Histopathological classification of childhood rhabdomyosarcoma: A report from the international society of pediatric oncology pathology panel

Caillaud, J M; Gérard-Marchant, R; Marsden, H. B.; Unnik, A. J. M. Van; Rodary, C; Rey, Annie; Flamant, F

doi:10.1002/mpo.2950170508

Cited by 58 publications

(24 citation statements)

References 12 publications

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“…Using methods similar to those of Palmer, in 1989, Caillaud et al (27) published a classification used to test a pediatric sarcoma registry from a large multi-institutional study, that of the International Society of Pediatric Oncology (SIOP). The IRS and SIOP studies diverged, however, in that the SIOP group concentrated on cytologic differentiation and cellular density rather than nuclear features (see Table 1).…”

Section: Siop Classificationmentioning

confidence: 99%

Pathologic Classification of Rhabdomyosarcomas and Correlations with Molecular Studies

2001

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Rhabdomyosarcoma, the most common soft-tissue malignancy of childhood and adolescence, comprises a group of differing pathobiologic entities linked by their common propensity for formation of neoplastic skeletal muscle, a feature that results from biological forces related to aberrant transcription signals and the resultant production of myogenic proteins. At a molecular level, however, rhabdomyosarcomas form a heterogeneous group that can be subdivided into distinct clinicopathologic entities based on morphologic appearance and genetic makeup. These differing morphologic features were recognized in the mid-1900s by Horn and Enterline with their division of rhabdomyosarcomas into embryonal, alveolar, botryoid, and pleomorphic subtypes. More recent histologic and biologic studies have resulted in description of additional entities, such as spindle cell and anaplastic rhabdomyosarcoma, and refinements in recognition of the original entities, such as solid-alveolar rhabdomyosarcoma. Familiarity with newer classifications and their relationship to molecular aberrations is key to stratifying patients on current therapeutic protocols and proposed innovative genetic therapies.

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Section: Siop Classificationmentioning

confidence: 99%

Pathologic Classification of Rhabdomyosarcomas and Correlations with Molecular Studies

2001

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“…Rhabdomyosarcoma (RMS), the most common soft tissue sarcoma in children, originates from mesenchymal tissues at various anatomic locations [1]. Overall survival (OS) rates for children with RMS have improved significantly over the last 30 years [2], primarily due to advances in multimodal therapy.…”

Section: Introductionmentioning

confidence: 99%

Improved care of rhabdomyosarcoma in Jordan using less intensive therapy

Al-Jumaily

Ayaad

Masarweh

et al. 2012

Pediatric Blood & Cancer

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BackgroundThe care of rhabdomyosarcoma (RMS) is complex due to its multimodal nature. By following standard protocols with acceptable toxicity and building local expertise, better outcome should be achievable.ProcedureA retrospective study was conducted of records of patients (n = 45; 31 males; median age 26 months) with RMS treated at King Hussein Cancer Center in Jordan from January 2004 to December 2008. Patient demographics, tumor characteristics, risk stratification, treatment plan, and outcomes were studied. In June 2006, the cyclophosphamide dose was lowered from 2.2 g/m2 to 1.2 g/m2 per cycle because of the significant toxicity with higher dose. Survival rates, hematological toxicities, period of hospitalization due to febrile neutropenia (FN), and response rate at week 12 of treatment were compared between low‐ and high‐dose cyclophosphamide groups.ResultsFour‐year progression‐free survival (PFS) and overall survival (OS) rates were 61% ± 7.5% and 72% ± 6.9%, respectively. There was a significant difference in outcome by risk group in 4‐year PFS (low‐risk, 88% ± 12%; intermediate‐risk 63% ± 9.3%; high‐risk, 14% ± 13%; P = 0.0001) and OS (low‐risk, 88% ± 12%; intermediate‐risk 79% ± 7.5%; high‐risk, 17% ± 15%; P = 0.0011). There was significant reduction in hematological toxicities, incidence of FN, and period of hospitalization for FN in patients given low‐dose cyclophosphamide but no significant difference in PFS between low‐ and high‐dose cyclophosphamide groups.ConclusionsSurvival rates of patients with RMS in some developing countries can be improved by following or modifying evidence‐based approaches successful in developed countries and establishing multidisciplinary strategies. Therapy intensity should be increased in developing countries only when evidence supports its utility. Pediatr Blood Cancer 2013; 60: 53–58. © 2012 Wiley Periodicals, Inc.

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“…1,2 Alveolar rhabdomyosarcomas most often occur in older children, with a median patient age of between 7 and 9 years, but are also relatively common in adolescents and young adults, and may rarely involve even elderly patients. [3][4][5] Although alveolar rhabdomyosarcoma was initially thought to be principally a tumor of the extremities, subsequent series have shown an equal occurrence in head and neck locations, including the orbit, sinuses and ear. 3,[6][7][8] In its classic form, alveolar rhabdomyosarcoma is comprised of distinctive nests of primitive-appearing round cells, which grow in a discohesive fashion, surrounded by hyalinized and highly vascular fibrous septae, producing a pseudoalveolar pattern.…”

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confidence: 99%

“…3,[6][7][8] In its classic form, alveolar rhabdomyosarcoma is comprised of distinctive nests of primitive-appearing round cells, which grow in a discohesive fashion, surrounded by hyalinized and highly vascular fibrous septae, producing a pseudoalveolar pattern. [3][4][5] Obvious rhabdomyoblastic differentiation, in the form of strap cells and cells with cross striations is seldom identified. Solid forms of alveolar rhabdomyosarcomas lack the prominent nested pattern and cellular discohesion seen in classic alveolar rhabdomyosarcomas and may closely mimic a variety of other 'small round cell tumors'.…”

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confidence: 99%

Aberrant expression of epithelial and neuroendocrine markers in alveolar rhabdomyosarcoma: a potentially serious diagnostic pitfall

Bahrami

Gown²,

Baird³

et al. 2008

Modern Pathology

162

119

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Alveolar rhabdomyosarcoma may be extremely difficult to distinguish from other primitive round cell neoplasms without ancillary immunohistochemistry and/or genetic study. Particularly in adults and in the head and neck locations, the differential diagnosis of alveolar rhabdomyosarcoma includes small cell carcinoma and neuroepithelial tumors, such as esthesioneuroblastoma. We have recently seen cases of genetically confirmed alveolar rhabdomyosarcoma, which were misdiagnosed owing to expression of cytokeratins and neuroendocrine markers. We studied a large group of well-characterized alveolar rhabdomyosarcomas for expression of such markers. Forty-four alveolar rhabdomyosarcomas (18 genetically confirmed) were retrieved from our archives and immunostained for wide-spectrum cytokeratin (OSCAR), low molecular weight cytokeratin (Cam5.2), synaptophysin, chromogranin A, and CD56 using commercially available antibodies. Cases were scored as 'negative', 'rare' (o5% positive cells), '1 þ ' (5-25%), '2 þ ' (26-50%) and '3 þ ' (451%). The tumors occurred in 23 males and 21 females at a mean age of 18 years (range, o1-64 years), and involved many sites. Fifty percent of cases (22 of 44) expressed wide-spectrum cytokeratin, and scored almost equally as rare, 1 þ , and 2 þ , but rarely 3 þ . Cam5.2 was positive in 52% (14 of 27). Forty-three percent of cases (16 of 37) expressed at least one of the specific neuroendocrine markers, 32% (12 of 37) expressed synaptophysin, 22% (eight of 36) expressed chromogranin A, and 11% expressed both. Expression of synaptophysin and chromogranin A was typically confined to rare cells but could be more widespread. Thirty-two percent of cases (12 of 37) expressed the wide-spectrum cytokeratin and at least one of the neuroendocrine markers, and 8% (three of 36) expressed cytokeratin and both neuroendocrine markers. CD56 expression was nearly ubiquitous. Aberrant expression of epithelial and neuroendocrine markers is relatively common in alveolar rhabdomyosarcoma, occurring in 30-40% of cases. These findings have significant implications for the diagnosis of alveolar rhabdomyosarcoma, particularly in adults and in the head and neck locations. Although expression of cytokeratin and/or synaptophysin alone does not necessarily indicate epithelial or neuroendocrine differentiation, coexpression of cytokeratin and neuroendocrine markers, and in particular the presence of chromogranin expression, suggest true epithelial and/or neuroendocrine differentiation in a subset of alveolar rhabdomyosarcomas. CD56 is not a specific neuroendocrine marker, and should not be used in the absence of synaptophysin/chromogranin. These findings emphasize the need to employ a panel of markers, to include desmin, myogenin/MyoD1, and genetic study in the diagnosis of primitive round cell neoplasms in all age groups and in all locations.

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Histopathological classification of childhood rhabdomyosarcoma: A report from the international society of pediatric oncology pathology panel

Cited by 58 publications

References 12 publications

Pathologic Classification of Rhabdomyosarcomas and Correlations with Molecular Studies

Pathologic Classification of Rhabdomyosarcomas and Correlations with Molecular Studies

Improved care of rhabdomyosarcoma in Jordan using less intensive therapy

Aberrant expression of epithelial and neuroendocrine markers in alveolar rhabdomyosarcoma: a potentially serious diagnostic pitfall

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