Dedifferentiated chondrosarcoma: current standards of care.

Dedifferentiated chondrosarcomas are aggressive variants of chondrosarcoma, associated with poor outcomes. Tumor biphasism is the norm. The majority of these tumors are symptomatic at presentation. Radiologically, large soft tissue masses with bony destruction predominate. Treatment protocols of these tumors are not well defined. Surgical resection forms the standard of care for localized disease. (Neo)adjuvant therapies remain controversial as the results from multiple (mainly retrospective) studies remain conflicting. Age at presentation, stage and ability to obtain negative resection margins are important prognostic factors. The overall prognosis is dismal. Newer and novel therapies targeting the complex genetic makeup of these tumors have renewed interest in the adjuvant setting that could hold promise in the near future.

Lay abstract Dedifferentiated chondrosarcomas are rare cancers composed of two components: a high-grade component and a low-grade component, with one abruptly blending into another. These rare tumors affect middle-aged individuals and present with pain and swelling in the affected site. X-rays and other scans often show tumor within the soft tissue with bony destruction. Although the precise treatment protocol is not well defined, surgery remains the standard of care for those where the tumor has not spread elsewhere. Once the disease spreads to other parts of the body, the outcome is very poor. The role of certain drugs targeting the tumor (chemotherapeutic agents) is controversial. This review briefly describes the genetic basis, treatment modalities involved and newer agents being developed for this lethal cancer.

Evaluation of Metronomic Therapy as a Low-Cost, Sustainable, Standard-of-Care Option in Desmoid Fibromatosis: Real-World Data From a Tertiary Care Center in India.

PURPOSE: Desmoid fibromatosis (DF) is a locally aggressive tumor with low mortality but significant morbidity. There is a lack of standard of care, and existing therapies are associated with significant barriers including access, cost, and toxicities. This study aimed to explore the efficacy and safety of the metronomic therapy (MT) in DF in a large, homogenous cohort from India. PATIENTS AND METHODS: This study involved histologically confirmed DF cases treated with MT comprising vinblastine (6 mg) and methotrexate (15 mg) both once a week, and tamoxifen (40 mg/m2) in two divided doses once daily between 2002 and 2018. RESULTS: There were 315 patients with a median age of 27 years; the commonest site was extremity (142 of 315; 45.0%). There were 159 (50.1%) male patients. Of the 123 (39.0%) prior treated patients, 119 had surgery. Of 315 patients, 263 (83.5%) received treatment at our institute (MT-151, 77-local treatment, 9-tyrosine kinase inhibitor, and 26 were observed). Among the MT cohort (n = 163, 61.2%), at a median follow-up of 36 (0.5-186) months, the 3-year progression-free and overall survival were 81.1% (95% CI, 74.3 to 88.4) and 99.2% (95% CI, 97.6 to 100), respectively. There were 35% partial responses. Ninety-two patients (56.4%) completed 1-year therapy, which was an independent prognosticator (P < .0001; hazard ratio, 0.177 [95% CI, 0.083 to 0.377]). MT was well tolerated. Predominant grade ≥3 toxicities were febrile neutropenia, 12 (7.4%) without any chemotoxicity-related death. The annual cost of MT was $130 US dollars. CONCLUSION: The novel, low-cost MT qualifies as one of the effective, less toxic, sustainable, standard-of-care options for the treatment of DF with global reach and merits wide recognition.

A Survey of Personnel Protective equipment's (PPE) Use and Comfort Levels Among Surgeons During Routine Cancer Surgery in the COVID-19 Pandemic.

Personnel protective equipment (PPE) are recommended during surgery even in COVID-19 negative patients especially in a high-prevalence region due to its higher false-negative rates. However, the use of PPE has not been universal mainly due to the perception of discomfort and associated stress and fatigue. This study was done to understand the pattern of PPE use by cancer surgeons during the pandemic and the associated discomfort, stress, and fatigue with its use. The survey, consisting of 29 questions, was circulated widely across the country by email and chat groups among cancer surgeons. The study was registered with the Clinical Trials Registry of India (CTRI/2020/08/027050). We received a total of 342 evaluable responses that could be included for analysis. All the respondents used appropriate PPE in different combinations. N-95 mask and the face shield were the two components of the PPE that gave rise to a lot of discomforts. Fogging (of face shields) (p < 0.001,OR 3.61), dryness of mouth (p < 0.001,OR 3.35), and breathing difficulty/feeling of suffocation (p = 0.034,OR 1.68) contributed to the stress, whereas headache (p < 0.001,OR 11.34) and breathing difficulty/feeling of suffocation (p < 0.001,OR 4.24) contributed to the fatigue associated with PPE use the most. PPE was routinely used during cancer surgery in COVID-19 negative patients during the pandemic. However, most surgeons experienced different degrees of discomfort, especially with the N-95 masks and eye protection. Supplementary Information: The online version contains supplementary material available at 10.1007/s13193-021-01316-6.