Retroperitoneal Malignant Peripheral Nerve Sheath Research Articles

Giant Retroperitoneal Malignant Peripheral Nerve Sheath Tumor Treated with Multiorgan Resection: a Case Report and Review of the Literature.

Malignant peripheral nerve sheath tumors (MPNST) are rare. Although they originate from Schwann cells or pluripotent neural crest cells, they constitute less than 10% of all soft tissue sarcomas and more than 60% develop on the basis of neurofibromatosis. It is difficult to diagnose MPNST. Although it mostly occurs in the head and neck region or upper extremities, only 1% of cases are located in the retroperitoneal region. The main treatment is surgery, but survival results are not satisfactory even after surgery with R0 resection. They are not sensitive to chemotherapy and radiotherapy and tend to recur locally. The mass detected by imaging in a 57-year-old male patient who admitted to hospital with the complaint of abdominal pain was excised with clear surgical margins. The tumor was located in the left upper quadrant of the abdomen and seemed to invade the pancreas and left kidney in the computed tomography images. The patient had no history of neurofibromatosis or radiation. In this study, it was aimed to present our case diagnosed with retroperitoneal MPNST and treated with multiorgan resection, which is a rare entity, and to increase the awareness of clinicians about the diagnosis, treatment and prognosis of this rare tumor.

Retroperitoneal malignant peripheral nerve sheath tumor in a preschool child

Abstract Background Malignant peripheral nerve sheath tumors (MPNSTs) are aggressive sarcomas that occur in adulthood and are located mainly on the trunk and lower limbs, with a high association with neurofibromatosis type 1 (NF1). Case report A 34-months-old female infant without NF1 with a palpable abdominal tumor is described. The tumor corresponded to a retroperitoneal MPNST. The diagnostic approach and management are presented, highlighting the complications and sequelae during the evolution of the patient. Conclusions Despite their low incidence, MPNSTs are important because of their aggressiveness, and should be considered upon the detection of a tumor located at paravertebral level or limbs, especially in patients with NF1. The cornerstone of the treatment lies in a complete surgical resection due to the high rate of recurrence and limited therapeutic response to radiotherapy and chemotherapy. This case presents clinical manifestations and complications that can be expected with these tumors and their harmful behavior. The absence of NF1 does not exclude the diagnosis.

Role of ultrasonography in detection of renal artery pseudoaneurysm caused by retroperitoneal malignant peripheral nerve sheath tumor in a patient with neurofibromatosis type 1.

Malignant peripheral nerve sheath tumors (MPNSTs) are extremely uncommon neoplasms, predominantly seen within the deep soft tissues of the extremities, in close proximity to the nerve trunks. Retroperitoneal MPNSTs are exceedingly rare, usually seen in association with neurofibromatosis type 1 (NF-1), and often result from malignant degeneration of a plexiform neurofibroma. These tumors are highly malignant and the prognosis is worsened if they occur in association with NF-1. Metastasis is not uncommon, but local invasion by these tumors is rarely reported. Renal artery pseudoaneurysms are mostly iatrogenic and rarely result from invasion by retroperitoneal neoplasms. Ultrasonography is a valuable tool in early diagnosis of these neoplasms and vascular complications, particularly in emergency cases requiring immediate operative intervention. To the best of our knowledge, no case of renal artery pseudoaneurysm caused by retroperitoneal MPNST has been reported to date. We present a case of a middle-aged female with Von Recklinghausen disease, complaining of sudden onset of severe abdominal pain, where ultrasound accurately diagnosed renal artery pseudoaneurysm caused by a large retroperitoneal MPNST, and who was later treated by radical excision of the tumor and nephrectomy.

Retroperitoneal Malignant Peripheral Nerve Sheath Tumor Replacing an Absent Kidney in a Child

Malignant peripheral nerve sheath tumors (MPNSTs) are nonrhabdomyosarcoma soft tissue sarcomas with rare occurrence in children specially in the retroperitoneum. We describe a young child who presented with an abdominal mass. Both ultrasound and computed tomography revealed a large right-sided abdominal mass in the anatomic place of right kidney, while no kidney or ureter was observed at that side. He underwent surgical resection of the tumor with a primary impression of Wilms tumor. To the authors' knowledge, this is the first case of retroperitoneal malignant peripheral nerve sheath tumor and absent kidney. This case suggests the very rare probability of association of MPNSTs in children with genitourinary tract anomalies such as renal agenesis.

Nearly identical near-haploid karyotype in a peritoneal mesothelioma and a retroperitoneal malignant peripheral nerve sheath tumor

The presence of a near-haploid karyotype is a rare finding in human malignancies, most frequently occurring in acute leukemia. In solid tumors, a near-haploid karyotype has been reported in fewer than 40 cases. We report two nearly identical near-haploid karyotypes from two distinctly different tumor types. The first case is a biphasic malignant mesothelioma from a 53-year-old white woman forming a large retroperitoneal mass. Cytogenetic evaluation revealed a primary hyperdiploid cell population as well as near-haploid and hypertetraploid populations with an overall karyotype of 27,XX,i(5)(p10),+7,add(15)(p11.2),+dic(1;20)(p13;p13)[2]/54,idemx2[90]/101–108,idemx4[19]. The second case is a large pelvic mass from a 48-year-old man. Histologic examination identified a malignant peripheral nerve sheath tumor displaying a karyotype of 26,X,+i(5)(p10),+7,der(15)t(1;15)(q12;p12),+20[5]/52,idemx2[20]. Herein we discuss the potential relationship between these two disparate neoplasms with nearly identical near-haploid karyotypes and present a literature review.

Retroperitoneal Malignant Peripheral Nerve Sheath Tumour Associated with Vertebral Involvement and Spinal Cord Compression

Malignant peripheral nerve sheath tumour (MPNST) is usually seen in soft tissue. Intraosseous MPNST is rare, and so is secondary extension. Skeletal involvement usually involves the mandible and other long bones. Vertebral involvement is usually metastatic in nature. Here we report a case of a large retroperitoneal MPNST involving the L1 vertebra and producing spinal cord compression in a patient with neurofibromatosis (NF).

A Case of Fibroblastic Low-Grade Malignant Peripheral Nerve Sheath Tumor—A True Neurofibrosarcoma

The authors report a case of low-grade retroperitoneal malignant peripheral nerve sheath tumor (MPNST) showing Schwannian and fibroblastic differentiation in individual tumor cells. The tumor was detected in a 29-year-old male and posed diagnostic difficulty because of the unusual morphologic and immunophenotypic features. Morphologic examination of the H&E sections revealed a rather circumscribed, highly vascular, moderately cellular spindle cell tumor. The neoplastic cells were arranged in vague, short fascicles, distributed haphazardly amid hemangiopericytoma-like vascular channels, and showed occasional whorls. Myxoid stroma and keloid-like collagen bundles were frequently seen. There were satellite nodules outside the main tumor mass and low mitotic activity but no necrosis. The tumor cells stained strongly and diffusely for both S-100 protein and CD34. Electron microscopy revealed cells with processes and focal lamina, and prominent rough endoplasmic reticulum. Although the capacity of MPNST to exhibit divergent differentiation is well known, fibroblastic differentiation is generally poorly and inconsistently documented. The present case represents an unambiguous demonstration of the co-expression within individual tumor cells of Schwannian and fibroblastic differentiation in a low-grade MPNST. The literature on this subject is reviewed.

Preoperative embolization of a retroperitoneal malignant nervous sheath tumor

Malignant peripheral nerve sheath tumors are rare tumors compromising about 5% of all malignant soft tissue tumors. Treatment of these tumors is surgical but surgical resection is not always easy. We are presenting a low grade retroperitoneal malignant peripheral nerve sheath tumor in which we used selective arterial embolization before surgery and surgical resection became possible.