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Osteoblastic Osteosarcoma Arising beneath an Osteochondroma in an 11-Year-Old Male with Multiple Hereditary Exostoses
INTRODUCTION: Multiple hereditary exostoses (MHE) is a rare autosomal dominant disorder characterized by the presence of multiple skeletal deformities. They are painless slow-growing lesions. Malignant transformation tends to occur later in adulthood and has only been seen in 1–5% of patients. OBJEC...
Autores principales: | , , , , , , |
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Formato: | Online Artículo Texto |
Lenguaje: | English |
Publicado: |
Hindawi
2018
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Materias: | |
Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6079448/ https://www.ncbi.nlm.nih.gov/pubmed/30123601 http://dx.doi.org/10.1155/2018/8280415 |
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author | Bukara, Emmanuel Buteera, Alex M. Karakire, Robert Manirakiza, Felix Muhumuza, Samuel Rudakemwa, Emmanuel Kyokunda, Lynnette |
author_facet | Bukara, Emmanuel Buteera, Alex M. Karakire, Robert Manirakiza, Felix Muhumuza, Samuel Rudakemwa, Emmanuel Kyokunda, Lynnette |
author_sort | Bukara, Emmanuel |
collection | PubMed |
description | INTRODUCTION: Multiple hereditary exostoses (MHE) is a rare autosomal dominant disorder characterized by the presence of multiple skeletal deformities. They are painless slow-growing lesions. Malignant transformation tends to occur later in adulthood and has only been seen in 1–5% of patients. OBJECTIVE: We describe the clinical, radiological, and pathological characteristics of a child with MHE who developed osteoblastic osteosarcoma beneath an osteochondroma. CASE PRESENTATION: An 11-year-old male Rwandan presented to our hospital with a two-week history of a dull persistent pain in his left distal femur and loss of weight and appetite. There was no relief with pain killers. He was a known case of multiple hereditary exostoses diagnosed at age 3. He began experiencing mild symptoms 6 months prior to admission which worsened in the last two weeks prior to his admission. On examination, he had multiple palpable bony swellings bilaterally on the proximal humeri and distal femurs. X-rays showed multiple exostoses and MRI showed a lesion with heterogeneous signal intensities that suggested malignant transformation. At surgery, a necrotic lesion beneath the exostosis was excised and sent for histopathological analysis which confirmed osteochondroma with an osteoblastic osteosarcoma in the marrow cavity. Chemotherapy and limb-salvaging surgery were done and he has recovered well. CONCLUSION: Osteosarcomas arising at sites of MHE have not been previously reported in Africa. These tumors rarely undergo malignant transformation. |
format | Online Article Text |
id | pubmed-6079448 |
institution | National Center for Biotechnology Information |
language | English |
publishDate | 2018 |
publisher | Hindawi |
record_format | MEDLINE/PubMed |
spelling | pubmed-60794482018-08-19 Osteoblastic Osteosarcoma Arising beneath an Osteochondroma in an 11-Year-Old Male with Multiple Hereditary Exostoses Bukara, Emmanuel Buteera, Alex M. Karakire, Robert Manirakiza, Felix Muhumuza, Samuel Rudakemwa, Emmanuel Kyokunda, Lynnette Case Rep Orthop Case Report INTRODUCTION: Multiple hereditary exostoses (MHE) is a rare autosomal dominant disorder characterized by the presence of multiple skeletal deformities. They are painless slow-growing lesions. Malignant transformation tends to occur later in adulthood and has only been seen in 1–5% of patients. OBJECTIVE: We describe the clinical, radiological, and pathological characteristics of a child with MHE who developed osteoblastic osteosarcoma beneath an osteochondroma. CASE PRESENTATION: An 11-year-old male Rwandan presented to our hospital with a two-week history of a dull persistent pain in his left distal femur and loss of weight and appetite. There was no relief with pain killers. He was a known case of multiple hereditary exostoses diagnosed at age 3. He began experiencing mild symptoms 6 months prior to admission which worsened in the last two weeks prior to his admission. On examination, he had multiple palpable bony swellings bilaterally on the proximal humeri and distal femurs. X-rays showed multiple exostoses and MRI showed a lesion with heterogeneous signal intensities that suggested malignant transformation. At surgery, a necrotic lesion beneath the exostosis was excised and sent for histopathological analysis which confirmed osteochondroma with an osteoblastic osteosarcoma in the marrow cavity. Chemotherapy and limb-salvaging surgery were done and he has recovered well. CONCLUSION: Osteosarcomas arising at sites of MHE have not been previously reported in Africa. These tumors rarely undergo malignant transformation. Hindawi 2018-07-12 /pmc/articles/PMC6079448/ /pubmed/30123601 http://dx.doi.org/10.1155/2018/8280415 Text en Copyright © 2018 Emmanuel Bukara et al. http://creativecommons.org/licenses/by/4.0/ This is an open access article distributed under the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited. |
spellingShingle | Case Report Bukara, Emmanuel Buteera, Alex M. Karakire, Robert Manirakiza, Felix Muhumuza, Samuel Rudakemwa, Emmanuel Kyokunda, Lynnette Osteoblastic Osteosarcoma Arising beneath an Osteochondroma in an 11-Year-Old Male with Multiple Hereditary Exostoses |
title | Osteoblastic Osteosarcoma Arising beneath an Osteochondroma in an 11-Year-Old Male with Multiple Hereditary Exostoses |
title_full | Osteoblastic Osteosarcoma Arising beneath an Osteochondroma in an 11-Year-Old Male with Multiple Hereditary Exostoses |
title_fullStr | Osteoblastic Osteosarcoma Arising beneath an Osteochondroma in an 11-Year-Old Male with Multiple Hereditary Exostoses |
title_full_unstemmed | Osteoblastic Osteosarcoma Arising beneath an Osteochondroma in an 11-Year-Old Male with Multiple Hereditary Exostoses |
title_short | Osteoblastic Osteosarcoma Arising beneath an Osteochondroma in an 11-Year-Old Male with Multiple Hereditary Exostoses |
title_sort | osteoblastic osteosarcoma arising beneath an osteochondroma in an 11-year-old male with multiple hereditary exostoses |
topic | Case Report |
url | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6079448/ https://www.ncbi.nlm.nih.gov/pubmed/30123601 http://dx.doi.org/10.1155/2018/8280415 |
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