Is hepatocellular carcinoma complicated with portal vein tumor thrombosis potentially curable by radiotherapy in the form of stereotactic body radiation therapy?

PURPOSE: The prognosis of hepatocellular carcinoma (HCC) with portal vein tumor thrombosis (PVTT) is dismal. Despite best treatment and care, the patients with this malignancy only showed 2.7-4 months of overall survival. It is debatable whether liver transplantation helps PVTT sufferers. The effectiveness of radiation therapy in treating HCC patients with PVTT should not be undervalued. By limiting the high dosage region to a small planning target volume, stereotactic radiation delivery has shifted toward hypofractionation, limiting the radiation exposure to healthy organs and tissues. Stereotactic body radiotherapy (SBRT) has a local control rate of 75-100%, depending on the treatment. The major limitation in SBRT for hepatocellular carcinoma with PVTT is the paucity of prospective evidence for longer periods beyond the first two years after treatment. More prospective studies/randomized clinical trials with a longer follow-up, larger sample size, and adequate statistical power are the dire need of the present situation to ascertain the curative effect of SBRT as primary therapy for advanced HCC with PVTT. CONCLUSION: SBRT can improve survival, particularly for patients receiving multidisciplinary treatment. This review sums up our most current understanding of how radiation therapy, notably SBRT, can be used to treat hepatocellular carcinoma when combined with PVTT. Recent research has led us to believe that irradiation in the form of SBRT may cure hepatocellular carcinoma complicated by PVTT.

Successful multimodality management of extrarenal extracranial malignant rhabdoid tumour of the left sciatic nerve mimicking a neurofibroma.

Extrarenal extracranial malignant rhabdoid tumour (MRT) is a rare and highly aggressive tumour representing <1% of paediatric soft tissue malignancies. Only a few cases of MRT of the thigh arising from the sciatic nerve have been reported in medical literature to date. A 5-year-old girl presented with progressively increasing painless lump in the posterior aspect of the left thigh. A contrast-enhanced magnetic resonance imaging (MRI) of the left thigh showed a 4.7 × 5 × 10.5 cm well-marginated, lobulated, homogeneously enhancing lesion in the posterior compartment of the left thigh along the course of the sciatic nerve. She underwent en bloc excision of the left sciatic nerve tumour and end-to-end anastomosis of the left sciatic nerve with a right sural nerve graft. Histopathological and immunohistochemical examination of the surgical specimen revealed a malignant rhabdoid tumour. INI-1 immunoexpression was lost in the tumour cells. The metastatic workup was essentially normal. Subsequently, she received post-operative radiotherapy to the tumour bed (50.4 Gray in 28 fractions over 5.5 weeks) followed by six cycles of multiagent chemotherapy with ICE (Ifosfamide, Carboplatin, and Etoposide) regimen. On the last follow-up visit, 20 months after surgery, she was in complete clinical and radiological response. Aggressive multimodality management comprising radical resection of tumour, post-operative radiotherapy to the tumour bed, and multiagent chemotherapy with ICE regimen can lead to favourable outcomes in patients with this rare tumour.

Laryngeal soft tissue sarcoma: Systematic review and individual patient data analysis of 300 cases.

BACKGROUND: Laryngeal sarcoma is rare. We performed a systematic review and individual patient analysis to evaluate the patterns of care, prognostic factors, and role of radiotherapy in laryngeal soft tissue sarcoma. METHODS: A systematic search on PubMed and Google scholar was done. An individual patient data analysis was done. RESULTS: Of the 300 cases of laryngeal sarcoma, 80% underwent surgery. 44% underwent larynx preservation surgery and 25% received radiotherapy with surgery. Median progression free survival (PFS) was 48 months and overall survival (OS) of 224 months for the entire cohort. Patients with large primary, cartilage invasion, and positive margins had numerically worse PFS. Cartilage invasion and primary tumor size >3 cm were the most common risk factors for adjuvant radiation therapy. Patients receiving radiotherapy were not associated with better survival. CONCLUSION: Laryngeal sarcoma associated with a good survival. Larynx preservation surgery is feasible in nearly half patients. Adjuvant radiotherapy may be warranted in patients poor prognostic factors.