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A malignant peripheral nerve sheath tumor (MPNST) is a form of cancer of the connective tissue surrounding peripheral nerves. Given its origin and behavior it is classified as a sarcoma. About half the cases are diagnosed in people with neurofibromatosis; the lifetime risk for an MPNST in patients with neurofibromatosis type 1 is 8–13%. [2]
New and more precisely defined entities include malignant melanotic nerve sheath tumor (formerly known as melanotic schwannoma) and hybrid nerve sheath tumors. [4] [5] The majority of peripheral nerve tumors are benign tumors of the nerve sheath (usually schwannomas); on rare occasions, they are metastatic tumors or originate from the nerve cells.
Each nerve sheath tumor type has a few different associated features on MRI imaging. Neurofibromas and malignant peripheral nerve sheath tumors can be difficult to distinguish from each other and may require additional testing, including PET scans (18 FDG-PET). [1] Image-guided needle biopsies may be performed if there is concern for malignancy ...
A nervous system tumor is a tumor that arises within the nervous system, either the central nervous system (CNS) or the peripheral nervous system (PNS). [1] [2] Nervous system primary tumors include various types of brain tumor and spinal tumors, such as gliomas, and meningiomas (of the CNS), and schwannomas (of the PNS) and can be either benign or malignant.
Malignant melanotic nerve sheath tumor (previously known as melanotic schwannoma) is a rare aggressive peripheral nerve sheath tumor that typically develops in conjunction with spinal or visceral autonomic nerves, consisting uniformly of Schwann cells displaying melanocytic differentiation.
A subcategory of malignant peripheral nerve sheath tumors in particular, malignant triton tumors, have an enhanced expression of rhabdomyoblast differentiation. [5] Its name was coined due to the tumor's ability to promote the growth of limbs on the backs' of triton salamanders in animal transplant models. [5]
It is classified as a malignant peripheral nerve sheath tumor with rhabdomyosarcomatous differentiation. [citation needed] The unusual name "triton" was first used in reference to observation of supernumerary limbs containing bone and muscle growing on the backs of triton salamanders after the implantation of sciatic nerve tissue. [1]
The only reliable way to determine whether a soft-tissue tumor is benign or malignant is through a biopsy. The two methods for acquisition of tumor tissue for cytopathological analysis are: Needle aspiration biopsy, via needle; Surgically, via an incision made into the tumor; A pathologist examines the tissue under a microscope.
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