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Malignant Peripheral Nerve Sheath Tumor

MPNST


Presentation

  • We present a rare case of MPNST of the tongue who presented with features of hypoglossal nerve palsy. Incisional biopsy showed a malignant spindle cell tumor in the sub-epithelial connective tissue. The tumor cells were immune-positive for S-100.[ncbi.nlm.nih.gov]
Walking with a Cane
  • At 3-month follow-up, the patient walked with a cane with no further deficits except mild ataxia, which resolved within 6 months. Magnetic resonance imaging revealed a small residual tumor adherent to the basilar artery.[ncbi.nlm.nih.gov]
Soft Tissue Swelling
  • Herein, this tumor is presented within a rare clinical setting, in a 45-year-old gentleman, with multiple soft tissue swellings and a previous history of surgical excision of a thigh mass.[ncbi.nlm.nih.gov]
Hyposmia
  • She had a 4-month history of progressive, unilateral right nasal obstruction, unilateral mucopurulent rhinorrhea, foul nasal odor, snoring, hyposmia, occasional epistaxis, and no headache, no facial numbness, without eye swelling and vision loss.[ncbi.nlm.nih.gov]
Snoring
  • She had a 4-month history of progressive, unilateral right nasal obstruction, unilateral mucopurulent rhinorrhea, foul nasal odor, snoring, hyposmia, occasional epistaxis, and no headache, no facial numbness, without eye swelling and vision loss.[ncbi.nlm.nih.gov]
Hip Pain
  • A 9-year-old female with neurofibromatosis type 2 (NF-2) presented with right hip pain and severe weakness of bilateral legs for 3 months.[ncbi.nlm.nih.gov]
Eye Swelling
  • She had a 4-month history of progressive, unilateral right nasal obstruction, unilateral mucopurulent rhinorrhea, foul nasal odor, snoring, hyposmia, occasional epistaxis, and no headache, no facial numbness, without eye swelling and vision loss.[ncbi.nlm.nih.gov]
Breast Mass
  • Further examination revealed a right renal mass and left breast mass.[ncbi.nlm.nih.gov]
  • Eventually, the patient developed a right breast mass that was diagnosed as metastatic MPNST. The patient refused any kind of treatment and died 6 months later in hospice care.[urologyannals.com]
Abnormal Gait
  • Occasionally she experienced left leg pain and abnormal gait. General physical examination revealed sensorial loss in the L5-S1 regions.[ncbi.nlm.nih.gov]
Mononeuropathy
  • BACKGROUND: Ulnar neuropathy at the elbow (UNE) is the second most common mononeuropathy of the upper extremity. One rare cause of UNE is nerve mass lesions, including intraneural ganglion cysts (IGCs).[ncbi.nlm.nih.gov]
Lethargy
  • Patients taking chemotherapy must be prepared for the side effects that come with any other chemotherapy treatment, such as; hair loss, lethargy, weakness, etc.[en.wikipedia.org]
Somnolence
  • A 29-year-old previously healthy male patient, except for a history of two previous surgeries for ulnar nerve entrapment and progressive left upper extremity weakness, presented with acute onset somnolence.[ncbi.nlm.nih.gov]
Foot Drop
  • He subsequently developed rapidly progressive motor weakness in the same myotome, which caused a foot drop. On examination, his straight-leg raise test was normal.[ncbi.nlm.nih.gov]

Workup

  • Imaging of the entire neuraxis is recommended for initial workup of these lesions, which are capable of intradural spread.[ncbi.nlm.nih.gov]
  • Radiographic evaluation is extremely important for metastatic workup of MPNST, being an aggressive tumor. Prognosis remains poor despite all treatments. Financial support and sponsorship Nil.[urologyannals.com]
  • Metastatic workup in the form of computerized tomography scan of chest, whole body bone scan, and bone marrow aspirate and biopsy was essentially normal.[ijmpo.org]
  • All of the colonic lesions were discovered as small (0.2-1.0 cm) polyps during the time of colonoscopy (3 at the time of routine screening, 2 for the workup of occult blood in the stool).[thedoctorsdoctor.com]

Treatment

  • The first-line treatment is surgical resection with wide margins. Chemotherapy (e.g. high-dose doxorubicin) and often radiotherapy are done as adjuvant and/or neoadjuvant treatment.[en.wikipedia.org]
  • Our case suggests that retroperitoneal MPNSTs can deteriorate rapidly even if NF1 independent, that aggressive treatment may not benefit large high-grade MPNSTs, and that novel and effective treatment is urgently needed.[ncbi.nlm.nih.gov]
  • All 21 patients showed treatment failure, with median time to treatment failure 7.8 months (range 2.1–128 months).[doi.org]

Prognosis

  • The prognosis of malignant peripheral nerve sheath tumor (MPNST) with neurofibromatosis type 1 (NF-1) is worse than that of a solitary MPNST, because of the tumor size and location difficult to resect completely.[ncbi.nlm.nih.gov]

Etiology

  • Nerve Sheath Neoplasms/genetics Neurofibroma, Plexiform/drug therapy* Neurofibroma, Plexiform/etiology Neurofibroma, Plexiform/genetics Neurofibromatosis 1/complications Neurofibromatosis 1/genetics Neurofibromin 1/genetics Nuclear Proteins/metabolism[ncbi.nlm.nih.gov]
  • (Etiology) The exact cause and mechanism of formation of Malignant Peripheral Nerve Sheath Tumor is unknown They are thought to occur due to chromosomal defects occurring on account of certain genetic mutations Approximately 3-25% of neurofibromas can[dovemed.com]
  • 7], [8], [9] It is an aggressive sarcoma of neural origin showing a close association with peripheral nerve or neurofiboma or may show features of neural differentiation. [2] Its development is thought to be a multistep and multigene process with an etiology[ijdr.in]
  • His surgical history was notable for removal of a gastrointestinal stromal tumor (GIST) 3 years earlier and excision of a soft-tissue mass of unknown etiology from his neck 15 years earlier. The patient appeared healthy with no evidence of cachexia.[mdmag.com]

Epidemiology

  • Introduction Overview arises from a peripheral nerve or neurofibroma also known as neurofibrosarcoma or malignant schwannoma Epidemiology demographics 30 to 55 years for solitary neurofibromas 20 to 40 years for neurofibromatosis type 1 (NF-1) Genetics[orthobullets.com]
  • From Wikibooks, open books for an open world Jump to navigation Jump to search Malignant Peripheral Nerve Sheath Tumor Epidemiology [ edit ] Account for 5-10% of soft tissue tumors Majority arise de novo or from neurofibromas; prior RT (median 15 years[en.wikibooks.org]
  • […] sheath tumours in neurofibromatosis 1 D G R Evans 1, M E Baser 2, J McGaughran 1, S Sharif 1, E Howard 1, A Moran 3 1 University Department of Medical Genetics, St Mary's Hospital, Manchester M13 0JH, UK 2 Los Angeles, California, USA 3 Centre for Cancer Epidemiology[doi.org]
  • Pediatric and adultmalignant peripheral nerve sheath tumors: an analysis of data from the surveillance, epidemiology, and end results program. J Neurooncol. 2014;116(3):609–616. 5. Demir HA, Varan A, Yalçn B, Akyüz C, Kutluk T, Büyükpamukçu M.[dovepress.com]
Sex distribution
Age distribution

Pathophysiology

  • Significant advances in the pathophysiologic features of NF1 have led to considering this new therapeutic approach.[doi.org]

Prevention

  • Current medical research have not established a way of preventing Malignant Peripheral Nerve Sheath Tumor Regular medical screening at periodic intervals with blood tests, scans, and physical examinations, are mandatory, due to its high metastasizing[dovemed.com]
  • There is considerable evidence to suggest that adjuvant radiation, but not adjuvant chemotherapy, is helpful in preventing local recurrence of MPNST. Postoperative follow-up strategies for MPNST vary greatly across practitioners.[jdc.jefferson.edu]
  • The mass was removed surgically in its entirety and radiation therapy was applied locally to prevent tumor regrowth. Nonetheless, the patient died 10 months after surgery from metastases to the lung and brain.[karger.com]
  • Epigenetic induction of the Ink4a/Arf locus prevents Schwann cell overproliferation during nerve regeneration and after tumorigenic challenge. Brain. 2013. 136: 2262-78 10.[surgicalneurologyint.com]
  • ., Cary, NC, USA) and EpiInfo v3.4.2 (Centers for Disease Control and Prevention, Atlanta, GA, USA). Results We included data for 21 patients (9 females; median age 31 years [range 18–79 years]). Patient characteristics are in Table 1.[doi.org]

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