Rare case of Intravascular papillary hemangioendothelioma presenting as Carpal tunnel syndrome.

Written By : Dr Supreeth D R |Medically Reviewed By : Dr. Kamal Kant Kohli Published On 2023-06-15T20:00:28+05:30 | Updated On 15 Jun 2023 8:00 PM IST

IVPH is a benign lesion previously reported in the nasal cavity, neck, upper extremities, and breast. Diagnosis with cross-sectional imaging can prove difficult, with histopathological examination necessary for diagnosis. IVPH resulting in carpal tunnel symptoms is quite rare.

The rare case has been reported by Vrajesh J. Shah et al in ‘Skeletal radiology’ journal.

The patient is a 37-year-old right-hand dominant woman who first presented at an outside facility for examination of a radial, volar right wrist mass. She initially noted the hard mass in 2016. She experienced nerve-like pain, numbness, and tingling from her wrist to thumb with exercise. She denied any prior trauma. Electromyography did not reveal nerve damage. Magnetic resonance imaging (MRI) suggested a peripheral nerve sheath tumor.

At the time, the patient declined surgical intervention as her symptoms did not greatly impede her daily life. She presented to the author’s hospital 3 years later, reporting interval growth of the mass and pain with hyperextension loading and minor accidental collisions. Focused examination of her right hand and wrist revealed a 3.0 × 2.0-cm mobile mass at the volar aspect of the right wrist, just beneath the palmaris longus tendon in the distal forearm, which was mildly tender to palpation. No subjective sensory deficits were noted to light touch. The patient had full strength, and no thenar atrophy was appreciated. There was a negative Tinel sign over the mass.

Multiplanar multisequence MRI of the right wrist was obtained before and after 4.5 mL of an intravenous gadolinium-based contrast. This showed a lobulated, predominantly T2 hyperintense, heterogeneously enhancing mass measuring 2.0 cm transverse × 1.2 cm dorsal-volar × 3.1 cm in length along the volar-radial aspect of the distal forearm/wrist. T1-weighted sequences demonstrated the “split fat” and “tail sign” indicating the neural origin of the mass. T2-weighted sequences revealed small, rounded areas of intermediate to low signal with the mass noted. The mass was located along the course of the median nerve, deep to the palmaris longus and flexor carpi radialis tendons with nodular areas of internal enhancement and was reported to be consistent with a peripheral nerve sheath tumor. The patient elected to undergo elective surgical removal of the symptomatic, enlarging mass.

An extensive carpal tunnel approach was drawn, and an incision was made in the distal forearm ulnar to palmaris longus. After careful dissection of the superficial soft tissue to the volar forearm fascia with loupe magnification, the palmaris longus was radially retracted to expose the median nerve. A large, fragile, seemingly vascular mass was examined with the median nerve. Under microscopic magnification, a meticulous dissection of the fragile mass from the fascicles of the median nerve was performed. The tumor appeared to have a “bag of worms” network of infltrative vascular channels interdigitated among the fascicles of the median nerves. A carpal tunnel release was performed to prevent nerve compression from the inflammatory response secondary to extensive fascicular manipulation. The wound was then copiously irrigated and reapproximated.

Histopathology of the specimen revealed multiple irregularly contoured vascular caverns lined by endothelial cells with dense fibrous connective tissue septae, features suggestive of a cavernous hemangioma. Additionally noted within the vascular spaces were multiple small organizing thrombi and focally prominent papillary endothelial hyperplasia typical of IVPH.

The patient recovered well after her right median nerve release and extensive internal neurolysis with removal of the IVPH.

The authors concluded that – “Carpal tunnel syndrome, in exceedingly rare occasions, can result from an IVPH. MRI findings may be confused with more common entities. Histopathological confirmation remains necessary for conclusive diagnosis.”

MBBS, Dip. Ortho, DNB ortho, MNAMS

Dr Supreeth D R (MBBS, Dip. Ortho, DNB ortho, MNAMS) is a practicing orthopedician with interest in medical research and publishing articles. He completed MBBS from mysore medical college, dip ortho from Trivandrum medical college and sec. DNB from Manipal Hospital, Bengaluru. He has expirence of 7years in the field of orthopedics. He has presented scientific papers & posters in various state, national and international conferences. His interest in writing articles lead the way to join medical dialogues. He can be contacted at editorial@medicaldialogues.in.

Dr. Kamal Kant Kohli

Dr Kamal Kant Kohli-MBBS, DTCD- a chest specialist with more than 30 years of practice and a flair for writing clinical articles, Dr Kamal Kant Kohli joined Medical Dialogues as a Chief Editor of Medical News. Besides writing articles, as an editor, he proofreads and verifies all the medical content published on Medical Dialogues including those coming from journals, studies,medical conferences,guidelines etc. Email: drkohli@medicaldialogues.in. Contact no. 011-43720751