Sclerosing Rhabdomyosarcoma of a Chest Wall in an Adult: A Case Report and Review of the Literature
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- Mikubo Masashi
- Department of Thoracic Surgery, Pathology, Mitsui Memorial Hospital, Chiyoda-ku, Tokyo, Japan
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- Ikeda Shingo
- Department of Thoracic Surgery, Pathology, Mitsui Memorial Hospital, Chiyoda-ku, Tokyo, Japan
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- Hoshino Tatsuhiro
- Department of Thoracic Surgery, Pathology, Mitsui Memorial Hospital, Chiyoda-ku, Tokyo, Japan
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- Yokota Toshiya
- Department of Thoracic Surgery, Pathology, Mitsui Memorial Hospital, Chiyoda-ku, Tokyo, Japan
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- Fujii Akiko
- Department of Thoracic Surgery, Pathology, Mitsui Memorial Hospital, Chiyoda-ku, Tokyo, Japan
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- Mori Masaya
- Department of Thoracic Surgery, Pathology, Mitsui Memorial Hospital, Chiyoda-ku, Tokyo, Japan
抄録
Sclerosing rhabdomyosarcoma (SRMS) is a newly recognized and rare variant of rhabdomyosarcoma. This soft tissue tumor has not yet been reported as a thoracic lesion. We report a case of a 26-year-old woman who presented with a large chest wall tumor. The tumor originated from the right anterior chest wall and protruded into the intra- and extrapleural cavity. A transcutaneous needle biopsy revealed spindle cells in an abundant hyalinized and fibrous stroma. Although the tumor was considered as a malignant soft-tissue neoplasm, a definitive diagnosis could not be established. A wide excision of the chest wall including the second, third and fourth rib and a part of sternum was performed. Histologically, cytoplasmic cross-striations were found in a portion of the tumor cells. The tumor cells were positive for muscle markers, and the tumor was diagnosed as rhabdomyosarcoma consistent with a sclerosing type of rhabdomyosarcoma. Eighteen months after the complete resection, the patient has pleural disseminations but is alive and undergoing chemotherapy. This case highlights the histologic features of a rare form of rhabdomyosarcoma, and emphasizes the importance of awareness of its existence and the utility of skeletal muscle markers in distinguishing sclerosing rhabdomyosarcoma from its mimics.
収録刊行物
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- Annals of Thoracic and Cardiovascular Surgery
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Annals of Thoracic and Cardiovascular Surgery 20 (Supplement), 642-645, 2014
Annals of Thoracic and Cardiovascular Surgery 編集委員会