open access

Vol 80, No 3 (2012)
CASE REPORTS
Published online: 2012-05-07
Submitted: 2013-02-22
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Malignant peripheral nerve sheath tumor associated with clear cell renal cell carcinoma — case report

Beata Gryglicka, Irena Węgrzyn-Szkutnik, Janusz Milanowski, Sławomir Mandziuk, Ludmiła Grzybowska-Szatkowska, Dariusz Surdyka, Elżbieta Czekajska-Chehab, Małgorzata Zdunek
Pneumonol Alergol Pol 2012;80(3):269-274.

open access

Vol 80, No 3 (2012)
CASE REPORTS
Published online: 2012-05-07
Submitted: 2013-02-22

Abstract

Malignant peripheral nerve sheath tumor (MPNST) is a rare malignant counterpart to benign neurogenes tumors such as schwannomas and neurofibromas and account for approximately 5–10 % of all soft tissue sarcomas. This neoplasm is also referred to older designations as a malignant schwannoma, malignant neurilemmoma or neurogenic sarcoma. A patient was a woman of 59 years old with a diagnosed malignant neurilemmoma, treated since 1993. Operated several times and subjected to radiotherapy due to the local recurrence of the tumors located in the soft tissues of the back until 2002; Treated with chemotherapy (doxorubicin) and operated due to lung metastases. The therapy resulted in a total remission that lasted 12 months. In 2004 a new small tumor was diagnosed in the right lung, which had been followed up until 2006. The patient did not give permission to a second surgery, treated with ifosfamide. In 2006 she was operated for renal cell carcinoma of the left kidney. In 2009, due to a following progression of neurilemmoma and a worsening overall condition, she was subsequently treated with a combination of gemcitabine and docetaxel. The treatment resulted in a slight improvement, but was stopped due to complications (pancytopenia). In 2010 another progression of the disease occurred, which resulted in pleural metastases and osteolytic lesions in the vertebrae (Th6 and L2).

Abstract

Malignant peripheral nerve sheath tumor (MPNST) is a rare malignant counterpart to benign neurogenes tumors such as schwannomas and neurofibromas and account for approximately 5–10 % of all soft tissue sarcomas. This neoplasm is also referred to older designations as a malignant schwannoma, malignant neurilemmoma or neurogenic sarcoma. A patient was a woman of 59 years old with a diagnosed malignant neurilemmoma, treated since 1993. Operated several times and subjected to radiotherapy due to the local recurrence of the tumors located in the soft tissues of the back until 2002; Treated with chemotherapy (doxorubicin) and operated due to lung metastases. The therapy resulted in a total remission that lasted 12 months. In 2004 a new small tumor was diagnosed in the right lung, which had been followed up until 2006. The patient did not give permission to a second surgery, treated with ifosfamide. In 2006 she was operated for renal cell carcinoma of the left kidney. In 2009, due to a following progression of neurilemmoma and a worsening overall condition, she was subsequently treated with a combination of gemcitabine and docetaxel. The treatment resulted in a slight improvement, but was stopped due to complications (pancytopenia). In 2010 another progression of the disease occurred, which resulted in pleural metastases and osteolytic lesions in the vertebrae (Th6 and L2).
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Keywords

MPNST; neurilemmoma malignum; schwannoma malignum

About this article
Title

Malignant peripheral nerve sheath tumor associated with clear cell renal cell carcinoma — case report

Journal

Advances in Respiratory Medicine

Issue

Vol 80, No 3 (2012)

Pages

269-274

Published online

2012-05-07

Bibliographic record

Pneumonol Alergol Pol 2012;80(3):269-274.

Keywords

MPNST
neurilemmoma malignum
schwannoma malignum

Authors

Beata Gryglicka
Irena Węgrzyn-Szkutnik
Janusz Milanowski
Sławomir Mandziuk
Ludmiła Grzybowska-Szatkowska
Dariusz Surdyka
Elżbieta Czekajska-Chehab
Małgorzata Zdunek

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