Intermediate Follow-up and Management of Previously Reported Malignant Peripheral Nerve Sheath Tumor of the Penis

Urology ◽  
2020 ◽  
Vol 135 ◽  
pp. 133-135 ◽  
Author(s):  
Hannah Agard ◽  
Neel Parekh ◽  
Curtis Clark ◽  
Eric Massanyi ◽  
Ananth Murthy ◽  
...  
Keyword(s):  
Peripheral Nerve ◽  
Nerve Sheath Tumor ◽  
Peripheral Nerve Sheath Tumor ◽  
Nerve Sheath

scholarly journals Cranial and Spinal Malignant Peripheral Nerve Sheath Tumor: A Pathological Enigma

2021 ◽  
Author(s):  
Ujwal Yeole ◽  
K. V. L. Narsinga Rao ◽  
Manish Beniwal ◽  
Sumitra Sivakoti ◽  
Vani Santosh ◽  
...  
Keyword(s):  
Peripheral Nerve ◽  
High Rate ◽  
Subtotal Resection ◽  
Nerve Sheath Tumor ◽  
Peripheral Nerve Sheath Tumor ◽  
Large Size ◽  
Nerve Sheath ◽  
The Mean ◽  
Radical Surgical Resection

Abstract Objective Malignant peripheral nerve sheath tumor (MPNST) arises from nerve sheaths, mostly seen in peripheral nerves but rare in craniospinal nerves. The information available in the literature to build up treatment strategy and improve clinical outcomes is scarce. We are reviewing cases from our institute, with emphasis on radiological features for early differentiation from its benign variants. Methods We analyzed pathologically diagnosed cases retrospectively from January 2007 to December 2018 at our institute. Clinicoradiological details and treatment parameters were collected from medical records for evaluation. Each case was contacted telephonically for final clinical follow-up at the time of writing the manuscript. Results A total of seven cases of MPNST were diagnosed in the last 10 years. It included four intracranial and three spinal cases. The mean age for the cohort was 34.3 years, with five females. We could achieve gross total resection (GTR) and subtotal resection in four (57.1%) and two (28.6%) cases, respectively. We could achieve an overall survival of 57.1% in the average follow-up of 28.2 months (range: 8–84 months). Conclusion MPNST is a rare tumor with a bad prognosis. Radical surgical resection is the mainstay of the treatment, but it is not always possible to achieve it because of the inaccessible location and large size of lesions. Preoperative diagnosis is challenging; however, few radiological findings may give a clue toward it. As a disease entity overall, it has a poor outcome with a high rate of fatality.

scholarly journals Malignant peripheral nerve sheath tumor of nasal cavity and paranasal sinus with 13 years of follow‐up—A case report and review of literature

2019 ◽  
Vol 7 (11) ◽  
pp. 2194-2201
Author(s):  
Toms Vengaloor Thomas ◽  
Anu Abraham ◽  
Eldrin Bhanat ◽  
Youssef Al Hmada ◽  
Ashley Albert ◽  
...  
Keyword(s):  
Case Report ◽  
Peripheral Nerve ◽  
Nasal Cavity ◽  
Paranasal Sinus ◽  
Nerve Sheath Tumor ◽  
Review Of Literature ◽  
Peripheral Nerve Sheath Tumor ◽  
Nerve Sheath

scholarly journals Metastatic malignant peripheral nerve sheath tumor. A diagnostic surprise

2021 ◽  
Vol 91 (2) ◽  
Author(s):  
Muniza Bai ◽  
Vishnukanth Govindaraj ◽  
Abhishek Singh Chauhan ◽  
Bheemanati Hanuman Srinivas ◽  
Jinkala Sree Rekha ◽  
...  
Keyword(s):  
Peripheral Nerve ◽  
Local Therapy ◽  
Distant Metastases ◽  
Soft Tissue Tumors ◽  
Cardiac Metastasis ◽  
Nerve Sheath Tumor ◽  
Peripheral Nerve Sheath Tumor ◽  
Left Hand ◽  
Nerve Sheath

Malignant peripheral nerve sheath tumours (MPNSTs) are rare soft tissue tumors that arise from pre-existing plexiform neurofibromas or within a normal peripheral nerve. They are aggressive tumors with high rates of recurrence and distant metastases, the most common sites of metastasis being the lung followed by bone.A 46 year old gentleman presented with breathlessness and chest pain three years after post amputation of left thumb for an ulcerative growth. CECT thorax showed a left upper lobe mass with pleural and pericardial effusion. Within a month of presentation he worsened and succumbed to the disease. Antemortem biopsy of the left hand ulcerative growth showed features suggestive of malignant peripheral nerve sheath tumour- epithelioid variant and post mortem liver and lung biopsy showed metastasis of MPNST. The diagnosis was a malignant peripheral nerve sheath tumor with lung, liver and cardiac metastasis. This case report aims to highlight the importance of upfront aggressive multimodality local therapy for achieving local disease control in patients presenting with localised MPNST and regular follow up for early detection of relapse and metastasis.

Malignant Peripheral Nerve Sheath Tumor Arising from a Solitary Neurofibroma in a Patient without Neurofibromatosis Type 1

2016 ◽  
Vol 78 (5) ◽  
pp. 516-521
Author(s):  
Fumihisa SAWADA ◽  
Eiichi MAKINO ◽  
Takenobu YAMAMOTO ◽  
Ryo TANAKA ◽  
Yutaka FUJIWARA ◽  
...  
Keyword(s):  
Peripheral Nerve ◽  
Neurofibromatosis Type 1 ◽  
Neurofibromatosis Type ◽  
Nerve Sheath Tumor ◽  
Peripheral Nerve Sheath Tumor ◽  
Nerve Sheath ◽  
Solitary Neurofibroma

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

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.

Chromosomal translocations inactivating CDKN2A support a single path for malignant peripheral nerve sheath tumor initiation

2021 ◽  
Author(s):  
Miriam Magallón-Lorenz ◽  
Juana Fernández-Rodríguez ◽  
Ernest Terribas ◽  
Edgar Creus-Batchiller ◽  
Cleofe Romagosa ◽  
...  
Keyword(s):  
Peripheral Nerve ◽  
Chromosomal Translocations ◽  
Nerve Sheath Tumor ◽  
Tumor Initiation ◽  
Peripheral Nerve Sheath Tumor ◽  
Nerve Sheath ◽  
Single Path
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