Composite uterine neoplasm with embryonal rhabdomyosarcoma and primitive neuroectodermal tumor components: rhabdomyosarcoma with divergent differentiation, variant of primitive neuroectodermal tumor, or unique entity?

Summary Three cases of composite uterine neoplasms comprised of primitive neuroectodermal tumor (PNET) and rhabdomyosarcoma (RMS) have previously been described, including only one wherein the rhabdomyosarcomatous component was of the embryonal subtype. Whether such composite neoplasms are a variant...

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Bibliographic Details
Published in:Human pathology 2013-04, Vol.44 (4), p.656-663
Main Authors: Cate, Frances, MD, Bridge, Julia A., MD, Crispens, Marta A., MD, Keedy, Vicki L., MD, Troutman, Ashley, BS, HT(ASCP) QIHC, Coffin, Cheryl M., MD, Fadare, Oluwole, MD
Format: Article
Language:English
Subjects:
Adult
Age
Aneuploidy
Biomarkers, Tumor - genetics
Biomarkers, Tumor - metabolism
Botryoid embryonal rhabdomyosarcoma
Cell Transformation, Neoplastic
Chemotherapy
Combined Modality Therapy
Disease-Free Survival
DNA, Neoplasm - analysis
Female
Hospitals
Humans
Hysterectomy
In Situ Hybridization, Fluorescence
Medical treatment
Neoplasms, Complex and Mixed - genetics
Neoplasms, Complex and Mixed - metabolism
Neoplasms, Complex and Mixed - pathology
Neuroectodermal Tumors, Primitive - genetics
Neuroectodermal Tumors, Primitive - metabolism
Neuroectodermal Tumors, Primitive - pathology
NMR
Nuclear magnetic resonance
Pathology
Primitive neuroectodermal tumor
Proprietary
Radiation therapy
Rhabdomyosarcoma
Rhabdomyosarcoma, Embryonal - genetics
Rhabdomyosarcoma, Embryonal - metabolism
Rhabdomyosarcoma, Embryonal - pathology
Treatment Refusal
Tumors
Uterine Neoplasms - genetics
Uterine Neoplasms - metabolism
Uterine Neoplasms - pathology
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Summary:Summary Three cases of composite uterine neoplasms comprised of primitive neuroectodermal tumor (PNET) and rhabdomyosarcoma (RMS) have previously been described, including only one wherein the rhabdomyosarcomatous component was of the embryonal subtype. Whether such composite neoplasms are a variant of RMS, a variant of PNET, or a unique entity is unknown. We report the clinicopathologic, immunohistochemical, and molecular cytogenetic findings in a case of uterine embryonal RMS with coexisting PNET that was diagnosed in a 25-year-old female. The tumor broadly involved the cervix and corpus uteri and resulted in uterine inversion. The 2 distinct components each showed classic morphologic features, including cartilage in the RMS component. The unique combination of histologic, immunohistochemical and molecular findings in composite neoplasms of this type raises a question of whether they should be classified and treated as RMS, PNET, or a unique high-grade sarcoma. A variety of clinicopathologic arguments are presented that support the notion that the current neoplasm is an embryonal rhabdomyosarcoma with divergent neuroectodermal and cartilaginous differentiation.
ISSN:0046-8177
1532-8392
DOI:10.1016/j.humpath.2012.09.008