Eur J Orthop Surg Traumatol (2009) 19:213–216 DOI 10.1007/s00590-008-0407-5
C A S E RE P O RT
Intramuscular hemangioma presenting with periosteal reaction: report of two cases and review Dhananjaya Sabat · Vinod Kumar · Ajay Gupta
Received: 27 July 2008 / Accepted: 5 November 2008 / Published online: 23 November 2008 © Springer-Verlag 2008
Abstract We describe two cases of intramuscular hemangioma who presented with periosteal reaction on radiographs. One of the patients had extensive involvement of Wbula and part of tibia whereas the other had localized involvement of radius. Both of the cases were diagnosed with MRI and conWrmed histologically. With adequate treatment, both of the patients were relieved of symptoms and remained asymptomatic till the last follow-up. Keywords reaction
Intramuscular · Hemangioma · Periosteal
Introduction Intramuscular hemangioma is a common, benign vascular malformation, usually found in the limbs. When this tumor is located adjacent to the bone, a periosteal reaction may occur mimicking a periosteal or parosteal lesion. We present two cases of histologically proven intramuscular hemangioma that presented with periosteal reaction. The Wrst case presented with extensive periosteal reaction like an aggressive bone tumor and the second presented with a localized periosteal reaction like an osteomyelitis.
D. Sabat (&) · V. Kumar · A. Gupta Department of Orthopaedic Surgery, Maulana Azad Medical College and Associated Lok Nayak Hospital, New Delhi, India e-mail:
[email protected]
Case reports Case report 1 A 22 years female presented with 7-year history of swelling in the lower one-third of right leg. The swelling was slowly increasing in size and was associated with intermittent pain, particularly at night. There was no history of prior trauma or infection. Examination revealed tender, compressible swelling overlying the anterior and lateral aspect of right leg and ankle (Fig. 1a). No local warmth or bruit was noted. All laboratorical studies were normal. Plain radiographs showed irregular periosteal reaction involving whole of the lower two-third of Wbula and medial cortex of lower third of tibia along with erosions (Fig. 1b). MRI and MR angiography of right leg showed heterogenous lesion showing multiple tortuous, serpiginous vessels involving the muscles of anterolateral compartment of right leg. The lesion was abutting almost the entire shaft of Wbula and lower end of tibia. The vascular channels were seen to be draining into a prominent anterior tibial vein. The anterior tibial artery was also prominent (Fig. 1c). FNAC was done which showed paucicellular smears, predominantly composed of neutrophils, lymphocytes, and occasional eosinophils. Few scattered spindle cells were appreciated with a background of fresh RBC’s suggestive of a hemangioma. As the lesion was a large one, intralesional sclerotherapy was done along with pressure garment application. After four session of sclerotherapy, there was considerable reduction in size of the swelling as well as in pain. After 3 years of follow-up, there is minimal pain persisting with no increase in symptoms or size of the lesion.
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Fig. 1 a Clinical swelling in left leg, b radiographs showing periosteal reaction, c MR angiography showing prominent anterior tibial artery and the hemangioma
Case report 2
Discussion
A 20-year-old female presented with swelling and persistent pain in the wrist of 2 years duration. The swelling and pain had gradually increased over the period. There was no history suggestive of trauma or infection. On physical examination, the swelling was localized to the lower third of forearm on the volar side. There was no warmth or bruit. Pronation was terminally restricted and painful. Plain radiographs showed irregular periosteal reaction of volar surface of distal radius (Fig. 2a). MRI revealed a soft tissue intramuscular tumor in pronator quadratus muscle with inhomogenous low signal intensity on the T1- weighted image, a high signal intensity on the T2-weighted image and enhancement in post-gadolinium images (Fig. 2b). Angiogram showed feeding vessel arising from anterior interosseus artery. The lesion was excised without any bony resection (Fig. 2c). Excision biopsy conWrmed the diagnosis of cavernous hemangioma. Patient was pain free and after 2-year follow up, no evidence of recurrence was seen.
Hemangiomas are benign lesions characterized by vascular spaces lined with endothelial cells. Considerable debate exists as to whether these lesions are neoplasms, hamartomas, or vascular malformations. Most commonly, hemangiomas are localized to a single area, but multiple hemangiomas may occur in a single individual in a process known as hemangiomatosis. Hemangioma usually occurs in the skin, subcutaneous tissue or muscle. Hemangioma of bone and periosteal region, e.g. periosteal [1, 2], subperiosteal [3], surface [4] and surface-based [5, 6] is rare. Hemangiomas of muscle and bone may be asymptomatic or may cause symptoms such as pain and swelling. The etiology of hemangiomas is still unclear. Angiogenesis likely plays a role in the vascular excess present. Cytokines, such as basic Wbroblast growth factor (bFGF) and vascular endothelial growth factor (VEGF), have been implicated in the process of angiogenesis. Excess levels of angiogenic factors such as these or decreased levels of
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Fig. 2 a Radiographs showing periosteal reaction at distal radius, b MRI showing intramuscular tumor in pronator quadratus with inhomogenous low signal intensity on T1, high signal intensity on T2 and enhancement on post-gadolinium images, c tumor after resection
angiogenesis inhibitors such as gamma-interferon, tumor necrosis factor-beta, and transforming growth factor-beta may play a role in the etiology of hemangiomas [7, 8]. Treatment may be considered if pain is substantial, but due to the poor success rate of treatment and the apparent limited natural history of the process, indications for treatment are limited. The more limited the extent of the disease, the more likely it is to be controlled with surgical excision. Similar cases were scarcely reported in literature [9–11]. All reported cases have been found to involve tibia and/or Wbula. Similar presentation in any of the upper limb bones has not been described previously. This condition mimics other periosteal or parosteal lesions such as osteosarcoma, chondrosarcoma, a ganglion, periostitis and chronic osteomyelitis. The plain radiographical Wndings of hemangioma in the periosteal region have been described as cortical thickening or depression, periosteal reaction, a soft tissue mass and osteopenia [12]. In both of our cases, the presence of a soft-tissue mass adjacent to the bone and the characteristic signal intensity on MRI suggested the diagnosis and both cases were proven histologically. Hemangiomas have characteristic MR imaging features that enable their diVerentiation from malignant soft-tissue masses. SpeciWcally, these features are the lobulated and
septate appearance of hemangiomas compared with malignant soft-tissue masses; the central low-intensity dot sign on T2-weighted imaging characteristically seen in hemangiomas; marked enhancement of hemangiomas after gadolinium administration on T1-weighted imaging; and the high T2 signal of hemangiomas compared with that of malignant soft-tissue masses. It is the summation of all these features, rather than any one particular feature taken in isolation, that will help the radiologist to make a diagnosis of hemangioma [13]. Most authors describing treatment of periosteal hemangioma recommend resection en bloc, including the aVected bone, to reduce the incidence of local recurrence [2, 5, 6]. No clear recommendations have been found regarding management of intramuscular hemangioma with periosteal reaction. In the Wrst case, we used sclerotherapy with pressure garments with satisfactory results. In the second we performed a resection of tumor without touching the bone with good results. From the success of treatment in our cases, it can be concluded that this sort of presentation needs nothing more than the conventional management of hemangioma and there is no need of bone resection. ConXict of interest statement this study.
No funds were received in support of
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