. Malignant Peripheral Nerve Sheath Tumor, MPNST. PORTNotes. In: OrthopaedicsOne - The Orthopaedic Knowledge Network. Created Feb 16, 2009 16:54. Last modified Oct 05, 2011 15:02 ver.10. Retrieved 2016-06-30, from http://www.orthopaedicsone.com/x/ZgEjAQ.
DEFINITION AND PATHOGENESIS
- (Malignant peripheral nerve sheath tumor, MPNST, neurofibrosarcoma, malignant schwannoma, neurofibrosarcoma, neurogenic sarcoma)
- Malignant nerve sheath tumor of Schwann or perineural cells or fibroblasts of the nerve sheath
- Criteria for dx:
- Tumor arrises from a peripheral nerve, OR
- Tumor arises from a preexisting neurofibroma or neurilemmoma, OR
- Tumor has hsitologic features that reflect Schwann cell differentiation
- Dense and hypodense fascicles ("marble-like" pattern)
- Spindled cells with irregular nuclei
- Immunohistochemisty stains (or EM) consistent with Schwann cell differentiation
- About 50% occur with neurofibromatosis (~ 4% of those patients develop malignant lesions)
- Lifetime risk of MPNST in NF1 reported to be 10-30%
- MPNST may be multiple
- Originates in radiated fields reportedly 2-29% of the time
- Age range 16-76
- Up to 11% may be XRT related
- Most pts between 20-50 (lower end of the spectrum with NF1)
- M>F (M=F in some studies) due to M predominance in NF
- Enlarging masses (1.5-32cm, mean 9.5cm), usually painful or tender on palpation, ± sensory or motor findings
- Sudden enlargement of a pre-existing mass in NF1
- Have caused sxs similar to CTS, and cubital tunnel syndrome (1st thoracic nerve root)
- Size not always predictive of malignancy
- Enlargement of preexisting mass, superficial or deep
- Extremities (45%), trunk (34%), head/neck (19%)
- Unusual location
- MRI may reveal area of inhomogeneity within the lesion
- Has been reported to demonstrate synchronous lesions
- Large mass within a nerve, typically > 5cm and between anatomical compartments
- May be arising within a preexisting neurofibroma
- Wavy, comma shaped nuclei, parallel orientation (nuclear palisading) <10%
- Hyperplastic perivascular changes
- Dense fascicles of cells alternating with myxoid areas
- 94% high grade
- Hyaline nodules = rosettes
- Mature islands of (heterologous) cartilage, bone, skeletal muscle
- Elongated nuclei with tapered ends
- Angulated nuclei
- Branching cytoplasmic processes from the cell body with microtubules, neurofilaments
- Focally + S-100 in 50-90%
- Vimentin +
- Cytokeratins -
- HMB-45 -
- p53 protein expression in 21%
- TP53 mutations in 24% (36% were from NF1 pts)
DIFFERENTIAL CLINICOPATHOLOGIC DIAGNOSIS
- Schwannoma (neurilemoma) with mitoses
- Ancient schwannoma
- Pleomorphic undifferentiated sarcoma (previously known as MFH)
- Monophasic synovial sarcoma
- Angiosarcoma arising in a preexisitng neurofibroma
DISEASE COURSE AND TREATMENT
- Wide excision ± chemotherapy/XRT
- Proximal perineural spread may occur
- Metastases to lung, liver, subcutaneous tissue, and bone
- Unusual metastases
- Long term survival ~ 50% (early detection and complete resection important)
- LR 22%
- 5- and 100-year disease-specific survival 60% and 45%
- Poor prognostic factors
- ? 5 cm size
- High tumor grade
- Truncal location
MALIGNANT TRITON TUMOR
- (Malignant schwannoma with rhabdomyoblastic differentiation)
- 67% occur in NF
- Similar clinically to malignant schwannoma
- Scattered rhabdomyoblasts within an otherwise typical malignant schwannoma
GLANDULAR MALIGNANT SCHWANNOMA
- Rare, with cuboidal or columnar glands producing epithelial mucins
- Well-differentiated glands scattered throughout a MPNST
- Usually associated with NF1
- Median age 30 yrs
MALIGNANT EPITHELIOID SCHWANNOMA
- ? of malignant schwannomas, with the appearance of non-pigmented melanoma
- ? of malignant schwannomas, with the appearance of non-pigmented melanoma
- 78% LR (intraneural extension proximally and distally)
- "Preservation of function" compromises resection
- Pain an ominous sign
- Hematogenous and lymphatic metastases have been reported
PLEXIFORM MPNST OF INFANCY AND EARLY CHILDHOOD
- MPNST induced by transplacental injection of ethylnitrosourea
- MPNST induced by administration of methylcholanthrene
Bertagna F, Bosio G, Biasiotto G, Savelli G, Rodella C, Giubbini R, Rosenbaum J, Alavi A. Plurifocal malignant peripheral nerve sheath tumor demonstrated by 18F-fluorodeoxyglucose positron emission tomography/computed tomograph. Jpn J Radiol. 2009 Oct;27(8):320-3. Pubmed
Bhargava R, Parham DM, Laster OE, Chari RS, Chen G, Fletcher BD: MR imaging differentiation of benign and malignant peripheral nerve sheath tumors: use of target sign. Pediatr Radiol 27:124-9,1997.
Carvajal JA, Cuartas E, Qadir R, Levi AD, Temple HT. Peripheral nerve sheath tumors of the foot and ankle. Foot Ankle Int. 2011 Feb;32(2):163-7. Erratum in: Foot Ankle Int. 2011 Apr;32(4):vi. Pubmed
Chen CY, Sun JS. Malignant peripheral nerve sheath tumour of the hand. J Hand Surg Eur Vol. 2010 Mar;35(3):246-8. Pubmed
Clemens MW, Murthy A, Przygodzki RM. Malignant peripheral nerve sheath tumor of the hand. Plast Reconstr Surg. 2009 Feb;123(2):83e-85e.
Coady MSE, Polacarz S, Page RE: Cutaneous malignant peripheral nerve sheath tumor (MPNST) of the hand: a review of current literature. J Hand Surg 18B:478-81,1993.
Enzinger FM, Weiss SW: Malignant Tumors of Peripheral Nerves. In Soft Tissue Tumors FM Enzinger and SW Weiss (Eds), C.V. Mosby Co., St. Louis (3rd ed) 1995, pp 889-928.
Hung YW, Tse WL, Cheng HS, Ho PC. Surgical excision for challenging upper limb nerve sheath tumours: a single centre retrospective review of treatment results. Hong Kong Med J. 2010 Aug;16(4):287-91.
Ghosh A, Talwar OP, Pradhan SV. Tumour and tumour-like conditions of peripheral nerve origin: ten years' experience. Kathmandu Univ Med J (KUMJ). 2010 Jan-Mar;8(29):97-101. Pubmed
Gibson R, Bell MJ: Malignant schwannoma of the brachial plexus: a lesson in presentation. J Hand Surg 16B:113,1991.
Hotta Y, Azuma H, Kondo T, Yamashina M: Von Recklinghausen's neurofibromatosis with neurofibrosarcoma. J Bone Joint Surg 73B:348-9,1991.
Hruban RH, Shiu MH, Senie RT, : Malignant peripheral nerve sheath tumors of the buttock and lower extremity: a study of 43 cases. Cancer 66:1253-65,1990.
Isler MH, Fogaca MF, Mankin HJ: Radiation induced malignant schwannoma arising in a neurofibroma. Clin Orthop 3215:251-5,1996.
Kitamura M, Wada N, Nagata S, Iizuka N, Jin YF, Tomoeda M, Yuki M, Naka N, Araki N, Yutani C, Tomita Y. Malignant peripheral nerve sheath tumor associated with neurofibromatosis type 1, with metastasis to the heart: a case report. Diagn Pathol. 2010 Jan 9;5:2.
Laskin WB, Weiss SW, Bratthauer GL: Epithelioid variant of malignant peripheral nerve sheath tumor (malignant epithelioid schwannoma). Am J Surg Pathol 15:1136-45,1991.
Lisowski LA, Bramer JA, de Jonge MC, Bras J, Van der Horst CM, de Kraker J. Metastatic malignant peripheral nerve sheath tumor in a newborn. J Pediatr Hematol Oncol. 2008 Dec;30(12):891-5.
Lee RM, Ong CP, Jacobsen AS, Chan MY, Hwang WS. Malignant peripheral nerve sheath tumor mimicking carotid body tumor--case report and review. J Pediatr Surg. 2011 Mar;46(3):554-8.
MacKay IR, Barua JM: Perineural tumour spread: an unusual cause of carpal tunnel syndrome. J Hand Surg 15B:104-5,1990.
Meek MF, Wolf R, Coert JH, Hoekstra HJ, Nicolai JP. Plexiform malignant peripheral nerve sheath tumour of infancy and childhood of the index finger: Surgical treatment. Scand J Plast Reconstr Surg Hand Surg. 2009;43(4):230-5.
Miguchi M, Takakura Y, Egi H, Hinoi T, Adachi T, Kawaguchi Y, Shinomura M, Tokunaga M, Okajima M, Ohdan H. Malignant peripheral nerve sheath tumor arising from the greater omentum: case report. World J Surg Oncol. 2011 Mar 21;9:33.
Nepka C, Karadana M, Karasavvidou F, Barbanis S, Kalodimos G, Koukoulis G. Fine needle aspiration cytology of a primary malignant peripheral nerve sheath tumor arising in the parotid gland: a case report. Acta Cytol. 2009 Jul-Aug;53(4):423-6.
Rizvi S, Mehboob J, Asghar AH, Mateen A, Raza T, Hameed A. Omental caking: a rare manifestation of malignant peripheral nerve sheath tumour. J Coll Physicians Surg Pak. 2010 Aug;20(8):554-5.
Rogalski RP, Louis DS: Neurofibrosarcomas of the upper extremity. J Hand Surg 16A:873-6,1991.
Stewart TW, Copeland MM. Neurogenic sarcoma. Am J Cancer 1931; 15:1235.
Stucky CC, Johnson KN, Gray RJ, Pockaj BA, Ocal IT, Rose PS, Wasif N. Malignant peripheral nerve sheath tumors (MPNST): THe Mayo Clinic experience. Ann Surg Oncol. 2011 Aug 23. Pubmed
Sandberg K, Nilsson J, Søe Nielsen N, Dahlin LB. Tumours of peripheral nerves in the upper extremity: a 22-year epidemiological study. Scand J Plast Reconstr Surg Hand Surg. 2009;43(1):43-9.
Tadiparthi S, Shokrollahi K, Fahmy FS. Re: malignant peripheral nerve sheath tumour of the hand: challenges in diagnosis and management of a child. J Hand Surg Eur Vol. 2008 Oct;33(5):677.
Than KD, Ghori AK, Wang AC, Pandey AS. Metastatic malignant peripheral nerve sheath tumor of the cauda equina. J Clin Neurosci. 2011 Jun;18(6):844-6.
van Vliet M, Kliffen M, Krestin GP, van Dijke CF. Soft tissue sarcomas at a glance: clinical, histological, and MR imaging features of malignant extremity soft tissue tumors. Eur Radiol. 2009 Jun;19(6):1499-511. Pubmed
Vauthey J-N, Woodruff JM, Brennan MF: Extremity malignant peripheral nerve sheath tumors (neurogenic sarcomas): a 10-year experience. Ann Surg Oncol 2:126-31,1995.
Verdijk RM, den Bakker MA, Dubbink HJ, Hop WC, Dinjens WN, Kros JM. TP53 mutation analysis of malignant peripheral nerve sheath tumors. J Neuropathol Exp Neurol. 2010 Jan;69(1):16-26. Pubmed
Wood MK, Erdmann MWH, Davies DM: Malignant schwannoma mistakenly diagnosed as carpal tunnel syndrome. J Hand Surg 18B:187-8,1993.
Wong SY, Teh M, Tan YO, Best PV: Malignant glandular triton tumor. Cancer 67:1076-83,1991.
Yousem SA, Colby TV, Urich H: Malignant epithelioid schwannoma arising in a benign schwannoma. Cancer 55:2799-2803,1985.
Zambrana F, Vicente F, García-Manrique T, Pereira S, Sáinz De Zaitigui J, De La Cruz Merino L. Primary intracranial malignant peripheral nerve sheath tumour responding to chemotherapy. Clin Transl Oncol. 2010 Mar;12(3):231-3.
Zhang J, Sun Y, Peng ZL. Malignant peripheral nerve sheath tumor of the vagina. Saudi Med J. 2009 May;30(5):705-7.