Personal and Familial Malignancy History in Patients with Malignant Peripheral Nerve Sheath Tumors with a Focus on Sporadic Tumors

Practical Approach to Histological Diagnosis of Peripheral Nerve Sheath Tumors: An Update

“…MPNSTs represent about 5% of all sarcomas and may arise from a peripheral nerve or a pre-existing neurofibroma; in about 50% of cases, these tumors arise in the context of NF1, while in the remaining cases they appear to be sporadic (40% of cases) or associated with a previous history of radiation [ 1 , 4 , 6 , 73 , 74 , 75 ]. A significant number of the sporadic tumors arise in the deep soft tissues without any anatomic evidence of association with a peripheral nerve or pre-existing neurofibroma (soft tissue MPNST) [ 73 , 74 , 75 ]. The sporadic tumors usually occur in patients with an age ranging from 30 to 50 years, while the NF1-asociated tumors can manifest in younger patients, including in childhood [ 73 , 74 , 75 ].…”

“…A significant number of the sporadic tumors arise in the deep soft tissues without any anatomic evidence of association with a peripheral nerve or pre-existing neurofibroma (soft tissue MPNST) [ 73 , 74 , 75 ]. The sporadic tumors usually occur in patients with an age ranging from 30 to 50 years, while the NF1-asociated tumors can manifest in younger patients, including in childhood [ 73 , 74 , 75 ]. The most common sites are the extremities, trunk, and head/neck region; among the major nerves involved, the sciatic nerve is the most common one, followed by the brachial and sacral plexuses and the paraspinal nerves.…”

“…The most common subtype of MPNSTs is the classic type, basically a spindle cell sarcoma that can be divided into low- and high-grades based on the following morphological features: (i) the loss of neurofibroma architecture; (ii) hypercellularity; (iii) cytological atypia; (iv) mitotic activity; and (v) tumor necrosis [ 1 , 73 , 74 , 75 ]. Low-grade MPNSTs are uncommon (10% of all MPNSTs) and usually arise from a pre-existing NF1-associated neurofibroma ( Figure 21 ) [ 1 , 76 ].…”

MPNST of the abdominal wall in a patient with lynch syndrome: A case report of a rare presentation and unique association

“…Sporadic MPNSTs usually occur in patients with an age ranging from 30 to 50 years. When associated with neurofibromatosis type 1 (NF1), these tumors can manifest in younger patients [ 6 , 7 ]. Frequently, MPNST causes neurologic deficits in the distribution of the involved nerves due to impingement or mass effect [ 3 ].…”

“…On the other hand, some studies have investigated the link between MPNST and familial carcinologic history [ 7 , 20 ]. When comparing the family history of a group of sporadic MPNST and a group of NF1-associated MPNST, patients with sporadic MPNST had a significantly higher rate of positive family history of malignancy than patients from the second group.…”