A Rare Case of Angiosarcoma of Tibia in a Young Age-a Case Report.

Angiosarcoma of bone is very rare entity, accounting for less than 1% of all primary bone sarcomas with associated poor prognosis. We hereby present a case report of angiosarcoma of tibia in a young boy and reviewed its literature and management of the disease. A 21-year young male presented to us with complaints of pain and swelling over left lower leg since last 3 months. On evaluation, MRI lower leg revealed multiloculated lesion of size 32×36×52 mm showing multiple fluid-fluid levels which was hyperintense on T2 images in left distal tibial metaphysis showing endosteal scalloping with cortical destruction along lateral border and abuts the physis. Image-guided biopsy was done. PET CT revealed no evidence of metastasis. Histopathology report revealed tumor involving bone and soft tissue comprising of plump ovoid to epitheloid/spindled cells in nests and focal fascicles with marked nuclear atypia, prominent nucleoli and mitotic figures with vascular pattern with thick walled vessels, many haemosiderophages. Differentials of telangiectatic osteosarcoma/adamantioma was made. On further IHC studies, tumor cells were found positive for SATB2, CD31, ERG while negative for CK, P40, DESMIN, MYOGENIN, TLE-1, S-100. Diagnosis of angiosarcoma of tibia was confirmed. Decision to give neo-adjuvant chemotherapy with doxorubin/Ifosfamide-based regimen was made followed by later with limb salvage surgery. He received 5 cycles of chemotherapy 3 weekly regimen which he tolerated well. Repeat MRI leg showed partial reduction in tumor size but there was presence of pathological fracture seen with some extraosseous component of tumor. Limb salvage surgery was not possible and he underwent below knee amputation. His postoperative recovery was eventful. Final histopathology confirmed diagnosis of angiosarcoma tibia. Case was discussed in multidisciplinary tumor board and he was started on alternate chemotherapy with Injection docetaxel and gemcitabine-based regimen. He had received 3 cycles of this regimen till now and is disease free from last 3 months. Angiosarcoma of bone is a very rare entity; the literature is limited regarding treatment and outcome of patients with this tumor. Most information is currently available from case reports, and treatments are based on guidelines for other types of primary bone sarcomas. We hereby present a case report of angiosarcoma of tibia in a young boy and reviewed its literature, diagnostic dilemmas associated with it and management of the disease after discussing in multidisciplinary board. Role of chemotherapy, surgery, and their sequencing is not well defined. Prospective trials are required to manage this rare entity.

Can a diagnosis of Leiomyosarcoma soft palate be missed in the era of commoner oral carcinomas? - Rare case report and review of literature.

Leiomyosarcomas (LMSs) of the head and neck are an extremely rare entity. Of all smooth muscle tumors, 4%-10% occur in the head and neck and only 0.06% in the oral cavity. Because of its infrequency, it has been associated with both delayed diagnosis and misdiagnosis. Here, we report the clinicopathological findings of a case of primary LMS of the soft palate in a 42-year-old male patient with an emphasis on the judicious use of ancillary diagnostic modalities to arrive at a definitive diagnosis. Intraorally, LMSs present as painless, lobulated, fixed masses of the submucosal tissues in middle-aged or older individuals. The treatment modalities and lymph nodal dissection criteria are dissimilar to more common oral carcinomas. Hence, definitive diagnosis is necessary.

A Randomized, Placebo-Controlled Study to Evaluate the Effect of Bio-Enhanced Turmeric Formulation on Radiation-Induced Oral Mucositis.

INTRODUCTION: Oral mucositis is the most common toxicity of chemoradiotherapy treatment of head and neck cancers. The present study was performed to evaluate the effect of a researched turmeric formulation on oral mucositis in patients receiving chemoradiotherapy for oral cancer. METHODS: This randomized double-blinded placebo-controlled trial included 60 patients with oral cancer who had undergone radical surgery. Patients were equally randomized into 3 arms. Bio-enhanced turmeric formulation (BTF) capsules (low dose [1 g/day] or high dose [1.5 g/day]) or placebo was administered daily for 6 weeks with concurrent chemoradiotherapy. Study endpoints included the impact of the treatment on chemoradiotherapy-induced oral mucositis along with dysphagia, oral pain, dermatitis, and weight loss. RESULTS: The incidence of grade 3 toxicity of oral mucositis, oral pain, dysphagia, and dermatitis was significantly lower in patients who received BTF than placebo. Twenty-five and 20% patients in BTF 1 g/day (p = 0.011) and 1.5 g/day (p = 0.004) arms, respectively, developed grade 3 oral mucositis compared to 65% patients in the placebo arm. Thirty-five and 30% patients in BTF 1 g/day (p = 0.027) and 1.5 g/day (p = 0.011) arms, respectively, developed grade 3 oral pain compared to 70% patients in the placebo arm. Twenty-five and 20% patients in BTF 1 g/day (p = 0.025) and 1.5 g/day (p = 0.010) arms, respectively, developed grade 3 dysphagia compared to 60% patients in the placebo arm. Ten and 5% patients in BTF 1 g/day (p = 0.114) and 1.5 g/day (p = 0.037) arms. respectively, developed grade 3 dermatitis compared to 30% patients in the placebo arm. Patients under BTF supplementation experienced significantly less weight loss and greater compliance with treatment than placebo. CONCLUSION: BTF (BCM-95®) can significantly reduce chemoradiotherapy-induced severe oral mucositis, dysphagia, oral pain, and dermatitis in oral cancer patients. TRIAL REGISTRATION: Clinical Trials Registry, India (Registration No. CTRI) (CTRI/2015/12/006413 dated December 4, 2015).

A randomized prospective study comparing acute toxicity, compliance and objective response rate between simultaneous integrated boost and sequential intensity-modulated radiotherapy for locally advanced head and neck cancer.

PURPOSE: Intensity-modulated radiotherapy (IMRT) provides higher dose to target volumes and limits the dose to normal tissues. IMRT may be applied using either simultaneous integrated boost (SIB-IMRT) or sequential boost (SEQ-IMRT) technique. The objectives of this study were to compare acute toxicity and objective response rates between SIB-IMRT and SEQ-IMRT in patients with locally advanced head and neck cancer. MATERIALS AND METHODS: Total 110 patients with locally advanced carcinoma of oropharynx, hypopharynx, and larynx were randomized equally into the two arms (SIB-IMRT vs. SEQ-IMRT). Patients in SIB-IMRT arm received dose of 66 Gy in 30 fractions, 5 days a week, over 6 weeks. SEQ-IMRT arm's patients received 70 Gy in 35 fractions over 7 weeks. Weekly concurrent cisplatin chemotherapy was given in both arms. Patients were assessed for acute toxicities during the treatment and for objective response at 3 months after the radiotherapy. RESULTS: Grade 3 dysphagia was significantly more with SIB-IMRT compared to SEQ-IMRT (72% vs. 41.2%; p = 0.006) but other toxicities including mucositis, dermatitis, xerostomia, weight-loss, incidence of nasogastric tube intubation and hospitalization for supportive management were similar in both the arms. Patients in SIB-IMRT arm showed better treatment-compliance and had significantly less treatment-interruption compared to SEQ-IMRT arm (p = 0.028). Objective response rates were similar in both the arms (p = 0.783). CONCLUSION: Concurrent chemoradiation with SIB-IMRT for locally advanced head and neck cancer is well-tolerated and results in better treatment-compliance, similar objective response rates, comparable incidence of mucositis and higher incidence of grade 3 dysphagia compared to SEQ-IMRT.