Combined Extraskeletal Chondrosarcoma of Left Orbit : A Rare Second Primary Tumor of Retinoblastoma Survivor A Case Report

Retinoblastoma, a rare eye malignant tumor of childhood, is generally responsive to chemotherapy or radiation therapy, but the survivors have high risk to development of second primary tumors (SPTs) due to genetic susceptibility and/or prior radiation therapy. The SPTs predominantly occured among heritable form of Retinoblastoma and show worse prognosis than Retinoblastoma itself. A 13 years old girl underwent enucleation to remove tumor mass within her left orbit. This tumor initially appeared 4 years after the patient undergoing right bulbar enucleation and receiving chemotherapy due to Retinoblastoma in her right eye. Clinical and radiologic examination suspected the left one as a late presentation of bilateral Retinoblastoma, but microscopic examination revealed a combined Extraskeletal Chondrosarcoma. The histologic type of this SPT is rare, so does it occurance at younger age and in a survivor with unilateral Retinoblastoma wihout a history of radiation therapy. The treatment of choice is combine surgery and chemo/radiation therapy, and the prognosis is poor.

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Study of the Incidence, Clinicopathological Profile, and Management of Second Primary Tumors in Patients with Index Head and Neck Tumors

Asian Journal of Oncology ◽

10.1055/s-0040-1713961 ◽

2020 ◽

Vol 6 (02) ◽

pp. 53-60

Author(s):

Chirag Jain ◽

Mansi Jain ◽

Ajay Kumar Dewan ◽

Pavani Medisetty ◽

Ankush Jajodia ◽

...

Keyword(s):

Squamous Cell Carcinoma ◽

Head And Neck ◽

Second Primary ◽

Primary Tumors ◽

Alive With Disease ◽

Clinicopathological Profile ◽

History Of ◽

Second Primary Tumors ◽

The Mean

Abstract Introduction This study was performed to study the incidence and clinicopathological profile of second primary tumors (SPTs) in patients with squamous cell carcinoma of head and neck at our institute. Materials and Methods In this study, we included the data of 120 patients who developed an SPT of the upper aerodigestive tract following treatment of their index tumor (IT). Since the online data of cancer patients in our cancer registry was available from January 2005, we started the study retrospectively from that time. At our institute, Rajiv Gandhi Cancer Institute and Research Centre, the incidence was found to be 8.4%. Warren and Gates criteria were followed for defining a second tumor. Results Our study results showed an incidence of 8.4% of SPTs among patients of head and neck squamous cell carcinoma (HNSCC). The mean age of the patients was 56.47 ± 10.42 years with a male predominance. The mean period of addiction in patients was 18.48 ± 4.63 years. It was found that patients with SPT had significant history of tobacco and alcohol use. The most common location for ITs and SPT was tongue and buccal mucosa. The main modality of treatment was surgery in all patient groups. Patients were followed up at three-month intervals for the first 2 years. The SPT was diagnosed with a confirmation biopsy. Majority of patients with SPT again underwent surgery with reconstruction with either free flaps or local flaps. Recurrence after SPT treatment was seen in 16.67% cases, and primarily, it was a locoregional recurrence. Only patients with at least 6 months follow-up posttreatment of SPT were included in this study. At the end of the study, 62.5% patients were disease free, 20.83% patients were alive with disease, and 16.67% patients were dead. Some of the patients who are alive with disease developed a third primary tumor which was managed as per guidelines. Conclusion The incidence of SPTs is 8.4% in our institute. This study adds to the theory of field cancerization proposed by Slaughter et al. We found a significant history of tobacco chewing in our patients who developed SPT. The clinical significance of this study is identifying the features of SPT in patients with HNSCC and allowing for a rational follow-up schedule. The most important part of treatment although still lies with the patient by quitting use of alcohol and tobacco.

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