Clinicopathologic analysis of spindle cell/sclerosing rhabdomyosarcoma

2014 ◽  
Vol 62 (6) ◽  
pp. 1011-1016 ◽  
Author(s):  
Naoko Yasui ◽  
Akihiko Yoshida ◽  
Hiroshi Kawamoto ◽  
Kan Yonemori ◽  
Ako Hosono ◽  
...  
Keyword(s):  
Spindle Cell ◽  
Sclerosing Rhabdomyosarcoma

Establishment and characterization of NCC-ssRMS2-C1: a novel patient-derived cell line of spindle cell/sclerosing rhabdomyosarcoma

Human Cell ◽  
2021 ◽  
Author(s):  
Ryuto Tsuchiya ◽  
Yuki Yoshimatsu ◽  
Rei Noguchi ◽  
Yooksil Sin ◽  
Takuya Ono ◽  
...  
Keyword(s):  
Cell Line ◽  
Spindle Cell ◽  
Sclerosing Rhabdomyosarcoma

scholarly journals Spindle cell/sclerosing rhabdomyosarcoma with intracranial invasion without destroying the bone of the skull base: a case report and literature review

2017 ◽  
Vol 6 (8) ◽  
pp. 205846011772731
Author(s):  
Daichi Momosaka ◽  
Osamu Togao ◽  
Akio Hiwatashi ◽  
Koji Yamashita ◽  
Koji Yoshimoto ◽  
...  
Keyword(s):  
Spindle Cell ◽  
Bone Destruction ◽  
Japanese Woman ◽  
World Health ◽  
Imaging Characteristics ◽  
The World ◽  
Sclerosing Rhabdomyosarcoma ◽  
Elderly Japanese ◽  
Health Organization ◽  
Worse Prognosis

Spindle cell/sclerosing rhabdomyosarcoma (ssRMS) is a new subtype of rhabdomyosarcoma included in the World Health Organization soft tissue and bone tumor classification in 2013. Despite the increasing number of reported cases of ssRMS, the imaging characteristics of ssRMS are not established. Herein, we present the case of an elderly Japanese woman with ssRMS of the masticator space with intracranial invasion without destruction of the adjacent bone. Attention should be paid to the presence of intracranial infiltration that may indicate a worse prognosis. Tumor growth without bone destruction could be a key finding to differentiate ssRMSs from conventional subtypes of rhabdomyosarcoma.

scholarly journals Clinical and molecular heterogeneity of head and neck spindle cell and sclerosing rhabdomyosarcoma

Oral Oncology ◽  
2016 ◽  
Vol 58 ◽  
pp. e6-e11 ◽  
Author(s):  
Adepitan A. Owosho ◽  
Sonja Chen ◽  
Shruti Kashikar ◽  
Lei Zhang ◽  
Chun-Liang Chen ◽  
...  
Keyword(s):  
Head And Neck ◽  
Spindle Cell ◽  
Molecular Heterogeneity ◽  
Sclerosing Rhabdomyosarcoma

Challenges in the Diagnosis of Pediatric Spindle Cell/Sclerosing Rhabdomyosarcoma

2020 ◽  
Vol 13 (4) ◽  
pp. 729-738
Author(s):  
Sonja Chen ◽  
Erin R. Rudzinski ◽  
Michael A. Arnold
Keyword(s):  
Spindle Cell ◽  
Sclerosing Rhabdomyosarcoma

Combined Sclerosing and Spindle Cell Rhabdomyosarcoma in Previous Craniotomy Site: A Case Report and a Review of the Literature

2018 ◽  
Vol 27 (3) ◽  
pp. 328-335
Author(s):  
Hongxing Gui ◽  
Elliott Lhospital ◽  
Arthur P. Staddon ◽  
Suneel N. Nagda ◽  
Eric L. Zager ◽  
...  
Keyword(s):  
Case Report ◽  
High Frequency ◽  
Spindle Cell ◽  
Clinical Course ◽  
Review Of The Literature ◽  
Poor Clinical Outcome ◽  
Cell Components ◽  
Sclerosing Rhabdomyosarcoma ◽  
Aggressive Clinical Course ◽  
Worse Prognosis

Sclerosing rhabdomyosarcoma (RMS) is a rare subtype of RMS with unique prominent stromal hyalinization and a pseudovascular architecture. It overlaps morphologically with spindle cell RMS and poses both diagnostic and therapeutic challenges because of its rarity and aggressive clinical course. In this article, we report a case of sclerosing RMS arising from a prior craniotomy site, which demonstrated both sclerosing and spindle cell components. A literature review of RMS with sclerosing morphology identified 122 cases. Our review documents the following: sclerosing RMS occurs in both childhood and adult populations, has a predilection for the head and neck areas, and has a worse prognosis in adults. Sclerosing RMS harbors a high frequency of MYOD1 mutations, conferring a poor clinical outcome. Sclerosing RMS and spindle RMS likely represent a morphologic spectrum of one entity.

scholarly journals MYOD1 (L122R) mutations are associated with spindle cell and sclerosing rhabdomyosarcomas with aggressive clinical outcomes

2016 ◽  
Vol 29 (12) ◽  
pp. 1532-1540 ◽  
Author(s):  
Bharat Rekhi ◽  
Pawan Upadhyay ◽  
Manoj P Ramteke ◽  
Amit Dutt
Keyword(s):  
Clinical Outcomes ◽  
Spindle Cell ◽  
Neck Region ◽  
Immunohistochemical Analysis ◽  
Embryonal Rhabdomyosarcoma ◽  
Head And Neck Region ◽  
Recurrent Mutations ◽  
Alive With Disease ◽  
Sclerosing Rhabdomyosarcoma ◽  
Aggressive Clinical Course

Abstract Recurrent mutations in the myogenic transcription factor MYOD1 and PIK3CA were initially described in a subset of embryonal rhabdomyosarcomas. Recently, two independent studies demonstrated presence of MYODI (L122R) mutations as the basis to re-classify a spindle cell rhabdomyosarcoma, along with a sclerosing rhabdomyosarcoma, distinct from an embryonal rhabdomyosarcoma. We analyzed a much larger cohort of 49 primary rhabdomyosarcoma tumor samples of various subtypes, collected over a period of 9 years, for the presence of MYOD1 (L122R), PIK3CA (H1047), and PIK3CA (E542/E545) mutations, along with immunohistochemical analysis of desmin, myogenin, and MYOD1. Although activating PIK3CA mutations were absent across the sample set analyzed, we report 20% MYOD1 (L122R) mutation in rhabdomyosarcomas, found exclusively in 10 of 21 spindle cell and sclerosing rhabdomyosarcomas, occurring mostly in the head and neck region along with extremity sites (64%), than the paratesticular and intra-abdominal sites. Furthermore, while all 10 MYOD1 mutant spindle cell and sclerosing rhabdomyosarcoma samples showed diffuse and strong MYOD1 immunoexpression, 7 of 31 samples of rhabdomyosarcoma with wild-type MYOD1 were negative for MYOD1 expression. Clinically, a striking correlation was found between MYOD1 mutation and the clinical outcomes available for 15 of 21 cases: 5 of 7 patients with spindle cell and sclerosing rhabdomyosarcomas, harboring MYOD1 mutation, were alive-with-disease and 2 of 8 patients with spindle cell and sclerosing rhabdomyosarcomas, with mutant MYOD1, were free-of-disease. Taken together, we present the first report of MYOD1 (L122R) mutation in the largest cohort of 49 rhabdomyosarcomas reported so far, that are associated with a relatively aggressive clinical course. Moreover, consistent with the earlier two studies, this study further reinforces a relationship between spindle cell and the sclerosing rhabdomyosarcoma—now recognized as a single subtype, distinct from an embryonal rhabdomyosarcoma.

scholarly journals Adult spindle cell/ sclerosing rhabdomyosarcoma of the buccal maxillary gingiva: Unique entity, a rare case report

2021 ◽  
Vol 6 (3) ◽  
pp. 217-221
Author(s):  
Shikha Chopra ◽  
Richa Jindal ◽  
Molly Joseph ◽  
Bhumika Gupta ◽  
Lipakshi Lakhiani ◽  
...  
Keyword(s):  
Rare Case ◽  
Spindle Cell ◽  
Striated Muscle ◽  
Clinical Course ◽  
Malignant Neoplasm ◽  
Small Mass ◽  
Muscle Cell Differentiation ◽  
Genetic Abnormalities ◽  
Rare Case Report ◽  
Sclerosing Rhabdomyosarcoma

Rhabdomyosarcoma is a malignant neoplasm of mesenchymal cells, which shows varying degrees of striated muscle cell differentiation. It predominantly occurs in children while rarely found in adults. Involvement of the oral cavity accounts for only 10-12% of all head and neck cases. Herewith, we report a rare case of oral spindle cell / sclerosing rhabdomyosarcoma in a 47-year-old male presented with a small mass involving the gingiva of right upper incisor. The mass was excised with a preoperative diagnosis of gingival epulis and subjected to histopathological and immunohistochemical examination which confirmed it to be spindle cell / sclerosing rhabdomyosarcoma. Data regarding its clinical course, genetic abnormalities and prognosis as a combined subtype is scant.

Establishment and characterization of NCC-ssRMS1-C1: a novel patient-derived spindle-cell/sclerosing rhabdomyosarcoma cell line

Human Cell ◽  
2020 ◽  
Vol 33 (3) ◽  
pp. 886-893 ◽  
Author(s):  
Yuki Yoshimatsu ◽  
Rei Noguchi ◽  
Ryuto Tsuchiya ◽  
Akane Sei ◽  
Jun Sugaya ◽  
...  
Keyword(s):  
Cell Line ◽  
Spindle Cell ◽  
Rhabdomyosarcoma Cell Line ◽  
Rhabdomyosarcoma Cell ◽  
Sclerosing Rhabdomyosarcoma
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