Orofacial overgrowth with peripheral nerve enlargement and perineuriomatous pseudo-onion bulb proliferations is part of the PIK3CA-related overgrowth spectrum

Abstract Context.—Peripheral nerve masses are frequently encountered in surgical pathology practice. However, once a peripheral nerve mass is determined not to be a nerve sheath neoplasm, differential diagnostic considerations drop off sharply. Objective.—To review our experience with surgically resected nerve masses. Design.—Retrospective search of pathology database. Rare neoplasms were studied by cytogenetic analysis or gene microarray. Results.—Four hundred fifty-eight cases were identified. After elimination of common lesions (mostly nerve sheath tumors), 37 cases (8%) remained, almost all of which were of non–nerve sheath origin: for example, hemangioma, metastatic neuroendocrine pancreatic carcinoma, meningiomas invading nerve fascicles, and primary extrarenal rhabdoid tumor and Ewing sarcoma of nerve. The latter showed rearrangement of the EWSR1 locus (22q12). The gene expression pattern of an undifferentiated sarcoma, presenting as ropelike nerve enlargement, clustered with malignant peripheral nerve sheath neoplasms but not other sarcomas or neuroepithelial tumors. Conclusions.—Diverse benign and malignant conditions can affect peripheral nerve.

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Peripheral nerve enlargement on nerve ultrasound parallels neuropathological changes in adult‐onset Krabbe disease

Muscle & Nerve ◽

10.1002/mus.27175 ◽

2021 ◽

Author(s):

Matteo Tagliapietra ◽

Francesco Crescenzo ◽

Barbara Castellotti ◽

Cinzia Gellera ◽

Diana Polo ◽

...

Keyword(s):

Peripheral Nerve ◽

Krabbe Disease ◽

Adult Onset ◽

Nerve Ultrasound ◽

Nerve Enlargement

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P-21 Relationship between IGF-I levels and peripheral nerve enlargement in acromegaly

Growth Hormone & IGF Research ◽

10.1016/s1096-6374(08)70106-0 ◽

2008 ◽

Vol 18 ◽

pp. S33 ◽

Cited By ~ 1

Author(s):

E. Resmini ◽

A. Tagliafico ◽

R. Nizzo ◽

F. Bianchi ◽

F. Minuto ◽

...

Keyword(s):

Peripheral Nerve ◽

Nerve Enlargement ◽

Igf I

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Facilitated migration of cells from a transected peripheral nerve over collagen fibers: in vivo observations

Proceedings, annual meeting, Electron Microscopy Society of America ◽

10.1017/s0424820100156420 ◽

1989 ◽

Vol 47 ◽

pp. 888-889

Author(s):

Arthur J. Wasserman ◽

Azam Rizvi ◽

George Zazanis ◽

Frederick H. Silver

Keyword(s):

Sciatic Nerve ◽

Peripheral Nerve ◽

Collagen Gel ◽

Collagen Fibers ◽

Control Group ◽

Guide Tube ◽

Sprague Dawley ◽

Silicone Tube ◽

Thin Sections

In cases of peripheral nerve damage the gap between proximal and distal stumps can be closed by suturing the ends together, using a nerve graft, or by nerve tubulization. Suturing allows regeneration but does not prevent formation of painful neuromas which adhere to adjacent tissues. Autografts are not reported to be as good as tubulization and require a second surgical site with additional risks and complications. Tubulization involves implanting a nerve guide tube that will provide a stable environment for axon proliferation while simultaneously preventing formation of fibrous scar tissue. Supplementing tubes with a collagen gel or collagen plus extracellular matrix factors is reported to increase axon proliferation when compared to controls. But there is no information regarding the use of collagen fibers to guide nerve cell migration through a tube. This communication reports ultrastructural observations on rat sciatic nerve regeneration through a silicone nerve stent containing crosslinked collagen fibers.Collagen fibers were prepared as described previously. The fibers were threaded through a silicone tube to form a central plug. One cm segments of sciatic nerve were excised from Sprague Dawley rats. A control group of rats received a silicone tube implant without collagen while an experimental group received the silicone tube containing a collagen fiber plug. At 4 and 6 weeks postoperatively, the implants were removed and fixed in 2.5% glutaraldehyde buffered by 0.1 M cacodylate containing 1.5 mM CaCl2 and balanced by 0.1 M sucrose. The explants were post-fixed in 1% OSO4, block stained in 1% uranyl acetate, dehydrated and embedded in Epon. Axons were counted on montages prepared at a total magnification of 1700x. Montages were viewed through a dissecting microscope. Thin sections were sampled from the proximal, middle and distal regions of regenerating sciatic plugs.

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