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

Toru Hirozane, Robert Nakayama, Sayaka Yamaguchi, Tomoaki Mori, Naofumi Asano, Keisuke Asakura, Kazutaka Kikuta, Miho Kawaida, Aya Sasaki, Hajime Okita, Seishi Nakatsuka, Tsutomu Ito

研究成果: Article査読

抄録

Background: Malignant 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 presentation: A 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. Conclusions: In cases of a previous history of microscopic venous invasion, recurrence can occur as a massive tumor thrombus that extends to the great vessels.

本文言語English
論文番号8
ジャーナルWorld Journal of Surgical Oncology
20
1
DOI
出版ステータスPublished - 2022 12月

ASJC Scopus subject areas

  • 外科
  • 腫瘍学

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