scholarly journals First documented case of intracranial falcine malignant peripheral nerve sheath tumor: illustrative case

2021 ◽  
Vol 2 (6) ◽  
Author(s):  
Renato J. Galzio ◽  
Mattia Del Maestro ◽  
Diamantoula Pagkou ◽  
Massimo Caulo ◽  
Sofia Asioli ◽  
...  

BACKGROUND The authors reported the first documented case of intracranial extraaxial nonneurofibromatosis type 1–related nontriton malignant peripheral nerve sheath tumor (MPNST) originating from the falx cerebri. OBSERVATIONS A 34-year-old man with headache, short-term memory deficit, postural instability, and blurred vision presented with a large heterogenous contrast-enhanced intraventricular cystic lesion originating from the free margin of the falx cerebri. The patient received surgery using the right posterior interhemispheric approach. Gross total resection was performed, and the inferior border of the falx cerebri was resected. The postoperative course was uneventful. Histological examination revealed hypercellular foci of neoplastic spindle cells with hyperchromatic and wavy nuclei. Hence, a diagnosis of MPNST was made based on concomitant immunochemistry findings, including mouse double minute 2 homolog focal positivity and geographic loss of H3K27me3. The patient received adjuvant radiotherapy, and recurrence was not observed. LESSONS Intracranial MPNSTs are extremely rare tumors, typically originating from the cranial nerves in the posterior cranial fossa. An even rarer variant of these tumors, referred to as malignant intracerebral nerve sheath tumors, may directly arise from the brain parenchyma. The authors reported the first case of an intracranial MPNST originating from the dura mater of the falx cerebri, acting as an extraaxial lesion with prevalent expansion in the right ventricle.

2019 ◽  
Vol 131 (6) ◽  
pp. 1835-1839 ◽  
Author(s):  
Hirokazu Takami ◽  
Christoph M. Prummer ◽  
Christopher S. Graffeo ◽  
Maria Peris-Celda ◽  
Caterina Giannini ◽  
...  

Glioblastoma (GBM) of the internal auditory canal (IAC) is exceedingly rare, with only 3 prior cases reported in the literature. The authors present the fourth case of cerebellopontine angle (CPA) and IAC GBM, and the first in which the lesion mimicked a vestibular schwannoma (VS) early in its natural history. A 55-year-old man presented with tinnitus, hearing loss, and imbalance. MRI identified a left IAC/CPA lesion measuring 8 mm, most consistent with a benign VS. Over the subsequent 4 months he developed facial weakness. The tumor grew remarkably to 24 mm and surgery was recommended; the main preoperative diagnosis was malignant peripheral nerve sheath tumor (MPNST). Resection proceeded via a translabyrinthine approach with resection of cranial nerves VII and VIII, followed by facial-hypoglossal nerve anastomosis. Intraoperative frozen section suggested malignant spindle cell neoplasm, but final histopathological and molecular testing confirmed the lesion to be a GBM. The authors report the first case in which absence of any brainstem interface effectively excluded a primary parenchymal tumor, in particular GBM, from the differential diagnosis. Given the dramatic differences in treatment and prognoses between malignant glioma and MPNST, this case emphasizes the importance of surgical intervention on an aggressively growing lesion, which provides both the best probability of local control and the critical tissue diagnosis.


2018 ◽  
Vol 32 (3) ◽  
pp. 469-472
Author(s):  
Varun Aggarwal ◽  
Amit Narang ◽  
Chandni Maheshwari ◽  
Divya Kavita

Abstract Malignant Peripheral Nerve Sheath Tumors (MPNSTs) are one of the very rare high grade malignancies usually affecting extremities or trunk. Incidence is 1/Lac. Intracranial MPNSTs are even rarer, schwannomatous and commonly affecting cranial nerves VIII &VII). Intra-cranial MPNSTs are usually sporadic, arising de novo. The second most common mode of origin is from malignant transformation from pre-existing schwannomas or neurofibroma. We present an extremely rare and probably the first case of intra-cranial malignant peripheral nerve sheath tumor of the olfactory nerve in a non neurofibrosis patient with no prior history of irradiation.


2017 ◽  
Vol 2017 ◽  
pp. 1-5 ◽  
Author(s):  
Lauren Pearson ◽  
Erinc Akture ◽  
Julien Wonderlick ◽  
Gregory Fuller ◽  
Maryam Zenali

Schwannoma is a benign peripheral nerve sheath tumor that typically involves cranial nerves of the head and neck region. Intraparenchymal occurrence of this tumor is uncommon. Even rarer in this site is the microcystic/reticular pattern of schwannoma. This histologic variant, first described in 2008, has a predilection for visceral organs. Herein, we report the first case of microcystic/reticular schwannoma of the frontal lobe.


2009 ◽  
Vol 46 (3) ◽  
pp. 444-448 ◽  
Author(s):  
E. C. Kappe ◽  
K. KÖHLER ◽  
I. v. Felbert ◽  
J. P. Teifke ◽  
J. TÓTH ◽  
...  

A 14-year-old Haflinger gelding presented with a protruding mass involving the cornea of the right eye. The mass was resected and submitted for histopathologic and immunohistochemical examination. The preliminary diagnosis was corneal sarcoma, most likely fibrosarcoma. The immunohistochemical results confirmed the mesenchymal origin of the neoplastic cells, which were most consistent with a malignant peripheral nerve sheath tumor. Corneal mesenchymal neoplasms are extremely uncommon tumors in human beings and domestic animals. The cause for this tumor was not determined; infection with bovine papillomavirus was not detected.


2014 ◽  
Vol 3 (1) ◽  
pp. 204798161351603 ◽  
Author(s):  
Yuki Iwama ◽  
Makoto Kunisada ◽  
Hajimu Goto ◽  
Yoshiharu Ohno ◽  
Junji Yamashita ◽  
...  

Malignant peripheral nerve sheath tumor (MPNST) involving bone is rare. We report a case of MPNST of the fifth toe. The lesion was located in the distal phalanx of the right fifth toe and extended into surrounding subcutaneous tissues. Findings on magnetic resonance imaging and histological features of the case are described and the literature is briefly reviewed.


2017 ◽  
Vol 2017 ◽  
pp. 1-4
Author(s):  
Marija Milković Periša ◽  
Tihana Džombeta ◽  
Jasminka Stepan Giljević ◽  
Božo Krušlin

Benign and malignant tumors are common in the setting of neurofibromatosis type 1 (NF1). Malignant peripheral nerve sheath tumor (MPNST) and angiosarcoma are rare tumors in children and adolescents and mostly occur in young patients in relation to NF1. Both histological types can be present in the same tumor mass in patients with NF1. We present a case of 12.5-year-old girl with NF1 who first presented with MPNST of the right inguinal region and 1.5 years later with unrelated angiosarcoma of the scalp.


2021 ◽  
Vol 3 (Supplement_6) ◽  
pp. vi15-vi15
Author(s):  
Yuko Watanabe ◽  
Hiroshi Igaki ◽  
Ayumu Arakawa ◽  
Shunsuke Yanagisawa ◽  
Masamichi Takahashi ◽  
...  

Abstract Introduction: Recurrent malignant peripheral nerve sheath tumor (MPNST) is intractable. Boron neutron capture therapy (BNCT) is a treatment using tumor-selective particle radiation, and is indicated for medical treatment for head and neck cancer, and also used for malignant glioma and malignant meningioma.<Case>20-year-old woman who has MPNST that extends from the subcutaneous tissue of the right neck to the posterior fossa. <Medical History>Chemotherapy and local irradiation(50.4Gy/28fr) for primary embryonal rhabdomyosarcoma of the right ear canal at the age six.<Current medical history>Right cervical tumor developed at 17-year-old, some chemotherapy regimens and tumor resections were performed as recurrence of rhabdomyosarcoma at the previous hospital. After she was diagnosed with MPNST in the pathology consultation at our hospital, she was irradiated with heavy ion beam 70.8Gy(RBE)/16fr and received additional chemotherapies at our department, but her tumor was refractory. Although BNCT for MPNST is not covered by health insurance in Japan, she wanted to try to be treated for BNCT. After confirming boron accumulation in the tumor (SUVmax 4.28) by FBPA-PET, tumor growth and hydrocephalus occurred while waiting for travel to Taiwan due to the spread of COVID-19 infection. She was performed tumor resection(NTR) and irradiated with SRS 20Gy/fr for the residual lesion, but tumor had a rapid recurrence from the margin of the excision cavity. Finally, she could travel about 3 months after the operation and underwent BNCT, that used neutrons and 10B-boronophenylalanine from the Tsinghua University research reactor in Taiwan. No serious adverse events including cerebral edema were observed, and dramatic tumor shrinkage was maintained after treatment. FBPA-PET of 3 months later showed accumulation in the part of the margin of the cavity, the recurrence was observed on MRI after 3 and a half months. Discussion: BNCT for refractory/recurrent MPNST showed acceptable safety and was able to prolong progression-free survival.


Author(s):  
Anuradha P. ◽  
Bathi Reddy ◽  
Suhail Gaur

<p>Malignant peripheral nerve sheath tumor (MPNST) refers to spindle cell sarcomas arising from or separating in the direction of cells of the peripheral nerve sheath. The MPNST of the parotid gland is an extremely rare tumor, accounts for &lt;5% of all soft tissue sarcomas, and carries a poor prognosis. Diagnosis of MPNSTs is usually based on histopathology aided by IHC, which reflects the Schwann cell differentiation in this neoplasm. Approximately 50-90% of MPNSTs are positive for S-100 protein. A 56-year-old male presented with right parotid swelling for 3 months and incomplete right eye closure. The patient was diagnosed with neurofibromatosis 1 (NF-1) and pulmonary tuberculosis in the past. On Examination, a hard, tender swelling measuring 5×4 cm involving the right parotid gland and right grade 2 LMN facial palsy was noted. Histopathology was suggestive of a MPNST.</p><p><strong> </strong></p>


Apmis ◽  
2016 ◽  
Vol 124 (5) ◽  
pp. 428-430 ◽  
Author(s):  
Jasmina Redzepagic ◽  
Faruk Skenderi ◽  
Jasmina Bajrovic ◽  
Vanesa Beslagic ◽  
Nermina Ibisevic ◽  
...  

2010 ◽  
Vol 10 (4) ◽  
pp. 328-330 ◽  
Author(s):  
Violeta Kolarov ◽  
Jelena Stanić ◽  
Živka Eri ◽  
Biljana Zvezdin ◽  
Marija Kojičić ◽  
...  

We report a case of intrathoracic malignant peripheral nerve sheath tumor in a 65-year old woman revealed after a few-month history of progressive dyspnea, appetite and body mass loss. The chest magnetic resonance (MR) examination revealed the presence of a large tumor occupying the mediastinum and a major portion of the right hemithorax. The diagnostic tumor sample was obtained by parasternal biopsy in local anesthesia. The surgical resection of the tumor could not be performed due to its excessive size, intrathoracic involvement and bad respiratory reserves of a patient. The chemotherapy and irradiation were performed as palliative measures. The lethal outcome appeared 10 months after the diagnosis was established.


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