ANGIOMYXOMA OF THE FOOT

Clinical Cases 17.02.2006
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Section: Musculoskeletal system
Case Type: Clinical Cases
Patient: 31 years, female
Authors: Fregolino A, Quartieri S, Cremona A, Spina N, Scavone G, Argento G. Azienda Ospedaliera Sant'Andrea Università di Roma "La Sapienza" II Facoltà di Medicina e Chirurgia Via di Grottarossa, 1035-00189 Roma
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AI Report

Clinical History

A 31 y.o. woman presented a swelling on the plantar aspect of the 4th inter-metatarsal space. There was no history of direct trauma; she had first noticed this swelling about two months before. There was neither pain nor difficulty in movement. She was puerperal of three months.

Imaging Findings

A 31 y.o. woman presented a swelling in the distal end of the 4th inter-metatarsal space. Plain films didn’t show the presence of calcifications; there was initial bone involvement with a single extrinsic erosion and periostal reaction. Ultrasound examination of the soft-tissue mass revealed a solid hypo-echoic mass, clearly distinguished from normal structures. Color Doppler US images showed intense vascularization around and intra-lesion. In the MR examination T1-weighted spin-echo (SE), PD-weighted turbo-spin-echo (TSE), T2-weighted gradient echo (GE) and GE-STIR sequences were acquired, with scans on the axial, coronal and sagittal planes, followed by fat-sat T1-w post-contrast acquisitions. The solid mass was 3,0 x 2,4 x 2,4 mm in size; it extended from the 4th intercapitometatarsal space and involved the distal phalanx of the 4th finger. The lesion presented a polilobate border and appeared hyperintense in the high contrast sequences and with intense post-contrast enhancement. Histological examination stated it was an aggressive angiomyxoma with leiomiomatosis aspects.

Discussion

Angiomyxoma (AM) is a rare benign soft-tissue tumor characterised by its myxoid and vascular components. Its compliant nature tends to grow to large size, displacing the adjacent structures, even if the tumor is usually locally infiltrated. AM is considered an aggressive neoplasm as it frequently tends to recur locally if not completely excised. But, due to the bulky and locally infiltrating nature of this tumor, a complete surgical excision is considered technically difficult. Having done the gross pathologic examination, AM appears as a large bulky and gelatinous mass, partially or completely encapsulated. Histologically, AM consists of spindle or stellate cells separated by a myxoid stroma with a large amount of fibroblasts, myofibroblasts, and variably sized vessels. Moreover, mitotic activity has been shown to be rare in the majority of cases. To summarise, surgical excision is the primary treatment for AM: although the tumor is histologically benign, locally aggressive behaviour and high recurrence rates require complete excision. Furthermore, as patients with AM demonstrate a low mitotic activity, radiation therapy or chemiotherapy is unlikely to have effect. What is highly recommended is a long-term follow-up with CT and, preferably, MR imaging, given that recurrences have been reported several years after first excision. The characteristics of angiomyxoma at the MR examination are: the tumor is isointense related to muscle on T1-weighted image, hyperintense on T2-w image and enhanced after gadolinium contrast with a typical internal pattern. The differential diagnosis includes myxoma, angiolipoma, lipoma and hemangioma. Soft-tissue lipoma demonstrate bright signal intensity on T1-w images and do not increase in signal intensity on T2 or fat-suppressed T2-w fast spin-echo sequences. On STIR images, the fat signal in these lesions is nulled. Lipoma do not show enhancement after intravenous injection of a paramagnetic material. Hemangiomas range from the cavernous type to the capillary type. Intra-muscular hemangiomas is associated with variable amounts of fat, smooth muscle, myxoid stroma, and hemosiderin. Hemangiomas demonstrate low to intermediate signal intensity on T1-w images and bright signal intensity on T2-w images. Because of paramagnetic effects, central haemorrhage with hemosiderin deposits or peripheral hemosiderin-laden macrophages demonstrate low signal intensity on T1 and T2-w images. Mixoma is poorly delimitated in the muscle. It demonstrate low intensity on T1-w images and high intensity on T2-w images, like to a cystic formation. Angiolipoma has an heterogeneous pattern on T1-w and T2-w images.

Differential Diagnosis List

Angiomyxoma of the foot

Final Diagnosis

Angiomyxoma of the foot

Liscense

Figures

The mass extended from the distal end of the 4th inter-metatarsal space and involved the proximal phalanx of the 4th toe, with an initial bone involvement with a single extrinsic erosion and periostal reaction.

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The mass extended from the distal end of the 4th inter-metatarsal space and involved the proximal phalanx of the 4th toe, with an initial bone involvement with a single extrinsic erosion and periostal reaction.

US: A solid mass with ipoechogenic echostructure and polilobate border ,clearly distinguished by normal structures. Color Doppler US images showed intense vascularization around and intralesion.

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US: A solid mass with ipoechogenic echostructure and polilobate border ,clearly distinguished by normal structures. Color Doppler US images showed intense vascularization around and intralesion.

MRI sequences T1 FS pre-contrast, performed in the axial plane.

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MRI sequences T1 FS pre-contrast, performed in the axial plane.

MRI sequences T1 FS post-contrast, performed in the axial plane.

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MRI sequences T1 FS post-contrast, performed in the axial plane.

MRI sequences STIR performed on the coronal planes.

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MRI sequences STIR performed on the coronal planes.

MRI sequences STIR performed on the sagittal planes. AM appeares contigous with bones and tendinous structures.

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MRI sequences STIR performed on the sagittal planes. AM appeares contigous with bones and tendinous structures.