Intravenous lobular capillary hemangioma originating in the iliac veins: A case report

Intravenous lobular capillary hemangioma originating in the iliac veins: A case report

Intravenous lobular capillary hemangioma originating in the iliac veins: A case report Sanjeev Pradhan, MD,a Hernan Bazan, MD,b Ronald Salem, MD,a and...

432KB Sizes 0 Downloads 55 Views

Intravenous lobular capillary hemangioma originating in the iliac veins: A case report Sanjeev Pradhan, MD,a Hernan Bazan, MD,b Ronald Salem, MD,a and Richard J. Gusberg, MD,a New Haven, Conn; and New Orleans, La Intravenous lobular capillary hemangioma is a rare benign lesion that can mimic other intravascular lesions encountered by vascular surgeons, such as angiosarcoma, papillary endothelial hyperplasia, and deep vein thrombus. These lesions have been described originating in the veins of the head, neck, and upper extremities. To our knowledge, there are no reported cases of intravenous capillary hemangioma arising from within a pelvic vein. We report a case of an elderly woman found to have an extensive intravenous lobular capillary hemangioma originating in the internal iliac vein. She was successfully treated with resection, including the tumor and normal iliac vein. Diagnosis of these lesions can be difficult. We describe the utility of duplex ultrasound and magnetic resonance venography in aiding preoperative diagnosis and surgical planning. The diagnosis is ultimately confirmed with histology that demonstrates multiple capillaries lined with flattened endothelial cells grouped in a lobular fashion admixed with fibromyxoid stroma containing collagenous fibers, spindle cells, and mitotic figures. Preoperative work-up should include a duplex ultrasound and magnetic resonance venogram. Treatment should be with resection and specimen processing to rule-out malignant vascular tumors. ( J Vasc Surg 2008; 47:1346-9.)

Intravenous lobular capillary hemangioma, also known as pyogenic granuloma, is a variant of lobular capillary hemangioma. These rare, benign lesions consist of capillary lobules admixed within a fibromyxoid stroma and are grossly polypoid, rubbery masses that extend from a fibrovascular stalk within the lumen of a vein.1-4 As the alternate term suggests, these lesions were once thought to represent a reactive proliferation of inflammatory cells due to trauma or infection. Cytologic examination, however, usually does not reveal the presence of inflammatory cells, thus favoring a neoplastic etiologic process.2,5 Intravascular capillary hemangiomas were first described in 1979, and the most recent review found ⬍30 cases that have been reported in the English literature.1,6 These lesions are commonly thought to arise in upper extremity or neck veins of young women.1,4,7 Although benign, the presence of such a lesion requires differentiation from other intravascular lesions such as angiosarcoma, papillary endothelial hyperplasia, and thrombus.1,6-8 We report a case of an elderly woman, previously treated for an iliofemoral deep venous thrombosis (DVT) and recurrence, found to have an extensive intravenous lobular capillary hemangioma originating in the internal iliac vein. To our knowledge, this is the first reported case of intravenous lobular capillary hemangioma arising in a pelvic vein. From the Department of Surgery, Sections of Vascular Surgery and Surgical Oncology, Yale University Medical School and Yale-New Haven Hospital, New Havena; and Department of Surgery, Section of Vascular Surgery, Louisiana State University Health Sciences Center, New Orleans.b Competition of interest: none. Reprint requests: Sanjeev Pradhan, MD, Yale University School of Medicine, Vascular Surgery, FMB 137, PO Box 208602, New Haven, CT 06520-8062 (e-mail: [email protected]). 0741-5214/$34.00 Copyright © 2008 by The Society for Vascular Surgery. doi:10.1016/j.jvs.2008.01.049

1346

CASE REPORT A nonsmoking, hypertensive, but otherwise healthy 75-yearold woman presented to the vascular center at Yale–New Haven Hospital with the diagnosis of a vascular tumor in the right iliac vein. A presumed right iliac deep venous thrombosis, diagnosed 3 years previously, had been treated with Coumadin (Bristol-Myers Squibb, Princeton, NJ). The result of a hypercoagulable work-up was negative at that time. A subsequent computed tomography (CT) scan, done because of abdominal pain and diarrhea, revealed an enhancing intraluminal tumor in the right internal iliac vein (Fig 1, A). A magnetic resonance venogram confirmed the presence of an incompletely obstructing 2.4- ⫻ 1.8-cm mass within the right internal iliac vein extending proximally to the distal inferior vena cava (Fig 1, B). An arteriogram demonstrated neovascularization of the intravenous mass (Fig 2). The patient denied leg pain, leg swelling, and weight loss. Her surgical history included a total abdominal hysterectomy and bilateral salpingo-oophorectomy for benign ovarian masses. The result of her physical examination was unremarkable, with no lymphadenopathy or abdominal masses, normal femoral and distal pulses, and a nonswollen and nontender right lower extremity. A chest radiograph and bone scan showed no metastatic disease. Results of assays for tumor markers were negative. Before operation, an inferior vena cava (IVC) filter was placed to protect against tumor or thrombus embolization during dissection and manipulation of the iliac vein. In the operating room, the right iliac system was approached from a retroperitoneal approach. After venous tributaries of the right internal iliac vein were ligated to provide adequate mobilization, control of the IVC, right external, and internal iliac veins was achieved. The presence of a mass within the internal iliac vein and extending into the IVC was confirmed by intraoperative ultrasound imaging. A venotomy was made on the internal iliac vein revealing the attachment site just distal to the iliac bifurcation. The involved segment of the internal iliac vein was resected, and the tumor was extracted from the common iliac vein and IVC.

JOURNAL OF VASCULAR SURGERY Volume 47, Number 6

Pradhan et al 1347

Fig 1. A, A computed tomography scan with intravenous and oral contrast demonstrates a heterogeneously enhancing lesion within the internal iliac vein (black arrow). B, A T2-weighted magnetic resonance venography image demonstrates an internal iliac mass isointense to the vein (white arrow).

Fig 2. Panels a-c, Arteriograms demonstrate opacification of the right internal iliac artery with filling of the internal iliac vein and, subsequently, the tumor within in a delayed fashion. Panel d, This panel demonstrates sustained enhancement by the tumor after contrast has washed out from the vein. Pathology of the tumor revealed an intravascular capillary hemangioma. The patient was seen in follow-up 2 months later and demonstrated no evidence of leg swelling or wound complications. No further imaging was completed.

DISCUSSION Cooper et al1 first described “intravenous pyogenic granulomas” found in the upper extremity and neck veins of 18 patients in 1979. Since then, ⬍10 additional cases have been reported in the English literature.3,4,6-9 Most patients present in their mid-to-late 30s and there is a slight female predilection.1 Most patients are asymptomatic, but those that are symptomatic present with extremity edema or a painless mass.1,6,10 There are no known associated risk factors; however, some reports suggest these lesions are linked with arteriovenous malformations.11-13

The differential diagnosis characteristically focuses on vascular related pathologies, including deep venous thrombosis, aneurysm, ganglion or other neural lesion, vascular tumor, and papillary endothelial hyperplasia.1,4,9 With no characteristic clinical features, the diagnosis of intravenous lobular capillary hemangioma relies on imaging studies. On duplex ultrasound (DUS) imaging, intravenous lobular capillary hemangiomas appear as smoothly contoured, internally nodular lesions confined within the vessel wall. These lesions demonstrate small areas of hypoechogenicity within a mostly echogenic background suggesting hypervascularity. Ultrasound can therefore be used to rule out aneurysms and localize the mass to within the vessel wall, ruling out neural lesions or extrinsic compressive lesions. On DUS, in contrast to intravenous lobular capillary hemangiomas, DVT is typically found within a dis-

JOURNAL OF VASCULAR SURGERY June 2008

1348 Pradhan et al

Fig 3. A, Low-power magnification (x10; hematoxylin and eosin stain) of the tumor shows that the capillaries are arranged in a lobular manner surrounded by fibromyxoid stroma. B, Under high-power magnification (x30; hematoxylin and eosin stain), the flattened endothelial cells of the capillaries can be seen. Of note, there is a paucity of inflammatory cells and pleomorphic cells.

tended, noncompressible vein containing an echogenic thrombus. Recanalized DVT appears as concentric low echogenic signal thrombi demonstrating turbulent peripheral flow, not intralesion flow, on DUS.4,9 Magnetic resonance imaging, specifically, a magnetic resonance venogram, can also be used to diagnose these lesions. On T1-weighted images, the lesion will be isointense to muscles and isointense to veins on T2-weighted images. Gadolinium contrast will result in heterogeneous enhancement of the intravenous lesion with small areas of low-signal intensity found within the lesion suggesting hypervascularity.9 In contrast, DVT is diagnosed as a filling defect within a vessel and hypointensity to muscle on T2-weighted imaging.14 Our patient underwent an arteriogram to better delineate the tumor and associated arterial anatomy. The arteriogram will also demonstrate neovascularization when present. Although an arteriogram is not essential to making the diagnosis, it can aid in preoperative planning. Radiologic imaging can help define the lesion, but histologic examination is necessary to make the diagnosis of lobular capillary hemangioma and rule out a malignancy. On gross examination, most lesions appear as a soft, whitishgray, polypoid mass extending from the vein wall by a fibrovascular stalk. There is a characteristic perivenous artery, contained within the stalk, that serves as the feeding vessel.1,3,10 Usually, no thrombus, necrosis, or hemorrhage is noted.1,6 Under low-power magnification, these lesions are noted to consist of multiple capillaries lined with flattened endothelial cells grouped in a lobular fashion (Fig 3, A). These lobules are separated by an edematous fibromyxoid stroma containing collagenous fibers, spindle cells, and mitotic figures.1,13 Interestingly, the luminal surface of the tumor will also have an endothelial lining.1 Unlike the implication of the former term, pyogenic granuloma, no inflammatory cells are present within the mass (Fig 3, B).6 In the past, recanalized DVT, which can be associated with infiltrating leukocytes, may have often been misdiagnosed as pyogenic granulomas. However, lobular capillary hemangiomas clearly have a distinct, noninfectious, noninflammatory appearance that should not be mistaken for DVT.

Histology can aid in differentiating an intravenous lobular capillary hemangioma from malignant intravascular tumors. Angiosarcomas, which often appear as pale, graywhite solid, fleshy tumors, are macroscopically indistinguishable from lobular capillary hemangiomas. On histologic examination, however, angiosarcomas are typically not lobular and display nuclear hyperchromasia, pleomorphism, and a high rate of mitoses. Frozen section is not generally useful because these tumors exhibit a spectrum of architecture from well differentiated to anaplastic. Staining for CD-31, CD-34, and von Willebrand factor is helpful in confirming the diagnosis.15 Hemangioendotheliomas, another vascular tumor, demonstrate an intermediate histology. Microscopically, these tumors exhibit either plump, cuboidal cells or more flattened, epithelioid cells.15 Another rare intravascular tumor that can be found in the venous system is leiomyosarcoma. These lesions represent a highly aggressive form of intravenous leiomyoma and portend a poor prognosis. Histology of these lesions demonstrates nuclear pleomorphism, high rates of mitoses, prominent necrosis. Immunohistochemistry will confirm the diagnosis of leiomyosarcoma.16 Benign tumors such as papillary endothelial hyperplasia can be distinguished from intravenous lobular capillary hemangioma by its papillary architecture, presence of hemosiderin deposits, and association with organized thrombi.3,9-11 CONCLUSION Intravenous lobular capillary hemangioma is a rare benign lesion, usually found in the veins of the upper extremity and neck.1,3,6,7 To our knowledge, our patient represents the first reported case of a lobular capillary hemangioma originating in an iliac vein. Controversy exists about the underlying pathogenesis of such lesions, but histologic examination demonstrates that these lesions are not of an inflammatory or infectious nature, as previously thought. With no characteristic clinical findings associated with these lesions, diagnosis can be difficult. Although the initial presentation can mimic a deep venous thrombosis, imaging studies suggest neoplasm. If the diagnosis of intravascular neoplasm is entertained based on DUS or CT

JOURNAL OF VASCULAR SURGERY Volume 47, Number 6

imaging, we recommend obtaining a magnetic resonance venogram, which is helpful in making the diagnosis and surgical planning. Careful review of existing imaging can aid in identifying primary tumors that may present with vascular infiltration. All grossly palpable tumors should be removed with the segment of involved vessel, along with 2 to 3 cm of normal-appearing tissue. Definitive diagnosis requires histologic examination of the excised lesion with a portion of the adherent vein. Special stains should be done to rule out malignancy. If the pathology reveals lobular capillary hemangioma, the prognosis is excellent. Although growth rates are not known, these tumors can continue to grow and recur if not completely resected; however, with complete resection, there is little risk of recurrence. REFERENCES 1. Cooper PH, McAllister HA, Helwig EB. Intravenous pyogenic granuloma. Am J Surg Pathol 1979;3:221-8. 2. Mills SE, Cooper PH, Fechner RE. Lobular capillary haemangioma: the underlying lesion of pyogenic granuloma. A study of 73 cases from the oral and nasal mucous membranes. Am J Surg Pathol 1980;4:470-9. 3. DiFazio F, Morgan J. Intravenous pyogenic granuloma of the hand. J Hand Surg 1989;14:310-2. 4. Maddison A, Tew K, Orell S. Intravenous lobular capillary haemangioma: ultrasound and histology findings. Australas Radiol 2006;50: 186-8. 5. Nichols GE, Gaffey MJ, Mills SE, Weiss LM. Lobular capillary haemangioma: an immunohistochemical study including steroid hormone receptor status. Am J Clin Pathol 1992;97:770-5.

Pradhan et al 1349

6. Qian LH, Hui YZ. Intravenous pyogenic granuloma: immunohistochemical consideration—a case report. Vasc Surg 2001;35:315-9. 7. Saad RW, Sau P, Mulvaney MP, James WD. Intravenous pyogenic granuloma. Int J Dermatol 1993;32:130-2. 8. Ulbright TM, Santa-Cruz DJ. Intravenous pyogenic granuloma. Cancer 1983;45:1646-52. 9. Ghekiere O, Galant C, Vande Berg B. Intravenous pyogenic granuloma or intravenous lobular capillary hemangioma. Skeletal Radiol 2005;34: 343-6. 10. Song MG, Kim HJ, Lee ES. Intravenous pyogenic granuloma. Int J Dermatol 2001;40:57-9. 11. Rusin LJ, Harrell ER. Arteriovenous fistula: cutaneous manifestations. Arch Dermatol 1976;112:1135-8. 12. Kim TH, Choi EH, Ahn SK, Lee SH. Vascular tumors arising in port-wine stains: two cases of pyogenic granuloma and a case of acquired tufted angioma. J Dermatol 1999;26:813-6. 13. Hung CH, Kuo HW, Chiu YK, Huang PH. Intravascular pyogenic granuloma arising in an acquired arteriovenous malformation: report of a case and review of the literature. Dermatol Surg 2004;30:1050-3. 14. Moody AR, Pollock JG, O’Connor AR, Bagnall M. Lower-limb deep venous thrombosis: direct MR imaging of the thrombus. Radiology 1998;209:349-55. 15. Schoen FJ. Diseases of organ systems: blood vessels. In Kumar V, Abbas AK, Fausto N, editors. Robbins and Cotran pathologic basis for disease. Philadelphia, PA: Elsevier Saunders; 2005. p. 445-51. 16. McDonald DK, Kalva SP, Fan C, Vasilyev A. Leiomyosarcoma of the uterus with intravascular tumor extension and pulmonary tumor embolism. Cardiovasc Intervent Radiol 2007;30:140-2.

Submitted Oct 22, 2007; accepted Jan 25, 2008.