Pathology – Research and Practice 210 (2014) 321–324
Contents lists available at ScienceDirect
Pathology – Research and Practice journal homepage: www.elsevier.com/locate/prp
Teaching case
Intraosseous angioleiomyoma the tibia: A case report Sean K. Lau ∗ Department of Pathology, City of Hope National Medical Center, Duarte, CA, United States
a r t i c l e
i n f o
Article history: Received 24 July 2013 Received in revised form 3 December 2013 Accepted 29 January 2014 Keywords: Angioleiomyoma Vascular leiomyoma Angiomyoma Bone
a b s t r a c t Angioleiomyoma (vascular leiomyoma/angiomyoma) is a morphologically distinctive tumor characterized by proliferating smooth muscle cells admixed with prominent vascular elements. The majority of angioleiomyomas involve the superficial soft tissues. Examples of this lesion originating in bone, particularly in the appendicular skeleton, are extremely rare. The present report details the clinicopathologic features of an unusual case of an intraosseous angioleiomyoma arising in the distal tibia. The skeletal tumor exhibited the typical histologic appearance and immunophenotypic features of this entity. Due to its rarity, angioleiomyoma of bone can pose problems in diagnosis. Awareness that angioleiomyoma can present as a primary intraosseous lesion is important so as not to confuse this neoplasm with more commonly encountered bone tumors. © 2014 Elsevier GmbH. All rights reserved.
Introduction Angioleiomyoma, also known as vascular leiomyoma or angiomyoma, is a histologically distinctive, benign neoplasm composed of smooth muscle and prominent blood vessels [1–4]. The tumor is relatively common, occurring predominantly in the superficial soft tissues of the extremities [1–4]. Primary angioleiomyoma of the bone is rare; only a few cases have been reported, the majority of which have arisen in the gnathic bones [3,5–14]. Intraosseous angioleiomyomas originating in the appendicular skeleton are exceedingly uncommon [15–17]. Their rarity at this particular site and unusual morphology may lead to diagnostic difficulties. In order to increase recognition and further understanding of the pathologic features of this tumor, and unusual case of an intraosseous angioleiomyoma involving the distal tibia is described herein.
Case report A 48-year-old female presented with a chief complaint of swelling and discomfort of the posterior medial aspect of the left ankle following heavy physical exercise. On physical examination the left ankle had some minimal edema with mild
∗ Correspondence to: Kaiser Permanente Orange County-Anaheim Medical Center, Department of Pathology, 3440 East La Palma Avenue, Anaheim, CA 92806, United States. Tel.: +1 714 644 6160; fax: +1 714 644 6161. E-mail address: [email protected] http://dx.doi.org/10.1016/j.prp.2014.01.010 0344-0338/© 2014 Elsevier GmbH. All rights reserved.
tenderness on deep palpation of the medial malleolus. Radiograph showed a 2.4 cm expansile lytic lesion involving the subarticular distal posterior tibia (Fig. 1A). The lesion was well circumscribed with some cortical thinning, but no cortical breakthrough or pathologic fracture. No extension into the joint space was observed. On magnetic resonance imaging, the lesion showed an isointense signal on T1 weighted images and slightly heterogeneous high signal intensity on T2 weighted images. Following a diagnostic incisional biopsy, curettage and allografting of the lesion was performed. At the time of surgery, the lesion was noted to be bloody with a firm rubbery consistency. The patient is without evidence of recurrent disease four years after surgery.
Materials and methods The excised tissue was fixed in 10% neutral buffered formalin. Paraffin sections were stained with hematoxylin and eosin for routine histology. Immunohistochemical staining was performed on paraffin embedded tissue using antibodies directed against the following: actin (clone HHF35, dilution 1:75, Dako, Carpinteria, CA), smooth muscle actin (clone 1A4, dilution 1:500, Dako), desmin (clone D33, dilution 1:300, Dako), keratin (OSCAR, dilution 1:150, Covance, Emeryville, CA), S-100 (polyclonal, dilution 1:3000, Dako), and CD34 (QBEnd10, Dako). Antigen retrieval was performed by heating slides in the PT Link module (Dako). Staining was performed using an automated immunostainer (Autostainer Link 48, Dako), followed by antibody detection using the EnVision FLEX
322
S.K. Lau / Pathology – Research and Practice 210 (2014) 321–324
Fig. 1. (A) Plain radiograph displaying a solitary, well defined, radiolucent lesion involving the distal tibia. (B) Intraosseous tumor composed of prominent blood vessels and intervening spindle cells (H&E, original magnification 100×).
visualization system (Dako). Appropriate positive and negative tissue controls were used throughout. Results Histologically, the tumor was characterized by the presence of numerous predominantly medium to large size thick walled blood vessels with an associated proliferation of oval to spindle-shaped cells. Although well circumscribed radiographically, microscopically in areas the tumor was observed to permeate between preexisting bone trabecula (Fig. 1B). The lesional vessels had small, narrow lumens and many showed mural hyalinization or myxoid change (Fig. 2A). The associated spindle cells were cytologically bland, exhibiting plump ovoid, vesicular nuclei with inconspicuous nucleoli and abundant eosinophilic cytoplasm. In some areas the tumor had a solid appearance, with the spindle cells arranged in a loose storiform pattern or as compact bundles with associated slit like vascular channels (Fig. 2B), while in others, the spindle cells were intimately associated with thick walled vessels (Fig. 2C) or exhibited a concentric orientation around vascular lumina (Fig. 2D). No cellular pleomorphism, mitotic figures, or areas of necrosis were observed. By immunohistochemical analysis, the lesional cells were positive for muscle specific actin and smooth muscle actin. The spindle cells were negative for keratin, desmin, CD34, and S-100 protein. Discussion Angioleiomyoma (vascular leiomyoma/angiomyoma) is a benign neoplasm occurring predominantly in the peripheral subcutaneous tissue, thought to arise from vascular smooth muscle. Skeletal involvement by angioleiomyoma is extremely uncommon [3,5–17]. Most reported examples of intraosseous angioleiomyoma have arisen in the jawbones [3,5–13]. Well documented cases of angioleiomyoma originating from the appendicular skeleton are few. In addition to the present case, to the best of my knowledge only three previous cases of primary intraosseous angioleiomyoma of the pelvic or long bones of the extremities have been described [15–17]. Affected sites have included the iliac crest [16], iliopubic ramus [17], and tibia [15]. The latter documented example of angioleiomyoma involving the tibia was located in the proximal
epimetaphyseal region [17], which differs from the present case occurring in the distal tibial epiphysis. The reported cases of intraosseous angioleiomyoma have exhibited a nearly equal sex distribution and have occurred over a wide age range [5–17]. Clinically, patients typically present with pain, though lesions involving the gnathic bones may be asymptomatic [5–12]. Plain radiographs typically show a radiolucent lesion with well-defined sclerotic margins. The tumor is benign in nature with no reported cases of recurrence or aggressive behavior [5–17]. Microscopically, angioleiomyomas have a characteristic appearance typified by prominent blood vessels with admixed spindleshaped smooth muscle cells. The constituent vasculature may have a thin walled, thick walled, or dilated cavernous type appearance [1–4]. The intervening spindle cells are haphazardly arranged and tend to merge with the smooth muscle cells comprising the walls of the vascular elements. Perivascular concentric growth of cells similar to that observed in myopericytoma may also be present [4,13]. The lesional cells are cytologically bland and generally lack mitotic activity. By immunohistochemistry angioleiomyomas characteristically express muscle specific actin, and smooth muscle associated antigens including smooth muscle actin, calponin, and h-caldesmon [4,13,18,19]. Desmin immunoreactivity tends to be more variable, with previous studies indicating lack of desmin expression in 11–18% of cases [4,13,18,19]. Angioleiomyomas are typically negative for keratins, CD31, CD34, and S-100 protein. The principal considerations in the morphologic differential diagnosis of intraosseous angioleiomyoma would include other more common skeletal vasoformative lesions. The majority of vascular tumors of bone including ordinary hemangiomas, hemangioendotheliomas, epithelioid hemangiomas, and epithelioid hemangioendotheliomas lack a prominent spindle cell component and are easily discernible from angioleiomyomas [20–22]. Important exceptions would include vascular lesions described as hemorrhagic epithelioid and spindle cell hemangioma [23,24] and spindle cell hemangioma [25] of bone. Hemorrhagic epithelioid and spindle cell hemangioma bears some morphologic resemblance to angioleiomyoma due to the presence of well-formed vessels of varying size and cytologically banal spindle cells with a loose fascicular architecture [23,24]. Angioleiomyomas however lack the conspicuous epithelioid cell component which is characteristic of hemorrhagic epithelioid and spindle cell hemangioma. Intraosseous spindle cell hemangioma is morphologically similar to its soft tissue counterpart and is characterized by cavernous vascular spaces with intermixed cellular areas of spindle cells [25]. The tumor often exhibits epithelioid cytology characterized by round cells with intracytoplasmic vacuoles, a feature which is useful for differentiating spindle cell hemangioma from angioleiomyoma. Angioleiomyoma should also be distinguished from myopericytoma, a tumor showing differentiation toward perivascular myoid cells. Similar to angioleiomyoma, myopericytomas generally arise in the dermis or subcutis, though rare examples of myopericytoma affecting the skeletal system, in particular the spinal vertebra, have been described [26–28]. Myopericytomas are composed predominantly of oval to spindle-shaped cells displaying a characteristic concentric perivascular pattern of growth [29,30]. The tumor is morphologically heterogeneous and can also exhibit areas resembling myofibroma, infantile hemangiopericytoma, and glomus tumor, as well as angioleiomyoma [4,13,29,30]. The distinction of angioleiomyoma from myopericytoma can be difficult; myopericytomas may show elongated spindle-shaped tumor cells resembling smooth muscle [4,13,29,30], while a concentric perivascular growth pattern, such as that observed in the present case, is a not uncommon feature of angioleiomyomas [4,13]. The two tumors also share a common immunophenotype, exhibiting
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Fig. 2. (A) Thick walled vessels with mural hyalinization (H&E, original magnification 200×). (B) Spindle cell component of tumor with a solid growth pattern and slit like vascular spaces. (H&E, original magnification 200×). (C) Vessels with thick muscular walls and intervening spindle cells. (H&E, original magnification 200×). (D) Concentric perivascular proliferation of spindle cells resembling myopericytoma (H&E, original magnification 200×).
positive immunoreactivity for muscle specific actin, smooth muscle actin, and h-caldesmon, though angioleiomyoma is more frequently positive for desmin, while myopericytoma is typically negative or only focally positive for this marker [4,13,29,30]. The histologic and immunophenotypic similarities between myopericytoma and angioleiomyoma have led to the suggestion that these neoplasms form a morphologic continuum and represent a single spectrum of tumors [4,13]. At present, tumors composed predominantly of vascular structures surrounded concentrically by myoid type cells are classified as myopericytoma, while those lesions exhibiting mostly spindle cells with thick walled vessels are recognized as angioleiomyoma [4,13,30]. In summary, although angioleiomyomas are relatively common in soft tissues, they are extraordinarily rare in bone, particularly in the skeletal extremities. The microscopic appearance of intraosseous angioleiomyoma is similar to that of its soft tissue counterpart. While the admixture of spindle cells and prominent vascular elements is histologically distinctive, the unusual anatomic location can lead to difficulties in recognition of this lesion. Careful attention to morphologic details, along with immunohistochemistry, can help to distinguish angioleiomyoma from other neoplasms which are more commonly encountered in bone. Intraosseous angioleiomyomas appear to behave in a clinically benign manner and conservative excision is considered appropriate management. References [1] J.T. Duhig, J.P. Ayer, Vascular leiomyoma. A study of sixty one cases, Arch. Pathol. 68 (1959) 424–430. [2] D. Magner, D.P. Hill, Encapsulated angiomyoma of the skin and subcutaneous tissues, Am. J. Clin. Pathol. 35 (1961) 137–141. [3] T. Hachisuga, H. Hashimoto, M. Enjoji, Angioleiomyoma. A clinicopathologic reappraisal of 562 cases, Cancer 54 (1984) 126–130. [4] A. Matsuyama, M. Hisaoka, H. Hashimoto, Angioleiomyoma: a clinicopathologic and immunohistochemical reappraisal with special reference to the correlation with myopericytoma, Hum. Pathol. 38 (2007) 645–651.
[5] R.M. Rhatigan, Z.E. Kim, Leiomyoma arising adjacent to a maxillary tooth socket: an intraosseous leiomyoma presenting as an odontogenic lesion, S. Med. J. 69 (1976) 493–494. [6] L.I. Goldblatt, R.B. Edesess, Central leiomyoma of the mandible, Oral Surg. Oral Med. Oral Pathol. 43 (1977) 591–597. [7] D.K. White, L.R. Selinger, A.S. Miller, M.M. Behr, D.D. Damm, Primary angioleiomyoma of the mandible, J. Oral Maxillofac. Surg. 43 (1985) 640– 644. [8] M.D. McMillan, J.W. Ferguson, T.B. Kardos, Mandibular vascular leiomyoma, Oral Surg. Oral Med. Oral Pathol. 62 (1986) 427–433. [9] A.P. Bhatt, V.R. Brave, Angiomyoma of the mandible: a case report, J. Ind. Dent. Assoc. 61 (1990) 298–299. [10] E.J. Burkes Jr., Vascular leiomyoma of the mandible: report of a case, J. Oral Maxillofac. Surg. 53 (1995) 65–66. [11] J.K. Brooks, N.G. Nikitakis, N.J. Goodman, B.A. Levy, Clinicopathologic characterization of oral angioleiomyomas, Oral Surg. Oral Med. Oral Pathol. Oral Radiol. Endod. 94 (2002) 221–227. [12] L. Suresh, E. Matsumura, L.E. Calixto, E. Ruckert, A. Aguirre, Intraosseous angiomyoma of the mandible, Gen. Dent. 55 (2007) 132–135. [13] F. Ide, K. Mishima, H. Yamada, I. Saito, N. Horie, T. Shimoyama, K. Kusama, Perivascular myoid tumors of the oral region: a clinicopathologic re-evaluation of 35 cases, J. Oral Pathol. Med. 37 (2008) 43–49. [14] M. Vijayasaradhi, S.G. Uppin, V. Sreedhar, C. Sundaram, M.K. Panigrahi, Frontal intradiploic angioleiomyoma, J. Neurosurg. Pediatr. 2 (2008) 266–268. [15] K. Tomoda, K. Iyama, A case of intraosseous angioleiomyoma, Acta. Orthop. Scand. 63 (1992) 568–570. [16] A. Vaillo-Vinagre, C. Ballestin-Carcavilla, S. Madero-Garcia, S. Pastor Garcia, A. Checa Garcia, F.J. Martinez-Tello, Primary angioleiomyoma of the iliac bone: clinical pathological study of one case with flow cytometric DNA content and S-phase fraction analysis, Skeletal Radiol. 29 (2000) 181–185. [17] J.M. Laffosse, A. Gomez-Brouchet, G. Giordano, N. Bonnevialle, J. Puget, Intraosseous leiomyoma: a report of two cases, Joint Bone Spine 74 (2007) 389–392. [18] L. Lundgren, T. Seidal, L.G. Kindblom, L. Angervall, Intermediate and fine filaments of vascular leiomyomas (angiomyoma), leiomyoma and leiomyosarcomas of large veins, APMIS 97 (1989) 637–645. [19] T. Hasegawa, K. Seki, P. Yang, T. Hirose, K. Hizawa, Mechanism of pain and cytoskeletal properties in angioleiomyomas: an immunohistochemical study, Pathol. Int. 44 (1994) 66–72. [20] D.E. Wenger, L.E. Wold, Benign vascular lesions of bone: radiologic and pathologic features, Skeletal Radiol. 29 (2000) 63–74. [21] D.E. Wenger, L.E. Wold, Malignant vascular lesions of bone: radiologic and pathologic features, Skeletal Radiol. 29 (2000) 619–631. [22] H.L. Evans, A.K. Raymond, A.G. Ayala, Vascular tumors of bone: a study of 17 cases other than ordinary hemangioma, with an evaluation of the relationship of hemangioendothelioma of bone to epithelioid hemangioma, epithelioid
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S.K. Lau / Pathology – Research and Practice 210 (2014) 321–324 hemangioendothelioma, and high-grade angiosarcoma, Hum. Pathol. 34 (2003) 680–689. S.B. Keel, A.E. Rosenberg, Hemorrhagic epithelioid and spindle cell hemangioma: a newly recognized, unique vascular tumor of bone, Cancer 85 (1999) 1966–1972. F.M. Maclean, J. Schatz, S.W. McCarthy, R.A. Scolyer, P. Stalley, S.F. Bonar, Epithelioid and spindle cell haemangioma of bone, Skeletal Radiol. 36 (2007) S50– S57. M. Hakozaki, T. Tajino, K. Watanabe, H. Yamada, S. Kikuchi, H. Hojo, T. Ishida, S. Konno, Intraosseous spindle cell hemangioma of the calcaneus: a case report and review of the literature, Ann. Diagn. Pathol. 16 (2012) 369–373. D.P. Cox, L. Giltman, Myopericytoma of the thoracic spine, Spine 28 (2003) E30–E32.
[27] B. Brunschweiler, N. Guedj, T. Lenoir, T. Faillot, L. Rillardon, P. Guigui, Oncogenous osteomalacia and myopericytoma of the thoracic spine, Spine 34 (2009) E857–E860. [28] N. Agrawal, K. Nag, Myopericytoma of the thoracic spine: a case report and review of literature, Spine J. (September) (2013), http://dx.doi.org/10. 1016/j.spinee.2013.06.050, pii: S1529-9430(13)00736-5 (Epub ahead of print). [29] S.R. Granter, K. Badizadegan, C.D. Fletcher, Myofibromatosis in adults, glomangiopericytoma, and myopericytoma: a spectrum of tumors showing perivascular myoid differentiation, Am. J. Surg. Pathol. 22 (1998) 513–525. [30] T. Mentzel, A.P. Dei Tos, Z. Sapi, H. Kutzner, Myopericytoma of skin and soft tissues: clinicopathologic and immunohistochemical study of 54 cases, Am. J. Surg. Pathol. 30 (2006) 104–113.
Contents lists available at ScienceDirect
Pathology – Research and Practice journal homepage: www.elsevier.com/locate/prp
Teaching case
Intraosseous angioleiomyoma the tibia: A case report Sean K. Lau ∗ Department of Pathology, City of Hope National Medical Center, Duarte, CA, United States
a r t i c l e
i n f o
Article history: Received 24 July 2013 Received in revised form 3 December 2013 Accepted 29 January 2014 Keywords: Angioleiomyoma Vascular leiomyoma Angiomyoma Bone
a b s t r a c t Angioleiomyoma (vascular leiomyoma/angiomyoma) is a morphologically distinctive tumor characterized by proliferating smooth muscle cells admixed with prominent vascular elements. The majority of angioleiomyomas involve the superficial soft tissues. Examples of this lesion originating in bone, particularly in the appendicular skeleton, are extremely rare. The present report details the clinicopathologic features of an unusual case of an intraosseous angioleiomyoma arising in the distal tibia. The skeletal tumor exhibited the typical histologic appearance and immunophenotypic features of this entity. Due to its rarity, angioleiomyoma of bone can pose problems in diagnosis. Awareness that angioleiomyoma can present as a primary intraosseous lesion is important so as not to confuse this neoplasm with more commonly encountered bone tumors. © 2014 Elsevier GmbH. All rights reserved.
Introduction Angioleiomyoma, also known as vascular leiomyoma or angiomyoma, is a histologically distinctive, benign neoplasm composed of smooth muscle and prominent blood vessels [1–4]. The tumor is relatively common, occurring predominantly in the superficial soft tissues of the extremities [1–4]. Primary angioleiomyoma of the bone is rare; only a few cases have been reported, the majority of which have arisen in the gnathic bones [3,5–14]. Intraosseous angioleiomyomas originating in the appendicular skeleton are exceedingly uncommon [15–17]. Their rarity at this particular site and unusual morphology may lead to diagnostic difficulties. In order to increase recognition and further understanding of the pathologic features of this tumor, and unusual case of an intraosseous angioleiomyoma involving the distal tibia is described herein.
Case report A 48-year-old female presented with a chief complaint of swelling and discomfort of the posterior medial aspect of the left ankle following heavy physical exercise. On physical examination the left ankle had some minimal edema with mild
∗ Correspondence to: Kaiser Permanente Orange County-Anaheim Medical Center, Department of Pathology, 3440 East La Palma Avenue, Anaheim, CA 92806, United States. Tel.: +1 714 644 6160; fax: +1 714 644 6161. E-mail address: [email protected] http://dx.doi.org/10.1016/j.prp.2014.01.010 0344-0338/© 2014 Elsevier GmbH. All rights reserved.
tenderness on deep palpation of the medial malleolus. Radiograph showed a 2.4 cm expansile lytic lesion involving the subarticular distal posterior tibia (Fig. 1A). The lesion was well circumscribed with some cortical thinning, but no cortical breakthrough or pathologic fracture. No extension into the joint space was observed. On magnetic resonance imaging, the lesion showed an isointense signal on T1 weighted images and slightly heterogeneous high signal intensity on T2 weighted images. Following a diagnostic incisional biopsy, curettage and allografting of the lesion was performed. At the time of surgery, the lesion was noted to be bloody with a firm rubbery consistency. The patient is without evidence of recurrent disease four years after surgery.
Materials and methods The excised tissue was fixed in 10% neutral buffered formalin. Paraffin sections were stained with hematoxylin and eosin for routine histology. Immunohistochemical staining was performed on paraffin embedded tissue using antibodies directed against the following: actin (clone HHF35, dilution 1:75, Dako, Carpinteria, CA), smooth muscle actin (clone 1A4, dilution 1:500, Dako), desmin (clone D33, dilution 1:300, Dako), keratin (OSCAR, dilution 1:150, Covance, Emeryville, CA), S-100 (polyclonal, dilution 1:3000, Dako), and CD34 (QBEnd10, Dako). Antigen retrieval was performed by heating slides in the PT Link module (Dako). Staining was performed using an automated immunostainer (Autostainer Link 48, Dako), followed by antibody detection using the EnVision FLEX
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Fig. 1. (A) Plain radiograph displaying a solitary, well defined, radiolucent lesion involving the distal tibia. (B) Intraosseous tumor composed of prominent blood vessels and intervening spindle cells (H&E, original magnification 100×).
visualization system (Dako). Appropriate positive and negative tissue controls were used throughout. Results Histologically, the tumor was characterized by the presence of numerous predominantly medium to large size thick walled blood vessels with an associated proliferation of oval to spindle-shaped cells. Although well circumscribed radiographically, microscopically in areas the tumor was observed to permeate between preexisting bone trabecula (Fig. 1B). The lesional vessels had small, narrow lumens and many showed mural hyalinization or myxoid change (Fig. 2A). The associated spindle cells were cytologically bland, exhibiting plump ovoid, vesicular nuclei with inconspicuous nucleoli and abundant eosinophilic cytoplasm. In some areas the tumor had a solid appearance, with the spindle cells arranged in a loose storiform pattern or as compact bundles with associated slit like vascular channels (Fig. 2B), while in others, the spindle cells were intimately associated with thick walled vessels (Fig. 2C) or exhibited a concentric orientation around vascular lumina (Fig. 2D). No cellular pleomorphism, mitotic figures, or areas of necrosis were observed. By immunohistochemical analysis, the lesional cells were positive for muscle specific actin and smooth muscle actin. The spindle cells were negative for keratin, desmin, CD34, and S-100 protein. Discussion Angioleiomyoma (vascular leiomyoma/angiomyoma) is a benign neoplasm occurring predominantly in the peripheral subcutaneous tissue, thought to arise from vascular smooth muscle. Skeletal involvement by angioleiomyoma is extremely uncommon [3,5–17]. Most reported examples of intraosseous angioleiomyoma have arisen in the jawbones [3,5–13]. Well documented cases of angioleiomyoma originating from the appendicular skeleton are few. In addition to the present case, to the best of my knowledge only three previous cases of primary intraosseous angioleiomyoma of the pelvic or long bones of the extremities have been described [15–17]. Affected sites have included the iliac crest [16], iliopubic ramus [17], and tibia [15]. The latter documented example of angioleiomyoma involving the tibia was located in the proximal
epimetaphyseal region [17], which differs from the present case occurring in the distal tibial epiphysis. The reported cases of intraosseous angioleiomyoma have exhibited a nearly equal sex distribution and have occurred over a wide age range [5–17]. Clinically, patients typically present with pain, though lesions involving the gnathic bones may be asymptomatic [5–12]. Plain radiographs typically show a radiolucent lesion with well-defined sclerotic margins. The tumor is benign in nature with no reported cases of recurrence or aggressive behavior [5–17]. Microscopically, angioleiomyomas have a characteristic appearance typified by prominent blood vessels with admixed spindleshaped smooth muscle cells. The constituent vasculature may have a thin walled, thick walled, or dilated cavernous type appearance [1–4]. The intervening spindle cells are haphazardly arranged and tend to merge with the smooth muscle cells comprising the walls of the vascular elements. Perivascular concentric growth of cells similar to that observed in myopericytoma may also be present [4,13]. The lesional cells are cytologically bland and generally lack mitotic activity. By immunohistochemistry angioleiomyomas characteristically express muscle specific actin, and smooth muscle associated antigens including smooth muscle actin, calponin, and h-caldesmon [4,13,18,19]. Desmin immunoreactivity tends to be more variable, with previous studies indicating lack of desmin expression in 11–18% of cases [4,13,18,19]. Angioleiomyomas are typically negative for keratins, CD31, CD34, and S-100 protein. The principal considerations in the morphologic differential diagnosis of intraosseous angioleiomyoma would include other more common skeletal vasoformative lesions. The majority of vascular tumors of bone including ordinary hemangiomas, hemangioendotheliomas, epithelioid hemangiomas, and epithelioid hemangioendotheliomas lack a prominent spindle cell component and are easily discernible from angioleiomyomas [20–22]. Important exceptions would include vascular lesions described as hemorrhagic epithelioid and spindle cell hemangioma [23,24] and spindle cell hemangioma [25] of bone. Hemorrhagic epithelioid and spindle cell hemangioma bears some morphologic resemblance to angioleiomyoma due to the presence of well-formed vessels of varying size and cytologically banal spindle cells with a loose fascicular architecture [23,24]. Angioleiomyomas however lack the conspicuous epithelioid cell component which is characteristic of hemorrhagic epithelioid and spindle cell hemangioma. Intraosseous spindle cell hemangioma is morphologically similar to its soft tissue counterpart and is characterized by cavernous vascular spaces with intermixed cellular areas of spindle cells [25]. The tumor often exhibits epithelioid cytology characterized by round cells with intracytoplasmic vacuoles, a feature which is useful for differentiating spindle cell hemangioma from angioleiomyoma. Angioleiomyoma should also be distinguished from myopericytoma, a tumor showing differentiation toward perivascular myoid cells. Similar to angioleiomyoma, myopericytomas generally arise in the dermis or subcutis, though rare examples of myopericytoma affecting the skeletal system, in particular the spinal vertebra, have been described [26–28]. Myopericytomas are composed predominantly of oval to spindle-shaped cells displaying a characteristic concentric perivascular pattern of growth [29,30]. The tumor is morphologically heterogeneous and can also exhibit areas resembling myofibroma, infantile hemangiopericytoma, and glomus tumor, as well as angioleiomyoma [4,13,29,30]. The distinction of angioleiomyoma from myopericytoma can be difficult; myopericytomas may show elongated spindle-shaped tumor cells resembling smooth muscle [4,13,29,30], while a concentric perivascular growth pattern, such as that observed in the present case, is a not uncommon feature of angioleiomyomas [4,13]. The two tumors also share a common immunophenotype, exhibiting
S.K. Lau / Pathology – Research and Practice 210 (2014) 321–324
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Fig. 2. (A) Thick walled vessels with mural hyalinization (H&E, original magnification 200×). (B) Spindle cell component of tumor with a solid growth pattern and slit like vascular spaces. (H&E, original magnification 200×). (C) Vessels with thick muscular walls and intervening spindle cells. (H&E, original magnification 200×). (D) Concentric perivascular proliferation of spindle cells resembling myopericytoma (H&E, original magnification 200×).
positive immunoreactivity for muscle specific actin, smooth muscle actin, and h-caldesmon, though angioleiomyoma is more frequently positive for desmin, while myopericytoma is typically negative or only focally positive for this marker [4,13,29,30]. The histologic and immunophenotypic similarities between myopericytoma and angioleiomyoma have led to the suggestion that these neoplasms form a morphologic continuum and represent a single spectrum of tumors [4,13]. At present, tumors composed predominantly of vascular structures surrounded concentrically by myoid type cells are classified as myopericytoma, while those lesions exhibiting mostly spindle cells with thick walled vessels are recognized as angioleiomyoma [4,13,30]. In summary, although angioleiomyomas are relatively common in soft tissues, they are extraordinarily rare in bone, particularly in the skeletal extremities. The microscopic appearance of intraosseous angioleiomyoma is similar to that of its soft tissue counterpart. While the admixture of spindle cells and prominent vascular elements is histologically distinctive, the unusual anatomic location can lead to difficulties in recognition of this lesion. Careful attention to morphologic details, along with immunohistochemistry, can help to distinguish angioleiomyoma from other neoplasms which are more commonly encountered in bone. Intraosseous angioleiomyomas appear to behave in a clinically benign manner and conservative excision is considered appropriate management. References [1] J.T. Duhig, J.P. Ayer, Vascular leiomyoma. A study of sixty one cases, Arch. Pathol. 68 (1959) 424–430. [2] D. Magner, D.P. Hill, Encapsulated angiomyoma of the skin and subcutaneous tissues, Am. J. Clin. Pathol. 35 (1961) 137–141. [3] T. Hachisuga, H. Hashimoto, M. Enjoji, Angioleiomyoma. A clinicopathologic reappraisal of 562 cases, Cancer 54 (1984) 126–130. [4] A. Matsuyama, M. Hisaoka, H. Hashimoto, Angioleiomyoma: a clinicopathologic and immunohistochemical reappraisal with special reference to the correlation with myopericytoma, Hum. Pathol. 38 (2007) 645–651.
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