Osteogenic Sarcoma in an Adolescent With Cystic Fibrosis: Successful Treatment Despite Significant Obstacles

Introduction: We describe the case of a 16-year old male with cystic fiborosis (CF) who presented with an osteosarcoma of his right distal tibia.Case Report: Treatment consisted of neoadjuvant chemotherapy of cisplatin, doxorubicin and high dose methotrexate followed by distal tibial resection and f...

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Main Authors: Thomas J. C. Ruffles, Ryan Black, Wayne Nicholls, Barbara Laing, Alan Isles
Format: Article
Language:English
Published: Frontiers Media S.A. 2018-09-01
Series:Frontiers in Pediatrics
Subjects:
Online Access:https://www.frontiersin.org/article/10.3389/fped.2018.00245/full
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spelling doaj-8d7b784d07f7488aa6bebc81db8c1f9d2020-11-24T20:51:43ZengFrontiers Media S.A.Frontiers in Pediatrics2296-23602018-09-01610.3389/fped.2018.00245412966Osteogenic Sarcoma in an Adolescent With Cystic Fibrosis: Successful Treatment Despite Significant ObstaclesThomas J. C. Ruffles0Thomas J. C. Ruffles1Ryan Black2Ryan Black3Wayne Nicholls4Barbara Laing5Alan Isles6Alan Isles7Department of Respiratory and Sleep Medicine, South Brisbane, QLD, AustraliaLady Cilento Children's Hospital and the Centre for Children's Health Research, South Brisbane, QLD, AustraliaDepartment of Respiratory and Sleep Medicine, South Brisbane, QLD, AustraliaDepartment of Physiotherapy, South Brisbane, QLD, AustraliaOncology Services Group, Lady Cilento Children's Hospital, South Brisbane, QLD, AustraliaWesley Medical Imaging, Brisbane, QLD, AustraliaDepartment of Respiratory and Sleep Medicine, South Brisbane, QLD, AustraliaLady Cilento Children's Hospital and the Centre for Children's Health Research, South Brisbane, QLD, AustraliaIntroduction: We describe the case of a 16-year old male with cystic fiborosis (CF) who presented with an osteosarcoma of his right distal tibia.Case Report: Treatment consisted of neoadjuvant chemotherapy of cisplatin, doxorubicin and high dose methotrexate followed by distal tibial resection and free fibula flap reconstruction and consolidation chemotherapy. Treatment was complicated by a pulmonary exacerbation, where Pseudomonas aeruginosa (PsA) and Staphylococcus aureus were grown on sputum culture which was treated with a 2-week course of intravenous piptazobactam and tobramycin. Mycobacterium intracellulare and Mycobacterium abscessus were also cultured following commencement of chemotherapy and successfully treated with a 6-month course of oral azithromycin, ethambutol, and moxifloxacin along with a 1-month course of inhaled amikacin. Pulmonary function improved during his treatment from baseline FEV1 of 3.8 l (93.9%) to 4.15 l (102.3% predicted) whilst nutritional status remained stable.Discussion: The combination of CF and osteosarcoma is rare with only one previous case reported (1). Our case is instructive as the patient faced the challenge of chronic PsA and the first reported culturing and successful treatment of non-tuberculous mycobacterium (NTM) during chemotherapy. Fatal outcomes have been reported previously for CF patients during immunosuppression (2). In concordance with our findings, a recent report noted an improvement in respiratory function in a child treated for leukemia (3). The anti-inflammatory nature of some chemotherapy agents could be responsible for the observed clinical improvement in CF with low dose methotrexate having been shown to increase FEV1 in adolescents with advanced CF (4). Whilst doxorubicin could improve pulmonary outcomes through increased total cellular CFTR protein expression and CFTR associated chloride secretion (5). It is hypothesized that the improved pulmonary function in patients with CF who require chemotherapy could be due to increased production of Multi-Drug Resistance Proteins (MDR) and Multi-Drug Resistant Associated Proteins (MRP) that may complement the depleted CFTR protein (6).Concluding Remarks: We report the well-tolerated management of osteosarcoma in a patient with CF including the first reported identification and eradication of NTM during chemotherapy. The observed positive pulmonary outcome following chemotherapy highlights several potential cellular mechanisms that deserve to be explored.https://www.frontiersin.org/article/10.3389/fped.2018.00245/fullcystic fibrosisosteogenic sarcomatibiachemotherapynon-tuberculous mycobacteria
collection DOAJ
language English
format Article
sources DOAJ
author Thomas J. C. Ruffles
Thomas J. C. Ruffles
Ryan Black
Ryan Black
Wayne Nicholls
Barbara Laing
Alan Isles
Alan Isles
spellingShingle Thomas J. C. Ruffles
Thomas J. C. Ruffles
Ryan Black
Ryan Black
Wayne Nicholls
Barbara Laing
Alan Isles
Alan Isles
Osteogenic Sarcoma in an Adolescent With Cystic Fibrosis: Successful Treatment Despite Significant Obstacles
Frontiers in Pediatrics
cystic fibrosis
osteogenic sarcoma
tibia
chemotherapy
non-tuberculous mycobacteria
author_facet Thomas J. C. Ruffles
Thomas J. C. Ruffles
Ryan Black
Ryan Black
Wayne Nicholls
Barbara Laing
Alan Isles
Alan Isles
author_sort Thomas J. C. Ruffles
title Osteogenic Sarcoma in an Adolescent With Cystic Fibrosis: Successful Treatment Despite Significant Obstacles
title_short Osteogenic Sarcoma in an Adolescent With Cystic Fibrosis: Successful Treatment Despite Significant Obstacles
title_full Osteogenic Sarcoma in an Adolescent With Cystic Fibrosis: Successful Treatment Despite Significant Obstacles
title_fullStr Osteogenic Sarcoma in an Adolescent With Cystic Fibrosis: Successful Treatment Despite Significant Obstacles
title_full_unstemmed Osteogenic Sarcoma in an Adolescent With Cystic Fibrosis: Successful Treatment Despite Significant Obstacles
title_sort osteogenic sarcoma in an adolescent with cystic fibrosis: successful treatment despite significant obstacles
publisher Frontiers Media S.A.
series Frontiers in Pediatrics
issn 2296-2360
publishDate 2018-09-01
description Introduction: We describe the case of a 16-year old male with cystic fiborosis (CF) who presented with an osteosarcoma of his right distal tibia.Case Report: Treatment consisted of neoadjuvant chemotherapy of cisplatin, doxorubicin and high dose methotrexate followed by distal tibial resection and free fibula flap reconstruction and consolidation chemotherapy. Treatment was complicated by a pulmonary exacerbation, where Pseudomonas aeruginosa (PsA) and Staphylococcus aureus were grown on sputum culture which was treated with a 2-week course of intravenous piptazobactam and tobramycin. Mycobacterium intracellulare and Mycobacterium abscessus were also cultured following commencement of chemotherapy and successfully treated with a 6-month course of oral azithromycin, ethambutol, and moxifloxacin along with a 1-month course of inhaled amikacin. Pulmonary function improved during his treatment from baseline FEV1 of 3.8 l (93.9%) to 4.15 l (102.3% predicted) whilst nutritional status remained stable.Discussion: The combination of CF and osteosarcoma is rare with only one previous case reported (1). Our case is instructive as the patient faced the challenge of chronic PsA and the first reported culturing and successful treatment of non-tuberculous mycobacterium (NTM) during chemotherapy. Fatal outcomes have been reported previously for CF patients during immunosuppression (2). In concordance with our findings, a recent report noted an improvement in respiratory function in a child treated for leukemia (3). The anti-inflammatory nature of some chemotherapy agents could be responsible for the observed clinical improvement in CF with low dose methotrexate having been shown to increase FEV1 in adolescents with advanced CF (4). Whilst doxorubicin could improve pulmonary outcomes through increased total cellular CFTR protein expression and CFTR associated chloride secretion (5). It is hypothesized that the improved pulmonary function in patients with CF who require chemotherapy could be due to increased production of Multi-Drug Resistance Proteins (MDR) and Multi-Drug Resistant Associated Proteins (MRP) that may complement the depleted CFTR protein (6).Concluding Remarks: We report the well-tolerated management of osteosarcoma in a patient with CF including the first reported identification and eradication of NTM during chemotherapy. The observed positive pulmonary outcome following chemotherapy highlights several potential cellular mechanisms that deserve to be explored.
topic cystic fibrosis
osteogenic sarcoma
tibia
chemotherapy
non-tuberculous mycobacteria
url https://www.frontiersin.org/article/10.3389/fped.2018.00245/full
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