Management of cranial chondroblastoma in adults; a pooled analysis.

OBJECTIVES: Chondroblastoma is a rare bone tumour that often affects children more than adults. The adult cranial affection is a unique presentation of the disease (less than 2%) that mandates specific management. Through this pooled-analysis, we aimed to examine this disease group and obtain reliable scientific conclusions. METHODS: The literature was searched for case-reports of cranial chondroblastoma. Afterwards, a pooled analysis was done for the selected articles. The data in question were the patient's age, gender, disease site, modalities of treatment, disease control, salvage treatment, and disease-specific survival. RESULTS: 122 case reports were retrieved from the literature. The median age at diagnosis was 38.5 years. Up to the moment, the standard of care remains complete resection. The relapse-free survival was 29.5 months, with a three-year relapse rate of 9% and disease-specific mortality of 0%. Most of the relapses were salvaged excellently with excision. The strongest predictor for relapse was the post-resection residual disease, with three-years relapse rates of 23.8% and 2.4% for positive and negative residuals, respectively. Adjuvant Radiotherapy failed to improve the relapse rates. CONCLUSION: Complete surgical excision is the standard of care. Post-surgical residual is the only adverse prognostic factor that predicts poor disease control.

New insights inside the interdigitating dendritic cell sarcoma-pooled analysis and review of literature.

Interdigitating dendritic cell sarcoma is a rare haematological neoplasm with high debatable management protocols. The data extracted from 127 case reports published between 1981 and 2018 were analysed. The median age at diagnosis was 58 years with a male to female ratio of 1.65:1. The median OS and PFS of IDCS were 12 and 6 months, respectively, with a disease-specific mortality rate of 36.4%. Two-thirds of patients had a localised disease, while 30% had a disseminated form with 1-year mortality rates of 21.1% and 78.9%, respectively. Twenty per cent of cases were associated with other malignancies. Histologically, the proliferation of large spindle-shaped cells with fascicular growth was described in 84.3% of cases. Based on Cox-regression model, surgical resection was the only treatment modality linked to survival improvement with no recorded survival benefits of radiotherapy and chemotherapy. The 1-year mortality rates in resected and non-resected disease were 17.8% and 63.2%, respectively (P < 0.0001).

Dermatofibrosarcoma of The Breast - Case Report and Review of Literature.

Dermatofibrosarcoma protuberans is a rare form of soft tissue sarcoma that tends to invade and recur locally. The most common site of this disease is head, neck and extremities; however, the involvement of the breast has been reported. We present a case with a dermatofibrosarcoma of the breast that is composed of low-grade spindle cells which are positive for CD43. She was treated by wide surgical excision followed by adjuvant external beam irradiation at a dose of 60 Gray. After a follow-up of 24 months, no local-regional or distant recurrence was detected.

59 years old male with local advanced adenoid cystic carcinoma of the hypopharynx - A case report and a review of the literature.

Minor Salivary Gland tumours are rare, representing 2% of the head and neck tumours and less than 1% of hypopharyngeal cancers. The most common subtype of minor salivary gland tumours is adenoid cystic carcinoma. Although there are several case reports discussed it, there are only two reports discussing hypopharyngeal presentation in the literature, and they were treated by surgical resection. Despite that, our case report still to be unique as it presents a case of irresectable locally advanced tumour subjected to a trial of induction therapy.