Case Report
Osteosarcoma of Mandible in Pregnancy - A Management Challenge.
Authors
Sandeep Bansal, Assistant Professor,
Lokesh Kumar, Senior Resident,
Neelam Choudhary*, Senior Medical Officer,
Rijuneeta, Additional Professor,
Ashok K Gupta, Professor
Department of Otolaryngology & Head–Neck Surgery, Department of Obstetrics and Gynaecology*,
Postgraduate Institute of Medical Education & Research,
Chandigarh-160012, India.
Address for Correspondence
Dr. Sandeep Bansal,
Assistant professor,
Dept. of Otlaryngology & Head Neck Surgery,
PGIMER, Sector 12, Chandigarh,India.
E-mail:
drsandeepb@rediffmail.com
Citation
Bansal S, Kumar L, Choudhary N, Rijuneeta, Gupta AK. Osteosarcoma of Mandible in Pregnancy - A Management Challenge. Online J Health Allied Scs.
2015;14(1):6. Available at URL:
http://www.ojhas.org/issue53/2015-1-6.html
Open Access Archives
http://cogprints.org/view/subjects/OJHAS.html
http://openmed.nic.in/view/subjects/ojhas.html
Submitted: Feb 24,
2015; Accepted: Mar 28, 2015; Published: Apr 10, 2015 |
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Introduction:
Osteogenic sarcoma is most common malignant tumor of bones. Around 6 to 8% of Osteosarcomas occur in the head and neck, most commonly in the maxilla and mandible. Occurrence of malignancy in pregnant women is a rare event, with an incidence of 0.07 to 0.1% of all pregnancies.[1] So far, soft tissue sarcomas that have occurred in long bones and pelvic bones of pregnant women have been reported. Here we are presenting first case report of osteogenic carcinoma of mandible that occurred in a primigravid pregnant woman and its management.
Case Report:
A 25 year old primigravid pregnant with eighteen weeks of amenorrhea presented to our department with complaints of painful, progressive swelling in the right lower jaw along with numbness of the lower lip for the past two months. She also gave a history of dental extraction one month back. The pain was sometimes often severe and was radiating to neck and right ear as well. She also had a recent development of restricted mouth opening from last fifteen days. On examination, there was a 10 x 8 cm firm, tender swelling over the right jaw with stretching of skin over it. Her mouth opening was restricted to one and half fingers; there was diffuse widening of the lower gums from retromolar trigone to premolars with loosened molars. The swelling was tender on examination. There were no palpable cervical lymph nodes.
On contrast-enhanced computerized tomography, there was a heterogeneously enhancing mass causing the erosion of the right side of mandibular body and ramus (Figure 1). On clinical suspicion of a malignant tumor, a deep incisional biopsy was taken from the intraoral bulge, which revealed Osteosarcoma. Preoperative ultrasound scan for fetal well being revealed a single live intrauterine fetus of age eighteen weeks and six days. As per parenteral interest, surgery was planned. She underwent wide local excision and segmental resection of the mandible with primary approximation of the surrounding soft tissues. Intraoperative frozen section also confirmed that the features are those of pleomorphic osteosarcoma.
Checks fetal scan in the postoperative day 1, revealed live fetus however thorough discussion with the patient and her relatives consented for medical termination of pregnancy. Termination of pregnancy was undertaken in obstetrics department with mifepristone and misoprostol. Patient recovered from operative and obstetric stress and was discharged in stable condition.
Postoperative histopathological examination showed tumor cells arranged in sheets intervened by thin fibrous septa. The cells were round to polygonal in shape with moderate pleomorphism, vesicular nuclei, prominent nucleoli with abundant amount of eosinophilic cytoplasm. Osteoid formation was seen at some foci along with osteoclastic gaint cells. Overlying mucosa was hyperplastic and ulcerated. Overall features were suggestive of Osteosarcoma. All the bony and soft tissue margins were free of tumor.
Post operatively she received concurrent chemoradiotherapy. External beam radiotherapy of 50 Gy /25 fractions over five weeks was given along with six cycles of Injection Cisplatin and Injection Adriamycin. After a close follow up for ten months, there was no evidence of locoregional recurrence (Figures 2). She was being planned for second stage reconstruction of mandible with free fibular osseous flap.
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Figure 1: CECT face showing expansile, lytic lesion involving the ramus and angle of mandible. |
Figure 2: Postoperative image after 10 months showing disease free status |
Discussion:
Osteosarcoma of jaw is very aggressive, malignant tumors account for 5 to 13% of all musculoskeletal osteosarcomas.[2] Osteosarcoma in long bones most commonly occur in young adults where as jaw osteosarcoma usually present at a later age group (mean age of 34 to 36 years).[3] Head and neck osteosarcomas behave distinctly from that of long bones.
The occurrence of cancer in pregnancy is an infrequent event accounting for 0.07 to 0.1% of all pregnancies.[1] The hormonal influence that act on the initiation and development of malignancies during pregnancy is highly controversial. However, there were studies which have proven the pregnancy related aggressiveness in the tumors.[4,5]
Influence of pregnancy on the natural process of bone and soft tissue sarcomas is controversial. Remmelink M etal[6] in his in vitro studies showed that, osteosarcoma cell lines may be affected by sex steroids, but effect was not related to the gender of the cell line.
In our case, patient presented two months after the development of swelling. Delayed presentation may be attributed to lack of signs and symptoms, local remedies, religious attitude, lack of interest on part of patient.[6] Mandibular osteosarcomas cause early compression of underlying nerve leading to paraesthesias and numbness in the lower lip.[7] Our patient also had numbness and tingling sensation in the lower lip.
Early diagnosis and aggressive radical resection plays a key role in achieving high survival rates.[2] In our case, wide local excision with segmental mandibulectomy was opted as a surgical procedure. Patient has consented for second stage osseus reconstruction. Even though, the fetal well being was ensured in the post operative period, the social and religious factors forced the couple to opt to go for medical termination of pregnancy at ninteen weeks and two days of gestation.
Although in our case, all the margins were free of tumor, in view of aggressive course of the disease, concurrent chemoradiotherapy was administered. In the series of 111 cases of mandibular osteosarcomas, reported by J Thariat etal[8], neoadjuvant chemotherapy was administered in 93% and postoperative chemotherapy in 54.7%. In this series, chemotherapy had improved disease free and metastasis free survival from 50% to 68%. We followed up the case for ten months and patient continued to be under follow up without evidence of loco regional recurrence. It was planned to perform free fibular osseous reconstruction of the resected mandibular segment.
Conclusion:
Osteosarcoma of mandible in pregnancy is very rare. So far, there is no other case reported in English literature. Delay in presentation to hospital can be attributed to illiteracy, lack of signs and symptoms, local remedies, religious attitude, lack of interest on part of patient. Early diagnosis, radical resection of the tumor with wide margins along with adjuvant chemotherapy play a major role in improving the disease free and overall survival.
References:
- Maxwell C, Shah V,Wunder JS, Bell R, Farine D.Maternal and Neonatal outcomes in pregnancies complicated by bone and soft-tissue tumors. Obstetrics and Gynecology 2004;104:344-348.
- Mardinger O, Givol N, Talmi YP, Taicher S, Saba K, Tel Aviv , Hashomer T. Osteosarcoma of the jaws. Oral Surg Oral Med Oral Pathol Oral Radiol Endod. 2001 Apr;91(4):445-51.
- Murphey MD, Robbin MR, McRae GA, et al. The many faces of osteosarcoma. Radiographics 1997; 17:1205-31.
- Aterman K, Fraser GM, Lea RH. Disseminated peritoneal leiomyomatosis. Virchows Arch (Pathol Anat) 1977;374:13-26.
- Taylor HB, Helwig EB. Dermatofibrosarcoma protuberans: a study of 115 cases. Cancer 1962;15:717-25.
- Merimsky O, Cesne AL. Soft tissue and bone sarcomas in association with pregnancy. Acta Oncologia. 1998; 37:721-727.
- Jitendra V. Kalburge, Sunil K. Sahuji, Vaishali etal. osteosarcoma of mandible. Journal of Clinical and Diagnostic Research. 2012 November;6(9):1597-1599.
- Thariat J, Schouman T, Brouchet A et al Osteosarcomas of the mandible: multidisciplinary management of a rare tumor of the young adult a cooperative study of the GSF-GETO, Rare Cancer Network, GETTEC/REFCOR and SFCE. Annals of Oncology 2012;1–8. doi:10.1093/annonc/mds507. Available at http://annonc.oxfordjournals.org/content/early/2012/11/05/annonc.mds507.full
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