Volume 6, Issue 1 , Pages 42-44, January 2011
Second osteosarcoma of mandible after rhabdomyosarcoma of the cheek
Article Outline
Abstract
Development of a second malignant neoplasm is one of the most serious complications of cancer treatment. Second osteosarcoma developing after rhabdomyosarcoma has been reported rarely in literature. Here, we report a 8.5-year-old male presented with second osteosarcoma of the mandible 3 years after completion of treatment of rhabdomyosarcoma of the cheek.
Keywords: Second malignancy, Osteosarcoma, Cheek, Rhabdomyosarcoma, Children
1. Introduction
The occurrence of a second malignancy represents a serious complication of pediatric cancer. Depending upon advances in diagnosis and cancer treatment and consequently increased survival rates after childhood cancers increased number of second malignant neoplasms (SMN) is not surprising [1], [2]. Second osteosarcoma developing after soft tissue sarcomas has been reported scarcely in a few series [1], [2], [3], [4]. Here, we report a case of second osteosarcoma of the mandible after rhabdomyosarcoma of the cheek.
2. Case report
A 3.5-year-old-boy was admitted with the complaint of swelling in his left cheek in June 2004, especially growing in the last 3 months. In physical examination there was a tough mass sized 8
cm×5cm covering the left cheek. Computed tomography (CT) of the neck revealed a well-demarcated, homogenous soft tissue mass of 50mm×45mm×40mm in size, in the left cheek (Fig. 1A) and bilateral prominent lymph nodes (2cm×1cm) at level II. Histopathological examination of the specimen after an incisional biopsy revealed spindle cell rhabdomyosarcoma. There was no metastasis on chest and abdominal CT examinations of the patient. A chemotherapy protocol consisting of cisplatin, doxorubicin, cyclophosphamide and vincristin was started. The patient responded well to the chemotherapy (Fig. 1B) and after 6 courses, the residual tumor of 1.5cm in diameter was resected totally. Histopathological examination revealed rhabdomyosarcoma with positive surgical margins. A radiotherapy consisting of 5040cGy to the primary tumor, 4140cGy to supraclavicular fossa and 5040cGy to bilateral cervical region metastasis was delivered in 28 fractions with three-dimensional conformal plans. After completion of radiotherapy at April 2005, a more intensive chemotherapy protocol consisting of ifosphamide, carboplatin and etoposide was given. After six courses the therapy was completed with clinical and radiological complete remission in December 2005. The patient was well and followed with no evidence of disease for 3 years. In January 2009 the patient presented with a swelling in right facial region that was partly within the previous radiation field. In physical examination there was a painful and tough mass on the mandible extending to the submandibular region. Plain X-ray showed a periosteal reaction in right side of the mandible. A CT and magnetic resonance (MR) examinations revealed an inhomogenous soft tissue mass with new bone formation, bony destruction and soft tissue component in right angle of the mandible extending to coronoid process. The mass had an epicenter in the mandible and ipsilateral masticator muscles as well as parotid gland (Fig. 2A and B). Curettage biopsy of the mass revealed chondroblastic osteosarcoma (Fig. 3). A salvage chemotherapy consisting of high dose ifosphamide and methotrexate was started. The patient was on chemotherapy with minimal regression in the mass at the time.
Fig. 1.
(A) Axial postcontrast CT study shows a huge homogenous soft tissue mass in the left cheek. (B) At 3 months follow-up imaging following six courses of chemotherapy a residual soft tissue mass of about 1.5
cm in diameter is seen.
Fig. 2.
(A) Axial postcontrast CT scan at 22 months of diagnosis of rhabdomyosarcoma seen on Fig. 1A shows a new separate mass centered around the mandible with destruction and new bone formation is seen. (B) On MR imaging, the mass enhances with Gadolinium profoundly and involvement of ipsilateral masticator muscles and parotid gland is seen.
Fig. 3.
Osteoid production and pleomorphic neoplastic cells, H
+E (460×).
3. Discussion
Second malignant neoplasms are serious events following therapy for primary tumors [1]. With current management strategies for childhood cancers, more than 70% of children diagnosed with cancer are expected to survive at least 5 years [4], [5]. Increased survival causes the increased risk of long-term complications including SMN [6]. Development of SMN is among the most deleterious effects of cancer treatment. Survivors of childhood cancer are at greater risk for the development of SMN.
Most common SMN reported is leukemias, sarcomas, central nervous system (CNS) tumors and thyroid gland carcinomas after childhood cancer [1], [2], [7]. Caglar et al. [1] have reported osteosarcoma as the most frequently occurring (22.2%) second malignant neoplasm in their series. In the same study 4 out of 6 patients with osteosarcoma occurred within the radiation field [1]. SMN after soft tissue sarcomas are also reported rarely and most of them are osteosarcoma, chondrosarcoma, CNS tumors and acute myelogenous leukemia [2]. In a large series with 1770 patients with rhabdomyosarcoma only 22 SMN have been reported after a median 8.4 years’ follow-up and bone sarcomas followed by AML were found as the most common SMN [3].
Rich et al. [2] have reported that the median time for the development of SMN is 11.4 years (1.5–21 years). Median time to the development of a second malignancy was reported to be 7 years in another study [3]. The lag time in a patient survived from osteosarcoma as a second malignancy after the treatment of rhabdomyosarcoma was 15 years in Heyn et al.’s study [3]. The lag time of 3 years after the completion of radiotherapy in our patient may be considered as suspicious of treatment induced second neoplasm. However, in Mayo Clinic experience radiation induced malignancies were reported to present at 1 year after treatment [8]. Short time interval can also be an indicator of poor prognosis or but it will be depicted after the completion of treatment.
Genetic predisposition or long-term effects of chemotherapy and radiotherapy can be responsible for the increased risk of SMN after childhood cancer [9], [10]. Genetic susceptibility may be accused for the development of SMN in cancer patients, however we could not differentiate any evidence of tendency for familial cancers. In the present case there was not any family member or relative with retinoblastoma, osteosarcoma, neurofibromatosis or Li-Fraumeni syndrome. We believe that our patient is not a presentation of metachroneous osteosarcoma. The present data led us to consider radiation induced second osteosarcoma because a mass of a discrete histopathology apart from the previous one appeared inside the previous irradiation field. There is extensive literature data reporting additive role of the combined chemo- and radiotherapy in the development of SMN [3]. Certain tumors are related directly to the treatment procedure itself. Osteosarcoma is reported as a second cancer that can frequently occur in an irradiation field.
The second cancer in our patient is interesting because of the occurrence of a second malignancy in a bony structure with a different histopathology after the treatment of a soft tissue sarcoma. Intensive chemotherapy and close proximity to radiation fields might be responsible for the development of second osteosarcoma in our patient.
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PII: S1871-4048(10)00013-4
doi:10.1016/j.pedex.2010.02.001
© 2010 Elsevier Ireland Ltd. All rights reserved.
Volume 6, Issue 1 , Pages 42-44, January 2011
