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Elena G. Poriazova, Oliana B. Boykinova, Ivan S. Ivanov, Maria I. Spasova, Georgi B. Bozhilov, Ani K. Kevorkyan and Stoyan S. Novakov

REFERENCES 1. Sanguinetti C, Specchia N, Gigante A, et al. Clinical and pathological aspects of solitary spinal neurofibroma. J. Bone Joint Surg Br 1993;75:141-7. 2. Seppälä MT, Haltia MJ, Sankila RJ, et al. Long-term outcome after removal of spinal neurofibroma. J Neurosurg 1995;82:572-7. 3. Mukerji G, Sherekar S, Yadav YR, et al. Pediatric intramedullary schwannoma without neurofibromatosis. Neurol India 2007;55:54-6. 4. Kim NR, Suh YL, Shin HJ. Thoracic pediatric intramedullary schwannoma: report of a case. Pediatr Neurosurg 2009

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Kazimierz Kobus and Piotr Wójcicki

References Katz AD, Mc Alpin C: Face and neck neurogenic neoplasma. Am J Surg 1993; 166 (4): 421-23. Landreneau RJ, Dowling RD, Ferson PF: Thoracoscopic resection of a posterior mediastinal neurogenic tumor. Neurosurgery 1999. Patil K, Mahima VG, Shetty SK et al.: Facial plexiform neurofibromatosis type I: a case report. J Indian Soc Pedod Dent 2007; 25 (1): 30-35. Marocchio LS, Oliveira DT, Pereira MC et al.: Spradic and multiple neurofibromas

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Marta Fijałkowska and Bogusław Antoszewski

Abstract

Neurofibromatosis type 1 (NF-1, von Recklinghausen disease) is included in the group of diseases known as phacomatosis. Clinical diagnosis of Recklinghausen disease is based on the presence of 2 from 7 criteria developed by a panel of expert. So far effective strategy of treatment of patients with NF-1 is not developed. One method of treatment are surgical excisions.

The aim of the study was to present own experience of treatment of patients with giant tumors in the course of Recklinghausen disease.

Material and methods. The Department of Plastic, Reconstructive and Aesthetic Surgery of Medical University in Łódź has under care 34 patients with NF-1. Fifteen patients (8 females and 7 males) with giant tumors were included in this analysis. The patients age was from 6 to 57 years. In analyzed group a retrospective review of surgical treatment was carried out on the basis of patients’ medical documentation.

Results. Ten from 15 analyzed patients were operated on in our Department and performed surgeries were aimed to partially reduce the tumors mass. Only one patient had his tumor excised in one-stage procedure, in rest of the patients the lesions were too extensive. Most patients in histopathological examinations were diagnosed with neurofibroma (8 patients), 1 - neurofibromaplexiforme and 1 - neurofibrolipoma.

Conclusions. Unpredictable course of Recklinghausen disease causes that follow-up examinations are necessary. Treatment of such patients is particularly indicated when the tumor mass causes difficulty in patients daily habits or is life-threatening. Also it is worth to remember that delayed decision of surgical treatment unable excision of the whole tumor in one-stage procedure. This in turn causes the risk of tumor re-growing and/or developing of malignancy

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Jagoda Balaban, Dragana Popović and Svetlana Pavlović

;112(29):9034–9. 13. Shah KN. The diagnostic and clinical significance of café-au-lait macules. Pediatr Clin North Am 2010;57(5):1131-53. 14. Tchernev G, Chokoeva AA, Patterson JW, Bakardzhiev I, Wollina U, Tana C. Plexiform neurofibroma- a case report. Medicine (Baltimore) 2016;95(6):e2663. 15. Staser K, Yang FC, Clapp DW. Pathogenesis of plexiform neurofibroma: tumor-stromal/hematopoietic interactions in tumor progression. Annu Rev Pathol 2012;7:469–95. 16. O’Brien DE, Brenner DS, Gutmann DH, Gereau RW. Assessment of pain and itch behavior in a mouse model of

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A. Bolčeková, A. Hlavatá, A. Zat'ková, M. Némethová, M. Holobradá, P. Sýkora, A. Gerinec, K. Husáková, D. Ilenčíková and L Kovács

References [1] Blazo MA, Lewis RA, Chintagumpala MM, Frazier M, Mc-Cluggage C Plon SE. Outcomes of systematic screening for optic pathway tumors in children with neurofibroma-tosis type 1. Am J Med Genet. 2004;127A:224-229. [2] Bolcekova A, Nemethova M, Zatkova A Hlinkova K, Pozgayova S, Hlavata A, Kadasi L, Durovcikova D, Gerinec A, Husakova K, Pavlovicova Z, Holobrada M, Kovacs L, Ilencikova D. Clustering of mutations in the 5’ tertile of the NF1 gene in Slovakia patients with optic pathway glioma, Neoplasma. 2013;60 (6):655-65. [3] Brown EW, Riccardi

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Anna Maria Dąbrowska and Agnieszka Zwolak

REFERENCES 1. Ghalayani P, Saberi Z, Sardari F. Neurofibromatosis type I (von Recklinghausen’s disease): a family case report and literature review. Dent Res J (Isfahan). 2012;9:483-8. 2. Dadlani R, Sadanand V, Ghosal N, Hegde SH. Congenital giant plexi-form neurofibroma with occipital calvarial dysplasia in association with meningoencephalocele in neurofibromatosis Type 1 and segmental neurofibromatosis. J Neurosurg Pediatrics. 2013;12:458-64. 3. Diggs-Andrews KA, Gutmann DH. Modeling cognitive dysfunction in neurofibromatosis-1. Trends

Open access

Daniel Brzeziński, Mariusz łochowski and Józef Kozak

Abstract

We reported two cases of the giant mediastinal neurogenic tumors. Patients were women in middle age. In both cases the chest X-ray and CT (computed tomography) demonstrated the huge mass in a pleural cavity without intraspinal invasion. Radical procedures with removal of the giant tumors were performed. The postoperative treatment course was uneventful. The pathology showed schwannoma and neurofibroma. The follow up examination (6-24 months) showed no recurrence of the disease.

Open access

Varun Aggarwal, Amit Narang, Chandni Maheshwari and Divya Kavita

Abstract

Malignant Peripheral Nerve Sheath Tumors (MPNSTs) are one of the very rare high grade malignancies usually affecting extremities or trunk. Incidence is 1/Lac. Intracranial MPNSTs are even rarer, schwannomatous and commonly affecting cranial nerves VIII &VII). Intra-cranial MPNSTs are usually sporadic, arising de novo. The second most common mode of origin is from malignant transformation from pre-existing schwannomas or neurofibroma. We present an extremely rare and probably the first case of intra-cranial malignant peripheral nerve sheath tumor of the olfactory nerve in a non neurofibrosis patient with no prior history of irradiation.

Open access

Łukasz Ulatowski and Anna Kaniewska

Abstract

The study presented a case of a patient with a neurilemoma of the median nerve. It presented as a six centemeters tumor, at the level of the proximal one third of the arm with only mild paraesthesias within frst to third ray of the hand. MRI showed the relationship of the tumor and the median nerve, and allowed for the preliminary diagnose of a benign peripheral neural sheath tumor (neurilemoma or neurofibroma). During the first operation the tumor has not been excised, because nerve reconstruction technique was not available. During the second procedure excision of the tumor has been performed without resection of the median nerve trunk with no postoperative deficiencies. The paper provides a detailed description of a surgical procedure. The presented case, like current publications shows that peripheral neural sheath tumor may be usually excised without resection of the nerve trunk, although the possibility of nerve fascicles injury or the need to excise them in the case of neurofibroma clearly suggest that these type of operations should be carried out in centers with microsurgical facilities.

Open access

Kristina Kostić, Miroslav Dinić, Željko Mijušković, Lidija Zolotarevski, Lidija Kandolf-Sekulović and Radoš Zečević

Abstract

Neurofibromatosis type I (NF1) is an autosomal dominant, multisystemic disease that usually affects the skin, nervous system and bones. Diagnosis is made by matching at least two of the following 7 diagnostic criteria: six or more caféau- lait macules over 15 mm in diameter, two or more neurofibromas, axillary and/or inguinal freckles, optic glioma, two or more Lisch’s nodules (iris hamartoma), changes in the bones in the form of sphenoid dysplasia, thinning of the cortex of long bones and existence of neurofibromatosis in the first degree relatives. We report three patients, two men and a woman aged 18 to 33 years, in whom the first changes occurred at puberty, and there was no positive family history in any of them. All three patients had café-au-lait spots over 15 mm in diameter and numerous localized neurofibromas on the skin of the trunk and extremities that were histologically verified. In two patients, ophthalmic examinations recorded Lisch’s nodules in the iris. In one of the patients, MRI of the head, revealed presence of oval lesions with diameters of 10-15 mm, which may correspond to neurofibromas, and in the other patient fibrous dysplasia of the femur and tibia were observed. Psychological testing in one patient revealed IQ at the lower limits of average (IQ 68). After the diagnosis of neurofibromatosis type I, the patients were given advice about the disease and a plan for the monitoring and control of possible symptoms, and also the possibility of genetic testing during pregnancy. A multidisciplinary approach is required for diagnosing and monitoring of patients with neurofibromatosis type 1.