Myogenin is a Specific Marker for Rhabdomyosarcoma: An Immunohistochemical Study in Paraffin-Embedded Tissues

Kumar, Shimareet; Perlman, Elizabeth J.; Harris, Cynthia; Raffeld, Mark; Tsokos, Maria

doi:10.1038/modpathol.3880179

Cited by 200 publications

(147 citation statements)

References 9 publications

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“…However, epithelioid sarcomas are easily distinguished from leiomyosarcomas by their lacking a representative area of a leiomyosarcoma, showing fascicles of elongated tumor cells with blunt-ended "cigar-shaped" atypical nuclei, and greater frequency of negativity for desmin and SMA. Evidence of rhabdomyoblastic differentiation appreciated by positivity for HHF35 and myogenin (15) was absent in epithelioid sarcomas.…”

Section: Discussionmentioning

confidence: 94%

Proximal-Type Epithelioid Sarcoma: A Clinicopathologic Study of 20 Cases

Hasegawa¹,

Matsuno²,

Shimoda³

et al. 2001

Modern Pathology

213

247

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We studied the clinicopathologic and immunohistochemical features of 20 cases of proximal-type epithelioid sarcoma to identify prognostic factors. The 20 patients ranged in age from 13 to 80 years (mean, 40 y); 12 patients were male and 8 were female. The tumors presented as deep soft-tissue or subcutaneous masses on the inguinal region in five, the thigh in four, the vulva in three, the axilla in three, and one each in the flank, chest wall, back, hip and perineum. The tumors ranged from 2 to 16 cm at their greatest diameter (mean: 7.8 cm). Histologically, 12 tumors (60%) were classified as the large-cell subtype, characterized by sheets of large cells with prominent nucleoli resembling poorly differentiated carcinoma, and a frequent rhabdoid phenotype, six (30%) were classified as the conventional subtype, and two ( Epithelioid sarcoma, first described by Enzinger in 1970 (1), is a rare soft-tissue sarcoma typically presenting as a subcutaneous or deep dermal mass in distal portions of the extremities of adolescents and young adults. Microscopically, most tumors are characterized by a granuloma-like pattern: nodules of spindled and epithelioid cells circumscribe areas of central hyalinization and necrosis. Fibrous histiocytoma-like and angiomatoid subtypes have also been reported as less common histologic variants (2, 3). Patients often develop multiple local recurrences of long duration, with subsequent metastases in 30 to 50% of cases (4). A more aggressive "proximal" or largecell type has been described in rare cases to occur as a deep-seated soft-tissue mass at proximal body sites and to show sheets of large cells with prominent nucleoli resembling a poorly differentiated carcinoma and a frequent rhabdoid phenotype (5). Thus, this tumor must be distinguished from other epithelioid lesions, such as extrarenal rhabdoid tumor, synovial sarcoma, angiosarcoma and melanoma, and its prognostic factors have not yet been fully investigated. We therefore studied 20 cases of proximal or axial epithelioid sarcoma by immunohistochemical analysis to examine the clinicopathologic features that correlate with a poor outcome in patients with these tumors.

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Section: Discussionmentioning

confidence: 94%

Proximal-Type Epithelioid Sarcoma: A Clinicopathologic Study of 20 Cases

Hasegawa¹,

Matsuno²,

Shimoda³

et al. 2001

Modern Pathology

213

247

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“…Whereas, myf4 was specific for rhabdomyosarcomas with no other staining, except for one case of Wilm's tumor, of the above mentioned tumors. Further information on myf4, from a recent publication, reveals that it is highly sensitive and specific for rhabdomyosarcoma compared with other pediatric tumors (40).…”

Section: Figurementioning

confidence: 99%

Pleomorphic Rhabdomyosarcoma in Adults: A Clinicopathologic Study of 38 Cases with Emphasis on Morphologic Variants and Recent Skeletal Muscle-Specific Markers

2001

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Pleomorphic rhabdomyosarcoma (PRMS) is a rare and controversial tumor of skeletal muscle phenotype. Diagnostic criteria for PRMS by combined histology and currently available immunohistochemistry have not been clearly defined. We report 38 pleomorphic rhabdomyosarcomas in adults, explore morphologic variants, and discuss our experience with both specific and nonspecific skeletal muscle markers in these tumors. Clinical data, morphology, and immunohistochemistry were reviewed. Electron microscopy was performed. Of 38 cases, there were 28 males and 10 females. Patient ages ranged from 21 to 81 years (median ‫؍‬ 54 y; mean ‫؍‬ 51 y). Tumors were located in the lower extremity (n ‫؍‬ 18), abdomen/retroperitoneum (n ‫؍‬ 6), chest/abdominal wall (n ‫؍‬ 5), spermatic cord/ testes (n ‫؍‬ 4), upper extremity (n ‫؍‬ 3), and one each in the mouth and orbit. Tumor sizes ranged from 1.5 to 15.0 cm (mean ‫؍‬ 7.3 cm; median ‫؍‬ 6.8 cm). The cases were divided into three variants, each with large, atypical, pleomorphic polygonal rhabdomyoblasts (PRMB) with abundant eosinophilic cytoplasm in varying numbers and different morphologic backgrounds of round or spindled rhabdomyoblasts (RMB). , fast skeletal muscle myosin, or myf4). In addition, all cases had some positivity for nonspecific muscle markers (desmin, MSA, SMA, myf3). Electron microscopy (EM), performed on eight selected cases from all three morphologic groups, demonstrated definitive skeletal muscle differentiation in all cases. Follow-up, available on 30 (79%) cases, revealed that 70% of patients died of disease (mean 20 months, range 1 month-108 months), 3% were alive with disease at 12 months (n ‫؍‬ 1); and 27% had no evidence of disease (mean, 83 mo; range, 18 to 108 mo). PRMS, a tumor of predominantly middle-aged adult males in the lower extremity, can be diagnosed by the morphologic presence of scattered PRMB with immunohistochemical evidence of at least one skeletal muscle-specific marker. There are three morphologic variants of PRMS. The appropriate diagnosis of PRMS is significant as it is a high-grade sarcoma, with an aggressive clinical course.

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“…2,4 Intranuclear transcription factors called myogenin and MyoD1 are also sensitive and specific markers for striated muscle differentiation and, as expressed in the present case, it is now well established that the positivity of one of these markers is necessary for diagnosis of rhabdomyosarcoma. [5][6][7] As for the prognosis, 2 25% of adult-type rhabdomyosarcoma patients died of cancer or therapeutic complications. The overall 5-year survival and metastasis-free survival rates were 53 and 63%, respectively.…”

Section: Usmentioning

confidence: 99%