Superficial malignant peripheral nerve sheath tumor from recurrent neurofibroma in the abdominal wall of a patient without neurofibromatosis type 1 / 영남의대학술지

Malignant peripheral nerve sheath tumor (MPNST) is rare, accounting for 5-10% of all soft tissue sarcomas. MPNST is characteristically aggressive and has a poor prognosis. Fifty percent of patients with MPNST have neurofibromatosis type 1 (NF1). NF-associated MPNST occurs more often at younger ages than sporadic MPNST, but the survival difference is controversial. Superficial MPNST from a recurrent neurofibroma is extremely rare and only a limited number of cases have been reported in the literature. Herein, we report an unusual case of superficial MPNST from a recurrent neurofibroma in a patient without NF1.

Malignant Peripheral Nerve Sheath Tumour– A Rare Malignancy in Mandible

Malignant peripheral nerve sheath tumour (MPNST) is biologically an aggressive tumour that is usually found in the extremities, trunk and infrequently found in the head and neck area, particularly in the jaws, arising from the cells associated with nerve sheath. MPNST of the mandible is a very uncommon tumour that develops either from a pre-existing neurofibroma orde novo. Because of the greater variability from one case to the next in overall appearance both clinically and histologically, a case of MPNST of the mandible in 25-year-old female patient is reported.

Evaluation of performance of various histological grading systems of soft tissue sarcomas and the prognosis (metastatic risk and survival rate).

Background: Histologic grade represents the most important prognostic factor for all soft tissue sarcomas and it is strongly associated with the advent of metastasis and patients survival. The main objective of this study is to test individual grading system with metastatic risk and patients survival rate (prognosis). Methods: Soft tissue sarcomas (250) were graded by FNCLCC, NCI & Mhyre Jensens grading system. Special stains & immunohistochemistry were employed whenever necessary. Results: FNCLCC system shows Grade 1 = 50 (20%), Grade 2 = 75 (30%) & Grade 3 = 125 (50%). NCI (Costa et al.) showed Grade 1 = 70 (28%) Grade 2 = 85 (34%) & Grade 3 = 95 (38%). Myhre Jensen showed Grade 1 = 84 (33%), Grade 98 (39%) & Grade 3 = 68 (27%). Undoubtedly, FNCLCC system is the best of all grading systems which is very well supported by statistical analysis in this study. Conclusions: FNCLCC grading system of soft tissue sarcomas is the best documented and tested system. This present study strongly recommends FNCLCC grading system of soft tissue sarcomas to be internationally accepted because the grading system has well defined criteria & so least possible chances of interobserver variability. The present study & few other previous studies highly recommend the mandatory use of FNCLCC grading system in histopathology report format.

Malignant Peripheral Nerve Sheath Tumors of the Bilateral Adrenal Glands: Are They Metachronous Primary Tumors: A Case Report

Malignant peripheral nerve sheath tumors (MPNSTs) have rarely been reported to occur in the adrenal gland and all of the reported cases were associated with neurofibromatosis, pheochromocytoma or ganglioneuroma. We present here a case of MPNST in the bilateral adrenal glands without any history of neurofibromatosis or combined tumor. Histologic examination showed the tumor cells had a spindle to ovoid shape, they were arranged in sweeping fascicles and there were frequent mitotic figures. The immunohistochemical and ultrastructural features of the tumor are also presented. To the best of our knowledge, this is the first report in the English medical literature about MPNSTs in the bilateral adrenal glands without any history of neurofibromatosis or combined tumor.

Malignant Peripheral Nerve Sheath Tumor of Scalp

PURPOSE: Malignant peripheral nerve sheath tumors (MPNSTs) are rare neoplasms, usually arising from somatic soft tissues or peripheral nerves. Primary MPNST of the scalp is extremely rare. The case is being reported for its rarity. METHODS: A 53-year-old female was presented with a scalp mass on vertex area. The tumor was localized in scalp skin and did not invade underlying periosteum or skull and treated with complete surgical excision followed by adjuvant chemotherapy and radiotherapy. RESULTS: Histologically, the tumor showed malignant spindle cells with focal S-100 positivity on immunohistochemistry and a diagnosis of MPNST was made. CONCLUSION: Authors experienced a rare case of primary scalp MPNST and report the case.

Intraosseous Nerve Sheath Tumors in the Jaws

Although the head and neck region is recognized as the most common location for peripheral nerve sheath tumors, central involvement, particularly in the jaw bones, is quite unusual. Neurofibroma is one of the most common nerve sheath tumors occurring in the soft tissue and generally appears in neurofibromatosis 1 (NF1 or von Recklinghausen's disease). Malignant peripheral nerve sheath tumors (MPNSTs) are uncommon sarcomas that almost always arise in the soft tissue. Here, we report four cases of intraosseous peripheral nerve sheath tumors occurring in the jaw bones and compare the clinical, radiologic, and pathologic findings in order to make a differential diagnosis.

A case of malignant peripheral nerve sheath tumor of pelvic cavity / 대한산부인과학회지

A 75-year-old female presented with a 1-month history of low abd pain. A 15 x 13 cm solid mass was noted in pelvic cavity by imaging study. The patient underwent an exploratory laparotomy, total abdominal hysterectomy, bilateral salpingo-oophorectomy, pelvic lymph node dissection and omentectomy. Immunohistochemistry aided in the final diagnosis of a malignant peripheral nerve sheath tumor. The patient had several poor prognosis factor and relapsed into tumor and died 5 month after surgery. An extremely rare case of malignant peripheral nerve sheath tumor of pelvic cavity is reported with a brief review of the literature.

Malignant peripheral nerve sheath tumor on palate: A case report

SUMMARY: The malignant peripheral nerve sheath tumor(MPNST) is an aggressive neoplasm and can either arise independently or result from malignant change in preexisting neurofibromatosis (von Recklinghausen's disease). Its histologic characteristics remain controversial, but currently it is believed that the schwann cell is the origin of the peripheral nerve sheath tumors. MPNST is an uncommon neoplasm of the head and neck region, and its presentation in the oral cavity is quite rare. In this study, we report a patient with a rare case of a MPNST involving the maxilla. A CASE REPORT: A 29-year-old female presented with a chief complaint of painless swelling with bleeding tendency on the left maxillary tuberosity area 2 months ago. Clinical examination showed a 5.0*3.0 cm2 sized, indurative swelling on the site. Conventional radiographs showed a relatively well-defined soft tissue mass involving the left maxillary sinus, and destruction of the anterior, posterolateral walls of the left maxillary sinus. Subtotal maxillectomy and split-thickness skin graft from thigh were undertaken. In histochemical and immunohistochemical studies, the specimen revealed positive reactivities to Vimentin and S-100 protein. Final diagnosis was made as MPNST.