|Year : 2010 | Volume
| Issue : 3 | Page : 307-309
Late recurrence in orbital rhabdomyosarcoma: Complete remission after multimodality management
Arun R Warrier1, Sanju Syriac2, Krishna Kumar Rathnam2
1 Department of Medical Oncology, Cancer Institute, Chennai- 36, India
2 Department of Medical Oncology, Cancer Institute (WIA), Chennai- 36, India
|Date of Web Publication||29-Nov-2010|
Arun R Warrier
Kumaran Hospital, No. 214, P.H. Road, Kilpauk, Chennai - 10
Source of Support: Cancer Institute (WIA), Chennai, Conflict of Interest: None
A case of orbital rhabdomyosarcoma had a localised recurrence 13 years after being treated with chemo radiotherapy. Late recurrences are rare in orbital RMS. Only two cases have been reported to have recurred after five years of follow up. Pathological similarity of both the lesions and occurrence outside the irradiated field excluded a radiationinduced second neoplasm. Immunohistochemistry staining with p 53 was positive. Patient had good response to chemotherapy and radiotherapy. Surgical resection of residue showed complete necrosis. Retreatment with combined modality therapy resulted in complete remission and the patient is on follow up.
Keywords: Chemotherapy, late recurrence, multimodality treatment, rhabdomyosarcoma
|How to cite this article:|
Warrier AR, Syriac S, Rathnam KK. Late recurrence in orbital rhabdomyosarcoma: Complete remission after multimodality management. J Can Res Ther 2010;6:307-9
|How to cite this URL:|
Warrier AR, Syriac S, Rathnam KK. Late recurrence in orbital rhabdomyosarcoma: Complete remission after multimodality management. J Can Res Ther [serial online] 2010 [cited 2020 May 30];6:307-9. Available from: http://www.cancerjournal.net/text.asp?2010/6/3/307/73326
| > Introduction|| |
Rhabdomyosarcoma is the most common soft tissue sarcoma in pediatric age group. Among the various sites, orbital presentation with embryonal histology has one of the best survival rates. We report a case of orbital rhabdomyosarcoma having a recurrence in the mandible 13 years after treatment with chemo radiotherapy. Late recurrences in rhabdomyosarcoma are very rare. In literature, only two cases have been reported as to have recurred after five years. Treatment with chemo radiotherapy followed by surgery of the residual mass resulted in complete remission.
| > Case Report|| |
A threeyearold boy presented in 1994 with proptosis. He had no fever or constitutional symptoms. CT scan revealed a mass over the medial aspect of the right orbit. [Figure 1]a Staging investigations including chest xay, bone marrow and bone scan were normal. Diagnostic biopsy was reported as embryonal rhabdomyosarcoma. 50 Gy EBRT was given to the orbit. This was followed by one year of combination chemotherapy comprising vincristine, cyclophosphamide and dacitnomycin alternating with adriamycin. Patient had treatment sequlae in the form of facial asymmetry and reduced vision in the right eye. Patient was asymptomatic and on regular follow up.
|Figure 1: a) CT scan of the orbit showing the soft tissue mass in 1994 |
b) Clinical image of the intraoral soft tissue mass in 2007
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In 2007, he presented with difficulty in mastication. On examination, a soft tissue growth in the left lower gingivum was seen in the molar and retro molar region. [Figure 1]b In the right eye, he had only perception of light. There was no lymphadenopathy or any other bony swellings. Hemogram, differential count, bone marrow examination and chest X-ray were normal. Bone scan revealed uptake in right orbit and left mandible. CT scan revealed bony sclerosis of right maxilla and a soft tissue lesion. Possibilities of second malignancy/recurrent disease were considered. Biopsy was done from the right maxilla which detected only sclerosis. Biopsy from the intraoral mass was consistent with embryonal rhabdomyosarcoma. The radiation treatment field for the case in 1994 was reviewed. The area of recurrence was not in the radiation field and even scatter radiation to the area was calculated to be few centigrays. Initial paraffin sections and those from the recurrence were compared and confirmed to be of same histological subtype [[Figure 2]a and b]. IHC for p 53 was positive [Figure 2]c. Final diagnosis was recurrent nonmetastatic rhabdomyosarcoma, head and neck, Group 1, stage 3.
|Figure 2: a) Primitive ovoid to spindle shaped cells in a myxoid stroma. (H and E, 20X) b) Spindle shaped cells with hyperchromatic nuclei in an abundant myxoid stroma. (H and E, 20X) c) Tumor cells show nuclear positivity for p53. 20X|
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After one cycle of chemotherapy, increase in tumour size was noted. Fifty gray of external beam radiation therapy to left mandible and retro molar trigone was given. Following this, there was good tumour response. Chemotherapy (vincristine, dacitnomycin and cyclophosphamide, alternating with ifosfamide and etoposide) was continued. After radiotherapy and eight courses of chemotherapy, there was a persistent intraoral swelling and patient was symptomatic. Left mandibulectomy revealed a 6 × 3 × 2.5 cm mass with 100% necrosis. As per protocol, 16 courses of chemotherapy were delivered up to October 2008. Patient continues to be in complete remission after six months of follow up.
| > Discussion|| |
Rhabdomyosarcoma is the most common pediatric soft tissue sarcoma. Although most cases of RMS are sporadic in nature, the disease has been associated with familial syndromes such as neurofibromatosis and the Li-Fraumeni syndrome. Embryonal histological subtype is seen in 50% of cases. About 10% patients present with orbital involvement.  Orbit is a favorable site and long term survival of more than 80% has been reported with chemo radiotherapy in various protocols. In orbital RMS, age less than three years and embryonal subtype have been associated with a favorable outcome.  Patients who were subjected to radiotherapy are at risk for reduction of vision, cataract formation and bony hypoplasia leading to facial asymmetry. 
Radiation-induced sarcoma remains uncommon and arises in 0.035 to 0.2% of all irradiated patients. The histological features of the original lesion and post radiation sarcomas are usually completely different. The most common histological subtypes of radiation-induced sarcomas are osteogenic, malignant fibrous histiocytoma, angiosarcoma, lymphangiosarcoma and spindle cell sarcoma.  Investigators from Memorial Sloan-Kettering Cancer Centre have opined that combination of radiation therapy and high dose cyclophosphamide and dactinomycin used in the treatment of rhabdomyosarcoma may be responsible for the development of second malignant neoplasms. 
Mutation of p 53 changes confirmation of the molecule through aminoacid substitutions, and the protein is stabilised. This leads to an over expression of the protein and an increased level within the cell. It has been reported that immunodetection of p53 in paraffin-embedded material is reproducible and accurately reflects over expression of the p53 protein in human tumors. 
Survival after relapse in recurrent RMS is influenced by age at initial presentation, histology as well as group and stage of the patient. Patients who received radiation had better event free survival, although overall survival was similar to non irradiated patients.  In the subset of orbital RMS, patients initially treated with vincristine and dacitnomycin had a five-year survival after relapse of 86%. 
The case is significant because only two cases of orbital RMS with late recurrence after five years have been reported.  The close proximity of the site of recurrence to the initially irradiated area raised the doubt of a second malignancy. Verification of the initial radiation field with the site of recurrence showed that chance of even scatter radiation was minimal. Pathological comparison of the initial slides and biopsy from recurrence confirmed the similar histology. Immunohistochemistry of p 53 being positive indicated toward an increased susceptibility for malignancy, as a result of p 53 mutation and over expression. Left hemimandibulectomy specimen showed complete necrosis. The surgery was essential for symptomatic relief as the residue was interfering with mastication and speech. Achieving a complete remission is only the beginning of a period of close follow up.  As any survivor of a curable pediatric neoplasm, this patient also requires interventions to reduce the side effects of treatment and also for early detection of curable malignancies.
| > Acknowledgement|| |
1) Dr. Shirley Sundersingh, MD. 2) Dr. Lakshmi Srinivas, MD, DM. 3) Dr. T G Sagar, MD, DM, Cancer Institute (WIA), Chennai.
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[Figure 1], [Figure 2]