Hand Soft Tissue Tumor Quiz 2 22/9/86

13 y old girl complained from CTS like symptoms and a soft tissue mass in the anterior surface of the wrist.

P/I: ◦From 2 years ago insidious onset of CTS symptoms ◦From one year ago the mass appeared with very slow enlarging

P/E: ◦Soft tumor 2*4 cm. in size ◦Mobile without definitive borders ◦Tinel sign is negative

Paraclinical findings ◦EMG-NCV showed mild to moderate CTS ◦Plain X-ray is normal ◦MRI, soft tissue tumor adjacent to flexor tendons in carpal tunnel

MRI

The patient underwent operation Extensile transverse carpal ligament release

Intra-operative photography

Proximal limit

Distal end

Internal architecture of the tumor

Questions ? What is the best diagnosis? What is the result of histo-pathology? What is the definitive treatment? What is the prognosis?

Answers ;

This is a case of DEJERINE SOTTAS DISEASE : a localized enlargement of a peripheral nerve caused by hypertrophic interstitial neuropathy. The most common site is median nerve.It cannot be excised whith out resecting the nerve with should not be done. Dividing the transvese carpal ligament may help to releive pain & occasionaly has decreased the size of the nerve distally. The swelling will subside spontanously after surgery

javabe case DEJEINE-SOUTASS ast. type 3 C-M-T be tore localize asab haye mohity ra dargir mikonad. be tore typic asabe median dar gir ast. be dalile dysmyelination, myelin haye asab daeman takhrib va bazsazi mishavad. va be in dalil asab besyar bozorg va hypertrophy mishavad. biopsy be tashkhis komak konanade ast tamame toole asab dargir ast darman serfan baz kardane toonele carp ast

Discusion EMG and NCV could revealed the peripheral neuropathies with the abnormal findings in other nerves. DEJEINE-SOUTASS is usually benign disease without progression Carpal ligament release is suitable choice.