- Case report
- Open Access
Intraneural hemangioma of the median nerve: A case report
© Doğramacı et al. 2008
- Received: 16 December 2007
- Accepted: 22 February 2008
- Published: 22 February 2008
Hemangiomas of the median nerve are very rare and, so far, only ten cases of intraneural hemangioma of this nerve have been reported in the literature. We present a case of 14-year-old girl who had a soft tissue mass in the region of the left wrist with signs and symptoms of carpal tunnel syndrome. Total removal of the mass was achieved using microsurgical epineural and interfasicular dissection. The symptoms were relieved completely, after this procedure, without any neurologic deficit. On follow-up two years later, no recurrence was observed. Whenever a child or young adult patient presents with CTS the possibility of a hemangioma involving the median nerve should be kept in mind in the differential diagnosis.
The carpal tunnel syndrome (CTS) is the most common neuropathy due to compression seen in adults. There are very few cases in the literature referring to patients of paediatric age . Most of these young patients had a metabolic disorder mucopolysaccharidosis or mucolipidosis. Other unusual causes of CTS in children are fibrolipomas of the median nerve or intraneural perineuroma or haemangioma, haemophilia (secondary to local bleeding), musculotendinous malformation, Klippel-Trenaunay syndrome, Poland's syndrome, scleroderma, benign localised form of gigantism, intensive sports practice, and primary familial CTS . Very rarely Schwannomas of the median nerve can be mistakenly diagnosed and present as carpal tunnel syndrome [2–4]. Lipofibromatous hamartoma of the median nerve at the wrist was reported, and caused macrodactyly of the digits, and also resulted in symptoms of carpal tunnel syndrome [5–7]. Again epithelioid sarcoma of the median nerve may present with symptoms and signs of carpal tunnel syndrome . An isolated malignant peripheral nerve sheath tumor of mild type has also been reported to present with symptoms and signs of carpal tunnel syndrome .
Posttraumatic neuroma-in-continuity of the median nerve causing median nerve compression is rare . Damage to the median nerve after vascular graft placement as a result of an occult mass has been documented in a single case .
Intraneural hemangioma of the median nerve is a rare condition and only ten cases have been described in the literature [12–20]. Due to mechanical compression, carpal tunnel syndrome (CTS) is the main presenting feature [12–18]. Raynaud's phenomenon may be an associated complaint .
Here we present a case of intraneural hemangioma of the median nerve of a 14-year-old female removed surgically by combined interfasicular and epineural resection, no recurrence observed during the two years of postoperative follow-up period.
A 14-year-old female student presented to our outpatient clinic with painful swelling in the volar surface of the right wrist of 3 years duration; associated with tingling and numbness in the thumb, index, middle and radial half of the ring fingers, difficulty in writing long paragraphs. There was no history of trauma and relevant medical condition.
Physical examination revealed a tender, soft mass, 3 × 5 × 2 cm in dimension in the volar aspect of the right wrist. Tinel sign was positive.
EMG examination was planned for this patient, but the patient refused to cooperate during the test and the test was not completed successfully.
After preoperative assessment, the patient was admitted for surgical treatment under the diagnosis of volar ganglion causing CTS.
The symptoms were relieved in the first three weeks after the operation. On clinical and ultrasound examination, no recurrence was observed in the first two years following the operation.
Benign intraneural hemangioma originating from peripheral nerves is rare. Most patients present in with a painful, soft mass along the path of a nerve with signs and symptoms of nerve compression and entrapment.
A thorough search through the literature revealed ten cases of hemangioma of the median nerve [12–19]. In all the described cases CTS is the presenting feature and in one case Raynaud's phenomenon was an associated presenting feature.
The tumor may not be easily recognised until it becomes painful and it is rarely diagnosed before surgery. In the differential diagnosis, lipoma, lipofibroma, hamartoma and intraneuronal Schwannoma must be considered [20, 21].
Ultrasonography may give useful information about the nerve's dynamic relation to the surrounding musculotendinous structures  and nerve conduction studies may reveal non specific features of compressive neuropathies . For appropriate planning of surgical therapy and preoperative diagnosis, MRI is essential and gives useful information regarding tumor location, size, extent and relationship of peripheral nerve.
Hemangioma shows a hyperintense signal on T1- and T2- weighted images with fat suppression sequences. Flow voids are usually apparent and feeding vessels may be visualized; these lesions are also noted to enhance after Gd-addition. On angiography an early and persistent tumoral blush is demonstrated .
Schwannoma is a slightly hypodense, solid tumor with no vascular contrast enhancement on CT. MRI shows intermediate signals on. T1-W, and T2-W imaging shows high signal intensity with some heterogenity . Lipomas exhibit signal characteristics consistent with those of normal adipose tissue: homogeneous hyperintensity on T1- and T2-weighted sequences . MR imaging findings of lipofibromatous hamartoma are pathognomonic which consist of serpiginous T1- and T2-weighted low-intensity structures containing and surrounded by fat (hyperintense on T1- and hypointense on T2-weighted fat suppression sequences), giving the lesion a spaghetti-like appearance on sagittal images, and a "coaxial cable-like" appearance on coronal images .
No certain protocol has been established to manage this difficult condition, however conservative treatment usually fails and surgery is the treatment of choice. When possible total resection of intraneural hemangiomas is curative, partial resection may relieve symptoms but recurrence may occur which may require en-bloc nerve resection and repair with nerve graft .
The longest period of follow-up without recurrence has been reported by Oztekin et al. . They reported a case of CTS due to a cavernous hemangioma of the median nerve, which was successfully removed by epineural resection, and no recurrence was observed over a 6 year follow-up period. Patel et al.  reported two cases of hemangioma of the median nerve which they treated by partial excision and resulted in recurrence in the third year, one of the recurred case managed by resection of median nerve and nerve grafting without recurrence, four years after surgery.
Chatillon et al.  reported the first case of using radiotherapy in the treatment of intraneural hemangioma. Preoperative embolization and postoperative radiotherapy combined with partial resection were beneficial in a case of intraneural hemangioma involving inferior trunk of brachial plexus and resulted in symptomatic relief and radiologic shrinkage in the size of the mass seen on serial follow-up MRI images, with a follow-up period of two years.
In our case, total resection of the hemangioma was achieved by combined epineural resection and interfasicular dissection with microsurgical resection technique, no neurologic complications observed postoperatively and no recurrence observed in the two year follow-up period.
The type of microsurgical dissection and resection should be decided at the time of surgery and careful preoperative planning using MRI, and if needed angiography, is essential for cystic lesions of the volar side of wrist. Excision of the affected nerve and grafting should be the last choice and should only be used in complicated cases and when there are frequent recurrences.
Whenever a child or young adult patient presents with CTS the possibility of a hemangioma involving the median nerve should be kept in mind in the differential diagnosis.
Written informed consent was obtained from the patient for publication of this Case report and accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal.
- Van Meir N, De Smet L: Carpal tunnel syndrome in children. Acta Orthop Belg 2003, 69:387–395.PubMedGoogle Scholar
- Aslam N, Kerr G: Multiple Schwannomas of the median nerve: a case report and literature review. Hand Surg 2003, 8:249–252.View ArticlePubMedGoogle Scholar
- Padua L, Pazzaglia C, Insola A, Aprile I, Caliandro P, Rampoldi M, Bertolini C, Tonali P: Schwannoma of the median nerve (even outside the wrist) may mimic carpal tunnel syndrome. Neurol Sci 2006, 26:430–434.View ArticlePubMedGoogle Scholar
- Aydin MD, Kotan D, Keles M: Acute median nerve palsy due to hemorrhaged schwannoma: case Report. JBPPNI 2007, 2:19.Google Scholar
- Bagatur AE: Lipofibromatous hamartoma of the median nerve. Acta Orthop Traumatol Turc 2002, 36:172–176.PubMedGoogle Scholar
- Lorenzoni PJ, Lange MC, Kay CS, Silvado CE, Scola RH, Werneck LC: Fibrolipomatous hamartoma of the median nerve: case report. Arq Neuropsiquiatr 2005,63(B):881–884.PubMedGoogle Scholar
- Bains R, Kotwal A, Saeed W: Recurrent carpal tunnel syndrome in a child due to fibrolipomatous hamartoma of the median nerve successfully treated by limited excision and decompression. J Plast Reconstr Aesthet Surg 2006, 59:1394–1397.View ArticlePubMedGoogle Scholar
- Harish S, Saifuddin A, Fajinmi M: Epithelioid sarcoma of the median nerve mimicking a peripheral nerve sheath tumour. Australas Radiol 2007, 51:71–74.View ArticlePubMedGoogle Scholar
- Ochsner F, Baumann RP, Kuntzer T: Carpal tunnel syndrome with an unusual cause: a malignant nerve sheath tumor of the median nerve. Rev Neurol (Paris) 2001, 157:1547–1549.Google Scholar
- Martinelli P, Poppi M, Gaist G, Padovani R, Pozzati E: Posttraumatic neuroma of the median nerve: a cause of carpal tunnel syndrome. Eur Neurol 1985, 24:13–15.View ArticlePubMedGoogle Scholar
- Goldstein LJ, Helfend LK, Kordestani RK: Postoperative edema after vascular access causing nerve compression secondary to the presence of a perineuronal lipoma: case report. Neurosurgery 2002, 50:412–414.View ArticlePubMedGoogle Scholar
- Coessens B, De Mey A, Lacotte B, Vandenbroeck D: Carpal tunnel syndrome due to an haemangioma of the median nerve in a 12-year-old child. Ann Chir Main Memb Super 1991, 10:255–257.View ArticlePubMedGoogle Scholar
- Kojima T, Ide Y, Marumo E, Ishikawa E, Yamashita H: Haemangioma of median nerve causing carpal tunnel syndrome. Hand 1976, 8:62–65.View ArticleGoogle Scholar
- Patel CB, Tsai TM, Kleinert HE: Hemangioma of the median nerve: a report of two cases. J Hand Surg [Am] 1986, 11:76–79.Google Scholar
- Peled I, Iosipovich Z, Rousso M, Wexler MR: Hemangioma of the median nerve. J Hand Surg [Am] 1980, 5:363–365.Google Scholar
- Prosser AJ, Burke FD: Haemangioma of the median nerve associated with Raynaud's phenomenon. J Hand Surg [Br] 1987, 12:227–228.Google Scholar
- Wood MB: Intraneural hemangioma: report of a case. Plast Reconstr Surg 1980, 65:74–76.View ArticlePubMedGoogle Scholar
- Oztekin HH, Karaarslan AA: Carpal tunnel syndrome due to a cavernous hemangioma of the median nerve. Acta Orthop Traumatol Turc 2003, 37:170–172.PubMedGoogle Scholar
- Petrovici V: Cavernous hemangioma of the palm with symptoms resembling carpal tunnel syndrome. Z Plast Chir 1980, 4:40–47.PubMedGoogle Scholar
- Chatillon CE, Guiot MC, Jacques L: Lipomatous, vascular, and chondromatous benign tumors of the peripheral nerves: Representative cases and review of the literature. Neurosurg Focus 2007, 22:E18.View ArticlePubMedGoogle Scholar
- Louis D: Peripheral nerve tumors of the upper extremity. Hand Clin 1987, 3:311–318.PubMedGoogle Scholar
- Kuo YL, Yao WJ, Chiu HY: Role of sonography in the preoperative assessment of neurilemmoma. J Clin Ultrasound 2005, 33:87–89.View ArticlePubMedGoogle Scholar
- MacDonell RAL, Schwartz MS, Swash M: Carpal Tunnel Syndrome: Which finger should be tested? An analysis sensory conduction in digital branches of the median nerve. Muscle Nerve 1990, 13:601–606.View ArticlePubMedGoogle Scholar
- Hems TE, Burge PD, Wilson DJ: The role of magnetic resonance imaging in the management of peripheral nerve tumours. J Hand Surg 1997,22(1):57–60.View ArticleGoogle Scholar
- Chiao HC, Marks KE, Bauer TW, Pflanze W: Intraneural lipoma of the sciatic nerve. Clin Orthop Relat Res 1987, 221:267–271.PubMedGoogle Scholar
- Marom EM, Helms CA: Fibrolipomatous hamartoma: pathognomonic on MR imaging. Skeletal Radiol 1999, 28:260–264.View ArticlePubMedGoogle Scholar
This article is published under license to BioMed Central Ltd. This is an Open Access article distributed under the terms of the Creative Commons Attribution License (http://creativecommons.org/licenses/by/2.0), which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.