Neurofibroma differential diagnosis

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Sara Mohsin, M.D.[2] Shanshan Cen, M.D. [3]

Overview

Neurofibroma must be differentiated from schwannoma, dermatofibrosarcoma protuberans (DFSP), ganglioneuroma, dermal neurotized melanocytic nevus, myxoid liposarcoma, solitary circumscribed neuroma, traumatic neuroma, superficial angiomyxoma, nerve sheath myxoma, malignant peripheral nerve sheath tumor, spindle cell lipoma, Leiomyoma, inflammatory myofibroblastic tumor, and acrochordon.

Differential Diagnosis

Neurofibroma must be differentiated from:[1][2][3]

Differentiating neurofibroma from other diseases
Disease entity Etiology (Genetic or others) Histopathological findings Immunohistochemical staining Risk factors Common site of involvement Clinical manifestations Other associated features
Neurofibroma

Can be sporadic or as a part of Neurofibromatosis 1 and 2

  • Uniphasic, low to moderate cellularity
  • Non-encapsulated
  • Random pattern, only rare palisading
  • No well formed verocy bodies
  • Cells separated by collagen bundles
  • Hypocellular with abundant mucinous matrix
  • No peripheral perineural capsule
  • Frequent mast cells
  • Contains neural fibroblasts and fibrillary collagen
  • Random proliferation of Schwann cells and scattered admixed axons
  • No nevoid cells
  • No epithelial component
  • Diffuse growth pattern
  • Scant cytoplasm
  • Wavy cells with buckled nuclei
  • Pseudomeissnerian bodies representing specific differentiation may be present
  • Lacks storiform pattern

Neurofibroma with degenerative atypia ("ancient change") has following microscopic features:

  • Localized cells with large pleomorphic nuclei, cytoplasmic nuclear inclusions, smudgy chromatin, and inconspicuous nuclei
  • Absent or very low mitotic activity
  • Low to moderate cellularity
Positive for:
  • Nerve often not identified, incorporates nerve, axons often present in lesion
  • Seldom cystic
  • Frequently multiple
  • Widespread soft tissue infiltration
  • Tends to displace adnexa
  • <2cm in diameter
  • Lacks distinct lobulation
  • Lacks fat
Schwannoma
  • Encapsulated
  • Biphasic (majority entirely hypercellular Antoni A & hypocellular Antoni B)
  • Palisading
  • Verocay bodies
  • Infrequent extracellular collagen
Positive for:
  • NF-2 associated
  • Nerve often identifiable
  • Eccentric to nerve, axons generally absent within lesion
  • Occasionally cystic
Palisaded encapsulated neuroma Positive for:
  • EMA

Negative for:

  • 90% lesions affect the face
  • No known familial association
Traumatic neuroma
  • Numerous well formed small nerve twigs
  • Limited soft tissue infiltration
  • Contains axons in haphazardly arranged nerves
  • History of trauma or surgery
Neurotized Melanocytic Nevus
  • Superficial classic nevoid melanocytes
  • Congenital and nested growth patterns
  • More abundant cytoplasm
  • Tends to surround adnexa
Positive for:

Negative for:

  • Factor XIIIa
Cutaneous Myxoma (Superficial angiomyxoma)
  • Spindle to stellate cells with oval nuclei
  • 1-5cm in diameter
Positive for:
  • Associated with Carney complex
Nerve sheath myxoma Positive for:
Malignant peripheral nerve sheath tumor
  • Generalized atypia
  • Increased mitotic activity
  • Diffuse hypercellularity
Positive for:
Dermatofibrosarcoma protuberans (DFSP)
  • More cellular
  • Distinct storiform pattern
  • Usually forms a mass
Strongly positive for:

Negative for:

  • Deep soft tissue of posterior neck
Spindle cell lipoma
  • Delicate encapsulation
  • Mostly cases contain fat
  • Floret cell formation
  • No degenerative atypia
Positive for:
  • CD34 (strongly)
  • S-100(stains only fat cells)
Ganglioneuroma Genes involved in the pathogenesis of ganglioneuroma include: Positive for:
  • S-100
Myxoid liposarcoma[4][5][6][7][8][9][10][11][12][13]

Atypical lipomatous tumor/well differentiated liposarcoma and dedifferentiated liposarcoma are associated with:

Myxoid liposarcoma is associated with:

Pleomorphicliposarcoma is associated with:

Well-differentiated liposarcoma:

De-differentiated liposarcoma:

Atypical lipomatous tumor/well differentiated liposarcoma is positive for:

_
Leiomyoma[14][15][16][17][18][19][20][21][15][18][22]

Positive for:

Negative for:

_
Inflammatory myofibroblastic tumor(IMT)[14][15][16][17][18][19][20][21][15][18][22]

Unknown underlying etiology, may be due to inflammatory reaction to:

Mutations such as:

Positive for:

Negative for:

Also known as:

Acrochordon

References

  1. Neurofibroma. Libre Pathology 2015. http://librepathology.org/wiki/index.php/Neurofibroma#cite_note-pmid15486243-2 Accessed on November 17, 2015
  2. http://surgpathcriteria.stanford.edu/peripheral-nerve/neurofibroma/
  3. http://surgpathcriteria.stanford.edu/peripheral-nerve/neurofibroma/
  4. Khin Thway, Rashpal Flora, Chirag Shah, David Olmos & Cyril Fisher (2012). "Diagnostic utility of p16, CDK4, and MDM2 as an immunohistochemical panel in distinguishing well-differentiated and dedifferentiated liposarcomas from other adipocytic tumors". The American journal of surgical pathology. 36 (3): 462–469. doi:10.1097/PAS.0b013e3182417330. PMID 22301498. Unknown parameter |month= ignored (help)
  5. J. Rosai, M. Akerman, P. Dal Cin, I. DeWever, C. D. Fletcher, N. Mandahl, F. Mertens, F. Mitelman, A. Rydholm, R. Sciot, G. Tallini, H. Van den Berghe, W. Van de Ven, R. Vanni & H. Willen (1996). "Combined morphologic and karyotypic study of 59 atypical lipomatous tumors. Evaluation of their relationship and differential diagnosis with other adipose tissue tumors (a report of the CHAMP Study Group)". The American journal of surgical pathology. 20 (10): 1182–1189. PMID 8827023. Unknown parameter |month= ignored (help)
  6. Dal Cin, Paola; Kools, Patrick; Sciot, Raf; De Wever, Ivo; Van Damme, Boudewijn; Van de Ven, Wim; Van Den Berghe, Herman (1993). "Cytogenetic and fluorescence in situ hybridization investigation of ring chromosomes characterizing a specific pathologic subgroup of adipose tissue tumors". Cancer Genetics and Cytogenetics. 68 (2): 85–90. doi:10.1016/0165-4608(93)90001-3. ISSN 0165-4608.
  7. Dei Tos, Angelo P.; Doglioni, Claudio; Piccinin, Sara; Sciot, Raf; Furlanetto, Alberto; Boiocchi, Mauro; Dal Cin, Paola; Maestro, Roberta; Fletcher, Christopher D. M.; Tallini, Giovanni (2000). "Coordinated expression and amplification of theMDM2,CDK4, andHMGI-C genes in atypical lipomatous tumours". The Journal of Pathology. 190 (5): 531–536. doi:10.1002/(SICI)1096-9896(200004)190:5<531::AID-PATH579>3.0.CO;2-W. ISSN 0022-3417.
  8. Dei Tos, A (2000). "Liposarcoma: New entities and evolving concepts". Annals of Diagnostic Pathology. 4 (4): 252–266. doi:10.1053/adpa.2000.8133. ISSN 1092-9134.
  9. M. D. Kraus, L. Guillou & C. D. Fletcher (1997). "Well-differentiated inflammatory liposarcoma: an uncommon and easily overlooked variant of a common sarcoma". The American journal of surgical pathology. 21 (5): 518–527. PMID 9158675. Unknown parameter |month= ignored (help)
  10. P. Argani, F. Facchetti, G. Inghirami & J. Rosai (1997). "Lymphocyte-rich well-differentiated liposarcoma: report of nine cases". The American journal of surgical pathology. 21 (8): 884–895. PMID 9255251. Unknown parameter |month= ignored (help)
  11. H. L. Evans (1979). "Liposarcoma: a study of 55 cases with a reassessment of its classification". The American journal of surgical pathology. 3 (6): 507–523. PMID 534388. Unknown parameter |month= ignored (help)
  12. A. P. Dei Tos, T. Mentzel, P. L. Newman & C. D. Fletcher (1994). "Spindle cell liposarcoma, a hitherto unrecognized variant of liposarcoma. Analysis of six cases". The American journal of surgical pathology. 18 (9): 913–921. PMID 8067512. Unknown parameter |month= ignored (help)
  13. D. C. Dahlin, K. K. Unni & T. Matsuno (1977). "Malignant (fibrous) histiocytoma of bone--fact or fancy?". Cancer. 39 (4): 1508–1516. PMID 192432. Unknown parameter |month= ignored (help)
  14. 14.0 14.1 Coffin CM, Watterson J, Priest JR, Dehner LP (1995). "Extrapulmonary inflammatory myofibroblastic tumor (inflammatory pseudotumor). A clinicopathologic and immunohistochemical study of 84 cases". Am J Surg Pathol. 19 (8): 859–72. PMID 7611533.
  15. 15.0 15.1 15.2 15.3 Wenig BM, Devaney K, Bisceglia M (1995). "Inflammatory myofibroblastic tumor of the larynx. A clinicopathologic study of eight cases simulating a malignant spindle cell neoplasm". Cancer. 76 (11): 2217–29. PMID 8635024.
  16. 16.0 16.1 Ramachandra S, Hollowood K, Bisceglia M, Fletcher CD (1995). "Inflammatory pseudotumour of soft tissues: a clinicopathological and immunohistochemical analysis of 18 cases". Histopathology. 27 (4): 313–23. PMID 8847061.
  17. 17.0 17.1 Häusler M, Schaade L, Ramaekers VT, Doenges M, Heimann G, Sellhaus B (2003). "Inflammatory pseudotumors of the central nervous system: report of 3 cases and a literature review". Hum Pathol. 34 (3): 253–62. doi:10.1053/hupa.2003.35. PMID 12673560.
  18. 18.0 18.1 18.2 18.3 Rabban JT, Zaloudek CJ, Shekitka KM, Tavassoli FA (2005). "Inflammatory myofibroblastic tumor of the uterus: a clinicopathologic study of 6 cases emphasizing distinction from aggressive mesenchymal tumors". Am J Surg Pathol. 29 (10): 1348–55. PMID 16160478.
  19. 19.0 19.1 Kovach SJ, Fischer AC, Katzman PJ, Salloum RM, Ettinghausen SE, Madeb R; et al. (2006). "Inflammatory myofibroblastic tumors". J Surg Oncol. 94 (5): 385–91. doi:10.1002/jso.20516. PMID 16967468.
  20. 20.0 20.1 Coffin CM, Dehner LP, Meis-Kindblom JM (1998). "Inflammatory myofibroblastic tumor, inflammatory fibrosarcoma, and related lesions: an historical review with differential diagnostic considerations". Semin Diagn Pathol. 15 (2): 102–10. PMID 9606802.
  21. 21.0 21.1 Berardi RS, Lee SS, Chen HP, Stines GJ (1983). "Inflammatory pseudotumors of the lung". Surg Gynecol Obstet. 156 (1): 89–96. PMID 6336632.
  22. 22.0 22.1 Coffin CM, Hornick JL, Fletcher CD (2007). "Inflammatory myofibroblastic tumor: comparison of clinicopathologic, histologic, and immunohistochemical features including ALK expression in atypical and aggressive cases". Am J Surg Pathol. 31 (4): 509–20. doi:10.1097/01.pas.0000213393.57322.c7. PMID 17414097.


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