Skeletal Radiol (2005) 34:343–346 DOI 10.1007/s00256-004-0860-0
Olivier Ghekiere Christine Galant Bruno Vande Berg
Received: 11 June 2004 Revised: 18 August 2004 Accepted: 18 August 2004 Published online: 24 November 2004 ISS 2004 O. Ghekiere · B. Vande Berg ()) Department of Radiology, Cliniques Universitaires St. Luc, Avenue Hippocrate 10, 1200 Brussels, Belgium e-mail:
[email protected] Tel.: +32-2-7642944 Fax: +32-2-7705574 C. Galant Department of Pathology, Cliniques Universitaires St. Luc, Avenue Hippocrate 10, 1200 Brussels, Belgium
CASE REPORT
Intravenous pyogenic granuloma or intravenous lobular capillary hemangioma
Abstract Lobular capillary hemangioma is a vascular neoplasm that commonly occurs as a cutaneous tumor. When it involves the skin and mucosal surfaces, ulceration and suppuration may occur, hence the classic term of pyogenic granuloma. Intravenous pyogenic granuloma is a rare solitary form of lobular capillary hemangioma that usually occurs in the veins of the neck and upper extremities. We report the ultrasonographic and magnetic resonance imaging findings of a pyogenic intravenous granuloma localized in the right cephalic vein. The imaging and
pathological findings and the differential diagnoses are discussed. Keywords Cephalic vein · Intravenous pyogenic granuloma · Ultrasound · Magnetic resonance imaging
Introduction
Case report
Lobular capillary hemangioma (LCH) is one of the most common acquired vascular neoplasms. It occurs commonly as a cutaneous tumor, although it also occurs on mucosal membranes, especially of the oral cavity [1]. When it involves the skin and mucosal surfaces, ulceration and suppuration may occur, hence the classic term of pyogenic granuloma [2]. Intravenous pyogenic granuloma (IVPG) is a rare solitary form of LCH and occurs usually in the veins of the neck and upper extremities [3, 4, 5, 6, 7]. IVPG is seen in adults, with a slight female predominance [3]. The clinical features are not characteristic enough to suggest the diagnosis [3, 8, 9]. We present a histologically proven case of IVPG localized in the right cephalic vein, and we discuss the sonographic, magnetic resonance imaging (MRI) and pathological findings.
A 50-year-old asymptomatic man presented with a focal swelling of the anterior aspect of the right arm that had appeared spontaneously a few weeks earlier. On physical examination, a small mass was palpable just above the right elbow. There was no skin change. Moderate pain was elicited at palpation. His medical history was unremarkable without history of drug abuse or trauma. Sonography was performed with an HDI 5000 unit (Philips Medical System, Best, The Netherlands) with a broad band frequency linear-array transducer (L12–5). A well-defined nodular mass was found that involved the cephalic vein (Fig. 1A). The lesion had a length of 17 mm and a diameter of 10 mm. The lesion was echogenic with small hypoechogenic areas within it. The lesion was adherent to the wall of the vein without blood flow around it, and it was not compressible. Color Doppler imaging showed hypervascularization within the lesion (Fig. 1B). Magnetic resonance imaging (MRI) of the right elbow was performed for further evaluation with a 1.5 T unit (Philips Medical System). Coronal (TR/TE;1299/100) and sagittal T2-weighted (TR/ TE;4016/100) fast spin-echo (FSE) images and transverse (TR/ TE;663/13) and sagittal (TR/TE;709/13) T1-weighted spin-echo (SE) images before and after contrast material injection (gadoterate meglumine, Guerbet, Aulnay-sous-Bois, France) were obtained. On
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Fig. 3 Coronal T2-weighted FSE image (TR/TE;1299/100) shows a high signal intensity mass that contains small central punctate areas of intermediate signal intensity equivalent of that of fat (arrow). The longitudinal axis of the lesion is parallel to that of the cephalic vein Fig. 1A, B Ultrasound examination. A Longitudinal ultrasound scan of the cephalic vein shows a well-defined nodular echogenic mass (arrow) with small hypoechogenic areas within it. B Axial color Doppler imaging shows hypervascularization within the lesion (arrow) MRI, a well-circumscribed nodular mass involving the right cephalic vein was seen. Adjacent fat planes and muscles were normal. On T2-weighted images, the lesion presented a high signal intensity with central spiculated areas of intermediate signal intensity
Fig. 2A—C Magnetic resonance images. A Sagittal T2-weighted FSE image (TR/TE; 4016/100) shows a high signal intensity mass with central spiculated areas of intermediate signal intensity (arrow). B Sagittal T1-weighted SE image (TR/TE; 709/13) shows that the signal of the lesion (arrow) is equivalent of that of adjacent
(Fig. 2A). The mass was delineated by a thin rim of low signal intensity (Fig. 3). On T1-weighted images, the signal of the lesion was equivalent of that of adjacent muscles (Fig. 2B). On T1weighted images obtained after intravenous injection of gadolinium, there was heterogeneous enhancement of the lesion signal intensity (Fig. 2C). The patient underwent surgical excision of a vascular segment of 2 cm containing the mass. At macroscopic inspection, an intraluminal polypoid well-circumscribed mass of 127 mm with reg-
muscles. C Sagittal T1-weighted SE image obtained after intravenous injection of gadolinium (TR/TE; 709/13) shows heterogeneous enhancement of the lesion with small punctate areas of low signal intensity (arrow)
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Fig. 4 A Low-power photograph of the lesion shows an intravascular nodule (arrow). B High-power view shows a tumor composed of lobulated capillaries and lined by endothelial cells containing mitotic figures (arrow) and supported by fibrous stroma. Some larger vascular structures are situated in the center of the lesion (H and E, 75) ular contours was found. It was in continuity with the venous wall. At histological examination, the lesion was composed of lobulated capillaries lined by endothelial cells containing mitotic figures and supported by fibrous stroma. Few prominent vascular structures were situated in the center of the lesion (Fig. 4). The histopathological findings were diagnostic for intravenous LCH or pyogenic granuloma.
Discussion LCH is one of the most common acquired vascular neoplasms. It occurs commonly as a fleshy cutaneous tumor, although it also occurs on mucosal membranes, especially of the oral cavity [1]. When it involves the skin and mucosal surfaces, ulceration and suppuration may occur, hence the classic term of pyogenic granuloma [2]. IVPG is a rare solitary form of LCH, first described in 1979 by Cooper et al. [3] It most frequently involves the veins of
the neck and upper extremities [3, 4, 5, 6, 7]. IVPG is seen from 15 to 66 years of age with an average age of presentation of 38 years and with a slight female predominance [3]. The clinical features are not characteristic enough to suggest the diagnosis, although we note that the history and physical findings were similar in all patients with a usually asymptomatic mass [3, 8, 9]. This solid tumor presents a polypoid form and is composed of epithelioidappearing endothelial cells [6, 7]. It represents a variant of the more common pyogenic granuloma of the extremities in which the capillary proliferation is entirely confined within a vein lumen [3, 5]. At ultrasound, the lesion was moderately echogenic, with small hypoechogenic areas within it. Presence of hypervascularity within the lesion permitted exclusion of venous thrombosis. A thrombus may show central channels of hypervascularity at the subacute stage, but does not show the same nodular pattern with well-delimited and smooth margins as in the current case. On MRI, the lesion was isointense to muscles on T1-weighted images and isointense to the vein content on T2-weighted images. It was delineated by a rim of low signal intensity. Heterogeneous enhancement was noted after gadolinium injection with small central areas of low signal intensity. The most striking feature was the intravascular topography of the lesion, which suggested a vascular tumor. Differential diagnoses should include venous thrombosis, venous aneurysm of the forearm, or any other vascular tumor. Venous thrombosis usually shows a filling defect or absence of flow within a vessel and is hypointense to muscle on T2-weighted images, mainly on gradient-recalled-echo MR images. Venous aneurysm of the forearm has a mildly hyperintense signal relative to muscle on T1-weighted images and a heterogeneous signal intensity within the mass on T2-weighted FSE images [10, 11, 12]. The possibility of a peripheral nerve sheath tumor should also be suspected because they may develop near vascular structures. However, in the current case the endovascular topography excluded that consideration. At histological examination, two different patterns of IVPG have been described: the classic uncomplicated lobular pattern of capillaries with mostly small lumina and the edematous pattern with stromal infiltration by inflammatory cells and dilated vessels [3]. The typical feature of IVPG includes an intraluminal polyp attached to the vein wall and composed of lobules of capillaries lined by flattened or rounded endothelial cells. There is an edematous fibromyxoid stroma containing elongated spindle cells with numerous mitotic figures. Vessel dilatation can be variable [3, 5, 8]. IVPG should be differentiated from other intravascular lesions including thrombus, intravascular papillary endothelial hyperplasia (IPEH), intravenous atypical vascular proliferation (IAVP), angiosarcoma, angiolymphoid hyperplasia with
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eosinophilia and histiocytoid hemangiomas [6, 9]. A thrombus can be distinguished from IVPG by the presence of the different stages of blood clot organization with prominent fibrin deposits, the relatively paucity of capillaries, and the lack of lobular arrangement of the intraluminal growth [4, 9]. IPEH is characterized by a complex papillary structure and frequently associated with hemosiderin pigment and organizing thrombi, in contrast to IVPG [4]. IAVP can be distinguished by the greater cellularity and atypia of the proliferating endothelial cells [13]. Angiosarcoma does not show a lobular
pattern, is usually seen in an older age group, and does not frequently involve the neck, arm or hand. It typically exhibits nuclear hyperchromasia, pleomorphism and numerous mitoses, which were absent in our case [3]. Angiolymphoid hyperplasia with eosinophilia and histiocytoid hemangiomas differ by the presence of prominent vascular channels lined by histiocytoid endothelial cells [6]. The treatment of choice is complete local excision with a small portion of the vein. The prognosis is excellent [3, 5].
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