Mandible ASPS 72 year old

[D.ap]

Alveolar soft part sarcoma of the mandible: A rare case

Divya Khosla1, Rohit Mahajan1, Awadhesh K Pandey1, Kislay Dimri1, Hitesh Verma2, Bhumika Bisht3, Harsh Mohan3
1 Department of Radiotherapy and Oncology, Government Medical College and Hospital, Chandigarh, India
2 Department of Otorhinolaryngology and Head and Neck Surgery, Government Medical College and Hospital, Chandigarh, India
3 Department of Pathology, Government Medical College and Hospital, Chandigarh, India


Correspondence Address:
Divya Khosla
Department of Radiotherapy and Oncology, Government Medical College and Hospital, Sector 32, Chandigarh - 160 030
India
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A 72-year-old woman presented with 3 months history of painful ulcer on the right side of the oral cavity associated with bleeding from ulcer and difficulty in chewing. There was no history of any addiction. On neck examination, a solitary right upper cervical lymph node measuring 1 cm × 1 cm was palpable. On examination, there was an ulcerative lesion on right alveolar ridge extending from lateral incisor till first molar and involving lower gingivobuccal sulcus. Laboratory examination comprising of hematology, liver, and renal functions were normal. Chest X-ray and ultrasound of abdomen and pelvis were normal. Orthopantomogram showed a lytic lesion involving the right side of the mandible. Preoperative biopsy was suggestive of sarcoma. The patient underwent wide local excision, segmental mandibulectomy, and supraomohyoid neck dissection. The mandibular reconstruction was done with iliac crest graft. Gross examination of the specimen revealed grey white tumor on the alveolar ridge measuring 3.5 cm × 2.3 cm × 1.2 cm. The tumor was soft to firm in consistency and eroded adjacent bone. Histopathological examination showed a relatively circumscribed tumor arranged in nests separated by thin fibrovascular septae [Figure 1]a]. The tumor cells were round to polygonal with distinct cell borders, vesicular nuclei, abundant granular eosinophilic cytoplasm and low mitotic activity. Periodic acid-schiff (PAS) positive diastase resistant cytoplasmic inclusions were present focally. The tumor cells showed diffuse cytoplasmic positivity for vimentin and focal positivity for S-100 [Figure 1]b] and were negative for chromogranin, smooth muscle actin, desmin and HMB-45. The posterior resection limit was involved by the tumor. Three out of 11 lymph nodes showed metastatic tumor deposits. The patient was advised postoperative radiotherapy in view of positive margins and lymph node metastasis but owing to poor compliance, she did not undergo adjuvant radiotherapy and came 4 months postsurgery with recurrence of disease at the primary site and appearance of cervical lymph nodes at level II. The lymph node measured 4 cm × 3 cm and was hard, fixed with overlying skin ulcerated. Fine needle aspiration cytology from the lymph node showed metastatic tumor deposits. Patient was started on chemotherapy with vincristine, adriamycin, and cyclophosphamide. She had progressive disease on chemotherapy. After 3 cycles, she was started on palliative radiotherapy of 35 Gy in 15 fractions. But there was no response in disease and her condition deteriorated. The patient was put on best supportive care in view of her old age, poor general condition and advanced disease neither responding to chemotherapy nor radiotherapy. She ultimately died of disease 10 months postsurgery.




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