Showing posts with label oncology. Show all posts
Showing posts with label oncology. Show all posts

Monday, April 6, 2015

Learning to Care at the End- how could there be a better title?

By: Lydia Furman, MD,  Assistant Editor

    If you just have time for a quick read today go to “Learning to Care at the End” by trainee doctor MacDonell-Yilmaz (doi:10.1542/peds.2014-4125).  This excellent and moving article is the second monthly feature from the Section on Residents Students and Fellowship Trainees (SOMSRFT). The underbelly of training in hematology-oncology for resident physicians is that this rotation is often one of the first in which infants, children and teenagers die. Much of pediatrics is a very positive world of getting better with treatment or recovering from a viral illness; but not every child with cancer can be cured. What Dr. MacDonell-Yilmaz so eloquently points out to us is that many supervising physicians are not yet skilled at supporting and communicating with a family whose child is potentially or actually facing death.  She brings us to the bedside of a dying child, and uses this as a “teachable moment.”    How can we educate residents and students about palliative care, and how can we support our trainees’ continued engagement with a family when their child’s prognosis is unknown or poor?
     She reviews relevant literature that underscores her points, not just that palliative care is important, but that trainees and their educators agree that this critical content area is not being adequately addressed during residency. Kudos to Dr. MacDonell-Yilmaz for tackling an emotionally difficult topic in a non-judgmental way that lifts the veil for all of us on a vital but incompletely addressed area of training. How many of us received support to talk to a family about their hopes for their dying child, or to the child him or herself about their dreams, wishes and sadnesses? It likely took one special fellow, or nurse or attending to bring us in from the cold, so to speak, and to guide us in this painful but profoundly necessary and rewarding conversation. Every trainee should be given this opportunity, and should be prepared to step up when the moment arrives.
     Articles for this Monthly Feature are written by trainees (additional non-trainee authors are welcome but cannot be the first author), and are selected by trainees, with Editor Dr. Bryan Sisk, senior pediatric resident, and Deputy Editor and pediatric intern Dr. Kristin Schwarz  running the show. Trainee authors who submit to this section receive detailed, thoughtful and collaborative review and mentoring from the editors, and are able to work through multiple revisions to bring their article to publication. Please think about encouraging or mentoring a trainee yourself to write for this section.  Check out our author guidelines here and send any questions or concerns our way on twitter and facebook.

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Thursday, July 31, 2014

Outcomes in Multifocal Neuroblastoma as Part of the Neurocristopathy Syndrome

Our Case Report Editor, Dr. Jeffery Malatack shares a case report we early released this month from our upcoming August issue.

By: Jeffrey Malatack, MD

Neuroblastoma rosettes
Photo by Maria Tsokos, US National Cancer Institute
Neuroblastoma has been appreciated for many years to have patient outcomes related to a variety of unique tumor features, including presence of stage 4S subtype, presence of opsoclonus/myoclonus and association with ROHHAD syndrome (Rapid onset Obesity with Hypothalamic dysfunction, Hypoventilation and Autonomic Dysregulation).

In each of these settings the prognosis of the malignancy is improved by what is believed to be an antitumor immune response. Neuroblastoma occurring as part of neurocristopathy syndrome has not previously been considered another special setting with an improved outcome. The neurocristopathy syndrome occurs due to a germline mutation of a neurogenesis regulator gene. The result is abnormal neural crest cell development resulting in congenital central hypoventilation syndrome, Hirschprung’s disease, and neuroblastoma which is often multifocal and disseminated in its presentation. Such widespread neuroblastoma has been considered highly aggressive and treated with either palliative intent or with very intense high dose chemotherapy.

Dr. Williams and colleagues (doi:10.1542/peds.2013-3340) writing from Australia report a patient with neurocristopathy syndrome who had multifocal neuroblastoma associated with the underlying germline mutation. The reported patient was treated conservatively with surgery and low dose chemotherapy. Following treatment he had extensive residual disease that has continued to mature from malignant to nonmalignant neural tumor despite no further treatment.

A literature review also provided in Dr. Williams’ report identified 26 similar patients presenting with multifocal neuroblastoma as part of the neurocristopathy syndrome. In all cases the neuroblastoma behaved in an indolent manner with no deaths from tumor reported where patients received appropriate treatment. These provocative findings suggest neurocristopathy-associated neuroblastoma should be treated conservatively, despite the aggressive appearance of the disease.

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