Malignant Peripheral Nerve Sheath Tumor in the Nasal Cavity of a Neonate: A Case Report

Malignant peripheral nerve sheath tumor (MPNST) is a rare tumor that can develop on the lining of nerves and within the network of nerve fibers in different organs, and it is commonly found in the head and neck, limbs, and trunk. These tumors can occur in patients of any age. They most commonly occur in adults aged 20 to 50 years; however, fewer cases of this tumor in children have been reported. To date, no neonatal case of MPNST in the nasal cavity has been reported. Here, we report the case of a 4-day-old female newborn who presented with a nasal mass that re-enlarged after surgery and was diagnosed as MPNST of the nasal cavity on the basis of pathological results. This is the first report of MPNST in the nasal cavity of a neonate. Differential diagnosis and treatment of nasal masses have been proposed in the related literature.

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Spinal Cord Metastasis of a Non-neurofibromatosis Type-1 Malignant Peripheral Nerve Sheath Tumor: An Unusual Manifestation of a Rare Tumor

Journal of Neuro-Oncology ◽

10.1007/s11060-004-4596-4 ◽

2005 ◽

Vol 74 (2) ◽

pp. 183-185 ◽

Cited By ~ 7

Author(s):

William S. Baek ◽

Peter Pytel ◽

Samir D. Undevia ◽

Helene Rubeiz

Keyword(s):

Spinal Cord ◽

Peripheral Nerve ◽

Neurofibromatosis Type 1 ◽

Neurofibromatosis Type ◽

Nerve Sheath Tumor ◽

Rare Tumor ◽

Unusual Manifestation ◽

Peripheral Nerve Sheath Tumor ◽

Nerve Sheath

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Inaccurate summary of peripheral nerve sheath tumors in extradigital sites. Comment on “Hybrid peripheral nerve sheath tumor of the nasal cavity showing schwannomatous, neurofibromatus, and perineuriomatous areas. Med Mol Morphol (2010) 43:82-85”

Medical Molecular Morphology ◽

10.1007/s00795-010-0526-z ◽

2010 ◽

Vol 43 (4) ◽

pp. 253-253 ◽

Cited By ~ 1

Author(s):

Teresa Pusiol ◽

Maria Grazia Zorzi ◽

Doriana Morichetti ◽

Francesco Piscioli

Keyword(s):

Peripheral Nerve ◽

Nasal Cavity ◽

Nerve Sheath Tumor ◽

Peripheral Nerve Sheath Tumor ◽

Nerve Sheath Tumors ◽

Peripheral Nerve Sheath Tumors ◽

Nerve Sheath

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Hybrid peripheral nerve sheath tumor of the nasal cavity showing schwannomatous, neurofibromatous, and perineuriomatous areas

Medical Molecular Morphology ◽

10.1007/s00795-008-0418-7 ◽

2010 ◽

Vol 43 (2) ◽

pp. 82-85 ◽

Cited By ~ 22

Author(s):

Naoto Kuroda ◽

Dmitry V. Kazakov ◽

Ondrej Hes ◽

Michal Michal ◽

Masakazu Goda ◽

...

Keyword(s):

Peripheral Nerve ◽

Nasal Cavity ◽

Nerve Sheath Tumor ◽

Peripheral Nerve Sheath Tumor ◽

Nerve Sheath

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Epithelioid malignant peripheral nerve sheath tumor arising in schwannoma

Rare Tumors ◽

10.1177/2036361320950862 ◽

2020 ◽

Vol 12 ◽

pp. 203636132095086 ◽

Cited By ~ 1

Author(s):

Nicholas Heatley ◽

Eva Kolson Kokohaare ◽

Dirk C Strauss ◽

Magnus Hallin ◽

Robin L Jones ◽

...

Keyword(s):

Differential Diagnosis ◽

Peripheral Nerve ◽

Rare Variant ◽

Nerve Sheath Tumor ◽

Peripheral Nerve Sheath Tumor ◽

Nerve Sheath ◽

Pathologic Features

Epithelioid malignant peripheral nerve sheath tumor (EMPNST, malignant epithelioid schwannoma) is a rare variant of malignant peripheral nerve sheath tumor that has morphologic and immunophenotypic overlap with a variety of epithelioid neoplasms. Because of its rarity it may be potentially underrecognized. We describe a case arising in the subcutis of the thigh in a 25 year-old female, and discuss the pathologic features and differential diagnosis.

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