Anterior abdominal wall malignant peripheral nerve sheath tumor in an infant

Oğuzkurt, Pelin; Kayaselçuk, Fazi̇let; Arda, İrfan Serdar; Barutçu, Özlem; Tuncer, İlhan; Öz, Sedat

doi:10.1053/jpsu.2001.28875

Cited by 12 publications

(6 citation statements)

References 8 publications

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“…sites of occurrence are at a spinal nerve root, the sciatic nerve, brachial plexus, sacral plexus, as well as peripheral nerves [2,18]. Other uncommonly reported sites of MPNST include: pancreas [19], thyroid [20], tongue [21], bile duct [22], skin [23], prostate [24], breast [25], mediastinum [26], rectum [27], colon [28], cervix [29], mesentery [30], liver [31], abdominal wall [32]. The sites of presentation do not differ significantly in NF1 and non-NF1 patients [2].…”

Section: Demographicsmentioning

confidence: 99%

Malignant peripheral nerve sheath tumor: Molecular pathogenesis and current management considerations

Grobmyer¹,

Reith²,

Shahlaee³

et al. 2008

Journal of Surgical Oncology

182

164

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Malignant Peripheral Nerve Sheath Tumors (MPNSTs) are rare tumors that often occur in patients with neurofibromatosis 1. Surgical resection represents the mainstay of treatment. Radiation and chemotherapy have a role in selected patients with MPNST. Accurate pathologic diagnosis remains a challenge in many cases of MPNST. There are many recent advances in the understanding of the molecular pathogenesis of MPNST which represent the best opportunities to develop new strategies for management of patients with MPNST.

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Section: Demographicsmentioning

confidence: 99%

Malignant peripheral nerve sheath tumor: Molecular pathogenesis and current management considerations

Grobmyer¹,

Reith²,

Shahlaee³

et al. 2008

Journal of Surgical Oncology

182

164

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“…The vast majorities of these lesions are localized and have no association with NF1, althoughany of these lesions may occur in NF1 [4]. Localized neurofibromas are well-delineated, firm lesions with a white and shiny surface and may appear in the dermis or subcutaneous tissues.…”

Section: Discussionmentioning

confidence: 99%

10.4021/jocmr2009.09.1264

et al. 2009

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Malignant schwannoma of the anterior abdominal wall nerves is extremely rare. Malignant peripheral nerve sheath tumors (MPNST) represent approximately 10% of all soft tissue sarcomas and it is found in 4% of patients with neurofibromatosis 1. We present a case of malignant schwannoma in a 28-year-old female patient with neurofibromatosis 1. She presented with a painful mass in the right upper quadrant of her abdomen. The tumor location was in the abdominal wall in explorative laparatomy and malignant schwannoma was diagnosed in pathologic assessment. The tumor recurred in 3 months and computed tomography showed two masses in the right side of abdominopelvic cavity. Thereafter, second complete surgical resection was performed and pathologic finding was the same. In spite of administering chemotherapy after second surgery,the tumor recurred and magnetic resonance imaging finding showed a huge heterogeneously enhancing mass with adhesion to the inner side of the abdominal wall. The patient died because of acute respiratory failure due to multiple bilateral pulmonary metastases. Tumor location and rapid recurrence was unique in our patient.KeywordsMalignant peripheral nerve sheath tumor; Malignant schwannoma; Abdominal wall

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“…4,15 The mean age of patients with tumors at diagnosis was 31.6 years as reported by Kourea et al,10 34.0 years as reported by Ducatman et al, 4 and 37.4 years as reported by Wanebo et al 16 Reports of these tumors in children are rare. [17][18][19][20] MPNSTs located in the extremities and trunk are usually high-grade and clinically aggressive, and the prognosis of patients with them is poor. Wanebo reported that five patients with primary intradural MPNST died between 2 months and 6 years after surgery with widespread metastatic disease.…”

Section: Discussionmentioning

confidence: 99%

Primary malignant peripheral nerve sheath tumor of the cauda equina in a child case report

Yone¹,

Ijiri²,

Hayashi³

et al. 2004

Spinal Cord

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Study design: A case report of primary malignant peripheral nerve sheath tumor (MPNST) of the cauda equina in a child is presented, and the literature is reviewed. Objective: To discuss the problems involved in the treatment of primary intradural MPNSTs. Setting: A department of orthopaedic surgery in Japan. Methods: A 4-year-old boy complained of low-back pain radiating to the left calf. MRI revealed an intradural tumor at L3-L5 level. Following laminectomy of L3, L4 and L5, the tumor was removed en bloc. Based on pathological and immunohistological findings, the tumor was diagnosed as an MPNST. Results: Although adjuvant chemotherapy was administered local recurrence and cerebral and spinal metastases of the tumor were found 6 months after the operation. Following additional incomplete removal of the recurrent tumor, radiation therapy was administered. Although recurrent and metastatic tumors disappeared or diminished in size by radiation, tumors increased in size thereafter, despite additional adjuvant chemotherapy. At 21 months after the first operation, he died of pneumonia. Conclusions: Reported clinical outcomes for patients with primary intradural MPNST are very poor. Although no gold standard for the treatment of tumors has been established yet, surgical removal of tumors combined with postoperative high-dose radiation may be recommended.

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Anterior abdominal wall malignant peripheral nerve sheath tumor in an infant

Cited by 12 publications

References 8 publications

Malignant peripheral nerve sheath tumor: Molecular pathogenesis and current management considerations

Malignant peripheral nerve sheath tumor: Molecular pathogenesis and current management considerations

10.4021/jocmr2009.09.1264

Primary malignant peripheral nerve sheath tumor of the cauda equina in a child case report

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