- Email: [email protected]
Vascular tumors of the abdominal wall
The American Journal of Surgery 187 (2004) 553–556
Scientific paper
Vascular tumors of the abdominal wall Stephanie R. Goldberg, M.D.a, Robert A. Halvorsen, M.D.b, James P. Neifeld, M.D.a,* a
Division of Surgical Oncology, Department of Surgery, Medical College of Virginia Hospitals, Virginia Commonwealth University, Richmond, VA, USA b Department of Radiology, Medical College of Virginia Hospitals, Virginia Commonwealth University, Richmond, VA, USA Manuscript received May 19, 2003; revised manuscript September 13, 2003
Abstract Background: Vascular tumors of the abdominal wall are rare, benign congenital malformations that may cause pain or cosmetic disfigurement and are of significance for their ability to mimic more clinically aggressive tumors. There have been no previous reports of vascular tumors of the abdominal wall. This investigation reports the clinical presentation, diagnosis, and treatment of three patients with vascular tumors of the abdominal wall. Methods: Magnetic resonance angiography (MRA) was used to identify feeding and draining vessels and to aid in operative planning for two patients; in a third patient, standard magnetic resonance imaging revealed the feeding vessels. Results: MRA accurately identified the feeding and draining vessels and aided in operative planning, thus facilitating complete tumor resection. To date, patients have not experienced tumor recurrence. Conclusions: These cases suggest that MRA can be employed as a noninvasive imaging technique and should be the standard preoperative modality to plan the operative approach to vascular tumors of the abdominal wall. © 2004 Excerpta Medica, Inc. All rights reserved. Keywords: Abdominal wall; Hemangioma; Magnetic resonance angiography; Vascular tumors
Vascular tumors are benign congenital malformations that may present at birth but often do not become clinically significant until adulthood. Although rare in occurrence, vascular tumors are of interest clinically because of their ability to mimic aggressive tumors, and they may cause pain and cosmetic disfigurement [1]. Intramuscular vascular tumors of the extremities have been reported, but vascular tumors of the abdominal wall have not been described [2–5]. Hemangiomas, the most common tumor of infancy, occur often in the head and neck region, although 25% are found in the trunk. Patients presenting with multiple cutaneous hemangiomas are at increased risk for concurrent hemangiomas of the liver, lungs, and gastrointestinal tract. Hemangiomas have also been noted to develop in the lymph nodes, spleen, thymus, urinary bladder, gallbladder, pancreas, adrenals, meninges, brain, and spinal cord. Clinical presentation of cutaneous hemangiomas may vary considerably. Color can range from a vivid crimson to bright
* Corresponding author. Tel.: ⫹1-804-828-7875; fax: ⫹1-804-8271016. E-mail address: [email protected]
scarlet to a bluish hue and, depending on the layer of skin in which the tumor resides, may even appear colorless. The color may change over time. Although rare, complications of hemangiomas include ulceration; obstruction of visual, auditory, or respiratory systems; hemorrhage; and highoutput congestive heart failure [6]. Pain can be a symptom of enlarging hemangiomas or those with spontaneous thrombosis. Data are lacking to predict whether intramuscular hemangiomas pose the same level of complication risk as cutaneous hemangiomas. Radiologic techniques have been used to aid in the diagnosis of vascular tumors. Although color ultrasonography is capable of differentiating venous from lymphatic malformations, magnetic resonance imaging (MRI) is the preferred technique to identify tumor extent. The use of computed axial tomography (CAT) has been superceded by the advent of MRI technology [4 – 6]. Hemangiomas rarely require treatment, yet patients may develop symptoms of pain, hemorrhage, or congestive heart failure requiring treatment or may desire treatment for cosmetic reasons. In children, hemangiomas may respond favorably to systemic high-dose corticosteroids or subcutaneous interferon-␣-2a [1,6]. Surgical resection can be an effective treatment in adults.
0002-9610/04/$ – see front matter © 2004 Excerpta Medica, Inc. All rights reserved. doi:10.1016/j.amjsurg.2003.12.042
554
S.R. Goldberg et al. / The American Journal of Surgery 187 (2004) 553–556
The purpose of this report is to describe a group of patients with hemangiomas of the abdominal wall and to describe clinical presentation, diagnostic evaluation, and operative approach.
Clinical material Case No. 1 A 33-year-old woman presented with a 3-year history of an abdominal wall mass that had gradually increased in size. Physical examination revealed a soft, nontender 5.0 ⫻ 7.0 – cm left lower quadrant abdominal wall mass with no visible skin discoloration. Fine-needle aspiration biopsy showed blood, rare spindle cells, and skeletal muscle. CAT scan findings sowed a 5.1 ⫻ 2.9 ⫻ 5.0 – cm lowerleft quadrant mass, densely enhancing, located between the oblique muscles of the abdominal wall. No internal calcifications were noted. No extension into abdominal musculature or into the peritoneal cavity was noted. Feeding vessels were suggested to be arising from the left iliac artery, but the precise origin and course was unclear. The density of the mass after contrast administration was equal to that of the aorta, which suggested a large amount of central vascular space. Thus, CAT scan findings suggested an extremely vascular abdominal wall tumor with features that could not determine whether it was a benign or malignant lesion. MRI and magnetic resonance angiography (MRA) were performed to better define the extent of the mass and to identify any feeding or draining vessels (Fig. 1A). MRI findings showed a large, oval-shaped enhancing mass in the left lower quadrant of the abdominal wall, probably arising from the internal oblique muscle. It measured 3.8 cm anterior–posterior ⫻ 5.8 cm in width ⫻ 6.0 cm in height and was in direct contact with the anterior surface of the transversalis muscle. MRA findings showed a large feeding artery arising from the left femoral artery, extending superiorly along the left pelvic sidewall, and branching below the tumor with one major branch extending along the medial surface entering at the mid-medial portion of the mass (Fig. 1B). A second branch artery was noted to enter at the caudal end of the mass. The left inferior epigastric vein was noted to be larger than the right, but no definite communication between it and the mass was clearly identified. Using the MRA findings to plan an operative approach and to aid in identifying the location and course of the feeding and draining vessels, a lower-left quadrant incision was made. The peritoneal cavity was entered to obtain a deep margin. At the inferior aspect of the mass, in close proximity to the iliac crest, 2 small vessels consistent with the feeding vessels described preoperatively on MRA were identified and ligated. The fascia was able to be closed primarily. The gross specimen measured 5.0 ⫻ 4.0 ⫻ 4.0 cm and consisted of a pink to whitish-tan multilobulated, encapsu-
Fig. 1. (A) T1-weighted axial MRI findings after intravenous contrast show a large oval-shaped mass (arrow) in the left lateral abdominal wall musculature. Note the large draining vein (open arrow). (B) Coronal MRA findings show a mass (arrow) and 2 arterial branches (open arrow heads) supplying the mass. Axial T1-weighted postcontrast MRI findings show multiple enlarged vessels within the mass (arrow) that have replaced and expanded the left rectus muscle. MRA ⫽ magnetic resonance angiography; MRI ⫽ magnetic resonance imaging.
lated mass within the surrounding muscular tissue. No necrosis was present. Microscopic evaluation was significant for a lobular proliferation of small vessels and occasional larger vessels with a distinct elastin layer highlighted by an elastic stain. Final pathology reflected a vascular tumor with features of juvenile hemangioma. The patient remains free of recurrence and asymptomatic 8 months after resection. Case No. 2 A 40-year-old woman with a history of 13 operations for recurrent cavernous hemangiomas on her left abdomen, hip, and upper thigh was referred for evaluation. Outside pathol-
S.R. Goldberg et al. / The American Journal of Surgery 187 (2004) 553–556
555
ogy specimens from previous operations were obtained and were consistent with cavernous hemangioma. The patient had been free of recurrence for 5 years until she noticed swelling and asymmetry of the left chest wall, left abdominal wall, left thigh, and left hip compared with the right. MRI findings of the abdomen and pelvis showed enlargement of the left rectus abdominous muscle secondary to fatty infiltration and multiple tortuous vessels. Abnormal vessels extended anteriorly to the muscle into the subcutaneous fat (Fig. 2A). Preoperative MRA findings were utilized as a tool to guide operative planning. MRA showed draining veins that extended from the lesion posteriorly into the left external iliac vein and a fusiform dilated left common iliac vein (Fig. 2B). The entire left abdominal wall, including the peritoneum, was removed. The two large feeding vessels were identified and divided, and an enlarged vein, previously seen on MRA and draining into the left external iliac vein, was also identified and divided. The abdominal wall was reconstructed with mesh. The gross specimen measured 19.0 ⫻ 12.0 ⫻ 6.0 cm and consisted of tannish-yellow lobulated, fibrofatty tissue surfaced by a 15.0 ⫻ 10.0 – cm tannish-gray to tannish-purple smooth, dusky, thick membrane. The cut surface revealed reddish-brown striated muscle with an abundant amount of attached tannish-yellow lobulated adipose tissue and multiple dilated blood vessels. Final pathology was diagnostic of cavernous hemangioma. The swelling of the left hip and leg resolved after surgery, and no recurrence has been detected 2 years after surgery. Case No. 3 A 37-year-old woman with a history of right-sided abdominal discomfort for 2 years and a notable abdominal bulge was referred for evaluation. Physical examination showed a soft right lateral abdominal wall mass that was slightly tender to palpation. CAT findings showed a predominately fat density mass that contained multiple abnormal vessels displacing the right external oblique muscle anteriorly. MRI findings showed an elongated elliptical mass measuring 1.6 ⫻ 5.0 ⫻ 10.0 cm between the internal and external oblique muscles with extension into the external oblique. Multiple serpiginous vessels were noted. Feeding vessels arose in the right rectus abdominous muscle, and draining veins were seen extending into the right transversalis muscle. The abdominal wall mass was excised through a rightsided transverse incision at the level of the umbilicus. The abdominal wall was resected from the anterior superior iliac spine to the level of the eleventh rib. The feeding and draining vessels seen on MRI were individually ligated. The peritoneal cavity was not entered. A double layer of Marlex (Davol, Cranston, Rhode Island) mesh was used to close the abdominal wall defect.
Fig. 2. (A) Axial-T2 weighted MRI findings demonstrate high signal within a mass (arrow) and multiple adjacent vessels (open arrows) representing feeding arteries. (B) Obliquely oriented 3-dimensional MRA findings show an oval-shaped mass (arrow), large draining vessels (open arrow), and a fusiform aneurysm of the left external iliac vein. MRA ⫽ magnetic resonance angiography; MRI ⫽ magnetic resonance imaging.
The gross specimen measured 8.7 ⫻ 7.5 ⫻ 5.8 cm and consisted of skeletal muscle, adipose tissue, fascia, and soft tissue. Multiple serial sections revealed reddish-tan skeletal muscle and adipose tissue with scattered areas of petechial hemorrhage. No gross lesion or mass was identifiable. Final pathology was a cavernous hemangioma. The patient remains asymptomatic without evidence of recurrence 1 year after surgery.
556
S.R. Goldberg et al. / The American Journal of Surgery 187 (2004) 553–556
Comments Vascular tumors are rare, benign congenital malformations that are often asymptomatic, although they may present clinically as enlarging painful masses. Although the risk of hemorrhagic complications is small, vascular tumors require an accurate diagnosis be made because of their ability to clinically mimic aggressive tumors such as sarcomas [1]. Diagnostic approaches to vascular tumors include the use of color Doppler imaging [3], MRI [4,5], and angiography [7]. CAT has not been demonstrated to be of diagnostic value and is not capable of distinguishing between benign and malignant vascular tumors. Hemangiomas are characterized microscopically as consisting of rapidly dividing endothelial cells organized as compact sinusoids and capillary-sized channels. After going through a proliferating phase characterized by neoangiogenesis, the tumors become lobular in configuration with fibrous septae containing large-caliber feeding and draining vessels. Hemangiomas subsequently undergo an involuting phase that is marked histologically by areas of hyperplasia and regression, ultimately leading to the deposition of interlobular and intralobular fibrous and fatty tissue, thus lending a cavernous appearance [6]. The rarity of vascular tumors, combined with the lack of literature and confusing classification scheme, has contributed to disappointing surgical outcomes and a high incidence of recurrence or worsening of symptoms [3]. Vascular tumors are challenging to the surgeon in that they have a tendency to recur if they are not completely resected and their feeding and draining vessels are not identified and divided. Attempts to identify feeding vessels in vascular tumors of the extremities formerly consisted of angiography, but more recently MRI and magnetic resonance venography have been used for preoperative planning [4,6,8]. The traditional use of arteriography for venous malformations is limited by an inability to completely opacify the tumor when implemented alone and is indicated only with concurrent therapeutic embolization or sclerosis. Both closed-system and direct-puncture venography are superior to arteriography in identifying abnormal postcapillary vascular spaces [3]. Imaging of vascular tumors is aimed at defining the vascular characteristics and extent of the tumor; MRA is capable of achieving both goals. The noninvasive nature and ability of MRA to define the extent of the lesion argue against the traditional use of arteriography and venography. Vascular tumors of the abdominal wall have not been previously reported. The 3 cases discussed in this report were diagnosed by MRI, and the extent of each mass was outlined by the scan. MRA was used as a preoperative,
noninvasive procedure to demonstrate the feeding and draining vessels. These tumors are soft and ill defined on clinical examination; therefore, a preoperative test is useful and necessary to try to define the extent of the mass and to identify the feeding and draining vessels to aid in the development of an operative plan. To date, there is a paucity of literature that has suggested the use of MRA as a tool to identify the feeding vessels, thus aiding in preoperative planning. In the first 2 cases presented, the quantity and course of the feeding vessels were accurately identified using MRA, thereby enabling wide tumor excision with ligation and division of the arterial and venous drainage. MRI alone did not provide adequate identification of the vessels in the first 2 cases, but it proved sufficient information to identify feeding and draining vessels in the third patient, therefore obviating the need for MRA. All three patients remain without recurrence (from 8 to 24 months after resection) including the patient referred after 13 previous operations for recurrent vascular tumors. These cases suggest that MRA should be considered an important component of diagnostic and preoperative planning for patients presenting with vascular tumors. Because of the rarity of vascular tumors in clinical practice, further studies are necessary to adequately assess the true value of MRA. MRA is an easily employed, noninvasive, and costeffective procedure that may minimize tumor recurrences resulting from failure to identify and resect feeding and draining vessels. This approach should contribute to overall improved surgical outcomes and patient satisfaction and minimize the expense of unnecessary radiologic and surgical procedures. References [1] Dohil MA, Baugh WP, Eichenfield LF. Pediatric dermatology: vascular and pigmented birthmarks. Pediatr Clin North Am 2000;47:783– 812. [2] Allen PW, Enzinger FM. Hemangioma of skeletal muscle. An analysis of 89 cases. Cancer 1972;29:8 –22. [3] Yakes WF. Extremity venous malformations: diagnosis and management. Semin Interv Radiol 1994;11:332–9. [4] Laor T, Burrows PE, Hoffer FA. Magnetic resonance venography of congenital vascular malformations of the extremities. Pediatr Radiol 1996;26:371– 80. [5] Laor T, Burrows PE. Congenital anomalies and vascular birthmarks of the lower extremities. MRI Clin North Am 1998;6:497–519. [6] Fishman SJ, Mulliken JB. Hemangiomas and vascular malformations of infancy and childhood. Pediatr Clin North Am 1993;40:1177–1200. [7] Dobson MJ, Hartley RWJ, Ashleigh R, et al. MR angiography and MR imaging of symptomatic vascular malformations. Clin Radiol 1997;52: 595– 602. [8] Gould ES, Potter HG, Huvos A, et al. Case report 671. Skeletal Radiol 1991;20:303–5.
Scientific paper
Vascular tumors of the abdominal wall Stephanie R. Goldberg, M.D.a, Robert A. Halvorsen, M.D.b, James P. Neifeld, M.D.a,* a
Division of Surgical Oncology, Department of Surgery, Medical College of Virginia Hospitals, Virginia Commonwealth University, Richmond, VA, USA b Department of Radiology, Medical College of Virginia Hospitals, Virginia Commonwealth University, Richmond, VA, USA Manuscript received May 19, 2003; revised manuscript September 13, 2003
Abstract Background: Vascular tumors of the abdominal wall are rare, benign congenital malformations that may cause pain or cosmetic disfigurement and are of significance for their ability to mimic more clinically aggressive tumors. There have been no previous reports of vascular tumors of the abdominal wall. This investigation reports the clinical presentation, diagnosis, and treatment of three patients with vascular tumors of the abdominal wall. Methods: Magnetic resonance angiography (MRA) was used to identify feeding and draining vessels and to aid in operative planning for two patients; in a third patient, standard magnetic resonance imaging revealed the feeding vessels. Results: MRA accurately identified the feeding and draining vessels and aided in operative planning, thus facilitating complete tumor resection. To date, patients have not experienced tumor recurrence. Conclusions: These cases suggest that MRA can be employed as a noninvasive imaging technique and should be the standard preoperative modality to plan the operative approach to vascular tumors of the abdominal wall. © 2004 Excerpta Medica, Inc. All rights reserved. Keywords: Abdominal wall; Hemangioma; Magnetic resonance angiography; Vascular tumors
Vascular tumors are benign congenital malformations that may present at birth but often do not become clinically significant until adulthood. Although rare in occurrence, vascular tumors are of interest clinically because of their ability to mimic aggressive tumors, and they may cause pain and cosmetic disfigurement [1]. Intramuscular vascular tumors of the extremities have been reported, but vascular tumors of the abdominal wall have not been described [2–5]. Hemangiomas, the most common tumor of infancy, occur often in the head and neck region, although 25% are found in the trunk. Patients presenting with multiple cutaneous hemangiomas are at increased risk for concurrent hemangiomas of the liver, lungs, and gastrointestinal tract. Hemangiomas have also been noted to develop in the lymph nodes, spleen, thymus, urinary bladder, gallbladder, pancreas, adrenals, meninges, brain, and spinal cord. Clinical presentation of cutaneous hemangiomas may vary considerably. Color can range from a vivid crimson to bright
* Corresponding author. Tel.: ⫹1-804-828-7875; fax: ⫹1-804-8271016. E-mail address: [email protected]
scarlet to a bluish hue and, depending on the layer of skin in which the tumor resides, may even appear colorless. The color may change over time. Although rare, complications of hemangiomas include ulceration; obstruction of visual, auditory, or respiratory systems; hemorrhage; and highoutput congestive heart failure [6]. Pain can be a symptom of enlarging hemangiomas or those with spontaneous thrombosis. Data are lacking to predict whether intramuscular hemangiomas pose the same level of complication risk as cutaneous hemangiomas. Radiologic techniques have been used to aid in the diagnosis of vascular tumors. Although color ultrasonography is capable of differentiating venous from lymphatic malformations, magnetic resonance imaging (MRI) is the preferred technique to identify tumor extent. The use of computed axial tomography (CAT) has been superceded by the advent of MRI technology [4 – 6]. Hemangiomas rarely require treatment, yet patients may develop symptoms of pain, hemorrhage, or congestive heart failure requiring treatment or may desire treatment for cosmetic reasons. In children, hemangiomas may respond favorably to systemic high-dose corticosteroids or subcutaneous interferon-␣-2a [1,6]. Surgical resection can be an effective treatment in adults.
0002-9610/04/$ – see front matter © 2004 Excerpta Medica, Inc. All rights reserved. doi:10.1016/j.amjsurg.2003.12.042
554
S.R. Goldberg et al. / The American Journal of Surgery 187 (2004) 553–556
The purpose of this report is to describe a group of patients with hemangiomas of the abdominal wall and to describe clinical presentation, diagnostic evaluation, and operative approach.
Clinical material Case No. 1 A 33-year-old woman presented with a 3-year history of an abdominal wall mass that had gradually increased in size. Physical examination revealed a soft, nontender 5.0 ⫻ 7.0 – cm left lower quadrant abdominal wall mass with no visible skin discoloration. Fine-needle aspiration biopsy showed blood, rare spindle cells, and skeletal muscle. CAT scan findings sowed a 5.1 ⫻ 2.9 ⫻ 5.0 – cm lowerleft quadrant mass, densely enhancing, located between the oblique muscles of the abdominal wall. No internal calcifications were noted. No extension into abdominal musculature or into the peritoneal cavity was noted. Feeding vessels were suggested to be arising from the left iliac artery, but the precise origin and course was unclear. The density of the mass after contrast administration was equal to that of the aorta, which suggested a large amount of central vascular space. Thus, CAT scan findings suggested an extremely vascular abdominal wall tumor with features that could not determine whether it was a benign or malignant lesion. MRI and magnetic resonance angiography (MRA) were performed to better define the extent of the mass and to identify any feeding or draining vessels (Fig. 1A). MRI findings showed a large, oval-shaped enhancing mass in the left lower quadrant of the abdominal wall, probably arising from the internal oblique muscle. It measured 3.8 cm anterior–posterior ⫻ 5.8 cm in width ⫻ 6.0 cm in height and was in direct contact with the anterior surface of the transversalis muscle. MRA findings showed a large feeding artery arising from the left femoral artery, extending superiorly along the left pelvic sidewall, and branching below the tumor with one major branch extending along the medial surface entering at the mid-medial portion of the mass (Fig. 1B). A second branch artery was noted to enter at the caudal end of the mass. The left inferior epigastric vein was noted to be larger than the right, but no definite communication between it and the mass was clearly identified. Using the MRA findings to plan an operative approach and to aid in identifying the location and course of the feeding and draining vessels, a lower-left quadrant incision was made. The peritoneal cavity was entered to obtain a deep margin. At the inferior aspect of the mass, in close proximity to the iliac crest, 2 small vessels consistent with the feeding vessels described preoperatively on MRA were identified and ligated. The fascia was able to be closed primarily. The gross specimen measured 5.0 ⫻ 4.0 ⫻ 4.0 cm and consisted of a pink to whitish-tan multilobulated, encapsu-
Fig. 1. (A) T1-weighted axial MRI findings after intravenous contrast show a large oval-shaped mass (arrow) in the left lateral abdominal wall musculature. Note the large draining vein (open arrow). (B) Coronal MRA findings show a mass (arrow) and 2 arterial branches (open arrow heads) supplying the mass. Axial T1-weighted postcontrast MRI findings show multiple enlarged vessels within the mass (arrow) that have replaced and expanded the left rectus muscle. MRA ⫽ magnetic resonance angiography; MRI ⫽ magnetic resonance imaging.
lated mass within the surrounding muscular tissue. No necrosis was present. Microscopic evaluation was significant for a lobular proliferation of small vessels and occasional larger vessels with a distinct elastin layer highlighted by an elastic stain. Final pathology reflected a vascular tumor with features of juvenile hemangioma. The patient remains free of recurrence and asymptomatic 8 months after resection. Case No. 2 A 40-year-old woman with a history of 13 operations for recurrent cavernous hemangiomas on her left abdomen, hip, and upper thigh was referred for evaluation. Outside pathol-
S.R. Goldberg et al. / The American Journal of Surgery 187 (2004) 553–556
555
ogy specimens from previous operations were obtained and were consistent with cavernous hemangioma. The patient had been free of recurrence for 5 years until she noticed swelling and asymmetry of the left chest wall, left abdominal wall, left thigh, and left hip compared with the right. MRI findings of the abdomen and pelvis showed enlargement of the left rectus abdominous muscle secondary to fatty infiltration and multiple tortuous vessels. Abnormal vessels extended anteriorly to the muscle into the subcutaneous fat (Fig. 2A). Preoperative MRA findings were utilized as a tool to guide operative planning. MRA showed draining veins that extended from the lesion posteriorly into the left external iliac vein and a fusiform dilated left common iliac vein (Fig. 2B). The entire left abdominal wall, including the peritoneum, was removed. The two large feeding vessels were identified and divided, and an enlarged vein, previously seen on MRA and draining into the left external iliac vein, was also identified and divided. The abdominal wall was reconstructed with mesh. The gross specimen measured 19.0 ⫻ 12.0 ⫻ 6.0 cm and consisted of tannish-yellow lobulated, fibrofatty tissue surfaced by a 15.0 ⫻ 10.0 – cm tannish-gray to tannish-purple smooth, dusky, thick membrane. The cut surface revealed reddish-brown striated muscle with an abundant amount of attached tannish-yellow lobulated adipose tissue and multiple dilated blood vessels. Final pathology was diagnostic of cavernous hemangioma. The swelling of the left hip and leg resolved after surgery, and no recurrence has been detected 2 years after surgery. Case No. 3 A 37-year-old woman with a history of right-sided abdominal discomfort for 2 years and a notable abdominal bulge was referred for evaluation. Physical examination showed a soft right lateral abdominal wall mass that was slightly tender to palpation. CAT findings showed a predominately fat density mass that contained multiple abnormal vessels displacing the right external oblique muscle anteriorly. MRI findings showed an elongated elliptical mass measuring 1.6 ⫻ 5.0 ⫻ 10.0 cm between the internal and external oblique muscles with extension into the external oblique. Multiple serpiginous vessels were noted. Feeding vessels arose in the right rectus abdominous muscle, and draining veins were seen extending into the right transversalis muscle. The abdominal wall mass was excised through a rightsided transverse incision at the level of the umbilicus. The abdominal wall was resected from the anterior superior iliac spine to the level of the eleventh rib. The feeding and draining vessels seen on MRI were individually ligated. The peritoneal cavity was not entered. A double layer of Marlex (Davol, Cranston, Rhode Island) mesh was used to close the abdominal wall defect.
Fig. 2. (A) Axial-T2 weighted MRI findings demonstrate high signal within a mass (arrow) and multiple adjacent vessels (open arrows) representing feeding arteries. (B) Obliquely oriented 3-dimensional MRA findings show an oval-shaped mass (arrow), large draining vessels (open arrow), and a fusiform aneurysm of the left external iliac vein. MRA ⫽ magnetic resonance angiography; MRI ⫽ magnetic resonance imaging.
The gross specimen measured 8.7 ⫻ 7.5 ⫻ 5.8 cm and consisted of skeletal muscle, adipose tissue, fascia, and soft tissue. Multiple serial sections revealed reddish-tan skeletal muscle and adipose tissue with scattered areas of petechial hemorrhage. No gross lesion or mass was identifiable. Final pathology was a cavernous hemangioma. The patient remains asymptomatic without evidence of recurrence 1 year after surgery.
556
S.R. Goldberg et al. / The American Journal of Surgery 187 (2004) 553–556
Comments Vascular tumors are rare, benign congenital malformations that are often asymptomatic, although they may present clinically as enlarging painful masses. Although the risk of hemorrhagic complications is small, vascular tumors require an accurate diagnosis be made because of their ability to clinically mimic aggressive tumors such as sarcomas [1]. Diagnostic approaches to vascular tumors include the use of color Doppler imaging [3], MRI [4,5], and angiography [7]. CAT has not been demonstrated to be of diagnostic value and is not capable of distinguishing between benign and malignant vascular tumors. Hemangiomas are characterized microscopically as consisting of rapidly dividing endothelial cells organized as compact sinusoids and capillary-sized channels. After going through a proliferating phase characterized by neoangiogenesis, the tumors become lobular in configuration with fibrous septae containing large-caliber feeding and draining vessels. Hemangiomas subsequently undergo an involuting phase that is marked histologically by areas of hyperplasia and regression, ultimately leading to the deposition of interlobular and intralobular fibrous and fatty tissue, thus lending a cavernous appearance [6]. The rarity of vascular tumors, combined with the lack of literature and confusing classification scheme, has contributed to disappointing surgical outcomes and a high incidence of recurrence or worsening of symptoms [3]. Vascular tumors are challenging to the surgeon in that they have a tendency to recur if they are not completely resected and their feeding and draining vessels are not identified and divided. Attempts to identify feeding vessels in vascular tumors of the extremities formerly consisted of angiography, but more recently MRI and magnetic resonance venography have been used for preoperative planning [4,6,8]. The traditional use of arteriography for venous malformations is limited by an inability to completely opacify the tumor when implemented alone and is indicated only with concurrent therapeutic embolization or sclerosis. Both closed-system and direct-puncture venography are superior to arteriography in identifying abnormal postcapillary vascular spaces [3]. Imaging of vascular tumors is aimed at defining the vascular characteristics and extent of the tumor; MRA is capable of achieving both goals. The noninvasive nature and ability of MRA to define the extent of the lesion argue against the traditional use of arteriography and venography. Vascular tumors of the abdominal wall have not been previously reported. The 3 cases discussed in this report were diagnosed by MRI, and the extent of each mass was outlined by the scan. MRA was used as a preoperative,
noninvasive procedure to demonstrate the feeding and draining vessels. These tumors are soft and ill defined on clinical examination; therefore, a preoperative test is useful and necessary to try to define the extent of the mass and to identify the feeding and draining vessels to aid in the development of an operative plan. To date, there is a paucity of literature that has suggested the use of MRA as a tool to identify the feeding vessels, thus aiding in preoperative planning. In the first 2 cases presented, the quantity and course of the feeding vessels were accurately identified using MRA, thereby enabling wide tumor excision with ligation and division of the arterial and venous drainage. MRI alone did not provide adequate identification of the vessels in the first 2 cases, but it proved sufficient information to identify feeding and draining vessels in the third patient, therefore obviating the need for MRA. All three patients remain without recurrence (from 8 to 24 months after resection) including the patient referred after 13 previous operations for recurrent vascular tumors. These cases suggest that MRA should be considered an important component of diagnostic and preoperative planning for patients presenting with vascular tumors. Because of the rarity of vascular tumors in clinical practice, further studies are necessary to adequately assess the true value of MRA. MRA is an easily employed, noninvasive, and costeffective procedure that may minimize tumor recurrences resulting from failure to identify and resect feeding and draining vessels. This approach should contribute to overall improved surgical outcomes and patient satisfaction and minimize the expense of unnecessary radiologic and surgical procedures. References [1] Dohil MA, Baugh WP, Eichenfield LF. Pediatric dermatology: vascular and pigmented birthmarks. Pediatr Clin North Am 2000;47:783– 812. [2] Allen PW, Enzinger FM. Hemangioma of skeletal muscle. An analysis of 89 cases. Cancer 1972;29:8 –22. [3] Yakes WF. Extremity venous malformations: diagnosis and management. Semin Interv Radiol 1994;11:332–9. [4] Laor T, Burrows PE, Hoffer FA. Magnetic resonance venography of congenital vascular malformations of the extremities. Pediatr Radiol 1996;26:371– 80. [5] Laor T, Burrows PE. Congenital anomalies and vascular birthmarks of the lower extremities. MRI Clin North Am 1998;6:497–519. [6] Fishman SJ, Mulliken JB. Hemangiomas and vascular malformations of infancy and childhood. Pediatr Clin North Am 1993;40:1177–1200. [7] Dobson MJ, Hartley RWJ, Ashleigh R, et al. MR angiography and MR imaging of symptomatic vascular malformations. Clin Radiol 1997;52: 595– 602. [8] Gould ES, Potter HG, Huvos A, et al. Case report 671. Skeletal Radiol 1991;20:303–5.