Sclerosing Rhabdomyosarcoma in Adults: Report of Four Cases of a Hyalinizing, Matrix-Rich Variant of Rhabdomyosarcoma That May Be Confused With Osteosarcoma, Chondrosarcoma, or Angiosarcoma

  title={Sclerosing Rhabdomyosarcoma in Adults: Report of Four Cases of a Hyalinizing, Matrix-Rich Variant of Rhabdomyosarcoma That May Be Confused With Osteosarcoma, Chondrosarcoma, or Angiosarcoma},
  author={Andrew L. Folpe and Jesse K. McKenney and Julia A Bridge and Sharon Whelan Weiss},
  journal={The American Journal of Surgical Pathology},
Rhabdomyosarcomas (RMSs) are classified into embryonal (ERMS), alveolar (ARMS), and pleomorphic (PRMS) subtypes. ERMS, including botryoid variants, typically occurs in young children, ARMS typically occurs in older children and young adults, and PRMS occurs in older adults. Although ARMSs show thin fibrous bands separating nests of cells, abundant extracellular matrix production is rare in RMS. In the course of reviewing hyalinizing sarcomas we discovered a distinctive RMS in adults that… 

Sclerosing Rhabdomyosarcoma: Report of a Case Arising in the Head and Neck of an Adult and Review of the Literature

Controversy is discussed regarding how sclerosing rhabdomyosarcoma might best fit into existing rhabDomyosARcoma classification schemes, based upon current clinicopathologic and molecular genetic evidence.

[Spindle cell rhabdomyosarcoma in adults: a new entity in the spectrum of malignant mesenchymal tumors of soft tissues].

Spindle cell and sclerosing RMS in adults arise predominantly in the head and neck region and on the extremities, and are composed of atypical spindled cells and scattered rhabdomyoblasts.

Sclerosing Rhabdomyosarcomas in Children and Adolescents: A Clinicopathologic Review of 13 Cases From the Intergroup Rhabdomyosarcoma Study Group and Children’s Oncology Group

  • Melissa C. ChilesD. Parham W. Meyer
  • Medicine
    Pediatric and developmental pathology : the official journal of the Society for Pediatric Pathology and the Paediatric Pathology Society
  • 2005
Sclerosing RMS may arise in children, have mixed ERMS-ARMS histology, originate from the head and neck, and lack strong myogenin staining.

Sclerosing Rhabdomyosarcoma: Presentation of a Rare Sarcoma Mimicking Myoepithelial Carcinoma of the Parotid Gland and Review of the Literature

A case of SRMS mimicking a myoepithelial carcinoma of the parotid gland is reported, highlighting the potential for misdiagnosis as a primary salivary gland epithelial malignancy.

Sclerosing Rhabdomyosarcoma in Childhood: Case Report and Review of the Literature

  • B. VadgamaN. SebireM. MaloneA. Ramsay
  • Medicine
    Pediatric and developmental pathology : the official journal of the Society for Pediatric Pathology and the Paediatric Pathology Society
  • 2004
The histology, immunohistochemistry, and molecular genetics matched those reported in the seven adult cases of sclerosing rhabdomyosarcoma, and this is the first case report, to the authors' knowledge, of this rare tumor arising in the pediatric age group.

Sclerosing rhabdomyosarcoma: a clinicopathologic and immunohistochemical study of five cases.

5 cases of sclerosing rhabdomyosarcoma are described, which are reported to represent a special variant of rhab DomyosARcoma and is possibly related to embryonal rhabDomyosArcoma, the most common malignant soft tissue tumor of childhood and adolescence.

Adult-type Rhabdomyosarcoma: Analysis of 57 Cases With Clinicopathologic Description, Identification of 3 Morphologic Patterns and Prognosis

Adult-type rhabdomyosarcoma is a rare sarcoma occurring mainly in the extremities and trunk wall with 2 main histologic patterns, spindle cell, and pleomorphic patterns, which represent the end of the spectrum of a single entity.

Sclerosing pseudovascular rhabdomyosarcoma—immunohistochemical, ultrastructural, and genetic findings indicating a distinct subtype of rhabdomyosarcoma

These findings represent a relatively circumscribed genetic defect pattern in sclerosing (pseudovascular) rhabdomyosarcoma that is somewhat different from patterns described in most other rhabDomyosARcoma subtypes.

Sclerosing rhabdomyosarcoma of the parotid gland in an adult.

Spindle cell/sclerosing rhabdomyosarcoma: case series from a single institution emphasizing morphology, immunohistochemistry and follow-up.

It is demonstrated there do present some clincopathologic relations between spindle cells rhabdomyosarcoma and sclerosing rhabDomyosARcoma, but the latter seems to have a better prognosis.



Sclerosing, pseudovascular rhabdomyosarcoma in adults. Clinicopathological and immunohistochemical analysis of three cases.

An unusual morphological variant of rhabdomyosarcoma arising in adult patients is described, which should be added to the morphological spectrum of these neoplasms.

Sclerosing Epithelioid Fibrosarcoma: A Variant of Fibrosarcoma Simulating Carcinoma

The data suggest that SEF is a relatively low-grade fibrosarcoma; yet it is fully malignant despite the presence of histologically benign-appearing foci, and the proliferation markers PCNA and K67 did not correlate with prognosis.

Sclerosing Epithelioid Fibrosarcoma: A Study of 16 Cases and Confirmation of a Clinicopathologically Distinct Tumor

Histologically, the SEFs were composed predominantly of small to moderate-size round to ovoid, relatively uniform cells, often with clear cytoplasm, embedded in a hyalinized fibrous stroma, and the only consistent immunohistochemical finding was a strong, diffuse reactions of tumor cells for vimentin.

Intergroup Rhabdomyosarcoma Study: Update for Pathologists

  • S. QualmanC. Coffin W. Crist
  • Medicine
    Pediatric and developmental pathology : the official journal of the Society for Pediatric Pathology and the Paediatric Pathology Society
  • 1998
The objective of this report is to clearly define emerging histopathologic categories of RMS as defined by the ICR, and to emphasize correlative immunohistochemical or molecular studies.

Rhabdomyosarcoma in adults.

The concept of pleomorphic rhabdomyosarcoma as a distinct clinicopathologic entity, presenting as an aggressive, predominantly spindle-cell tumor arising in the skeletal musculature of middle-aged adults and defined by the presence of large, pleomorphic tumor cells, which show, at least focally, immunophenotypic or ultrastructural sarcomeric muscle differentiation, is discussed and supported.

Alveolar rhabdomyosarcoma. An analysis of 110 cases

The microscopic picture varied, but in most cases recognition was readily accomplished on the basis of the characteristic pseudoalveolar pattern and the presence of differentiated rhabdomyoblasts and multinucleated giant cells having peripherally placed nuclei.

The diagnosis and classification of childhood rhabdomyosarcoma.

  • M. Tsokos
  • Medicine
    Seminars in diagnostic pathology
  • 1994
Comparative analysis of several proposed classification schemes has led to the establishment of well-defined criteria for the major subtypes of rhabdomyosarcoma and to the recognition of subtypes with prognostic significance.

Rhabdomyosarcoma in middle‐aged and elderly individuals

The present investigation indicates that a pleomorphic type of rhabdomyosarcoma, indistinguishable from embryonal, botryoid and alveolar rhabdomsarcomas, is extremely rare or non‐existent.

Alveolar and poorly differentiated rhabdomyosarcoma

The results of the investigation indicate that immunohistochemistry is a more useful tool than electron microscopy in the diagnosis of poorly differentiated rhabdomyosarcoma and that the criteria for the diagnosis may need to be reformulated.

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

The results indicate that staining for myogenin is an extremely reliable and specific marker for rhabdomyoblastic differentiation and gives consistent and easily interpretable results in routinely fixed tissues.