Malignant Peripheral Nerve Sheath Tumor of Bone in Children and Adolescents

Malignant peripheral nerve sheath tumor (MPNST) of bone is a rare entity. We have examined three lesions that fit standard histopathologic criteria for MPNST of soft tissues but that arose in the skeleton of three children aged 6 to 13 years. None was affected by neurofibromatosis 1 (NF1). Histologic features typical of MPNST included spindle cells with comma-shaped nuclei, tactoid bodies, nuclear palisading, hyaline bands, and schwannoma-like and curlicue foci. Epithelioid foci were seen in two cases, and heterologous differentiation in one. Immunohistochemistry revealed positivity for S-100 (1 positive/3 tested), vimentin (3/3), glial fibrillary acidic protein (2/3), CD34 (1/1), and CD68 (1/2). Studies for CD99 (0/3), epithelial membrane antigen (0/3), cytokeratin (0/3), CD57 (0/3), and HMB-45 (0/2) were negative. Ultrastructural findings in one of two cases examined included interlacing, attenuated cytoplasmic processes, microtubules, and rare dense-core granules. We conclude that MPNST may arise as a primary bone neoplasm in children without NF1.

Download Full-text

RARE-20. MALIGNANT PERIPHERAL NERVE SHEATH TUMOR OF A CRANIAL NERVE IN AN INFANT WITH NEUROCUTANEOUS MELANOSIS

Neuro-Oncology ◽

10.1093/neuonc/noaa222.731 ◽

2020 ◽

Vol 22 (Supplement_3) ◽

pp. iii446-iii446

Author(s):

Lacey Carter ◽

Naina Gross ◽

Rene McNall-Knapp ◽

,and Jo Elle Peterson

Keyword(s):

Peripheral Nerve ◽

Cranial Nerve ◽

Leptomeningeal Disease ◽

Nerve Sheath Tumor ◽

Peripheral Nerve Sheath Tumor ◽

Neurocutaneous Melanosis ◽

Spindle Cell Neoplasm ◽

Lower Back ◽

Nerve Sheath ◽

S 100

Abstract At one month of age, a female presented with a giant congenital nevus along lower back and thighs and hydrocephalus. A ventriculoperitoneal shunt was placed. An MRI was done at six months, initially reported as normal. At eleven months of age, five months after original MRI, patient presented with dysconjugate gaze and lethargy. MRI showed new 3.8 x 3.7 x 3.4 cm right cerebellopontine angle mass extending into Meckel’s cave and foramen ovale along with leptomeningeal disease extending from the mass along the entire length of the spinal cord. Retrospective review of prior MRI revealed subtle leptomeningeal enhancement concerning for neurocutaneous melanosis (NCM). Given the leptomeningeal disease, family elected for open biopsy and debulking of lesion instead of aggressive resection. Histologically, the mass showed hypercellular spindle cell neoplasm with mitotic activity and necrosis mixed with remnants of normal cranial nerve. GFAP was negative, excluding a glioma. HMB-45, MITF, panmelanoma, and Melan-A were negative, excluding melanoma. A negative myogenin stain ruled out ectomesenchymoma. S-100 protein and SOX-10 positivity with variable loss of staining for trimethylation of histone H3 K27 were indicative of malignant peripheral nerve sheath tumor (MPNST). Given the course of the mass, trigeminal nerve MPNST was presumed. Given the poor prognosis of intracranial MPNST and NCM, family elected to forgo treatment and was discharged with hospice. She died 25 days after surgery. Cranial nerve MPNST is rare. MPNST in patients with NCM has not previously been reported to our knowledge.

Download Full-text

Malignant Peripheral Nerve Sheath Tumor of the Vestibulocochlear Nerve and Brainstem

Neurosurgery ◽

10.1227/neu.0b013e318223bc2a ◽

2011 ◽

Vol 69 (5) ◽

pp. E1152-E1165 ◽

Cited By ~ 6

Author(s):

Kristophe J. Karami ◽

Prashant S. Kelkar ◽

Michael P. Verdon ◽

Inga S. Grills ◽

Dennis I. Bojrab ◽

...

Keyword(s):

Radiation Therapy ◽

Peripheral Nerve ◽

Soft Tissues ◽

Gamma Knife Radiosurgery ◽

Surgical Approaches ◽

Nerve Sheath Tumor ◽

Vestibulocochlear Nerve ◽

Peripheral Nerve Sheath Tumor ◽

Peripheral Nerve Sheath Tumors ◽

Nerve Sheath

Abstract BACKGROUND AND IMPORTANCE Malignant peripheral nerve sheath tumors are the most common malignant mesenchymal tumors of soft tissues, but they are very rare when found to arise from a cranial nerve and when not in association with neurofibromatosis. These tumors are highly malignant and carry a poor prognosis with survival usually less than 6 months. CLINICAL PRESENTATION The authors report the case of a 23-year-old female with no history of phakomatoses, previous irradiation, or known genetic disorders, who presented with a malignant peripheral nerve sheath tumor of the vestibulocochlear nerve and brainstem. Multiple staged skull base approaches were carried out with maximal possible resection. Adjunctive therapies including standard radiation therapy, intensity-modulated radiation therapy, and stereotactic gamma knife radiosurgery were used with an ultimate patient survival of 27 months. CONCLUSION To our knowledge, this is the first report describing a patient with a malignant peripheral nerve sheath tumor of the vestibulocochlear nerve and brainstem treated with staged surgical approaches in conjunction with multiple forms of radiotherapy and having a significant survival of more than 2 years.

Download Full-text

Malignant peripheral nerve sheath tumor of the uterine cervix expressing both S-100 protein and HMB-45

Journal of Obstetrics and Gynaecology Research ◽

10.1111/j.1447-0756.2009.01078.x ◽

2009 ◽

Vol 35 (6) ◽

pp. 1136-1141 ◽

Cited By ~ 8

Author(s):

Na Rae Kim ◽

Dong-Hae Chung ◽

Chan Yong Park ◽

Seung Yeon Ha

Keyword(s):

Peripheral Nerve ◽

Uterine Cervix ◽

Nerve Sheath Tumor ◽

Peripheral Nerve Sheath Tumor ◽

Nerve Sheath ◽

S 100 ◽

Hmb 45

Download Full-text

Case report and immunohistochemical study of a malignant peripheral nerve sheath tumor in a dog

Brazilian Journal of Veterinary Research and Animal Science ◽

10.11606/issn.1678-4456.bjvras.2017.132447 ◽

2017 ◽

Vol 54 (4) ◽

pp. 420

Author(s):

Thaís De Almeida Moreira ◽

Leandro Willian Borges ◽

Talita Lopes Serra ◽

Diego Petrocino Caetano ◽

Marcelo Carrijo da Costa ◽

...

Keyword(s):

Case Report ◽

Peripheral Nerve ◽

Immunohistochemical Study ◽

Nerve Sheath Tumor ◽

Peripheral Nerve Sheath Tumor ◽

Nerve Sheath ◽

S 100

O tumor maligno da bainha do nervo periférico é raro e pouco descrito em cães. Classicamente, o termo “neurofibroma/sarcoma” é empregado quando o tumor é composto por células de Schwann e células perineurais. Neste relato são descritos os achados clínicos, histopatológicos e imuno-histoquímicos de um caso de tumor maligno da bainha do nervo periférico, provavelmente neurofibrossarcoma, localizado no subcutâneo do membro pélvico de um cão da raça Rottweiler. Os achados histopatológicos associados às observações imuno-histoquímicas contribuíram para o diagnóstico, sendo observada positividade para proteína S-100, vimentina e CD57, permitindo a diferenciação da neoplasia em questão de outros tumores malignos com características morfológicas similares.

Download Full-text

Malignant Triton Tumor without Von Recklinghausen’s Neurofibromatosis in a 16 year-old Indonesian Girl: A Case Report

Ophthalmologica Indonesiana ◽

10.35749/journal.v43i1.129 ◽

2017 ◽

Vol 43 (1) ◽

pp. 3

Author(s):

Neni Anggraini ◽

Nurjati Chairani Siregar

Keyword(s):

Visual Acuity ◽

Case Report ◽

Early Detection ◽

Peripheral Nerve ◽

Correct Diagnosis ◽

Nerve Sheath Tumor ◽

Malignant Triton Tumor ◽

Peripheral Nerve Sheath Tumor ◽

Nerve Sheath ◽

S 100

Background: To diagnose malignant peripheral nerve sheath tumor with rhabdomyoblastic differentiation in malignant triton tumor which has a rare incidence. Case Illustration: We reported a case in a 16 year-old girl who presented with progressively painless proptosis of the left eye and decreased visual acuity. There was no evidence of Recklinghausen’s disease. She underwent surgery and radiotherapy. The histopatologic findings showed malignant peripheral nerve sheath tumor (MPNST). Four years later, she suffered recurrence proptosis and decreased visual acuity. Histomorphology supported by immunostaining with S-100 protein confirmed the diagnosis of malignant triton tumor. She underwent chemotherapy and enucleation. Conclusion: This case highlights the prudent use of immunohistochemistry that is essential in making an early detection and a correct diagnosis.

Download Full-text