Abstract

In cases of peripheral nerve sheath tumors, current guidelines do not recommend routine abdominal imaging to stage the disease, as extra-pulmonary metastasis is considered rare. We report a case of large peripheral nerve sheath tumor in a 40 year-old-female with neurofibromatosis type 1 who had isolated adrenal metastasis. She underwent primary and adrenal metastasis resection.

Highlights

  • A similar recommendation is made by the European Society of Medical Oncology (ESMO)/ European Sarcoma Network Working Group[7]

  • We report a case of a malignant peripheral nerve sheath tumor of the thigh, without evidence of concomitant pulmonary metastases, which was found to have metastasized to the adrenal gland

  • The above case describes a patient with a malignant peripheral nerve sheath tumor (MPNST) of the thigh with pathologically confirmed metastases to the adrenal gland, yet without evidence of pulmonary metastases

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Summary

Introduction

Soft tissue sarcomas of the extremity are a rare, comprising less than 1% of all malignancies[1]. We report a case of a malignant peripheral nerve sheath tumor of the thigh, without evidence of concomitant pulmonary metastases, which was found to have metastasized to the adrenal gland. This case raises the question if routine abdominal imaging should be performed along with pulmonary imaging in metastatic peripheral nerve sheath tumors. The patient underwent a laparoscopic adrenalectomy, with gross pathological exams revealing two tumor nodules and with a histological exam revealing an intermixed “collision” tumor involving pheochromocytoma and sarcoma, consistent with metastatic peripheral nerve sheath tumor. The patient refused radiation therapy and did not follow up with oncology

Discussion
Conclusions
Kane JM 3rd
Findings
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