|LETTER TO EDITOR
|Year : 2019 | Volume
| Issue : 2 | Page : 258-259
Outcomes in rhabdomyosarcoma: Indian perspective
Saurav Verma, Shalabh Arora, Sameer Rastogi
Department of Medical Oncology, AIIMS, New Delhi, India
|Date of Web Publication||20-Dec-2019|
Department of Medical Oncology, AIIMS, New Delhi
Source of Support: None, Conflict of Interest: None
|How to cite this article:|
Verma S, Arora S, Rastogi S. Outcomes in rhabdomyosarcoma: Indian perspective. Cancer Res Stat Treat 2019;2:258-9
In their article, Bhuvan et al. reported disease characteristics and outcomes of rhabdomyosarcoma (RMS) patients treated at a tertiary care center. They reported higher incidence of RMS at parameningeal sites (44%) and extremity (24%) as compared to the Intergroup Rhabdomyosarcoma Study (IRS-IV) (25% and 13%, respectively). The lack of patients with head and neck (excluding parameningeal sites) and orbit as primary site, which together constituted 16% in IRS-IV study, is intriguing and may be explained by the late presentation of patients in our population, resulting in a more extensive stage at the initial presentation. This unfavorable site presentation may be a reason for dismal outcomes in the Indian population compared to Western literature.
In their study, Bhuvan et al. showed that the most common histology was embryonal RMS (66%), followed by alveolar (9%), pleomorphic (4%), and not otherwise specified (NOS) (21%), with no spindle cell RMS. However, in the IRS-IV study, frequency of various tumor subtypes was embryonal/botryoid/spindle cell (70%), alveolar (20%), undifferentiated sarcoma (4%), and others classified as sarcoma NOS because of inadequate tissue or poor tissue fixation (6%). The high and low proportion of sarcoma NOS and alveolar sarcoma, respectively, calls for an astute pathological evaluation in sarcoma patients by a specialist sarcoma pathologist.
A comprehensive staging workup (fluorodeoxyglucose positron emission tomography–computed tomography [CT] scan or CT scan of the thorax, bilateral bone marrow aspiration and biopsies, bone scan for identifying the sites of metastases, besides CT or magnetic resonance imaging for local staging) is essential for treatment planning. Because histologic classification based on morphology alone is challenging, use of myogenic factors in immunohistochemistry (including myogenic differentiation 1; MyoD1) and molecular genetic studies (reverse transcription polymerase chain reaction or fluorescence in situ hybridization) to assess for fusion gene status of FOXO1 for prognostic stratification and guiding treatment is essential and needs to be incorporated into routine clinical practice.
Regarding local therapy in non-metastatic disease, complete surgical excision of the primary site was performed in 34% of patients, and radiotherapy to the primary site was given in 55% of patients, and this may account for high (50%) relapse rate. Complete surgical excision and radiotherapy to local site are cornerstones to achieve excellent local control in RMS. To improve RMS outcomes in India, besides systemic chemotherapy, we need to incorporate surgery and radiotherapy as part of the multimodality treatment, with dedicated sarcoma clinics and multidisciplinary teams. Patients with IRS group Stage III, Group I disease and all patients with residual microscopic disease (Group II) should receive radiotherapy, besides patients with alveolar histology and Group I disease, regardless of fusion status.
Treatment abandonment is a significant contributing factor responsible for worse outcomes in oncology in developing countries. Interventions like multidisciplinary psychosocial teams consisting of psychologists, social workers, child life, and palliative care providers to identify and target families at risk of abandonment are needed to decrease abandonment rate. Such interventions may also improve compliance to both local and systemic treatment and outcomes.
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Conflicts of interest
There are no conflicts of interest.
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