Malignant peripheral nerve sheath tumors (MPNST) – Clinicopathological study and treatment outcome of twenty-four cases

Kar, Madhabananda; Deo, S. V. S.; Shukla, Nootan Kumar; Malik, Ajay; DattaGupta, Sidharth; Mohanti, B.K.; Thulkar, Sanjay

doi:10.1186/1477-7819-4-55

Cited by 173 publications

(89 citation statements)

References 22 publications

Supporting

Mentioning

Contrasting

Unclassified

Order By: Relevance

“…Despite the fact that most MPNST are diagnosed in patients with von Recklinghausen's disease, [1][2][3] no renal or perirenal cases were reported. The diagnosis of renal or perirenal MPNST remains challenging and was established by pathologic examination in all reported cases.…”

Section: Discussionmentioning

confidence: 99%

“…[2][3][4]6 Conflicting evidence suggests that adjuvant radiotherapy might play a role in the management of MPNST [1][2][3]6 However, no studies found that chemotherapy improves survival. [1][2][3]5,7 We report the fourth case of MPNST in the renal parenchyma (Table 1). [8][9][10] Martinot and colleagues 8 reported the first case in 1960 of a 70-year-old male whose left kidney was almost totally replaced by tumour tissue; this patient died on postoperative day 5.…”

Section: Discussionmentioning

confidence: 99%

“…[2][3][4][5] MPNST show poor prognosis, with reported rates of 5-year disease specific survival of 16% to 60%. [1][2][3][4][5] The most effective treatment remains surgical resection and margin status is of primary importance. [2][3][4]6 Conflicting evidence suggests that adjuvant radiotherapy might play a role in the management of MPNST [1][2][3]6 However, no studies found that chemotherapy improves survival.…”

Section: Discussionmentioning

confidence: 99%

“…[1][2][3][4][5] The most effective treatment remains surgical resection and margin status is of primary importance. [2][3][4]6 Conflicting evidence suggests that adjuvant radiotherapy might play a role in the management of MPNST [1][2][3]6 However, no studies found that chemotherapy improves survival. [1][2][3]5,7 We report the fourth case of MPNST in the renal parenchyma (Table 1).…”

Section: Discussionmentioning

confidence: 99%

“…These tumours are often associated with Von Recklinghausen's disease. [1][2][3] Preoperative diagnosis remains challenging and is based on histology and positive immunohistochemical studies for S-100 protein. Occurrence is usually in the head and neck region or in the limbs.…”

mentioning

confidence: 99%

See 4 more Smart Citations

Malignant peripheral nerve sheath tumour of the renal parenchyma presenting as a fast growing atypical renal cyst

Ouellet

Doueik

Sabbagh

2013

CUAJ

View full text Add to dashboard Cite

Malignant peripheral nerve sheath tumours (MPNST) of the kidney are very rare, with only 3 cases reported in the English and French literature. However, we report the first case of fast growing atypical renal cyst where a magnetic resonance imaging was an interesting adjunct to the computed tomography scan in imaging this rare tumour. IntroductionMalignant peripheral nerve sheath tumours (MPNST), also known as malignant schwannoma, arise from Schwann cells which are derived from the neural crest. These tumours are often associated with Von Recklinghausen's disease. [1][2][3] Preoperative diagnosis remains challenging and is based on histology and positive immunohistochemical studies for S-100 protein. Occurrence is usually in the head and neck region or in the limbs. [2][3][4][5] Renal or perirenal MPNST are exceptionally rare findings. We report a case of renal malignant peripheral nerve sheath tumour that highlights the importance of a magnetic resonance imaging (MRI) in such situation. Case reportA 70-year-old ex-smoking male known for hypertension, diabetes mellitus, dyslipidemia, obesity (body mass index 40) and chronic renal failure (estimated glomerular filtration rate 45.4 mL/min/1.73 m 2 ) was referred to our institution for a growing renal cyst. The patient was asymptomatic and physical examination was unremarkable. Personal and familial history was negative for von Recklinghausen's disease. Laboratory studies, including complete blood count, electrolytes, urinalysis, glycemia and hepatic profile, were normal except for a high level of serum creatinine and normocytic anemia secondary to chronic renal disease. A computed tomography (CT) scan confirmed multiple simple cysts on both kidneys, except for an 8-cm left mid-upper pole dense cyst. A controlled CT scan 6 months later showed a slight growth and peripheral rim enhancement of this cyst with subtle changes in its content (Fig. 1, part A). An ultrasound confirmed some internal echoes compatible with cellular content. A MRI was recommended by our radiologist 2 months later, and showed a slightly hypointense left renal lesion (Fig. 1, part B) with heterogeneous enhancement following gadolinium administration on T 1 -weighted images (Fig. 1, part C). Lesion also appeared heterogeneously hyperintense on T 2 -weighted images. (Fig. 1, part D). Considering the differences between the CT and the MRI images, our radiologist suggested a repeat abdominal CT scan 3 months later which showed significant growth of the left renal mass to 14 cm with heterogeneous internal enhancement (Fig. 1, part E) suggesting a renal tumour. There was focal fat stranding involving the perirenal space. The renal vein remained permeable and without hydronephrosis.The patient underwent a transperitoneal laparoscopic radical nephrectomy. Lymph nodes were explored during surgery and none were found. Ipsilateral adrenal gland was not removed because it was radiologically normal.Gross examination of the specimen showed a 17 × 14 × 10-cm kidney weighting about 3 kg. The tumour wa...

show abstract

Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

mentioning

confidence: 99%

See 3 more Smart Citations

Malignant peripheral nerve sheath tumour of the renal parenchyma presenting as a fast growing atypical renal cyst

Ouellet

Doueik

Sabbagh

2013

CUAJ

View full text Add to dashboard Cite

show abstract

Intrapelvic malignant nerve sheath tumors presenting as acute urinary retention: A case report and literature review

Ariafar

Ahmed

Hosseini

et al. 2022

Clinical Case Reports

View full text Add to dashboard Cite

show abstract

Germline and somaticNF1 gene mutation spectrum in NF1-associated malignant peripheral nerve sheath tumors (MPNSTs)

et al. 2007

View full text Add to dashboard Cite

About 10% of neurofibromatosis type 1 (NF1) patients develop malignant peripheral nerve sheath tumors (MPNSTs) and represent considerable patient morbidity and mortality. Elucidation of the genetic mechanisms by which inherited and acquired NF1 disease gene variants lead to MPNST development is important. A study was undertaken to identify the constitutional and somatic NF1 mutations in 34 MPNSTs from 27 NF1 patients. The NF1 germline mutations identified in 22 lymphocytes DNA from these patients included seven novel mutations and a large 1.4-Mb deletion. The NF1 germline mutation spectrum was similar to that previously identified in adult NF1 patients without MPNST. Somatic NF1 mutations were identified in tumor DNA from 31 out of 34 MPNSTs, of which 28 were large genomic deletions. The high prevalence (>90%) of such deletions in MPNST contrast with the =or<20% found in benign neurofibromas and is indicative of the involvement of different mutational mechanisms in these tumors. Coinactivation of the TP53 gene by deletion, or by point mutation along with NF1 gene inactivation, is known to exacerbate disease symptoms in NF1, therefore TP53 gene inactivation was screened. DNA from 20 tumors showed evidence for loss of heterozygosity (LOH) across the TP53 region in 11 samples, with novel TP53 point mutations in four tumors.

show abstract

Malignant peripheral nerve sheath tumors (MPNST) – Clinicopathological study and treatment outcome of twenty-four cases

Cited by 173 publications

References 22 publications

Malignant peripheral nerve sheath tumour of the renal parenchyma presenting as a fast growing atypical renal cyst

Malignant peripheral nerve sheath tumour of the renal parenchyma presenting as a fast growing atypical renal cyst

Intrapelvic malignant nerve sheath tumors presenting as acute urinary retention: A case report and literature review

Germline and somaticNF1 gene mutation spectrum in NF1-associated malignant peripheral nerve sheath tumors (MPNSTs)

Contact Info

Product

Resources

About