Extraneural Perineurioma - Tnasicon Poster

A CASE REPORT - EXTRANEURAL SOFT TISSUE PERINEURIOMA – A UNIQUE AND
RARE NEOPLASM OF THE PERIPHERAL NERVE SHEATH

Dr. RAMKI ARUNACHALAM GANESH, Prof. Dr. B.V. SREEDEVI, Dr. S. BALAJI
DEPARTMENT OF GENERAL SURGERY, SREE BALAJI MEDICAL COLLEGE & HOSPITAL, CHENNAI
INTRODUCTION CASE REPORT

Perineuriomas are quite rare benign A 45 year old female presented with complaints of painless swelling over right foot
peripheral nerve sheath tumors, that dorsum over first metatarsal region since over four years. Swelling was insidious in
onset, initially small in size and gradually increased to attain its current size of 4x3
has a predilection for the extremities.
cms. On examination, the swelling had limited mobility, non-tender, skin over swelling
Lazarus and Trombetta were the first was pinchable, with no discoloration/ discharge. There was no edema or induration
to describe it in 1978. Perineuroimas surrounding the swelling.
are classically divided into two Musculoskeletal, vascular and neurological examinations were unremarkable. There
types. Intraneural perineurioma, was no prior history of trauma. There was no other similar swelling anywhere else in
Figure 1: Radiograph of the Figure 2: Soft tissue mass
emerges directly within the nerve the body. Her laboratory investigations sent were within normal limits. Her X ray foot patients right foot exhibiting noted over the first metatarsal
no bony abnormalities region of the patient’s right
trunk. Extraneural perineurioma taken showed no fractures, no calcification or any other periosteal reaction or foot
osteomyelitic changes. Excision biopsy was advised for the patient. After obtaining
manifests as a well-circumscribed
informed consent and necessary preparation, excision biopsy was done.
subcutaneous or, in rare cases, deep
A linear incision was placed over the skin above the swelling. Incision was deepened by
soft tissue or cutaneous mass that is blunt dissection. A firm, greyish white mass originating from the extensor hallucis
not associated with a distinguishable longus (EHL) tendon sheath was revealed. The mass excised intoto was approximately
nerve. 4x3x1.5 cms in size and was sent for histopathological examination. The EHL tendon
When compared to intraneural was found to be intact. Wound was thoroughly washed with normal saline. After
ensuring complete hemostasis, skin was sutured with 3-0 Ethilon.
perineuriomas, extraneural
Post-operative course of the patient was uneventful and their no neural deficit or any Figure 3: Intraoperative picture Figure 4: Excised Soft tissue
perineuriomas are more frequent. other complications. There was no evidence of tumor recurrence 12 months post
revealing the soft tissue tumor. tumor
Extraneural perineurioma are further surgery.

subclassified into classical, The histopathological examination of the lesion showed well defined proliferation of
Figure 5: HPE slide showing
sclerosing and reticular/retiform slender spindle cells in short whorl like pattern, consistent with Soft tissue extraneural features of perrineurioma.
subtypes. perineurioma. The Immunohistochemical analysis of the lesion was positive for EMA
and Negative for S-100 ruling out schwanomma and characteristic of extraneural
perineurioma.
RESEARCH POSTER PRESENTATION DESIGN © 2019
www.PosterPresentations.com
A CASE REPORT - EXTRANEURAL SOFT TISSUE PERINEURIOMA – A UNIQUE AND
RARE NEOPLASM OF THE PERIPHERAL NERVE SHEATH
Dr. RAMKI ARUNACHALAM GANESH, Prof. Dr. B.V. SREEDEVI, Dr. S. BALAJI
DEPARTMENT OF GENERAL SURGERY, SREE BALAJI MEDICAL COLLEGE & HOSPITAL, CHENNAI
DISCUSSION CONCLUSION
Perineuriomas have unique morphological, ultrastructural, and The histologic characteristics of many other benign and
immunophenotypic characteristics, which set them apart from the
considerably more prevalent nerve sheath tumours. Very thin cytoplasmic
malignant soft tissue tumours overlap with those of perineurioma,
processes with numerous pinocytotic vesicles, an incomplete external a rare and underappreciated benign neoplasm. The right diagnosis
lamina, and frequent junctional complexes are the characteristics is primarily based on the immunohistochemical profile and
exhibited by perineurial cells. Perineurial cells exhibit positive histologic findings, but in challenging cases, ultrastructural
immunoreactivity for the EMA, type-4 collagen, and laminin, but not for studies may be necessary. Although cytogenetic and molecular
the S-100 protein, nuerofilaments, or CD34. They can be distinguished
from Schwann cells, which have a continuous basement membrane and
genetic studies still have a limited role in the diagnosis of
positively stain for the S-100 protein, by their lack of both of these perineuriomas, they may be crucial in ruling out serious
features. Firm, non-encapsulated, well-circumscribed lesions in the differential diagnoses like DFSP and low-grade fibromyxoid
dermis, subcutaneous tissue, or deep soft tissue are the hallmarks of soft sarcoma.
tissue perineurioma. These tumours appear white to grey upon gross
inspection. They exhibit elongated, spindled cells with loose storiform,
short fascicular, and whorled growth patterns under the microscope.
REFERENCES
1. Hornick J.L. ,Fletcher C.D. , Fletcher J.A. ,Perineuroma. Fletcher D.M.C, Bridge Julia A,Hogendoorn Pancras C.W. , Mertens
Both Intraneural and Soft tissue perineurioma has almost equal Fredrik editors.WHO, Classification of Soft tissue and Bone, 5th edition, Lyon 2013 p 176-7
2. Lazarus SS, Trombetta LD. Ultrastructural identification of a benign perineurial cell tumor. Cancer. 1978;41(5):1823–9.
prevalence in both the sexes. In a study conducted by Hornick et al, 3. Graadt van Roggen JF, McMenamin ME, Belchis DA, Nielsen GP, Rosenberg AE, Fletcher CD. Reticular perineurioma: a
distinctive variant of soft tissue perineurioma. Am J Surg Pathol. 2001;25(4):485–93.
comprising of 81 cases of soft tissue perineurioma between 1994 and 4. Hornick JL, Fletcher CD. Soft tissue perineurioma: clinicopathologic analysis of 81 cases including those with atypical
2003, the majority of the tumours developed in the subcutaneous histologic features. Am J Surg Pathol. 2005;29(7):845–58.
5. Fetsch JF, Miettinen M. Sclerosing perineurioma: a clinicopathologic study of 19 cases of a distinctive soft tissue lesion with a
tissues, with the remaining ones developing in the dermis or deep predilection for the fingers and palms of young adults. Am J Surg Pathol. 1997;21(12):1433–42.
soft tissue layers. The lower extremity (n = 36), upper extremity (n = 6. Hirose T, Scheithauer BW, Sano T. Perineurial malignant peripheral nerve sheath tumor (MPNST): a clinicopathologic,
immunohistochemical, and ultrastructural study of seven cases. Am J Surg Pathol. 1998;22(11):1368–78.
19), or trunk (n = 15) were the areas where tumours were most likely 7. Nishio J, Iwasaki H, Hayashi H, Nabeshima K, Naito M. Soft tissue perineurioma of the foot with 10q24 rearrangements:
unique MRI features with histopathologic correlation. Skelet Radiol. 2014;43(7):1017–22.
to develop, with the head and neck region, retroperitoneum, and 8. Klein CJ, Wu Y, Jentoft ME, Mer G, Spinner RJ, Dyck PJ, et al. Genomic analysis reveals frequent TRAF7 mutations in
paratesticular accounting for the remaining cases. intraneural perineuriomas. Ann Neurol. 2017;81(2):316–21.
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A CASE REPORT - EXTRANEURAL SOFT TISSUE PERINEURIOMA – A UNIQUE AND

RARE NEOPLASM OF THE PERIPHERAL NERVE SHEATH

INTRODUCTION CASE REPORT

Extraneural perineurioma are further surgery.

RESEARCH POSTER PRESENTATION DESIGN © 2019

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