Recurrent malignant peripheral nerve sheath tumor presenting as an asymptomatic intravenous thrombus extending to the heart: a case report

World Journal of Surgical Oncology

Tsutomu Ito¹  Sayaka Yamaguchi²  Naofumi Asano²  Tomoaki Mori²  Toru Hirozane²  Robert Nakayama²  Kazutaka Kikuta³  Hajime Okita⁴  Miho Kawaida⁴  Aya Sasaki⁵  Seishi Nakatsuka⁶  Keisuke Asakura⁷ 

[1] Department of Cardiovascular Surgery, Keio University School of Medicine, Tokyo, Japan;Department of Orthopedic Surgery, Keio University School of Medicine, 35 Shinanomachi, Shinjuku-ku, 160-8582, Tokyo, Japan;Department of Orthopedic Surgery, Keio University School of Medicine, 35 Shinanomachi, Shinjuku-ku, 160-8582, Tokyo, Japan;Division of Musculoskeletal Oncology and Orthopedic Surgery, Tochigi Cancer Center, Tochigi, Japan;Department of Pathology, Keio University School of Medicine, Tokyo, Japan;Department of Pathology, Tokyo Dental College Ichikawa General Hospital, Chiba, Japan;Department of Radiology, Keio University School of Medicine, Tokyo, Japan;Division of Thoracic Surgery, Department of Surgery, Keio University School of Medicine, Tokyo, Japan;

关键词: MPNST;    Tumor thrombus;    Venous invasion;   

DOI  :  10.1186/s12957-021-02473-2

来源: Springer

【 摘 要 】

BackgroundMalignant peripheral nerve sheath tumor (MPNST) is a rare soft tissue sarcoma mainly treated via surgical resection. Herein, we report a case of MPNST wherein a massive tumor thrombus extended to the major veins and heart.Case presentationA 39-year-old female with a history of neurofibromatosis type 1 developed MPNST from the right radial nerve. In addition to adjuvant chemotherapy, she underwent wide tumor resection and concomitant radial nerve resection, followed by postoperative radiotherapy. Histological evaluation revealed marked venous invasion. The 2-year follow-up CT revealed an asymptomatic recurrent tumor thrombus extending from the right subclavian vein to the heart. An urgent life-saving operation was performed to ligate the base of the right subclavian vein and remove the entire intravenous thrombus that extended to the right ventricle. The remaining tumor in the right subclavian vein increased in size 3 months after thrombectomy. After confirming the absence of any metastatic lesions, the patient underwent extended forequarter amputation to achieve surgical remission. One year later, a new metastasis to the right diaphragm was safely resected. The patient remains alive without any evidence of disease 2 years after the extended forequarter amputation.ConclusionsIn cases of a previous history of microscopic venous invasion, recurrence can occur as a massive tumor thrombus that extends to the great vessels.

【 授权许可】

CC BY