Perineural hemangiomas of the upper extremity: Report of four cases Perineural hemangiomas in the upper extremity have been the subject of few reports. We found only eight such cases reported in the literature. We report four additional cases in which there were neurologic symptoms. The specific diagnosis of cavernous hemangioma was not made preoperatively
in any of the patients. The greater the extent of intrafascicular
more difficult is total eradication
of the hemangioma.
Dean S. Louis, MD, and Paul T. Forth,
V
ascular malformations may occur in just about any location in the body.‘. * At times they may even be life-threatening. Major arteriovenous malformations may lead to congestive heart failure, encroach upon vital structures, lead to hemmorrhage, or cause a consumption coagulopathy (the Hasbach-Merritt syndrome). When such problems do occur, the diagnosis has usually been known for some time and visualized by vascular imaging techniques. Less obvious presentations may result from perineural hemangiomas, which, although symptomatic, are not lifethreatening. During the past 20 years, we have seen four patients with hemangiomas in perineural locations in the upper extremity. In all four patients the diagnosis was not suspected at the time of the initial surgery. Case reports Case 1. A 2 1-year-old college student with an established diagnosis of neurofibromatosis had been followed up for more than 3 years because of vague pain in her right forearm. The pain was initially episodic in nature, but it had become almost constant and had radiated in the median nerve distribution. After a mass was felt in the proximal third of the forearm,
From the Section of Orthopaedic Surgery. University of Michigan, Ann Arbor, Mich. Received for publication March 26. 1991: accepted in revised form Aug. 23. 1991. No benefits in any form have &en received or will be received from a commercial party related directly or indirectly to the subject of this article. Reprint requests: Dean S. Louis, MD. Section of Orthopedics. versity of Michigan, Ann Arbor, MI 48109. 3/I/33720
308
THE JOURNAL
OF HAND SURGERY
Uni-
involvement,
the
(J HAND SURG1992;17A:308-11.)
MD, Ann Arbor, Mich.
an exploratory procedure was performed. The median nerve was seen to have a vascular malformation within the epineural sheath, but this was extrafascicular in location (Fig. 1). With the use of magnification, the observed malformation was removed from the nerve without difficulty. At follow-up 6 months postoperatively, the symptoms were no longer present. The long period of evaluation before the mass came evident indicates the difficulty in making this specific diagnosis.
Case 2. A lo-year-old girl was seen because of a painful mass in the posterior part of the arm. A previous biopsy had resulted in extensive bleeding, and the patient was referred for our evaluation. There was a firmness in the posterior medial part of the arm in the distal third. Palpation resulted in discomfort in the ulnar nerve distribution. At operation an extensive hemangioma was visualized extending for a distance of about 15 cm both above and below the medial epicondyle (Fig. 2). Its location was intraepineural and extrafascicular in the ulnar nerve. All visible abnormal tissue was removed. Twelve years after this resection the patient is free of symptoms. A preoperative angiogram was not performed. Although it might have been helpful in making the diagnosis preoperatively, the outcome was not influenced by the absence of the study. Case 3. A 29-year-old secretary was seen because of a recurrent mass between the webs of the long and ring fingers of her right hand. She had undergone excision of a painful mass in the same area on three separate occasions. Pathologic findings indicated a hemangioma. An arteriogram revealed a vascular malformation in the area of the web space of the long and ring fingers. Exploration revealed a vascular web that encircled the long finger metacarpal and both digital nerves. All visible abnormal tissue was removed with the aid of magnification. There was extensive residual scarring from the previous operations. Postoperatively the patient experienced persistent tingling and numbness in the long finger. which created difficulty for her when she used a typewriter.
Vol. 17A. No. 2 March 1992
Perineurul
hemangiomas
oj. upper extremic
Fig. 1. Intraepineural hemangioma present in the median nerve just at the point where it emerges from beneath the two heads of the flexor digitorum superticialis. With the use of magnification. the tumor was completely excised, and complete relief of all symptoms was achieved.
Fig. 2. Another example of a more diffuse type of intraepineural, This was completely excised with the use of magnification.
but extrafascicular,
hemangioma.
309
310
Louis and Fortin
The Journal of HAND SURGERY
Fig. 3. An example of a combined extraneural hemangioma (open large arrow) and an additional diffuse intrafascicular, intraneural hemangioma (double-ended arrow). The hemangioma here involves the ulnar nerve and its dorsal sensory branch distally. Both of these are seen under the tip of the hemostat. The lesion can be seen to extend in both directions.
Fig. 4. Histologic specimen from case 4. The multiple vascular spaces are seen in the midst of a homogeneous and uniform stroma. This is representative of the lesions presented in this small series. (Original magnification X 25 .)
Vol. 17A, No. 2 March 1992
Case 4. A healthy 13-year-old girl was seen because of a painful mass at the ulnar border of her forearm distally. Exploration revealed an extensive mass that involved the ulnar nerve and appeared to be composed of blood vessels. The patient was referred to a hand surgeon. who explored the area 6 months later but found that there was little separation between the fascicles and the vascular malformation. Because of the fear of nerve injury only a limited biopsy was performed (Fig. 3). Histologic study revealed multiple vascular spaces within a homogeneous stroma (Fig. 4). Nine years later there has been no progression or recurrence of the mass. Function has not changed. Discussion Vascular malformations are rare and perplexing anomalies that to a large extent frustrate surgeons’ efforts to restore normal local function. These anomalies may occur throughout the body.‘, ’ Upper extremity involvement has been the subject of a few reports3-’ Eight reported cases have involved nerves of the upper extremity.3-8 Involvement of the nerves has been of three types. Type I is an intraneural extrafascicular malformation that is relatively easily removed with magnification. Type II is an intrafascicular encompassing type that is deemed unresectable because of the potential for loss of nerve function secondary to the required dissection. Type III has both intraneural and extraneural components. Losl? reported a case of massive involvement of the ulnar nerve in which the entire mass and the adjacent nerve were resected. He reviewed the literature up to the time of his report and included one other case of Satoh that involved the median nerve in the forearm in a 64-year-old man with a 1Cyear follow-up but without further details. Since that time, Kojima et al..3 Peled et al. ,’ Kon and Vuursteen4 Pate1 et al. .’ and Prosser and Burke” have contributed six additional cases. All of these hemangiomas occurred in female patients between 4 and 19 years of age. Five of these had median nerve involvement, and one involved a digital nerve.J Treatment varied from excision and nerve grafting’ ’ to limited local resection to preserve existing function. In the case reported by Prosser and Burke’ there was an associated Raynaud’s phenomenon. Limited resections were done on four occasions over a 25-year period. The patient was reported to have maintained function at the time of the 25-year follow-up. A compressive neurop-
Peritleurul hemangiomus
gf upper extremity
311
athy may occur, as in the ulnar neuropathy reported by Losl? and the carpal tunnel compression reported by Kojima et al.’ Hemangiomas that involve nerves may cause symptoms of pain. paresthesia and numbness. and accompanying signs of weakness and atrophy may also be evident. The extent to which these symptoms and signs are present will vary, depending on the size and location of the hemangiomas. Nerves compressed by hemangiomas that are external to the nerve will respond well to removal of the adjacent hemangioma.’ Cases in which the hemangioma is intraneural but essentially extrafascicular tend to do well with local excision alone, as in our cases 1 and 2. Cases in which there is extensive involvement of the hemangioma with the fascicular structure of the nerve are the most difficult ones to manage. In such situations limited local excision may lead to recurrence. as in our cases 3 and 4. Total excision with nerve grafting may eliminate the hemangioma. but it will certainly cause other deficits. Our four cases. like seven of the previously reported eight cases in the upper extremity. involved female patients. REFERENCES I. Weber TR, Connors RH, Tracy TF Jr, Bailey PV. Complex hemangiomas of infants and children. Arch Surg 1990:125:1017-21. 2. Griffin JM. Vasconez LO. Schatten WE. Congenital arteriovenous malformations of the upper extremity. Plast Reconstr Surg 1978;62:49-57. 3. Kojima T, lde Y. Marumo E, lshikawa E. Yamasha H. Haemangioma of median nerve causing carpal tunnel syndrome. Hand 1976;8:62-5. 4. Kon M. Vuursteen PJ. An intraneural hemangioma of a digital nerve: case report. J HAND SURG 1981;6:357-8. 5. Losli EJ. Intrinsic hemangiomas of the peripheral nerves: a report of two cases and a review of the literature. Arch Path01 1952;53:226-32. 6. Sato S. uber das cavern&e Angiom des periphenschen nervensystem. Arch Klin Chir 19 I3;100:552. Pate1 CB. Tsai T. Kleinert HE. Hemangioma of the median nerve: a report of two cases. J HANI) SURG 1986;l IA: 76-9. Peled 1, Iosipovich Z, Rousso M, Wexler MR. Hemangioma of the median nerve. J HAND SURG 1980;5:363-5. Prosser AJ. Burke FD. Haemangioma of the median nerve associated with Raynaud’s phenomenon. J HAND SURG 12B:227-8.
in any of the patients. The greater the extent of intrafascicular
more difficult is total eradication
of the hemangioma.
Dean S. Louis, MD, and Paul T. Forth,
V
ascular malformations may occur in just about any location in the body.‘. * At times they may even be life-threatening. Major arteriovenous malformations may lead to congestive heart failure, encroach upon vital structures, lead to hemmorrhage, or cause a consumption coagulopathy (the Hasbach-Merritt syndrome). When such problems do occur, the diagnosis has usually been known for some time and visualized by vascular imaging techniques. Less obvious presentations may result from perineural hemangiomas, which, although symptomatic, are not lifethreatening. During the past 20 years, we have seen four patients with hemangiomas in perineural locations in the upper extremity. In all four patients the diagnosis was not suspected at the time of the initial surgery. Case reports Case 1. A 2 1-year-old college student with an established diagnosis of neurofibromatosis had been followed up for more than 3 years because of vague pain in her right forearm. The pain was initially episodic in nature, but it had become almost constant and had radiated in the median nerve distribution. After a mass was felt in the proximal third of the forearm,
From the Section of Orthopaedic Surgery. University of Michigan, Ann Arbor, Mich. Received for publication March 26. 1991: accepted in revised form Aug. 23. 1991. No benefits in any form have &en received or will be received from a commercial party related directly or indirectly to the subject of this article. Reprint requests: Dean S. Louis, MD. Section of Orthopedics. versity of Michigan, Ann Arbor, MI 48109. 3/I/33720
308
THE JOURNAL
OF HAND SURGERY
Uni-
involvement,
the
(J HAND SURG1992;17A:308-11.)
MD, Ann Arbor, Mich.
an exploratory procedure was performed. The median nerve was seen to have a vascular malformation within the epineural sheath, but this was extrafascicular in location (Fig. 1). With the use of magnification, the observed malformation was removed from the nerve without difficulty. At follow-up 6 months postoperatively, the symptoms were no longer present. The long period of evaluation before the mass came evident indicates the difficulty in making this specific diagnosis.
Case 2. A lo-year-old girl was seen because of a painful mass in the posterior part of the arm. A previous biopsy had resulted in extensive bleeding, and the patient was referred for our evaluation. There was a firmness in the posterior medial part of the arm in the distal third. Palpation resulted in discomfort in the ulnar nerve distribution. At operation an extensive hemangioma was visualized extending for a distance of about 15 cm both above and below the medial epicondyle (Fig. 2). Its location was intraepineural and extrafascicular in the ulnar nerve. All visible abnormal tissue was removed. Twelve years after this resection the patient is free of symptoms. A preoperative angiogram was not performed. Although it might have been helpful in making the diagnosis preoperatively, the outcome was not influenced by the absence of the study. Case 3. A 29-year-old secretary was seen because of a recurrent mass between the webs of the long and ring fingers of her right hand. She had undergone excision of a painful mass in the same area on three separate occasions. Pathologic findings indicated a hemangioma. An arteriogram revealed a vascular malformation in the area of the web space of the long and ring fingers. Exploration revealed a vascular web that encircled the long finger metacarpal and both digital nerves. All visible abnormal tissue was removed with the aid of magnification. There was extensive residual scarring from the previous operations. Postoperatively the patient experienced persistent tingling and numbness in the long finger. which created difficulty for her when she used a typewriter.
Vol. 17A. No. 2 March 1992
Perineurul
hemangiomas
oj. upper extremic
Fig. 1. Intraepineural hemangioma present in the median nerve just at the point where it emerges from beneath the two heads of the flexor digitorum superticialis. With the use of magnification. the tumor was completely excised, and complete relief of all symptoms was achieved.
Fig. 2. Another example of a more diffuse type of intraepineural, This was completely excised with the use of magnification.
but extrafascicular,
hemangioma.
309
310
Louis and Fortin
The Journal of HAND SURGERY
Fig. 3. An example of a combined extraneural hemangioma (open large arrow) and an additional diffuse intrafascicular, intraneural hemangioma (double-ended arrow). The hemangioma here involves the ulnar nerve and its dorsal sensory branch distally. Both of these are seen under the tip of the hemostat. The lesion can be seen to extend in both directions.
Fig. 4. Histologic specimen from case 4. The multiple vascular spaces are seen in the midst of a homogeneous and uniform stroma. This is representative of the lesions presented in this small series. (Original magnification X 25 .)
Vol. 17A, No. 2 March 1992
Case 4. A healthy 13-year-old girl was seen because of a painful mass at the ulnar border of her forearm distally. Exploration revealed an extensive mass that involved the ulnar nerve and appeared to be composed of blood vessels. The patient was referred to a hand surgeon. who explored the area 6 months later but found that there was little separation between the fascicles and the vascular malformation. Because of the fear of nerve injury only a limited biopsy was performed (Fig. 3). Histologic study revealed multiple vascular spaces within a homogeneous stroma (Fig. 4). Nine years later there has been no progression or recurrence of the mass. Function has not changed. Discussion Vascular malformations are rare and perplexing anomalies that to a large extent frustrate surgeons’ efforts to restore normal local function. These anomalies may occur throughout the body.‘, ’ Upper extremity involvement has been the subject of a few reports3-’ Eight reported cases have involved nerves of the upper extremity.3-8 Involvement of the nerves has been of three types. Type I is an intraneural extrafascicular malformation that is relatively easily removed with magnification. Type II is an intrafascicular encompassing type that is deemed unresectable because of the potential for loss of nerve function secondary to the required dissection. Type III has both intraneural and extraneural components. Losl? reported a case of massive involvement of the ulnar nerve in which the entire mass and the adjacent nerve were resected. He reviewed the literature up to the time of his report and included one other case of Satoh that involved the median nerve in the forearm in a 64-year-old man with a 1Cyear follow-up but without further details. Since that time, Kojima et al..3 Peled et al. ,’ Kon and Vuursteen4 Pate1 et al. .’ and Prosser and Burke” have contributed six additional cases. All of these hemangiomas occurred in female patients between 4 and 19 years of age. Five of these had median nerve involvement, and one involved a digital nerve.J Treatment varied from excision and nerve grafting’ ’ to limited local resection to preserve existing function. In the case reported by Prosser and Burke’ there was an associated Raynaud’s phenomenon. Limited resections were done on four occasions over a 25-year period. The patient was reported to have maintained function at the time of the 25-year follow-up. A compressive neurop-
Peritleurul hemangiomus
gf upper extremity
311
athy may occur, as in the ulnar neuropathy reported by Losl? and the carpal tunnel compression reported by Kojima et al.’ Hemangiomas that involve nerves may cause symptoms of pain. paresthesia and numbness. and accompanying signs of weakness and atrophy may also be evident. The extent to which these symptoms and signs are present will vary, depending on the size and location of the hemangiomas. Nerves compressed by hemangiomas that are external to the nerve will respond well to removal of the adjacent hemangioma.’ Cases in which the hemangioma is intraneural but essentially extrafascicular tend to do well with local excision alone, as in our cases 1 and 2. Cases in which there is extensive involvement of the hemangioma with the fascicular structure of the nerve are the most difficult ones to manage. In such situations limited local excision may lead to recurrence. as in our cases 3 and 4. Total excision with nerve grafting may eliminate the hemangioma. but it will certainly cause other deficits. Our four cases. like seven of the previously reported eight cases in the upper extremity. involved female patients. REFERENCES I. Weber TR, Connors RH, Tracy TF Jr, Bailey PV. Complex hemangiomas of infants and children. Arch Surg 1990:125:1017-21. 2. Griffin JM. Vasconez LO. Schatten WE. Congenital arteriovenous malformations of the upper extremity. Plast Reconstr Surg 1978;62:49-57. 3. Kojima T, lde Y. Marumo E, lshikawa E. Yamasha H. Haemangioma of median nerve causing carpal tunnel syndrome. Hand 1976;8:62-5. 4. Kon M. Vuursteen PJ. An intraneural hemangioma of a digital nerve: case report. J HAND SURG 1981;6:357-8. 5. Losli EJ. Intrinsic hemangiomas of the peripheral nerves: a report of two cases and a review of the literature. Arch Path01 1952;53:226-32. 6. Sato S. uber das cavern&e Angiom des periphenschen nervensystem. Arch Klin Chir 19 I3;100:552. Pate1 CB. Tsai T. Kleinert HE. Hemangioma of the median nerve: a report of two cases. J HANI) SURG 1986;l IA: 76-9. Peled 1, Iosipovich Z, Rousso M, Wexler MR. Hemangioma of the median nerve. J HAND SURG 1980;5:363-5. Prosser AJ. Burke FD. Haemangioma of the median nerve associated with Raynaud’s phenomenon. J HAND SURG 12B:227-8.
