Isolated malignant peripheral nerve sheath tumor of kidney capsule (original) (raw)
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A Case of Malignant Peripheral Nerve Sheath Tumour of the Kidney
Nephro-urology monthly, 2023
Introduction: Malignant peripheral nerve sheath tumor (MPNST) of the kidney is an uncommon neoplasm. We present a rare case of MPNST successfully managed by surgical intervention. Case Presentation: A 23-year-old male patient presented with dull aching pain in the right flank associated with hematuria. On examination, an abdominal mass with firm consistency occupied the right lumbar quadrant with the fullness of the right renal angle. Contrast-enhanced computed tomography (CECT) showed heterogeneously hypoenhancing lobulated mass (9.2 × 6.4 × 7.6 cm) arising exophytically from the anterior cortex of the middle and lower poles of the right kidney with necrosis. It was compressing the pelvicalyceal system causing mild hydronephrosis. Kidney function tests revealed normal results. Urine cytology for malignant cells was negative. The patient underwent an open radical nephrectomy. Renal mass was adhered to ascending colon and duodenum and was abutting the liver. The histopathological report suggested malignant peripheral nerve sheath tumors with margins microscopically clear of tumor cells. S-100 and Vimentin were stained positively. The patient has been under regular follow-up (every three months) for the last year. Conclusions: If feasible, wide local excision is considered the preferred approach, as prognosis mainly depends on the completeness of surgical resection.
Malignant peripheral nerve sheath tumor of kidney—A case report
International Journal of Surgery Case Reports, 2013
INTRODUCTION: Malignant peripheral nerve sheath tumors (MPNSTs) are very rare soft-tissue tumors. These tumors originate from Schwann cells or pluripotent perineural cells of the neural crest. An isolated MPNST of the kidney is extremely rare and has been reported in only six cases to date. PRESENTATION OF CASE: A 30-year-old female with an unremarkable past medical and surgical history presented with an enlarging, right-sided abdominal mass for the last 3 months. A CT scan of the abdomen and pelvis showed a large exophytic mass arising from the right kidney. A metastatic work-up was negative, so a right radical nephrectomy was performed. Histopathological and immunohistochemical studies confirmed the diagnosis of an MPNST. DISCUSSION: MPNSTs are rare and aggressive soft-tissue sarcomas that are usually associated with neurofibromatosis type 1 (NF-1) but that also occur post-radiation and sporadically. These tumors are highly malignant neoplasms with a high recurrence rate and distant metastases. Complete surgical excision remains the mainstay of treatment due to the limited response of MPNSTs to both chemotherapy and radiotherapy. CONCLUSION: MPNSTs arising from the kidney are very rare. Complete surgical resection provides the only hope for cure. Postoperative chemotherapy and radiotherapy are still controversial, although postoperative radiotherapy is currently recommended by an oncology consensus group.
Case Report on Malignant Peripheral Nerve Sheath Tumor
Journal of Pharmaceutical Negative Results, 2022
Malignant peripheral nerve sheath tumours (MPNST) are an uncommon type of cancer that spreads from the brain stem into the body and affects the covering of the nerves. Anywhere in the body can develop MPNST, but the deep tissues of the arms, legs, and trunk are where they most frequently occur. They result in discomfort and lethargy, mostly in the affected region, and it may develop a bulge or tumour. A 22-year-old woman presented in cancer ward with the complaints of right lower limb pain with the pain score of 4/10, the intensity of pain aggravates while sitting or performing activity and relieves while at rest, also felt heaviness since 7 months. A vast blood investigation was carried out. MRI and Biopsy were done to make her diagnosis. After following investigations, she was diagnosed as a "malignant peripheral nerve sheath tumours". Initially she was started on I.V. fluids, analgesics and injectables on the day of admission. Retroperitoneal mass excision was performed under general anaesthesia. Patient's prognosis remains good.
Sciatic Nerve Tumor: Malignant Peripheral Nerve Sheath Tumor: Case Report
The Egyptian Journal of Hospital Medicine
Background: Malignant peripheral nerve sheath tumour (MPNST) is rare. It is a type of peripheral nerve sheath tumour that is cancerous (malignant). Most peripheral nerve sheath tumours are not cancerous (benign). A tumour is a lump or growth in the body. Objective: This paper reports a case of a malignant peripheral nerve sheath tumour revealed from the Magnetic Resonance Imaging (MRI) examination and other processes, with remarkable unusual size of the tumour. Case report: A 36 years old male patient with a three months history of massive right-thigh medial side swelling came to the clinic. The patient reported that he fell while walking before the beginning of the symptoms. He then visited the clinic with medial side thigh pain, limping, and massive swelling in the medial side of his right thigh. The provider recommended a triple assessment; 1-clinical, 2-laboratory, and 3-radiological. The clinical evaluation showed that the swelling was about 20 cm x 10 cm diffuse fusiform swelling, with tense stretched skin over cystic to a firm consistency. Conclusion: There is usually a poor MPNST prognosis, so the doctors must be sure about the diagnosis before treating the condition. Typically, there are three ways to treat MPNST: surgery, chemotherapy and radiotherapy.
Journal of Neurosurgery, 2005
ERIPHERAL non-neural sheath tumors are rare. A review of the literature over the last three decades demonstrates a paucity of case reports or small series of patients for each of these tumors, with the exception of the more common ganglion cysts of the peripheral nerve, about which many reports have been published. Localized hypertrophic neuropathy of the peripheral nerve originates from hyperplasia of Schwann and/or perineurial cells. Localized hypertrophic neuropathy is thought by some to represent a hypertrophic lesion. Some investigators, however, have posited a tumor basis for these lesions because they have a clonal abnormality involving a loss of 22q11.2qter and the NF2 tumor suppressor gene is localized to 22q11.2-q12. 17 If classified as a tumor, LHN is usually specified as a non-neural sheath tumor rather than a neural sheath tumor, and is, therefore, included in this review. Benign non-neural sheath tumors also include lipo-mas and vascular tumors such as venous angiomas, hemangiopericytomas, glomus tumors, and hemangioblastomas. Myositis ossificans, osteochondromas, ganglioneuromas, meningiomas, cystic hygromas, myoblastomas or granular cell tumors, and epidermoid cysts are also benign PNNSTs. Malignant PNNSTs arise from nonneural tissues and directly extend or metastasize to a nerve. Examples include breast or pulmonary cancers, which directly extend or metastasize to the perineurium or any potential space surrounding it. Osteogenic or soft-tissue sarcomas displace or adhere to the nerve or encase it and, in a few cases, actually invade the nerve. Lymphomas and melanomas can metastasize and secondarily involve a nerve by epineurial invasion or infiltration of the subperineurial zone, the interfascicular epineurium, and eventually the fascicle(s) themselves. In this paper we report the results of a retrospective evaluation of the charts of 146 patients with benign or malignant tumors of non-neural sheath origin that were located in the brachial and pelvic plexi and other major nerves and were surgically treated at the LSUHSC within a 30-year period. This is the first published report to address the issue of the incidence of benign and malignant PNNSTs with regard to their locations; a critical analysis was performed to determine if there is a correlation between tumor type and loca
British Journal of Surgery, 2021
We present this rare case which was led by spinal surgeons at Leeds after being deemed not suitable for operative input by a national centre. A 54-year-old lady who presented with 2-month history of worsening left flank pain on a background of four years. She was otherwise fit and well. CT confirmed aggressive left-sided paraspinal tumour with epidural encroachment, extending from T8 to T10 vertebral levels. Its deep surface was well-defined and extending superficially through the chest wall with destruction of ribs. The mass grew in size in course of days requiring urgent attention due to risk of cord compression. Biopsy proved high grade MPNST. Malignant peripheral nerve sheath tumor (MPNST) is a rare type of sarcoma, 5-10% of sarcoma cases. MPNST is most common in young adults and middle-aged adults. About 25% to 50% of people with MPNST have NF1. 8% to 13% of people with NF1 will get MPNST in their lifetime. An MDT approach involving spinal, thoracic, vascular and plastic surgeo...