Symptoms associated with malignancy of peripheral nerve sheath tumours: a retrospective study of 69 patients with neurofibromatosis 1

Valeyrie‐Allanore, Laurence; Ismaili, Nabil; Bastuji‐Garin, Sylvie; Zeller, J; Wechsler, Janine; Revuz, J; Wolkenstein, Pierre

doi:10.1111/j.1365-2133.2005.06558.x

Cited by 86 publications

(59 citation statements)

References 20 publications

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“…Neurofibroma with NF1 is associated in less than 1% of all cases [1]. However, it is estimated that 4.5% of NF1 patients develop mediastinal tumors [2]. Neurofibroma of the intrathoracic vagusnerve is a benign, slow-growing neurogenic tumor that arises from the nerve sheath.…”

Section: Discussionmentioning

confidence: 99%

Unusual Presentation of a Mediastinal Tumour: A Case Report

Kumar¹,

Roy²,

Agarwal³

et al. 2017

WJCD

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Neurofibroma arising from intrathoracic vagus nerve is a rare entity. We present a 6-year-old child with stigmata of neurofibromatosis along with a lump in the back. She also had history of on and off cough. On investigation there was a large mediastinal mass abutting the trachea. She underwent a posterolateral thoracotomy with partial resection of the lump. Intraoperatively the mass appeared to arise from the vagus nerve. Post operative stay was uneventful. Biopsy of the mass revealed a benign neurofibroma with no evidence of invasion. She is presently asymptomatic after 1 year of follow-up.

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Section: Discussionmentioning

confidence: 99%

Unusual Presentation of a Mediastinal Tumour: A Case Report

Kumar¹,

Roy²,

Agarwal³

et al. 2017

WJCD

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“…Immunohistochemistry, clinical features, and the possible association with neurofibromatosis all contribute to the establishment of an accurate diagnosis. 70,71 The characteristic histological findings include fascicles of spindled cells with marked hypercellularity and high mitotic activity. 66,71 Immunohistochemical study usually discloses focal positivity for S-100 protein and negativity for cytokeratins, smooth muscle actin, HMB45, and alpha-1-antitrypsin.…”

Section: Liposarcoma (Atypical Lipomatous Tumors) Of the Vulvamentioning

confidence: 99%

“…71,72 Therapy consists of wide surgical excision of the primary tumor. 70,72 Postsurgical radiotherapy has been reported with good effect in order to prevent the risk of recurrence. 73 In cases involving locally advanced disease and large tumors, external-beam radiation, anterior pelvic exenteration with pelvic reconstruction, and adjuvant chemotherapy represent the treatment of choice.…”

Section: Liposarcoma (Atypical Lipomatous Tumors) Of the Vulvamentioning

confidence: 99%

“…73 In cases involving locally advanced disease and large tumors, external-beam radiation, anterior pelvic exenteration with pelvic reconstruction, and adjuvant chemotherapy represent the treatment of choice. 70,73 In cases where surgery is contraindicated, high-dose radiation therapy can be considered. 73 The prognosis is generally poor, especially for children.…”

Section: Liposarcoma (Atypical Lipomatous Tumors) Of the Vulvamentioning

confidence: 99%

“…69 Besides pain, subjective complaints are associated with neural involvement and manifest with paresthesia, difficulty in walking or sitting, and a periodically burning sensation, numbness, or tingling. 70 The diagnosis of MPNST is based on histological examination that reveals a predominant spindle cell proliferation, frequently with nerve involvement. Immunohistochemistry, clinical features, and the possible association with neurofibromatosis all contribute to the establishment of an accurate diagnosis.…”

Section: Liposarcoma (Atypical Lipomatous Tumors) Of the Vulvamentioning

confidence: 99%

See 2 more Smart Citations

Vulvar sarcomas: Short guideline for histopathological recognition and clinical management. Part 2

Chokoeva¹,

Tchernev

Cardoso

et al. 2015

Int J Immunopathol Pharmacol

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Malignant tumors of the female reproductive system are a serious health and social problem, as they are the second most common cause of death among women, after breast cancer. Vulvar tumors represent only 4% of all gynecological neoplasms, and they are fourth in frequency after tumors of the cervix, uterus, and ovary. Ninety-eight percent of all vulvar tumors are benign and only 2% are malignant. Sarcomas of the vulva comprise approximately 1-3% of all vulvar cancers. They are characterized by rapid growth, high metastatic potential, frequent recurrences, aggressive behavior, and high mortality rate. In Part 1 of this paper, we presented the most common forms of sarcoma of the vulva: leiomyosarcoma, epithelioid sarcoma, malignant rhabdoid tumor, and rhabdomyosarcoma. The second part of this review will focus mainly on the rarest variants of vulvar sarcoma: low-grade fibromyxoid sarcoma, synovial sarcoma, monophasic synovial sarcoma, carcinosarcoma, Ewing sarcoma, myeloid sarcoma, dermatofibrosarcoma protuberans, malignant fibrous histiocytoma, angiomatoid fibrous histiocytoma, liposarcoma, malignant peripheral nerve sheath tumor, and malignant mesothelioma.

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Imaging in gynecological disease (26): clinical and ultrasound characteristics of benign retroperitoneal pelvic peripheral‐nerve‐sheath tumors

Fischerová

Santos²,

Wong

et al. 2023

Ultrasound in Obstet & Gyne

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ObjectiveTo describe the clinical and sonographic characteristics of benign, retroperitoneal, pelvic peripheral nerve sheath tumors (PNST).MethodsThis was a retrospective, single, gynecologic oncology center study conducted between 1 January 2018 and 31 August 2022. All ultrasound images, clips, and final specimens of benign PNSTs were reviewed by the authors to describe (1) the ultrasound appearance of the tumors using the terminology of the International Ovarian Tumor Analysis (IOTA), Morphological Uterus Sonographic Assessment (MUSA) and Vulvar International Tumor Analysis (VITA) groups on a predefined ultrasound assessment form, (2) the origin of the tumors in relation to nerves and pelvic anatomy, and (3) the correlation between ultrasound features and histotopograms. A review of literature on benign, retroperitoneal, pelvic PNSTs with preoperative ultrasound examination was performed.ResultsFive women (mean age 53 years) with benign, retroperitoneal, pelvic PNSTs were identified: four with schwannomas and one with a neurofibroma, all of which were sporadic and solitary. All patients had good quality ultrasound images and clips and final biopsies of surgically excised tumors, except for one patient managed conservatively with a tru‐cut biopsy. In four of these cases, the findings were incidental. Size range for the five PNSTs was from 31‐50 mm. All five PNSTs were solid, moderately vascular tumors, with non‐uniform echogenicity, well‐circumscribed by hyperechogenic epineurium, and had no acoustic shadowing. Most of the masses were round (n = 4 (80%)), and contained small, irregular, anechoic, cystic spaces (n = 3 (60%)), and hyperechoic areas (n = 4 (80%)). A literature search identified 47 cases of retroperitoneal schwannomas and neurofibromas, the characteristics of which were compared with the cases in our series.ConclusionsOn ultrasound, benign PNSTs were solid, non‐uniform, moderately vascular tumors without acoustic shadowing. Most were round, containing small, irregular, anechoic, cystic spaces, and hyperechoic areas, consistent with degenerative changes on pathology. All tumors were well‐circumscribed by a hyperechogenic rim composed of epineurium. No imaging characteristics reliably differentiated between schwannomas and neurofibromas. In fact, they overlap with the ultrasound appearance of malignant tumors. Hence, ultrasound‐guided biopsy plays a pivotal role in diagnosis, and if confirmed as benign PNSTs, these tumors can undergo ultrasound surveillance.This article is protected by copyright. All rights reserved.

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Symptoms associated with malignancy of peripheral nerve sheath tumours: a retrospective study of 69 patients with neurofibromatosis 1

Abstract: From a practical point of view, pain, neurological deficit and enlargement of a pre-existing peripheral nerve sheath tumour in NF1 must lead to deep surgical biopsy to rule out malignant transformation.

Cited by 86 publications

References 20 publications

Unusual Presentation of a Mediastinal Tumour: A Case Report

Unusual Presentation of a Mediastinal Tumour: A Case Report

Vulvar sarcomas: Short guideline for histopathological recognition and clinical management. Part 2

Imaging in gynecological disease (26): clinical and ultrasound characteristics of benign retroperitoneal pelvic peripheral‐nerve‐sheath tumors

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