Case Report
Intravenous Lobular Capillary Hemangioma Occurring in the Cephalic Vein of a 39-year-old Adult Sung Eun Ahn, MD,1 Ji Seon Park, MD,1 Kyung Nam Ryu, MD,1 Wook Jin, MD,2 So Young Park, MD2 1
Department of Radiology, Kyung Hee University Hospital, South Korea Department of Radiology, Kyung Hee University Hospital at Gangdong, School of Medicine, Kyung Hee University, South Korea
2
Received 18 March 2013; accepted 10 September 2013
ABSTRACT: Intravenous lobular capillary hemangiomas are extremely uncommon and mostly occur in the veins of the neck and upper extremities. Here, we report the clinical and sonographic features of an intravenous lobular capillary hemangioma localized in the right cephalic vein, and we discuss its pathoC logic findings and differential diagnoses. V 2013 Wiley Periodicals, Inc. J Clin Ultrasound 42:375–378, 2014; Published online in Wiley Online Library (wileyonlinelibrary.com). DOI: 10.1002/jcu.22110 Keywords: cephalic vein; intravenous lobular capillary hemangioma; intravenous pyogenic granuloma; ultrasonography
L
obular capillary hemangiomas are relatively common acquired vascular neoplasms, occurring mostly on cutaneous and mucosal surfaces, especially of the oral cavity.1,2 Intravenous lobular capillary hemangiomas (IVLCH) are rare variants of lobular capillary hemangioma. To date, there are only a few case reports of ultrasound findings of IVLCH. We report a histologically proven case of IVLCH localized in the right cephalic vein and discuss the clinical and sonographic features, pathologic findings, and differential diagnoses of IVLCH.
CASE REPORT
A 39-year-old woman presented with a painless soft mass of the dorsoradial aspect of the right Correspondence to: J. S. Park C 2013 Wiley Periodicals, Inc. V
VOL. 42, NO. 6, JULY/AUGUST 2014
distal forearm that had appeared spontaneously 2 months earlier. On physical examination, there was neither tenderness nor skin change. She had no prior history of drug abuse, intravenous catheter insertion, or trauma of the right upper extremity. Sonography was performed using an IU 22 system (Philips Medical Systems, Bothell, WA) with a broadband high-frequency linear transducer (L17–5). On gray-scale sonography, a well-defined nodular intraluminal mass was found dilating the cephalic vein (Figure 1A). The size of the mass was approximately 1.4 3 0.8 3 1.8 cm, and the lesion was hyperechoic with small hypoechoic areas within it. There was no evidence of any extravascular component; the venous wall was preserved, and the surrounding soft tissues maintained their integrity (Figure 1B). Color Doppler imaging showed intense hypervascularization within the lesion (Figure 1C), which adhered to the venous wall and was surrounded by blood flow. The lesion was incompressible. A superficial vein about 10 cm in length, cranially apart from the main mass, showed segmental wall-thickening, suggesting associated phlebitis (Figure 1D). The distal part of this superficial vein was intact. On the basis of these findings, we diagnosed an intravenous benign tumor and decided its surgical removal. The patient underwent surgical excision of a 3-cm-long vascular segment containing the mass. At macroscopic inspection, an intraluminal polypoid well-circumscribed mass with regular contours was found, in continuity with the venous wall. 375
AHN ET AL
FIGURE 1. (A) Longitudinal gray-scale sonogram of the right cephalic vein showing a well-defined intravenous nodular echogenic mass. (B) Axial sonographic scan showing small hypoechogenic areas (arrow) within the lesion. (C) Color Doppler imaging showing hypervascularization within the entire mass. (D) Longitudinal section showing segmental wass thickening (arrows) along a superficial vein, about 10 cm cranially from the mass.
At histologic examination, the lesion was composed of a lobulated angiomatoid proliferation of small blood vessels that were lined by an endothelium and a stroma containing smooth muscle fibers (Figure 2). The histopathological findings led to the final diagnosis of intravenous lobular capillary hemangioma.
DISCUSSION
Lobular capillary hemangioma is a relatively commonly acquired vascular neoplasm, which occurs mainly on cutaneous and mucosal surfaces, where ulceration and suppuration may occur. Lobular capillary hemangiomas were previously thought to occur reactively to trauma or pyogenic infection, hence were formerly known as “pyogenic granulomas.”1,2
IVLCH is a rare variant of lobular capillary hemangioma and occurs mostly in the veins of the neck and upper extremities. It was first described in 1979 and, to date, only a few case reports of ultrasound findings of this lesion have been published.3 The average age of presentation is 38 years (range 15–66 years) and female patients are affected slightly more frequently than male patients. IVLCH do not present with characteristic clinical features ensuring its diagnosis. Ghekiere et al2 noted that, according to the literature, the clinical history and physical findings were similar in all patients presenting with an asymptomatic mass. Our case also presented as a palpable mass without any other symptoms. The sonographic manifestations of IVLCH, as shown in this case, are a well-defined echogenic nodular intraluminal mass, with small
FIGURE 2. (A) Low-power microscope photogram of the lesion showing an intravascular nodule, attached to the venous wall (H&E stain, 312.5). (B) High-power view showing that the tumor was composed of a lobulated angiomatoid proliferation of small blood vessels lined by an endothelium and a stroma containing smooth muscle fibers (H&E stain, 3200).
376
JOURNAL OF CLINICAL ULTRASOUND
INTRAVENOUS LOBULAR CAPILLARY HEMANGIOMA
hypoechogenic internal areas. When compared with histopathological findings, the echogenic nodular intraluminal mass is thought to be lobulating capillaries and the hypoechogenic areas are thought to be edematous fibromyxoid stroma. There was no extravascular extension and the venous wall was preserved. We did not record spectral Doppler signals in our case, but Maddison et al4 demonstrated typically arterial waveforms within the mass, contrasting with the typically venous flow modulation of the vein downstream from the lesion, which allows the diagnosis of intravascular soft tissue neoplasm while excluding a resolving thrombus or an arteriovenous fistula. In our case, the superficial vein located cranially from the mass showed segmental wall thickening. Venous segment was not excised at surgery, so that the exact histopathological diagnosis was not confirmed. However, it is plausible that inflammation (phlebitis) of this superficial vein cranial to the mass was associated with the development of the IVLCH. However, the presumed relationship between IVLCH and phlebitis is not clear: superficial thrombophlebitis is relatively common but IVLCH has not been reported as a common consequence. Histopathologically, IVLCH appear as a single polypoid mass, attached to the venous wall through a fibrovascular stalk or a broad base. In most cases, a small muscular artery is located near the stalk, as a feeding vessel. The polypoid mass is composed of lobulating capillaries separated by edematous fibromyxoid stroma containing elongated spindle cells with mitotic features. The lobules of capillaries are lined by flattened or rounded endothelial cells presenting mitotic features.2,4 The surface of the tumor is also covered by endothelial cells. In rare cases, IVLCH may contain a component of intravascular papillary endothelial hyperplasia.3–6 Although the exact pathogenesis of IVLCH remains unclear, it is generally considered a benign neoplastic process.7–9 The sonographic and clinical differential diagnoses of IVLCH include primary intravascular neoplasms (eg, intravascular leiomyosarcoma, primary intravascular lymphoma), organizing thrombus, and reactive entities such as intravascular papillary endothelial hyperplasia. Involvement of venous walls and surrounding soft tissues would be evocative of a malignant tumor. Intense hypervascularity within the lesion enabled us to exclude venous thrombosis. Rarely, arterial blood flow can be VOL. 42, NO. 6, JULY/AUGUST 2014
detected in small channels within the venous thrombus, but this feature is mainly associated with tumor or infected thrombus. Moreover, venous thrombosis does not show the nodular pattern with well-delimited and smooth margins associated with marked hypervascularity that was prominent in the present case. Intravascular papillary endothelial hyperplasia (Masson tumor) is a benign vascular lesion formed by papillary proliferation of endothelial cells, which can occur together with organization and recanalization of a thrombus, usually at the hands and fingers. Sonographic and clinical examination do not allow distinguishing IVLCH from Masson tumor.9,10 The diagnosis is based largely on microscopic examination. Masson tumor may occur as a pure (intravascular) form involving a blood vessel, or as a focal change (mixed form) arising in a pre-existing vascular lesion, or in an extravascular, organizing hematoma. The treatment of choice for IVLCH is complete excision of the mass along with a small portion of the vein. The prognosis is excellent, with no tendency for recurrence or dissemination. In conclusion, IVLCH can be suspected when the sonographic examination shows a welldefined intravenous nodular echogenic mass with intense vascularity and no evidence of extravenous extension, especially if the patient presents or reports an asymptomatic soft tissue mass.
REFERENCES 1. Ghersin E, Nitecki S, Brook OR, et al. Intraluminal pyogenic granuloma of the basilic vein: color duplex sonographic manifestations. J Ultrasound Med 2004;23:443. 2. Ghekiere O, Galant C, Vande Berg B, et al. Intravenous pyogenic granuloma or intravenous lobular capillary hemangioma. Skeletal Radiol 2005;34: 343. 3. Cooper PH, McAllister HA, Helwig EB. Intravenous pyogenic granuloma. A study of 18 cases. Am J Surg Pathol 1979;3:221. 4. Maddison A, Tew K, Orell S. Intravenous lobular capillary haemangioma: ultrasound and histology findings. Australas Radiol 2006;50: 186. 5. Inaloz HS, Patel G, Knight AG. Recurrent intravascular papillary endothelial hyperplasia developing from a pyogenic granuloma. J Eur Acad Dermatol Venereol 2001;15:156. 6. Pantanowitz L, Duke WH. Intravascular lesions of the hand. Diagn Pathol 2008;30:24.
377
AHN ET AL 7. 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. 8. Nichols GE, Gaffey MJ, Mills SE, et al. Lobular capillary heamangioma. An immunohistochemical study including steroid hormone receptor status. Am J Clin Pathol 1992;97:770.
378
9. Lysyy O, Schwartz I, Kolander Y, et al. Sonographic features of intravascular papillary endothelial hyperplasia (Masson’s Tumor) in the forearm. J Clin Ultrasound 2011;39:301. 10. Lee SJ, Choo HJ, Park JS, et al. Imaging finding of intravascular papillary endothelial hyperplasia presenting in extremities: correlation with pathological findings. Skeletal Radiol 2010;39: 783.
JOURNAL OF CLINICAL ULTRASOUND
Intravenous Lobular Capillary Hemangioma Occurring in the Cephalic Vein of a 39-year-old Adult Sung Eun Ahn, MD,1 Ji Seon Park, MD,1 Kyung Nam Ryu, MD,1 Wook Jin, MD,2 So Young Park, MD2 1
Department of Radiology, Kyung Hee University Hospital, South Korea Department of Radiology, Kyung Hee University Hospital at Gangdong, School of Medicine, Kyung Hee University, South Korea
2
Received 18 March 2013; accepted 10 September 2013
ABSTRACT: Intravenous lobular capillary hemangiomas are extremely uncommon and mostly occur in the veins of the neck and upper extremities. Here, we report the clinical and sonographic features of an intravenous lobular capillary hemangioma localized in the right cephalic vein, and we discuss its pathoC logic findings and differential diagnoses. V 2013 Wiley Periodicals, Inc. J Clin Ultrasound 42:375–378, 2014; Published online in Wiley Online Library (wileyonlinelibrary.com). DOI: 10.1002/jcu.22110 Keywords: cephalic vein; intravenous lobular capillary hemangioma; intravenous pyogenic granuloma; ultrasonography
L
obular capillary hemangiomas are relatively common acquired vascular neoplasms, occurring mostly on cutaneous and mucosal surfaces, especially of the oral cavity.1,2 Intravenous lobular capillary hemangiomas (IVLCH) are rare variants of lobular capillary hemangioma. To date, there are only a few case reports of ultrasound findings of IVLCH. We report a histologically proven case of IVLCH localized in the right cephalic vein and discuss the clinical and sonographic features, pathologic findings, and differential diagnoses of IVLCH.
CASE REPORT
A 39-year-old woman presented with a painless soft mass of the dorsoradial aspect of the right Correspondence to: J. S. Park C 2013 Wiley Periodicals, Inc. V
VOL. 42, NO. 6, JULY/AUGUST 2014
distal forearm that had appeared spontaneously 2 months earlier. On physical examination, there was neither tenderness nor skin change. She had no prior history of drug abuse, intravenous catheter insertion, or trauma of the right upper extremity. Sonography was performed using an IU 22 system (Philips Medical Systems, Bothell, WA) with a broadband high-frequency linear transducer (L17–5). On gray-scale sonography, a well-defined nodular intraluminal mass was found dilating the cephalic vein (Figure 1A). The size of the mass was approximately 1.4 3 0.8 3 1.8 cm, and the lesion was hyperechoic with small hypoechoic areas within it. There was no evidence of any extravascular component; the venous wall was preserved, and the surrounding soft tissues maintained their integrity (Figure 1B). Color Doppler imaging showed intense hypervascularization within the lesion (Figure 1C), which adhered to the venous wall and was surrounded by blood flow. The lesion was incompressible. A superficial vein about 10 cm in length, cranially apart from the main mass, showed segmental wall-thickening, suggesting associated phlebitis (Figure 1D). The distal part of this superficial vein was intact. On the basis of these findings, we diagnosed an intravenous benign tumor and decided its surgical removal. The patient underwent surgical excision of a 3-cm-long vascular segment containing the mass. At macroscopic inspection, an intraluminal polypoid well-circumscribed mass with regular contours was found, in continuity with the venous wall. 375
AHN ET AL
FIGURE 1. (A) Longitudinal gray-scale sonogram of the right cephalic vein showing a well-defined intravenous nodular echogenic mass. (B) Axial sonographic scan showing small hypoechogenic areas (arrow) within the lesion. (C) Color Doppler imaging showing hypervascularization within the entire mass. (D) Longitudinal section showing segmental wass thickening (arrows) along a superficial vein, about 10 cm cranially from the mass.
At histologic examination, the lesion was composed of a lobulated angiomatoid proliferation of small blood vessels that were lined by an endothelium and a stroma containing smooth muscle fibers (Figure 2). The histopathological findings led to the final diagnosis of intravenous lobular capillary hemangioma.
DISCUSSION
Lobular capillary hemangioma is a relatively commonly acquired vascular neoplasm, which occurs mainly on cutaneous and mucosal surfaces, where ulceration and suppuration may occur. Lobular capillary hemangiomas were previously thought to occur reactively to trauma or pyogenic infection, hence were formerly known as “pyogenic granulomas.”1,2
IVLCH is a rare variant of lobular capillary hemangioma and occurs mostly in the veins of the neck and upper extremities. It was first described in 1979 and, to date, only a few case reports of ultrasound findings of this lesion have been published.3 The average age of presentation is 38 years (range 15–66 years) and female patients are affected slightly more frequently than male patients. IVLCH do not present with characteristic clinical features ensuring its diagnosis. Ghekiere et al2 noted that, according to the literature, the clinical history and physical findings were similar in all patients presenting with an asymptomatic mass. Our case also presented as a palpable mass without any other symptoms. The sonographic manifestations of IVLCH, as shown in this case, are a well-defined echogenic nodular intraluminal mass, with small
FIGURE 2. (A) Low-power microscope photogram of the lesion showing an intravascular nodule, attached to the venous wall (H&E stain, 312.5). (B) High-power view showing that the tumor was composed of a lobulated angiomatoid proliferation of small blood vessels lined by an endothelium and a stroma containing smooth muscle fibers (H&E stain, 3200).
376
JOURNAL OF CLINICAL ULTRASOUND
INTRAVENOUS LOBULAR CAPILLARY HEMANGIOMA
hypoechogenic internal areas. When compared with histopathological findings, the echogenic nodular intraluminal mass is thought to be lobulating capillaries and the hypoechogenic areas are thought to be edematous fibromyxoid stroma. There was no extravascular extension and the venous wall was preserved. We did not record spectral Doppler signals in our case, but Maddison et al4 demonstrated typically arterial waveforms within the mass, contrasting with the typically venous flow modulation of the vein downstream from the lesion, which allows the diagnosis of intravascular soft tissue neoplasm while excluding a resolving thrombus or an arteriovenous fistula. In our case, the superficial vein located cranially from the mass showed segmental wall thickening. Venous segment was not excised at surgery, so that the exact histopathological diagnosis was not confirmed. However, it is plausible that inflammation (phlebitis) of this superficial vein cranial to the mass was associated with the development of the IVLCH. However, the presumed relationship between IVLCH and phlebitis is not clear: superficial thrombophlebitis is relatively common but IVLCH has not been reported as a common consequence. Histopathologically, IVLCH appear as a single polypoid mass, attached to the venous wall through a fibrovascular stalk or a broad base. In most cases, a small muscular artery is located near the stalk, as a feeding vessel. The polypoid mass is composed of lobulating capillaries separated by edematous fibromyxoid stroma containing elongated spindle cells with mitotic features. The lobules of capillaries are lined by flattened or rounded endothelial cells presenting mitotic features.2,4 The surface of the tumor is also covered by endothelial cells. In rare cases, IVLCH may contain a component of intravascular papillary endothelial hyperplasia.3–6 Although the exact pathogenesis of IVLCH remains unclear, it is generally considered a benign neoplastic process.7–9 The sonographic and clinical differential diagnoses of IVLCH include primary intravascular neoplasms (eg, intravascular leiomyosarcoma, primary intravascular lymphoma), organizing thrombus, and reactive entities such as intravascular papillary endothelial hyperplasia. Involvement of venous walls and surrounding soft tissues would be evocative of a malignant tumor. Intense hypervascularity within the lesion enabled us to exclude venous thrombosis. Rarely, arterial blood flow can be VOL. 42, NO. 6, JULY/AUGUST 2014
detected in small channels within the venous thrombus, but this feature is mainly associated with tumor or infected thrombus. Moreover, venous thrombosis does not show the nodular pattern with well-delimited and smooth margins associated with marked hypervascularity that was prominent in the present case. Intravascular papillary endothelial hyperplasia (Masson tumor) is a benign vascular lesion formed by papillary proliferation of endothelial cells, which can occur together with organization and recanalization of a thrombus, usually at the hands and fingers. Sonographic and clinical examination do not allow distinguishing IVLCH from Masson tumor.9,10 The diagnosis is based largely on microscopic examination. Masson tumor may occur as a pure (intravascular) form involving a blood vessel, or as a focal change (mixed form) arising in a pre-existing vascular lesion, or in an extravascular, organizing hematoma. The treatment of choice for IVLCH is complete excision of the mass along with a small portion of the vein. The prognosis is excellent, with no tendency for recurrence or dissemination. In conclusion, IVLCH can be suspected when the sonographic examination shows a welldefined intravenous nodular echogenic mass with intense vascularity and no evidence of extravenous extension, especially if the patient presents or reports an asymptomatic soft tissue mass.
REFERENCES 1. Ghersin E, Nitecki S, Brook OR, et al. Intraluminal pyogenic granuloma of the basilic vein: color duplex sonographic manifestations. J Ultrasound Med 2004;23:443. 2. Ghekiere O, Galant C, Vande Berg B, et al. Intravenous pyogenic granuloma or intravenous lobular capillary hemangioma. Skeletal Radiol 2005;34: 343. 3. Cooper PH, McAllister HA, Helwig EB. Intravenous pyogenic granuloma. A study of 18 cases. Am J Surg Pathol 1979;3:221. 4. Maddison A, Tew K, Orell S. Intravenous lobular capillary haemangioma: ultrasound and histology findings. Australas Radiol 2006;50: 186. 5. Inaloz HS, Patel G, Knight AG. Recurrent intravascular papillary endothelial hyperplasia developing from a pyogenic granuloma. J Eur Acad Dermatol Venereol 2001;15:156. 6. Pantanowitz L, Duke WH. Intravascular lesions of the hand. Diagn Pathol 2008;30:24.
377
AHN ET AL 7. 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. 8. Nichols GE, Gaffey MJ, Mills SE, et al. Lobular capillary heamangioma. An immunohistochemical study including steroid hormone receptor status. Am J Clin Pathol 1992;97:770.
378
9. Lysyy O, Schwartz I, Kolander Y, et al. Sonographic features of intravascular papillary endothelial hyperplasia (Masson’s Tumor) in the forearm. J Clin Ultrasound 2011;39:301. 10. Lee SJ, Choo HJ, Park JS, et al. Imaging finding of intravascular papillary endothelial hyperplasia presenting in extremities: correlation with pathological findings. Skeletal Radiol 2010;39: 783.
JOURNAL OF CLINICAL ULTRASOUND
