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Fulltext The Value of H3K27me3 Immunohistochemistry in Differentiating Malignant Peripheral Nerve Sheath Tumour with Its Histologic Mimickers

Mustapar N, Zawawi MSF, Tuan Sharif SE

Asian Pac J Cancer Prev, 2020 Mar 01;21(3):699-705.
PMID: 32212796 DOI: 10.31557/APJCP.2020.21.3.699

BACKGROUND: Diagnosis of malignant peripheral nerve sheath tumor (MPNST) is rather challenging due to its divergent morphologic heterogeneity and lack of specific ancillary test. The emergence of H3K27 trimethylation (H3K27me3) as a new immunohistochemistry (IHC) marker for MPNST have recently available to assist pathologists in differentiating MPNST from other histologic mimics. We aim to study the expression pattern of H3K27me3 in MPNST and its histologic mimickers and their association with the clinicopathological data.
METHODOLOGY: A total of 59 benign and malignant spindle cell tumours (18 MPNST and 41 of its histologic mimickers which included 10 schwannoma, 13 neurofibroma, 4 synovial sarcoma, 3 fibrosarcoma, 2 gastrointestinal stromal tumour (GIST), 4 leiomyosarcoma, 1 spindle cell liposarcoma, 1 solitary fibrous tumour, 2 low grade fibromyxoid sarcoma and 1 unclassified spindle cell sarcoma), diagnosed from January 1998 to April 2018 in Hospital Universiti Sains Malaysia (HUSM) were tested for H3K27me3 by IHC. The MPNST histological grade was assessed based on the French Fe'de' ration Nationale des Centres de LutteContre le Cancer (FNCLCC) for 3 tiers system (low grade, intermediate grade and high grade). The clinicopathological data were retrieved from the patients' record.
RESULTS: A total of 61.1% (11/18 MPNST) showed loss of H3K27me3 expression which is statistically significant as compared to its histologic mimics (p<0.001). Similar findings (p=0.026) were also observed in high grade MPNST (81.8%), intermediate grade MPNST (100%) and 0% in low grade MPNST.
CONCLUSION: H3K27me3, combined with other panel of markers, is useful in MPNST diagnosis to differentiate it from the histological mimickers.

Matched MeSH terms: Nerve Sheath Neoplasms/diagnosis*; Nerve Sheath Neoplasms/metabolism
Ulcerated bleeding palisaded encapsulated neuroma of the tongue

Dhanuka S, Rodrigues G, Carnello S

Malays J Pathol, 2019 Aug;41(2):229-232.
PMID: 31427561

INTRODUCTION: Palisaded encapsulated neuroma (PEN) is a benign lesion of Schwann cells and its occurrence in the oral mucosa especially the tongue is very rare.
CASE REPORT: This article describes a case of a 41-year-old male, a chronic smoker with an actively bleeding, ulcerated, solitary, firm lesion on the lateral border of the tongue which had bled thrice before. A differential diagnosis of pyogenic granuloma, haemangioma, fibroma, nerve sheath tumour, salivary gland tumour and malignancy was made and surgically excised. Histopathology of the excised specimen revealed a well-circumscribed lesion with spindle-shaped cells arranged in interlacing fascicles and with the help of immunohistochemical markers confirmed it to be a PEN.
DISCUSSION: To our knowledge, this is the first description of an ulcerated PEN presented with an active bleed.

Matched MeSH terms: Nerve Sheath Neoplasms
A rare case of cervical hypoglossal nerve neurofibromas in a patient with type 1 neurofibromatosis

Lum SG, Baki MM, Yunus MRM

Braz J Otorhinolaryngol, 2021 Feb 25.
PMID: 33707119 DOI: 10.1016/j.bjorl.2021.01.006

BACKGROUND: Neurofibromas are benign peripheral nerve sheath tumours. Hypoglossal nerve neurofibromas in cervical region are relatively rare, bilateral occurrence is extremely rare.
METHODS: A 32-year-old man with type 1 neurofibromatosis presented with bilateral neck masses. Magnetic resonance imaging showed parapharyngeal masses consistent with neurogenic tumours, most likely neurofibromas.
RESULTS: Surgical exploration through lateral cervical approach revealed unexpected finding of the tumour that arose from the hypoglossal nerve. The tumour had totally engulfed the nerve with no normal nerve fascicles identifiable, thus resected in toto. In the postoperative course, the patient developed right hypoglossal palsy and vocal fold palsy treated with augmentation of the paralysed vocal fold with temporary injection material.
CONCLUSIONS: The authors described a patient with type 1 neurofibromatosis with neurofibroma originating from cervical part of hypoglossal nerve. This paper discussed this rare condition and the management on how to improve the treatment outcome.

Matched MeSH terms: Nerve Sheath Neoplasms
Fulltext Primary Ewing’s sarcoma of the lumbosacral spine: a case report

Zamzuri, Z., Mohd Adham, S.Y., Saufi, M.A., Azian, A.A., Fadhli, M.

International Medical Journal Malaysia, 2012;11(1):47-50.
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Ewing's sarcoma is a rare tumor first discovered by James Ewing in 1921. It is more common in bone or skeletal component compared to soft tissue or extraosseous Ewing's sarcoma. Among soft tissue Ewing’s, spinal cord involvement is rarer with only nine cases reported. We report a case of nine-year-old Malay girl who presented with low back pain for two months following a fall with progressive neurological deficits of bilateral lower limb. Magnetic resonance imaging was suggestive of a well-defined margin of intradural extramedullary tumor. With nerve sheath tumor in mind, surgical excision with laminectomy L2-S1 was performed. Intraoperative finding was an extradural mass from L3-L5 with extension to bilateral neuroforamen. Histopathology report defined a round cell tumour of Ewing’s sarcoma from the mass.

Matched MeSH terms: Nerve Sheath Neoplasms
Fulltext Malignant Peripheral Nerve Sheath Tumor: Treat or Not Treat?

Bin Samsuddin MF, Bin Omar MA

Asian J Neurosurg, 2019 4 3;14(1):283-285.
PMID: 30937055 DOI: 10.4103/ajns.AJNS_332_17

Malignant peripheral nerve sheath tumors (MPNSTs) are uncommon, biologically aggressive soft tissue sarcomas of neural origin that poses tremendous challenges to effective therapy. MPNSTs are among the most challenging mesenchymal malignancies to treat with poor prognosis. They usually affect young and middle-aged adults, tend toward early metastasis, and often demonstrate resistance to chemotherapy. We present a case of a 23-year-old female who initially presented with the right temporal swelling for 1 month associated with constitutional symptom which progressively worsening. The right craniotomy and excision biopsy were done with histopathological examination results suggestive of MPNST. Thorax-abdominal-pelvic computed tomography and magnetic resonance imaging further revealed multiple metastatic lesions involving spine, retroperitoneal, pelvic, chest wall, and lungs. This case illustrates the typical presentation of MPNST with its known poorly outcome.

Matched MeSH terms: Nerve Sheath Neoplasms
Fulltext Bilateral Mirror Image Cervical Neurofibroma in an Adult with Neurofibromatosis Type 1

Pandey S, Singh K, Sharma V, Khan MT, Ghosh A, Santhosh D

Malays J Med Sci, 2017 Mar;24(1):117-120.
PMID: 28381935 DOI: 10.21315/mjms2017.24.1.13

Neurofibromatosis type 1 (NF1) is an autosomal dominant disorder characterised by various phenotypic features like hyperpigmented spots, neurofibromas, Lisch nodules, skeletal abnormalities and tendency to develop neoplasms. Only few cases of Non-Familial Spinal Neurofibromatosis-1 (Non-FSNF1) have been described in literature with tumors involving the spinal roots at every level being even rarer. We reported an interesting case of bilateral symmetrical cervical neurofibroma with multiple spinal neurofibromas appearing as mirror image on CT, associated with non familial NF-1 as a rare presentation in a 25-year-old adult male.

Matched MeSH terms: Nerve Sheath Neoplasms