scholarly journals Spinal Canal Involvement in Solitary Infantile Myofibromatosis: Case Report

2017 ◽  
Vol 06 (03) ◽  
pp. 223-227
Author(s):  
S.N. Gautam ◽  
Siddharth Mittal
Keyword(s):  
Spinal Canal ◽  
Smooth Muscle Actin ◽  
Lower Limbs ◽  
Nerve Sheath Tumor ◽  
Infantile Myofibromatosis ◽  
Nerve Sheath ◽  
Biopsy Report ◽  
S 100 ◽  
History Of ◽  
Intradural Space

AbstractInfantile myofibromatosis involving the spinal canal is very rare; only 11 cases have been reported so far in the literature. The authors present a case of an 18-month-old male child who presented with the history of dribbling of urine and weakness in bilateral lower limbs since 2 months. MRI of spine revealed single intramedullary intradural space-occupying lesion (SOL) at D1 to D2.The patient underwent laminectomy with excision of SOL with biopsy report suggestive of benign nerve sheath tumor, and immunohistochemistry report revealed desmin negative, smooth muscle actin positive, and S-100 focally positive infantile myofibromatosis. The patient gradually recovered and had a clear stream of urine with improved movements and tone of bilateral lower limbs at the time of discharge.

Cerebral Malignant Nerve Sheath Tumor, Triton Tumor Variant: Case Report

2003 ◽  
Vol 6 (2) ◽  
pp. 168-172 ◽  
Author(s):  
Leticia Bornstein-Quevedo ◽  
Fabiola Peralta-Olvera ◽  
Alfonso Marhx-Bracho ◽  
Rodolfo Rodríguez-Jurado ◽  
Beatriz de Leon-Bojorge
Keyword(s):  
Clinical Evidence ◽  
Immunohistochemical Study ◽  
Nerve Sheath Tumor ◽  
Intraparenchymal Hemorrhage ◽  
Malignant Triton Tumor ◽  
Nerve Sheath ◽  
Spindle Cells ◽  
S 100 ◽  
History Of ◽  
The Right

A case of a cerebral malignant triton tumor in a 3-year-old boy with a 2-month history of frontal headache and no clinical evidence of neurofibromatosis is reported. The computed tomography (CT) scan showed a large, irregular tumor in the right parietooccipital lobe. A partial surgical resection was performed. Histologically, the tumor was highly cellular and consisted of spindle cells with hyperchromatic and pleomorphic nuclei. Focally, neoplastic cells with rhabdomyoblastic features were found. The immunohistochemical study showed that tumor cells were positive for S-100 protein and CD57, and the rhabdomyoblasts expressed desmin, Myo-D1, and myoglobin. During the postoperative period, a massive intraparenchymal hemorrhage was identified and surgical drainage was performed. The patient worsened and died 10 days after the first surgery. Postmortem study was not authorized. Six cases of cerebral malignant nerve sheath tumor have been described; however, primary intraparenchymal malignant triton tumor has not been previously described.

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.

scholarly journals Metastatic small cell carcinoma of the lung with prominent spindle cell morphology and hemangiopericytoma-like vascular pattern: A sarcoma mimicker

2020 ◽  
Vol 8 ◽  
pp. 2050313X2098117
Author(s):  
Esra Nsour ◽  
Ali Al Khader ◽  
Bushra Al-Tarawneh
Keyword(s):  
Cell Carcinoma ◽  
Small Cell Carcinoma ◽  
Spindle Cell ◽  
Small Cell ◽  
Nerve Sheath Tumor ◽  
Vascular Pattern ◽  
Nerve Sheath ◽  
History Of ◽  
Wall Mass ◽  
Aggressive Clinical Behavior

Small cell carcinoma is a malignant neuroendocrine tumor with aggressive clinical behavior. Histologically, the tumor is characterized by the proliferation of small, round, blue cells. Here, we present the case of a 50-year-old man with a 1-month history of enlarging chest wall mass. Microscopic examination of the lesion revealed a highly cellular neoplasm composed of closely packed, atypical spindle cells with scant cytoplasm, inconspicuous nucleoli, and brisk mitotic activity. The hemangiopericytoma-like vascular pattern was prominent. Areas showing a fibrosarcoma-like fascicular pattern were also observed. The tumor was immunohistochemically positive for TTF1, synaptophysin, and chromogranin, confirming small cell carcinoma. Further investigations revealed a lung origin and widespread metastases. The tumor in this case closely mimicked synovial sarcoma or malignant peripheral nerve sheath tumor. Small cell carcinoma demonstrates a hemangiopericytoma-like pattern that can mimic sarcoma histologically. This is a serious pitfall that can significantly affect the speed of diagnosis and management.

scholarly journals Malignant Peripheral Nerve Sheath Tumor of the Infraorbital Nerve

2016 ◽  
Vol 9 (2) ◽  
pp. 170-174
Author(s):  
JoséLuis D'Addino ◽  
Laura Piccoletti ◽  
MaríaMercedes Pigni ◽  
Maria José Rodriguez Arenas de Gordon
Keyword(s):  
Peripheral Nerve ◽  
Chronic Atrial Fibrillation ◽  
Radical Resection ◽  
Infraorbital Nerve ◽  
Malignant Schwannoma ◽  
Ocular Motility ◽  
Nerve Sheath Tumor ◽  
Peripheral Nerve Sheath Tumor ◽  
Nerve Sheath ◽  
History Of

The objective of this study is to report a large, rare, and ulcerative infiltrated skin lesion. Its diagnosis, therapeutic management, and progress are described. The patient is a 78-year-old white man, who presented with a 12-month ulcerative perforated lesion that had affected and infiltrated the skin, with easy bleeding. He had a history of hypertension, although controlled, was a 40-year smoker, had chronic atrial fibrillation, diabetes, and microangiopathy. During the consultation, the patient also presented with ocular obstruction due to an inability to open the eye. He mentioned having reduced vision. The computed tomography scan showed upper maxilla osteolysis without eye involvement. We underwent a radical resection in which upper maxilla and the anterior orbital margin were included. We used a Becker-type flap that allowed us to rebuild the cheek and to complete a modified neck dissection. Progress was favorable; the patient recovered ocular motility and his vision improved to 20/200. The final biopsy result was “malignant peripheral nerve sheath tumor, malignant schwannoma.” Malignant schwannoma of the peripheral nerve is extremely rare. The total resection and reconstruction being completed in one surgery represented a challenge due to the difficulty in obtaining tissues in addition to the necessity of an oncological resection.

scholarly journals Retrospective canine skin peripheral nerve sheath tumors data with emphasis on histologic, immunohistochemical and prognostic factors

2015 ◽  
Vol 35 (12) ◽  
pp. 965-974 ◽  
Author(s):  
Gisele S. Boos ◽  
Daniele M. Bassuino ◽  
Fabiana Wurster ◽  
Neusa B. Castro ◽  
Tatiane T.N. Watanabe ◽  
...  
Keyword(s):  
Peripheral Nerve ◽  
Protein S ◽  
Skin Neoplasms ◽  
Tumor Location ◽  
Benign Tumors ◽  
Nerve Sheath Tumor ◽  
Nerve Sheath Tumors ◽  
Peripheral Nerve Sheath Tumors ◽  
Nerve Sheath ◽  
S 100

Abstract: In this retrospective study was determined the frequency of canine skin peripheral nerve sheath tumors (PNST) in cases diagnosed by the Setor de Patologia Veterinária of the Universidade Federal do Rio Grande do Sul (SPV-UFRGS), Brazil, between the years 2000 and 2012. The canine profiles, as well as histological, immunohistochemical and prognostic aspects of the tumors were based on 70 samples, comprising 40 females, 29 males and one unspecified sample. Between 2000 and 2012, 2,984 skin tumors of dogs were diagnosed in the SPV-UFRGS, totaling 2.34% of skin neoplasms in dogs. Animals that comprised the largest amount of samples (43%) were those with no breed (SRD), followed by German Shepherds (10%). Females were more affected than males (40/70 - 57% and 29/70 - 41% respectively). Skin PNST of this research showed predominant localization on the limbs (40% in the forelimbs and 29% in the hindlimbs); affecting adult dogs, mostly aged between 8 and 11 years (54%). The samples were routinely processed for hematoxylin and eosin, and were also evaluated by toluidine blue and Masson's trichrome staining, and immunohistochemistry (IHC) anti-vimentin, -S-100, -GFAP, -actin, von Willebrand factor and neurofilament. Anisocytosis and anisokaryosis, mitotic index, intratumoral necrosis, invasion of adjacent tissues, tumor location, local recurrence and metastasis were related to the diagnosis of benign (49/70) or malignant tumor (21/70). The Antoni A histological pattern was observed more frequently in benign tumors. The immunohistochemistry helped to diagnose PNST, and anti-vimentin and anti-protein S-100 showed the highest rates of immunostaining. Throughout statistical analysis of animals with tumor recurrence, it was found that the chance of an animal with a malignant peripheral nerve sheath tumor to develop recurrence is 4.61 times higher than in an animal that had a benign tumor.

scholarly journals A Review of the Literature on Primary Leiomyosarcoma of the Prostate Gland

2015 ◽  
Vol 2015 ◽  
pp. 1-15 ◽  
Author(s):  
Anthony Kodzo-Grey Venyo
Keyword(s):  
Cytogenetic Study ◽  
Smooth Muscle Actin ◽  
Prostate Gland ◽  
Hormonal Treatment ◽  
Previous Treatment ◽  
Multicenter Trial ◽  
Perineal Pain ◽  
S 100 ◽  
History Of ◽  
Primary Leiomyosarcoma

Primary leiomyosarcoma of the prostate (PLSOP) is rare, with less than 200 cases reported so far. PLSOPs present with lower urinary tract symptoms, haematuria, and perineal pain; may or may not be associated with a history of previous treatment for adenocarcinoma of prostate by means of radiotherapy and or hormonal treatment; may afflict children and adult male. Examination may reveal benign enlarged prostate and hard enlarged mass. PLSOPs may be diagnosed by histological examination findings of spindle-shaped carcinoma cells in prostate specimens. Immunohistochemical staining tends to be positive for vimentin, CD44, smooth muscle actin, and calponin, focally positive for desmin, and at times positive for keratin. They stain negatively for PSA, S-100, CD34, CD117, and cytokeratin. Cytogenetic study on primary leiomyosarcoma of the prostate gland may show clonal chromosomal rearrangement involving Chromosomes 2, 3, 9, 11, and 19. On the whole the prognosis is poor. Surgery with or without chemotherapy would appear to be the mainstay of treatment for PLSOPs that are operable, but generally there is no consensus opinion on the best therapeutic approach. Most cases of PLSOPs are diagnosed in an advanced stage of the disease. A global multicenter trial is required to find therapies that would improve the prognosis.

Primary malignant peripheral nerve sheath tumor of the spine with acute hydrocephalus: a rare clinical entity

2014 ◽  
Vol 21 (3) ◽  
pp. 367-371 ◽  
Author(s):  
Yaxiong Li ◽  
Fengshi Fan ◽  
Jianguo Xu ◽  
Jie An ◽  
Weining Zhang
Keyword(s):  
Peripheral Nerve ◽  
English Language ◽  
Standard Of Care ◽  
Nerve Sheath Tumor ◽  
Nerve Sheath Tumors ◽  
Peripheral Nerve Sheath Tumors ◽  
Nerve Sheath ◽  
History Of ◽  
Brain Ct Scan ◽  
The Right

Primary malignant peripheral nerve sheath tumors (MPNSTs) are extremely rare in patients without a history of neurofibromatosis; only 18 cases have been reported in the English-language literature to this point. The authors report their experience with 1 new case of a primary MPNST. A 33-year-old woman presented with low-back pain radiating to the right calf that progressed over 1 year. Magnetic resonance imaging of the spine revealed an intradural extramedullary lesion at the T12–L1 level. The patient was diagnosed with primary MPNST, underwent two surgical excisions and radiation therapy, and developed leptomeningeal metastases as well as brain metastases. The patient revisited the emergency room with sudden loss of consciousness. A brain CT scan displayed bilateral lateral ventricle enlargement, for which a ventriculoperitoneal shunt was inserted. These symptoms have not been described in any previous report. Primary spinal MPNST is an exceedingly rare entity, and the overall prognosis is very poor. To the authors' knowledge, no standard of care for primary spinal MPNSTs has yet been established. All 19 cases of primary spinal MPNSTs are reviewed, and the authors discuss their clinical, radiological, and therapeutic features and outcomes.

Spinal malignant nerve-sheath tumor or cellular schwannoma? A striking difference in prognosis

1993 ◽  
Vol 79 (4) ◽  
pp. 528-532 ◽  
Author(s):  
Matti T. Seppälä ◽  
Matti J. J. Haltia
Keyword(s):  
Malignant Tumors ◽  
Spinal Tumors ◽  
Striking Difference ◽  
Malignant Neoplasms ◽  
Nerve Sheath Tumor ◽  
Content Type ◽  
Nerve Sheath Tumors ◽  
Nerve Sheath ◽  
Cellular Schwannoma ◽  
S 100

✓ Cellular schwannoma, a recently delineated entity, has a histological appearance mimicking that of malignant neoplasms. The aim of this study was to determine the outcome for patients treated for a spinal cellular schwannoma or malignant nerve-sheath tumor. A histological re-examination was conducted of 283 spinal tumors, considered to originate from a nerve root, that were treated in the Department of Neurosurgery between 1953 and 1985. After re-examination, 50 of these were determined to be other tumors or nonneoplastic lesions. The review yielded eight cellular schwannomas and six malignant nerve-sheath tumors out of 233 of nerve-sheath origin. Immunohistochemical staining with a commercially available polyclonal antibody against S-100 protein was positive in all cases of cellular schwannoma, but negative for the malignant tumors. Clinical outcome was favorable for patients with cellular schwannomas, but uniformly poor for those with the malignant tumors.

Recurrence of acoustic neurilemoma as a malignant spindle-cell neoplasm

1990 ◽  
Vol 73 (6) ◽  
pp. 946-950 ◽  
Author(s):  
Catriona A. McLean ◽  
John D. Laidlaw ◽  
David S. B. Brownbill ◽  
Michael F. Gonzales
Keyword(s):  
Cerebellopontine Angle ◽  
Spindle Cell ◽  
Positive Staining ◽  
Nerve Sheath Tumor ◽  
Content Type ◽  
Spindle Cell Neoplasm ◽  
Nerve Sheath ◽  
Cell Neoplasm ◽  
Acoustic Nerve ◽  
S 100

✓ A 75-year-old man presented with a right cerebellopontine angle tumor 11 months after complete macroscopic resection of a right acoustic neurilemoma. Histological examination of the recurrent tumor showed a malignant spindle-cell neoplasm with positive staining for S-100 protein. The patient had no stigmata of von Recklinghausen's disease. It is proposed that this recurrence represents progression from a benign to a malignant acoustic nerve-sheath tumor, an event that is extremely rare outside the clinicopathological context of neurofibromatosis.

scholarly journals Fibrosarcoma of Mandible: A Case Report

2011 ◽  
Vol 2011 ◽  
pp. 1-4
Author(s):  
Monal B. Yuwanati ◽  
J. V. Tupkari
Keyword(s):  
Smooth Muscle Actin ◽  
Third Molar ◽  
Final Diagnosis ◽  
Posterior Teeth ◽  
Mandibular Third Molar ◽  
Mesenchymal Neoplasm ◽  
S 100 ◽  
History Of ◽  
Posterior Area ◽  
Molar Region

Fibrosarcoma is a malignant mesenchymal neoplasm of fibroblasts that rarely affects the oral cavity and can cause local recurrences or metastasis. In this paper, a case of fibrosarcoma in the posterior area of mandible in a 44-year-old woman is described. Clinical examination revealed a growth on right mandibular third molar region extending on the buccal and the lingual side. There was history of extraction of posterior teeth. Radiologically, there was a diffuse bone loss. Microscopically, the tumor showed an intense proliferation of fibroblasts of variable size and shape. These cells were arranged in parallel bands and partly crossing each other. The cells exhibited increased mitotic activity and nuclear pleomorphism. Immunohistochemically the cells showed immunoreactivity only for vimentin while they exhibited negativity towards S-100 protein, cytokeratin cocktail, HMB-45, desmin, smooth muscle actin, and epithelial membrane antigen (EMA). Based on findings the final diagnosis of fibrosarcoma was made.

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