases of embryonal rhabdomyosarcoma, composed mainly of elongated spindle cells arranged in a fasciculated or storiform pattern, were retrieved from the files of the German-Italian Cooperative Soft Tissue Sarcoma Study. The term spindle cell rhabdomyosarcoma is proposed to designate this histotype. Spindle cell rhabdomyosarcoma predilected male patients (18 males, three females) and involved mostly the paratesticular area (12 cases) and the head and neck region (six cases). Histologically, all cases were characterized by a uniform proliferation of elongated spindle cells with eosinophilic and fibrillar cytoplasm mimicking smooth muscle fibers; immunocytochemical studies disclosed high expression of the muscle markers titin, desmin, and myoglobin. Clinical information was available in 17 cases; according to the Intergroup Rhabdomyosarcoma Study (IRS) grouping system, 13 were classified in group I, two in group II, and two in group III. Sixteen patients were well and alive 24 to 100 months after diagnosis; one patient died from disease progression 24 months after diagnosis. Analysis of our results determined that spindle cell rhabdomyosarcoma constitutes a rare variant of the embryonal form, showing a high degree of skeletal muscle differentiation and a low malignant potential; it should therefore be distinguished from classical forms of embryonal rhabdomyosarcoma.

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Author and article information

Contributors

Hans-Ulrich Schildhaus:

ORCID: http://orcid.org/0000-0002-9856-7050

hans-ulrich.schildhaus@med.uni-goettingen.de

Suvi Lokka: lokka@patho-nordhessen.de

Werner Fenner: w.fenner@mvz-hannmuenden.de

Jens Küster: j.kuester@mvz-hannmuenden.de

Ingrid Kühnle: ikuehnle@med.uni-goettingen.de

Ernst Heinmöller: heinmoeller@patho-nordhessen.de

Journal

Journal ID (nlm-ta): Diagn Pathol

Journal ID (iso-abbrev): Diagn Pathol

Title: Diagnostic Pathology

Publisher: BioMed Central (London )

ISSN (Electronic): 1746-1596

Publication date (Electronic): 29 June 2016

Publication date PMC-release: 29 June 2016

Publication date Collection: 2016

Volume: 11

Electronic Location Identifier: 56

Affiliations

[ ]Department of Pathology, University Medical Center Göttingen, Göttingen, Germany

[ ]Institute of Pathology Nordhessen, Germaniastr. 7, 34119 Kassel, Germany

[ ]Deparment of Urology, Nephrologisches Zentrum, Vogelsang 105, 34346 Hann. Münden, Germany

[ ]Department of Pediatric Hematology and Oncology, University Medical Center Göttingen, Göttingen, Germany

Author information

Hans-Ulrich Schildhaus http://orcid.org/0000-0002-9856-7050

Article

Publisher ID: 507

DOI: 10.1186/s13000-016-0507-1

PMC ID: 4928285

PubMed ID: 27357857

SO-VID: 2708e114-6519-4705-98d2-1d4a0a5c8ec4

License:

Open AccessThis article is distributed under the terms of the Creative Commons Attribution 4.0 International License ( http://creativecommons.org/licenses/by/4.0/), which permits unrestricted use, distribution, and reproduction in any medium, provided you give appropriate credit to the original author(s) and the source, provide a link to the Creative Commons license, and indicate if changes were made. The Creative Commons Public Domain Dedication waiver ( http://creativecommons.org/publicdomain/zero/1.0/) applies to the data made available in this article, unless otherwise stated.

History

Date received : 10 December 2015

Date accepted : 25 June 2016

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ScienceOpen disciplines: Pathology

Keywords: embryonal rhabdomyosarcoma,prostate,adults

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ScienceOpen disciplines: Pathology

Keywords: embryonal rhabdomyosarcoma, prostate, adults

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Spindle cell embryonal rhabdomyosarcoma of the prostate in an adult patient – case report and review of clinicopathological features

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Most cited references 23

Chromosomes in Ewing's sarcoma. I. An evaluation of 85 cases of remarkable consistency of t(11;22)(q24;q12).

Classification of rhabdomyosarcomas and related sarcomas. Pathologic aspects and proposal for a new classification--an Intergroup Rhabdomyosarcoma Study.

Spindle cell rhabdomyosarcoma. A prognostically favorable variant of rhabdomyosarcoma.

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