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Dedifferentiated Osteosarcoma of the Distal Ulna: A Case Report
Osteosarcoma is the most common malignant primary bone tumor that occurs most frequently in the second decade of life but rarely in patients over 40 years of age. The most common primary sites of osteosarcoma are the distal femur followed by proximal tibia and proximal humerus, and involvement of th...
Autores principales: | , , , , , , , , , , |
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Formato: | Online Artículo Texto |
Lenguaje: | English |
Publicado: |
S. Karger AG
2021
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Materias: | |
Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8460975/ https://www.ncbi.nlm.nih.gov/pubmed/34703440 http://dx.doi.org/10.1159/000518266 |
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author | Tsujisaka, Ryosuke Nakayama, Robert Sekita, Tetsuya Asano, Naofumi Kikuta, Kazutaka Oguro, Sota Takeuchi, Katsuhito Sasaki, Aya Okita, Hajime Nakamura, Masaya Matsumoto, Morio |
author_facet | Tsujisaka, Ryosuke Nakayama, Robert Sekita, Tetsuya Asano, Naofumi Kikuta, Kazutaka Oguro, Sota Takeuchi, Katsuhito Sasaki, Aya Okita, Hajime Nakamura, Masaya Matsumoto, Morio |
author_sort | Tsujisaka, Ryosuke |
collection | PubMed |
description | Osteosarcoma is the most common malignant primary bone tumor that occurs most frequently in the second decade of life but rarely in patients over 40 years of age. The most common primary sites of osteosarcoma are the distal femur followed by proximal tibia and proximal humerus, and involvement of the wrist is extremely rare. Moreover, dedifferentiated osteosarcoma is also a rare condition that progresses to high-grade osteosarcoma from low-grade osteosarcoma, usually central low-grade osteosarcoma or parosteal osteosarcoma that bears MDM2 and/or CDK4 gene amplifications. We herein report an extremely rare case of dedifferentiated osteosarcoma arising in the distal ulna of an adult over 40 years of age. The patient was a 46-year-old man with a 2-month history of pain in his left swollen wrist. The initial radiological findings suggested a benign bone tumor in the distal ulna, and the lesion was marginally excised at the nearby hospital. Although the pathological diagnosis at the nearby hospital suggested a benign cartilaginous tumor, the tumor recurred in an aggressive manner 8 months after the initial surgery. The patient was referred to our hospital, and an incisional biopsy showed a high-grade osteosarcoma. The primary tumor was retrospectively re-evaluated at our hospital and diagnosed as low-grade osteosarcoma. Since neoadjuvant chemotherapy failed to shrink the tumor, the patient had to undergo below the elbow amputation to cure the disease. Although the tumor was negative for MDM2 nor CDK4, the definitive diagnosis of dedifferentiated osteosarcoma was made according to the clinical course and the histological findings. Lung metastases were found 10 months after the amputation, which were successfully treated by neoadjuvant chemotherapy and surgery. The patient has been doing well with no evidence of disease for 1 year and 6 months. Surprisingly, the literature review revealed that many low-grade osteosarcomas of the distal ulna progressed to high-grade dedifferentiated osteosarcomas. One should bear in mind that the diagnosis and treatment for bone-forming tumors of the distal ulna should be made very carefully because, although rare, it is possible that the tumor may initially appear as a benign or low-grade malignant tumor and may progress to high-grade osteosarcoma. |
format | Online Article Text |
id | pubmed-8460975 |
institution | National Center for Biotechnology Information |
language | English |
publishDate | 2021 |
publisher | S. Karger AG |
record_format | MEDLINE/PubMed |
spelling | pubmed-84609752021-10-25 Dedifferentiated Osteosarcoma of the Distal Ulna: A Case Report Tsujisaka, Ryosuke Nakayama, Robert Sekita, Tetsuya Asano, Naofumi Kikuta, Kazutaka Oguro, Sota Takeuchi, Katsuhito Sasaki, Aya Okita, Hajime Nakamura, Masaya Matsumoto, Morio Case Rep Oncol Case Report Osteosarcoma is the most common malignant primary bone tumor that occurs most frequently in the second decade of life but rarely in patients over 40 years of age. The most common primary sites of osteosarcoma are the distal femur followed by proximal tibia and proximal humerus, and involvement of the wrist is extremely rare. Moreover, dedifferentiated osteosarcoma is also a rare condition that progresses to high-grade osteosarcoma from low-grade osteosarcoma, usually central low-grade osteosarcoma or parosteal osteosarcoma that bears MDM2 and/or CDK4 gene amplifications. We herein report an extremely rare case of dedifferentiated osteosarcoma arising in the distal ulna of an adult over 40 years of age. The patient was a 46-year-old man with a 2-month history of pain in his left swollen wrist. The initial radiological findings suggested a benign bone tumor in the distal ulna, and the lesion was marginally excised at the nearby hospital. Although the pathological diagnosis at the nearby hospital suggested a benign cartilaginous tumor, the tumor recurred in an aggressive manner 8 months after the initial surgery. The patient was referred to our hospital, and an incisional biopsy showed a high-grade osteosarcoma. The primary tumor was retrospectively re-evaluated at our hospital and diagnosed as low-grade osteosarcoma. Since neoadjuvant chemotherapy failed to shrink the tumor, the patient had to undergo below the elbow amputation to cure the disease. Although the tumor was negative for MDM2 nor CDK4, the definitive diagnosis of dedifferentiated osteosarcoma was made according to the clinical course and the histological findings. Lung metastases were found 10 months after the amputation, which were successfully treated by neoadjuvant chemotherapy and surgery. The patient has been doing well with no evidence of disease for 1 year and 6 months. Surprisingly, the literature review revealed that many low-grade osteosarcomas of the distal ulna progressed to high-grade dedifferentiated osteosarcomas. One should bear in mind that the diagnosis and treatment for bone-forming tumors of the distal ulna should be made very carefully because, although rare, it is possible that the tumor may initially appear as a benign or low-grade malignant tumor and may progress to high-grade osteosarcoma. S. Karger AG 2021-08-16 /pmc/articles/PMC8460975/ /pubmed/34703440 http://dx.doi.org/10.1159/000518266 Text en Copyright © 2021 by S. Karger AG, Basel https://creativecommons.org/licenses/by-nc/4.0/This article is licensed under the Creative Commons Attribution-NonCommercial-4.0 International License (CC BY-NC) (http://www.karger.com/Services/OpenAccessLicense). Usage and distribution for commercial purposes requires written permission. |
spellingShingle | Case Report Tsujisaka, Ryosuke Nakayama, Robert Sekita, Tetsuya Asano, Naofumi Kikuta, Kazutaka Oguro, Sota Takeuchi, Katsuhito Sasaki, Aya Okita, Hajime Nakamura, Masaya Matsumoto, Morio Dedifferentiated Osteosarcoma of the Distal Ulna: A Case Report |
title | Dedifferentiated Osteosarcoma of the Distal Ulna: A Case Report |
title_full | Dedifferentiated Osteosarcoma of the Distal Ulna: A Case Report |
title_fullStr | Dedifferentiated Osteosarcoma of the Distal Ulna: A Case Report |
title_full_unstemmed | Dedifferentiated Osteosarcoma of the Distal Ulna: A Case Report |
title_short | Dedifferentiated Osteosarcoma of the Distal Ulna: A Case Report |
title_sort | dedifferentiated osteosarcoma of the distal ulna: a case report |
topic | Case Report |
url | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8460975/ https://www.ncbi.nlm.nih.gov/pubmed/34703440 http://dx.doi.org/10.1159/000518266 |
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