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Malignant peripheral nerve sheath tumor with divergent differentiation  [cached]
Suresh T,Harendra Kumar M,Prasad C.S.B.R,Kalyani R
Indian Journal of Pathology and Microbiology , 2009,
Abstract: A malignant peripheral nerve sheath tumor (MPNST) is an uncommon spindle cell sarcoma accounting for approximately 5% of all soft tissue sarcomas. A 55-year-old female with a right suprarenal tumor showed MPNST with additional foci of epithelioid, rhabdomyoblastic, osteogenic and lipogenic differentiation. Although the capacity of MPNST to undergo epithelioid, rhabdomyoblastic, osteogenic and very rarely lipogenic differentiation is reported in literature, the occurrence of all these differentiation in one case has not been described in literature before. To the best of our knowledge, this is only the second MPNST case with lipomatous differentiation
Malignant peripheral nerve sheath tumor of the breast: case report
Kajal Dhingra, Shramana Mandal, Somak Roy, Nita Khurana
World Journal of Surgical Oncology , 2007, DOI: 10.1186/1477-7819-5-142
Abstract: A 38-year-old female presented with complaints of painless, hard breast lump for three months which was clinically suspected to be a ductal carcinoma with inconclusive fine needle aspiration cytology. Histopathology revealed a malignant spindle cell tumor which was confirmed to be malignant peripheral nerve sheath tumor on the basis of immunopositivity for vimentin, neurone specific enolase and S-100.To the best of our knowledge only six such case reports have been published in literature. The differential diagnosis of malignant peripheral nerve sheath tumor should be considered by the clinician as well as the pathologists in the work-up of a breast neoplasm as treatment and prognosis of this rare malignancy is different.Malignant peripheral nerve sheath tumor (MPNST) is a rare soft tissue sarcoma of the ectomesenchymal origin. It is the malignant counterpart of benign soft tissue tumors like neurofibromas and schwannomas and may follow them. Common sites include deeper soft tissues, usually in the proximity of a nerve trunk. MPNST in the breast is often unsuspected and the diagnosis may be missed unless clinical suspicion is high and immunohistochemistry carried outA 38-year old female patient presented to the surgical out patient department with complaints of a rapidly growing right breast lump for three months. There was no history of prior breast mass, pain, trauma, bleeding, discharge, or family history of breast cancer. On examination there was a single 3.5 × 3 × 3 cm, ill defined non tender, firm, fixed mass in the right upper outer quadrant. There was no retraction or ulceration of the overlying skin. Fine needle aspiration cytology (FNAC) suggested a malignant spindle cell tumor probably a malignant phylloides. An excisional biopsy was advised to confirm the diagnosis and to guide the further management.Grossly the specimen was a skin covered lumpectomy specimen measuring 5 × 4.4 × 4 cms. The cut sections revealed a firm infiltrative unencapsulated gray whi
Malignant Peripheral Nerve Sheath Tumor in the Maxilla: Report of a Rare Case
Jahanshah Salehinejad,Atefeh Nasseh,Amir Hossein Jafarian,Nazanin Bashardoust
Journal of Dental Materials and Techniques , 2013,
Abstract: Malignant peripheral nerve sheath tumor (MPNST) is a rare malignant tumor that develops either from a preexisting neurofibroma or de novo. The cell of origin is believed to be the Schwann cell and possibly other nerve sheath cells. In this report, we describe a rare case of MPNST that arise from the socket of second left maxillary molar that has been already extracted in a young man. He was referred to a dentist’s office with a tumor-like mass of soft tissue on his left maxillary gingiva. Biopsy and histopathologic examination was performed and based on histologic and immuno-histochemical findings, the diagnosis of MPNST was made. MPNST is a rare malignant tumor in the oral cavity. Dentists must be careful and conscious because this rare malignancy can occur in gingiva and can mimic the clinical feature of any benign gingival enlargements.
Malignant peripheral nerve sheath tumor in a patient with Werner’s syndrome
M. A. Rosa,G. Falcone,V. De Santis,O. Azzaro
Journal of Orthopaedics and Traumatology , 2003, DOI: 10.1007/s10195-003-0018-1
Abstract: Werner’s syndrome is a rare autosomal recessive disorder characterized by features of premature aging. It is attributed to an autosomal recessive mutation on chromosome 8p12 where the Werner gene is located. This gene encodes a protein homologous to the RecQ-type DNA helicase, an enzyme that unwinds double-stranded DNA into single-stranded DNA. The association of Werner’s syndrome with malignant tumors is widely reported in the literature. Both bone and soft tissue sarcomas have been described. We report a patient with Werner’s syndrome who developed a malignant peripheral nerve sheath tumor of the left popliteal fossa.
Malignant peripheral nerve sheath tumor in a patient with Werner's syndrome
M. A. Rosa,G. Falcone,V. De Santis,O. Azzaro
Journal of Orthopaedics and Traumatology , 2003, DOI: 10.1007/BF02637324
Abstract: Werner's syndrome is a rare autosomal recessive disorder characterized by features of premature aging. It is attributed to an autosomal recessive mutation on chromosome 8p12 where the Werner gene is located. This gene encodes a protein homologous to the RecQ-type DNA helicase, an enzyme that unwinds double-stranded DNA into single-stranded DNA. The association of Werner's syndrome with malignant tumors is widely reported in the literature. Both bone and soft tissue sarcomas have been described. We report a patient with Werner's syndrome who developed a malignant peripheral nerve sheath tumor of the left popliteal fossa.
Malignant peripheral nerve sheath tumor of the cervical vagus nerve in a neurofibromatosis type 1 patient - An unusual presentation  [cached]
Ashok Gupta,Sandeep Bansal,Sanjeev Bhagat,Amanjit Bahl
Online Journal of Health & Allied Sciences , 2010,
Abstract: Malignant peripheral nerve sheath tumors (MPNST’S) of the head and neck comprise 2% to 6% of head and neck sarcomas. These tumors may arise as sporadic variants or in patients with neurofibromatosis (NF). Development of these MPNST’s is one of the serious complications of neurofibromatosis type 1(NF1). To our knowledge there are only two reported cases of MPNST’s arising in the cervical vagal nerve, occurring in NF1 patients. We present here an NF1 patient who developed an MPNST of the cervical vagus nerve and presented only with a cervical swelling and hoarseness.
Malignant Peripheral Nerve Sheath Tumor of the Small Bowel: An Unusual Presentation with Fatal Outcome  [PDF]
Amina Mohtaram,Siham Mesmoudi,Hind M’rabti,Amal Rami,Rachida Latib,Zakia Bernoussi,Imane Aaribi,Meryam Ben Ameur El Youbi,Hassan Errihani
Case Reports in Oncological Medicine , 2013, DOI: 10.1155/2013/423867
Abstract: Malignant peripheral nerve sheath tumor of the small bowel is an extremely rare disease. Histologic distinction from other types of soft tissue sarcoma especially fibrosarcoma and leiomyosarcoma requires electron microscopy. Complete surgery remains the only curative treatment. However, late diagnosis makes curative surgery more difficult. The contribution of chemotherapy to incomplete surgery has been proved without controlled studies. We report a case of this type of lesion discovered following a small bowel perforation. 1. Introduction Malignant peripheral nerve sheath tumors are defined as any tumor arising from a peripheral nerve or exhibiting nerve sheath differentiation. The incidence of malignant peripheral nerve sheath tumors is 1/100?000, corresponding to 3–12% of soft tissue sarcomas [1]. They are commonly arising on the trunk, extremities, head and neck, and paravertebral region [2]. Malignant peripheral nerve sheath tumors arising from the gastrointestinal tract are extremely rare, with a few cases being reported in the literature for the small bowel [2, 3]. Because the clinical symptoms in this localization are usually nonspecific, the diagnostic is often late and makes wide excision more difficult. In this report, we describe a malignant peripheral nerve sheath tumors of small bowel discovered following an intestinal perforation. 2. Case Report A 18-year-old patient without pathological antecedents especially neurofibromatosis presented with abdominal pain associated with diarrhea and vomiting. The clinical examination revealed an abdominal defence. The patient underwent emergency surgery for peritonitis. At laparotomy, the surgeon discovered a tumour in the proximal jejunum associated with peritonitis in the right hypochondrium and carcinomatosis implants of the colon, omentum, and mesentery. Tumor excision with 40?cm resection of the small intestine was performed. Macroscopic examination showed a 40?cm resected small bowel, containing a tumor measuring 20 × 19 × 12 with parietal development. The tumor had a gray-white appearance with small cysts and a few areas of hemorrhage. Histologically, the tumor showed malignant spindle cells. The cytoplasm was scant abundant with anisokaryosis. The mitotic activity was 9 mitoses per 10 high-power fields. Myxoid stroma reaction was found, and there were negative surgical margins (Figure 1). Figure 1: Microscopic findings of small bowel showing (a) cellular spindle cell tumor in fascicles with (b) cytonuclear atypia and high mitotic index (HE ×40). On immunohistochemical staining, the tumor was
Overexpression of Cyclooxygenase-2 in Malignant Peripheral Nerve Sheath Tumor and Selective Cyclooxygenase-2 Inhibitor-Induced Apoptosis by Activating Caspases in Human Malignant Peripheral Nerve Sheath Tumor Cells  [PDF]
Michiyuki Hakozaki, Takahiro Tajino, Shinichi Konno, Shinichi Kikuchi, Hitoshi Yamada, Michiro Yanagisawa, Jun Nishida, Hiroyuki Nagasawa, Takashi Tsuchiya, Akira Ogose, Masafumi Abe, Hiroshi Hojo
PLOS ONE , 2014, DOI: 10.1371/journal.pone.0088035
Abstract: Background Cyclooxygenase-2 (COX-2) is a key enzyme in the conversion of arachidonic acid to prostanoids, and its activation is associated with carcinogenesis as well as inflammation. The antitumor effect of selective COX-2 inhibitors has been noted in various malignancies. Malignant peripheral nerve sheath tumor (MPNST) is a rare and aggressive soft tissue sarcoma for which effective treatments have not yet been established. The purpose of this study was to investigate a potential therapeutic role of COX-2 in MPNST. Methods We evaluated the expression of COX-2 in 44 cases of high-grade MPNST using immunohistochemical staining and compared the staining results with the characteristics and outcome of the patients. We also investigated the antitumor effect of etodolac, a selective COX-2 inhibitor, on MPNST cells in vitro using the MPNST cell line, FMS-1. Results Overexpression of COX-2 (≥50% positive cells) was observed in 29 cases (65.9%), was significantly associated with a poor overall survival (P = 0.0495), and was considered an independent risk factor for a poor outcome by the results of both univariate and multivariate analysis. Etodolac induced apoptosis of FMS-1 cells through the activation of caspase-8, -9, and -3. Moreover, several caspase inhibitors significantly inhibited etodolac-induced apoptosis. Conclusions Selective COX-2 inhibitors including etodolac had an antitumor effect on MPNST cells, and their use holds promise as a novel therapeutic strategy for patients with MPNST to improve their prognoses.
Retroperitoneal Malignant Peripheral Nerve Sheath Tumor Replacing an Absent Kidney in a Child  [PDF]
Samin Alavi,M. T. Arzanian,Yalda Nilipour
Case Reports in Oncological Medicine , 2013, DOI: 10.1155/2013/627472
Abstract: Malignant peripheral nerve sheath tumors (MPNSTs) are nonrhabdomyosarcoma soft tissue sarcomas with rare occurrence in children specially in the retroperitoneum. We describe a young child who presented with an abdominal mass. Both ultrasound and computed tomography revealed a large right-sided abdominal mass in the anatomic place of right kidney, while no kidney or ureter was observed at that side. He underwent surgical resection of the tumor with a primary impression of Wilms tumor. To the authors’ knowledge, this is the first case of retroperitoneal malignant peripheral nerve sheath tumor and absent kidney. This case suggests the very rare probability of association of MPNSTs in children with genitourinary tract anomalies such as renal agenesis. 1. Introduction Soft tissue sarcomas (STSs) account for less than 1% of all cancer diagnoses in the general population. They are more common in children, representing approximately 7% of all cancers in patients younger than 20 years. Rhabdomyosarcomas comprise approximately half of the pediatric STSs. The remaining known as nonrhabdomyosarcoma soft tissue sarcomas (NRSTSs) are a heterogeneous group of neoplasms [1]. Malignant peripheral nerve sheath tumors (MPNST) are defined as any malignant tumor deriving from or differentiating into cells of the peripheral nerve sheaths with nonspecific symptoms and a high risk of local recurrence and distant metastasis [2]. MPNST is particularly rare with an incidence of 0.001% in the general population [1, 2]. It is a very rare spindle cell sarcoma in children accounting for approximately 5–10% of nonrhabdomyosarcoma soft tissue sarcomas [2, 3]. Herein, we report the first case of retroperitoneal MPNST that was associated with absent right kidney and compare our findings with the previous reports in the literature. 2. Case Presentation A 12-year-old boy was referred to pediatric clinic with abdominal pain lasting for one month. He was also complaining from nausea and vomiting during the week prior to his referral. On physical examination a huge abdominal mass mainly in the right side of the abdomen was detected. Abdominal ultrasonography showed a large solid/cystic, fluid filled mass measuring ?cm in upper abdomen assumed to be originated from right kidney. In computed tomography with contrast the right kidney was not visualized, but a huge heterogeneous cystic, septated mass measuring ?cm in the anatomic area of right kidney, extending upwardly to the diaphragm and liver and anteriorly to the pancreas, was noticed. Absence of right kidney was consistent with its
Intraosseous malignant peripheral nerve sheath tumor with focal epithelioid differentiation of the thoracic spine  [cached]
Miyakoshi Naohisa,Nishikawa Yuji,Shimada Yoichi,Okada Kyoji
Neurology India , 2007,
Abstract: The authors describe an extremely rare case with malignant peripheral nerve sheath tumor (MPNST) with focal epithelioid differentiation presenting as an intraosseous lesion of the spine. A 75-year-old woman presented with progressive paraplegia caused by epidural mass arising from the posterior element of the T7 vertebra. At surgery, the lesion was noted to originate from the T7 vertebra and separate from the dura and spinal nerve roots. The patient died of tumor metastases to the lungs six months after the initial presentation. Histological diagnosis was MPNST. However, the tumor also contained cystic structures lined by epithelioid cells, requiring differentiation from synovial sarcoma. From the histological and immunohistochemical features, as well as the absence of SYT-SSX fusion gene expression, the diagnosis of MPNST with focal epithelioid differentiation was made. This is the first case report of intraosseous MPNST of the spine with a peculiar biphasic appearance.
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