MALIGNANT PERIPHERAL NERVE SHEATH TUMOR– BIOLOGICALLY AGGRESSIVE SOFT TISSUE SARCOMAS: CASE REPORT

  • Todor Hadzi - Manchev General Public Hospital “ 8 Septemvri” Skopje, R.of North Macedonia
  • Ljubomir Ognjenovikj University Clinic of Abdominal surgery Skopje, R.of North Macedonia
  • Zoran Karadzov University Clinic of Abdominal surgery Skopje, R.of North Macedonia
  • Stojan Gjosev University Clinic of Abdominal surgery Skopje, R.of North Macedonia
  • Dragan Hadzi-Manchev University Clinic of Abdominal surgery Skopje, R.of North Macedonia
  • Darko Dzambaz University Clinic of Abdominal surgery Skopje, R.of North Macedonia
  • Velimir Shumenkovski General Public Hospital Ohrid, R.of North Macedonia

Abstract

Primary retroperitoneal tumors are an exceedingly rare clinical problem. Masses in the retroperitoneum can be categorized as one of three entities: lymphomas, extragonadal germ cell tumors, and sarcomas. Malignant peripheral nerve sheath tumors are uncommon, biologically aggressive soft tissue sarcomas of neural origin that pose tremendous challenges to effective therapy. We present a young adult with retroperitoneal tumor. The patient presented a lumbar pain on the left side. Echotomography of the abdomen was madeand cystic tumor under the pancreas was detected. CT showed retroperitoneal tumor with hypodense features,with defined borders with size of 5,6x9,6 cm. Blood results and tumor markers showed no abnormalities. The patient had a surgery and  whole extirpation was made without damaging the surrounding organs.This case illustrate early diagnosis of MPNST is key to reduce mortality, with complete surgical extirpation with clear margins as treatment of choice.


Key words: Retroperitoneal tumor, soft tissue sarcomas, surgical extirpation


https://doi.org/10.55302/JMS2141101hm


 

References

1.Fletcher CDM, Bridge JA, Hogendoorn PCW. et al. WHO Classification of Tumours of Soft Tissue and Bone. Lyon, France: IARC; 2013. [Google Scholar]
2.Ng VY, Scharschmidt TJ, Mayerson JL, et al. Incidence and survival in sarcoma in the United States: A focus on musculoskeletal lesions. Anticancer Res. 2013;33:2597–2604. [PubMed] [Google Scholar]
3.Widemann BC. Current status of sporadic and neurofibromatosis type 1-associated malignant peripheral nerve sheath tumors. Curr Oncol Rep. 2009;11:322–328. [PMC free article] [PubMed] [Google Scholar]
4.Ferner RE, Golding JF, Smith M, et al. [18F]2-fluoro-2-deoxy-D-glucose positron emission tomography (FDG PET) as a diagnostic tool for neurofibromatosis 1 (NF1) associated malignant peripheral nerve sheath tumours (MPNSTs): A long-term clinical study. Ann Oncol. 2008;19:390–394. [PubMed] [Google Scholar]
5.Beert E, Brems H, Daniëls B, et al. Atypical neurofibromas in neurofibromatosis type 1 are premalignant tumors. Genes Chromosomes Cancer. 2011;50:1021–1032. [PubMed] [Google Scholar]
6.Zou C, Smith KD, Liu J, et al. Clinical, pathological, and molecular variables predictive of malignant peripheral nerve sheath tumor outcome. Ann Surg. 2009;249:1014–1022. [PubMed] [Google Scholar]
7.LaFemina J, Qin LX, Moraco NH, et al. Oncologic outcomes of sporadic, neurofibromatosis-associated, and radiation-induced malignant peripheral nerve sheath tumors. Ann Surg Oncol. 2013;20:66–72. [PMC free article] [PubMed] [Google Scholar]
8.Stucky CC, Johnson KN, Gray RJ, et al. Malignant peripheral nerve sheath tumors (MPNST): The Mayo Clinic experience. Ann Surg Oncol. 2012;19:878–885. [PubMed] [Google Scholar]
9.Dunn GP, Spiliopoulos K, Plotkin SR, et al. Role of resection of malignant peripheral nerve sheath tumors in patients with neurofibromatosis type 1. J Neurosurg. 2013;118:142–148. [PubMed] [Google Scholar]
10.Pervaiz N, Colterjohn N, Farrokhyar F, et al. A systematic meta-analysis of randomized controlled trials of adjuvant chemotherapy for localized resectable soft-tissue sarcoma. Cancer. 2008;113:573–581. [PubMed] [Google Scholar]
11.Porter DE, Prasad V, Foster L, et al. Survival in malignant peripheral nerve sheath tumours: A comparison between sporadic and neurofibromatosis type 1-associated tumours. Sarcoma. 2009;2009:756395. [PMC free article] [PubMed] [Google Scholar]
12.Ferrari A, Miceli R, Rey A, et al. Non-metastatic unresected paediatric non-rhabdomyosarcoma soft tissue sarcomas: Results of a pooled analysis from United States and European groups. Eur J Cancer. 2011;47:724–731. [PMC free article] [PubMed] [Google Scholar]
13.Carli M, Ferrari A, Mattke A, et al. Pediatric malignant peripheral nerve sheath tumor: The Italian and German soft tissue sarcoma cooperative group. J Clin Oncol. 2005;23:8422–8430. [PubMed] [Google Scholar]
14.Kolberg M, Høland M, Agesen TH, et al. Survival meta-analyses for >1800 malignant peripheral nerve sheath tumor patients with and without neurofibromatosis type 1. Neuro-oncol. 2013;15:135–147. [PMC free article] [PubMed] [Google Scholar]
Published
2021-03-30
How to Cite
HADZI - MANCHEV, Todor et al. MALIGNANT PERIPHERAL NERVE SHEATH TUMOR– BIOLOGICALLY AGGRESSIVE SOFT TISSUE SARCOMAS: CASE REPORT. Journal of Morphological Sciences, [S.l.], v. 4, n. 1, p. 101-104, mar. 2021. ISSN 2545-4706. Available at: <http://jms.mk/jms/article/view/vol4no1-16>. Date accessed: 29 mar. 2024.
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Articles